Factors associated with bronchiectasis in patients with moderate–severe chronic obstructive pulmonary disease

PubMed Central

Jin, Jianmin; Yu, Wenling; Li, Shuling; Lu, Lijin; Liu, Xiaofang; Sun, Yongchang

2016-01-01

Abstract A high prevalence of bronchiectasis was found by chest computed tomography (CT) in patients with moderate–severe chronic obstructive pulmonary disease (COPD), and it was shown to be associated with more severe symptoms, higher frequency of exacerbations and mortality. The risk factors for bronchiectasis in COPD are not yet clarified. High-resolution computed tomography (HRCT) of chest was performed in patients with moderate–severe COPD, and the presence and the extent of bronchiectasis were evaluated by two radiologists. Demographic data, respiratory symptoms, lung function, previous pulmonary tuberculosis, serum inflammatory markers, serum total immunoglobulin E (T-IgE), and sputum culture of Pseudomonas aeruginosa were compared between those with and without bronchiectasis. Multivariate logistic regression analysis was used to determine the independent factors associated with bronchiectasis. We enrolled 190 patients with stable COPD, of which 87 (87/190, 45.8%) had bronchiectasis on HRCT. Compared with those without bronchiectasis, COPD patients with bronchiectasis were more likely to be males (P = 0.021), had a lower body mass index (BMI) (P = 0.019), a higher prevalence of previous tuberculosis (P = 0.005), longer history of dyspnea (P < 0.001), more severe dyspnea (P = 0.041), higher frequency of acute exacerbation (P = 0.002), higher serum concentrations of C-reactive protein (CRP) (P = 0.017), fibrinogen (P = 0.016), and T-IgE [P = 0.004; for log10(T-IgE), P <0.001]. COPD patients with bronchiectasis also showed poorer lung function (for FEV1/FVC, P = 0.013; for FEV1%predicted, P = 0.012; for global initiative for chronic obstructive lung disease (GOLD) grades, P = 0.035), and a higher positive rate of sputum P aeruginosa (P = 0.020). Logistic regression analysis demonstrated that male gender (P = 0.021), previous tuberculosis (P = 0.021), and increased level of serum T-IgE [for log10(T-IgE), P < 0.001] were risk factors for coexistent bronchiectasis. More notably, the level of serum T-IgE [log10(T-IgE)] was positively correlated with the extent of bronchiectasis in COPD patients (r = 0.208, P = 0.05). Higher serum T-IgE, male gender, and previous tuberculosis are independent risk factors for coexistent bronchiectasis in COPD. The association of T-IgE with the extent of bronchiectasis also suggests that further investigations are needed to explore the potential role of IgE in the pathogenesis of bronchiectasis in COPD. PMID:27442646

COPD-related bronchiectasis; independent impact on disease course and outcomes.

PubMed

Gatheral, Timothy; Kumar, Neelam; Sansom, Ben; Lai, Dilys; Nair, Arjun; Vlahos, Ioannis; Baker, Emma H

2014-12-01

COPD and radiographic bronchiectasis frequently coexist but the effect of this on the clinical course of COPD is not fully understood. We determined the impact of bronchiectasis on clinical outcomes in COPD patients, independent of coexisting emphysema and bronchial wall thickening (BWT). COPD patients admitted with first exacerbation 1998-2008 were identified retrospectively using ICD10 codes J44.0,1,8,9. Patients with suitable CT scans were graded for severity of bronchiectasis, emphysema and BWT on a 5 point scale (0-absent, 1-minor, 2-mild, 3-moderate, 4-severe). 406 patients (71 ± 11 years, 56% male, FEV1 52 ± 23% predicted) were included; 278 (69%) patients had bronchiectasis: minor, 112 (40%); mild, 81 (29%); moderate, 62 (22%); severe 23 (8%). Bronchiectasis severity correlated with severity of BWT (p < 0.001) but not emphysema (p = 0.090). Bronchiectasis independently determined sputum isolation of Pseudomonas aeruginosa (Odds ratio (OR) 1.39 (95% CI 1.07 to 1.80), p = 0.013) and atypical mycobacteria (OR 2.44 (95% CI 1.04 to 5.69), p = 0.040), annual respiratory admissions (p = 0.044) and inpatient days (p < 0.001), but did not predict survival (p = 0.256). Radiographic bronchiectasis in COPD patients is associated with increased respiratory infection and hospitalisation, independent of coexisting emphysema and BWT. COPD-related bronchiectasis is therefore an important diagnosis with potential implications for treatment.

Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis.

PubMed

Nikolic, Aleksandra

2018-05-12

Bronchiectasis is characterized by deregulated inflammatory response and recurrent bacterial infection resulting in progressive lung damage and an irreversible dilatation of bronchi and bronchioles. Generally accepted model of the development of bronchiectasis is the "vicious cycle hypothesis" that proposes compromising of the mucociliary clearance by an initial event, which leads to the infection of the respiratory tract followed by further impairment of mucociliary function, bacterial proliferation, and more inflammation. Bronchiectasis is a very common symptom in patients with cystic fibrosis (CF), while bronchiectasis unrelated to CF is heterogeneous pathology of unknown cause with a large number of potential contributory factors and poorly understood pathogenesis. It is presumed that bronchiectasis unrelated to CF is a multifactorial condition predisposed by genetic factors. Different molecules have been implicated in the onset and development of idiopathic bronchiectasis, as well as modulation of the disease severity and response to therapy. Most of these molecules are involved in the processes that contribute to the homeostasis of the lung tissue, especially mucociliary clearance, protease-antiprotease balance, and immunomodulation. Evaluation of the studies performed towards investigation of the role these molecules play in bronchiectasis identifies genetic variants that may be of potential importance for clinical management of the disease, and also of interest for future research efforts. This review focuses on the molecules with major roles in lung homeostasis and their involvement in bronchiectasis unrelated to CF.

Computed Tomography Findings of Bronchiectasis in Different Respiratory Phases Correlate with Pulmonary Function Test Data in Adults.

PubMed

do Amaral, Ricardo Holderbaum; Nin, Carlos S; de Souza, Vinicius V S; Alves, Giordano R T; Marchiori, Edson; Irion, Klaus; Meirelles, Gustavo S P; Hochhegger, Bruno

2017-06-01

To investigate bronchiectasis variations in different computed tomography (CT) respiratory phases, and their correlation with pulmonary function test (PFT) data, in adults. Retrospective data analysis from 63 patients with bronchiectasis according to CT criteria selected from the institution database and for whom PFT data were also available. Bronchiectasis diameter was measured on inspiratory and expiratory phases. Its area and matched airway-vessel ratios in both phases were also calculated. Finally, PFT results were compared with radiological measurements. Bronchiectatic airways were larger on inspiration than on expiration (mean cross-sectional area, 69.44 vs. 40.84 mm 2 ; p < 0.05) as were airway-vessel ratios (2.1 vs. 1.4; p < 0.05). Cystic bronchiectasis cases showed the least variation in cross-sectional area (48%). Mean predicted values of forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) were 81.5 and 77.2%, respectively, in the group in which bronchiectasis could not be identified on expiratory images, and 58.3 and 56.0%, respectively, in the other group (p < 0.05). Variation in bronchiectasis area was associated with poorer lung function (r = 0.32). Bronchiectasis detection, diameter, and area varied significantly according to CT respiratory phase, with non-reducible bronchiectasis showing greater lung function impairment.

Bronchiectasis diagnosed after renal transplantation: a retrospective multicenter study.

PubMed

Dury, Sandra; Colosio, Charlotte; Etienne, Isabelle; Anglicheau, Dany; Merieau, Elodie; Caillard, Sophie; Rivalan, Joseph; Thervet, Eric; Essig, Marie; Babinet, François; Subra, Jean-François; Toubas, Olivier; Rieu, Philippe; Launois, Claire; Perotin-Collard, Jeanne-Marie; Lebargy, François; Deslée, Gaëtan

2015-11-07

Bronchiectasis is characterized by abnormal, permanent and irreversible dilatation of the bronchi, usually responsible for daily symptoms and frequent respiratory complications. Many causes have been identified, but only limited data are available concerning the association between bronchiectasis and renal transplantation. We conducted a retrospective multicenter study of cases of bronchiectasis diagnosed after renal transplantation in 14 renal transplantation departments (French SPIESSER group). Demographic, clinical, laboratory and CT scan data were collected. Forty-six patients were included (mean age 58.2 years, 52.2 % men). Autosomal dominant polycystic kidney disease (32.6 %) was the main underlying renal disease. Chronic cough and sputum (50.0 %) were the major symptoms leading to chest CT scan. Mean duration of symptoms before diagnosis was 1.5 years [0-12.1 years]. Microorganisms were identified in 22 patients, predominantly Haemophilus influenzae. Hypogammaglobulinemia was observed in 46.9 % patients. Bronchiectasis was usually extensive (84.8 %). The total bronchiectasis score was 7.4 ± 5.5 with a significant gradient from apex to bases. Many patients remained symptomatic (43.5 %) and/or presented recurrent respiratory tract infections (37.0 %) during follow-up. Six deaths (13 %) occurred during follow-up, but none were attributable to bronchiectasis. These results highlight that the diagnosis of bronchiectasis should be considered in patients with de novo respiratory symptoms after renal transplantation. Further studies are needed to more clearly understand the mechanisms underlying bronchiectasis in this setting.

Patient information, education and self-management in bronchiectasis: facilitating improvements to optimise health outcomes.

PubMed

Hester, Katy L M; Newton, Julia; Rapley, Tim; De Soyza, Anthony

2018-05-22

Bronchiectasis is an incurable lung disease characterised by irreversible airway dilatation. It causes symptoms including chronic productive cough, dyspnoea, and recurrent respiratory infections often requiring hospital admission. Fatigue and reductions in quality of life are also reported in bronchiectasis. Patients often require multi-modal treatments that can be burdensome, leading to issues with adherence. In this article we review the provision of, and requirement for, education and information in bronchiectasis. To date, little research has been undertaken to improve self-management in bronchiectasis in comparison to other chronic conditions, such as COPD, for which there has been a wealth of recent developments. Qualitative work has begun to establish that information deficit is one of the potential barriers to self-management, and that patients feel having credible information is fundamental when learning to live with and manage bronchiectasis. Emerging research offers some insights into ways of improving treatment adherence and approaches to self-management education; highlighting ways of addressing the specific unmet information needs of patients and their families who are living with bronchiectasis. We propose non-pharmacological recommendations to optimise patient self-management and symptom recognition; with the aim of facilitating measurable improvements in health outcomes for patients with bronchiectasis.

Noncystic Fibrosis Bronchiectasis: Regional Abnormalities and Response to Airway Clearance Therapy Using Pulmonary Functional Magnetic Resonance Imaging.

PubMed

Svenningsen, Sarah; Guo, Fumin; McCormack, David G; Parraga, Grace

2017-01-01

Evidence-based treatment and management for patients with bronchiectasis remain challenging. There is a need for regional disease measurements as focal distribution of disease is common. Our objective was to evaluate the ability of magnetic resonance imaging (MRI) to detect regional ventilation impairment and response to airway clearance therapy (ACT) in patients with noncystic fibrosis (CF) bronchiectasis, providing a new way to objectively and regionally evaluate response to therapy. Fifteen participants with non-CF bronchiectasis and 15 age-matched healthy volunteers provided written informed consent to an ethics board-approved Health Insurance Portability and Accountability Act-compliant protocol and underwent spirometry, plethysmography, computed tomography (CT), and hyperpolarized 3 He MRI. Bronchiectasis patients also completed a Six-Minute Walk Test, the St. George's Respiratory questionnaire, and Patient Evaluation Questionnaire (PEQ), and returned for a follow-up visit after 3 weeks of daily oscillatory positive expiratory pressure use. CT evidence of bronchiectasis was qualitatively reported by lobe, and MRI ventilation defect percent (VDP) was measured for the entire lung and individual lobes. CT evidence of bronchiectasis and abnormal VDP (14 ± 7%) was observed for all bronchiectasis patients and no healthy volunteers. There was CT evidence of bronchiectasis in all lobes for 3 patients and in 3 ± 1 lobes (range = 1-4) for 12 patients. VDP in lobes with CT evidence of bronchiectasis (19 ± 12%) was significantly higher than in lobes without CT evidence of bronchiectasis (8 ± 5%, P = .001). For patients, VDP in lung lobes with (P < .0001) and without CT evidence of bronchiectasis (P = .006) was higher than in healthy volunteers (3 ± 1%). For all patients, mean PEQ-ease-bringing-up-sputum (P = .048) and PEQ-patient-global-assessment (P = .01) were significantly improved post-oscillatory positive expiratory pressure. An improvement in regional VDP greater than the minimum clinical important difference was observed for 8 of the 14 patients evaluated. There was CT and MRI evidence of structure-function abnormalities in patients with bronchiectasis; in approximately half, there was evidence of ventilation improvements after airway clearance therapy. Copyright © 2017 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.

Pharmacotherapy for Non-Cystic Fibrosis Bronchiectasis: Results From an NTM Info & Research Patient Survey and the Bronchiectasis and NTM Research Registry.

PubMed

Henkle, Emily; Aksamit, Timothy R; Barker, Alan F; Curtis, Jeffrey R; Daley, Charles L; Anne Daniels, M Leigh; DiMango, Angela; Eden, Edward; Fennelly, Kevin; Griffith, David E; Johnson, Margaret; Knowles, Michael R; Leitman, Amy; Leitman, Philip; Malanga, Elisha; Metersky, Mark L; Noone, Peadar G; O'Donnell, Anne E; Olivier, Kenneth N; Prieto, Delia; Salathe, Matthias; Thomashow, Byron; Tino, Gregory; Turino, Gerard; Wisclenny, Susan; Winthrop, Kevin L

2017-12-01

Non-cystic fibrosis bronchiectasis ("bronchiectasis") is a chronic inflammatory lung disease often associated with nontuberculous mycobacteria (NTM) infection. Very little data exist to guide bronchiectasis management decisions. We sought to describe patterns of inhaled corticosteroid (ICS) and antibiotic therapy in the United States. We invited 2,000 patients through NTM Info & Research (NTMir) to complete an anonymous electronic survey. We separately queried baseline clinical and laboratory data from the US Bronchiectasis and NTM Research Registry (BRR). Among 511 NTMir survey responders with bronchiectasis, whose median age was 67 years, 85 (17%) reported asthma and 99 (19%) reported COPD. History of ICS use was reported by 282 (55%), 171 (61%) of whom were treated 1 year or longer, and 150 (53%) were currently taking ICSs. Fewer reported ever taking azithromycin for non-NTM bronchiectasis (203 responders [40%]) or inhaled tobramycin (78 responders [15%]). The median age of 1,912 BRR patients was 69 years; 528 (28%) had asthma and 360 (19%) had COPD. Among 740 patients (42%) without NTM, 314 were taking ICSs at baseline. Among patients without NTM who were taking ICSs, only 178 (57%) had a concurrent diagnosis of COPD or asthma that could explain ICS use. Fewer were taking suppressive macrolides (96 patients [13%]), and of the 70 patients (10%) taking inhaled suppressive antibiotics, 48 (68%) had chronic Pseudomonas aeruginosa infection. ICS use was common in two national samples of patients with bronchiectasis, with relatively few patients taking suppressive antibiotic therapies. Further research is needed to clarify the safety and effectiveness of these therapies in patients with bronchiectasis. Copyright © 2017 American College of Chest Physicians. All rights reserved.

High prevalence of bronchiectasis in adults: analysis of CT findings in a health screening program.

PubMed

Kwak, Hyun Jung; Moon, Ji-Yong; Choi, Yo Won; Kim, Tae Hyung; Sohn, Jang Won; Yoon, Ho Joo; Shin, Dong Ho; Park, Sung Soo; Kim, Sang-Heon

2010-12-01

Bronchiectasis is one of the common chronic respiratory diseases and associated with respiratory morbidity and mortality. However, neither its prevalence nor its etiology is well-defined. We aimed to estimate the prevalence and risk factors of bronchiectasis in adults. In a retrospective study, we analyzed radiologic findings on chest computed tomography (CT) images performed as part of a health-screening program. From January to December 2008, 1,409 (24.6%) of 5,727 participants in the screening program of a health promotion center at a university hospital underwent chest CT scans based on the subject's decision. Bronchiectasis was diagnosed, if there was abnormal bronchial dilatation in any area of both lungs on chest CT. Respiratory symptoms, smoking status, and past medical history were also analyzed to define clinical characteristics and risk factors of bronchiectasis. Of 1,409 patients (aged 23-86 years), who were screened for respiratory diseases using chest CT for one year in a health promotion center, 129 patients (9.1%) were diagnosed with bronchiectasis. The prevalence of bronchiectasis was higher in females than in males (11.5% vs. 7.9%, p = 0.022) and increased with age. Respiratory symptoms were reported in 53.7% of subjects. Previous history of tuberculosis (TB) (OR 4.61, 95% CI 2.39-8.88, p = 0.001) and age (OR 2.49, 95% CI 1.56-3.98, p = 0.001) were significantly associated with bronchiectasis. This retrospective analysis of chest CT findings in health screening examinees revealed a very high prevalence of bronchiectasis in adults. Previous TB infection is one of the major causes of bronchiectasis.

The pathophysiology of bronchiectasis

PubMed Central

King, Paul T

2009-01-01

Bronchiectasis is defined by permanent and abnormal widening of the bronchi. This process occurs in the context of chronic airway infection and inflammation. It is usually diagnosed using computed tomography scanning to visualize the larger bronchi. Bronchiectasis is also characterized by mild to moderate airflow obstruction. This review will describe the pathophysiology of noncystic fibrosis bronchiectasis. Studies have demonstrated that the small airways in bronchiectasis are obstructed from an inflammatory infiltrate in the wall. As most of the bronchial tree is composed of small airways, the net effect is obstruction. The bronchial wall is typically thickened by an inflammatory infiltrate of lymphocytes and macrophages which may form lymphoid follicles. It has recently been demonstrated that patients with bronchiectasis have a progressive decline in lung function. There are a large number of etiologic risk factors associated with bronchiectasis. As there is generally a long-term retrospective history, it may be difficult to determine the exact role of such factors in the pathogenesis. Extremes of age and smoking/chronic obstructive pulmonary disease may be important considerations. There are a variety of different pathogens involved in bronchiectasis, but a common finding despite the presence of purulent sputum is failure to identify any pathogenic microorganisms. The bacterial flora appears to change with progression of disease. PMID:20037680

Bronchiectasis in Children: Current Concepts in Immunology and Microbiology.

PubMed

Pizzutto, Susan J; Hare, Kim M; Upham, John W

2017-01-01

Bronchiectasis is a complex chronic respiratory condition traditionally characterized by chronic infection, airway inflammation, and progressive decline in lung function. Early diagnosis and intensive treatment protocols can stabilize or even improve the clinical prognosis of children with bronchiectasis. However, understanding the host immunologic mechanisms that contribute to recurrent infection and prolonged inflammation has been identified as an important area of research that would contribute substantially to effective prevention strategies for children at risk of bronchiectasis. This review will focus on the current understanding of the role of the host immune response and important pathogens in the pathogenesis of bronchiectasis (not associated with cystic fibrosis) in children.

Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening.

PubMed

Stick, Stephen M; Brennan, Siobhain; Murray, Conor; Douglas, Tonia; von Ungern-Sternberg, Britta S; Garratt, Luke W; Gangell, Catherine L; De Klerk, Nicholas; Linnane, Barry; Ranganathan, Sarath; Robinson, Phillip; Robertson, Colin; Sly, Peter D

2009-11-01

To determine the prevalence of bronchiectasis in young children with cystic fibrosis (CF) diagnosed after newborn screening (NBS) and the relationship of bronchiectasis to pulmonary inflammation and infection. Children were diagnosed with CF after NBS. Computed tomography and bronchoalveolar lavage were performed with anesthesia (n = 96). Scans were analyzed for the presence and extent of abnormalities. The prevalence of bronchiectasis was 22% and increased with age (P = .001). Factors associated with bronchiectasis included absolute neutrophil count (P = .03), neutrophil elastase concentration (P = .001), and Pseudomonas aeruginosa infection (P = .03). Pulmonary abnormalities are common in infants and young children with CF and relate to neutrophilic inflammation and infection with P. aeruginosa. Current models of care for infants with CF fail to prevent respiratory sequelae. Bronchiectasis is a clinically relevant endpoint that could be used for intervention trials that commence soon after CF is diagnosed after NBS.

The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC): experiences from a successful ERS Clinical Research Collaboration.

PubMed

Chalmers, James D; Crichton, Megan; Goeminne, Pieter C; Loebinger, Michael R; Haworth, Charles; Almagro, Marta; Vendrell, Montse; De Soyza, Anthony; Dhar, Raja; Morgan, Lucy; Blasi, Francesco; Aliberti, Stefano; Boyd, Jeanette; Polverino, Eva

2017-09-01

In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare diseases such as cystic fibrosis, there has been little research and few clinical trials in bronchiectasis. Guidelines are primarily based on expert opinion and treatment is challenging because of the heterogeneous nature of the disease. In an effort to address decades of underinvestment in bronchiectasis research, education and clinical care, the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) was established in 2012 as a collaborative pan-European network to bring together bronchiectasis researchers. The European Respiratory Society officially funded EMBARC in 2013 as a Clinical Research Collaboration, providing support and infrastructure to allow the project to grow. EMBARC has now established an international bronchiectasis registry that is active in more than 30 countries both within and outside Europe. Beyond the registry, the network participates in designing and facilitating clinical trials, has set international research priorities, promotes education and has participated in producing the first international bronchiectasis guidelines. This manuscript article the development, structure and achievements of EMBARC from 2012 to 2017. To understand the role of Clinical Research Collaborations as the major way in which the European Respiratory Society can stimulate clinical research in different disease areasTo understand some of the key features of successful disease registriesTo review key epidemiological, clinical and translational studies of bronchiectasis contributed by the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) project in the past 5 yearsTo understand the key research priorities identified by EMBARC for the next 5 years.

The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC): experiences from a successful ERS Clinical Research Collaboration

PubMed Central

Crichton, Megan; Goeminne, Pieter C.; Loebinger, Michael R.; Haworth, Charles; Almagro, Marta; Vendrell, Montse; De Soyza, Anthony; Dhar, Raja ; Morgan, Lucy; Blasi, Francesco; Aliberti, Stefano; Boyd, Jeanette; Polverino, Eva

2017-01-01

In contrast to airway diseases like chronic obstructive pulmonary disease or asthma, and rare diseases such as cystic fibrosis, there has been little research and few clinical trials in bronchiectasis. Guidelines are primarily based on expert opinion and treatment is challenging because of the heterogeneous nature of the disease. In an effort to address decades of underinvestment in bronchiectasis research, education and clinical care, the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) was established in 2012 as a collaborative pan-European network to bring together bronchiectasis researchers. The European Respiratory Society officially funded EMBARC in 2013 as a Clinical Research Collaboration, providing support and infrastructure to allow the project to grow. EMBARC has now established an international bronchiectasis registry that is active in more than 30 countries both within and outside Europe. Beyond the registry, the network participates in designing and facilitating clinical trials, has set international research priorities, promotes education and has participated in producing the first international bronchiectasis guidelines. This manuscript article the development, structure and achievements of EMBARC from 2012 to 2017. Educational aims To understand the role of Clinical Research Collaborations as the major way in which the European Respiratory Society can stimulate clinical research in different disease areas To understand some of the key features of successful disease registries To review key epidemiological, clinical and translational studies of bronchiectasis contributed by the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) project in the past 5 years To understand the key research priorities identified by EMBARC for the next 5 years PMID:28894479

The Objective Assessment of Cough Frequency in Bronchiectasis.

PubMed

Spinou, Arietta; Lee, Kai K; Sinha, Aish; Elston, Caroline; Loebinger, Michael R; Wilson, Robert; Chung, Kian Fan; Yousaf, Nadia; Pavord, Ian D; Matos, Sergio; Garrod, Rachel; Birring, Surinder S

2017-10-01

Cough in bronchiectasis is associated with significant impairment in health status. This study aimed to quantify cough frequency objectively with a cough monitor and investigate its relationship with health status. A secondary aim was to identify clinical predictors of cough frequency. Fifty-four patients with bronchiectasis were compared with thirty-five healthy controls. Objective 24-h cough, health status (cough-specific: Leicester Cough Questionnaire LCQ and bronchiectasis specific: Bronchiectasis Health Questionnaire BHQ), cough severity and lung function were measured. The clinical predictors of cough frequency in bronchiectasis were determined in a multivariate analysis. Objective cough frequency was significantly raised in patients with bronchiectasis compared to healthy controls [geometric mean (standard deviation)] 184.5 (4.0) vs. 20.6 (3.2) coughs/24-h; mean fold-difference (95% confidence interval) 8.9 (5.2, 15.2); p < 0.001 and they had impaired health status. There was a significant correlation between objective cough frequency and subjective measures; LCQ r = -0.52 and BHQ r = -0.62, both p < 0.001. Sputum production, exacerbations (between past 2 weeks to 12 months) and age were significantly associated with objective cough frequency in multivariate analysis, explaining 52% of the variance (p < 0.001). There was no statistically significant association between cough frequency and lung function. Cough is a common and significant symptom in patients with bronchiectasis. Sputum production, exacerbations and age, but not lung function, were independent predictors of cough frequency. Ambulatory objective cough monitoring provides novel insights and should be further investigated as an outcome measure in bronchiectasis.

Comorbidities and the risk of mortality in patients with bronchiectasis: an international cohort study

PubMed Central

McDonnell, Melissa J; Aliberti, Stefano; Goeminne, Pieter C.; Restrepo, Marcos I.; Finch, Simon; Pesci, Alberto; Dupont, Lieven J; Fardon, Thomas C.; Wilson, Robert; Loebinger, Michael R; Skrbic, Dusan; Obradovic, Dusanka; De Soyza, Anthony; Ward, Chris; Laffey, John G.; Rutherford, Robert M.; Chalmers, James D.

2017-01-01

Background Patients with bronchiectasis often suffer from concurrent comorbidities but their nature, prevalence and impact on disease severity and outcome is poorly understood. We aimed to evaluate comorbidities in bronchiectasis patients and determine their prognostic value on disease severity and mortality. Methods An observational cohort analysis of 986 bronchiectasis patients across four European centres was performed for score derivation. Comorbidity diagnoses were based on standardised definitions obtained on full review of hard copy and electronic records, prescriptions and investigator definitions. Weibull parametric survival analysis was used to model the prediction of 5-year mortality to construct the Bronchiectasis Aetiology Comorbidity Index (BACI). We tested the BACI as a predictor of outcomes and explored whether the BACI added further prognostic information when used alongside the Bronchiectasis Severity Index (BSI). Findings Median number of comorbidities per patient was 4 (IQR 2-6), range 0-20. Thirteen comorbidities independently predicting mortality were integrated into the BACI. The overall hazard ratio for death conferred by a one point increase in the BACI was 1.18 (1.14-1.23), p<0.0001. The BACI predicted 5-year mortality, hospitalisations, exacerbations and health-related quality of life across all BSI risk strata (p<0.0001). When used in conjunction with the BSI, the combined model was superior to either model alone. The BACI was validated in two independent international cohorts. Interpretation Multimorbidity is frequent in bronchiectasis and can negatively influence survival. The BACI complements the BSI in assessing mortality and disease outcomes in patients with bronchiectasis. Funding 1. European Bronchiectasis Network (EMBARC).2. Health Research Board Ireland. PMID:27864036

Proximal and distal gastro-oesophageal reflux in chronic obstructive pulmonary disease and bronchiectasis.

PubMed

Lee, Annemarie L; Button, Brenda M; Denehy, Linda; Roberts, Stuart J; Bamford, Tiffany L; Ellis, Samantha J; Mu, Fi-Tjen; Heine, Ralf G; Stirling, Robert G; Wilson, John W

2014-02-01

The aims of this observational study were (i) to examine the prevalence of symptomatic and clinically silent proximal and distal gastro-oesophageal reflux (GOR) in adults with chronic obstructive pulmonary disease (COPD) or bronchiectasis, (ii) the presence of gastric aspiration, and (iii) to explore the possible clinical significance of this comorbidity in these conditions. Twenty-seven participants with COPD, 27 with bronchiectasis and 17 control subjects completed reflux symptom evaluation and dual-channel 24 h oesophageal pH monitoring. In those with lung disease, pepsin levels in sputum samples were measured using enzyme-linked immunosorbent assay, with disease severity (lung function and high-resolution computed tomography) also measured. The prevalence of GOR in COPD was 37%, in bronchiectasis was 40% and in control subjects was 18% (P = 0.005). Of those diagnosed with GOR, clinically silent reflux was detected in 20% of participants with COPD and 42% with bronchiectasis. While pepsin was found in 33% of COPD and 26% of bronchiectasis participants, the presence of pepsin in sputum was not related to a diagnosis of GOR based on oesophageal pH monitoring in either condition. Neither a diagnosis of GOR nor the presence of pepsin was associated with increased severity of lung disease in COPD or bronchiectasis. The prevalence of GOR in COPD or bronchiectasis is twice that of the control population, and the diagnosis could not be based on symptoms alone. Pepsin was detected in sputum in COPD and bronchiectasis, suggesting a possible role of pulmonary aspiration, which requires further exploration. © 2013 The Authors. Respirology © 2013 Asian Pacific Society of Respirology.

Impacts of Co-Existing Chronic Rhinosinusitis on Disease Severity and Risks of Exacerbations in Chinese Adults with Bronchiectasis

PubMed Central

Guan, Wei-jie; Gao, Yong-hua; Li, Hui-min; Yuan, Jing-jing; Chen, Rong-chang; Zhong, Nan-shan

2015-01-01

Background Mounting evidence supports the notion of “one airway, one disease.” Objective To determine whether chronic rhinosinusitis (CRS) poses adverse impacts on Chinese adults with bronchiectasis. Methods We enrolled 148 consecutive adults with clinically stable bronchiectasis. CRS diagnosed based on the 2012 EP3OS criteria. We systematically evaluated the bronchiectasis etiology, radiology, lung function, sputum bacteriology, airway inflammatory biomarkers, Bronchiectasis Severity Index, cough sensitivity and healthcare resource utilization. All patients were prospectively followed-up for 1 year to examine the frequency of bronchiectasis exacerbations (BEs). Results Forty-seven patients (31.8%) were diagnosed as having CRS. Bronchiectasis etiologies did not vary statistically between CRS and no-CRS group. There was a trend towards non-statistically higher Bronchiectasis Severity Index [6.4±3.4 vs. 5.0(6.0), P = 0.19], a higher proportion of patients with BEs needing hospitalization before enrollment (48.9% vs. 29.7%, P = 0.13), poorer FVC [78.2±19.8% vs. 82.2(16.8)%, P = 0.54] and FEV1 [68.2±24.8% vs. 74.8(21.2)%, P = 0.29], a higher prevalence of Pseudomonas aeruginosa isolated (36.2% vs. 26.7%, P = 0.27) or colonized in sputum (36.2% vs. 21.8%, P = 0.12) and greater capsaicin cough sensitivity [C2: 3.9(123.0) μmol/L vs. 11.7(123.0) μmol/L, P = 0.81; C5: 62.5(996.0) μmol/L vs. 250.0(973.0) μmol/L, P = 0.32]. Patients with CRS had significantly greater risks of experiencing BEs during follow-up (P = 0.02 for negative binominal regression test). Conclusion Chinese adults with bronchiectasis appear to have a lower prevalence of CRS than that in western countries. There was a trend towards greater adverse impacts on bronchiectasis in patients with CRS. Studies with greater sample sizes might help to resolve this issue. In future clinical practice, physicians should be vigilant to the screening of concomitant CRS in bronchiectasis so as to better improve patient’s healthcare. Our findings may be of clinical significance in that proper treatment of upper airway symptoms due to CRS will be the prevention of infection or re-infection of the tracheobronchial tree, which should be addressed for the future management of bronchiectasis. PMID:26340660

Quality of Life and Bronchial Hyper-Responsiveness in Subjects With Bronchiectasis: Validation of the Seattle Obstructive Lung Disease Questionnaire in Bronchiectasis.

PubMed

Bulcun, Emel; Arslan, Mesut; Ekici, Aydanur; Ekici, Mehmet

2015-11-01

Bronchiectasis can adversely affect quality of life. However, the tests examining quality of life in bronchiectasis are not sufficient. We examined the validity of a measure designed for COPD, the Seattle Obstructive Lung Disease Questionnaire (SOLQ), in bronchiectasis. In addition, we aimed to compare the quality of life of subjects with bronchiectasis and bronchial hyper-responsiveness with that of those without to identify the effective factors. We studied 78 subjects with clinically stable bronchiectasis and 41 healthy controls matched for age and sex. Subjects were assessed by the SOLQ. A detailed history, physical examination, the Medical Outcomes Study 36-Item Short Form questionnaire, the Hospital Anxiety and Depression Scale, and spirometric measurements were obtained. Cronbach α coefficients, which reflected internal consistency, were >0.70 for all SOLQ components except for treatment satisfaction. SOLQ component scores correlated with all of the component scores of the Medical Outcomes Study 36-Item Short Form questionnaire and the Hospital Anxiety and Depression Scale, confirming their concurrent validity. All SOLQ scores correlated positively with percent-of-predicted FEV1, whereas the physical function, treatment satisfaction, and emotional function correlated negatively with the exacerbation frequency in Pearson analysis. Emotional and physical functions were positively associated with percent-of-predicted FEV1 in linear regression analysis. Compared with subjects without bronchial hyper-responsiveness, those with bronchial hyper-responsiveness had lower FEV1/FVC and more exacerbations/y. Compared with bronchiectasis subjects without bronchial hyper-responsiveness, those with bronchial hyper-responsiveness had significantly lower SOLQ, physical function, and coping skills scores but not emotional function and treatment satisfaction. The SOLQ is a valid instrument for determining quality of life in subjects with bronchiectasis. Subjects with bronchiectasis and bronchial hyper-responsiveness had a poorer quality of life, lower baseline spirometric values, and more frequent exacerbations, suggesting more severe disease. Copyright © 2015 by Daedalus Enterprises.

Geographic variation in the aetiology, epidemiology and microbiology of bronchiectasis.

PubMed

Chandrasekaran, Ravishankar; Mac Aogáin, Micheál; Chalmers, James D; Elborn, Stuart J; Chotirmall, Sanjay H

2018-05-22

Bronchiectasis is a disease associated with chronic progressive and irreversible dilatation of the bronchi and is characterised by chronic infection and associated inflammation. The prevalence of bronchiectasis is age-related and there is some geographical variation in incidence, prevalence and clinical features. Most bronchiectasis is reported to be idiopathic however post-infectious aetiologies dominate across Asia especially secondary to tuberculosis. Most focus to date has been on the study of airway bacteria, both as colonisers and causes of exacerbations. Modern molecular technologies including next generation sequencing (NGS) have become invaluable tools to identify microorganisms directly from sputum and which are difficult to culture using traditional agar based methods. These have provided important insight into our understanding of emerging pathogens in the airways of people with bronchiectasis and the geographical differences that occur. The contribution of the lung microbiome, its ethnic variation, and subsequent roles in disease progression and response to therapy across geographic regions warrant further investigation. This review summarises the known geographical differences in the aetiology, epidemiology and microbiology of bronchiectasis. Further, we highlight the opportunities offered by emerging molecular technologies such as -omics to further dissect out important ethnic differences in the prognosis and management of bronchiectasis.

The development and validation of the Bronchiectasis Health Questionnaire.

PubMed

Spinou, Arietta; Siegert, Richard J; Guan, Wei-Jie; Patel, Amit S; Gosker, Harry R; Lee, Kai K; Elston, Caroline; Loebinger, Michael R; Wilson, Robert; Garrod, Rachel; Birring, Surinder S

2017-05-01

Health-related quality of life or health status is significantly impaired in bronchiectasis. There is a paucity of brief, simple-to-use, disease-specific health status measures. The aim of this study was to develop and validate the Bronchiectasis Health Questionnaire (BHQ), a new health status measure that is brief and generates a single overall score.Patients with bronchiectasis were recruited from two outpatient clinics, during a clinically stable stage. The development of the questionnaire followed three phases: item generation and item reduction using Rasch analysis, validation, and repeatability testing. The BHQ was translated into 11 languages using standardised methodology.206 patients with bronchiectasis completed a preliminary 65-item questionnaire. 55 items were removed due to redundancy or poor fit to the Rasch model. The final version of the BHQ consisted of 10 items. Internal consistency was good (Cronbach's α=0.85). Convergent validity of the BHQ with the St George's Respiratory Questionnaire was high (r= -0.82; p<0.001) and moderate with lung function (forced expiratory volume in 1 s % predicted r= -0.27; p=0.001). There was a significant association between BHQ scores and number of exacerbations of bronchiectasis in the last 12 months (p<0.001), hospital admissions (p=0.001) and computed tomography scan bronchiectasis pulmonary lobe counts (p<0.001). BHQ scores were significantly worse in patients with sputum bacterial colonisation versus no colonisation (p=0.048). The BHQ was highly repeatable after 2 weeks (intraclass correlation coefficient 0.89).The BHQ is a brief, valid and repeatable, self-completed health status questionnaire for bronchiectasis that generates a single total score. It can be used in the clinic to assess bronchiectasis from the patient's perspective. Copyright ©ERS 2017.

Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis.

PubMed

Tepper, Leonie A; Utens, Elisabeth M W J; Caudri, Daan; Bos, Aukje C; Gonzalez-Graniel, Karla; Duivenvoorden, Hugo J; van der Wiel, Els C W; Quittner, Alexandra L; Tiddens, Harm A W M

2013-08-01

Cystic fibrosis (CF) is primarily characterised by bronchiectasis and trapped air on chest computed tomography (CT). The revised Cystic Fibrosis Questionnaire respiratory symptoms scale (CFQ-R RSS) measures health-related quality of life. To validate bronchiectasis, trapped air and CFQ-R RSS as outcome measures, we investigated correlations and predictive values for pulmonary exacerbations. CF patients (aged 6-20 years) underwent CT, CFQ-R RSS and 1-year follow-up. Bronchiectasis and trapped air were scored using the CF-CT scoring system. Correlation coefficients and backward multivariate modelling were used to identify predictors of pulmonary exacerbations. 40 children and 32 adolescents were included. CF-CT bronchiectasis (r = -0.38, p<0.001) and CF-CT trapped air (r = -0.35, p = 0.003) correlated with CFQ-R RSS. Pulmonary exacerbations were associated with: bronchiectasis (rate ratio 1.10, 95% CI 1.02-1.19; p = 0.009), trapped air (rate ratio 1.02, 95% CI 1.00-1.05; p = 0.034) and CFQ-R RSS (rate ratio 0.95, 95% CI 0.91-0.98; p = 0.002). The CFQ-R RSS was an independent predictor of pulmonary exacerbations (rate ratio 0.96, 95% CI 0.94-0.97; p<0.001). Bronchiectasis, trapped air and CFQ-R RSS were associated with pulmonary exacerbations. The CFQ-R RSS was an independent predictor. This study further validated bronchiectasis, trapped air and CFQ-R RSS as outcome measures in CF.

Survival in Patients with Advanced Non-cystic Fibrosis Bronchiectasis Versus Cystic Fibrosis on the Waitlist for Lung Transplantation.

PubMed

Hayes, Don; Kopp, Benjamin T; Tobias, Joseph D; Woodley, Frederick W; Mansour, Heidi M; Tumin, Dmitry; Kirkby, Stephen E

2015-12-01

Survival in non-cystic fibrosis (CF) bronchiectasis is not well studied. The United Network for Organ Sharing database was queried from 1987 to 2013 to compare survival in adult patients with non-CF bronchiectasis to patients with CF listed for lung transplantation (LTx). Each subject was tracked from waitlist entry date until death or censoring to determine survival differences between the two groups. Of 2112 listed lung transplant candidates with bronchiectasis (180 non-CF, 1932 CF), 1617 were used for univariate Cox and Kaplan-Meier survival function analysis, 1173 for multivariate Cox models, and 182 for matched-pairs analysis based on propensity scores. Compared to CF, patients with non-CF bronchiectasis had a significantly lower mortality by univariate Cox analysis (HR 0.565; 95 % CI 0.424, 0.754; p < 0.001). Adjusting for potential confounders, multivariate Cox models identified a significant reduction in risk for death associated with non-CF bronchiectasis who were lung transplant candidates (HR 0.684; 95 % CI 0.475, 0.985; p = 0.041). Results were consistent in multivariate models adjusting for pulmonary hypertension and forced expiratory volume in one second. Non-CF bronchiectasis with advanced lung disease was associated with significantly lower mortality hazard compared to CF bronchiectasis on the waitlist for LTx. Separate referral and listing criteria for LTx in non-CF and CF populations should be considered.

Characterization of bronchiectasis in the elderly.

PubMed

Bellelli, Giuseppe; Chalmers, James D; Sotgiu, Giovanni; Dore, Simone; McDonnell, Melissa J; Goeminne, Pieter C; Dimakou, Katerina; Skrbic, Dusan; Lombi, Andrea; Pane, Federico; Obradovic, Dusanka; Fardon, Thomas C; Rutherford, Robert M; Pesci, Alberto; Aliberti, Stefano

2016-10-01

Although bronchiectasis particularly affects people ≥65 years of age, data describing clinical characteristics of the disease in this population are lacking. This study aimed at evaluating bronchiectasis features in older adults and elderly, along with their clinical outcomes. This was a secondary analysis of six European databases of prospectively enrolled adult outpatients with bronchiectasis. Bronchiectasis characteristics were compared across three study groups: younger adults (18-65 years), older adults (66-75 years), and elderly (and ≥76 years). 3-year mortality was the primary study outcome. Among 1258 patients enrolled (median age: 66 years; 42.5% males), 50.9% were ≥65 years and 19.1 ≥ 75 years old. Elderly patients were more comorbid, had worse quality of life and died more frequently than the others. Differences were detected among the three study groups with regard to neither the etiology nor the severity of bronchiectasis, nor the prevalence of chronic infection with P. aeruginosa. In multivariate regression model, age (OR: 1.05; p-value: <0.0001), low BMI (OR: 2.63; p-value: 0.02), previous hospitalizations (OR: 2.06; p-value: 0.006), and decreasing FEV 1 (OR: 1.02; p-value: 0.001) were independent predictors of 3-year mortality, after adjustment for covariates. Bronchiectasis does not substantially differ across age groups. Poor outcomes in elderly patients with bronchiectasis might be directly related to individual's frailty that should be further investigated in clinical studies. Copyright © 2016 Elsevier Ltd. All rights reserved.

The Saudi Thoracic Society guidelines for diagnosis and management of noncystic fibrosis bronchiectasis

PubMed Central

Al-Jahdali, Hamdan; Alshimemeri, Abdullah; Mobeireek, Abdullah; Albanna, Amr S.; Al Shirawi, Nehad N.; Wali, Siraj; Alkattan, Khaled; Alrajhi, Abdulrahman A.; Mobaireek, Khalid; Alorainy, Hassan S.; Al-Hajjaj, Mohamed S.; Chang, Anne B.; Aliberti, Stefano

2017-01-01

This is the first guideline developed by the Saudi Thoracic Society for the diagnosis and management of noncystic fibrosis bronchiectasis. Local experts including pulmonologists, infectious disease specialists, thoracic surgeons, respiratory therapists, and others from adult and pediatric departments provided the best practice evidence recommendations based on the available international and local literature. The main objective of this guideline is to utilize the current published evidence to develop recommendations about management of bronchiectasis suitable to our local health-care system and available resources. We aim to provide clinicians with tools to standardize the diagnosis and management of bronchiectasis. This guideline targets primary care physicians, family medicine practitioners, practicing internists and respiratory physicians, and all other health-care providers involved in the care of the patients with bronchiectasis. PMID:28808486

Predictors of adherence to treatment in bronchiectasis.

PubMed

McCullough, Amanda R; Tunney, Michael M; Stuart Elborn, J; Bradley, Judy M; Hughes, Carmel M

2015-07-01

We aimed to determine if beliefs about treatment, clinical factors and quality of life predicted adherence to treatment in patients with bronchiectasis. We recruited participants with confirmed bronchiectasis to a one-year study. We calculated adherence to treatment using medication possession ratios and self-report. Baseline Beliefs about Medicines, clinical, demographic and Quality of Life Questionnaire-Bronchiectasis data were collected. We used logistic regression to determine predictors of adherence to treatment during the subsequent year. Seventy-five participants were recruited. Beliefs about harm, age and total number of prescribed medications were predictors of adherence to inhaled antibiotics. Concerns about medication, age and Quality of Life Questionnaire-Bronchiectasis Treatment Burden were predictors of adherence to other respiratory medicines. Beliefs about necessity of airway clearance and age were predictors of adherence to airway clearance. Beliefs about treatment, age, number of prescribed medications and perceived treatment burden predicted subsequent adherence in bronchiectasis, thereby, providing potential targets for future interventions in this population. Clinicians can use these data to identify patients with bronchiectasis who might be at risk of non-adherence i.e. those who are younger, have concerns about medications, who do not think airway clearance is necessary or who are prescribed numerous medications. Copyright © 2015 Elsevier Ltd. All rights reserved.

Comorbidities and the risk of mortality in patients with bronchiectasis: an international multicentre cohort study.

PubMed

McDonnell, Melissa J; Aliberti, Stefano; Goeminne, Pieter C; Restrepo, Marcos I; Finch, Simon; Pesci, Alberto; Dupont, Lieven J; Fardon, Thomas C; Wilson, Robert; Loebinger, Michael R; Skrbic, Dusan; Obradovic, Dusanka; De Soyza, Anthony; Ward, Chris; Laffey, John G; Rutherford, Robert M; Chalmers, James D

2016-12-01

Patients with bronchiectasis often have concurrent comorbidities, but the nature, prevalence, and impact of these comorbidities on disease severity and outcome are poorly understood. We aimed to investigate comorbidities in patients with bronchiectasis and establish their prognostic value on disease severity and mortality rate. An international multicentre cohort analysis of outpatients with bronchiectasis from four European centres followed up for 5 years was done for score derivation. Eligible patients were those with bronchiectasis confirmed by high-resolution CT and a compatible clinical history. Comorbidity diagnoses were based on standardised definitions and were obtained from full review of paper and electronic medical records, prescriptions, and investigator definitions. Weibull parametric survival analysis was used to model the prediction of the 5 year mortality rate to construct the Bronchiectasis Aetiology Comorbidity Index (BACI). We tested the BACI as a predictor of outcomes and explored whether the BACI added further prognostic information when used alongside the Bronchiectasis Severity Index (BSI). The BACI was validated in two independent international cohorts from the UK and Serbia. Between June 1, 2006, and Nov 22, 2013, 1340 patients with bronchiectasis were screened and 986 patients were analysed. Patients had a median of four comorbidities (IQR 2-6; range 0-20). 13 comorbidities independently predicting mortality rate were integrated into the BACI. The overall hazard ratio for death conferred by a one-point increase in the BACI was 1·18 (95% CI 1·14-1·23; p<0·0001). The BACI predicted 5 year mortality rate, hospital admissions, exacerbations, and health-related quality of life across all BSI risk strata (p<0·0001 for mortality and hospital admissions, p=0·03 for exacerbations, p=0·0008 for quality of life). When used in conjunction with the BSI, the combined model was superior to either model alone (p=0·01 for combined vs BACI; p=0·008 for combined vs BSI). Multimorbidity is frequent in bronchiectasis and can negatively affect survival. The BACI complements the BSI in the assessment and prediction of mortality and disease outcomes in patients with bronchiectasis. European Bronchiectasis Network (EMBARC). Copyright © 2016 Elsevier Ltd. All rights reserved.

Quantitative CT Measures of Bronchiectasis in Smokers.

PubMed

Diaz, Alejandro A; Young, Thomas P; Maselli, Diego J; Martinez, Carlos H; Gill, Ritu; Nardelli, Pietro; Wang, Wei; Kinney, Gregory L; Hokanson, John E; Washko, George R; San Jose Estepar, Raul

2017-06-01

Bronchiectasis is frequent in smokers with COPD; however, there are only limited data on objective assessments of this process. The objective was to assess bronchovascular morphology, calculate the ratio of the diameters of bronchial lumen and adjacent artery (BA ratio), and identify those measurements able to discriminate bronchiectasis. We collected quantitative CT (QCT) measures of BA ratios, peak wall attenuation, wall thickness (WT), wall area, and wall area percent (WA%) at matched fourth through sixth airway generations in 21 ever smokers with bronchiectasis (cases) and 21 never-smoking control patients (control airways). In cases, measurements were collected at both bronchiectatic and nonbronchiectatic airways. Logistic analysis and the area under receiver operating characteristic curve (AUC) were used to assess the predictive ability of QCT measurements for bronchiectasis. The whole-lung and fourth through sixth airway generation BA ratio, WT, and WA% were significantly greater in bronchiectasis cases than control patients. The AUCs for the BA ratio to predict bronchiectasis ranged from 0.90 (whole lung) to 0.79 (fourth-generation). AUCs for WT and WA% ranged from 0.72 to 0.75 and from 0.71 to 0.75. The artery diameters but not bronchial diameters were smaller in bronchiectatic than both nonbronchiectatic and control airways (P < .01 for both). Smoking-related increases in the BA ratio appear to be driven by reductions in vascular caliber. QCT measures of BA ratio, WT, and WA% may be useful to objectively identify and quantify bronchiectasis in smokers. ClinicalTrials.gov; No.: NCT00608764; URL: www.clinicaltrials.gov. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Diffuse cylindrical bronchiectasis due to eosinophilic bronchopneumopathy in a dog

PubMed Central

Meler, Erika; Pressler, Barrak M.; Heng, Hock Gan; Baird, Debra K.

2010-01-01

A miniature pinscher-cross was evaluated for chronic coughing. Computed tomography and bronchoscopy revealed severe, diffuse, cylindrical bronchiectasis secondary to eosinophilic bronchopneumopathy. Computed tomography is the gold standard for diagnosis of bronchiectasis in humans, and should be further investigated in dogs as a means of characterizing severity and pattern of disease. PMID:20885829

The Role of the Immune Response in the Pathogenesis of Bronchiectasis.

PubMed

King, Paul T

2018-01-01

Bronchiectasis is a prevalent respiratory condition characterised by permanent and abnormal dilation of the lung airways (bronchi). There are a large variety of causative factors that have been identified for bronchiectasis; all of these compromise the function of the immune response to fight infection. A triggering factor may lead to the establishment of chronic infection in the lower respiratory tract. The bacteria responsible for the lower respiratory tract infection are usually found as commensals in the upper respiratory tract microbiome. The consequent inflammatory response to infection is largely responsible for the pathology of this condition. Both innate and adaptive immune responses are activated. The literature has highlighted the central role of neutrophils in the pathogenesis of bronchiectasis. Proteases produced in the lung by the inflammatory response damage the airways and lead to the pathological dilation that is the pathognomonic feature of bronchiectasis. The small airways demonstrate infiltration with lymphoid follicles that may contribute to localised small airway obstruction. Despite aggressive treatment, most patients will have persistent disease. Manipulating the immune response in bronchiectasis may potentially have therapeutic potential.

European Respiratory Society guidelines for the management of adult bronchiectasis.

PubMed

Polverino, Eva; Goeminne, Pieter C; McDonnell, Melissa J; Aliberti, Stefano; Marshall, Sara E; Loebinger, Michael R; Murris, Marlene; Cantón, Rafael; Torres, Antoni; Dimakou, Katerina; De Soyza, Anthony; Hill, Adam T; Haworth, Charles S; Vendrell, Montserrat; Ringshausen, Felix C; Subotic, Dragan; Wilson, Robert; Vilaró, Jordi; Stallberg, Bjorn; Welte, Tobias; Rohde, Gernot; Blasi, Francesco; Elborn, Stuart; Almagro, Marta; Timothy, Alan; Ruddy, Thomas; Tonia, Thomy; Rigau, David; Chalmers, James D

2017-09-01

Bronchiectasis in adults is a chronic disorder associated with poor quality of life and frequent exacerbations in many patients. There have been no previous international guidelines.The European Respiratory Society guidelines for the management of adult bronchiectasis describe the appropriate investigation and treatment strategies determined by a systematic review of the literature.A multidisciplinary group representing respiratory medicine, microbiology, physiotherapy, thoracic surgery, primary care, methodology and patients considered the most relevant clinical questions (for both clinicians and patients) related to management of bronchiectasis. Nine key clinical questions were generated and a systematic review was conducted to identify published systematic reviews, randomised clinical trials and observational studies that answered these questions. We used the GRADE approach to define the quality of the evidence and the level of recommendations. The resulting guideline addresses the investigation of underlying causes of bronchiectasis, treatment of exacerbations, pathogen eradication, long term antibiotic treatment, anti-inflammatories, mucoactive drugs, bronchodilators, surgical treatment and respiratory physiotherapy.These recommendations can be used to benchmark quality of care for people with bronchiectasis across Europe and to improve outcomes. Copyright ©ERS 2017.

The Multiple Faces of Non-Cystic Fibrosis Bronchiectasis. A Cluster Analysis Approach.

PubMed

Martínez-García, Miguel Á; Vendrell, Montserrat; Girón, Rosa; Máiz-Carro, Luis; de la Rosa Carrillo, David; de Gracia, Javier; Olveira, Casilda

2016-09-01

The clinical presentation and prognosis of non-cystic fibrosis bronchiectasis are both very heterogeneous. To identify different clinical phenotypes for non-cystic fibrosis bronchiectasis and their impact on prognosis. Using a standardized protocol, we conducted a multicenter observational cohort study at six Spanish centers with patients diagnosed with non-cystic fibrosis bronchiectasis before December 31, 2005, with a 5-year follow-up from the bronchiectasis diagnosis. A cluster analysis was used to classify the patients into homogeneous groups by means of significant variables corresponding to different aspects of bronchiectasis (clinical phenotypes): age, sex, body mass index, smoking habit, dyspnea, macroscopic appearance of sputum, number of exacerbations, chronic colonization with Pseudomonas aeruginosa, FEV1, number of pulmonary lobes affected, idiopathic bronchiectasis, and associated chronic obstructive pulmonary disease. Survival analysis (Kaplan-Meier method and log-rank test) was used to evaluate the comparative survival of the different subgroups. A total of 468 patients with a mean age of 63 (15.9) years were analyzed. Of these, 58% were females, 39.7% had idiopathic bronchiectasis, and 29.3% presented with chronic Pseudomonas aeruginosa colonization. Cluster analysis showed four clinical phenotypes: (1) younger women with mild disease, (2) older women with mild disease, (3) older patients with severe disease who had frequent exacerbations, and (4) older patients with severe disease who did not have frequent exacerbations. The follow-up period was 54 months, during which there were 95 deaths. Mortality was low in the first and second groups (3.9% and 7.6%, respectively) and high for the third (37%) and fourth (40.8%) groups. The third cluster had a higher proportion of respiratory deaths than the fourth (77.8% vs. 34.4%; P < 0.001). Using cluster analysis, it is possible to separate patients with bronchiectasis into distinct clinical phenotypes with different prognoses.

Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants

PubMed Central

Birchard, Katherine R.; Lowe, Jared R.; Patrone, Michael V.

2016-01-01

Rationale: Patients with idiopathic bronchiectasis are predominantly female and have an asthenic body morphotype and frequent nontuberculous mycobacterial respiratory infections. They also demonstrate phenotypic features (scoliosis, pectus deformity, mitral valve prolapse) that are commonly seen in individuals with heritable connective tissue disorders. Objectives: To determine whether lumbar dural sac size is increased in patients with idiopathic bronchiectasis as compared with control subjects, and to assess whether dural sac size is correlated with phenotypic characteristics seen in individuals with heritable connective tissue disorders. Methods: Two readers blinded to diagnosis measured anterior–posterior and transverse dural sac diameter using L1–L5 magnetic resonance images of 71 patients with idiopathic bronchiectasis, 72 control subjects without lung disease, 29 patients with cystic fibrosis, and 24 patients with Marfan syndrome. We compared groups by pairwise analysis of means, using Tukey’s method to adjust for multiple comparisons. Dural sac diameter association with phenotypic and clinical features was also tested. Measurements and Main Results: The L1–L5 (average) anterior–posterior dural sac diameter of the idiopathic bronchiectasis group was larger than those of the control group (P < 0.001) and the cystic fibrosis group (P = 0.002). There was a strong correlation between increased dural sac size and the presence of pulmonary nontuberculous mycobacterial infection (P = 0.007) and long fingers (P = 0.003). A trend toward larger dural sac diameter was seen in those with scoliosis (P = 0.130) and those with a family history of idiopathic bronchiectasis (P = 0.149). Conclusions: Individuals with idiopathic bronchiectasis have an enlarged dural sac diameter, which is associated with pulmonary nontuberculous mycobacterial infection, long fingers, and family history of idiopathic bronchiectasis. These findings support our hypothesis that “idiopathic” bronchiectasis development reflects complex genetic variation in heritable connective tissue and associated transforming growth factor-β–related pathway genes. PMID:27409985

Trends and Burden of Bronchiectasis-Associated Hospitalizations in the United States, 1993-2006

PubMed Central

Seitz, Amy E.; Olivier, Kenneth N.; Steiner, Claudia A.; Montes de Oca, Ruben; Holland, Steven M.

2010-01-01

Background: Current data on bronchiectasis prevalence, trends, and risk factors are lacking; such data are needed to estimate the burden of disease and for improved medical care and public health resource allocation. The objective of the present study was to estimate the trends and burden of bronchiectasis-associated hospitalizations in the United States. Methods: We extracted hospital discharge records containing International Classification of Diseases, 9th Revision, Clinical Modification codes for bronchiectasis (494, 494.0, and 494.1) as any discharge diagnosis from the State Inpatient Databases from the Agency for Healthcare Research and Quality. Discharge records were extracted for 12 states with complete and continuous reporting from 1993 to 2006. Results: The average annual age-adjusted hospitalization rate from 1993 to 2006 was 16.5 hospitalizations per 100,000 population. From 1993 to 2006, the age-adjusted rate increased significantly, with an average annual percentage increase of 2.4% among men and 3.0% among women. Women and persons aged > 60 years had the highest rate of bronchiectasis-associated hospitalizations. The median cost for inpatient care was 7,827 US dollars (USD) (range, 13-543,914 USD). Conclusions: The average annual age-adjusted rate of bronchiectasis-associated hospitalizations increased from 1993 to 2006. This study furthers the understanding of the impact of bronchiectasis and demonstrates the need for further research to identify risk factors and reasons for the increasing burden. PMID:20435655

Capsaicin cough sensitivity in bronchiectasis.

PubMed

Torrego, A; Haque, R A; Nguyen, L T; Hew, M; Carr, D H; Wilson, R; Chung, K F

2006-08-01

Bronchiectasis is a suppurative airway disease characterised by persistent cough and sputum production associated with bronchial dilatation. A study was undertaken to determine whether cough sensitivity is increased in bronchiectatic patients. Twenty two patients with bronchiectasis and 20 healthy non-smoking controls matched for age and sex were recruited into the study. Quality of life (Leicester Cough Questionnaire score), total cough symptom score, and extent of bronchiectasis on HRCT scans were recorded. Cough sensitivity was assessed using incremental inhalation of capsaicin concentrations; the concentration at which 5 or more coughs occurred (C5) was recorded. Patients with bronchiectasis had increased sensitivity to capsaicin compared with controls (mean (SE) log10 C5 1.22 (0.20) v 1.89 (0.21); p<0.03). Capsaicin sensitivity correlated positively with the Leicester Cough Questionnaire score (r = 0.64; p = 0.005) and inversely with the total cough symptom score (r = -0.58; p = 0.004), but not with the extent of the disease. It also correlated with forced expiratory volume in 1 second (FEV1) in litres (r = 0.58; p = 0.005) but not with FEV1 % predicted. Capsaicin sensitivity was not related to the presence of infected sputum or to corticosteroid or bronchodilator use. : Patients with bronchiectasis have a sensitive cough reflex which reflects the severity of cough symptoms. A measure of cough severity could be part of health assessment for patients with bronchiectasis.

Bronchoscopy, Imaging, and Concurrent Diseases in Dogs with Bronchiectasis: (2003-2014).

PubMed

Johnson, L R; Johnson, E G; Vernau, W; Kass, P H; Byrne, B A

2016-01-01

Bronchiectasis is a permanent and debilitating sequel to chronic or severe airway injury, however, diseases associated with this condition are poorly defined. To evaluate results of diagnostic tests used to document bronchiectasis and to characterize underlying or concurrent disease processes. Eighty-six dogs that had bronchoscopy performed and a diagnosis of bronchiectasis. Retrospective case series. Radiographs, computed tomography, and bronchoscopic findings were evaluated for features of bronchiectasis. Clinical diagnoses of pneumonia (aspiration, interstitial, foreign body, other), eosinophilic bronchopneumopathy (EBP), and inflammatory airway disease (IAD) were made based on results of history, physical examination, and diagnostic testing, including bronchoalveolar lavage fluid analysis and microbiology. Bronchiectasis was diagnosed in 14% of dogs (86/621) that had bronchoscopy performed. Dogs ranged in age from 0.5 to 14 years with duration of signs from 3 days to 10 years. Bronchiectasis was documented during bronchoscopy in 79/86 dogs (92%), thoracic radiology in 50/83 dogs (60%), and CT in 34/34 dogs (100%). Concurrent airway collapse was detected during bronchoscopy in 50/86 dogs (58%), and focal or multifocal mucus plugging of segmental or subsegmental bronchi was found in 41/86 dogs (48%). Final diagnoses included pneumonia (45/86 dogs, 52%), EBP (10/86 dogs, 12%) and IAD (31/86 dogs, 36%). Bacteria were isolated in 24/86 cases (28%), with Streptococcus spp, Pasteurella spp, enteric organisms, and Stenotrophomonas isolated most frequently. Bronchiectasis can be anticipated in dogs with infectious or inflammatory respiratory disease. Advanced imaging and bronchoscopy are useful in making the diagnosis and identifying concurrent respiratory disease. Copyright © 2015 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

Bronchiectasis in a diverse US population: effects of ethnicity on etiology and sputum culture.

PubMed

McShane, Pamela J; Naureckas, Edward T; Strek, Mary E

2012-07-01

Previous studies of patients with bronchiectasis have found that the cause is idiopathic in the majority of cases, but these studies were done in homogeneous populations. We hypothesized that the etiology of bronchiectasis can be determined in a higher percentage of patients in a diverse US population and will differ significantly based on ethnicity. One hundred twelve patients with bronchiectasis confirmed by chest CT scan entered the study. Data from 106 patients were available for full evaluation. Clinical questionnaire, pulmonary function tests, sputum microbiology, laboratory data, and immune function testing were done. Results were analyzed by ethnicity and etiology. Patients were 61.6% European American (EA), 26.8% African American (AA), 8.9% Hispanic American (HA), and 2.7% Asian American. A cause of bronchiectasis was determined in 93.3% of patients. In 63.2% of patients, bronchiectasis was caused by immune dysregulation, including deficiency (n = 18 [17%]), autoimmune disease (n = 33 [31.1%]), hematologic malignancy (n = 15 [14.2%]), and allergic bronchopulmonary aspergillosis (n = 1 [0.9%]). Rheumatoid arthritis was the cause of bronchiectasis in 28.6% of AA patients vs 6.2% of EA patients (P < .05). Hematologic malignancy was the etiology in 20.0% of the EA patients vs none of the AA patients (P = .02). A significantly higher percentage of HA patients had Pseudomonas aeruginosa in their sputum compared with AA and EA patients (P = .01). The etiology of bronchiectasis can be determined in the majority of patients in a heterogeneous US population and is most often due to immune dysregulation. Rheumatoid arthritis is more likely in AA patients than EA patients. HA patients are more likely to have P aeruginosa in their sputum.

Singing for children and adults with bronchiectasis.

PubMed

Irons, Jung Yoon; Kenny, Dianna Theadora; Chang, Anne B

2010-02-17

Bronchiectasis is a common respiratory disease, especially in developing countries. Its cause varies from chronic infection to rare immune deficiencies. Bronchiectasis can be present with other respiratory diseases, such as chronic obstructive pulmonary disease (COPD). People with bronchiectasis may suffer from chronic cough, fatigue, shortness of breath, chest pain and coughing up blood. Their lung function may decline with time. These can also have a negative impact on their quality of life. Thus, a holistic management is needed to provide treatment and support. Therapies which include breathing manoeuvres, such as singing, may have health benefits for respiratory function and psychological well being. To evaluate the effects of a singing intervention as a therapy on the quality of life, morbidity, respiratory muscle strength and pulmonary function of children and adults with bronchiectasis. We searched the Cochrane Airways Group (CAG) trials register, the Cochrane Central Register of Controlled Trials, major allied complementary databases, and clinical trials registers. Professional organisations and individuals were also contacted. CAG performed searches in February, and additional searches were carried out in June 2009. Randomised controlled trials in which singing (as an intervention) is compared with either a sham intervention or no singing in patients with bronchiectasis. Two authors independently reviewed the titles, abstracts and citations to assess potential relevance for full review. No eligible trials were identified and thus no data were available for analysis. No meta-analysis could be performed. In the absence of data, we cannot draw any conclusion to support or refute the adoption of singing as an intervention for people with bronchiectasis. Given the simplicity of the potentially beneficial intervention, future randomised controlled trials are required to evaluate singing therapy for people with bronchiectasis.

Role of vitamin D in cystic fibrosis and non-cystic fibrosis bronchiectasis

PubMed Central

Moustaki, Maria; Loukou, Ioanna; Priftis, Kostas N; Douros, Konstantinos

2017-01-01

Bronchiectasis is usually classified as cystic fibrosis (CF) related or CF unrelated (non-CF); the latter is not considered an orphan disease any more, even in developed countries. Irrespective of the underlying etiology, bronchiectasis is the result of interaction between host, pathogens, and environment. Vitamin D is known to be involved in a wide spectrum of significant immunomodulatory effects such as down-regulation of pro-inflammatory cytokines and chemokines. Respiratory epithelial cells constitutively express 1α-hydroxylase leading to the local transformation of the inactive 25(OH)-vitamin D to the active 1,25(OH)2-vitamin D. The latter through its autocrine and paracrine functions up-regulates vitamin D dependent genes with important consequences in the local immunity of lungs. Despite the scarcity of direct evidence on the involvement of vitamin D deficiency states in the development of bronchiectasis in either CF or non-CF patients, it is reasonable to postulate that vitamin D may play some role in the pathogenesis of lung diseases and especially bronchiectasis. The potential contribution of vitamin D deficiency in the process of bronchiectasis is of particular clinical importance, taking into consideration the increasing prevalence of vitamin D deficiency worldwide and the significant morbidity of bronchiectasis. Given the well-established association of vitamin D deficiency with increased inflammation, and the indicative evidence for harmful consequences in lungs, it is intriguing to speculate that the administration of vitamin D supplementation could be a reasonable and cost effective supplementary therapeutic approach for children with non-CF bronchiectasis. Regarding CF patients, maybe in the future as more data become available, we have to re-evaluate our policy on the most appropriate dosage scheme for vitamin D. PMID:28828295

Changing epidemiology of non-cystic fibrosis bronchiectasis.

PubMed

Bahçeci, Semiha; Karaman, Sait; Nacaroğlu, Hikmet Tekin; Yazıcı, Selçuk; Girit, Saniye; Ünsal-Karkıner, Şule; Can, Demet

2016-01-01

Non-cystic fibrosis bronchiectasis again becomes a major health problem due to inappropriate antibiotic use and increasing frequency of protracted bacterial bronchitis. The aim was to determine the changes in etiology of bronchiectasis. Patients who admitted to Behçet Uz Children Hospital between 2005 and 2015 (n=110) were retrospectively examined. The etiology of bronchiectasis was detected as; primary ciliary dyskinesia 26.4%, protracted bacterial bronchitis 22.8%, primary immune deficiency 11.8%, bronchiolitis obliterans 8.2%, lung disease secondary to gastro-esophageal reflux 3.7%, foreign body aspiration 2.7%, tuberculosis %2.7, congenital malformation 1.8% and asthma 1.8%, respectively. In 15.4% of cases, etiology was not identified clearly. 91% of the patients were medically treated. In ten years, the frequency of asthma and tuberculosis in etiology had decreased but primary ciliary dyskinesia and primary immune deficiency had increased. Non-cystic fibrosis bronchiectasis can be followed up for a long time with medical treatment.

National BTS bronchiectasis audit 2012: is the quality standard being adhered to in adult secondary care?

PubMed

Hill, Adam T; Routh, Chris; Welham, Sally

2014-03-01

A significant step towards improving care of patients with non-cystic fibrosis bronchiectasis was the creation of the British Thoracic Society (BTS) national guidelines and the quality standard. A BTS bronchiectasis audit was conducted between 1 October and 30 November 2012, in adult patients with bronchiectasis attending secondary care, against the BTS quality standard. Ninety-eight institutions took part, submitting a total of 3147 patient records. The audit highlighted the variable adoption of the quality standard. It will allow the host institutions to benchmark against UK figures and drive quality improvement programmes to promote the quality standard and improve patient care.

Association between environmental factors and hospitalisations for bronchiectasis in Badalona, Barcelona, Spain (2007-2015).

PubMed

Garcia-Olivé, Ignasi; Radua, Joaquim; Sánchez-Berenguer, Dan; Hernández-Biette, Agnes; Raya-Márquez, Patricia; Stojanovic, Zoran; Martínez-Rivera, Carlos; Fernandez Serrano, Silvia; Ruiz Manzano, Juan

2018-04-13

The relationship between environmental factors and the exacerbation of respiratory diseases has been widely studied. However, there are no studies examining the relationship between these factors and bronchiectasis exacerbations. Our objective was to analyse the association between various environmental factors and hospitalisation for bronchiectasis. This was a retrospective observational study conducted at two hospitals in Badalona (Barcelona). The number of hospital admissions for exacerbation of bronchiectasis between 2007 and 2015 was obtained. Through multiple regression we analysed the relationship between the number of exacerbations and mean monthly values of temperature, SO 2 , NO, NO 2 , O 3 and CO. Temperature, SO 2 , NO, NO 2 , O 3 and CO were significantly associated with an increase in admissions due to exacerbation of bronchiectasis. By controlling the effect of temperature on the pollution variables, only SO 2 maintained statistical significance (P=.008). We have detected an increase in hospital admissions for exacerbation of bronchiectasis with increases in the atmospheric concentration of SO 2 and the decrease in temperature. Prospective studies with different geographical locations to confirm these results are needed. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

To investigate the prevention of OM-85 on bronchiectasis exacerbations (iPROBE) in Chinese patients: study protocol for a randomized controlled trial

PubMed Central

2014-01-01

Background Non-cystic fibrosis bronchiectasis is characterized by the irreversible dilatation of the medium-sized bronchi as a result of airway injury from recurrent or chronic inflammation and lower respiratory tract infections. Bronchiectasis airways are commonly colonized with bacterial species. Infections of the airways play important role in bronchiectasis exacerbations. The non-specific prevention of recurrent airway infections by immunostimulating agents has gained growing interest. OM-85, consisting of extracts of eight kinds of bacteria important in respiratory infections, could support the respiratory tract resistance to the pathogens. OM-85 has been shown to be a benefit by decreasing the risk of acute exacerbation of chronic obstructive pulmonary disease (COPD) in several perspective clinical trials. Exacerbation of bronchiectasis substantially contributes to a more rapid decline in lung function, reduced quality of life, and healthcare costs. In this context, we plan to conduct a clinical trial to investigate the PReventive effect of OM-85 on Bronchiectasis Exacerbation in Chinese patients (iPROBE). Methods/Design This study is designed as a prospective, randomized, double blind, placebo-controlled multicenter trial. A total of 244 patients with bronchiectasis, who have had at least one exacerbation of bronchiectasis in the previous year, will be included. The subjects will randomly receive two courses of 7 mg of OM-85 or a matching placebo. The treatment dose of OM-85 will be one daily capsule taken orally for 10 days each month for 3 consecutive months at the beginning of the study, followed by 3 months of no drug. This schedule will repeat until the patient has been seen for one year. Discussion We will investigate whether long-term treatment with an oral immunostimulant (OM-85) could decrease exacerbations of bronchiectasis over a one-year period. We will also assess other relevant outcomes, including the rate of event-based exacerbation, lung function parameters, and total scores judged by the St George’s respiratory questionnaire, Leicester cough questionnaire, and inflammatory index. We hope that this study will provide new information on the preventive effects of OM-85 on bronchiectasis exacerbations and will address a knowledge gap for this understudied disease. Trial registration This study is registered at http://www.clinicaltrials.gov (identifier NCT01968421) on 19 October 2013. PMID:24773830

The etiologies of non-CF bronchiectasis in childhood: a systematic review of 989 subjects.

PubMed

Brower, Kelly S; Del Vecchio, Michael T; Aronoff, Stephen C

2014-12-10

While cystic fibrosis (CF) is the most common cause of bronchiectasis in childhood, non-CF bronchiectasis is associated with a wide variety of disorders. The objective of this study was to determine the relative prevalence and specific etiologies on non-CF bronchiectasis in childhood. EMBASE, Medline, OVID Cochrane Reviews, Directory of Open Access Journals, Open Science Directory, EPSCO information services, and OAlster were searched electronically and the bibliographies of selected studies were searched manually. The search was conducted independently by 2 authors. (1) any clinical trial, observational study or cross-sectional case series of 10 or more patients with a description of the conditions associated with bronchiectasis; (2) subjects aged 21 years or younger; (3) cystic fibrosis was excluded and; (4) the diagnosis was confirmed by computed tomography of the chest. Patient number, age range, inclusion criteria, diagnostic criteria, patient source, and categorical and specific etiology. From 491 studies identified, 12 studies encompassing 989 children with non-CF bronchiectasis were selected. Sixty-three percent of the subjects had an underlying disorder. Infectious (17%), primary immunodeficiency (16%), aspiration (10%), ciliary dyskinesia (9%), congenital malformation (3%), and secondary immunodeficiency (3%) were the most common disease categories; 999 etiologies were identified. Severe pneumonia of bacterial or viral etiology and B cell defects were the most common disorders identified. The majority of children with non-CF bronchiectasis have an underlying disorder. A focused history and laboratory investigated is recommended.

Effects of pulmonary rehabilitation in bronchiectasis: A retrospective study.

PubMed

Ong, H K; Lee, A L; Hill, C J; Holland, A E; Denehy, L

2011-01-01

There is limited information about the benefits of pulmonary rehabilitation (PR) in patients with bronchiectasis. This study aimed to evaluate the effects of an out-patient PR program in patients with a primary diagnosis of bronchiectasis and to compare them with a matched COPD group who completed the same PR program. A retrospective review was conducted of patients with bronchiectasis or COPD who completed 6 to 8 weeks of PR at two tertiary institutions. The outcome measures were the 6-minute walk distance (6MWD) and Chronic Respiratory Disease Questionnaire (CRQ). Ninety-five patients with bronchiectasis completed the PR (48 male; FEV(1) 63 [24] % predicted; age 67 [10] years). Significant improvements in 6MWD (mean change 53.4 m, 95% CI 45.0 to 61.7) and CRQ total score (mean change 14.0 units, 95% CI 11.3 to 16.7) were observed immediately following PR. In patients with complete follow-up (n = 37), these improvements remained significantly higher than baseline at 12 months (20.5 m, 95% CI 1.4 to 39.5 for 6MWD; 12.1 points, 95% CI 5.7 to 18.4 for CRQ total score). The time trend and changes in the 6MWD and CRQ scores were not significantly different between the bronchiectasis and the COPD groups (all p > 0.05). This study supports the inclusion of patients with bronchiectasis in existing PR programs. Further prospective RCTs are warranted to substantiate these findings.

Extrapulmonary features of bronchiectasis: muscle function, exercise capacity, fatigue, and health status.

PubMed

Ozalp, Ozge; Inal-Ince, Deniz; Calik, Ebru; Vardar-Yagli, Naciye; Saglam, Melda; Savci, Sema; Arikan, Hulya; Bosnak-Guclu, Meral; Coplu, Lutfi

2012-06-11

There are limited number of studies investigating extrapulmonary manifestations of bronchiectasis. The purpose of this study was to compare peripheral muscle function, exercise capacity, fatigue, and health status between patients with bronchiectasis and healthy subjects in order to provide documented differences in these characteristics for individuals with and without bronchiectasis. Twenty patients with bronchiectasis (43.5 ± 14.1 years) and 20 healthy subjects (43.0 ± 10.9 years) participated in the study. Pulmonary function, respiratory muscle strength (maximal expiratory pressure - MIP - and maximal expiratory pressure - MEP), and dyspnea perception using the Modified Medical Research Council Dyspnea Scale (MMRC) were determined. A six-minute walk test (6MWT) was performed. Quadriceps muscle, shoulder abductor, and hand grip strength (QMS, SAS, and HGS, respectively) using a hand held dynamometer and peripheral muscle endurance by a squat test were measured. Fatigue perception and health status were determined using the Fatigue Severity Scale (FSS) and the Leicester Cough Questionnaire (LCQ), respectively. Number of squats, 6MWT distance, and LCQ scores as well as lung function testing values and respiratory muscle strength were significantly lower and MMRC and FSS scores were significantly higher in patients with bronchiectasis than those of healthy subjects (p < 0.05). In bronchiectasis patients, QMS was significantly associated with HGS, MIP and MEP (p < 0.05). The 6MWT distance was significantly correlated to LCQ psychological score (p < 0.05). The FSS score was significantly associated with LCQ physical and total and MMRC scores (p < 0.05). The LCQ psychological score was significantly associated with MEP and 6MWT distance (p < 0.05). Peripheral muscle endurance, exercise capacity, fatigue and health status were adversely affected by the presence of bronchiectasis. Fatigue was associated with dyspnea and health status. Respiratory muscle strength was related to peripheral muscle strength and health status, but not to fatigue, peripheral muscle endurance or exercise capacity. These findings may provide insight for outcome measures for pulmonary rehabilitation programs for patients with bronchiectasis.

Depression and anxiety symptoms in bronchiectasis: associations with health-related quality of life.

PubMed

Olveira, Casilda; Olveira, Gabriel; Gaspar, Inmaculada; Dorado, Antonio; Cruz, Ivette; Soriguer, Federico; Quittner, Alexandra L; Espildora, Francisco

2013-04-01

Bronchiectasis causes pulmonary infections and loss of lung function, resulting in chronic respiratory symptoms and worsening health-related quality of life. The aims of this study were to measure symptoms of depression and anxiety in a sample of patients with bronchiectasis and evaluate their relationship to health outcomes and health-related quality of life. This cross-sectional study included adolescents and adults with bronchiectasis. Patients completed the hospital anxiety and depression scale and the St. George respiratory questionnaire. Health outcome data, including clinical, radiological and spirometric values, were recorded from medical charts. Ninety-three participants with bronchiectasis of any aetiology were recruited: 20 % had elevated depression-related scores and 38 % had elevated anxiety-related scores. Increased symptoms of depression and anxiety were significantly associated with age; anxiety was associated with more frequent exacerbations. Regression analyses indicated that after controlling for demographic (gender and age) and clinical variables (exacerbations frequency, daily sputum, aetiology and spirometry), both depression and anxiety symptoms predicted significantly worse health-related quality of life. In comparison with other predictors, psychological symptoms explained the largest amount of variance in health-related quality of life. Symptoms of depression and anxiety were significant predictors of health-related quality of life in patients with bronchiectasis, independently of respiratory involvement, gender, age or other variables.

Viruses in bronchiectasis: a pilot study to explore the presence of community acquired respiratory viruses in stable patients and during acute exacerbations.

PubMed

Mitchell, Alicia B; Mourad, Bassel; Buddle, Lachlan; Peters, Matthew J; Oliver, Brian G G; Morgan, Lucy C

2018-05-22

Bronchiectasis is a chronic respiratory condition. Persistent bacterial colonisation in the stable state with increased and sometimes altered bacterial burden during exacerbations are accepted as key features in the pathophysiology. The extent to which respiratory viruses are present during stable periods and in exacerbations is less well understood. This study aimed to determine the incidence of respiratory viruses within a cohort of bronchiectasis patients with acute exacerbations at a teaching hospital and, separately, in a group of patients with stable bronchiectasis. In the group of stable patients, a panel of respiratory viruses were assayed for using real time quantitative PCR in respiratory secretions and exhaled breath. The Impact of virus detection on exacerbation rates and development of symptomatic infection was evaluated. Routine hospital-based viral PCR testing was only requested in 28% of admissions for an exacerbation. In our cohort of stable bronchiectasis patients, viruses were detected in 92% of patients during the winter season, and 33% of patients during the summer season. In the 2-month follow up period, 2 of 27 patients presented with an exacerbation. This pilot study demonstrated that respiratory viruses are commonly detected in patients with stable bronchiectasis. They are frequently detected during asymptomatic viral periods, and multiple viruses are often present concurrently.

Quantitative and qualitative computed tomographic characteristics of bronchiectasis in 12 dogs.

PubMed

Cannon, Matthew S; Johnson, Lynelle R; Pesavento, Patricia A; Kass, Philip H; Wisner, Erik R

2013-01-01

Bronchiectasis is an irreversible dilatation of the bronchi resulting from chronic airway inflammation. In people, computed tomography (CT) has been described as the noninvasive gold standard for diagnosing bronchiectasis. In dogs, normal CT bronchoarterial ratios have been described as <2.0. The purpose of this retrospective study was to describe quantitative and qualitative CT characteristics of bronchiectasis in a cohort of dogs with confirmed disease. Inclusion criteria for the study were thoracic radiography, thoracic CT, and a diagnosis of bronchiectasis based on bronchoscopy and/or histopathology. For each included dog, a single observer measured CT bronchoarterial ratios at 6 lobar locations. Qualitative thoracic radiography and CT characteristics were recorded by consensus opinion of two board-certified veterinary radiologists. Twelve dogs met inclusion criteria. The mean bronchoarterial ratio from 28 bronchiectatic lung lobes was 2.71 ± 0.80 (range 1.4 to 4.33), and 23/28 measurements were >2.0. Averaged bronchoarterial ratios from bronchiectatic lung lobes were significantly larger (P < 0.01) than averaged ratios from nonbronchiectatic lung lobes. Qualitative CT characteristics of bronchiectasis included lack of peripheral airway tapering (12/12), lobar consolidation (11/12), bronchial wall thickening (7/12), and bronchial lumen occlusion (4/12). Radiographs detected lack of airway tapering in 7/12 dogs. In conclusion, the most common CT characteristics of bronchiectasis were dilatation, a lack of peripheral airway tapering, and lobar consolidation. Lack of peripheral airway tapering was not visible in thoracic radiographs for some dogs. For some affected dogs, bronchoarterial ratios were less than published normal values. © 2013 Veterinary Radiology & Ultrasound.

The heterogeneity of systemic inflammation in bronchiectasis.

PubMed

Saleh, Aarash D; Chalmers, James D; De Soyza, Anthony; Fardon, Thomas C; Koustas, Spiro O; Scott, Jonathan; Simpson, A John; Brown, Jeremy S; Hurst, John R

2017-06-01

Systemic inflammation in bronchiectasis is poorly studied in relation to aetiology and severity. We hypothesized that molecular patterns of inflammation may define particular aetiology and severity groups in bronchiectasis. We assayed blood concentrations of 31 proteins from 90 bronchiectasis patients (derivation cohort) and conducted PCA to examine relationships between these markers, disease aetiology and severity. Key results were validated in two separate cohorts of 97 and 79 patients from other centres. There was significant heterogeneity in protein concentrations across the derivation population. Increasing severity of bronchiectasis (BSI) was associated with increasing fibrinogen (rho = 0.34, p = 0.001 -validated in a second cohort), and higher fibrinogen was associated with worse lung function, Pseudomonas colonisation and impaired health-status. There were generally similar patterns of inflammation in patients with idiopathic and post-infectious disease. However, patients with primary immunodeficiency had exaggerated IL-17 responses, validated in a second cohort (n = 79, immunodeficient 12.82 pg/ml versus idiopathic/post-infectious 4.95 pg/ml, p = 0.001), and thus IL-17 discriminated primary immunodeficiency from other aetiologies (AUC 0.769 (95%CI 0.661-0.877)). Bronchiectasis is associated with heterogeneity of systemic inflammatory proteins not adequately explained by differences in disease aetiology or severity. More severe disease is associated with enhanced acute-phase responses. Plasma fibrinogen was associated with bronchiectasis severity in two cohorts, Pseudomonas colonisation and health status, and offers potential as a useful biomarker. Crown Copyright © 2017. Published by Elsevier Ltd. All rights reserved.

Secreted mucins and airway bacterial colonization in non-CF bronchiectasis.

PubMed

Sibila, Oriol; Suarez-Cuartin, Guillermo; Rodrigo-Troyano, Ana; Fardon, Thomas C; Finch, Simon; Mateus, Eder Freddy; Garcia-Bellmunt, Laia; Castillo, Diego; Vidal, Silvia; Sanchez-Reus, Ferran; Restrepo, Marcos I; Chalmers, James D

2015-10-01

Secreted mucins play a key role in antibacterial defence in the airway, but have not previously been characterized in non-cystic fibrosis (CF) bronchiectasis patients. We aim to investigate the relationship between secreted mucins levels and the presence of bacterial colonization due to potentially pathogenic microorganisms (PPM) in the airways of stable bronchiectasis patients. Clinically stable bronchiectasis patients were studied prospectively at two centres. Patients with other pulmonary conditions were excluded. Spontaneous sputum was subject to bacterial culture, and secreted mucins (MUC2, MUC5AC and MUC5B) were measured in sputum supernatants by ELISA. A total of 50 patients were included. PPM were identified from sputum samples in 30 (60%), with Pseudomonas aeruginosa (n = 10) and Haemophilus influenzae (n = 10) as the most common PPM. There were no baseline differences among airway colonized and non-colonized patients. Patients with airways colonized by PPM presented higher levels of airway MUC2. No differences in MUC5AC levels were found among groups, whereas MUC5B levels were undetectable. Patients with P. aeruginosa colonization expressed the highest levels of MUC2. High levels of MUC2 and MUC5AC are also correlated with disease severity using the Bronchiectasis Severity Index. Airway MUC2 levels were higher in bronchiectasis patients colonized with PPM compared with those without airway colonization, especially in patients with P. aeruginosa. These findings suggest that airway-secreted mucins levels may play a role in the pathogenesis of airway infection in non-CF bronchiectasis. © 2015 Asian Pacific Society of Respirology.

Risk factors for bronchiectasis in children with cystic fibrosis.

PubMed

Sly, Peter D; Gangell, Catherine L; Chen, Linping; Ware, Robert S; Ranganathan, Sarath; Mott, Lauren S; Murray, Conor P; Stick, Stephen M

2013-05-23

Bronchiectasis develops early in the course of cystic fibrosis, being detectable in infants as young as 10 weeks of age, and is persistent and progressive. We sought to determine risk factors for the onset of bronchiectasis, using data collected by the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) intensive surveillance program. We examined data from 127 consecutive infants who received a diagnosis of cystic fibrosis after newborn screening. Chest computed tomography (CT) and bronchoalveolar lavage (BAL) were performed, while the children were in stable clinical condition, at 3 months and 1, 2, and 3 years of age. Longitudinal data were used to determine risk factors associated with the detection of bronchiectasis from 3 months to 3 years of age. The point prevalence of bronchiectasis at each visit increased from 29.3% at 3 months of age to 61.5% at 3 years of age. In multivariate analyses, risk factors for bronchiectasis were presentation with meconium ileus (odds ratio, 3.17; 95% confidence interval [CI], 1.51 to 6.66; P=0.002), respiratory symptoms at the time of CT and BAL (odds ratio, 2.27; 95% CI, 1.24 to 4.14; P=0.008), free neutrophil elastase activity in BAL fluid (odds ratio, 3.02; 95% CI, 1.70 to 5.35; P<0.001), and gas trapping on expiratory CT (odds ratio, 2.05; 95% CI, 1.17 to 3.59; P=0.01). Free neutrophil elastase activity in BAL fluid at 3 months of age was associated with persistent bronchiectasis (present on two or more sequential scans), with the odds seven times as high at 12 months of age and four times as high at 3 years of age. Neutrophil elastase activity in BAL fluid in early life was associated with early bronchiectasis in children with cystic fibrosis. (Funded by the National Health and Medical Research Council of Australia and Cystic Fibrosis Foundation Therapeutics.)

Airway clearance techniques for bronchiectasis.

PubMed

Lee, Annemarie L; Burge, Angela; Holland, Anne E

2013-05-31

People with non-cystic fibrosis bronchiectasis commonly experience chronic cough and sputum production and these features may be associated with progressive decline in clinical status. Airway clearance techniques (ACTs) are often prescribed to facilitate expectoration of sputum from the lungs, but the efficacy of these techniques in a stable clinical state or during an acute exacerbation of bronchiectasis is unclear. Primary: to determine the effects of ACTs on the rate of acute exacerbations, incidence of hospitalisation and health-related quality of life in individuals with acute and stable bronchiectasis.Secondary: to determine whether a) ACTs are safe for individuals with acute and stable bronchiectasis and b) ACTs have beneficial effects on physiology and symptoms in individuals with acute and stable bronchiectasis. We searched the Cochrane Airways Group Specialised Register of trials from inception to October 2012, PEDro in October 2012 and handsearched relevant journals. Randomised controlled parallel and cross-over trials that compared an ACT to no treatment, sham ACT or directed coughing in participants with bronchiectasis. We used standard methodological procedures expected by The Cochrane Collaboration. Five studies involving 51 participants met the inclusion criteria of the review, all of which were cross-over design. Four studies were on adults with stable bronchiectasis, and the other study was on clinically stable children with bronchiectasis. Three studies were single treatment sessions, two were longer-term studies. The interventions varied and some control groups received a sham intervention while others were inactive. The methodological quality of the studies was variable and the studies were not able to blind participants and personal. Heterogeneity between studies precluded these data from meta-analysis and the review was therefore narrative.One study on 20 adults comparing an airway oscillatory device with no treatment found no significant difference in the number of exacerbations at 12 weeks (low-quality evidence). No data were available to assess the impact of ACTs on the time to exacerbation, duration of, incidence of hospitalisation or total number of hospitalised days. The same study reported clinically significant improvements in health-related quality of life in both disease-specific and cough-related measures. While based on a small number of participants and the data were skewed, the median difference in the change in total St George's Respiratory Questionnaire (SGRQ) score over three months in this study was 8.5 units (P value = 0.005 (Wilcoxon), low-quality evidence). Two studies reported mean increases in volume of sputum expectorated with airway oscillatory devices in the short term of 8.4 mL (95% CI 3.4 to 13.4 mL) and in the long term of 3 mL (P value = 0.02), with no significant effect on lung function. One study reported an immediate reduction in pulmonary hyperinflation in adults with non-positive expiratory pressure (PEP) ACTs (difference in functional residual capacity (FRC) of 19%, P value < 0.05) and with airway oscillatory devices (difference in FRC of 30%, P value < 0.05) compared to no ACTs. A similar decrease in pulmonary hyperinflation (difference in FRC of 6%) was found in children using an airway oscillatory device for 3 months compared to sham therapy. No studies reported on the effects of gas exchange, people's symptoms or antibiotic usage. ACTs appear to be safe for individuals (adults and children) with stable bronchiectasis, where there may be improvements in sputum expectoration, selected measures of lung function and health-related quality of life. The role of these techniques in people with an acute exacerbation of bronchiectasis is unknown. In view of the chronic nature of bronchiectasis, more data are needed to establish the clinical value of ACTs over the short and long term on patient-important outcomes, including symptoms, on physiological outcomes which may clarify the rationale for each technique and on long-term parameters that impact on disease progression in individuals with stable bronchiectasis. This is necessary in order to provide further guidance of specific ACT prescription for people with bronchiectasis. It may also be important to establish the comparative effect of different types of ACTs in people with bronchiectasis.

Assessment of CF lung disease using motion corrected PROPELLER MRI: a comparison with CT.

PubMed

Ciet, Pierluigi; Serra, Goffredo; Bertolo, Silvia; Spronk, Sandra; Ros, Mirco; Fraioli, Francesco; Quattrucci, Serena; Assael, M Baroukh; Catalano, Carlo; Pomerri, Fabio; Tiddens, Harm A W M; Morana, Giovanni

2016-03-01

To date, PROPELLER MRI, a breathing-motion-insensitive technique, has not been assessed for cystic fibrosis (CF) lung disease. We compared this technique to CT for assessing CF lung disease in children and adults. Thirty-eight stable CF patients (median 21 years, range 6-51 years, 22 female) underwent MRI and CT on the same day. Study protocol included respiratory-triggered PROPELLER MRI and volumetric CT end-inspiratory and -expiratory acquisitions. Two observers scored the images using the CF-MRI and CF-CT systems. Scores were compared with intra-class correlation coefficient (ICC) and Bland-Altman plots. The sensitivity and specificity of MRI versus CT were calculated. MRI sensitivity for detecting severe CF bronchiectasis was 0.33 (CI 0.09-0.57), while specificity was 100% (CI 0.88-1). ICCs for bronchiectasis and trapped air were as follows: MRI-bronchiectasis (0.79); CT-bronchiectasis (0.85); MRI-trapped air (0.51); CT-trapped air (0.87). Bland-Altman plots showed an MRI tendency to overestimate the severity of bronchiectasis in mild CF disease and underestimate bronchiectasis in severe disease. Motion correction in PROPELLER MRI does not improve assessment of CF lung disease compared to CT. However, the good inter- and intra-observer agreement and the high specificity suggest that MRI might play a role in the short-term follow-up of CF lung disease (i.e. pulmonary exacerbations). PROPELLER MRI does not match CT sensitivity to assess CF lung disease. PROPELLER MRI has lower sensitivity than CT to detect severe bronchiectasis. PROPELLER MRI has good to very good intra- and inter-observer variability. PROPELLER MRI can be used for short-term follow-up studies in CF.

Fat-free mass depletion and inflammation in patients with bronchiectasis.

PubMed

Olveira, Gabriel; Olveira, Casilda; Gaspar, Inmaculada; Porras, Nuria; Martín-Núñez, Gracia; Rubio, Elehazara; Colomo, Natalia; Rojo-Martínez, Gemma; Soriguer, Federico

2012-12-01

Fat-free mass depletion has been related to increased inflammatory activity and to increased morbidity and mortality in chronic respiratory diseases. The aims of our study were to determine the nutritional status and serum levels of adipocytokines and inflammatory cytokines in patients with bronchiectasis of any etiology and their relation with respiratory parameters. A cross-sectional study was designed that included patients aged >14 years with diagnostic criteria for bronchiectasis. Anthropometric parameters; a diet questionnaire; hand grip dynamometry; levels of leptin, adiponectin, interleukin-6 (IL-6), tumor necrosis factor-α, and ultrasensitive C-reactive protein; as well as respiratory parameters (ie, clinical, radiologic, and spirometric values) were assessed. Ninety-three clinically stable patients were recruited, 43 with cystic fibrosis, 31 with noncystic fibrosis bronchiectasis, and 19 with cystic fibrosis transmembrane conductance regulator-related bronchiectasis. Fat-free mass depletion was present in 31% of patients, with no differences according to the etiology of the bronchiectasis. Correlations were found between inflammatory cytokines (ie, IL-6) and exacerbations, bronchorrea, forced expiratory volume in 1 second, and Bhalla score. Patients with worse respiratory disease severity, malnutrition, and diabetes had significantly higher levels of IL-6. Adiponectin correlated significantly and positively with fat mass and fat mass index and negatively with fat-free mass, fat-free mass index, and hand dynamometry. Leptin correlated positively with body mass index, fat mass and fat mass index, and negatively with fat-free mass, fat-free mass index, and dynamometry. Patients with bronchiectasis present a high percentage of fat-free mass depletion, independent of the etiology of the disease. The levels of inflammatory cytokines (especially IL-6) may be useful markers of disease severity. Adiponectin levels were higher in patients with fat-free mass depletion. Copyright © 2012 Academy of Nutrition and Dietetics. Published by Elsevier Inc. All rights reserved.

Anxiety and depression in adult outpatients with bronchiectasis: Associations with disease severity and health-related quality of life.

PubMed

Gao, Yong-Hua; Guan, Wei-Jie; Zhu, Ya-Nan; Chen, Rong-Chang; Zhang, Guo-Jun

2018-04-01

Anxiety and depression might frequently affect bronchiectasis patients, but data in Chinese patients, including their association with disease severity assessed with Bronchiectasis Severity Index (BSI) and FACED score, are limited. To investigate the rate, risk factors, association with disease severity and impact of anxiety and depression on health-related quality of life (HRQoL) in adult outpatients with steady-state bronchiectasis. This cross-sectional study included 163 outpatients (102 females; mean age, 45.8 years) and 80 healthy subjects (47 females; mean age, 47.1 years). Demographic, clinical indices, radiology, spirometry, aetiology, sputum bacteriology, Hospital Anxiety and Depression Scales (HADS), Pittsburgh Sleep Quality Index (PSQI) and St. George's Respiratory Questionnaire (SGRQ) were assessed. Patients with steady-state bronchiectasis had a higher rate of depression (HADS-depression >7) (30.1% vs 10.0%, P = .001) and anxiety (HADS-anxiety >7; 39.9% vs 6.3%, P < .001) compared with healthy subjects. Notably, no significant differences in the rate of anxiety and depression were found across different disease severity, assessed with BSI and FACED score (all P > .05). In multivariate model, factors associated with anxiety included younger age (OR = 1.05), education below college graduate (OR = 4.55) and sleep disturbance (PSQI ≥ 6; OR = 2.95); whereas sleep disturbance was the sole factor associated with depression (OR = 5.98). Patients with either depression or anxiety had more markedly impaired HRQoL affecting most domains than those without. Anxiety and depression are common in bronchiectasis and can negatively affect HRQoL, but not related to disease severity. Prompt assessment and treatment of these mental disorders, regardless of bronchiectasis severity, are advocated and might improve HRQoL. © 2017 John Wiley & Sons Ltd.

Sleep disturbances and health-related quality of life in adults with steady-state bronchiectasis.

PubMed

Gao, Yonghua; Guan, Weijie; Xu, Gang; Lin, Zhiya; Tang, Yan; Lin, Zhimin; Li, Huimin; Gao, Yang; Luo, Qun; Zhong, Nanshan; Chen, Rongchang

2014-01-01

Sleep disturbances are common in patients with chronic lung diseases, but little is known about the prevalence in patients with bronchiectasis. A cross sectional study was conducted to investigate the prevalence and determinants associated with sleep disturbances, and the correlation between sleep disturbances and quality of life (QoL) in adults with steady-state bronchiectasis. One hundred and forty-four bronchiectasis patients and eighty healthy subjects were enrolled. Sleep disturbances, daytime sleepiness, and QoL were measured by utilizing the Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS) and St. George Respiratory Questionnaire (SGRQ), respectively. Demographic, clinical indices, radiology, spirometry, bacteriology, anxiety and depression were also assessed. Adults with steady-state bronchiectasis had a higher prevalence of sleep disturbances (PSQI>5) (57% vs. 29%, P<0.001), but not daytime sleepiness (ESS≥10) (32% vs. 30%, P = 0.76), compared with healthy subjects. In the multivariate model, determinants associated with sleep disturbances in bronchiectasis patients included depression (OR, 10.09; 95% CI, 3.46-29.37; P<0.001), nocturnal cough (OR, 1.89; 95% CI, 1.13-3.18; P = 0.016), aging (OR, 1.04; 95% CI, 1.01-1.07; P = 0.009) and increased 24-hour sputum volume (OR, 2.01; 95% CI, 1.22-3.33; P = 0.006). Patients with sleep disturbances had more significantly impaired QoL affecting all domains than those without. Only 6.2% of patients reported using a sleep medication at least weekly. In adults with steady-state bronchiectasis, sleep disturbances are more common than in healthy subjects and are related to poorer QoL. Determinants associated with sleep disturbances include depression, aging, nighttime cough and increased sputum volume. Assessment and intervention of sleep disturbances are warranted and may improve QoL.

Capsaicin Cough Sensitivity and the Association with Clinical Parameters in Bronchiectasis

PubMed Central

Lin, Zhi-ya; Tang, Yan; Li, Hui-min; Lin, Zhi-min; Zheng, Jin-ping; Chen, Rong-chang; Zhong, Nan-shan

2014-01-01

Background Cough hypersensitivity has been common among respiratory diseases. Objective To determine associations of capsaicin cough sensitivity and clinical parameters in adults with clinically stable bronchiectasis. Methods We recruited 135 consecutive adult bronchiectasis patients and 22 healthy subjects. History inquiry, sputum culture, spirometry, chest high-resolution computed tomography (HRCT), Leicester Cough Questionnaire scoring, Bronchiectasis Severity Index (BSI) assessment and capsaicin inhalation challenge were performed. Cough sensitivity was measured as the capsaicin concentration eliciting at least 2 (C2) and 5 coughs (C5). Results Despite significant overlap between healthy subjects and bronchiectasis patients, both C2 and C5 were significantly lower in the latter group (all P<0.01). Lower levels of C5 were associated with a longer duration of bronchiectasis symptoms, worse HRCT score, higher 24-hour sputum volume, BSI and sputum purulence score, and sputum culture positive for P. aeruginosa. Determinants associated with increased capsaicin cough sensitivity, defined as C5 being 62.5 µmol/L or less, encompassed female gender (OR: 3.25, 95%CI: 1.35–7.83, P<0.01), HRCT total score between 7–12 (OR: 2.57, 95%CI: 1.07–6.173, P = 0.04), BSI between 5–8 (OR: 4.05, 95%CI: 1.48–11.06, P<0.01) and 9 or greater (OR: 4.38, 95%CI: 1.48–12.93, P<0.01). Conclusion Capsaicin cough sensitivity is heightened in a subgroup of bronchiectasis patients and associated with the disease severity. Gender and disease severity, but not sputum purulence, are independent determinants of heightened capsaicin cough sensitivity. Current testing for cough sensitivity diagnosis may be limited because of overlap with healthy subjects but might provide an objective index for assessment of cough in future clinical trials. PMID:25409316

Clinical Determinants of Incremental Shuttle Walk Test in Adults with Bronchiectasis.

PubMed

Yildiz, Sulenur; Inal-Ince, Deniz; Calik-Kutukcu, Ebru; Vardar-Yagli, Naciye; Saglam, Melda; Arikan, Hulya; Coplu, Lutfi

2018-06-01

Exercise capacity is impaired in patients with bronchiectasis. Incremental shuttle walk test (ISWT) stresses cardiorespiratory system physiologically to symptom-limited maximal exercise capacity. The purpose of this study was to investigate the clinical determinants of ISWT in adults with non-cystic fibrosis (CF) bronchiectasis. Forty-one clinically stable bronchiectasis patients aged 18-72 years (27 females, 14 males) participated in the study. Subjects' demographics and physical characteristics were recorded. Bronchiectasis Severity Index was used to identify disease severity. Pulmonary function test was performed. Dyspnea perception was assessed using the modified Medical Research Council Dyspnea Scale. Maximum inspiratory and expiratory pressures were measured. Peripheral muscle strength using a hand held dynamometer was measured. ISWT was performed to determine exercise capacity. Fatigue Severity Scale, Hospital Anxiety and Depression Scale, Leicester Cough Questionnaire were used to determine fatigue, psychosocial status, and quality of life. Patients' mean ISWT distance was 469.5 m. The ISWT distance was significantly related with age (r = - 0.472), height (r = 0.469), gender (r = 0.520), FEV 1 (r = 0.651), and FVC (r = 0.545, p < 0.05). Quadriceps muscle strength was higher in males (p = 0.001) as compared to females. Age and gender were identified as independent predictors of the ISWT, explaining 42% of variance in ISWT distance (r = 0.649, r 2  = 0.421, F (2,38)  = 13.794, p < 0.001). The clinical determinants of ISWT in clinically stable patients with non-CF bronchiectasis are age and gender. Pulmonary function, dyspnea perception, muscle strength, disease severity, fatigue, psychosocial factors, and health-related quality of life seems to have an independent effect on ISWT in this group of patients with bronchiectasis.

Effect of Air Pollution on Exacerbations of Bronchiectasis in Badalona, Spain, 2008-2016.

PubMed

Garcia-Olivé, Ignasi; Stojanovic, Zoran; Radua, Joaquim; Rodriguez-Pons, Laura; Martinez-Rivera, Carlos; Ruiz Manzano, Juan

2018-05-17

Air pollution has been widely associated with respiratory diseases. Nevertheless, the association between air pollution and exacerbations of bronchiectasis has been less studied. To analyze the effect of air pollution on exacerbations of bronchiectasis. This was a retrospective observational study conducted in Badalona. The number of daily hospital admissions and emergency room visits related to exacerbation of bronchiectasis (ICD-9 code 494.1) between 2008 and 2016 was obtained. We used simple Poisson regressions to test the effects of daily mean temperature, SO2, NO2, CO, and PM10 levels on bronchiectasis-related emergencies and hospitalizations on the same day and 1-4 days after. All p values were corrected for multiple comparisons. SO2 was significantly associated with an increase in the number of hospitalizations (lags 0, 1, 2, and 3). None of these associations remained significant after correcting for multiple comparisons. The number of emergency room visits was associated with higher levels of SO2 (lags 0-4). After correcting for multiple comparisons, the association between emergency room visits and SO2 levels was statistically significant for lag 0 (p = 0.043), lag 1 (p = 0.018), and lag 3 (p = 0.050). The number of emergency room visits for exacerbation of bronchiectasis is associated with higher levels of SO2. © 2018 S. Karger AG, Basel.

The generalizability of bronchiectasis randomized controlled trials: A multicentre cohort study.

PubMed

Chalmers, James D; McDonnell, Melissa J; Rutherford, Robert; Davidson, John; Finch, Simon; Crichton, Megan; Dupont, Lieven; Hill, Adam T; Fardon, Thomas C; De Soyza, Anthony; Aliberti, Stefano; Goeminne, Pieter

2016-03-01

Randomized controlled trials (RCTs) for bronchiectasis have experienced difficulties with recruitment and in reaching their efficacy end-points. To estimate the generalizability of such studies we applied the eligibility criteria for major RCTs in bronchiectasis to 6 representative observational European Bronchiectasis cohorts. Inclusion and exclusion criteria from 10 major RCTs were applied in each cohort. Demographics and outcomes were compared between patients eligible and ineligible for RCTs. 1672 patients were included. On average 33.0% were eligible for macrolide trials, 15.0% were eligible for inhaled antibiotic trials, 15.9% for the DNAse study and 47.7% were eligible for a study of dry powder mannitol. Within these groups, some trials were highly selective with only 1-9% of patients eligible. Eligible patients were generally more severe with higher mortality during follow-up (mean 17.2 vs 9.0% for macrolide studies, 19.2%% vs 10.7% for inhaled antibiotic studies), and a higher frequency of exacerbations than ineligible patients. As up to 93% of patients were ineligible for studies, however, numerically more deaths and exacerbations occurred in ineligible patient across studies (mean 56% of deaths occurred in ineligible patients across all studies). Our data suggest that patients enrolled in RCT's in bronchiectasis are only partially representative of patients in clinical practice. The majority of mortality and morbidity in bronchiectasis occurs in patients ineligible for many current trials. Copyright © 2016 Elsevier Ltd. All rights reserved.

[Transport of mucoid mucus in healthy individuals and patients with chronic obstructive pulmonary disease and bronchiectasis].

PubMed

Lima Afonso, J; Tambascio, J; Dutra de Souza, H C; Jardim, J R; Baddini Martinez, J A; Gastaldi, A C

2013-01-01

To characterise and compare the in vitro transport properties of respiratory mucoid secretion in individuals with no lung disease and in stable patients with chronic obstructive pulmonary disease (COPD) and bronchiectasis. Samples of mucus were collected, from 21 volunteers presenting no lung disease who had undergone surgery, from 10 patients presenting chronic COPD, and from 16 patients with bronchiectasis. Mucociliary transport (MCT), transport by cough (SCM), and contact angle (CAM) were evaluated. MCT was found to be greater in healthy individuals (1.0±0.19) than in COPD (0.91±0.17) and bronchiectasis (0.76±0.23) patients (p<0.05), whereas SCM was greater in COPD patients (16.31±7.35 cm) than in patients with bronchiectasis (12.16±6.64 cm) and healthy individuals (10.50±25.8 cm) (p<0.05). No significant differences were observed between the groups regarding CAM. Mucus from healthy individuals allows better mucociliary transport compared to that from patients with lung diseases. However, the mucus from COPD patients allows a better transport by coughing, demonstrating that these individuals have adapted to a defence mechanism compared to patients with bronchiectasis, who have impairment in their ciliary and cough transport mechanisms. Copyright © 2012 Sociedade Portuguesa de Pneumologia. Published by Elsevier España. All rights reserved.

Validation of a Spanish version of the Leicester Cough Questionnaire in non-cystic fibrosis bronchiectasis.

PubMed

Muñoz, Gerard; Buxó, Maria; de Gracia, Javier; Olveira, Casilda; Martinez-Garcia, Miguel Angel; Giron, Rosa; Polverino, Eva; Alvarez, Antonio; Birring, Surinder S; Vendrell, Montserrat

2016-05-01

The Leicester Cough Questionnaire (LCQ) has been validated in non-cystic fibrosis bronchiectasis (NCFBC). The present study aimed to create and validate a Spanish version of the LCQ (LCQ-Sp) in NCFBC. The LCQ-Sp was developed following a standardized protocol. For reliability, we assessed internal consistency and the change in score over a 15-day period in stable state. For responsiveness, we assessed the change in scores between visit 1 and the first exacerbation. For validity, we evaluated convergent validity through correlation with the Saint George's Respiratory Questionnaire (SGRQ) and discriminant validity. Two hundred fifty-nine patients (118 mild bronchiectasis, 90 moderate bronchiectasis and 47 severe bronchiectasis) were included. Internal consistency was high for the total scoring and good for the different domains (Cronbach's α: 0.86-0.91). The test-retest reliability shows an intraclass correlation coefficient of 0.87 for the total score. The mean LCQ-Sp score at visit 1 decreased at the beginning of an exacerbation (15.13 ± 4.06 vs. 12.24 ± 4.64; p < 0.001). The correlation between LCQ-Sp and SGRQ scores was -0.66 (p < 0.01). The differences in the LCQ-Sp total score between the different groups of severity were significant (p < 0.001). The LCQ-Sp discriminates disease severity, is responsive to change when faced with exacerbations and is reliable for use in bronchiectasis. © The Author(s) 2016.

Validation of a Spanish version of the Leicester Cough Questionnaire in non-cystic fibrosis bronchiectasis

PubMed Central

Muñoz, Gerard; Buxó, Maria; de Gracia, Javier; Olveira, Casilda; Martinez-Garcia, Miguel Angel; Giron, Rosa; Polverino, Eva; Alvarez, Antonio; Birring, Surinder S

2016-01-01

The Leicester Cough Questionnaire (LCQ) has been validated in non-cystic fibrosis bronchiectasis (NCFBC). The present study aimed to create and validate a Spanish version of the LCQ (LCQ-Sp) in NCFBC. The LCQ-Sp was developed following a standardized protocol. For reliability, we assessed internal consistency and the change in score over a 15-day period in stable state. For responsiveness, we assessed the change in scores between visit 1 and the first exacerbation. For validity, we evaluated convergent validity through correlation with the Saint George’s Respiratory Questionnaire (SGRQ) and discriminant validity. Two hundred fifty-nine patients (118 mild bronchiectasis, 90 moderate bronchiectasis and 47 severe bronchiectasis) were included. Internal consistency was high for the total scoring and good for the different domains (Cronbach’s α: 0.86–0.91). The test–retest reliability shows an intraclass correlation coefficient of 0.87 for the total score. The mean LCQ-Sp score at visit 1 decreased at the beginning of an exacerbation (15.13 ± 4.06 vs. 12.24 ± 4.64; p < 0.001). The correlation between LCQ-Sp and SGRQ scores was −0.66 (p < 0.01). The differences in the LCQ-Sp total score between the different groups of severity were significant (p < 0.001). The LCQ-Sp discriminates disease severity, is responsive to change when faced with exacerbations and is reliable for use in bronchiectasis. PMID:26902541

Validation of a visual analogue score (LRTI-VAS) in non-CF bronchiectasis.

PubMed

Altenburg, Josje; Wortel, Kim; de Graaff, Casper S; van der Werf, Tjip S; Boersma, Wim G

2016-03-01

Quality of life in patients with non-cystic fibrosis (non-CF) bronchiectasis is largely defined by respiratory symptoms. To date, no disease-specific tool for symptom measurement in this patient group was available. We developed the lower respiratory tract infections - visual analogue scale (LRTI-VAS) in order to quickly and conveniently quantify symptoms in non-CF bronchiectasis. This study aimed to validate LRTI-VAS for use in non-CF bronchiectasis. This study included outpatients with radiologically proven bronchiectasis and no evidence of CF. Results of LRTI-VAS were compared with other markers of disease activity {lung function parameters, oxygen saturation and three health-related quality of life questionnaires [Medical Outcomes Study Short-Form 36 Health Survey (SF-36), St Georges Respiratory Questionnaire (SGRQ) and Leicester Cough Questionnaire (LCQ)]} and validity, reliability and responsiveness were assessed. Thirty stable and 30 exacerbating participants completed the LRTI-VAS questionnaire. When testing for repeatability on two separate occasions, no statistically significant difference between total scores was found {1.4 [standard deviation (SD)] 5.3}, P = 0.16). Internal consistency was high across items (Cronbach's alpha 0.86). Correlation with SGRQ, SF-36 and LCQ total scores was high. Following antibiotic treatment, mean (SD) LRTI-VAS total score improved from 18.1 (SD 9.9) to 26.1 (SD 6.6) (P < 0.001). LRTI-VAS showed excellent validity, reliability and responsiveness to change and therefore appears a reliable tool for symptom measurement in non-CF bronchiectasis. © 2014 John Wiley & Sons Ltd.

Cardiovascular Outcomes after a Respiratory Tract Infection among Adults with Non-Cystic Fibrosis Bronchiectasis: A General Population-based Study.

PubMed

Navaratnam, Vidya; Root, Adrian A; Douglas, Ian; Smeeth, Liam; Hubbard, Richard B; Quint, Jennifer K

2018-03-01

Studies suggest that adults with bronchiectasis are at increased risk of cardiovascular comorbidities. We aimed to quantify the relative risk of incident cardiovascular events after a respiratory tract infection among adults with bronchiectasis. Using UK electronic primary care records, we conducted a within-person comparison using the self-controlled case series method. We calculated the relative risk of first-time cardiovascular events (either first myocardial infarction or stroke) after a respiratory tract infection compared with the individual's baseline risk. Our cohort consisted of 895 adult men and women with non-cystic fibrosis bronchiectasis with a first myocardial infarction or stroke and at least one respiratory tract infection. There was an increased rate of first-time cardiovascular events in the 91-day period after a respiratory tract infection (incidence rate ratio, 1.56; 95% confidence interval, 1.20-2.02). The rate of a first cardiovascular event was highest in the first 3 days after a respiratory tract infection (incidence rate ratio, 2.73; 95% confidence interval, 1.41-5.27). These data suggest that respiratory tract infections are strongly associated with a transient increased risk of first-time myocardial infarction or stroke among people with bronchiectasis. As respiratory tract infections are six times more common in people with bronchiectasis than the general population, the increased risk has a disproportionately greater impact in these individuals. These findings may have implications for including cardiovascular risk modifications in airway infection treatment pathways in this population.

Matrix metalloproteinase activation by free neutrophil elastase contributes to bronchiectasis progression in early cystic fibrosis.

PubMed

Garratt, Luke W; Sutanto, Erika N; Ling, Kak-Ming; Looi, Kevin; Iosifidis, Thomas; Martinovich, Kelly M; Shaw, Nicole C; Kicic-Starcevich, Elizabeth; Knight, Darryl A; Ranganathan, Sarath; Stick, Stephen M; Kicic, Anthony

2015-08-01

Neutrophil elastase is the most significant predictor of bronchiectasis in early-life cystic fibrosis; however, the causal link between neutrophil elastase and airway damage is not well understood. Matrix metalloproteinases (MMPs) play a crucial role in extracellular matrix modelling and are activated by neutrophil elastase. The aim of this study was to assess if MMP activation positively correlates with neutrophil elastase activity, disease severity and bronchiectasis in young children with cystic fibrosis.Total MMP-1, MMP-2, MMP-7, MMP-9, tissue inhibitor of metalloproteinase (TIMP)-2 and TIMP-1 levels were measured in bronchoalveolar lavage fluid collected from young children with cystic fibrosis during annual clinical assessment. Active/pro-enzyme ratio of MMP-9 was determined by gelatin zymography. Annual chest computed tomography imaging was scored for bronchiectasis.A higher MMP-9/TIMP-1 ratio was associated with free neutrophil elastase activity. In contrast, MMP-2/TIMP-2 ratio decreased and MMP-1 and MMP-7 were not detected in the majority of samples. Ratio of active/pro-enzyme MMP-9 was also higher in the presence of free neutrophil elastase activity, but not infection. Across the study cohort, both MMP-9/TIMP-1 and active MMP-9 were associated with progression of bronchiectasis.Both MMP-9/TIMP-1 and active MMP-9 increased with free neutrophil elastase and were associated with bronchiectasis, further demonstrating that free neutrophil elastase activity should be considered an important precursor to cystic fibrosis structural disease. Copyright ©ERS 2015.

Adrenal suppression in bronchiectasis and the impact of inhaled corticosteroids.

PubMed

Holme, J; Tomlinson, J W; Stockley, R A; Stewart, P M; Barlow, N; Sullivan, A L

2008-10-01

The present study identified three patients with bronchiectasis receiving inhaled corticosteroids (ICSs) who had symptomatic adrenal suppression secondary to ICS. The prevalence of adrenal suppression is unknown in bronchiectasis. The frequency of adrenal suppression and the impact of ICS use in bronchiectasis patients were examined. In total, 50 outpatients (33 receiving ICSs) underwent a short Synacthen test and completed a St George's Respiratory Questionnaire (SGRQ). Symptoms of adrenal suppression, steroid use and lung function were compared between subjects who were suppressed and those who were not. Adrenal suppression was evident in 23.5% of subjects who did not receive ICSs and 48.5% of those who did. Basal cortisol and the increments by which cortisol increased 30 min after Synacthen were lower in suppressed than in nonsuppressed subjects. The incremental cortisol rise was negatively correlated with SGRQ impacts and total score, suggesting a worse quality of life in those who had an impaired adrenal response. The greatest frequency of generalised symptoms was seen in the suppressed group. A significant proportion of subjects with bronchiectasis have evidence of adrenal suppression, and this is increased when inhaled corticosteroids are also used. Impairment of the cortisol response to stimulation is associated with poorer health status.

Pseudomonas aeruginosa isolates in severe chronic obstructive pulmonary disease: characterization and risk factors

PubMed Central

2014-01-01

Background Patients with severe chronic obstructive pulmonary disease (COPD) are at increased risk of infection by P. aeruginosa. The specific role of bronchiectasis in both infection and chronic colonization by this microorganism in COPD, however, remains ill defined. To evaluate the prevalence and risk factors for P. aeruginosa recovery from sputum in outpatients with severe COPD, characterizing P. aeruginosa isolates by pulsed-field gel electrophoresis (PFGE) and focusing on the influence of bronchiectasis on chronic colonization in these patients. Methods A case-cohort study of 118 patients with severe COPD attended at a Respiratory Day Unit for an acute infectious exacerbation and followed up over one year. High-resolution CT scans were performed during stability for bronchiectasis assessment and sputum cultures were obtained during exacerbation and stability in all patients. P. aeruginosa isolates were genotyped by PFGE. Determinants of the recovery of P. aeruginosa in sputum and chronic colonization by this microorganism were assessed by multivariate analysis. Results P. aeruginosa was isolated from 41 of the 118 patients studied (34.7%). Five of these 41 patients (12.2%) with P. aeruginosa recovery fulfilled criteria for chronic colonization. In the multivariate analysis, the extent of bronchiectasis (OR 9.8, 95% CI: 1.7 to 54.8) and the number of antibiotic courses (OR 1.7, 95% CI: 1.1 to 2.5) were independently associated with an increased risk of P. aeruginosa isolation. Chronic colonization was unrelated to the presence of bronchiectasis (p=0.75). In patients with chronic colonization the isolates of P. aeruginosa retrieved corresponded to the same clones during the follow-up, and most of the multidrug resistant isolates (19/21) were harbored by these patients. Conclusions The main risk factors for P. aeruginosa isolation in severe COPD were the extent of bronchiectasis and exposure to antibiotics. Over 10% of these patients fulfilled criteria for chronic colonization by P. aeruginosa and showed clonal persistence, independently of the presence of bronchiectasis. PMID:24964956

Sleep Disturbances and Health-Related Quality of Life in Adults with Steady-State Bronchiectasis

PubMed Central

Lin, Zhiya; Tang, Yan; Lin, Zhimin; Li, Huimin; Gao, Yang; Luo, Qun; Zhong, Nanshan; Chen, Rongchang

2014-01-01

Background Sleep disturbances are common in patients with chronic lung diseases, but little is known about the prevalence in patients with bronchiectasis. A cross sectional study was conducted to investigate the prevalence and determinants associated with sleep disturbances, and the correlation between sleep disturbances and quality of life (QoL) in adults with steady-state bronchiectasis. Methods One hundred and forty-four bronchiectasis patients and eighty healthy subjects were enrolled. Sleep disturbances, daytime sleepiness, and QoL were measured by utilizing the Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS) and St. George Respiratory Questionnaire (SGRQ), respectively. Demographic, clinical indices, radiology, spirometry, bacteriology, anxiety and depression were also assessed. Results Adults with steady-state bronchiectasis had a higher prevalence of sleep disturbances (PSQI>5) (57% vs. 29%, P<0.001), but not daytime sleepiness (ESS≥10) (32% vs. 30%, P = 0.76), compared with healthy subjects. In the multivariate model, determinants associated with sleep disturbances in bronchiectasis patients included depression (OR, 10.09; 95% CI, 3.46–29.37; P<0.001), nocturnal cough (OR, 1.89; 95% CI, 1.13–3.18; P = 0.016), aging (OR, 1.04; 95% CI, 1.01–1.07; P = 0.009) and increased 24-hour sputum volume (OR, 2.01; 95% CI, 1.22–3.33; P = 0.006). Patients with sleep disturbances had more significantly impaired QoL affecting all domains than those without. Only 6.2% of patients reported using a sleep medication at least weekly. Conclusions In adults with steady-state bronchiectasis, sleep disturbances are more common than in healthy subjects and are related to poorer QoL. Determinants associated with sleep disturbances include depression, aging, nighttime cough and increased sputum volume. Assessment and intervention of sleep disturbances are warranted and may improve QoL. PMID:25036723

Treatment adherence and health outcomes in patients with bronchiectasis.

PubMed

McCullough, Amanda R; Tunney, Michael M; Quittner, Alexandra L; Elborn, J Stuart; Bradley, Judy M; Hughes, Carmel M

2014-07-01

We aimed to determine adherence to inhaled antibiotics, other respiratory medicines and airway clearance and to determine the association between adherence to these treatments and health outcomes (pulmonary exacerbations, lung function and Quality of Life Questionnaire-Bronchiectasis [QOL-B]) in bronchiectasis after 12 months. Patients with bronchiectasis prescribed inhaled antibiotics for Pseudomonas aeruginosa infection were recruited into a one-year study. Participants were categorised as "adherent" to medication (medication possession ratio ≥80% using prescription data) or airway clearance (score ≥80% in the Modified Self-Reported Medication-Taking Scale). Pulmonary exacerbations were defined as treatment with a new course of oral or intravenous antibiotics over the one-year study. Spirometry and QOL-B were completed at baseline and 12 months. Associations between adherence to treatment and pulmonary exacerbations, lung function and QOL-B were determined by regression analyses. Seventy-five participants were recruited. Thirty-five (53%), 39 (53%) and 31 (41%) participants were adherent to inhaled antibiotics, other respiratory medicines, and airway clearance, respectively. Twelve (16%) participants were adherent to all treatments. Participants who were adherent to inhaled antibiotics had significantly fewer exacerbations compared to non-adherent participants (2.6 vs 4, p = 0.00) and adherence to inhaled antibiotics was independently associated with having fewer pulmonary exacerbations (regression co-efficient = -0.51, 95% CI [-0.81,-0.21], p < 0.001). Adherence to airway clearance was associated with lower QOL-B Treatment Burden (regression co-efficient = -15.46, 95% CI [-26.54, -4.37], p < 0.01) and Respiratory Symptoms domain scores (regression co-efficient = -10.77, 95% CI [-21.45; -0.09], p < 0.05). There were no associations between adherence to other respiratory medicines and any of the outcomes tested. Adherence to treatment was not associated with FEV1 % predicted. Treatment adherence is low in bronchiectasis and affects important health outcomes including pulmonary exacerbations. Adherence should be measured as part of bronchiectasis management and future research should evaluate bronchiectasis-specific adherence strategies.

Fungi in cystic fibrosis and non-cystic fibrosis bronchiectasis.

PubMed

Moss, Richard B

2015-04-01

Bronchiectasis is a pathologic bronchial dilatation with loss of function that can result from multiple inflammatory and infectious injuries to the conducting airways of the lung. Molds, particularly the filamentous fungus Aspergillus fumigatus, have been implicated as a common cause of both cystic fibrosis (CF) and non-CF bronchiectasis, the latter primarily in patients with severe asthma. The pathogenesis of mold-associated bronchiectasis is usually due to atopic sensitization to mold allergens in the presence of active chronic endobronchial fungal infection with host innate and adaptive immune deviation to a Th2-dominated inflammation, a condition known as allergic bronchopulmonary aspergillosis (ABPA) (or allergic bronchopulmonary mycosis if a non-Aspergillus mold is implicated). Diagnostic criteria of ABPA continue to evolve, while treatment relies upon downregulation of the allergic inflammatory response with immunomodulatory agents and antifungal pharmacotherapy. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis and non-cystic fibrosis bronchiectasis.

PubMed

Alyasin, Soheila; Moghtaderi, Mozhgan; Farjadian, Shirin; Babaei, Maryam; Teshnizi, Saeed Hosseini

2018-01-01

Aspergillus sensitization (AS) and allergic bronchopulmonary aspergillosis (ABPA) can occur as a cause of permanent lung damage in patients with cystic fibrosis (CF) and non-CF bronchiectasis. The aim of this study was to determine the frequency of AS and ABPA in patients with CF and non-CF bronchiectasis in southwestern Iran. This cross-sectional study was conducted on 33 patients with CF and 27 patients with non-CF bronchiectasis from southwestern Iran who were referred to Namazi Hospital affiliated to Shiraz University of Medical Sciences from July 2015 to February 2016. Skin prick test to Aspergillus fumigatus, peripheral blood eosinophil count, total serum IgE, specific IgE and IgG against Aspergillus fumigatus as well as radiologic chest studies were done for each patient. Statistical analysis was done by Mann-Whitney U test, Fisher Exact test, and Kappa weighted in SPSS software version 18. Level of significance was set at p<0.05. Nine patients with CF (27.3%) and one patient with non-CF bronchiectasis (3.7%) had positive skin tests to Aspergillus. There was 81.2% agreement between positive skin test and specific IgE to Aspergillus fumigatus (p<0.001). Three patients with CF (9%) met the diagnostic criteria for ABPA, whereas ABPA was not seen in patients with non-CF bronchiectasis. ABPA was low in this study, considering more frequency of AS in patients with cystic fibrosis, clinicians should keep in mind the diagnosis of ABPA for those CF patients that do not respond to usual medical therapy and have positive skin tests to Aspergillus allergens.

Hospital admissions for non-cystic fibrosis bronchiectasis in New Zealand.

PubMed

Bibby, Susan; Milne, Richard; Beasley, Richard

2015-09-04

To investigate hospital admissions for non-cystic fibrosis bronchiectasis during July 1, 2008 to June 30, 2013; and to describe their distribution and annual cost in New Zealand. Admissions with a principal diagnosis of bronchiectasis (ICD10 J47), excluding cystic fibrosis, and length of stay <90 days were analysed by age, sex, ethnicity, socioeconomic deprivation, DHB, re-admissions and seasonality. There were 5,494 admissions with a mean annual rate of 25.7 (age adjusted rate 20.4) per 100,000. Admission rates peaked in childhood and in the elderly, and increased steeply with socioeconomic deprivation. Age-adjusted rates were 38% higher for women, 4.9-fold higher for Māori and 9.1-fold higher for Pacific peoples. Counties Manukau had the highest unadjusted rate for any DHB (49.4 per 100,000). The overall 30 day readmission rate was 12.4%. Admissions peaked in winter and spring. The estimated cost in financial year 2012/13 was NZD 5.34M. Hospital admissions for bronchiectasis are concentrated in socioeconomically disadvantaged young and elderly Māori and Pacific peoples; are more common in winter and spring, and incur a high annual cost. Evidence-based interventions to reduce the disproportionate burden of bronchiectasis in Māori and Pacific children and the elderly is a public health priority.

Airway clearance techniques for bronchiectasis.

PubMed

Lee, Annemarie L; Burge, Angela T; Holland, Anne E

2015-11-23

People with non-cystic fibrosis bronchiectasis commonly experience chronic cough and sputum production, features that may be associated with progressive decline in clinical and functional status. Airway clearance techniques (ACTs) are often prescribed to facilitate expectoration of sputum from the lungs, but the efficacy of these techniques in a stable clinical state or during an acute exacerbation of bronchiectasis is unclear. Primary: to determine effects of ACTs on rates of acute exacerbation, incidence of hospitalisation and health-related quality of life (HRQoL) in individuals with acute and stable bronchiectasis. Secondary: to determine whether:• ACTs are safe for individuals with acute and stable bronchiectasis; and• ACTs have beneficial effects on physiology and symptoms in individuals with acute and stable bronchiectasis. We searched the Cochrane Airways Group Specialised Register of trials from inception to November 2015 and PEDro in March 2015, and we handsearched relevant journals. Randomised controlled parallel and cross-over trials that compared an ACT versus no treatment, sham ACT or directed coughing in participants with bronchiectasis. We used standard methodological procedures as expected by The Cochrane Collaboration. Seven studies involving 105 participants met the inclusion criteria of this review, six of which were cross-over in design. Six studies included adults with stable bronchiectasis; the other study examined clinically stable children with bronchiectasis. Three studies provided single treatment sessions, two lasted 15 to 21 days and two were longer-term studies. Interventions varied; some control groups received a sham intervention and others were inactive. The methodological quality of these studies was variable, with most studies failing to use concealed allocation for group assignment and with absence of blinding of participants and personnel for outcome measure assessment. Heterogeneity between studies precluded inclusion of these data in the meta-analysis; the review is therefore narrative.One study including 20 adults that compared an airway oscillatory device versus no treatment found no significant difference in the number of exacerbations at 12 weeks (low-quality evidence). Data were not available for assessment of the impact of ACTs on time to exacerbation, duration or incidence of hospitalisation or total number of hospitalised days. The same study reported clinically significant improvements in HRQoL on both disease-specific and cough-related measures. The median difference in the change in total St George's Respiratory Questionnaire (SGRQ) score over three months in this study was 7.5 units (P value = 0.005 (Wilcoxon)). Treatment consisting of high-frequency chest wall oscillation (HFCWO) or a mix of ACTs prescribed for 15 days significantly improved HRQoL when compared with no treatment (low-quality evidence). Two studies reported mean increases in sputum expectoration with airway oscillatory devices in the short term of 8.4 mL (95% confidence interval (CI) 3.4 to 13.4 mL) and in the long term of 3 mL (P value = 0.02). HFCWO improved forced expiratory volume in one second (FEV1) by 156 mL and forced vital capacity (FVC) by 229.1 mL when applied for 15 days, but other types of ACTs showed no effect on dynamic lung volumes. Two studies reported a reduction in pulmonary hyperinflation among adults with non-positive expiratory pressure (PEP) ACTs (difference in functional residual capacity (FRC) of 19%, P value < 0.05; difference in total lung capacity (TLC) of 703 mL, P value = 0.02) and with airway oscillatory devices (difference in FRC of 30%, P value < 0.05) compared with no ACTs. Low-quality evidence suggests that ACTs (HFCWO, airway oscillatory devices or a mix of ACTs) reduce symptoms of breathlessness and cough and improve ease of sputum expectoration compared with no treatment (P value < 0.05). ACTs had no effect on gas exchange, and no studies reported effects of antibiotic usage. Among studies exploring airway oscillating devices, investigators reported no adverse events. ACTs appear to be safe for individuals (adults and children) with stable bronchiectasis and may account for improvements in sputum expectoration, selected measures of lung function, symptoms and HRQoL. The role of these techniques in acute exacerbation of bronchiectasis is unknown. In view of the chronic nature of bronchiectasis, additional data are needed to establish the short-term and long-term clinical value of ACTs for patient-important outcomes and for long-term clinical parameters that impact disease progression in individuals with stable bronchiectasis, allowing further guidance on prescription of specific ACTs for people with bronchiectasis.

Upper airway involvement in bronchiectasis is marked by early onset and allergic features

PubMed Central

Nassrallah, Najwan; Jrbashyan, Jenny; Uri, Nechama; Stein, Nili; Adir, Yochai

2018-01-01

The association of bronchiectasis with chronic rhinosinusitis (CRS) has been reported. However, apart from primary ciliary dyskinesia (PCD) and cystic fibrosis (CF), predisposing conditions have not been established. We aimed to define clinical and laboratory features that differentiate patients with bronchiectasis with upper airway symptoms (UASs) and without PCD from patients without UASs. We reviewed charts of adults with bronchiectasis, excluding CF and PCD. UASs were defined as nasal discharge most days of the year, sinusitis or nasal polyps. Laboratory data included IgG, total IgE, blood eosinophils, sputum bacteriology and lung function. A radiologist blinded to UAS presence scored bronchiectasis (Reiff score) and sino-nasal pathology (Lund–Mackay score). Of 197 patients, for the 70 (35%) with UASs, symptoms started earlier (34±25 versus 46±24 years; p=0.001), disease duration was longer (median 24 versus 12 years; p=0.027), exacerbations were more frequent (median 3 versus 2 per year; p=0.14), and peripheral blood eosinophil (median 230 versus 200 μL−1; p=0.015) and total IgE (median 100 versus 42 IU·mL−1; p=0.085) levels were higher. The sinus computed tomography score was independently associated with exacerbations, with 1 point on the Lund–Mackay score associated with a 1.03-fold increase in the number of exacerbations per year (95% CI 1.0–1.05; p=0.004). These findings may implicate a higher disease burden in patients with UASs. We hypothesise that UASs precede and may in some cases lead to the development of bronchiectasis. PMID:29362708

Lung clearance index is a repeatable and sensitive indicator of radiological changes in bronchiectasis.

PubMed

Rowan, Stephen A; Bradley, Judy M; Bradbury, Ian; Lawson, John; Lynch, Tom; Gustafsson, Per; Horsley, Alex; O'Neill, Katherine; Ennis, Madeleine; Elborn, J Stuart

2014-03-01

In bronchiectasis there is a need for improved markers of lung function to determine disease severity and response to therapy. To assess whether the lung clearance index is a repeatable and more sensitive indicator of computed tomography (CT) scan abnormalities than spirometry in bronchiectasis. Thirty patients with stable bronchiectasis were recruited and lung clearance index, spirometry, and health-related quality of life measures were assessed on two occasions, 2 weeks apart when stable (study 1). A separate group of 60 patients with stable bronchiectasis was studied on a single visit with the same measurements and a CT scan (study 2). In study 1, the intervisit intraclass correlation coefficient for the lung clearance index was 0.94 (95% confidence interval, 0.89 to 0.97; P < 0.001). In study 2, the mean age was 62 (10) years, FEV1 76.5% predicted (18.9), lung clearance index 9.1 (2.0), and total CT score 14.1 (10.2)%. The lung clearance index was abnormal in 53 of 60 patients (88%) and FEV1 was abnormal in 37 of 60 patients (62%). FEV1 negatively correlated with the lung clearance index (r = -0.51, P < 0.0001). Across CT scores, there was a relationship with the lung clearance index, with little evidence of an effect of FEV1. There were no significant associations between the lung clearance index or FEV1 and health-related quality of life. The lung clearance index is repeatable and a more sensitive measure than FEV1 in the detection of abnormalities demonstrated on CT scan. The lung clearance index has the potential to be a useful clinical and research tool in patients with bronchiectasis.

Serum Albumin and Disease Severity of Non-Cystic Fibrosis Bronchiectasis.

PubMed

Lee, Seung Jun; Kim, Hyo-Jung; Kim, Ju-Young; Ju, Sunmi; Lim, Sujin; Yoo, Jung Wan; Nam, Sung-Jin; Lee, Gi Dong; Cho, Hyun Seop; Kim, Rock Bum; Cho, Yu Ji; Jeong, Yi Yeong; Kim, Ho Cheol; Lee, Jong Deog

2017-08-01

A clinical classification system has been developed to define the severity and predict the prognosis of subjects with non-cystic fibrosis (CF) bronchiectasis. We aimed to identify laboratory parameters that are correlated with the bronchiectasis severity index (BSI) and FACED score. The medical records of 107 subjects with non-CF bronchiectasis for whom BSI and FACED scores could be calculated were retrospectively reviewed. The correlations between the laboratory parameters and BSI or FACED score were assessed, and multiple-linear regression analysis was performed to identify variables independently associated with BSI and FACED score. An additional subgroup analysis was performed according to sex. Among all of the enrolled subjects, 49 (45.8%) were male and 58 (54.2%) were female. The mean BSI and FACED scores were 9.43 ± 3.81 and 1.92 ± 1.59, respectively. The serum albumin level (r = -0.49), bilirubin level (r = -0.31), C-reactive protein level (r = 0.22), hemoglobin level (r = -0.2), and platelet/lymphocyte ratio (r = 0.31) were significantly correlated with BSI. Meanwhile, serum albumin (r = -0.37) and bilirubin level (r = -0.25) showed a significant correlation with the FACED score. Multiple-linear regression analysis showed that the serum bilirubin level was independently associated with BSI, and the serum albumin level was independently associated with both scoring systems. Subgroup analysis revealed that the level of uric acid was also a significant variable independently associated with the BSI in male bronchiectasis subjects. Several laboratory variables were identified as possible prognostic factors for non-CF bronchiectasis. Among them, the serum albumin level exhibited the strongest correlation and was identified as an independent variable associated with the BSI and FACED scores. Copyright © 2017 by Daedalus Enterprises.

Improvement in health status following bronchopulmonary hygiene physical therapy in patients with bronchiectasis.

PubMed

Mutalithas, Kugathasan; Watkin, Gillian; Willig, Briony; Wardlaw, Andrew; Pavord, Ian D; Birring, Surinder S

2008-08-01

Chronic productive cough is a common symptom in patients with bronchiectasis that is associated with a reduction in health-related quality of life (QOL). Bronchopulmonary hygiene physical therapy (BHPT) is widely prescribed for patients with bronchiectasis, although the evidence for its efficacy is limited. We set out to prospectively evaluate the impact of BHPT on health-related QOL in patients with non-cystic fibrosis bronchiectasis. We assessed cough symptoms (0-100mm visual analogue scale; VAS) and cough-related QOL in 53 patients with stable non-cystic fibrosis bronchiectasis at baseline and >4 weeks after outpatient-based BHPT. Cough specific health status was assessed with the Leicester Cough Questionnaire (LCQ; total score range 3-21, higher scores representing better QOL). All patients with bronchiectasis complained of cough as the major symptom and had mean (SEM) FEV(1) of 2.1 (0.1)L. Cough-related health status was reduced at baseline; mean (SEM) LCQ score 14.3 (0.6). There were significant improvements in cough symptoms (mean cough VAS before 43.3 (3.6) vs after 27.5 (3.1); mean difference 15.8; 95% CI of difference 9.6-22; p<0.0001) and cough-related health status after BHPT (mean LCQ total score before 14.2 vs after 17.3; mean difference 3.1; 95% confidence interval of difference 2.4-3.9; p<0.001). A significant improvement was seen in all LCQ health-related domains (physical, psychological and social; all p<0.001). Our findings suggest that bronchopulmonary hygiene physical therapy can lead to a significant improvement in cough-related quality of life.

Factors Associated With Quality of Life in Subjects With Stable COPD.

PubMed

Ekici, Aydanur; Bulcun, Emel; Karakoc, Tulay; Senturk, Erol; Ekici, Mehmet

2015-11-01

The purpose of this study was to assess the impact of bronchiectasis, anxiety and depression, and parameters of disease severity on health-related quality of life (HRQOL) in subjects with COPD. Sixty-two subjects with stable COPD were selected for the study. The presence of bronchiectasis in all subjects with COPD was investigated by high-resolution computed tomography. Pulmonary function tests were performed. Dyspnea was assessed using the Modified Medical Research Council (MMRC) dyspnea scale. Psychological disorders were investigated using the Hospital Anxiety and Depression Scale (HADS), and the HRQOL was examined using the St George Respiratory Questionnaire (SGRQ). High-resolution computed tomography revealed that 44 of 62 (70.9%) subjects with COPD had bronchiectasis. There were no differences in pulmonary function tests, symptoms, activities, impact, SGRQ total scores, and HADS scores between COPD subjects with and without bronchiectasis. Pearson correlation analyses showed that there was no significant association between the presence of bronchiectasis, bronchial wall thickness, or severity of bronchial dilatation and all subscales of the SGRQ or HADS scores. Additionally, HADS scores showed significant positive association with all subscales of the SGRQ in all subjects. In linear regression analysis, a statistically significant relationship was found between the SGRQ total score and percent-of-predicted FEV1, percent-of-predicted diffusing capacity of the lung for carbon monoxide, MMRC score, and anxiety score, but the SGRQ total score was not associated with age, body mass index, total number of hospitalizations, PaO2 , or depression score. The presence of bronchiectasis in subjects with COPD does not impact HRQOL and psychological disorders. However, disease severity, dyspnea levels, and anxiety scores predict poor HRQOL. Copyright © 2015 by Daedalus Enterprises.

A preliminary quality of life questionnaire-bronchiectasis: a patient-reported outcome measure for bronchiectasis.

PubMed

Quittner, Alexandra L; Marciel, Kristen K; Salathe, Matthias A; O'Donnell, Anne E; Gotfried, Mark H; Ilowite, Jonathan S; Metersky, Mark L; Flume, Patrick A; Lewis, Sandra A; McKevitt, Matthew; Montgomery, A Bruce; O'Riordan, Thomas G; Barker, Alan F

2014-08-01

The Quality of Life Questionnaire-Bronchiectasis (QOL-B) is the first disease-specific, patient-reported outcome measure for patients with bronchiectasis. Content validity, cognitive testing, responsivity to open-label treatment, and psychometric analyses are presented. Reviews of literature, existing measures, and physician input were used to generate the initial QOL-B. Modifications following preliminary cognitive testing (N = 35 patients with bronchiectasis) generated version (V) 1.0. An open-ended patient interview study (N = 28) provided additional information and was content analyzed to derive saturation matrices, which summarized all disease-related topics mentioned by each participant. This resulted in QOL-B V2.0. Psychometric analyses were carried out using results from an open-label phase 2 trial, in which 89 patients were enrolled and treated with aztreonam for inhalation solution. Responsivity to open-label treatment was observed. Additional analyses generated QOL-B V3.0, with 37 items on eight scales: respiratory symptoms; physical, role, emotional, and social functioning; vitality; health perceptions; and treatment burden. For each scale, scores are standardized on a 0-to-100-point scale; higher scores indicate better health-related quality of life. No total score is calculated. A final cognitive testing study (N = 40) resulted in a minor change to one social functioning scale item (QOL-B V3.1). Content validity, cognitive testing, responsivity to open-label treatment, and initial psychometric analyses supported QOL-B items and structure. This interim QOL-B is a promising tool for evaluating the efficacy of new therapies for patients with bronchiectasis and for measuring symptoms, functioning, and quality of life in these patients on a routine basis. A final psychometric validation study is needed and is forthcoming. ClinicalTrials.gov; No.: NCT00805025; URL: www.clinicaltrials.gov.

Pulmonary rehabilitation in patients with bronchiectasis: pulmonary function, arterial blood gases, and the 6-minute walk test.

PubMed

van Zeller, Mafalda; Mota, Patrícia Caetano; Amorim, Adelina; Viana, Paulo; Martins, Paula; Gaspar, Luís; Hespanhol, Venceslau; Gomes, Isabel

2012-01-01

Information regarding the effects of pulmonary rehabilitation (PR) on pulmonary function (PF), arterial blood gases (ABG), and 6-minute walk distance (6MWD) in patients with bronchiectasis is scant in the literature. To evaluate the effects of PR on these indices in this population, a retrospective evaluation of those who attended PR from 2007 to 2010, was made. Pulmonary rehabilitation lasted a mean of 12 weeks and included cycle ergometer exercise for 30 minutes, 3 times per week, with additional upper limbs and quadriceps training. PF, ABG, and 6MWD were evaluated before and after PR to determine the potential influence of gender, exacerbations, underlying cause of bronchiectasis, severity of obstruction, and colonization with bacteria. Forty-one patients (48.8% males; median age, 54 years) were included; 25 had severe obstruction and 19 were colonized with bacteria. Following PR, no significant changes were detected in PF or ABG. Median 6MWD before PR was 425 m and post-PR was 450 m (P = .431). Outcomes did not show any interaction with gender, colonization, or exacerbations. However, patients with idiopathic bronchiectasis did show a significant improvement in forced vital capacity in percent of predicted and residual volume after PR (P = .016 and .048, respectively). Patients with severe obstruction showed a statistically significant decrease in percent of predicted residual volume (P = .025). There appears to be a beneficial impact of PR on PF in certain groups of patients with bronchiectasis. In addition, PR indications and protocols for patients with bronchiectasis may need to be adapted to accommodate specific patients, so that expressive exercise capacity improvement can be achieved.

Free-breathing pulmonary 1H and Hyperpolarized 3He MRI: comparison in COPD and bronchiectasis.

PubMed

Capaldi, Dante P I; Sheikh, Khadija; Guo, Fumin; Svenningsen, Sarah; Etemad-Rezai, Roya; Coxson, Harvey O; Leipsic, Jonathon A; McCormack, David G; Parraga, Grace

2015-03-01

In this proof-of-concept demonstration, we aimed to quantitatively and qualitatively compare pulmonary ventilation abnormalities derived from Fourier decomposition of free-breathing (1)H magnetic resonance imaging (FDMRI) to hyperpolarized (3)He MRI in subjects with chronic obstructive pulmonary disease (COPD) and bronchiectasis. All subjects provided written informed consent to a protocol approved by a local research ethics board and Health, Canada, and they underwent MRI, computed tomography (CT), spirometry, and plethysmography during a single 2-hour visit. Semiautomated segmentation was used to generate ventilation defect measurements derived from FDMRI and (3)He MRI, and these were compared using analysis of variance and Pearson correlations. Twenty-six subjects were evaluated including 12 COPD subjects (67 ± 9 years) and 14 bronchiectasis subjects (70 ± 11 years). For COPD subjects, FDMRI and (3)He MRI ventilation defect percent (VDP) was 7 ± 6% and 24 ± 14%, respectively (P < .001; bias = -16 ± 9%). In COPD subjects, FDMRI was significantly correlated with (3)He MRI VDP (r = .88; P = .0001), (3)He MRI apparent diffusion coefficient (r = .71; P < .05), airways resistance (r = .60; P < .05), and RA950 (r = .80; P < .01). In subjects with bronchiectasis, FDMRI VDP (5 ± 3%) and (3)He MRI VDP (18 ± 9%) were significantly different (P < .001) and not correlated (P > .05). The Dice similarity coefficient (DSC) for FDMRI and (3)He MRI ventilation was 86 ± 7% for COPD and 86 ± 4% for bronchiectasis subjects (P > .05); the DSC for FDMRI ventilation defects and CT RA950 was 19 ± 20% in COPD and 2 ± 3% in bronchiectasis subjects (P < .01). FDMRI and (3)He MRI VDP were strongly related in COPD but not in bronchiectasis subjects. In COPD only, FDMRI ventilation defects were spatially related with (3)He ventilation defects and emphysema. Copyright © 2015 AUR. Published by Elsevier Inc. All rights reserved.

Treatment adherence and health outcomes in patients with bronchiectasis

PubMed Central

2014-01-01

Background We aimed to determine adherence to inhaled antibiotics, other respiratory medicines and airway clearance and to determine the association between adherence to these treatments and health outcomes (pulmonary exacerbations, lung function and Quality of Life Questionnaire-Bronchiectasis [QOL-B]) in bronchiectasis after 12 months. Methods Patients with bronchiectasis prescribed inhaled antibiotics for Pseudomonas aeruginosa infection were recruited into a one-year study. Participants were categorised as “adherent” to medication (medication possession ratio ≥80% using prescription data) or airway clearance (score ≥80% in the Modified Self-Reported Medication-Taking Scale). Pulmonary exacerbations were defined as treatment with a new course of oral or intravenous antibiotics over the one-year study. Spirometry and QOL-B were completed at baseline and 12 months. Associations between adherence to treatment and pulmonary exacerbations, lung function and QOL-B were determined by regression analyses. Results Seventy-five participants were recruited. Thirty-five (53%), 39 (53%) and 31 (41%) participants were adherent to inhaled antibiotics, other respiratory medicines, and airway clearance, respectively. Twelve (16%) participants were adherent to all treatments. Participants who were adherent to inhaled antibiotics had significantly fewer exacerbations compared to non-adherent participants (2.6 vs 4, p = 0.00) and adherence to inhaled antibiotics was independently associated with having fewer pulmonary exacerbations (regression co-efficient = -0.51, 95% CI [-0.81,-0.21], p < 0.001). Adherence to airway clearance was associated with lower QOL-B Treatment Burden (regression co-efficient = -15.46, 95% CI [-26.54, -4.37], p < 0.01) and Respiratory Symptoms domain scores (regression co-efficient = -10.77, 95% CI [-21.45; -0.09], p < 0.05). There were no associations between adherence to other respiratory medicines and any of the outcomes tested. Adherence to treatment was not associated with FEV1 % predicted. Conclusions Treatment adherence is low in bronchiectasis and affects important health outcomes including pulmonary exacerbations. Adherence should be measured as part of bronchiectasis management and future research should evaluate bronchiectasis-specific adherence strategies. PMID:24980161

Adult Non-Cystic Fibrosis Bronchiectasis Is Characterised by Airway Luminal Th17 Pathway Activation

PubMed Central

Chen, Alice C.-H.; Martin, Megan L.; Lourie, Rohan; Rogers, Geraint B.; Burr, Lucy D.; Hasnain, Sumaira Z.; Bowler, Simon D.; McGuckin, Michael A.; Serisier, David J.

2015-01-01

Background Non-cystic fibrosis (CF) bronchiectasis is characterised by chronic airway infection and neutrophilic inflammation, which we hypothesised would be associated with Th17 pathway activation. Methods Th17 pathway cytokines were quantified in bronchoalveolar lavage fluid (BALF), and gene expression of IL-17A, IL-1β, IL-8 and IL-23 determined from endobronchial biopsies (EBx) in 41 stable bronchiectasis subjects and 20 healthy controls. Relationships between IL-17A levels and infection status, important clinical measures and subsequent Pseudomonas aeruginosa infection were determined. Results BALF levels of all Th17 cytokines (median (IQR) pg/mL) were significantly higher in bronchiectasis than control subjects, including IL-17A (1.73 (1.19, 3.23) vs. 0.27 (0.24, 0.35), 95% CI 1.05 to 2.21, p<0.0001) and IL-23 (9.48 (4.79, 15.75) vs. 0.70 (0.43, 1.79), 95% CI 4.68 to 11.21, p<0.0001). However, BALF IL-17A levels were not associated with clinical measures or airway microbiology, nor predictive of subsequent P. aeruginosa infection. Furthermore, gene expression of IL-17A in bronchiectasis EBx did not differ from control. In contrast, gene expression (relative to medians of controls) in bronchiectasis EBx was significantly higher than control for IL1β (4.12 (1.24, 8.05) vs 1 (0.13, 2.95), 95% CI 0.05 to 4.07, p = 0.04) and IL-8 (3.75 (1.64, 11.27) vs 1 (0.54, 3.89), 95% CI 0.32 to 4.87, p = 0.02) and BALF IL-8 and IL-1α levels showed significant relationships with clinical measures and airway microbiology. P. aeruginosa infection was associated with increased levels of IL-8 while Haemophilus influenzae was associated with increased IL-1α. Conclusions and Clinical Relevance Established adult non-CF bronchiectasis is characterised by luminal Th17 pathway activation, however this pathway may be relatively less important than activation of non-antigen-specific innate neutrophilic immunity. PMID:25822228

Chronic rhinosinusitis is associated with higher prevalence and severity of bronchiectasis in patients with COPD

PubMed Central

Yang, Xia; Xu, Yali; Jin, Jianmin; Li, Ruimin; Liu, Xiaofang; Sun, Yongchang

2017-01-01

Background and purpose Bronchiectasis revealed by high-resolution computed tomography (HRCT) is common in chronic obstructive pulmonary disease (COPD), but the causes and risk factors remain to be determined. Chronic rhinosinusitis (CRS) is closely associated with bronchiectasis or COPD, but whether it is associated with comorbid bronchiectasis in COPD (COPD-Bx) is unknown. Patients and methods Patients with stable COPD were enrolled consecutively and evaluated for the presence of CRS by questionnaire and paranasal sinus computed tomography. The presence and severity of bronchiectasis on lung HRCT were evaluated by the Smith and severity scores. COPD symptoms were evaluated by COPD Assessment Test (CAT) and Modified British Medical Research Council Questionnaire. The sputum cell differentials and concentrations of interleukin (IL)-6, IL-8, IL-5, matrix metalloproteinases-9 (MMP-9), and tissue inhibitor of matrix metalloproteinases-1 were measured. Results We enrolled 136 patients with stable COPD, of which 66 (48.5%) were diagnosed with CRS according to the European Position Paper on Rhinosinusitis and Nasal Polyps (EP3OS) criteria. The prevalence of bronchiectasis was 57.6% in patients with CRS, but 37.1% in those without CRS (P=0.017). COPD-Bx patients with CRS showed a significantly higher severity score of bronchiectasis than those without CRS (P=0.034). COPD patients with CRS had a higher percentage of eosinophils, higher levels of IL-8, IL-6, and MMP-9 in sputum as compared to those without CRS. In COPD-Bx patients with CRS, the percentage of eosinophils and the levels of IL-6 and MMP-9 in sputum were increased as compared to those without CRS. In all the subjects, Sino-Nasal Outcome Test-20 correlated with CAT score (r=0.315, P<0.01) and in COPD patients with CRS, Lund–MacKay scores correlated with forced expiratory volume in 1 s (% pred) (r=−0.251, P<0.05). Conclusions CRS was associated with COPD-Bx and this was probably due to increased airway inflammation. PMID:28260873

Continuous versus intermittent antibiotics for bronchiectasis.

PubMed

Donovan, Tim; Felix, Lambert M; Chalmers, James D; Milan, Stephen J; Mathioudakis, Alexander G; Spencer, Sally

2018-06-03

Bronchiectasis is a chronic airway disease characterised by a destructive cycle of recurrent airway infection, inflammation and tissue damage. Antibiotics are a main treatment for bronchiectasis. The aim of continuous therapy with prophylactic antibiotics is to suppress bacterial load, but bacteria may become resistant to the antibiotic, leading to a loss of effectiveness. On the other hand, intermittent prophylactic antibiotics, given over a predefined duration and interval, may reduce antibiotic selection pressure and reduce or prevent the development of resistance. This systematic review aimed to evaluate the current evidence for studies comparing continuous versus intermittent administration of antibiotic treatment in bronchiectasis in terms of clinical efficacy, the emergence of resistance and serious adverse events. To evaluate the effectiveness of continuous versus intermittent antibiotics in the treatment of adults and children with bronchiectasis, using the primary outcomes of exacerbations, antibiotic resistance and serious adverse events. On 1 August 2017 and 4 May 2018 we searched the Cochrane Airways Review Group Specialised Register (CAGR), CENTRAL, MEDLINE, Embase, PsycINFO, CINAHL, and AMED. On 25 September 2017 and 4 May 2018 we also searched www.clinicaltrials.gov, the World Health Organization (WHO) trials portal, conference proceedings and the reference lists of existing systematic reviews. We planned to include randomised controlled trials (RCTs) of adults or children with bronchiectasis that compared continuous versus intermittent administration of long-term prophylactic antibiotics of at least three months' duration. We considered eligible studies reported as full-text articles, as abstracts only and unpublished data. Two review authors independently screened the search results and full-text reports. We identified 268 unique records. Of these we retrieved and examined 126 full-text reports, representing 114 studies, but none of these studies met our inclusion criteria. No randomised controlled trials have compared the effectiveness and risks of continuous antibiotic therapy versus intermittent antibiotic therapy for bronchiectasis. High-quality clinical trials are needed to establish which of these interventions is more effective for reducing the frequency and duration of exacerbations, antibiotic resistance and the occurrence of serious adverse events.

[The place of surgery in bilateral sequelae bronchiectasis].

PubMed

Issoufou, I; Rabiou, S; Belliraj, L; Ammor, F Z; Harmouchi, H; Diarra, A S; Lakranbi, M; Serraj, M; Ouadnouni, Y; Smahi, M

2017-06-01

The aim of our study is to report our surgery results in bilateral sequelae bronchiectasis and to assess its impact on the life quality of our patients. This is a retrospective descriptive study in thoracic surgery department of Teaching Hospital Hassan II of Fez in Morocco. It involved all patients with bilateral bronchiectasis which is predominant on a few lobes or segments (localized) and who underwent surgery during the period 2010-2015. The epidemiological, clinical and paraclinical data, the surgery results, the evolution and the impact on life quality were assessed. From a total of 47 patients with bilateral bronchiectasis, 13 were operated, thus a frequency of 27.6%. The average age was 32years, ranging from 15 to 54years. Women were in majority (61.5%) representing a sex ratio of 1.6. The association of chronic bronchorrhea and hemoptysis was the main reason of medical consultation in 46.16%, followed by isolated chronic bronchorrhea in 38.46%. Surgical resection involved the left side in 61.5% of cases. The left lower lobectomy was the most accomplished gesture. An improvement in symptoms was found in 11 patients (84.6%) as a decrease in bronchorrhea, hemoptysis episodes and decreasing use of antibiotics. Bilateral bronchiectasis surgery can be performed with acceptable morbidity and mortality in well-selected patients with an improvement in symptoms. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Validation of the Leicester Cough Questionnaire in non-cystic fibrosis bronchiectasis.

PubMed

Murray, M P; Turnbull, K; MacQuarrie, S; Pentland, J L; Hill, A T

2009-07-01

Health-related quality of life is a potentially important marker for evaluating existing and new therapies in bronchiectasis. The Leicester Cough Questionnaire (LCQ) is a symptom specific questionnaire designed to assess the impact of cough severity, a major symptom of bronchiectasis. This study aimed to validate the LCQ in bronchiectasis. The validity, responsiveness and reliability of the LCQ were assessed as follows: ability to discriminate severe and mild disease; change in score following antibiotic treatment for exacerbations; repeatability over a 6-month period in stable disease; and comparison with the St George's Respiratory Questionnaire (SGRQ). In total, 120 patients (51 with severe disease, 29 with moderate disease and 40 with mild disease) completed the LCQ and SGRQ. The area under the receiver-operator curve was good for both severe and mild disease (0.84 and 0.80 respectively, p<0.0001). Following 2 weeks' antibiotic treatment, the median LCQ score (interquartile range) improved from 11.3 (9.3-13.7) to 17.8 (15-18.8) (p<0.0001). The LCQ score was repeatable over 6 months in stable disease (intraclass correlation coefficient of 0.96 (95%CI 0.93-0.97), p<0.0001). Correlation between the LCQ and SGRQ scores was -0.7 in both stable disease and exacerbations (p<0.0001). The LCQ can discriminate disease severity, is responsive to change and is reliable for use in non-cystic fibrosis bronchiectasis.

Clinical determinants of the 6-Minute Walk Test in bronchiectasis.

PubMed

Lee, A L; Button, B M; Ellis, S; Stirling, R; Wilson, J W; Holland, A E; Denehy, L

2009-05-01

The 6-Minute Walk Test (6MWT) is a widely used measurement of functional exercise capacity in chronic lung disease. While exercise intolerance has been identified in patients with bronchiectasis, the clinical determinants of the 6MWT in this population have not been examined. The aim of this study was to 1) establish the relationship between the 6-Minute Walk Distance (6MWD), disease severity and Health-Related Quality of Life (HRQOL) and 2) identify predictors of exercise tolerance in adults with bronchiectasis. The 6MWT was performed in 27 patients with bronchiectasis (mean [SD] FEV(1) 73.9% predicted [23.4]). Disease severity was assessed using spirometry and HRCT scoring while HRQOL was evaluated using the St George's Respiratory Questionnaire (SGRQ) and the Short-Form 36 (SF-36). The relationships were evaluated using correlation and multiple regression. The 6MWD correlated positively with FVC (r=0.52, p<0.01), generations of bronchopulmonary divisions (r(s)=0.38, p<0.05) and SF-36 physical summary (r=0.71, p<0.001) while a negative correlation was observed between all domains of the SGRQ (all correlations r>0.5, p<0.001). Multiple regression analysis indicated that the SGRQ activity, symptom scores and generations of bronchial divisions involved were identified as independent predictors of the 6MWD, explaining 76% of the variance. Measures of HRQOL demonstrated a stronger association with the 6MWD compared to physiological measures of disease severity in patients with predominantly mild to moderate bronchiectasis.

The BRICS (Bronchiectasis Radiologically Indexed CT Score): A Multicenter Study Score for Use in Idiopathic and Postinfective Bronchiectasis.

PubMed

Bedi, Pallavi; Chalmers, James D; Goeminne, Pieter C; Mai, Cindy; Saravanamuthu, Pira; Velu, Prasad Palani; Cartlidge, Manjit K; Loebinger, Michael R; Jacob, Joe; Kamal, Faisal; Schembri, Nicola; Aliberti, Stefano; Hill, Uta; Harrison, Mike; Johnson, Christopher; Screaton, Nicholas; Haworth, Charles; Polverino, Eva; Rosales, Edmundo; Torres, Antoni; Benegas, Michael N; Rossi, Adriano G; Patel, Dilip; Hill, Adam T

2018-05-01

The goal of this study was to develop a simplified radiological score that could assess clinical disease severity in bronchiectasis. The Bronchiectasis Radiologically Indexed CT Score (BRICS) was devised based on a multivariable analysis of the Bhalla score and its ability in predicting clinical parameters of severity. The score was then externally validated in six centers in 302 patients. A total of 184 high-resolution CT scans were scored for the validation cohort. In a multiple logistic regression model, disease severity markers significantly associated with the Bhalla score were percent predicted FEV 1 , sputum purulence, and exacerbations requiring hospital admission. Components of the Bhalla score that were significantly associated with the disease severity markers were bronchial dilatation and number of bronchopulmonary segments with emphysema. The BRICS was developed with these two parameters. The receiver operating-characteristic curve values for BRICS in the derivation cohort were 0.79 for percent predicted FEV 1 , 0.71 for sputum purulence, and 0.75 for hospital admissions per year; these values were 0.81, 0.70, and 0.70, respectively, in the validation cohort. Sputum free neutrophil elastase activity was significantly elevated in the group with emphysema on CT imaging. A simplified CT scoring system can be used as an adjunct to clinical parameters to predict disease severity in patients with idiopathic and postinfective bronchiectasis. Copyright © 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Observational study of sleep, respiratory mechanics and quality of life in patients with non-cystic fibrosis bronchiectasis: a protocol study

PubMed Central

Faria Júnior, Newton Santos; Oliveira, Luis Vicente Franco; Perez, Eduardo Araújo; de Oliveira, Ezequiel Fernandes; Apostolico, Nadua; Pereira, Nixon Alves; dos Santos, Israel dos Reis; Urbano, Jessica Julioti; Souza, Ismael Dias; Polonio, Igor Bastos; Romaldini, José Gustavo Barian; Pereira, Déborah Madeu; Alves, Vera Lúcia dos Santos; Souza, Ângela Honda; Nascimento, Oliver Augusto; Jardim, José Roberto; Stirbulov, Roberto

2015-01-01

Introduction Bronchiectasis is a chronic disorder characterised by permanent and irreversible abnormal dilation of the bronchi and bronchioles, primarily caused by repeated cycles of pulmonary infections and inflammation, which lead to reduced mucociliary clearance and to the excessive production of sputum. Patients with non-cystic fibrosis bronchiectasis may be predisposed to hypoxemia during sleep, or to symptoms that may lead to arousals and thereby reduce the quality of life, because of the irreversible dilation of the bronchi and the presence of secretions and airflow obstruction. Methods and analysis For this cross-sectional observational study, patients with a clinical diagnosis of non-cystic fibrosis bronchiectasis will be recruited from the Bronchiectasis Clinic of the Pneumology Department of the Santa Casa de Misericordia Hospital and the Federal University of São Paulo (São Paulo, Brazil). Patients of either sex will be included if high-resolution CT of the thorax and classic sweat test confirms they have non-cystic fibrosis bronchiectasis, are between 18 and 80 years old, use long-acting bronchodilators, are clinically stable for a least 1 month, agree to participate in the study and they sign a statement of informed consent. The first part of the study will involve a clinical evaluation, maximal respiratory pressures, spirometry and the Saint George's Respiratory Questionnaire. The Sleep Laboratory of the Master's and Doctoral Postgraduate Program in Rehabilitation Sciences of the Nove de Julho University (São Paulo, Brazil) will perform the polysomnographic studies, Berlin Questionnaire, Epworth Sleepiness Scale, waist and neck circumferences, modified Mallampati classification and tonsil index. Ethics and dissemination This protocol has been approved by the Human Research Ethics Committees of Santa Casa de Misericordia Hospital (process number 178/2012) and Human Research Ethics Committee of Nove de Julho University (process number 370474/2010). All participants will sign a statement of informed consent. The study findings will be published in peer-reviewed journals and presented at conferences. PMID:26169808

Prevention of exacerbations in patients with stable non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis of pharmacological and non-pharmacological therapies.

PubMed

Abu Dabrh, Abd Moain; Hill, Adam T; Dobler, Claudia C; Asi, Noor; Farah, Wigdan H; Haydour, Qusay; Wang, Zhen; Benkhadra, Khalid; Prokop, Larry J; Murad, Mohammad Hassan

2018-04-20

Several pharmacological and non-pharmacological therapies are used to treat stable bronchiectasis of non-cystic fibrosis (CF) aetiology. We conducted a systematic review and meta-analysis to assess the evidence of the effectiveness of pharmacological and non-pharmacological treatment options in patients with stable non-CF bronchiectasis with a focus on reducing exacerbations. Multiple databases were searched through September 2017. Outcomes included the number of patients with exacerbation events, mean number of exacerbations, hospitalisations, mortality, quality of life measures, and safety and adverse effects. Meta-analysis was conducted using the random effects model. 30 randomised controlled trials enrolled subjects with non-CF bronchiectasis using different interventions. Moderate-quality evidence supported the effect of long-term antibiotics (≥3 months) on lowering the number of patients experiencing exacerbation events (relative risk 0.77 (95% CI 0.68 to 0.89)), reducing number of exacerbations (incidence rate ratio 0.62 (95% CI 0.49 to 0.78)), improving forced expiratory volume (litre) in the first second (FEV 1 ) (weighted mean difference (WMD); 0.02 (95% CI 0.00 to 0.04)), decreasing sputum purulence scores (numerical scale of 1-8) (WMD -0.90 (95% CI -1.58 to -0.22)) and improving quality of life scores assessed by the St George's Respiratory Questionnaire (WMD -6.07 (95% CI -10.7 to -1.43)). Bronchospasm increased with inhaled antibiotics while diarrhoea increased particularly with oral macrolide therapy. Moderate-quality evidence supports long-term antibiotic therapy for preventing exacerbations in stable non-CF bronchiectasis. However, data about the optimum agent, mode of therapy and length of treatment are limited. There is paucity of high-quality evidence to support the management of stable non-CF bronchiectasis including prevention of exacerbations. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

[Lung transplant therapy for suppurative diseases].

PubMed

de Pablo, A; López, S; Ussetti, P; Carreño, M C; Laporta, R; López García-Gallo, C; Ferreiro, M J

2005-05-01

Lung transplantation is a valid therapeutic approach for patients with bronchiectasis. The objective of the present study was to evaluate our experience with bronchiectasis patients and compare the results in patients with cystic fibrosis to results in those with bronchiectasis caused by other processes. We carried out a retrospective study of bronchiectasis patients treated by lung transplantation in order to analyze demographic, functional and microbiological characteristics before and after transplantation, and survival. From 1991 to 2002 lung transplants were performed on 171 patients, 44 of whom had suppurative lung disease (27 had cystic fibrosis and 17 had bronchiectasis caused by other processes). There were no significant differences in the demographic variables between the 2 groups. At transplantation, lung function variables showed severe bronchial obstruction (mean [SD] forced expiratory volume in 1 second of 808 [342] mL and forced vital capacity of 1,390 [611] mL) and respiratory insufficiency (PaO2 at 52 [10] mm Hg and PaCO2 at 48 [9] mm Hg). Only PaO2 was significantly lower in patients with bronchiectasis from causes other than cystic fibrosis. Airway colonization was present in 91% of the patients; Pseudomonas spp germs were detected in 64% of the cases and were multiresistant in 9%. In the early postoperative period germs were isolated in 59% of the cases, half of which involved the same germ as had been isolated before transplantation. One year after lung transplantation, 34% of the patients continued to have bronchial colonization. Survival at 1 year was 79% and at 5 years, 49%, with no significant difference between the patients with cystic fibrosis and those with other suppurative diseases, nor between the patients with and without Pseudomonas colonization. Only 2 patients had died of bacterial pneumonia at 1 month after transplantation. Although airway colonization in patients with suppurative diseases complicates postoperative management, the results in terms of survival are good.

Sedentary behaviour and physical activity in bronchiectasis: a cross-sectional study.

PubMed

Bradley, Judy M; Wilson, Jason J; Hayes, Kate; Kent, Lisa; McDonough, Suzanne; Tully, Mark A; Bradbury, Ian; Kirk, Alison; Cosgrove, Denise; Convery, Rory; Kelly, Martin; Elborn, Joseph Stuart; O'Neill, Brenda

2015-05-13

The impact of bronchiectasis on sedentary behaviour and physical activity is unknown. It is important to explore this to identify the need for physical activity interventions and how to tailor interventions to this patient population. We aimed to explore the patterns and correlates of sedentary behaviour and physical activity in bronchiectasis. Physical activity was assessed in 63 patients with bronchiectasis using an ActiGraph GT3X+ accelerometer over seven days. Patients completed: questionnaires on health-related quality-of-life and attitudes to physical activity (questions based on an adaption of the transtheoretical model (TTM) of behaviour change); spirometry; and the modified shuttle test (MST). Multiple linear regression analysis using forward selection based on likelihood ratio statistics explored the correlates of sedentary behaviour and physical activity dimensions. Between-group analysis using independent sample t-tests were used to explore differences for selected variables. Fifty-five patients had complete datasets. Average daily time, mean(standard deviation) spent in sedentary behaviour was 634(77)mins, light-lifestyle physical activity was 207(63)mins and moderate-vigorous physical activity (MVPA) was 25(20)mins. Only 11% of patients met recommended guidelines. Forced expiratory volume in one-second percentage predicted (FEV1% predicted) and disease severity were not correlates of sedentary behaviour or physical activity. For sedentary behaviour, decisional balance 'pros' score was the only correlate. Performance on the MST was the strongest correlate of physical activity. In addition to the MST, there were other important correlate variables for MVPA accumulated in ≥10-minute bouts (QOL-B Social Functioning) and for activity energy expenditure (Body Mass Index and QOL-B Respiratory Symptoms). Patients with bronchiectasis demonstrated a largely inactive lifestyle and few met the recommended physical activity guidelines. Exercise capacity was the strongest correlate of physical activity, and dimensions of the QOL-B were also important. FEV1% predicted and disease severity were not correlates of sedentary behaviour or physical activity. The inclusion of a range of physical activity dimensions could facilitate in-depth exploration of patterns of physical activity. This study demonstrates the need for interventions targeted at reducing sedentary behaviour and increasing physical activity, and provides information to tailor interventions to the bronchiectasis population. NCT01569009 ("Physical Activity in Bronchiectasis").

Bronchiectasis in the Last Five Years: New Developments

PubMed Central

Khoo, Jun Keng; Venning, Victoria; Wong, Conroy; Jayaram, Lata

2016-01-01

Bronchiectasis, a chronic lung disease characterised by cough and purulent sputum, recurrent infections, and airway damage, is associated with considerable morbidity and mortality. To date, treatment options have been limited to physiotherapy to clear sputum and antibiotics to treat acute infections. Over the last decade, there has been significant progress in understanding the epidemiology, pathophysiology, and microbiology of this disorder. Over the last five years, methods of assessing severity have been developed, the role of macrolide antibiotic therapy in reducing exacerbations cemented, and inhaled antibiotic therapies show promise in the treatment of chronic Pseudomonas aeruginosa infection. Novel therapies are currently undergoing Phase 1 and 2 trials. This review aims to address the major developments within the field of bronchiectasis over this time. PMID:27941638

Fully automated lobe-based airway taper index calculation in a low dose MDCT CF study over 4 time-points

NASA Astrophysics Data System (ADS)

Weinheimer, Oliver; Wielpütz, Mark O.; Konietzke, Philip; Heussel, Claus P.; Kauczor, Hans-Ulrich; Brochhausen, Christoph; Hollemann, David; Savage, Dasha; Galbán, Craig J.; Robinson, Terry E.

2017-02-01

Cystic Fibrosis (CF) results in severe bronchiectasis in nearly all cases. Bronchiectasis is a disease where parts of the airways are permanently dilated. The development and the progression of bronchiectasis is not evenly distributed over the entire lungs - rather, individual functional units are affected differently. We developed a fully automated method for the precise calculation of lobe-based airway taper indices. To calculate taper indices, some preparatory algorithms are needed. The airway tree is segmented, skeletonized and transformed to a rooted acyclic graph. This graph is used to label the airways. Then a modified version of the previously validated integral based method (IBM) for airway geometry determination is utilized. The rooted graph, the airway lumen and wall information are then used to calculate the airway taper indices. Using a computer-generated phantom simulating 10 cross sections of airways we present results showing a high accuracy of the modified IBM. The new taper index calculation method was applied to 144 volumetric inspiratory low-dose MDCT scans. The scans were acquired from 36 children with mild CF at 4 time-points (baseline, 3 month, 1 year, 2 years). We found a moderate correlation with the visual lobar Brody bronchiectasis scores by three raters (r2 = 0.36, p < .0001). The taper index has the potential to be a precise imaging biomarker but further improvements are needed. In combination with other imaging biomarkers, taper index calculation can be an important tool for monitoring the progression and the individual treatment of patients with bronchiectasis.

Automated CT Scan Scores of Bronchiectasis and Air Trapping in Cystic Fibrosis

PubMed Central

Swiercz, Waldemar; Heltshe, Sonya L.; Anthony, Margaret M.; Szefler, Paul; Klein, Rebecca; Strain, John; Brody, Alan S.; Sagel, Scott D.

2014-01-01

Background: Computer analysis of high-resolution CT (HRCT) scans may improve the assessment of structural lung injury in children with cystic fibrosis (CF). The goal of this cross-sectional pilot study was to validate automated, observer-independent image analysis software to establish objective, simple criteria for bronchiectasis and air trapping. Methods: HRCT scans of the chest were performed in 35 children with CF and compared with scans from 12 disease control subjects. Automated image analysis software was developed to count visible airways on inspiratory images and to measure a low attenuation density (LAD) index on expiratory images. Among the children with CF, relationships among automated measures, Brody HRCT scanning scores, lung function, and sputum markers of inflammation were assessed. Results: The number of total, central, and peripheral airways on inspiratory images and LAD (%) on expiratory images were significantly higher in children with CF compared with control subjects. Among subjects with CF, peripheral airway counts correlated strongly with Brody bronchiectasis scores by two raters (r = 0.86, P < .0001; r = 0.91, P < .0001), correlated negatively with lung function, and were positively associated with sputum free neutrophil elastase activity. LAD (%) correlated with Brody air trapping scores (r = 0.83, P < .0001; r = 0.69, P < .0001) but did not correlate with lung function or sputum inflammatory markers. Conclusions: Quantitative airway counts and LAD (%) on HRCT scans appear to be useful surrogates for bronchiectasis and air trapping in children with CF. Our automated methodology provides objective quantitative measures of bronchiectasis and air trapping that may serve as end points in CF clinical trials. PMID:24114359

Assessment of the quantity of microorganisms associated with bronchiectasis in saliva, sputum and nasal lavage after periodontal treatment: a study protocol of a randomised controlled trial

PubMed Central

Pinto, Erika Horácio; Longo, Priscila Larcher; de Camargo, Caroline Cristina Batista; Dal Corso, Simone; Lanza, Fernanda De Cordoba; Stelmach, Rafael; Athanazio, Rodrigo; Fernandes, Kristianne Porta Santos; Mayer, Marcia Pinto Alves; Bussadori, Sandra Kalil; Mesquita Ferrari, Raquel Agnelli; Horliana, Anna Carolina Ratto Tempestini

2016-01-01

Introduction The association between periodontal disease (PD) and chronic obstructive pulmonary disease (COPD) has been widely studied, with aspiration of periodontal pathogens being one of the most accepted causal mechanisms for pulmonary exacerbation. Periodontal treatment (PT) was associated with a decrease in these exacerbations. Bronchiectasis is a pulmonary disease that has many similarities to COPD; however, there are no studies correlating this condition to PD thus far. This study will evaluate if PT reduces proinflammatory cytokines in serum and saliva, as well as halitosis and the amount of microorganisms associated with exacerbation of bronchiectasis in saliva, sputum and nasal lavage 3 months after PT. Methods and analysis A total of 182 patients with PD and bronchiectasis will be randomly allocated to group 1 (positive control; scaling and root planing (SRP)+oral hygiene (OH)) or group 2 (experimental; SRP+photodynamic therapy+OH). After 3 months, samples of saliva, nasal lavage and sputum will be collected to determine the level of Pseudomonas aeruginosa, Staphylococcus aureus and Porphyromonas gingivalis by quantitative PCR. This protocol will determine the efficacy of PT in reducing the most likely niches of bronchiectasis exacerbation by comparing pre- and post-treatment microbiology samples. Furthermore, there will be assessment of oral halitosis and verification of inflammatory cytokines in serum and saliva. Ethics and dissemination This protocol has been approved by the Research Ethics Committee of Universidade Nove de Julho. Data will be published in a peer-reviewed journal. Trial registration number NCT02514226. PMID:27084279

Positive expiratory pressure therapy versus other airway clearance techniques for bronchiectasis.

PubMed

Lee, Annemarie L; Burge, Angela T; Holland, Anne E

2017-09-27

People with bronchiectasis experience chronic cough and sputum production and require the prescription of airway clearance techniques (ACTs). A common type of ACT prescribed is positive expiratory pressure (PEP) therapy. A previous review has suggested that ACTs including PEP therapy are beneficial compared to no treatment in people with bronchiectasis. However, the efficacy of PEP therapy in a stable clinical state or during an acute exacerbation compared to other ACTs in bronchiectasis is unknown. The primary aim of this review was to determine the effects of PEP therapy compared with other ACTs on health-related quality of life (HRQOL), rate of acute exacerbations, and incidence of hospitalisation in individuals with stable or an acute exacerbation of bronchiectasis.Secondary aims included determining the effects of PEP therapy upon physiological outcomes and clinical signs and symptoms compared with other ACTs in individuals with stable or an acute exacerbation of bronchiectasis. We searched the Cochrane Airways Group Specialised Register of Trials, PEDro and clinical trials registries from inception to February 2017 and we handsearched relevant journals. Randomised controlled parallel and cross-over trials that compared PEP therapy versus other ACTs in participants with bronchiectasis. We used standard methodological procedures as outlined by Cochrane. Nine studies involving 213 participants met the inclusion criteria, of which seven were cross-over in design. All studies included adults with bronchiectasis, with eight including participants in a stable clinical state and one including participants experiencing an acute exacerbation. Eight studies used oscillatory PEP therapy, using either a Flutter or Acapella device and one study used Minimal PEP therapy. The comparison intervention differed between studies. The methodological quality of studies was poor, with cross-over studies including suboptimal or no washout period, and a lack of blinding of participants, therapists or personnel for outcome measure assessment in most studies. Clinical heterogeneity between studies limited meta-analysis.Daily use of oscillatory PEP therapy for four weeks was associated with improved general health according to the Short-Form 36 questionnaire compared to the active cycle of breathing technique (ACBT). When applied for three sessions over one week, minimal PEP therapy resulted in similar improvement in cough-related quality of life as autogenic drainage (AD) and L'expiration Lente Totale Glotte Ouverte en Decubitus Lateral (ELTGOL). Oscillatory PEP therapy twice daily for four weeks had similar effects on disease-specific HRQOL (MD -0.09, 95% CI -0.37 to 0.19; low-quality evidence). Data were not available to determine the incidence of hospitalisation or rate of exacerbation in clinically stable participants.Two studies of a single session comparison of oscillatory PEP therapy and gravity-assisted drainage (GAD) with ACBT had contrasting findings. One study found a similar sputum weight produced with both techniques (SMD 0.54g (-0.38 to 1.46; 20 participants); the other found greater sputum expectoration with GAD and ACBT (SMD 5.6 g (95% CI 2.91 to 8.29: 36 participants). There was no difference in sputum weight yielded between oscillatory PEP therapy and ACBT with GAD when applied daily for four weeks or during an acute exacerbation. Although a single session of oscillatory PEP therapy was associated with less sputum compared to AD (median difference 3.1 g (95% CI 1.5 to 4.8 g; one study, 31 participants), no difference between oscillatory PEP therapy and seated ACBT was evident. PEP therapy had a similar effect on dynamic and static measures of lung volumes and gas exchange as all other ACTs. A single session of oscillatory PEP therapy (Flutter) generated a similar level of fatigue as ACBT with GAD, but greater fatigue was noted with oscillatory PEP therapy compared to ACBT alone. The degree of breathlessness experienced with PEP therapy did not differ from other techniques. Among studies exploring adverse events, only one study reported nausea with use of oscillatory PEP therapy. PEP therapy appears to have similar effects on HRQOL, symptoms of breathlessness, sputum expectoration, and lung volumes compared to other ACTs when prescribed within a stable clinical state or during an acute exacerbation. The number of studies and the overall quality of the evidence were both low. In view of the chronic nature of bronchiectasis, additional information is needed to establish the long-term clinical effects of PEP therapy over other ACTs for outcomes that are important to people with bronchiectasis and on clinical parameters which impact on disease progression and patient morbidity in individuals with stable bronchiectasis. In addition, the role of PEP therapy during an acute exacerbation requires further exploration. This information is necessary to provide further guidance for prescription of PEP therapy for people with bronchiectasis.

Bronchiectasis exacerbation study on azithromycin and amoxycillin-clavulanate for respiratory exacerbations in children (BEST-2): study protocol for a randomized controlled trial.

PubMed

Chang, Anne B; Grimwood, Keith; Wilson, Andrew C; van Asperen, Peter P; Byrnes, Catherine A; O'Grady, Kerry-Ann F; Sloots, Theo P; Robertson, Colin F; Torzillo, Paul J; McCallum, Gabrielle B; Masters, Ian B; Buntain, Helen M; Mackay, Ian M; Ungerer, Jacobus; Tuppin, Joanne; Morris, Peter S

2013-02-20

Bronchiectasis unrelated to cystic fibrosis (CF) is being increasingly recognized in children and adults globally, both in resource-poor and in affluent countries. However, high-quality evidence to inform management is scarce. Oral amoxycillin-clavulanate is often the first antibiotic chosen for non-severe respiratory exacerbations, because of the antibiotic-susceptibility patterns detected in the respiratory pathogens commonly associated with bronchiectasis. Azithromycin has a prolonged half-life, and with its unique anti-bacterial, immunomodulatory, and anti-inflammatory properties, presents an attractive alternative. Our proposed study will test the hypothesis that oral azithromycin is non-inferior (within a 20% margin) to amoxycillin-clavulanate at achieving resolution of non-severe respiratory exacerbations by day 21 of treatment in children with non-CF bronchiectasis. This will be a multicenter, randomized, double-blind, double-dummy, placebo-controlled, parallel group trial involving six Australian and New Zealand centers. In total, 170 eligible children will be stratified by site and bronchiectasis etiology, and randomized (allocation concealed) to receive: 1) azithromycin (5 mg/kg daily) with placebo amoxycillin-clavulanate or 2) amoxycillin-clavulanate (22.5 mg/kg twice daily) with placebo azithromycin for 21 days as treatment for non-severe respiratory exacerbations. Clinical data and a parent-proxy cough-specific quality of life (PC-QOL) score will be obtained at baseline, at the start and resolution of exacerbations, and on day 21. In most children, blood and deep-nasal swabs will also be collected at the same time points. The primary outcome is the proportion of children whose exacerbations have resolved at day 21. The main secondary outcome is the PC-QOL score. Other outcomes are: time to next exacerbation; requirement for hospitalization; duration of exacerbation, and spirometry data. Descriptive viral and bacteriological data from nasal samples and blood inflammatory markers will be reported where available. Currently, there are no published randomized controlled trials (RCT) to underpin effective, evidence-based management of acute respiratory exacerbations in children with non-CF bronchiectasis. To help address this information gap, we are conducting two RCTs. The first (bronchiectasis exacerbation study; BEST-1) evaluates the efficacy of azithromycin and amoxycillin-clavulanate compared with placebo, and the second RCT (BEST-2), described here, is designed to determine if azithromycin is non-inferior to amoxycillin-clavulanate in achieving symptom resolution by day 21 of treatment in children with acute respiratory exacerbations. Australia and New Zealand Clinical Trials Register (ANZCTR) number http://ACTRN12612000010897. http://www.anzctr.org.au/trial_view.aspx?id=347879.

Bronchiectasis exacerbation study on azithromycin and amoxycillin-clavulanate for respiratory exacerbations in children (BEST-2): study protocol for a randomized controlled trial

PubMed Central

2013-01-01

Background Bronchiectasis unrelated to cystic fibrosis (CF) is being increasingly recognized in children and adults globally, both in resource-poor and in affluent countries. However, high-quality evidence to inform management is scarce. Oral amoxycillin-clavulanate is often the first antibiotic chosen for non-severe respiratory exacerbations, because of the antibiotic-susceptibility patterns detected in the respiratory pathogens commonly associated with bronchiectasis. Azithromycin has a prolonged half-life, and with its unique anti-bacterial, immunomodulatory, and anti-inflammatory properties, presents an attractive alternative. Our proposed study will test the hypothesis that oral azithromycin is non-inferior (within a 20% margin) to amoxycillin-clavulanate at achieving resolution of non-severe respiratory exacerbations by day 21 of treatment in children with non-CF bronchiectasis. Methods This will be a multicenter, randomized, double-blind, double-dummy, placebo-controlled, parallel group trial involving six Australian and New Zealand centers. In total, 170 eligible children will be stratified by site and bronchiectasis etiology, and randomized (allocation concealed) to receive: 1) azithromycin (5 mg/kg daily) with placebo amoxycillin-clavulanate or 2) amoxycillin-clavulanate (22.5 mg/kg twice daily) with placebo azithromycin for 21 days as treatment for non-severe respiratory exacerbations. Clinical data and a parent-proxy cough-specific quality of life (PC-QOL) score will be obtained at baseline, at the start and resolution of exacerbations, and on day 21. In most children, blood and deep-nasal swabs will also be collected at the same time points. The primary outcome is the proportion of children whose exacerbations have resolved at day 21. The main secondary outcome is the PC-QOL score. Other outcomes are: time to next exacerbation; requirement for hospitalization; duration of exacerbation, and spirometry data. Descriptive viral and bacteriological data from nasal samples and blood inflammatory markers will be reported where available. Discussion Currently, there are no published randomized controlled trials (RCT) to underpin effective, evidence-based management of acute respiratory exacerbations in children with non-CF bronchiectasis. To help address this information gap, we are conducting two RCTs. The first (bronchiectasis exacerbation study; BEST-1) evaluates the efficacy of azithromycin and amoxycillin-clavulanate compared with placebo, and the second RCT (BEST-2), described here, is designed to determine if azithromycin is non-inferior to amoxycillin-clavulanate in achieving symptom resolution by day 21 of treatment in children with acute respiratory exacerbations. Trial registration Australia and New Zealand Clinical Trials Register (ANZCTR) number http://ACTRN12612000010897. http://www.anzctr.org.au/trial_view.aspx?id=347879 PMID:23421781

Short-Term Effect of Autogenic Drainage on Ventilation Inhomogeneity in Adult Subjects With Stable Non-Cystic Fibrosis Bronchiectasis.

PubMed

Poncin, William; Reychler, Grégory; Leeuwerck, Noémie; Bauwens, Nathalie; Aubriot, Anne-Sophie; Nader, Candice; Liistro, Giuseppe; Gohy, Sophie

2017-05-01

Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from a multiple-breath washout test, is a promising tool for assessing airway function in patients with non-cystic fibrosis bronchiectasis. However, it is unknown whether ventilation inhomogeneity could improve after successful elimination of excessive secretions within bronchiectasis. The objective of this work was to assess the short-term effects of lung secretion clearance using the autogenic drainage technique on standard lung function tests and LCI in subjects with non-cystic fibrosis bronchiectasis. Nitrogen-based multiple-breath washout, spirometry, and body plethysmography tests were performed 30 min before autogenic drainage in adults with stable non-cystic fibrosis bronchiectasis. The autogenic drainage session was followed by a 5-min break, after which the tests were repeated in the same order. Sputum expectorated during autogenic drainage was quantified as dry weight and correlated with change between post- and pre-measurements (Δ). Paired t test or Wilcoxon signed-rank tests were used to compare pre- and post-autogenic drainage measurement outcomes. A P value of ≤.05 was considered as statistically significant. Twenty-four subjects were studied (18 females, median age [range]: 65 [21-81] y). Mean ± SD LCI significantly improved after autogenic drainage (10.88 ± 2.62 vs 10.53 ± 2.35, P = .042). However, only 20% of subjects with mucus hyperproduction during autogenic drainage had a ΔLCI that exceeded measurement variability. The percent of predicted slow vital capacity (SVC%) also slightly improved (88.7 ± 19.3% vs 90 ± 19.1%, P = .02). ΔLCI was inversely related to dry sputum weight (r = -.48, P = .02) and ΔSVC% (r = -.64, P = .001). ΔSVC% also correlated with dry sputum weight (r = 0.46, P = .02). In adults with non-cystic fibrosis bronchiectasis and mucus hypersecretion, autogenic drainage improved ventilation inhomogeneity. LCI change may be the result of the maximum recruited lung volume and the amount of cleared mucus secretion. (ClinicalTrials.gov registration NCT02411981.). Copyright © 2017 by Daedalus Enterprises.

The validity of health-related quality of life questionnaires in bronchiectasis: a systematic review and meta-analysis.

PubMed

Spinou, Arietta; Fragkos, Konstantinos C; Lee, Kai K; Elston, Caroline; Siegert, Richard J; Loebinger, Michael R; Wilson, Robert; Garrod, Rachel; Birring, Surinder S

2016-08-01

A range of questionnaires have been used to assess health-related quality of life (HRQOL) in bronchiectasis. A systematic review was conducted to evaluate their psychometric properties and assess associations between HRQOL and clinical measures. Five electronic databases were searched. Studies eligible for inclusion were those that investigated the validity of HRQOL questionnaires and/or their association with other outcomes in adults with bronchiectasis. Patients with cystic fibrosis were excluded. The identified questionnaires were assessed for convergent, discriminant and cross-cultural translation validity; missing data, floor and ceiling effects, internal consistency, responsiveness and test-retest reliability. A meta-analysis was conducted to estimate the strength of associations between HRQOL and clinical measures. From 1918 studies identified, 43 studies were included in the systematic review, of which 38 were suitable for the meta-analysis. Nine HRQOL questionnaires were identified, with the most widely used being: St George's Respiratory Questionnaire, Leicester Cough Questionnaire, Quality of Life-Bronchiectasis and Short Form-36. HRQOL questionnaires had moderate to good internal consistency and good test-retest reliability. Only 8 of 18 studies that used translated HRQOL questionnaires reported or referred to the validity of the translated questionnaire. There was a stronger correlation (mean r (95% CI)) between HRQOL and subjective outcome measures, such as dyspnoea (0.55 (0.41 to 0.68)) and fatigue (0.42 (0.23 to 0.58)) compared with objective measures; exercise capacity (-0.41 (-0.54 to -0.24)), FEV1% predicted (-0.31 (-0.40 to -0.23)) and extent of bronchiectasis on CT scan (0.35 (0.03 to 0.61)); all p<0.001. This review supports most HRQOL questionnaires used in bronchiectasis have good psychometric properties. There was a weak to moderate association between HRQOL and objective outcome measures. This suggests that HRQOL questionnaires assess a unique aspect of health not captured by objective measures. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Increased disease activity, severity and autoantibody positivity in rheumatoid arthritis patients with co-existent bronchiectasis.

PubMed

Perry, Elizabeth; Eggleton, Paul; De Soyza, Anthony; Hutchinson, David; Kelly, Clive

2017-12-01

Patients with rheumatoid arthritis (RA) and co-existent bronchiectasis (BRRA) have a five-fold increased mortality compared to rheumatoid arthritis alone. Yet previous studies have found no difference in clinical and serological markers of RA disease severity between BRRA patients and RA alone. However, RA disease activity measures such as Disease Activity Score of 28 joints - C-reactive protein (DAS28-CRP) and anti-cyclic citrullinated peptide antibodies (anti-CCP) have not been studied, so we assessed these parameters in patients with BRRA and RA alone. BRRA patients (n = 53) had high-resolution computed tomography proven bronchiectasis without any interstitial lung disease and ≥ 2 respiratory infections/year. RA alone patients (n = 50) had no clinical or radiological evidence of lung disease. DAS28-CRP, rheumatoid factor (immunoglobulin M) and anti-CCP were measured in all patients, together with detailed clinical and radiology records. In BRRA, bronchiectasis predated RA in 58% of patients. BRRA patients had higher DAS28 scores (3.51 vs. 2.59), higher levels of anti-CCP (89% vs. 46%) and rheumatoid factor (79% vs. 52%) (P = 0.003) compared to RA alone. Where hand and foot radiology findings were recorded, 29/37 BRRA (78%) and 13/30 (43%) RA alone had evidence of erosive change (P = 0.003). There were no significant differences between groups in smoking history or disease-modifying anti-rheumatic drug/biologic therapy. Increased levels of RA disease activity, severity and RA autoantibodies are demonstrated in patients with RA and co-existent bronchiectasis compared to patients with RA alone, despite lower tobacco exposure. This study demonstrates that BRRA is a more severe systemic disease than RA alone. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

Validity and Reliability of the Chronic Respiratory Disease Questionnaire in Elderly Individuals with Mild to Moderate Non-Cystic Fibrosis Bronchiectasis.

PubMed

Vodanovich, Domagoj A; Bicknell, Thomas J; Holland, Anne E; Hill, Catherine J; Cecins, Nola; Jenkins, Sue; McDonald, Christine F; Burge, Angela T; Thompson, Philip; Stirling, Robert G; Lee, Annemarie L

2015-01-01

The chronic respiratory disease questionnaire (CRDQ) is designed to assess health-related quality of life (HRQOL) in chronic respiratory conditions, but its reliability, validity and responsiveness in individuals with mild to moderate non-cystic fibrosis (CF) bronchiectasis are unclear. This study aimed to determine measurement properties of the CRDQ in non-CF bronchiectasis. Participants with non-CF bronchiectasis involved in a randomised controlled trial of exercise training were recruited. Internal consistency was assessed using Cronbach's α. Over 8 weeks, reliability was evaluated using intra-class correlation coefficients and Bland-Altman analysis for measures of agreement. Convergent and divergent validity was assessed by correlations with the other HRQOL questionnaires and the Hospital Anxiety and Depression Scale (HADS). The responsiveness to exercise training was assessed using effect sizes and standardised response means. Eighty-five participants were included (mean age ± SD, 64 ± 13 years). Internal consistency was adequate (>0.7) for all CRDQ domains and the total score. Test-retest reliability ranged from 0.69 to 0.85 for each CRDQ domain and was 0.82 for the total score. Dyspnoea (CRDQ) was related to St George's respiratory questionnaire (SGRQ) symptoms only (r = 0.38), with no relationship to the Leicester cough questionnaire (LCQ) or HADS. Moderate correlations were found between the total score of the CRDQ, the SGRQ (rs = -0.49) and the LCQ score (rs = 0.51). Lower CRDQ scores were associated with higher anxiety and depression (rs = -0.46 to -0.56). The responsiveness of the CRDQ was small (effect size 0.1-0.24). The CRDQ is a valid and reliable measure of HRQOL in mild to moderate non-CF bronchiectasis, but responsiveness was limited. © 2015 S. Karger AG, Basel.

Inhaled Colistin in Patients with Bronchiectasis and Chronic Pseudomonas aeruginosa Infection

PubMed Central

Foweraker, Juliet E.; Wilkinson, Peter; Kenyon, Robert F.; Bilton, Diana

2014-01-01

Rationale: Chronic infection with Pseudomonas aeruginosa is associated with an increased exacerbation frequency, a more rapid decline in lung function, and increased mortality in patients with bronchiectasis. Objectives: To perform a randomized placebo-controlled study assessing the efficacy and safety of inhaled colistin in patients with bronchiectasis and chronic P. aeruginosa infection. Methods: Patients with bronchiectasis and chronic P. aeruginosa infection were enrolled within 21 days of completing a course of antipseudomonal antibiotics for an exacerbation. Participants were randomized to receive colistin (1 million IU; n = 73) or placebo (0.45% saline; n = 71) via the I-neb twice a day, for up to 6 months. Measurements and Main Results: The primary endpoint was time to exacerbation. Secondary endpoints included time to exacerbation based on adherence recorded by the I-neb, P. aeruginosa bacterial density, quality of life, and safety parameters. All analyses were on the intention-to-treat population. Median time (25% quartile) to exacerbation was 165 (42) versus 111 (52) days in the colistin and placebo groups, respectively (P = 0.11). In adherent patients (adherence quartiles 2–4), the median time to exacerbation was 168 (65) versus 103 (37) days in the colistin and placebo groups, respectively (P = 0.038). P. aeruginosa density was reduced after 4 (P = 0.001) and 12 weeks (P = 0.008) and the St. George’s Respiratory Questionnaire total score was improved after 26 weeks (P = 0.006) in the colistin versus placebo patients, respectively. There were no safety concerns. Conclusions: Although the primary endpoint was not reached, this study shows that inhaled colistin is a safe and effective treatment in adherent patients with bronchiectasis and chronic P. aeruginosa infection. Clinical trial registered with http://www.isrctn.org/ (ISRCTN49790596) PMID:24625200

Inhaled mannitol for non-cystic fibrosis bronchiectasis: a randomised, controlled trial.

PubMed

Bilton, Diana; Tino, Gregory; Barker, Alan F; Chambers, Daniel C; De Soyza, Anthony; Dupont, Lieven J A; O'Dochartaigh, Conor; van Haren, Eric H J; Vidal, Luis Otero; Welte, Tobias; Fox, Howard G; Wu, Jian; Charlton, Brett

2014-12-01

Bronchiectasis is characterised by excessive production of mucus and pulmonary exacerbations. Inhaled osmotic agents may enhance mucociliary clearance, but few long-term clinical trials have been conducted. To determine the impact of inhaled mannitol on exacerbation rates in patients with non-cystic fibrosis (CF) bronchiectasis. Secondary endpoints included time to first exacerbation, duration of exacerbations, antibiotic use for exacerbations and quality of life (QOL) (St George's Respiratory Questionnaire, SGRQ). Patients with non-CF bronchiectasis and a history of chronic excess production of sputum and ≥2 pulmonary exacerbations in the previous 12 months were randomised (1:1) to 52 weeks treatment with inhaled mannitol 400 mg or low-dose mannitol control twice a day. Patients were 18-85 years of age, baseline FEV1 ≥40% and ≤85% predicted and a baseline SGRQ score ≥30. 461 patients (233 in the mannitol and 228 in the control arm) were treated. Baseline demographics were similar in the two arms. The exacerbation rate was not significantly reduced on mannitol (rate ratio 0.92, p=0.31). However, time to first exacerbation was increased on mannitol (HR 0.78, p=0.022). SGRQ score was improved on mannitol compared with low-dose mannitol control (-2.4 units, p=0.046). Adverse events were similar between groups. Mannitol 400 mg inhaled twice daily for 12 months in patients with clinically significant bronchiectasis did not significantly reduce exacerbation rates. There were statistically significant improvements in time to first exacerbation and QOL. Mannitol therapy was safe and well tolerated. NCT00669331. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Self-administered acupressure reduces the symptoms that limit daily activities in bronchiectasis patients: pilot study findings.

PubMed

Maa, Suh-Hwa; Tsou, Tsung-Shan; Wang, Kwua-Yun; Wang, Chun-Hua; Lin, Horng-Chyuan; Huang, Yi-Hsiang

2007-04-01

To examine and compare the effects of acupressure on the perceived health-related quality of life of the participants with bronchiectasis. In an attempt to offer comfort, pain control and symptom management, nursing is becoming increasingly involved in offering complementary-alternative medicine as part of its caring-healing focus in comprehensive patient care. Acupressure is one such modality that is being increasingly used by both medical and nursing professionals. While acupressure has been reported to have beneficial effects in patients with respiratory disease, the benefits to bronchiectasis patients have remained uncertain. A randomized, partially blinded study consisting of three groups. Thirty-five out-patients of both genders, aged 59.46 SD 11.52 years, who were suffering from bronchiectasis, were randomly split into one of three groups: standard care with supplemental acupressure for eight weeks (11 participants); standard care with supplemental sham acupressure for eight weeks (11 participants); and standard care alone (13 participants). Outcomes were determined by changes in daily sputum amounts, sputum self-assessment, six-minute walking distance, breathing difficulty (measured on the dyspnea visual analogue scale) and health-related quality of life (measured by the Saint George Respiratory Questionnaire). The sputum self-assessment score improved over time for the sham acupressure participants (P = 0.03), when compared with the controls. For acupressure participants, the Saint George respiratory questionnaire activity component scores also improved over time, compared with controls (P = 0.01) after adjustment for covariates (treatment, time, age, sex and baseline values). Other variables did not differ between the standard care alone group and the other two groups. Eight weeks of self-administered acupressure could be useful in reducing the effects of bronchiectasis on a patient's daily activities. Acupressure may be regarded as a viable nursing intervention.

Inhaled, dual release liposomal ciprofloxacin in non-cystic fibrosis bronchiectasis (ORBIT-2): a randomised, double-blind, placebo-controlled trial

PubMed Central

Serisier, David J; Bilton, Diana; De Soyza, Anthony; Thompson, Philip J; Kolbe, John; Greville, Hugh W; Cipolla, David; Bruinenberg, Paul; Gonda, Igor

2013-01-01

Background The delivery of antipseudomonal antibiotics by inhalation to Pseudomonas aeruginosa-infected subjects with non-cystic fibrosis (CF) bronchiectasis is a logical extension of treatment strategies successfully developed in CF bronchiectasis. Dual release ciprofloxacin for inhalation (DRCFI) contains liposomal ciprofloxacin, formulated to optimise airway antibiotic delivery. Methods Phase II, 24-week Australian/New Zealand multicentre, randomised, double-blind, placebo-controlled trial in 42 adult bronchiectasis subjects with ≥2 pulmonary exacerbations in the prior 12 months and ciprofloxacin-sensitive P aeruginosa at screening. Subjects received DRCFI or placebo in three treatment cycles of 28 days on/28 days off. The primary outcome was change in sputum P aeruginosa bacterial density to the end of treatment cycle 1 (day 28), analysed by modified intention to treat (mITT). Key secondary outcomes included safety and time to first pulmonary exacerbation—after reaching the pulmonary exacerbation endpoint subjects discontinued study drug although remained in the study. Results DRCFI resulted in a mean (SD) 4.2 (3.7) log10 CFU/g reduction in P aeruginosa bacterial density at day 28 (vs −0.08 (3.8) with placebo, p=0.002). DRCFI treatment delayed time to first pulmonary exacerbation (median 134 vs 58 days, p=0.057 mITT, p=0.046 per protocol). DRCFI was well tolerated with a similar incidence of systemic adverse events to the placebo group, but fewer pulmonary adverse events. Conclusions Once-daily inhaled DRCFI demonstrated potent antipseudomonal microbiological efficacy in adults with non-CF bronchiectasis and ciprofloxacin-sensitive P aeruginosa. In this modest-sized phase II study, DRCFI was also well tolerated and delayed time to first pulmonary exacerbation in the per protocol population. PMID:23681906

Vitamin D3 supplementation in adults with bronchiectasis: A pilot study.

PubMed

Bartley, Jim; Garrett, Jeff; Camargo, Carlos A; Scragg, Robert; Vandal, Alain; Sisk, Rose; Milne, David; Tai, Ray; Jeon, Gene; Cursons, Ray; Wong, Conroy

2018-01-01

Vitamin D supplementation prevents acute respiratory infections and, through modulating innate and adaptive immunity, could have a potential role in bronchiectasis management. The primary aims of this pilot study were to assess serum 25-hydroxyvitamin D (25(OH)D) levels in New Zealand adults with bronchiectasis, and their 25(OH)D levels after vitamin D 3 supplementation. Adults with bronchiectasis received an initial 2.5 mg vitamin D 3 oral loading dose and 0.625 mg vitamin D 3 weekly for 24 weeks. The primary outcome was serum 25(OH)D levels before and after vitamin D 3 supplementation. Secondary outcomes (time to first infective exacerbation, exacerbation frequency, spirometry, health-related quality of life measures, sputum bacteriology and cell counts and chronic rhinosinusitis) were also assessed. This study is registered with the Australian New Zealand Clinical Trials Registry (ACTRN 12612001222831). The initial, average 25(OH)D level was 71 nmol/L (95% confidence interval (CI): [58, 84]), rising to 218 nmol/L (95% CI: [199, 237]) at 12 weeks and 205 nmol/L (95% CI: [186, 224]) at 24 weeks. The initial serum cathelicidin level was 25 nmol/L (95% CI: [17, 33]), rising to 102 nmol/L (95% CI: [48, 156]) at 12 weeks and 151 nmol/L (95% CI: [97, 205]) at 24 weeks. Over the 24-week study period, we observed statistically significant changes of 1.11 (95% CI: [0.08, 2.14]) in the Leicester Cough Questionnaire and -1.97 (95% CI: [-3.71, -0.23]) in the Dartmouth COOP charts score. No significant adverse effects were recorded. Many New Zealand adults with bronchiectasis have adequate 25(OH)D levels. Weekly vitamin D 3 supplementation significantly improved 25(OH)D levels.

[Respiratory manifestations of yellow nail syndrome: report of two cases and literature review].

PubMed

Li, S; Huang, H; Xu, K; Xu, Z J

2018-03-12

Objective: To describe the clinical characteristics of respiratory manifestations of yellow nail syndrome. Methods: We conducted a retrospective analysis of 2 patients with respiratory diseases associated with yellow nail syndrome. Their clinical and chest radiological data were collected. We searched PubMed, Wanfang and CNKI databases with the keywords "yellow nail syndrome, yellow nail and lung" in Chinese and English. And the relevant literatures, including 6 articles in Chinese and 81 articles in English, were reviewed. Results: Our 2 patients were male, one 60 years old and the other 76. Typical yellow nails were present in their fingers, and one of them also showed toe yellow nails. One patient was admitted for refractory respiratory infection and he was diagnosed with diffuse bronchiectasis. The respiratory symptoms could be relieved with antibiotics according to the results of sputum microbiological analysis. The other patient was admitted for cough and exertional dyspnea, and refractory pleural effusions were revealed bilaterally. He received repeated effusion drainage by thoracentesis, and Octreotide was tried recently. A total of 373 cases were reviewed in Chinese and English literatures. Pleural effusions (152 cases) and diffuse bronchiectasis (121 cases) were the most common reported respiratory manifestations. Lymphoedema was present in almost all cases with pleural effusion associated with yellow nail syndrome, and the effusion was usually exudative and lymphocyte predominant. Pleurodesis and decortication were effective for them. But, somatostatin analogues had been tried effectively for these patients recently. On the other hand, literatures showed that diffuse bronchiectasis in yellow nail syndrome was less severe than idiopathic diffuse bronchiectasis, and might benefit from long-term macrolide antibiotics. Conclusions: Yellow nail syndrome is a very rare disorder. Besides yellow nail, respiratory manifestations are the main clinical presentations. Diffuse bronchiectasis and recurrent pleural effusions are the common manifestations.

An Epidemiological Overview of Chronic Obstructive Pulmonary Disease: What Can Real-Life Data Tell Us about Disease Management?

PubMed

Soriano, Joan B

2017-03-15

Chronic obstructive pulmonary disease (COPD) is a common condition, associated with increasing age and smoking exposure. COPD is a leading cause of morbidity, mortality and health care expenditure worldwide; yet, only 10-15% of all cases are identified medically. Alpha-1-antitrypsin deficiency (AATD) is responsible for about 1% of COPD cases but is also largely under-recognised, leading to diagnostic delay and missed treatment opportunities in patients who remain undetected. New evidence has recently highlighted the extent of overlap between COPD and bronchiectasis and the implications of comorbidity on clinical course and mortality. COPD with comorbid bronchiectasis deserves to be given research priority. This article overviews the epidemiology of COPD and examines the implications of overlap between COPD and AATD and between COPD and bronchiectasis.

Diagnosis and management of bronchiectasis.

PubMed

Smith, Maeve P

2017-06-19

KEY POINTS Following a diagnosis of bronchiectasis, it is important to investigate for an underlying cause. Goals of management are to suppress airway infection and inflammation, to improve symptoms and health-related quality of life. There are now validated scoring tools to help assess disease severity, which can help to stratify management. Good evidence supports the use of both exercise training and long-term macrolide therapy in long-term disease management.

Spanish Guidelines on Treatment of Bronchiectasis in Adults.

PubMed

Martínez-García, Miguel Ángel; Máiz, Luis; Olveira, Casilda; Girón, Rosa Maria; de la Rosa, David; Blanco, Marina; Cantón, Rafael; Vendrell, Montserrat; Polverino, Eva; de Gracia, Javier; Prados, Concepción

2018-02-01

In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest therapies available for bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure quality of content, and are linked to a large amount of online information that includes a wealth of references. The guidelines are focused on the treatment of bronchiectasis from both a multidisciplinary perspective, including specialty areas and the different healthcare levels involved, and a multidimensional perspective, including a comprehensive overview of the specific aspects of the disease. A series of recommendations have been drawn up, based on an in-depth review of the evidence for treatment of the underlying etiology, the bronchial infection in its different forms of presentation using existing therapies, bronchial inflammation, and airflow obstruction. Nutritional aspects, management of secretions, muscle training, management of complications and comorbidities, infection prophylaxis, patient education, home care, surgery, exacerbations, and patient follow-up are addressed. Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.

The Likelihood of Preventing Respiratory Exacerbations in Children and Adolescents with either Chronic Suppurative Lung Disease or Bronchiectasis

PubMed Central

O’Grady, Kerry-Ann F; Grimwood, Keith

2017-01-01

Chronic suppurative lung disease (CSLD) and bronchiectasis in children and adolescents are important causes of respiratory morbidity and reduced quality of life (QoL), also leading to subsequent premature death during adulthood. Acute respiratory exacerbations in pediatric CSLD and bronchiectasis are important markers of disease control clinically, given that they impact upon QoL and increase health-care-associated costs and can adversely affect future lung functioning. Preventing exacerbations in this population is, therefore, likely to have significant individual, familial, societal, and health-sector benefits. In this review, we focus on therapeutic interventions, such as drugs (antibiotics, mucolytics, hyperosmolar agents, bronchodilators, corticosteroids, non-steroidal anti-inflammatory agents), vaccines and physiotherapy, and care-planning, such as post-hospitalization management and health promotion strategies, including exercise, diet, and reducing exposure to environmental toxicants. The review identified a conspicuous lack of moderate or high-quality evidence for preventing respiratory exacerbations in children and adolescents with CSLD or bronchiectasis. Given the short- and long-term impact of exacerbations upon individuals, their families, and society as a whole, large studies addressing interventions at the primary and tertiary prevention phases are required. This research must include children and adolescents in both developing and developed countries and address long-term health outcomes. PMID:28393062

Mediterranean diet is associated on symptoms of depression and anxiety in patients with bronchiectasis.

PubMed

Olveira, Casilda; Olveira, Gabriel; Espildora, Francisco; Girón, Rosa-María; Vendrell, Montserrat; Dorado, Antonio; Martínez-García, Miguel-Ángel

2014-01-01

The aim was to measure symptoms of depression and anxiety in patients with bronchiectasis and evaluate their relationship with a Mediterranean diet. This cross-sectional study recruited patients with bronchiectasis at four Spanish centers. Patients completed the hospital anxiety and depression scale (HADS) and the Mediterranean diet questionnaire (PREDIMED). Demographic, health and outcome data were recorded from medical charts. Logistic regression was used to determine the predictors of elevated symptoms of depression and anxiety (HADS≥11). Of the 205 participants recruited, 37 (18.0%) had elevated anxiety-related scores and 26 (12.7%) had elevated depression-related scores (HADS≥11). Increased symptoms of depression were significantly associated with being unemployed, a lower education, older age, comorbidity, major dyspnea, worse quality of life (QOL) and a lower PREDIMED score. Increased symptoms of anxiety were significantly associated with more exacerbations, worse QOL and a lower PREDIMED score. Regression analyses indicated that, after adjustment, QOL and the PREDIMED score predicted elevated symptoms of depression and QOL predicted elevated symptoms of anxiety. The prevalence of elevated symptoms of depression and anxiety is high in patients with bronchiectasis and greater adherence to a Mediterranean diet is associated with a lower likelihood of having these symptoms, particularly for depression. Copyright © 2014 Elsevier Inc. All rights reserved.

Bronchiectasis: Current Concepts in Pathogenesis, Immunology, and Microbiology.

PubMed

Boyton, Rosemary J; Altmann, Daniel M

2016-05-23

Bronchiectasis is a disorder of persistent lung inflammation and recurrent infection, defined by a common pathological end point: irreversible bronchial dilatation arrived at through diverse etiologies. This suggests an interplay between immunogenetic susceptibility, immune dysregulation, bacterial infection, and lung damage. The damaged epithelium impairs mucus removal and facilitates bacterial infection with increased cough, sputum production, and airflow obstruction. Lung infection is caused by respiratory bacterial and fungal pathogens, including Pseudomonas aeruginosa, Haemophilus, Aspergillus fumigatus, and nontuberculous mycobacteria. Recent studies have highlighted the relationship between the lung microbiota and microbial-pathogen niches. Disease may result from environments favoring interleukin-17-driven neutrophilia. Bronchiectasis may present in autoimmune disease, as well as conditions of immune dysregulation, such as combined variable immune deficiency, transporter associated with antigen processing-deficiency syndrome, and hyperimmunoglobulin E syndrome. Differences in prevalence across geography and ethnicity implicate an etiological mix of genetics and environment underpinning susceptibility.

Evaluation of a novel information resource for patients with bronchiectasis: study protocol for a randomised controlled trial.

PubMed

Hester, Katy L M; Newton, Julia; Rapley, Tim; De Soyza, Anthony

2016-04-23

There is currently little patient information on bronchiectasis, a chronic lung disease with rising prevalence. Previous work shows that patients and their families want more information, which could potentially improve their understanding and self-management. Using interviews and focus groups, we have co-developed a novel patient and carer information resource, aiming to meet their identified needs. The aims and objectives are: 1. To assess the potential impact of the information resource 2. To evaluate and refine the intervention 3. To establish the feasibility of carrying out a multi-centre randomised controlled trial to determine its effect on understanding, self-management and health outcomes This is a feasibility study, with a single-centre, randomised controlled trial design, comparing use of a novel patient information resource to usual care in bronchiectasis. Additionally, patients and carers will be invited to focus groups to discuss their views on both the intervention itself and the trial process. The study duration for each participant will be 3 months from the study entry date. A total of 70 patients will be recruited to the study, and a minimum of 30 will be randomised to each arm. Ten participants (and their carers if applicable) will be invited to attend focus groups on completion of the study visits. Participants will be adults with bronchiectasis diagnosed as per national bronchiectasis guidelines. Once consented, participants will be randomised to the intervention or control arm using random permuted blocks to ensure treatment group numbers are evenly balanced. Randomisation will be web-based. Those randomised to the intervention will receive the information resource (website and booklet) and instructions on its use. Outcome measures (resource satisfaction, resource use and alternative information seeking, quality of life questionnaires, unscheduled healthcare visits, exacerbation frequency, bronchiectasis knowledge questionnaire and lung function tests) will be recorded at baseline, 2 weeks and 3 months. All outcome measures will be used in assessing feasibility and acceptability of a future definitive trial. Feasibility outcomes include recruitment, retention and study scale form completion rates. Focus groups will strengthen qualitative data for resource refinement and to identify participant views on the trial process, which will also inform feasibility assessments. Questionnaires will also be used to evaluate and refine the resource. ISRCTN84229105.

Pseudomonas aeruginosa isolation in patients with non-cystic fibrosis bronchiectasis: a retrospective study.

PubMed

Wang, Hong; Ji, Xiao-Bin; Mao, Bei; Li, Cheng-Wei; Lu, Hai-Wen; Xu, Jin-Fu

2018-03-14

Pseudomonas aeruginosa (P. aeruginosa) occupies an important niche in the pathogenic microbiome of bronchiectasis. The objective of this study is to evaluate the clinical characteristics and prognostic value of P. aeruginosa in Chinese adult patients with bronchiectasis. This retrospective and follow-up study enrolled 1188 patients diagnosed with bronchiectasis at Shanghai Pulmonary Hospital between January 2011 and December 2012. The patients' clinical data including anthropometry, clinical symptoms, serum biomarkers, radiographic manifestations and lung function indices were reviewed. The median follow-up duration (IQR) was 44 (40-54) months, during which 289 patients were lost to follow-up. Data from 899 patients were collected and analysed for the outcomes of mortality, annual exacerbation frequency and health-related quality of life. P. aeruginosa was isolated from 232 patients, alongside other pathogens such as Aspergillus (n=75) and Candida albicans (n=72). There were 74 deaths (12% of patients with P. aeruginosa , 7.3% of those without) over the course of the follow-up. The isolation of P. aeruginosa was a risk factor for all-cause mortality (HR, 3.07; 95% CI 1.32 to 7.15) and was associated with high rates of exacerbations (ie, ≥3 exacerbations per year of follow-up) (HR, 2.40; 95% CI 1.20 to 4.79). Patients with P. aeruginosa also had worse scores on the Hospital Anxiety and Depression Scale (anxiety, p=0.005; depression, p<0.001), the Leicester Cough Questionnaire (p=0.033) and the modified Medical Research Council scale (p=0.001) compared with those without P. aeruginosa . Isolation of P. aeruginosa in patients with bronchiectasis is a significant prognostic indicator and should be a major factor in the clinical management of the disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Expert patient self-management program versus usual care in bronchiectasis: a randomized controlled trial.

PubMed

Lavery, Katherine A; O'Neill, Brenda; Parker, Michael; Elborn, J Stuart; Bradley, Judy M

2011-08-01

To investigate the efficacy of a disease-specific Expert Patient Programme (EPP) compared with usual care in patients with bronchiectasis. Proof-of-concept randomized controlled trial. Regional respiratory center. Adult patients (N=64; age, >18y) with a primary diagnosis of bronchiectasis based on a respiratory physician's assessment including a computed tomographic scan. Patients were randomly assigned to an intervention (usual care plus EPP; n=32) or control group (usual care only; n=32). The primary outcome measure was the Chronic Disease Self-efficacy Scale (CDSS). Other outcome measures included the Revised Illness Perception Questionnaire (IPQ-R), the St Georges Respiratory Questionnaire, and standard EPP questionnaires. Data were collected at baseline, postintervention, and 3 and 6 months postintervention. This disease-specific EPP for patients with bronchiectasis significantly improved self-efficacy in 6 of 10 subscales (CDSS subscales: exercise regularly [P=.02]; get information about disease [P=.03]; obtain help from community, family, and friends [P=.06]; communicate with physician [P=.85]; manage disease in general [P=.05]; do chores [P=.04]; social/recreational activities [P=.03]; manage symptoms [P<.01]; manage shortness of breath [P=.08]; control/manage depression [P=.01]) compared with usual care. There was no improvement on IPQ-R score. Patients who received the intervention reported more symptoms and decreased quality of life between 3 and 6 months postintervention and an increase in some components of self reported health care use. Patients receiving the disease-specific EPP indicated they were satisfied with the intervention and learned new self-management techniques. There were no significant differences in lung function over time. This original study indicates that a disease-specific EPP results in short-term improvements in self-efficacy. Based on these positive preliminary findings, a larger adequately powered study is justified to investigate the efficacy of a disease-specific EPP in patients with bronchiectasis. Copyright © 2011 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Bronchiectasis Rheumatoid Overlap Syndrome Is an Independent Risk Factor for Mortality in Patients With Bronchiectasis: A Multicenter Cohort Study.

PubMed

De Soyza, Anthony; McDonnell, Melissa J; Goeminne, Pieter C; Aliberti, Stefano; Lonni, Sara; Davison, John; Dupont, Lieven J; Fardon, Thomas C; Rutherford, Robert M; Hill, Adam T; Chalmers, James D

2017-06-01

This study assessed if bronchiectasis (BR) and rheumatoid arthritis (RA), when manifesting as an overlap syndrome (BROS), were associated with worse outcomes than other BR etiologies applying the Bronchiectasis Severity Index (BSI). Data were collected from the BSI databases of 1,716 adult patients with BR across six centers: Edinburgh, United Kingdom (608 patients); Dundee, United Kingdom (n = 286); Leuven, Belgium (n = 253); Monza, Italy (n = 201); Galway, Ireland (n = 242); and Newcastle, United Kingdom (n = 126). Patients were categorized as having BROS (those with RA and BR without interstitial lung disease), idiopathic BR, bronchiectasis-COPD overlap syndrome (BCOS), and "other" BR etiologies. Mortality rates, hospitalization, and exacerbation frequency were recorded. A total of 147 patients with BROS (8.5% of the cohort) were identified. There was a statistically significant relationship between BROS and mortality, although this relationship was not associated with higher rates of BR exacerbations or BR-related hospitalizations. The mortality rate over a mean of 48 months was 9.3% for idiopathic BR, 8.6% in patients with other causes of BR, 18% for RA, and 28.5% for BCOS. Mortality was statistically higher in patients with BROS and BCOS compared with those with all other etiologies. The BSI scores were statistically but not clinically significantly higher in those with BROS compared with those with idiopathic BR (BSI mean, 7.7 vs 7.1, respectively; P < .05). Patients with BCOS had significantly higher BSI scores (mean, 10.4), Pseudomonas aeruginosa colonization rates (24%), and previous hospitalization rates (58%). Both the BROS and BCOS groups have an excess of mortality. The mechanisms for this finding may be complex, but these data emphasize that these subgroups require additional study to understand this excess mortality. Copyright © 2017 American College of Chest Physicians. All rights reserved.

CFTR gene variant IVS8-5T in disseminated bronchiectasis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Pignatti, P.F.; Bombieri, C.; Benetazzo, M.

1996-04-01

Obstructive pulmonary disease includes asthma, chronic obstructive pulmonary disease (COPD; i.e., pulmonary emphysema and chronic bronchitis), bronchiectasis, and cystic fibrosis (CF). It represents a leading cause of death in developed countries. Both familial clustering of non-CF obstructive pulmonary disease and familial aggregation of impaired lung function have been described. This suggests that genetic factors contribute to non-CF obstructive pulmonary disease, even if it is difficult to determine the relative contribution of environmental factors. 11 refs., 1 tab.

Culture and PCR Detection of Haemophilus influenzae and Haemophilus haemolyticus in Australian Indigenous Children with Bronchiectasis

PubMed Central

Binks, M. J.; Grimwood, K.; Chang, A. B.; Leach, A. J.; Smith-Vaughan, H.

2012-01-01

A PCR for protein D (hpd#3) was used to differentiate nontypeable Haemophilus influenzae (NTHI) from Haemophilus haemolyticus. While 90% of nasopharyngeal specimens and 100% of lower-airway specimens from 84 Indigenous Australian children with bronchiectasis had phenotypic NTHI isolates confirmed as H. influenzae, only 39% of oropharyngeal specimens with phenotypic NTHI had H. influenzae. The nasopharynx is therefore the preferred site for NTHI colonization studies, and NTHI is confirmed as an important lower-airway pathogen. PMID:22553240

[Acquired hypogammaglobulinemia associated with thymoma: Good syndrome].

PubMed

Aouadi, Samira; Ghrairi, Najla; Braham, Emna; Kaabi, Manel; Maâlej, Sonia; Elgharbi, Leila Douik

2017-01-01

Good syndrome (GS) is defined as the association between thymoma and immune deficiency. It is often complicated by broncho-pulmonary bacterial infections and rhinosinusitis. This disease accounts for only 5% of all parathymic syndromes. These recurrent respiratory infections can cause bronchiectasis associated with Good syndrome. We report the case of a 52-year old woman hospitalized for non resolutive infectious pneumonitis. Chest CT scan showed bronchiectasis associated with thymoma confirmed by biopsy. The discovery of hypogammaglobulinemia allowed the diagnosis of Good syndrome.

Effects of exercise on secretion transport, inflammation, and quality of life in patients with noncystic fibrosis bronchiectasis

PubMed Central

dos Santos, Daniele Oliveira; de Souza, Hugo Celso Dutra; Baddini-Martinez, José Antônio; Ramos, Ercy Mara Cipulo; Gastaldi, Ada Clarice

2018-01-01

Abstract Background: Bronchiectasis is characterized by pathological and irreversible bronchial dilatation caused by the inefficient mucus and microorganism clearance and progression of inflammatory processes. The most frequent characteristic is the increase in bronchial mucus production resulting in slower transport and damage to the mucociliary transport. Aims: To evaluate the effects of exercise on mucus transport, inflammation, and resistance of the respiratory and autonomic nervous systems and subsequent effects on quality of life in patients with bronchiectasis who are enrolled in a pulmonary rehabilitation program. Methods: Sixty subjects of both sexes between 18 and 60 years (30 volunteers with clinically stable bronchiectasis and 30 healthy volunteers) will be included. Participants with chronic obstructive pulmonary disease, decompensated cardiovascular or metabolic diseases, neuromuscular and musculoskeletal diseases, and active smokers will be excluded. Volunteers will be randomly allocated to the pulmonary rehabilitation or control groups. The primary outcomes will be nasal transport time as evaluated by nasal saccharin transport time, analysis of nasal lavage, enzyme immunoassay of exhaled expiration, and analysis of the mucus properties. The secondary outcomes will include pulmonary function tests, impulse oscillometry, heart rate variability analysis, and quality of life questionnaires. Discussion: In addition to the benefits for patients already described in the literature, the additional benefit of mucus removal may contribute to optimizing treatments and better control of the disease. Conclusion: This protocol could provide new information about the unclear mechanisms regarding exercise to aid in the removal of secretions. PMID:29443739

A pilot study of pulmonary rehabilitation and chest physiotherapy versus chest physiotherapy alone in bronchiectasis.

PubMed

Mandal, P; Sidhu, M K; Kope, L; Pollock, W; Stevenson, L M; Pentland, J L; Turnbull, K; Mac Quarrie, S; Hill, A T

2012-12-01

The aim of our study was to assess the efficacy of pulmonary rehabilitation in addition to regular chest physiotherapy in non cystic fibrosis bronchiectasis. Thirty patients with clinically significant bronchiectasis and limited exercise tolerance were randomized into either the control group receiving chest physiotherapy (8 weeks) or into the intervention group, receiving pulmonary rehabilitation in addition to chest physiotherapy (8 weeks). Both groups were encouraged to maintain their exercise program and or chest physiotherapy, following completion of the study. End of training (8 weeks) No improvement in control group. In the intervention group, incremental shuttle walk test (ISWT) improved by 56.7 m (p = 0.03), endurance walk test (EWT) by 193.3 m (p = 0.01), Leicester Cough Questionnaire (LCQ) improved by 2.6 units (p < 0.001) and St. George's Respiratory Questionnaire (SGRQ) by 8 units (p < 0.001). At 20 weeks (12 weeks post end of training) No improvement in control group. In the intervention group, ISWT improved by 80 m (p = 0.04) and EWT by 247.5 m (p = 0.003). LCQ improved by 4.4 units (p < 0.001) and SGRQ by 4 units (p < 0.001). Pulmonary rehabilitation in addition to regular chest physiotherapy, improves exercise tolerance and health related quality of life in non cystic fibrosis bronchiectasis and the benefit was sustained at 12 weeks post end of pulmonary rehabilitation. Clinical trials regn no. NCT00868075. Copyright © 2012 Elsevier Ltd. All rights reserved.

Efficacy of azithromycin in the treatment of bronchiectasis.

PubMed

Lourdesamy Anthony, Albert I; Muthukumaru, Umadevi

2014-11-01

We evaluated the efficacy of a 12-week oral treatment with azithromycin in adult patients with bronchiectasis. The objectives were to demonstrate that this treatment reduces sputum volume, improves quality of life and to assess the lengths of effects after cessation of therapy. Seventy-eight patients with bronchiectasis confirmed by high-resolution computed tomography were included in this study. Subjects received oral azithromycin or placebo in a randomized manner for 12 weeks followed by placebo for another 12 weeks. Sputum volume, St George's Respiratory Questionnaire (SGRQ) score and spirometry were recorded at baseline, 12 weeks and 24 weeks, respectively. End-point measurements were compared from baseline to the end of each study phase. Sixty-eight subjects were included in the analysis. Mean 24-h sputum volume significantly decreased (P < 0.01) during the active treatment phase and remained low during the control phase (P < 0.01). The mean SGRQ total score with azithromycin decreased (i.e. improved health status) from baseline by more than the 4 points at the end of 12 and 24 weeks. Lung functions remained stable during oral azithromycin therapy and the subsequent control phase. Twelve weeks administration of azithromycin in bronchiectasis produces significant reductions in mean sputum volume, health status and stabilization of lung function values. Sputum volume reduction and the improvement of quality of life were sustained for 12 weeks after cessation of azithromycin. (Clinicaltrials.gov number NCT02107274). © 2014 Asian Pacific Society of Respirology.

Changes in B cell immunophenotype in common variable immunodeficiency: cause or effect – is bronchiectasis indicative of undiagnosed immunodeficiency?

PubMed Central

Bright, P; Grigoriadou, S; Kamperidis, P; Buckland, M; Hickey, A; Longhurst, H J

2013-01-01

Common variable immunodeficiency (CVID) is the most common severe primary immunodeficiency, but the pathology of this condition is poorly understood. CVID involves a defect in the production of immunoglobulin from B cells, with a subsequent predisposition to infections. Approximately 10–20% of cases are inherited, but even in families with a genetic defect the penetrance is far from complete. A classification system for CVID has been suggested (EUROclass) based on B cell immunophenotyping, but it has not been shown that altered B cell immunophenotype is not a consequence of the complications and treatment of CVID. This study compares the EUROclass B cell immunophenotype of CVID patients (n = 30) with suitable disease controls with bronchiectasis (n = 11), granulomatous disease (Crohn's disease) (n = 9) and neurological patients on immunoglobulin treatment (n = 6). The results of this study correlate with previous literature, that alterations in B cell immunophenotype are associated strongly with CVID. Interestingly, three of the 11 bronchiectasis patients without known immunodeficiency had an altered B cell immunophenotype, suggesting the possibility of undiagnosed immunodeficiency, or that bronchiectasis may cause a secondary alteration in B cell immunophenotype. This study showed a significant difference in B cell immunophenotype between CVID patients compared to disease control groups of granulomatous disease and immunoglobulin treatment. This suggests that granulomatous disease (in Crohn's disease) and immunoglobulin treatment (for chronic neurological conditions) are not causal of an altered B cell immunophenotype in these control populations. PMID:23286946

Lung transplantation for non-cystic fibrosis bronchiectasis: analysis of a 13-year experience.

PubMed

Beirne, Paul Adrian; Banner, Nicholas R; Khaghani, Asghar; Hodson, Margaret E; Yacoub, Magdi H

2005-10-01

Lung transplantation is a well-established treatment for end-stage cystic fibrosis, and there are considerable data on medium- and long-term results. However, less information exists about transplantation for non-cystic fibrosis bronchiectasis. Between December 1988 and June 2001, 22 patients (12 men, 10 women) underwent transplantation for bronchiectasis not due to cystic fibrosis. Procedures were bilateral sequential single-lung transplants (BSSLTX) in 4 patients, en bloc double lung transplants (DLTX) in 5, heart-lung transplants (HLTX) in 6, and single-lung transplants (SLTX) in 7. Lifelong outpatient follow-up was continued at a minimum of every 6 months. One-year Kaplan-Meier survival for all patients was 68% (95% confidence interval [CI], 54%-91%), and 5-year survival was 62% (95% CI, 41-83%). One-year survival after SLTX was 57% (95% CI, 20%-94%) vs 73% (95% CI, 51-96%) for those receiving 2 lungs. At 6 months, mean forced expiratory volume in 1 second was 73% predicted (range, 58%-97%), and mean forced vital capacity was 68% predicted (range, 53%-94%) after receiving 2 lungs (n = 10); in the SLTX group at 6 months, mean forced expiratory volume in 1 second was 50% predicted (range, 34%-61%), and mean forced vital capacity was 53% predicted (range 46-63%) (n = 4). Survival and lung function after transplantation for non-cystic fibrosis bronchiectasis was similar to that after transplantation for cystic fibrosis. A good outcome is possible after single lung transplantation in selected patients.

Prevalence of Osteopenia and Osteoporosis in Patients with Noncystic Fibrosis Bronchiectasis.

PubMed

Diehl, Nathan; Johnson, Margaret M

2016-12-01

The objective of our study was to define the prevalence of osteoporosis and osteopenia in patients with noncystic fibrosis bronchiectasis (NCFB). We conducted a retrospective chart review of all patients with physician-diagnosed NCFB evaluated at Mayo Clinic Florida between January 1, 2011 and June 3, 2013. A total of 113 patients with physician-diagnosed NCFB and confirmatory findings on computed tomography scan were identified. The cohort was overwhelmingly women (90%) with a mean age of 72 ± 10.6 and a body mass index of 24.8 ± 6.8. The medical history indicated that 30% (34) had osteoporosis, 39% (44) had osteopenia, and 9% (10) had normal bone density. In 25 (22%) of the subjects, bone density was unknown or undocumented. Most were never smokers (55.7%) or past smokers (41.6%) and airflow obstruction was present in 58% of the 84 subjects who had undergone pulmonary function tests. In total, 57 patients (50.44%) and 45 patients (39.82%) had been prescribed proton pump inhibitors and inhaled corticosteroids, respectively. Bone mineral density testing was performed during the study period in 70 (62%) of the subjects. Decreased bone density consistent with osteoporosis was present in 19 (27%); 41 (59%) had osteopenia, and bone density was normal in 10 (14%) subjects. Diminished bone density was present in 82.8% (24/29) of patients younger than age 70, with 27.6% (8/29) having osteoporosis. There was a greater incidence of diminished bone density in those with reduced body mass index (100% vs 82%), but this difference did not reach statistical significance ( P = 0.10). Forty-seven and 32% of patients with diminished bone density were using proton pump inhibitor therapy and inhaled corticosteroids, respectively. This study suggested that diminished bone density is common in patients with bronchiectasis, with >85% of this cohort having osteoporosis or osteopenia confirmed by bone density testing. Although the prevalence of both bronchiectasis and diminished bone density increases with advancing age and female sex, these data suggest a greater prevalence than expected based on demographic risks. Medications that may predispose individuals to diminished bone density are not uncommonly prescribed in patients with bronchiectasis. Provider awareness of the substantial prevalence of diminished bone density in patients with bronchiectasis may improve patient care by prompting appropriate screening for and treatment of osteoporosis and osteopenia. In light of these observations, judicious use of medications that may predispose to diminished bone density is warranted.

Long-term azithromycin for Indigenous children with non-cystic-fibrosis bronchiectasis or chronic suppurative lung disease (Bronchiectasis Intervention Study): a multicentre, double-blind, randomised controlled trial.

PubMed

Valery, Patricia C; Morris, Peter S; Byrnes, Catherine A; Grimwood, Keith; Torzillo, Paul J; Bauert, Paul A; Masters, I Brent; Diaz, Abbey; McCallum, Gabrielle B; Mobberley, Charmaine; Tjhung, Irene; Hare, Kim M; Ware, Robert S; Chang, Anne B

2013-10-01

Indigenous children in high-income countries have a heavy burden of bronchiectasis unrelated to cystic fibrosis. We aimed to establish whether long-term azithromycin reduced pulmonary exacerbations in Indigenous children with non-cystic-fibrosis bronchiectasis or chronic suppurative lung disease. Between Nov 12, 2008, and Dec 23, 2010, we enrolled Indigenous Australian, Maori, and Pacific Island children aged 1-8 years with either bronchiectasis or chronic suppurative lung disease into a multicentre, double-blind, randomised, parallel-group, placebo-controlled trial. Eligible children had had at least one pulmonary exacerbation in the previous 12 months. Children were randomised (1:1 ratio, by computer-generated sequence with permuted block design, stratified by study site and exacerbation frequency [1-2 vs ≥3 episodes in the preceding 12 months]) to receive either azithromycin (30 mg/kg) or placebo once a week for up to 24 months. Allocation concealment was achieved by double-sealed, opaque envelopes; participants, caregivers, and study personnel were masked to assignment until after data analysis. The primary outcome was exacerbation (respiratory episodes treated with antibiotics) rate. Analysis of the primary endpoint was by intention to treat. At enrolment and at their final clinic visits, children had deep nasal swabs collected, which we analysed for antibiotic-resistant bacteria. This study is registered with the Australian New Zealand Clinical Trials Registry; ACTRN12610000383066. 45 children were assigned to azithromycin and 44 to placebo. The study was stopped early for feasibility reasons on Dec 31, 2011, thus children received the intervention for 12-24 months. The mean treatment duration was 20·7 months (SD 5·7), with a total of 902 child-months in the azithromycin group and 875 child-months in the placebo group. Compared with the placebo group, children receiving azithromycin had significantly lower exacerbation rates (incidence rate ratio 0·50; 95% CI 0·35-0·71; p<0·0001). However, children in the azithromycin group developed significantly higher carriage of azithromycin-resistant bacteria (19 of 41, 46%) than those receiving placebo (four of 37, 11%; p=0·002). The most common adverse events were non-pulmonary infections (71 of 112 events in the azithromycin group vs 132 of 209 events in the placebo group) and bronchiectasis-related events (episodes or investigations; 22 of 112 events in the azithromycin group vs 48 of 209 events in the placebo group); however, study drugs were well tolerated with no serious adverse events being attributed to the intervention. Once-weekly azithromycin for up to 24 months decreased pulmonary exacerbations in Indigenous children with non-cystic-fibrosis bronchiectasis or chronic suppurative lung disease. However, this strategy was also accompanied by increased carriage of azithromycin-resistant bacteria, the clinical consequences of which are uncertain, and will need careful monitoring and further study. National Health and Medical Research Council (Australia) and Health Research Council (New Zealand). Copyright © 2013 Elsevier Ltd. All rights reserved.

Validation of a Quality of Life Questionnaire for Bronchiectasis: psychometric analyses of the Spanish QOL-B-V3.0.

PubMed

Olveira, Casilda; Olveira, Gabriel; Espildora, Francisco; Giron, Rosa-Maria; Muñoz, Gerard; Quittner, Alexandra L; Martinez-Garcia, Miguel-Angel

2014-05-01

Bronchiectasis is a chronic disease, leading to worsening of health-related quality of life. This study evaluated the psychometric properties of a new patient-reported outcome for non-cystic fibrosis bronchiectasis, the Quality of Life Questionnaire Bronchiectasis, translated into Spanish (QOL-B-Sp-V3.0). This prospective study recruited clinically stable patients with non-cystic fibrosis bronchiectasis at 4 Spanish centers. Health status was assessed with multiple indicators (dyspnea, exacerbations, bronchorrhea, etc.), microbiological, radiological, spirometric, and anthropometric parameters plus St-George Respiratory Questionnaire (SGRQ). Psychometric analyses included internal consistency, test-retest reliability, convergent validity, predictive validity, and responsivity to change. The 207 stable patients (mean age 57.2 years) had a Bhalla score of 11.53 ± 7.39 and FEV1% of 68.3 ± 22.2 %. One hundred and sixty-one stable patients repeated the test 2 weeks later, and 80 patients who had an exacerbation within 6 months of the assessment also repeated it. Internal consistency was high across all scales (Cronbach's alpha >0.70). Thirty-six of 37 items correlated more strongly with their assigned scale than a competing scale. Test-retest coefficients were strong (intraclass correlations r = 0.68-0.88). All scales, except Treatment Burden, discriminated significantly between patients with mild, moderate, and severe disease according to FEV1% and other respiratory parameters. Strong convergence was found between the QOL-B-Sp-V3.0 and SGRQ. Significant correlations were found between QOL-B-Sp-V3.0 and various clinical, spirometric, radiological, and anthropometric variables. Significant differences were found on all QOL-B-Sp-V3.0 scales, except emotional functioning, between the baseline responses and onset of an exacerbation; robust sensitivity to change was observed on the Respiratory Symptoms scale. The QOL-B-Sp-V3.0 questionnaire demonstrated strong reliability and validity. Scores were reproducible after 2 weeks, and it discriminated between patients who varied in severity and was responsive to changes related to exacerbation.

Applying the Transtheoretical Model to Physical Activity Behavior in Individuals With Non-Cystic Fibrosis Bronchiectasis.

PubMed

Wilson, Jason J; Kirk, Alison; Hayes, Kate; Bradbury, Ian; McDonough, Suzanne; Tully, Mark A; O'Neill, Brenda; Bradley, Judy M

2016-01-01

The transtheoretical model has been successful in promoting health behavior change in general and clinical populations. However, there is little knowledge about the application of the transtheoretical model to explain physical activity behavior in individuals with non-cystic fibrosis bronchiectasis. The aim was to examine patterns of (1) physical activity and (2) mediators of behavior change (self-efficacy, decisional balance, and processes of change) across stages of change in individuals with non-cystic fibrosis bronchiectasis. Fifty-five subjects with non-cystic fibrosis bronchiectasis (mean age ± SD = 63 ± 10 y) had physical activity assessed over 7 d using an accelerometer. Each component of the transtheoretical model was assessed using validated questionnaires. Subjects were divided into groups depending on stage of change: Group 1 (pre-contemplation and contemplation; n = 10), Group 2 (preparation; n = 20), and Group 3 (action and maintenance; n = 25). Statistical analyses included one-way analysis of variance and Tukey-Kramer post hoc tests. Physical activity variables were significantly (P < .05) higher in Group 3 (action and maintenance) compared with Group 2 (preparation) and Group 1 (pre-contemplation and contemplation). For self-efficacy, there were no significant differences between groups for mean scores (P = .14). Decisional balance cons (barriers to being physically active) were significantly lower in Group 3 versus Group 2 (P = .032). For processes of change, substituting alternatives (substituting inactive options for active options) was significantly higher in Group 3 versus Group 1 (P = .01), and enlisting social support (seeking out social support to increase and maintain physical activity) was significantly lower in Group 3 versus Group 2 (P = .038). The pattern of physical activity across stages of change is consistent with the theoretical predictions of the transtheoretical model. Constructs of the transtheoretical model that appear to be important at different stages of change include decisional balance cons, substituting alternatives, and enlisting social support. This study provides support to explore transtheoretical model-based physical activity interventions in individuals with non-cystic fibrosis bronchiectasis. (ClinicalTrials.gov registration NCT01569009.). Copyright © 2016 by Daedalus Enterprises.

A Comprehensive Analysis of the Impact of Pseudomonas aeruginosa Colonization on Prognosis in Adult Bronchiectasis.

PubMed

Finch, Simon; McDonnell, Melissa J; Abo-Leyah, Hani; Aliberti, Stefano; Chalmers, James D

2015-11-01

Eradication and suppression of Pseudomonas aeruginosa is a key priority in national guidelines for bronchiectasis and is a major focus of drug development and clinical trials. An accurate estimation of the clinical impact of P. aeruginosa in bronchiectasis is therefore essential. Data derived from 21 observational cohort studies comparing patients with P. aeruginosa colonization with those without it were pooled by random effects meta-analysis. Data were collected for key longitudinal clinical outcomes of mortality, hospital admissions, exacerbations, and lung function decline, along with cross-sectional outcomes such as quality of life. In the aggregate, the included studies comprised 3,683 patients. P. aeruginosa was associated with a highly significant and consistent increase in all markers of disease severity, including mortality (odds ratio [OR], 2.95; 95% confidence interval [CI], 1.98-4.40; P < 0.0001), hospital admissions (OR, 6.57; 95% CI, 3.19-13.51; P < 0.0001), and exacerbations (mean difference, 0.97/yr; 95% CI, 0.64-1.30; P < 0.0001). The patients with P. aeruginosa also had worse quality of life on the basis of their St. George's Respiratory Questionnaire results (mean difference, 18.2 points; 95% CI, 14.7-21.8; P < 0.0001). Large differences in lung function and radiological severity were also observed. The definitions of colonization were inconsistent among the studies, but the findings were robust regardless of the definition used. P. aeruginosa is associated with an approximately threefold increased risk of death and an increase in hospital admissions and exacerbations in adult bronchiectasis.

The effects of pulmonary rehabilitation in patients with non-cystic fibrosis bronchiectasis: protocol for a randomised controlled trial.

PubMed

Lee, Annemarie L; Cecins, Nola; Hill, Catherine J; Holland, Anne E; Rautela, Linda; Stirling, Robert G; Thompson, Phillip J; McDonald, Christine F; Jenkins, Sue

2010-02-02

Non-cystic fibrosis bronchiectasis is characterised by sputum production, exercise limitation and recurrent infections. Although pulmonary rehabilitation is advocated for this patient group, its effects are unclear. The aims of this study are to determine the short and long term effects of pulmonary rehabilitation on exercise capacity, cough, quality of life and the incidence of acute pulmonary exacerbations. This randomised controlled trial aims to recruit 64 patients with bronchiectasis from three tertiary institutions. Participants will be randomly allocated to the intervention group (supervised, twice weekly exercise training with regular review of airway clearance therapy) or a control group (twice weekly telephone support). Measurements will be taken at baseline, immediately following the intervention and at six and 12 months following the intervention period by a blinded assessor. Exercise capacity will be measured using the incremental shuttle walk test and the six-minute walk test. Quality of life and health status will be measured using the Chronic Respiratory Questionnaire, Leicester Cough Questionnaire, Assessment of Quality of Life Questionnaire and the Hospital Anxiety and Depression Scale. The rate of hospitalisation will be captured as well as the incidence of acute pulmonary exacerbations using a daily symptom diary. Results from this study will help to determine the efficacy of supervised twice-weekly pulmonary rehabilitation upon exercise capacity and quality of life in patients with bronchiectasis and will contribute to clinical practice guidelines for physiotherapists in the management of this population. This study protocol is registered with ClinicalTrials.gov (NCT00885521).

The burden of disease in pediatric non-cystic fibrosis bronchiectasis.

PubMed

Kapur, Nitin; Masters, I Brent; Newcombe, Peter; Chang, Anne B

2012-04-01

The burden of disease in children with non-cystic fibrosis (non-CF) bronchiectasis is unknown. Our study aimed to identify the determinants of quality of life (QOL) and parental mental health in this group of patients and their parents and to evaluate the effect of exacerbations on these parameters. Parents of 69 children (median age 7 years) with non-CF bronchiectasis prospectively completed two questionnaires (parent-proxy cough-specific quality of life [PC-QOL] and the Depression, Anxiety, and Stress Scale [DASS]) at stable and exacerbation states. Data on clinical, investigational, and lung function parameters were also collected. During the stable state, the median interquartile range (IQR) PC-QOL score was 6.5 (5.3-6.9) and the DASS 21-item questionnaire score was 6 (0-20). Being of a young age correlated with a worse QOL (r(5) = 0.242, P = .04) but radiologic extent, lung function, underlying cause, environmental tobacco smoke exposure, and chronic upper-airway disease did not influence these scores. Exacerbations caused significant worsening in the PC-QOL scores (median [IQR], 4.6 [3.8-5.4]; P = .001) and DASS scores (median [IQR], 22 [9-42]; P < .001; 38% with elevated anxiety, 54% with abnormal depression/stress scores during exacerbation). The presence of viral infection, hypoxia, and hospitalization did not influence the exacerbation PC-QOL and DASS scores. There is a significant burden of disease, especially during exacerbation, on parents of children with bronchiectasis. Prevention, early detection, and appropriate treatment of exacerbations are likely to reduce psychologic morbidity in this group.

Thoracoscopic pneumonectomy for severe bronchiectasis in a 9-year-old female.

PubMed

Anselmo, Dean M; Perez, Iris A; Shaul, Donald B

2008-10-01

Thoracoscopic total pneumonectomy has not been previously described in the pediatric surgical literature. In this paper, we describe a case of pneumonectomy performed through a minimally invasive approach in a 9-year-old female with Down's syndrome and gastroesophageal reflux disease. The patient suffered from multiple recurrent aspiration pneumonias, which progressed to bronchiectasis of the entire left lung. As a result, the patient was hypoxemic and required continuous supplemental oxygen. Preoperative perfusion scans showed diminished perfusion of the left lung. Thoracoscopy was performed by using 3-5 mm trocars and one 12-mm trocar. Insufflation pressure was maintained at 5 mm Hg. Dissection was performed at the hilum by using hook electrocautery and the LigaSure device (ValleyLab, Boulder, CO). The pulmonary artery, veins, and left mainstem bronchus were sequentially divided by using a 35-mm ENDO GIA vascular stapler (Ethicon Endo-Surgery, Cincinnati, OH). There were no intraoperative complications. Eight months following surgery, her health is improved and she no longer requires supplemental oxygen. Thoracoscopic pneumonectomy is a safe, technically feasible approach for severe bronchiectasis in children.

Oral versus inhaled antibiotics for bronchiectasis.

PubMed

Spencer, Sally; Felix, Lambert M; Milan, Stephen J; Normansell, Rebecca; Goeminne, Pieter C; Chalmers, James D; Donovan, Tim

2018-03-27

Bronchiectasis is a chronic inflammatory disease characterised by a recurrent cycle of respiratory bacterial infections associated with cough, sputum production and impaired quality of life. Antibiotics are the main therapeutic option for managing bronchiectasis exacerbations. Evidence suggests that inhaled antibiotics may be associated with more effective eradication of infective organisms and a lower risk of developing antibiotic resistance when compared with orally administered antibiotics. However, it is currently unclear whether antibiotics are more effective when administered orally or by inhalation. To determine the comparative efficacy and safety of oral versus inhaled antibiotics in the treatment of adults and children with bronchiectasis. We identified studies through searches of the Cochrane Airways Group's Specialised Register (CAGR), which is maintained by the Information Specialist for the group. The Register contains trial reports identified through systematic searches of bibliographic databases including the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, CINAHL, AMED, and PsycINFO, and handsearching of respiratory journals and meeting abstracts. We also searched ClinicalTrials.gov and the WHO trials portal. We searched all databases in March 2018 and imposed no restrictions on language of publication. We planned to include studies which compared oral antibiotics with inhaled antibiotics. We would have considered short-term use (less than four weeks) for treating acute exacerbations separately from longer-term use as a prophylactic (4 weeks or more). We would have considered both intraclass and interclass comparisons. We planned to exclude studies if the participants received continuous or high-dose antibiotics immediately before the start of the trial, or if they have received a diagnosis of cystic fibrosis (CF), sarcoidosis, active allergic bronchopulmonary aspergillosis or active non-tuberculous Mycobacterial infection. Two review authors independently applied study inclusion criteria to the searches and we planned for two authors to independently extract data, assess risk of bias and assess overall quality of the evidence using GRADE criteria. We also planned to obtain missing data from the authors where possible and to report results with 95% confidence intervals (CIs). We identified 313 unique records through database searches and a further 21 records from trial registers. We excluded 307 on the basis of title and abstract alone and a further 27 after examining full-text reports. No studies were identified for inclusion in the review. There is currently no evidence indicating whether orally administered antibiotics are more beneficial compared to inhaled antibiotics. The recent ERS bronchiectasis guidelines provide a practical approach to the use of long-term antibiotics. New research is needed comparing inhaled versus oral antibiotic therapies for bronchiectasis patients with a history of frequent exacerbations, to establish which approach is the most effective in terms of exacerbation prevention, quality of life, treatment burden, and antibiotic resistance.

Nurse-led versus doctor-led care for bronchiectasis.

PubMed

Lawton, Kathryn; Royals, Karen; Carson-Chahhoud, Kristin V; Campbell, Fiona; Smith, Brian J

2018-06-20

Specialist nursing roles to manage stable disease populations are being used to meet the needs of both patients and health services. With increasing cost pressures on health departments, alternative models such as nurse-led care are gaining momentum as a substitute for traditional doctor-led care. This review evaluates the safety, effectiveness, and health outcomes of nurses practising in autonomous roles while using advanced practice skills, within the context of bronchiectasis management in subacute, ambulatory, and/or community care. To compare the effectiveness of nurse-led care versus doctor-led care in the management of stable bronchiectasis. We searched the Cochrane Airways Group Specialised Register and bibliographies of selected papers in addition to grey literature such as electronic clinical trials registries. Searches were current as of March 2018. Randomised controlled trials were eligible for inclusion in the review. Two reviewers extracted and entered data from included studies. Primary outcomes were numbers of exacerbations requiring treatment with antibiotics, hospital admissions, and emergency department attendances. We included one United Kingdom (UK) study in the review. In this randomised controlled trial, a total of 80 participants, with a mean age of 58 years, were treated for 12 months by a specialist nurse or doctor, then were crossed over to the other clinician for the next 12 months. Two participants died during the study period. Six participants failed to cross over to nurse-led care because of unstable bronchiectasis. Overall, the level of study completion was high.Data show no difference in the numbers of exacerbations requiring treatment with antibiotics (rate ratio 1.09, 95% confidence interval (CI) 0.91 to 1.30, 80 participants, moderate-certainty evidence). Investigators reported more hospital admissions in the nurse-led care group (rate ratio 1.52, 95% CI 1.04 to 2.23, 80 participants, moderate-certainty evidence) and did not report emergency department attendance.For secondary outcomes, participants in the nurse-led care group used more healthcare resources during the first year of the trial. Increased admissions and greater use of resources made treatment costs for nurse-led groups' higher. Total costs for both years of the study were £8,464 and £5,228 for nurse-led care compared with doctor-led care. However, by the second year, treatment costs were almost equitable between the two groups, which may reflect the nurses' learning of how to better treat people with bronchiectasis. No statistically significant changes were observed in quality of life, exercise capacity, mortality, or lung function. Wide confidence intervals led to uncertainty regarding these results. Adverse events were not an outcome for this review. This update of the review shows that only one trial met review criteria. Review authors were unable to demonstrate effectiveness of nurse-led care compared with doctor-led care on the basis of findings of a single study. The included study reported no significant differences, but limited evidence means that differences in clinical outcomes between nurse-led care and usual care within the setting of a specialist clinic remain unclear. Further research is required to determine whether nurse-led care is cost-effective, if guidelines and protocols for bronchiectasis management are followed does this increases costs and how effective nurse-led management of bronchiectasis is in other clinical settings such as inpatient and outreach.

Chronic atelectasis of the left lower lobe: a clinicopathological condition equivalent to middle lobe syndrome

PubMed Central

Hamad, Abdel-Mohsen; Elmistekawy, Elsayed; Elatafy, Elatafy

2012-01-01

OBJECTIVES Middle lobe syndrome is a well-known clinical condition. In this retrospective study, we report our experience with a similar clinicopathological condition affecting the left lower lobe. METHODS The data of 17 patients with atelectasis or bronchiectasis of the left lower lobe who underwent lobectomy during the period from January 2000 to December 2011 were reviewed. Demographic, clinical, radiological and surgical data were collected. RESULTS Seventeen patients were included in this study, only one adult male patient of 52 years and 16 children. The paediatric patients were 10 boys and 6 girls, their age ranged from 2 to 11 years, mean 6.19 ± 2.6 years. Most patients presented with recurrent respiratory infection 15/17 (88.2%). The lag time before referral to surgery ranged from 3 to 48 months, mean 17.59 ± 13.1 months. Radiological signs of bronchiectasis were found in 11 (64.7%) patients. Bronchoscopy showed patent lower lobe bronchus in all patients. The criteria for lobectomy were evidence of bronchiectasis [11 (64.71%) patients], persistent atelectasis of the lobe after bronchoscopy and intensive medical therapy for a maximum of 2 months [6 (35.29%) patients]. Histopathological examination showed bronchiectasis in 11 (64.71%) patients, fibrosing pneumonitis in 4 (23.53%) patients and peribronchial inflammation in 2 (11.76%) patients. Most patients were doing well 1 year after surgery. CONCLUSIONS Chronic atelectasis of the left lower lobe is a clinicopathological condition equivalent to middle lobe syndrome. Impaired collateral ventilation together with airway plugging with secretion is an accepted explanation. Surgical resection is indicated for bronchiectatic lobe or failure of 2-month intensive medical therapy to resolve lobar atelectasis. PMID:22761114

Chronic atelectasis of the left lower lobe: a clinicopathological condition equivalent to middle lobe syndrome.

PubMed

Hamad, Abdel-Mohsen; Elmistekawy, Elsayed; Elatafy, Elatafy

2012-10-01

Middle lobe syndrome is a well-known clinical condition. In this retrospective study, we report our experience with a similar clinicopathological condition affecting the left lower lobe. The data of 17 patients with atelectasis or bronchiectasis of the left lower lobe who underwent lobectomy during the period from January 2000 to December 2011 were reviewed. Demographic, clinical, radiological and surgical data were collected. Seventeen patients were included in this study, only one adult male patient of 52 years and 16 children. The paediatric patients were 10 boys and 6 girls, their age ranged from 2 to 11 years, mean 6.19 ± 2.6 years. Most patients presented with recurrent respiratory infection 15/17 (88.2%). The lag time before referral to surgery ranged from 3 to 48 months, mean 17.59 ± 13.1 months. Radiological signs of bronchiectasis were found in 11 (64.7%) patients. Bronchoscopy showed patent lower lobe bronchus in all patients. The criteria for lobectomy were evidence of bronchiectasis [11 (64.71%) patients], persistent atelectasis of the lobe after bronchoscopy and intensive medical therapy for a maximum of 2 months [6 (35.29%) patients]. Histopathological examination showed bronchiectasis in 11 (64.71%) patients, fibrosing pneumonitis in 4 (23.53%) patients and peribronchial inflammation in 2 (11.76%) patients. Most patients were doing well 1 year after surgery. Chronic atelectasis of the left lower lobe is a clinicopathological condition equivalent to middle lobe syndrome. Impaired collateral ventilation together with airway plugging with secretion is an accepted explanation. Surgical resection is indicated for bronchiectatic lobe or failure of 2-month intensive medical therapy to resolve lobar atelectasis.

Lung function, symptoms and inflammation during exacerbations of non-cystic fibrosis bronchiectasis: a prospective observational cohort study.

PubMed

Brill, Simon E; Patel, Anant R C; Singh, Richa; Mackay, Alexander J; Brown, Jeremy S; Hurst, John R

2015-02-07

Exacerbations of non-cystic fibrosis bronchiectasis cause significant morbidity but there are few detailed data on their clinical course and associated physiological changes. The biology of an exacerbation has not been previously described. This was a prospective observational cohort study of 32 outpatients with non-cystic fibrosis bronchiectasis conducted between August 2010 and August 2012. Patients completed a symptom diary card and measured their peak expiratory flow rate (PEFR) daily. Exacerbations were defined as oral antibiotic treatment taken for a worsening of respiratory symptoms. Symptoms and peak flow at exacerbation were analysed, and further measurements including the COPD Assessment Test (CAT) and inflammatory markers were also compared to baseline values. At baseline, health status was significantly related to lung function, prognostic severity and systemic inflammation. 51 exacerbations occurred in 22 patients. Exacerbation symptoms began a median (interquartile range) of 4 (2, 7) days before treatment started and the median exacerbation duration was 16 (10, 29) days. 16% had not recovered by 35 days. At exacerbation, mean PEFR dropped by 10.6% (95% confidence interval 6.9-14.2, p < 0.001) and mean CAT score increased by 6.3 units (3.6-9.1, p = 0.001), median symptom count by 4 (2.25, 6, p < 0.001), and mean CRP by 9.0mg/L (2.3-15.8, p = 0.011). Exacerbations where PEFR fell by ≥10% were longer with more symptoms at onset. Exacerbations of non-CF bronchiectasis are inflammatory events, with worsened symptoms, lung function and health status, and a prolonged recovery period. Symptom diary cards, PEFR and CAT scores are responsive to changes at exacerbation and may be useful tools for their detection and monitoring.

Prevalence and impact of bronchiectasis in alpha1-antitrypsin deficiency.

PubMed

Parr, David G; Guest, Peter G; Reynolds, John H; Dowson, Lee J; Stockley, Robert A

2007-12-15

alpha(1)-Antitrypsin (AAT) deficiency is associated with increased risk of chronic obstructive pulmonary disease (COPD), in particular emphysema, but airway disease is less well described. To assess the prevalence of airways disease in subjects with AAT deficiency and to identify the relationship between radiological airway abnormalities and clinical phenotype. We characterized the computed tomographic phenotype of 74 subjects (PiZ), using visual scoring of airway disease and densitometric assessment of emphysema. Computed tomographic measurements were related to physiology, health status (St. George's Respiratory Questionnaire), and emphysema severity, and the relative impact of airway disease and emphysema severity on health status and airflow obstruction was compared by stepwise regression. Bronchiectatic changes were seen in 70 subjects, and a subgroup with a bronchiectasis-predominant phenotype was identified. Clinically significant bronchiectasis (radiologic bronchiectasis in 4 or more bronchopulmonary segments together with symptoms of regular sputum production) occurred in 20 subjects (27%). AAT-deficient index cases had higher airway disease scores (P < 0.05), more severe emphysema (P < 0.001), and greater impairment of physiology (P < 0.001) and health status (P < 0.05) than nonindex cases. Airway disease scores correlated with health status, and bronchial wall thickening correlated with FEV(1). Regression analysis indicated that emphysema severity had the strongest associations for health status (r = 0.505, P < 0.001) and FEV(1) (r = 0.699, P < 0.001), but the addition of airway disease score improved the regression models (r = 0.596, P = 0.002 and r = 0.783, P < 0.001, respectively). Emphysema is the predominant component of COPD in AAT deficiency, but the prevalence and impact of airway disease are greater than currently recognized. Consequently, future therapeutic strategies in AAT deficiency should also target this component of COPD.

Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis.

PubMed

Kellett, Fiona; Robert, Niven M

2011-12-01

Sputum retention is a distressing feature of non-cystic fibrosis bronchiectasis and has been shown to contribute to the vicious cycle of infection seen in this disease. In a previous study we demonstrated that nebulised 7% hypertonic saline was both safe and effective in this patient population. Patients with a clinical diagnosis of non-cystic fibrosis bronchiectasis, confirmed by HRCT, were entered into a randomised single blind cross-over study to evaluate 0.9% sodium chloride (IS) and 7% hypertonic saline (HS). Following a 4 week run in patients received a random order active HS or IS daily for 3 months. A 4 week wash-out phase was included between phases. We report lung function, quality of life, and health care utilisation responses. 32 patients mean age 56.6 years (SD 14.6), 16 male, were recruited of which 28 were randomised and completed the study. Lung function (%change from baseline) improved in HS vs. IS (FEV(1): 15.1, 1.8 p<0.01; FVC: 11.2, 0.7 p<0.01. SGRQ improved significantly from baseline (HS 6.0, IS 1.2; p<0.05). There were reductions in annualised antibiotic usage (HS 2.4, IS 5.4 courses per patient per year), annualised emergency health care utilisation visits were reduced (HS 2.1, IS 4.9 events per patient per year). There were also improvements in sputum viscosity and ease of expectoration (visual analogue scale). Regular use of 7% hypertonic saline improves lung function, quality of life and health care utilisation in non-cystic fibrosis bronchiectasis patients. Copyright © 2011 Elsevier Ltd. All rights reserved.

Multidimensional severity assessment in bronchiectasis: an analysis of seven European cohorts

PubMed Central

McDonnell, M J; Aliberti, S; Goeminne, P C; Dimakou, K; Zucchetti, S C; Davidson, J; Ward, C; Laffey, J G; Finch, S; Pesci, A; Dupont, L J; Fardon, T C; Skrbic, D; Obradovic, D; Cowman, S; Loebinger, M R; Rutherford, R M; De Soyza, A; Chalmers, J D

2016-01-01

Introduction Bronchiectasis is a multidimensional disease associated with substantial morbidity and mortality. Two disease-specific clinical prediction tools have been developed, the Bronchiectasis Severity Index (BSI) and the FACED score, both of which stratify patients into severity risk categories to predict the probability of mortality. Methods We aimed to compare the predictive utility of BSI and FACED in assessing clinically relevant disease outcomes across seven European cohorts independent of their original validation studies. Results The combined cohorts totalled 1612. Pooled analysis showed that both scores had a good discriminatory predictive value for mortality (pooled area under the curve (AUC) 0.76, 95% CI 0.74 to 0.78 for both scores) with the BSI demonstrating a higher sensitivity (65% vs 28%) but lower specificity (70% vs 93%) compared with the FACED score. Calibration analysis suggested that the BSI performed consistently well across all cohorts, while FACED consistently overestimated mortality in ‘severe’ patients (pooled OR 0.33 (0.23 to 0.48), p<0.0001). The BSI accurately predicted hospitalisations (pooled AUC 0.82, 95% CI 0.78 to 0.84), exacerbations, quality of life (QoL) and respiratory symptoms across all risk categories. FACED had poor discrimination for hospital admissions (pooled AUC 0.65, 95% CI 0.63 to 0.67) with low sensitivity at 16% and did not consistently predict future risk of exacerbations, QoL or respiratory symptoms. No association was observed with FACED and 6 min walk distance (6MWD) or lung function decline. Conclusion The BSI accurately predicts mortality, hospital admissions, exacerbations, QoL, respiratory symptoms, 6MWD and lung function decline in bronchiectasis, providing a clinically relevant evaluation of disease severity. PMID:27516225

Patient participation in ERS guidelines and research projects: the EMBARC experience

PubMed Central

Timothy, Alan; Polverino, Eva; Almagro, Marta; Ruddy, Thomas; Powell, Pippa; Boyd, Jeanette

2017-01-01

The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) is a European Respiratory Society (ERS) Clinical Research Collaboration dedicated to improving research and clinical care for people with bronchiectasis. EMBARC has created a European Bronchiectasis Registry, funded by the ERS and by the European Union (EU) Innovative Medicines Initiative Programme. From the outset, EMBARC had the ambition to be a patient-focussed project. In contrast to many respiratory diseases, however, there are no specific patient charities or European patient organisations for patients with bronchiectasis and no existing infrastructure for patient engagement. This article describes the experience of EMBARC and the European Lung Foundation in establishing a patient advisory group and then engaging this group in European guidelines, an international registry and a series of research studies. Patient involvement in research, clinical guidelines and educational activities is increasingly advocated and increasingly important. Genuine patient engagement can achieve a number of goals that are critical to the success of an EU project, including focussing activities on patient priorities, allowing patients to direct the clinical and research agenda, and dissemination of guidelines and research findings to patients and the general public. Here, we review lessons learned and provide guidance for future ERS task forces, EU-funded projects or clinical research collaborations that are considering patient involvement. Educational aims To understand the different ways in which patients can contribute to clinical guidelines, research projects and educational activities. To understand the barriers and potential solutions to these barriers from a physician’s perspective, in order to ensure meaningful patient involvement in clinical projects. To understand the barriers and potential solutions from a patient’s perspective, in order to meaningfully involve patients in clinical projects. PMID:28894480

Patient participation in ERS guidelines and research projects: the EMBARC experience.

PubMed

Chalmers, James D; Timothy, Alan; Polverino, Eva; Almagro, Marta; Ruddy, Thomas; Powell, Pippa; Boyd, Jeanette

2017-09-01

The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) is a European Respiratory Society (ERS) Clinical Research Collaboration dedicated to improving research and clinical care for people with bronchiectasis. EMBARC has created a European Bronchiectasis Registry, funded by the ERS and by the European Union (EU) Innovative Medicines Initiative Programme. From the outset, EMBARC had the ambition to be a patient-focussed project. In contrast to many respiratory diseases, however, there are no specific patient charities or European patient organisations for patients with bronchiectasis and no existing infrastructure for patient engagement. This article describes the experience of EMBARC and the European Lung Foundation in establishing a patient advisory group and then engaging this group in European guidelines, an international registry and a series of research studies. Patient involvement in research, clinical guidelines and educational activities is increasingly advocated and increasingly important. Genuine patient engagement can achieve a number of goals that are critical to the success of an EU project, including focussing activities on patient priorities, allowing patients to direct the clinical and research agenda, and dissemination of guidelines and research findings to patients and the general public. Here, we review lessons learned and provide guidance for future ERS task forces, EU-funded projects or clinical research collaborations that are considering patient involvement. To understand the different ways in which patients can contribute to clinical guidelines, research projects and educational activities.To understand the barriers and potential solutions to these barriers from a physician's perspective, in order to ensure meaningful patient involvement in clinical projects.To understand the barriers and potential solutions from a patient's perspective, in order to meaningfully involve patients in clinical projects.

[EFFICACY OF IVIG TREATMENT IN BRONCHIECTASIS ASSOCIATED WITH IGG SUBCLASS DEFICIENCY].

PubMed

Shostak, Yael; Kramer, Mordechai R

2017-11-01

Bronchiectasis is characterized by an abnormal dilatation of the bronchi leading to a chronic inflammatory process, airway blockage and impaired clearance of secretions. The damage to the airways is usually progressive and is the result of several pathogenic processes. In the past, healing of infections (especially pulmonary tuberculosis) was the main cause of airway dilatation and progression of chronic inflammation. Today, congenital illnesses, anatomical defects and immune deficiency play an important role in the pathogenesis of bronchiectasis formation. The immunoglobulin repertoire is vital for effective host protection against a wide variety of pathogens. Primary antibody deficiency diseases are defects of the humoral arm of the immune system and involve an absence/reduced levels of one or more immunoglobulin classes/subclasses or defects of specific antibody formation. Immunoglobulin G (IGG) subclass deficiency can occur in a healthy person and could be without clinical significance. However, in recent years there is emerging evidence that in patients with recurrent infections, early diagnosis of antibody deficiency affects the prognosis and prevention of ongoing lung damage. The use of IVIG has contributed significantly to the survival rate in primary antibody deficiencies. There is limited literature on the treatment of IVIG for patients with IGG subclass deficiency. However, all studies presented so far demonstrated that immunoglobulin therapy reduced the rate of bacterial infections, days of antibiotic usage, hospital admissions and significantly increased patients' quality of life. Therefore, in the appropriate clinical setting, ie: a patient with bronchiectasis and recurrent infections, it is justified to test whether there are humoral immune defects such as IGG subclass deficiency. In a patient with proven deficiency, we should recommend to start IVIG treatment until clinical benefit is achieved.

ANTIBIOTIC THERAPY OF ABSCESS OF THE LUNG AND BRONCHIECTASIS

PubMed Central

Hewitt, William L.

1952-01-01

Since the fusospirochetal group of bacteria are the commonest etiologic agents in abscess of the lung, aqueous crystalline penicillin is the agent of first choice in the majority of cases. Streptomycin is indicated for a small group of cases in which Klebsiella is the etiologic agent. Aureomycin, chloramphenicol or terramycin may produce an excellent therapeutic response either initially or after therapeutic failure with penicillin. Administration of antibiotics by inhalation should be carried out in conjunction with systemic forms of treatment. In the treatment of bronchiectasis, the antibiotics are most useful in the control of acute exacerbations of pulmonary infection which punctuate the course of this disease. PMID:14935877

Early bronchiectasis in cystic fibrosis detected by surveillance CT.

PubMed

Pillarisetti, Naveen; Linnane, Barry; Ranganathan, Sarath

2010-08-01

There is emerging evidence that cystic fibrosis lung disease begins early in infancy. Newborn screening allows early detection and surveillance of pulmonary disease and the possibility of early intervention in this life-shortening condition. We report two children with cystic fibrosis who underwent a comprehensive assessment from diagnosis that included measurement of lung function, limited-slice high-resolution CT and BAL performed annually. Early aggressive surveillance enabled significant lung disease and bronchiectasis to be detected during the first few years of life and led to a change in management, highlighting a clinical role for CT scanning during the preschool years in children with cystic fibrosis.

Understanding COPD-overlap syndromes.

PubMed

Poh, Tuang Yeow; Mac Aogáin, Micheál; Chan, Adrian Kwok Wai; Yii, Anthony Chau Ang; Yong, Valerie Fei Lee; Tiew, Pei Yee; Koh, Mariko Siyue; Chotirmall, Sanjay Haresh

2017-04-01

Chronic obstructive pulmonary disease accounts for a large burden of lung disease. It can 'overlap' with other respiratory diseases including bronchiectasis, fibrosis and obstructive sleep apnea (OSA). While COPD alone confers morbidity and mortality, common features with contrasting clinical outcomes can occur in COPD 'overlap syndromes'. Areas covered: Given the large degree of heterogeneity in COPD, individual variation to treatment is adopted based on its observed phenotype, which in turn overlaps with features of other respiratory disease states such as asthma. This is coined asthma-COPD overlap syndrome ('ACOS'). Other examples of such overlapping clinical states include bronchiectasis-COPD ('BCOS'), fibrosis-COPD ('FCOS') and OSA-COPD ('OCOS'). The objective of this review is to highlight similarities and differences between the COPD-overlap syndromes in terms of risk factors, pathophysiology, diagnosis and potential treatment differences. Expert commentary: As a consequence of COPD overlap syndromes, a transition from the traditional 'one size fits all' treatment approach is necessary. Greater treatment stratification according to clinical phenotype using a precision medicine approach is now required. In this light, it is important to recognize and differentiate COPD overlap syndromes as distinct disease states compared to individual diseases such as asthma, COPD, fibrosis or bronchiectasis.

A comparison of preoperative and postoperative nutritional states of lung transplant recipients.

PubMed

Madill, J; Maurer, J R; de Hoyos, A

1993-08-01

Malnutrition is a documented problem in some types of endstage lung disease (ESLD). Recently, isolated lung transplants have successfully reversed the respiratory failure of patients suffering from ESLD. In this study, we compare the preoperative and postoperative nutritional states of lung transplant recipients using weight-to-height ratios, anthropometric measurements, subjective global assessment, and biochemical blood values. Patients with emphysema, cystic fibrosis, and other types of bronchiectasis, but not patients with pulmonary fibrosis or pulmonary hypertension, were malnourished preoperatively. All groups had normal biochemical profiles. Caloric intake of patients with cystic fibrosis and bronchiectasis was increased above predicted basal energy expenditure levels. By six months to one year postoperatively, all groups of malnourished patients had significantly improved their nutritional status. Emphysema patients improved nutrition by maintaining preoperative caloric intake levels--however, both cystic fibrosis and bronchiectasis patients were able to achieve the same goal with significantly decreased caloric intakes. We conclude that malnourished ESLD patients receiving isolated lung grafts are able to achieve normal nutrition within one year posttransplant. Since this occurs in all cases with a reduced, or at best maintained, caloric intake, more study is needed to elucidate the factors that contribute to ESLD malnutrition.

Coexistence of bronchiectasis and rheumatoid arthritis: revisited.

PubMed

Wilczynska, Maria M; Condliffe, Alison M; McKeon, Damian J

2013-04-01

The presence of bronchiectasis (BR) in patients with rheumatoid arthritis (RA) has been recognized for many decades; nevertheless, little research has been undertaken in this area. It is important to recognize that BR coexistent with RA differs from the other types of BR. The purpose of this descriptive review was to delineate the epidemiology, etiology, risk factors, pulmonary function testing, imaging, prognosis and management of concomitant BR and RA. To inform our study we searched the PubMed, EMBASE, CINAHL, and MEDLINE databases, using combinations of the following key words: computed tomography, lung function tests, rheumatoid arthritis, bronchiectasis, biological agents, and interstitial lung disease. The number of published papers covering this topic is limited, but several relevant conclusions can be drawn. Patients with concomitant RA and BR have worse obstructive airways disease, increased susceptibility to recurrent pulmonary infections, faster lung function decline, and higher mortality, compared with subjects with either RA or BR alone. The use of disease-modifying anti-rheumatic drugs (both biological and non-biological) for RA in RA-BR patients imparts a further challenge in managing these patients. Although there are not any published guidelines on the management of coexisting RA-BR, we have attempted to provide such recommendations, based on the literature review and our experience.

The relationship between psychological symptoms, lung function and quality of life in children and adolescents with non-cystic fibrosis bronchiectasis.

PubMed

Bahali, Kayhan; Gedik, Ahmet Hakan; Bilgic, Ayhan; Cakir, Erkan; Ustabas Kahraman, Feyza; Keskin Osmanoglu, Nurcan; Uzuner, Selcuk; Kilicoglu, Ali Guven

2014-01-01

The aim of this study was to evaluate the relationship between psychological symptoms and quality of life (QOL) and clinical variables in a cohort of children and adolescents with non-cystic fibrosis (non-CF) bronchiectasis. Seventy-six patients (aged 8-17years) participated in this study. Questionnaires were used to evaluate the psychological status and QOL of the patients and healthy controls. The patient and control groups were divided into child and adolescent groups to exclude the effect of puberty on psychological status. No significant difference was found between patient and control groups for mean depression and trait anxiety scores. Only the child-rated physical health QOL scores were significantly lower for patients than the controls. Also, excepting physical health scores in adolescent group, all of the parent-rated QOL scores were significantly lower in both group and total subjects. Regarding determinants of QOL, age of children and FEV1/FVC percent predicted had positive effects, while dyspnea severity and trait anxiety had negative effects, for the sample as a whole. Non-CF bronchiectasis is associated with poorer QOL in childhood. The impact of the disease on QOL occurs through both clinical and psychological variables. Copyright © 2014 Elsevier Inc. All rights reserved.

Validation of the Mandarin Chinese version of the Leicester Cough Questionnaire in bronchiectasis.

PubMed

Gao, Y-H; Guan, W-J; Xu, G; Gao, Y; Lin, Z-Y; Tang, Y; Lin, Z-M; Li, H-M; Luo, Q; Zhong, N-S; Birring, S S; Chen, R-C

2014-12-01

The Leicester Cough Questionnaire (LCQ) has been validated for assessing cough-specific health status in bronchiectasis. We translated the LCQ into Mandarin Chinese and investigated its validity, reliability and responsiveness. The LCQ was translated into Mandarin Chinese using the forward-backward translation procedure. A total of 144 out-patients completed the Mandarin Chinese version of the LCQ (LCQ-MC), the Hospital Anxiety and Depression Scale (HADS) and the St George's Respiratory Questionnaire. Reassessments were performed during exacerbations and at 6 months. Concurrent validation, internal consistency, repeatability and responsiveness were determined. Minor cultural adaptations were made to the wording of LCQ-MC. No other difficulties were found during the translation process, with all items easily adapted to acceptable Mandarin Chinese. The questionnaire was not changed in terms of content layout and the order of the questions. In cognitive debriefing interviews, participants reported that the questionnaire was acceptable, relevant, comprehensive and easy to complete. The LCQ-MC showed good concurrent validity, internal consistency and test-retest reliability. Responsiveness was shown by significant changes in LCQ-MC scores between steady state, the first exacerbation and following 2-week antibiotic treatment (both interval changes, P < 0.01) CONCLUSION: The LCQ-MC is a valid, reliable and responsive instrument for determining cough-specific health status in Chinese bronchiectasis patients.

Evaluation of obstructive sleep apnea in non-cystic fibrosis bronchiectasis: A cross-sectional study

PubMed Central

Urbano, Jessica Julioti; Santos, Israel Reis; Silva, Anderson Soares; Perez, Eduardo Araújo; Souza, Ângela Honda; Nascimento, Oliver Augusto; Jardim, José Roberto; Insalaco, Giuseppe; Oliveira, Luis Vicente Franco; Stirbulov, Roberto

2017-01-01

The relationship between sleep disorders and bronchiectasis has not been well described. We hypothesize that, due to the irreversible dilatation of the bronchi, the presence of secretions, and airflow obstruction, patients with non-cystic fibrosis bronchiectasis may be predisposed to hypoxemia during sleep, or to symptoms that may lead to arousal. A cross-sectional observational study was performed involving 49 patients with a clinical diagnosis of non-cystic fibrosis bronchiectasis (NCFB). All patients underwent clinical evaluation, spirometry, and polysomnography, and were evaluated for the presence of excessive daytime sleepiness (EDS) and risk of obstructive sleep apnea (OSA). The mean age of the participants was 50.3 ± 13.6 years; 51.1% of patients were male and had a mean body mass index of 23.8 ± 3.4 kg/m2. The mean total sleep time (TST) was 325.15 ± 64.22 min with a slight reduction in sleep efficiency (84.01 ± 29.2%). Regarding sleep stages, stage 1 sleep and REM sleep were abnormal. OSA was present in 40.82% of the patients. The mean arousal index was 5.6 ± 2.9/h and snoring was observed in 71.43% of the patients. The oxygen desaturation index (ODI) was 14.35 ± 15.36/h, mean minimum oxygen saturation (SpO2 nadir) was 83.29 ± 7.99%, and mean TST with an SpO2 less than 90% was 30.21 ± 60.48 min. EDS was exhibited by 53.06% of the patients and 55.1% were at high risk of developing OSA. The patients infected by Pseudomonas aeruginosa had higher apnea-hypopnea indices, ODI, and TST with SpO2 < 90%, and lower values of SpO2 nadir. Adult patients with clinically stable NCFB, especially those infected by Pseudomonas aeruginosa, display EDS and a high prevalence of OSA, associated with considerable oxygen desaturation during sleep. PMID:28972989

Evaluation of obstructive sleep apnea in non-cystic fibrosis bronchiectasis: A cross-sectional study.

PubMed

Faria Júnior, Newton Santos; Urbano, Jessica Julioti; Santos, Israel Reis; Silva, Anderson Soares; Perez, Eduardo Araújo; Souza, Ângela Honda; Nascimento, Oliver Augusto; Jardim, José Roberto; Insalaco, Giuseppe; Oliveira, Luis Vicente Franco; Stirbulov, Roberto

2017-01-01

The relationship between sleep disorders and bronchiectasis has not been well described. We hypothesize that, due to the irreversible dilatation of the bronchi, the presence of secretions, and airflow obstruction, patients with non-cystic fibrosis bronchiectasis may be predisposed to hypoxemia during sleep, or to symptoms that may lead to arousal. A cross-sectional observational study was performed involving 49 patients with a clinical diagnosis of non-cystic fibrosis bronchiectasis (NCFB). All patients underwent clinical evaluation, spirometry, and polysomnography, and were evaluated for the presence of excessive daytime sleepiness (EDS) and risk of obstructive sleep apnea (OSA). The mean age of the participants was 50.3 ± 13.6 years; 51.1% of patients were male and had a mean body mass index of 23.8 ± 3.4 kg/m2. The mean total sleep time (TST) was 325.15 ± 64.22 min with a slight reduction in sleep efficiency (84.01 ± 29.2%). Regarding sleep stages, stage 1 sleep and REM sleep were abnormal. OSA was present in 40.82% of the patients. The mean arousal index was 5.6 ± 2.9/h and snoring was observed in 71.43% of the patients. The oxygen desaturation index (ODI) was 14.35 ± 15.36/h, mean minimum oxygen saturation (SpO2 nadir) was 83.29 ± 7.99%, and mean TST with an SpO2 less than 90% was 30.21 ± 60.48 min. EDS was exhibited by 53.06% of the patients and 55.1% were at high risk of developing OSA. The patients infected by Pseudomonas aeruginosa had higher apnea-hypopnea indices, ODI, and TST with SpO2 < 90%, and lower values of SpO2 nadir. Adult patients with clinically stable NCFB, especially those infected by Pseudomonas aeruginosa, display EDS and a high prevalence of OSA, associated with considerable oxygen desaturation during sleep.

Multidimensional severity assessment in bronchiectasis: an analysis of seven European cohorts.

PubMed

McDonnell, M J; Aliberti, S; Goeminne, P C; Dimakou, K; Zucchetti, S C; Davidson, J; Ward, C; Laffey, J G; Finch, S; Pesci, A; Dupont, L J; Fardon, T C; Skrbic, D; Obradovic, D; Cowman, S; Loebinger, M R; Rutherford, R M; De Soyza, A; Chalmers, J D

2016-12-01

Bronchiectasis is a multidimensional disease associated with substantial morbidity and mortality. Two disease-specific clinical prediction tools have been developed, the Bronchiectasis Severity Index (BSI) and the FACED score, both of which stratify patients into severity risk categories to predict the probability of mortality. We aimed to compare the predictive utility of BSI and FACED in assessing clinically relevant disease outcomes across seven European cohorts independent of their original validation studies. The combined cohorts totalled 1612. Pooled analysis showed that both scores had a good discriminatory predictive value for mortality (pooled area under the curve (AUC) 0.76, 95% CI 0.74 to 0.78 for both scores) with the BSI demonstrating a higher sensitivity (65% vs 28%) but lower specificity (70% vs 93%) compared with the FACED score. Calibration analysis suggested that the BSI performed consistently well across all cohorts, while FACED consistently overestimated mortality in 'severe' patients (pooled OR 0.33 (0.23 to 0.48), p<0.0001). The BSI accurately predicted hospitalisations (pooled AUC 0.82, 95% CI 0.78 to 0.84), exacerbations, quality of life (QoL) and respiratory symptoms across all risk categories. FACED had poor discrimination for hospital admissions (pooled AUC 0.65, 95% CI 0.63 to 0.67) with low sensitivity at 16% and did not consistently predict future risk of exacerbations, QoL or respiratory symptoms. No association was observed with FACED and 6 min walk distance (6MWD) or lung function decline. The BSI accurately predicts mortality, hospital admissions, exacerbations, QoL, respiratory symptoms, 6MWD and lung function decline in bronchiectasis, providing a clinically relevant evaluation of disease severity. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Oral supplement enriched in HMB combined with pulmonary rehabilitation improves body composition and health related quality of life in patients with bronchiectasis (Prospective, Randomised Study).

PubMed

Olveira, Gabriel; Olveira, Casilda; Doña, Esperanza; Palenque, Francisco Javier; Porras, Nuria; Dorado, Antonio; Godoy, Ana M; Rubio-Martínez, Elehazara; Rojo-Martínez, Gemma; Martín-Valero, Rocío

2016-10-01

Pulmonary Rehabilitation (PR) is recommended for bronchiectasis but there is no data about its effect on body composition. The aim of this study is to assess the effect of Pulmonary Rehabilitation (PR) for 12 weeks in normally-nourished non-cystic-fibrosis bronchiectasis patients compared with the effect of PR plus a hyperproteic oral nutritional supplement enriched with beta-hydroxy-beta-methylbutyrate (HMB) on body composition, muscle strength, quality of life and serum biomarkers. single center randomized controlled trial, parallel treatment design: Participants were randomly assigned to receive PR for 12 weeks or PR plus ONS (PRONS) (one can per day). Outcome assessments were performed at baseline, 12 weeks and 24 weeks: body composition (Dual-energy X-Ray Absorptiometry (DEXA), mid-arm muscle circumference (MAMC), phase angle by Bio-impedance), health related quality of life (Spanish QOL-B-V3.0, Physical Functioning Scale), handgrip strength, diet questionnaire, and plasma levels of prealbumin, myostatin and somatomedin-c. Thirty patients were randomized (15 per group) without differences in clinical and respiratory variables. In the PRONS group bone mineral density (BMD), mean and maximum handgrip dynamometry, MAMC, QOLB and prealbumin were significantly increased from baseline at 12 and 24 weeks and Fat free Mass (FFM) and FFM index, at 12 weeks. In the PR group only mean handgrip dynamometry and prealbumin were significantly increased at 12 and 24 weeks. In both groups plasma myostatin was reduced at 12 weeks (without significant differences). The addition of a hyperproteic ONS enriched with HMB to Pulmonary Rehabilitation could improve body composition, BMD, muscle strength and health related quality of life in bronchiectasis patients. Clinical Trials Number NCT02048397. Copyright © 2015 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.

Quality of Life Questionnaire-Bronchiectasis: final psychometric analyses and determination of minimal important difference scores.

PubMed

Quittner, Alexandra L; O'Donnell, Anne E; Salathe, Matthias A; Lewis, Sandra A; Li, Xiaoming; Montgomery, A Bruce; O'Riordan, Thomas G; Barker, Alan F

2015-01-01

The Quality of Life-Bronchiectasis (QOL-B), a self-administered, patient-reported outcome measure assessing symptoms, functioning and health-related quality of life for patients with non-cystic fibrosis (CF) bronchiectasis, contains 37 items on 8 scales (Respiratory Symptoms, Physical, Role, Emotional and Social Functioning, Vitality, Health Perceptions and Treatment Burden). Psychometric analyses of QOL-B V.3.0 used data from two double-blind, multicentre, randomised, placebo-controlled, phase III trials of aztreonam for inhalation solution (AZLI) in 542 patients with non-CF bronchiectasis and Gram-negative endobronchial infection. Excellent internal consistency (Cronbach's α ≥0.70) and 2-week test-retest reliability (intraclass correlation coefficients ≥0.72) were demonstrated for each scale. Convergent validity with 6 min walk test was observed for Physical and Role Functioning scores. No floor or ceiling effects (baseline scores of 0 or 100) were found for the Respiratory Symptoms scale (primary endpoint of trials). Baseline Respiratory Symptoms scores discriminated between patients based on baseline FEV₁% predicted in only one trial. The minimal important difference score for the Respiratory Symptoms scale was 8.0 points. AZLI did not show efficacy in the two phase III trials. QOL-B responsivity to treatment was assessed by examining changes from baseline QOL-B scores at study visits at which protocol-defined pulmonary exacerbations were reported. Mean Respiratory Symptoms scores decreased 14.0 and 14.2 points from baseline for placebo-treated and AZLI-treated patients with exacerbations, indicating that worsening respiratory symptoms were reflected in clinically meaningful changes in QOL-B scores. Previously established content validity, reliability and responsivity of the QOL-B are confirmed by this final validation study. The QOL-B is available for use in clinical trials and routine clinical practice. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Defining the content and delivery of an intervention to Change AdhereNce to treatment in BonchiEctasis (CAN-BE): a qualitative approach incorporating the Theoretical Domains Framework, behavioural change techniques and stakeholder expert panels.

PubMed

McCullough, Amanda R; Ryan, Cristín; O'Neill, Brenda; Bradley, Judy M; Elborn, J Stuart; Hughes, Carmel M

2015-08-22

Low patient adherence to treatment is associated with poorer health outcomes in bronchiectasis. We sought to use the Theoretical Domains Framework (TDF) (a framework derived from 33 psychological theories) and behavioural change techniques (BCTs) to define the content of an intervention to change patients' adherence in bronchiectasis (Stage 1 and 2) and stakeholder expert panels to define its delivery (Stage 3). We conducted semi-structured interviews with patients with bronchiectasis about barriers and motivators to adherence to treatment and focus groups or interviews with bronchiectasis healthcare professionals (HCPs) about their ability to change patients' adherence to treatment. We coded these data to the 12 domain TDF to identify relevant domains for patients and HCPs (Stage 1). Three researchers independently mapped relevant domains for patients and HCPs to a list of 35 BCTs to identify two lists (patient and HCP) of potential BCTs for inclusion (Stage 2). We presented these lists to three expert panels (two with patients and one with HCPs/academics from across the UK). We asked panels who the intervention should target, who should deliver it, at what intensity, in what format and setting, and using which outcome measures (Stage 3). Eight TDF domains were perceived to influence patients' and HCPs' behaviours: Knowledge, Skills, Beliefs about capability, Beliefs about consequences, Motivation, Social influences, Behavioural regulation and Nature of behaviours (Stage 1). Twelve BCTs common to patients and HCPs were included in the intervention: Monitoring, Self-monitoring, Feedback, Action planning, Problem solving, Persuasive communication, Goal/target specified:behaviour/outcome, Information regarding behaviour/outcome, Role play, Social support and Cognitive restructuring (Stage 2). Participants thought that an individualised combination of these BCTs should be delivered to all patients, by a member of staff, over several one-to-one and/or group visits in secondary care. Efficacy should be measured using pulmonary exacerbations, hospital admissions and quality of life (Stage 3). Twelve BCTs form the intervention content. An individualised selection from these 12 BCTs will be delivered to all patients over several face-to-face visits in secondary care. Future research should focus on developing physical materials to aid delivery of the intervention prior to feasibility and pilot testing. If effective, this intervention may improve adherence and health outcomes for those with bronchiectasis in the future.

Alpha-1 antitrypsin blood test

MedlinePlus

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Long term radiological features of radiation-induced lung damage.

PubMed

Veiga, Catarina; Landau, David; McClelland, Jamie R; Ledermann, Jonathan A; Hawkes, David; Janes, Sam M; Devaraj, Anand

2018-02-01

To describe the radiological findings of radiation-induced lung damage (RILD) present on CT imaging of lung cancer patients 12 months after radical chemoradiation. Baseline and 12-month CT scans of 33 patients were reviewed from a phase I/II clinical trial of isotoxic chemoradiation (IDEAL CRT). CT findings were scored in three categories derived from eleven sub-categories: (1) parenchymal change, defined as the presence of consolidation, ground-glass opacities (GGOs), traction bronchiectasis and/or reticulation; (2) lung volume reduction, identified through reduction in lung height and/or distortions in fissures, diaphragm, anterior junction line and major airways anatomy, and (3) pleural changes, either thickening and/or effusion. Six patients were excluded from the analysis due to anatomical changes caused by partial lung collapse and abscess. All remaining 27 patients had radiological evidence of lung damage. The three categories, parenchymal change, shrinkage and pleural change were present in 100%, 96% and 82% respectively. All patients had at least two categories of change present and 72% all three. GGOs, reticulation and traction bronchiectasis were present in 44%, 52% and 37% of patients. Parenchymal change, lung shrinkage and pleural change are present in a high proportion of patients and are frequently identified in RILD. GGOs, reticulation and traction bronchiectasis are common at 12 months but not diagnostic. Copyright © 2017 Elsevier B.V. All rights reserved.

The clinical utility of long-term humidification therapy in chronic airway disease.

PubMed

Rea, Harold; McAuley, Sue; Jayaram, Lata; Garrett, Jeffrey; Hockey, Hans; Storey, Louanne; O'Donnell, Glenis; Haru, Lynne; Payton, Matthew; O'Donnell, Kevin

2010-04-01

Persistent airway inflammation with mucus retention in patients with chronic airway disorders such as COPD and bronchiectasis may lead to frequent exacerbations, reduced lung function and poor quality of life. This study investigates if long-term humidification therapy with high flow fully humidified air at 37 degrees C through nasal cannulae can improve these clinical outcomes in this group of patients. 108 patients diagnosed with COPD or bronchiectasis were randomised to daily humidification therapy or usual care for 12 months over which exacerbations were recorded. Lung function, quality of life, exercise capacity, and measures of airway inflammation were also recorded at baseline, 3 and 12 months. Patients on long-term humidification therapy had significantly fewer exacerbation days (18.2 versus 33.5 days; p = 0.045), increased time to first exacerbation (median 52 versus 27 days; p = 0.0495) and reduced exacerbation frequency (2.97/patient/year versus 3.63/patient/year; p = 0.067) compared with usual care. Quality of life scores and lung function improved significantly with humidification therapy compared with usual care at 3 and 12 months. Long-term humidification therapy significantly reduced exacerbation days, increased time to first exacerbation, improved lung function and quality of life in patients with COPD and bronchiectasis. Clinical trial registered with www.actr.org.au; Number ACTRN2605000623695. Copyright 2010 Elsevier Ltd. All rights reserved.

Bronchiectasis

MedlinePlus

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Bronchiectasis

MedlinePlus

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Self-management for bronchiectasis.

PubMed

Kelly, Carol; Grundy, Seamus; Lynes, Dave; Evans, David Jw; Gudur, Sharada; Milan, Stephen J; Spencer, Sally

2018-02-07

Bronchiectasis is a long term respiratory condition with an increasing rate of diagnosis. It is associated with persistent symptoms, repeated infective exacerbations, and reduced quality of life, imposing a burden on individuals and healthcare systems. The main aims of therapeutic management are to reduce exacerbations and improve quality of life. Self-management interventions are potentially important for empowering people with bronchiectasis to manage their condition more effectively and to seek care in a timely manner. Self-management interventions are beneficial in the management of other airways diseases such as asthma and COPD (chronic obstructive pulmonary disease) and have been identified as a research priority for bronchiectasis. To assess the efficacy, cost-effectiveness and adverse effects of self-management interventions for adults and children with non-cystic fibrosis bronchiectasis. We searched the Cochrane Airways Specialised Register of trials, clinical trials registers, reference lists of included studies and review articles, and relevant manufacturers' websites up to 13 December 2017. We included all randomised controlled trials of any duration that included adults or children with a diagnosis of non-cystic fibrosis bronchiectasis assessing self-management interventions delivered in any form. Self-management interventions included at least two of the following elements: patient education, airway clearance techniques, adherence to medication, exercise (including pulmonary rehabilitation) and action plans. Two review authors independently screened searches, extracted study characteristics and outcome data and assessed risk of bias for each included study. Primary outcomes were, health-related quality of life, exacerbation frequency and serious adverse events. Secondary outcomes were the number of participants admitted to hospital on at least one occasion, lung function, symptoms, self-efficacy and economic costs. We used a random effects model for analyses and standard Cochrane methods throughout. Two studies with a total of 84 participants were included: a 12-month RCT of early rehabilitation in adults of mean age 72 years conducted in two centres in England (UK) and a six-month proof-of-concept RCT of an expert patient programme (EPP) in adults of mean age 60 years in a single regional respiratory centre in Northern Ireland (UK). The EPP was delivered in group format once a week for eight weeks using standardised EPP materials plus disease-specific education including airway clearance techniques, dealing with symptoms, exacerbations, health promotion and available support. We did not find any studies that included children. Data aggregation was not possible and findings are reported narratively in the review.For the primary outcomes, both studies reported health-related quality of life, as measured by the St George's Respiratory Questionnaire (SGRQ), but there was no clear evidence of benefit. In one study, the mean SGRQ total scores were not significantly different at 6 weeks', 3 months' and 12 months' follow-up (12 months mean difference (MD) -10.27, 95% confidence interval (CI) -45.15 to 24.61). In the second study there were no significant differences in SGRQ. Total scores were not significantly different between groups (six months, MD 3.20, 95% CI -6.64 to 13.04). We judged the evidence for this outcome as low or very low. Neither of the included studies reported data on exacerbations requiring antibiotics. For serious adverse events, one study reported more deaths in the intervention group compared to the control group, (intervention: 4 of 8, control: 2 of 12), though interpretation is limited by the low event rate and the small number of participants in each group.For our secondary outcomes, there was no evidence of benefit in terms of frequency of hospital admissions or FEV1 L, based on very low-quality evidence. One study reported self-efficacy using the Chronic Disease Self-Efficacy scale, which comprises 10 components. All scales showed significant benefit from the intervention but effects were only sustained to study endpoint on the Managing Depression scale. Further details are reported in the main review. Based on overall study quality, we judged this evidence as low quality. Neither study reported data on respiratory symptoms, economic costs or adverse events. There is insufficient evidence to determine whether self-management interventions benefit people with bronchiectasis. In the absence of high-quality evidence it is advisable that practitioners adhere to current international guidelines that advocate self-management for people with bronchiectasis.Future studies should aim to clearly define and justify the specific nature of self-management, measure clinically important outcomes and include children as well as adults.

Prolonged antibiotics for non-cystic fibrosis bronchiectasis in children and adults.

PubMed

Hnin, Khin; Nguyen, Chau; Carson, Kristin V; Evans, David J; Greenstone, Michael; Smith, Brian J

2015-08-13

The vicious cycle hypothesis for bronchiectasis predicts that bacterial colonisation of the respiratory tract perpetuates inflammatory change. This damages the mucociliary escalator, preventing bacterial clearance and allowing persistence of pro-inflammatory mediators. Conventional treatment with physiotherapy and intermittent antibiotics is believed to improve the condition of people with bronchiectasis, although no conclusive data show that these interventions influence the natural history of the condition. Various strategies have been tried to interrupt this cycle of infection and inflammation, including prolonging antibiotic treatment with the goal of allowing the airway mucosa to heal. To determine the benefits of prolonged antibiotic therapy in the treatment of patients with bronchiectasis. We searched the Cochrane Airways Group Trials Register and reference lists of identified articles. Searches were current as of February 2014. Randomised trials examining the use of prolonged antibiotic therapy (for four or more weeks) in the treatment of bronchiectasis compared with placebo or usual care. Two review authors independently assessed trial quality and extracted data. We contacted study authors to ask for missing information. Eighteen trials met the inclusion criteria, randomly assigning a total of 1157 participants. Antibiotics were given for between four weeks and 83 weeks. Limited meta-analysis was possible because of the diversity of outcomes reported in these trials. Based on the number of participants with at least one exacerbation, the meta-analysis showed significant effects in favour of the intervention (odds ratio (OR) 0.31, 95% confidence interval (CI) 0.19 to 0.52; P value < 0.00001), with events occurring in 271 per 1000 people in the intervention arm (95% CI 126 to 385) and in 546 per 1000 in the control population, based on evidence of moderate quality. A non-statistically significant reduction in hospitalisation favoured the use of prolonged antibiotics with a moderate quality grade of supporting evidence (37 per 1000 in the intervention arm (95% CI 13 to 96) and 87 per 1000 in control (OR 0.40, 95% CI 0.14 to 1.11; P value = 0.08). Drug resistance developed in 36 of 220 participants taking antibiotics compared with 10 of 211 participants given placebo or standard therapy (OR 3.48, 95% CI 1.20 to 10.07; P value = 0.02), translating to natural frequencies of 155 per 1000 in the intervention arm (95% CI 59 to 346) and 50 per 1000 in the control arm. The intervention was well tolerated with no overall significant difference in withdrawal between treatment and placebo groups (OR 0.91, 95% CI 0.56 to 1.49). Diarrhoea was commonly reported as an adverse event, particularly with an oral intervention. Available evidence shows benefit associated with use of prolonged antibiotics in the treatment of patients with bronchiectasis, at least halving the odds of exacerbation (with 275 fewer exacerbations per every 1000 people treated in the antibiotic arm compared with the control arm) and hospitalisation (50 fewer hospitalisations per 1000 people in the antibiotic arm compared with the control arm). However, the risk of emerging drug resistance is increased more than threefold. This review is limited by diversity of trials and by evidence of moderate to low quality. Further randomised controlled trials with adequate power and standardised end points are required.

Inspiratory muscle training in bronchiectasis patients: a prospective randomized controlled study.

PubMed

Liaw, Mei-Yun; Wang, Yi-Hsi; Tsai, Yu-Chin; Huang, Kuo-Tung; Chang, Pei-Wen; Chen, Yung-Che; Lin, Meng-Chih

2011-06-01

To investigate the efficacy and feasibility of home-based inspiratory muscle training in patients with bronchiectasis. A prospective, single-blind, randomized, controlled study. Outpatient clinic of a tertiary care medical centre. Twenty-six patients with bronchiectasis were randomly divided into inspiratory muscle training and control groups. In the inspiratory muscle training group (n = 13), the training programme started with an intensity of 30% maximal inspiratory pressure (MIP), which was increased by 2 cmH(2)O each week, for 30 minutes daily, 5 days a week for eight weeks. The control group (n = 13) did not receive inspiratory muscle training. Main outcome measures included spirometry, resting oxyhaemoglobin saturation by pulse oximetry (SpO(2)), lowest SpO(2) and Borg Scale during 6-minute walking tests, 6-minute walking distance (6MWD), 6-minute walking work (6M(work)), MIP, maximal expiratory pressure (MEP) and St George's Respiratory Questionnaire. There were significant differences in change from baseline in 6MWD (411.9 (133.5) vs. 473.2 (117.2) m, P = 0.021), 6M(work) (21 051.0 (8286.7) vs. 23 915.5 (8343.0) kg-m, P = 0.022), MIP (60.8 (21.8) vs. 84.6 (29.0) cmH(2)O, P = 0.004), and MEP (72.3 (31.1) vs. 104.2 (35.7) cmH(2)O, P = 0.004) in the inspiratory muscle training group. Significant improvements in both MIP (23.8 (25.3) vs. 2.3 (16.4) cmH(2)O, adjusted P-value = 0.005) and MEP (31.9 (30.8) vs. 11.5 (20.8) cmH(2)O, adjusted P-value = 0.038) levels after adjusting for age by linear regression analysis were observed between groups. An eight-week home-based inspiratory muscle training is feasible and effective in improving both inspiratory and expiratory muscle strength, but has no effect on respiratory function and quality of life in patients with bronchiectasis.

Inhaled mannitol for the treatment of mucociliary dysfunction in patients with bronchiectasis: effect on lung function, health status and sputum.

PubMed

Daviskas, Evangelia; Anderson, Sandra D; Gomes, Kerry; Briffa, Peter; Cochrane, Belinda; Chan, H-Kim; Young, Iven H; Rubin, Bruce K

2005-01-01

Inhaled mannitol increases mucus clearance in patients with bronchiectasis by an unclear mechanism. The effect of mannitol on lung function, health status and sputum properties was investigated. Nine patients with bronchiectasis inhaled 400 mg of mannitol once daily for 12 days. Health status was assessed using the St George's Respiratory Questionnaire (SGRQ). Sputum was analysed for viscosity, elasticity, spinnability, surface tension, contact angle, solids, mucociliary transportability (MCTR) on a frog palate, and cough transportability (CTR) on a simulated cough machine. Lung function was unchanged with treatment (baseline FEV1 82.0 +/- 16.2%) apart from an improvement in FEF from 85.4 +/- 13% (baseline) to 90.7 +/- 14.4% (P < 0.05; 12th treatment; visit 7). The total SGRQ score (mean +/- SD) of 49.3 +/- 13.8 at baseline, decreased by 12.4 +/- 10.2 (P < 0.01; visit 7) and 10.1 +/- 9.4 units (P < 0.02) 6-10 days after treatment cessation. The baseline subscores for symptoms (72.9 +/- 11.8), activity (44.7 +/- 20.9) and impact (44.4 +/- 14.3) were reduced by 0.8 +/- 9.1 (P > 0.7), 8.4 +/- 16.0 (P > 0.1) and 19.2 +/- 13.7 (P < 0.005) units, respectively (visit 7). Mannitol reduced the baseline (mean +/- SE) surface tension from 94.5 +/- 1.4 to 84.7 +/- 2.1 mN/m (P < 0.0001), contact angle from 51.1 +/- 2.8 to 33.2 +/- 2.4 degrees (P < 0.0001), spinnability from 11.6 +/- 0.4 to 10.0 +/- 0.2 mm (P < 0.005), and solids from 5.7 +/- 0.4 to 4.3 +/- 0.7% (P < 0.02), acutely (visit 7). Viscosity, elasticity and MCTR did not change significantly, while CTR was increased from 25.8 +/- 1.0 to 34.1 +/- 2.7 mm (P < 0.003). Mannitol significantly improved the health status over 12 days and this improvement was maintained for 6-10 days after cessation of treatment. In addition, mannitol reduced the tenacity, increased the hydration of mucus acutely and improved cough clearability in patients with bronchiectasis.

The short and long term effects of exercise training in non-cystic fibrosis bronchiectasis--a randomised controlled trial.

PubMed

Lee, Annemarie L; Hill, Catherine J; Cecins, Nola; Jenkins, Sue; McDonald, Christine F; Burge, Angela T; Rautela, Linda; Stirling, Robert G; Thompson, Philip J; Holland, Anne E

2014-04-15

Exercise training is recommended for non-cystic fibrosis (CF) bronchiectasis, but the long-term effects are unclear. This randomised controlled trial aimed to determine the effects of exercise training and review of airway clearance therapy (ACT) on exercise capacity, health related quality of life (HRQOL) and the incidence of acute exacerbations in people with non-CF bronchiectasis. Participants were randomly allocated to 8 weeks of supervised exercise training and review of ACT, or control. Primary outcomes of exercise capacity and HRQOL (Chronic respiratory disease questionnaire) and secondary outcomes of cough-related QOL (Leicester cough questionnaire) and psychological symptoms (Hospital anxiety and depression scale) were measured at baseline, following completion of the intervention period and at 6 and 12 months follow up. Secondary outcomes of the exacerbation rate and time to first exacerbation were analysed over 12 months. Eighty-five participants (mean FEV1 74% predicted; median Modified Medical Research Council Dyspnoea grade of 1 (IQR [1-3]) were included. Exercise training increased the incremental shuttle walk distance (mean difference to control 62 m, 95% CI 24 to 101 m) and the 6-minute walking distance (mean difference to control 41 m, 95% CI 19 to 63 m), but these improvements were not sustained at 6 or 12 months. Exercise training reduced dyspnoea (p = 0.009) and fatigue (p = 0.01) but did not impact on cough-related QOL or mood. Exercise training reduced the frequency of acute exacerbations (median 1[IQR 1-3]) compared to the control group (2[1-3]) over 12 months follow up (p = 0.012), with a longer time to first exacerbation with exercise training of 8 months (95% CI 7 to 9 months) compared to the control group (6 months [95% CI 5 to 7 months], p = 0.047). Exercise training in bronchiectasis is associated with short term improvement in exercise capacity, dyspnoea and fatigue and fewer exacerbations over 12 months. ClinicalTrials.gov (NCT00885521).

The short and long term effects of exercise training in non-cystic fibrosis bronchiectasis – a randomised controlled trial

PubMed Central

2014-01-01

Background Exercise training is recommended for non-cystic fibrosis (CF) bronchiectasis, but the long-term effects are unclear. This randomised controlled trial aimed to determine the effects of exercise training and review of airway clearance therapy (ACT) on exercise capacity, health related quality of life (HRQOL) and the incidence of acute exacerbations in people with non-CF bronchiectasis. Methods Participants were randomly allocated to 8 weeks of supervised exercise training and review of ACT, or control. Primary outcomes of exercise capacity and HRQOL (Chronic respiratory disease questionnaire) and secondary outcomes of cough-related QOL (Leicester cough questionnaire) and psychological symptoms (Hospital anxiety and depression scale) were measured at baseline, following completion of the intervention period and at 6 and 12 months follow up. Secondary outcomes of the exacerbation rate and time to first exacerbation were analysed over 12 months. Results Eighty-five participants (mean FEV1 74% predicted; median Modified Medical Research Council Dyspnoea grade of 1 (IQR [1–3]) were included. Exercise training increased the incremental shuttle walk distance (mean difference to control 62 m, 95% CI 24 to 101 m) and the 6-minute walking distance (mean difference to control 41 m, 95% CI 19 to 63 m), but these improvements were not sustained at 6 or 12 months. Exercise training reduced dyspnoea (p = 0.009) and fatigue (p = 0.01) but did not impact on cough-related QOL or mood. Exercise training reduced the frequency of acute exacerbations (median 1[IQR 1–3]) compared to the control group (2[1–3]) over 12 months follow up (p = 0.012), with a longer time to first exacerbation with exercise training of 8 months (95% CI 7 to 9 months) compared to the control group (6 months [95% CI 5 to 7 months], p = 0.047). Conclusions Exercise training in bronchiectasis is associated with short term improvement in exercise capacity, dyspnoea and fatigue and fewer exacerbations over 12 months. Trial registry ClinicalTrials.gov (NCT00885521). PMID:24731015

Lung surgery - discharge

MedlinePlus

... Read More Bronchiectasis Chronic obstructive pulmonary disease Lung cancer Lung cancer - non-small cell Lung cancer - small cell ... team. Related MedlinePlus Health Topics COPD Emphysema Lung Cancer Lung Diseases Pleural Disorders Browse the Encyclopedia A.D. ...

The Effect of Lt to Rt Shunt Using Veno-veno-arterial Extracorporeal Membrane Oxygenation (ECMO) on Coronary Oxygenation in Lung Transplantation Patients

ClinicalTrials.gov

2016-08-03

Interstitial Pulmonary Fibrosis ARDS; COPD (Chronic Obstructive Pulmonary Disease); Bronchiectasis; Lymphangioleiomyomatosis; Primary Pulmonary Hypertension; ARDS (Acute Respiratory Distress Syndrome)

Genetics Home Reference: primary ciliary dyskinesia

MedlinePlus

... mutations explain only 2% of primary ciliary dykinesia. Respiration. 2008;76(2):198-204. doi: 10.1159/ ... MR. Genetic causes of bronchiectasis: primary ciliary dyskinesia. Respiration. 2007;74(3):252-63. Review. Citation on ...

Radiographic appearance of bronchoalveolar carcinoma in nine cats.

PubMed

Ballegeer, Elizabeth A; Forrest, Lisa J; Stepien, Rebecca L

2002-01-01

Thoracic radiographs of nine cats with confirmed bronchoalveolar carcinoma (BAC) were reviewed retrospectively. Radiographic appearance of BAC was divided into three categories: mixed bronchoalveolar pattern, ill-defined alveolar mass, or mass with cavitation. In addition to these radiographic signs, all nine cats had evidence of some form of bronchial disease. Cavitary lesions were the most common finding (n = 5). In addition, three cats in this category had diffuse bronchointerstitial opacity and one cat had focal peribronchial cuffing. Five cats had either a mixed bronchoalveolar pattern with bronchiectasis (n = 3) or an ill-defined alveolar mass with peribronchial cuffing (n = 2). One cat had both a mixed bronchoalveolar pattern and a cavitary mass. Each of these nine cats had some form of bronchial disease (bronchointerstitial pattern, peribronchial cuffing, or bronchiectasis), which aids in the radiographic diagnosis of bronchoalveolar carcinoma and may represent airway metastasis.

Rene Theophile Hyacinthe Laënnec (1781–1826): The Man Behind the Stethoscope

PubMed Central

Roguin, Ariel

2006-01-01

Rene Theophile Hyacinthe Laënnec (1781–1826) was a French physician who, in 1816, invented the stethoscope. Using this new instrument, he investigated the sounds made by the heart and lungs and determined that his diagnoses were supported by the observations made during autopsies. Laënnec later published the first seminal work on the use of listening to body sounds, De L’auscultation Mediate (On Mediate Auscultation). Laënnec is considered the father of clinical auscultation and wrote the first descriptions of bronchiectasis and cirrhosis and also classified pulmonary conditions such as pneumonia, bronchiectasis, pleurisy, emphysema, pneumothorax, phthisis and other lung diseases from the sounds he heard with his invention. Laënnec perfected the art of physical examination of the chest and introduced many clinical terms still used today. PMID:17048358

Multiple bronchoceles in a non-asthmatic patient with allergic bronchopulmonary aspergillosis.

PubMed

Amin, Muhammad Umar; Mahmood, Rabia

2008-09-01

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction due to a fungus, Aspergillus fumigatus. It is typically seen in patients with long-standing asthma. Our patient was a non-asthmatic 18 years old male who presented with chronic cough for 2 years. Peripheral blood eosinophilia and elevated scrum IgE were observed. His x-ray chest revealed v-shaped opacity in the left upper lobe close to the hilum. High resolution computed tomographic scan of the chest revealed multiple dilated bronchi filled with mucous (bronchoceles) and central bronchiectasis (CB) involving main segmental bronchi. Central bronchiectasis (CB) was typical of ABPA but bronchocele formation was a rare manifestation of the disease. The patient was managed with oral prednisolone and was relieved of his symptoms. Occurrence of ABPA in non-asthmatics is very rare and deserves reporting.

Molecular identification and genotyping of Pseudomonas aeruginosa isolated from cystic fibrosis and non-cystic fibrosis patients with bronchiectasis.

PubMed

Eusebio, Nadia; Amorim, Adelina A; Gamboa, Fernanda; Araujo, Ricardo

2015-03-01

There is no standard methodology for the molecular identification and genotyping of Pseudomonas aeruginosa which are frequently isolated in bronchiectasis patients. Hence, the main goal of this work was to propose a methodology capable to simultaneously identify and genotype, in less than 6 h, clinical P. aeruginosa collected from cystic fibrosis (CF) and non-CF patients with bronchiectasis. Molecular analyses were conducted in clinical isolates by testing the newly colony-PCR strategy and SNaPaer assay. A total of 207 isolates of P. aeruginosa were collected from clinical samples. To assess the assay specificity, other Gram-negative non-aeruginosa bacteria, namely Pseudomonas and Burkholderia, were tested. The complete group of 23 markers included in the SNaPaer panel was observed exclusively in P. aeruginosa; more than 18 markers failed in other bacteria. A total of 43 SnaP profiles were obtained for clinical P. aeruginosa, being the profiles highly patient-specific. Six CF patients were colonized with P. aeruginosa isolates with very distinct SnaP profiles, particularly following adjustments on antibiotic therapy, thus suggesting changes on the dynamics and dominance of these bacteria. SnaPaer proved to be a good and reliable tool for identification and genotyping of clinical P. aeruginosa in a single-tube multiplex PCR. Combined with the proposed colony-PCR strategy, SnaPaer assay facilitates the molecular analysis of P. aeruginosa. © FEMS 2014. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Mounier-Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases.

PubMed

Akgedik, Recep; Karamanli, Harun; Kizilirmak, Deniz; Kurt, Ali Bekir; Öztürk, Hasan; Yildirim, Berna Botan; Çakir, Lütfullah

2018-03-01

Mounier-Kuhn syndrome (MKS) is a congenital disorder characterized by tracheobronchomegaly resulting from the absence of elastic fibers in the trachea and main bronchi or atrophy and thinning of the smooth muscle layer. In this syndrome, dead space associated with tracheobronchomegaly increases and discharge of secretions decreases because of ineffective coughing. The most common complications are recurrent lower respiratory tract infections and bronchiectasis. We examined the clinical characteristics, radiological features, and related complications of patients with MKS. The cases were obtained between September 2007 and November 2015. Computed tomography scans of the chest were used to diagnose tracheobronchomegaly. All cases (a total of 11) were males with a mean age of 63 ± 13 (range, 38-80) years. The mean diameter of the trachea was 31.53 ± 2.99 mm; the mean transverse diameter was 31.69 ± 3.10 mm and the mean sagittal diameter was 31.36 ± 3.01 mm. Complaints at the time of presentation included chronic cough, purulent sputum, dyspnea, and hemoptysis. There were recurrent pulmonary infections in seven cases, bronchiectasis in six, and tracheal diverticulum in four at the time of diagnosis. In this article, 11 cases with various rarely seen complications are presented and evaluated in the light of current literature. We recommend that if chronic cough, recurrent pulmonary infections, and bronchiectasis seen in a patient, MKS should be kept in mind. © 2017 John Wiley & Sons Ltd.

Clubbing of the fingers or toes

MedlinePlus

... occur in people with bronchiectasis , cystic fibrosis , or lung abscess Infection of the lining of the heart chambers and heart valves (infectious endocarditis). This can be caused by ... substances Lung disorders in which the deep lung tissues become ...

Pathology of pulmonary aspergillomas.

PubMed

Shah, Rajeev; Vaideeswar, Pradeep; Pandit, Shobhana P

2008-01-01

Aspergilloma refers to a fungal ball formed by saprophytic overgrowth of Aspergillus species and is seen secondary to cavitatory/cystic respiratory diseases. Paucity of clinical and pathological data of aspergilloma in India prompted us to analyze cases of aspergilloma over 15 years. The clinical features were recorded in all and correlated with detailed pathological examination. Aspergillomas were identified in 41 surgical excisions or at autopsy. There was male predominance; half the patients were in their fourth decade. Episodic hemoptysis was the commonest mode of presentation (85.4%). Forty aspergillomas were complex, occurring in cavitatory lesions (82.9%) or in bronchiectasis (14.6%). Simple aspergilloma was seen as an incidental finding in only one. Tuberculosis was the etiological factor in 31 patients, producing cavitatory or bronchiectatic lesions; other causes were chronic lung abscess and bronchiectasis (unrelated to tuberculosis). Surgical resections are endorsed in view of high risk of unpredictable, life-threatening hemoptysis.

RESPIRE 1: a phase III placebo-controlled randomised trial of ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis.

PubMed

De Soyza, Anthony; Aksamit, Timothy; Bandel, Tiemo-Joerg; Criollo, Margarita; Elborn, J Stuart; Operschall, Elisabeth; Polverino, Eva; Roth, Katrin; Winthrop, Kevin L; Wilson, Robert

2018-01-01

We evaluated the efficacy and safety of ciprofloxacin dry powder for inhalation (DPI) in patients with non-cystic fibrosis bronchiectasis, two or more exacerbations in the previous year and pre-defined bacteria in sputum.In this phase III, double-blind, placebo-controlled trial, patients were randomised 2:1 to twice-daily ciprofloxacin DPI 32.5 mg or placebo in two treatment regimens consisting of on/off treatment cycles of 14 or 28 days for 48 weeks. The primary end-points were time to first exacerbation and frequency of exacerbations.A total of 416 patients were randomised to the 14-day on/off regimen (ciprofloxacin DPI (n=137) and placebo (n=68)) or the 28-day on/off regimen (ciprofloxacin DPI (n=141) and placebo (n=70)). Ciprofloxacin DPI 14 days on/off significantly prolonged time to first exacerbation versus pooled placebo (median time >336 versus 186 days; hazard ratio 0.53, 97.5% CI 0.36-0.80; p=0.0005) and reduced the frequency of exacerbations compared with matching placebo by 39% (mean number of exacerbations 0.6 versus 1.0; incidence rate ratio 0.61, 97.5% CI 0.40-0.91; p=0.0061). Outcomes for ciprofloxacin DPI 28 days on/off were not statistically significantly different from placebo. The safety profile of ciprofloxacin DPI was favourable.Ciprofloxacin DPI was well tolerated and has the potential to be an effective treatment option in non-cystic fibrosis bronchiectasis. Copyright ©ERS 2018.

Inhaled Antibiotics for Lower Airway Infections

PubMed Central

Quon, Bradley S.; Goss, Christopher H.

2014-01-01

Inhaled antibiotics have been used to treat chronic airway infections since the 1940s. The earliest experience with inhaled antibiotics involved aerosolizing antibiotics designed for parenteral administration. These formulations caused significant bronchial irritation due to added preservatives and nonphysiologic chemical composition. A major therapeutic advance took place in 1997, when tobramycin designed for inhalation was approved by the U.S. Food and Drug Administration (FDA) for use in patients with cystic fibrosis (CF) with chronic Pseudomonas aeruginosa infection. Attracted by the clinical benefits observed in CF and the availability of dry powder antibiotic formulations, there has been a growing interest in the use of inhaled antibiotics in other lower respiratory tract infections, such as non-CF bronchiectasis, ventilator-associated pneumonia, chronic obstructive pulmonary disease, mycobacterial disease, and in the post–lung transplant setting over the past decade. Antibiotics currently marketed for inhalation include nebulized and dry powder forms of tobramycin and colistin and nebulized aztreonam. Although both the U.S. Food and Drug Administration and European Medicines Agency have approved their use in CF, they have not been approved in other disease areas due to lack of supportive clinical trial evidence. Injectable formulations of gentamicin, tobramycin, amikacin, ceftazidime, and amphotericin are currently nebulized “off-label” to manage non-CF bronchiectasis, drug-resistant nontuberculous mycobacterial infections, ventilator-associated pneumonia, and post-transplant airway infections. Future inhaled antibiotic trials must focus on disease areas outside of CF with sample sizes large enough to evaluate clinically important endpoints such as exacerbations. Extrapolating from CF, the impact of eradicating organisms such as P. aeruginosa in non-CF bronchiectasis should also be evaluated. PMID:24673698

Inhaled antibiotics for lower airway infections.

PubMed

Quon, Bradley S; Goss, Christopher H; Ramsey, Bonnie W

2014-03-01

Inhaled antibiotics have been used to treat chronic airway infections since the 1940s. The earliest experience with inhaled antibiotics involved aerosolizing antibiotics designed for parenteral administration. These formulations caused significant bronchial irritation due to added preservatives and nonphysiologic chemical composition. A major therapeutic advance took place in 1997, when tobramycin designed for inhalation was approved by the U.S. Food and Drug Administration (FDA) for use in patients with cystic fibrosis (CF) with chronic Pseudomonas aeruginosa infection. Attracted by the clinical benefits observed in CF and the availability of dry powder antibiotic formulations, there has been a growing interest in the use of inhaled antibiotics in other lower respiratory tract infections, such as non-CF bronchiectasis, ventilator-associated pneumonia, chronic obstructive pulmonary disease, mycobacterial disease, and in the post-lung transplant setting over the past decade. Antibiotics currently marketed for inhalation include nebulized and dry powder forms of tobramycin and colistin and nebulized aztreonam. Although both the U.S. Food and Drug Administration and European Medicines Agency have approved their use in CF, they have not been approved in other disease areas due to lack of supportive clinical trial evidence. Injectable formulations of gentamicin, tobramycin, amikacin, ceftazidime, and amphotericin are currently nebulized "off-label" to manage non-CF bronchiectasis, drug-resistant nontuberculous mycobacterial infections, ventilator-associated pneumonia, and post-transplant airway infections. Future inhaled antibiotic trials must focus on disease areas outside of CF with sample sizes large enough to evaluate clinically important endpoints such as exacerbations. Extrapolating from CF, the impact of eradicating organisms such as P. aeruginosa in non-CF bronchiectasis should also be evaluated.

Review: Quality of Life in Children with Non-cystic Fibrosis Bronchiectasis.

PubMed

Nathan, Anna Marie; de Bruyne, Jessie Anne; Eg, Kah Peng; Thavagnanam, Surendran

2017-01-01

Non-cystic fibrosis bronchiectasis (NCFB) has gained renewed interest, due to its increasing health-care burden. Annual mortality statistics in England and Wales showed that under 1,000 people die from bronchiectasis each year, and this number is increasing by 3% yearly. Unfortunately, there is a severe lack of well-powered, randomized controlled trials to guide clinicians how to manage NCFB effectively. Quality-of-life (QOL) measures in NCFB are an important aspect of clinical care that has not been studied well. Commonly used disease-specific questionnaires in children with NCFB are the St George's Respiratory Questionnaire, Short Form-36, the Leicester Cough Questionnaire, and the Parent Cough-Specific Quality of Life questionnaire (PC-QOL). Of these, only the PC-QOL can be used in young children, as it is a parent-proxy questionnaire. We reviewed pediatric studies looking at QOL in children with NCFB and cystic fibrosis. All types of airway clearance techniques appear to be safe and have no significant benefit over each other. Number of exacerbations and hospitalizations correlated with QOL scores, while symptom subscales correlated with lung function, worse QOL, frequent antibiotic requirements, and duration of regular follow-up in only one study. There was a correlation between QOL and age of diagnosis in children with primary ciliary dyskinesia. Other studies have shown no relationship between QOL scores and etiology of NCFB as well as CT changes. As for treatments, oral azithromycin and yoga have demonstrated some improvement in QOL scores. In conclusion, more studies are required to accurately determine important factors contributing to QOL.

Bronchopulmonary Disease Caused by Flagellated Protozoa Infection in 15 Chinese Children.

PubMed

Liu, Jinrong; Li, Shaogang; Li, Huimin; Fan, Yimu; Yang, Haiming; Xu, Hui; Shen, Yuelin; Zhao, Shunying

2017-04-01

Bronchopulmonary disease caused by flagellated protozoa infection (BPFP) is thought to be rare in children but may be an emerging or underestimated disease, especially in developing countries. In this study, we retrospectively reviewed records of 15 patients who were presented with a cough, wheezing or bronchopulmonary disease of unknown causes during admission, and patients who were finally diagnosed with BPFP from January 2014 to January 2015 were enrolled. Protozoa were observed in bronchoalveolar lavage fluid by light microscopy. A total of 15 pediatric cases (11 male and 4 female individuals, from 1 year 8 months to 12 years 1 month of age) with flagellated protozoa infection diagnosed by light microscopy were recruited. The course of the disease at the time of diagnosis was from 10 days to 11 months. Patients presented with a fever (N = 9), cough (N = 11), wheezing (N = 5) and chest pain (N = 5). Laboratory data showed elevated peripheral blood leucocytes (N = 6), eosinophilic granulocytes (N = 3), C-reactive protein (N = 5) and immunoglobulin E (N = 3). Bronchoscopy revealed a mucus plug (N = 3) and bronchiectasis (N = 1). Lung computed tomography results indicated ground-glass opacification (N = 2), atelectasis (N = 3), bronchiectasis (N = 1), bronchial wall thickening (N = 3) or nodular opacity (N = 6, including 1 case of pulmonary embolism). All children responded to metronidazole for a 2- to 5-week treatment period. Patients with BPFP often have a chronic or recurrent course and present with recurrent fever, cough, wheezing and chest pain. Chest imaging may reveal ground-glass opacification, atelectasis, bronchiectasis or nodular opacity (including pulmonary embolism). BPFP responds favorably to metronidazole treatment.

Etiologies, Investigations and Outcomes of Patients Presenting With Hemoptysis

ClinicalTrials.gov

2016-09-22

Hemoptysis; Haemoptysis; Lung Disease; Pneumonia; Tuberculosis; Bronchiectasis; Respiratory Tract Infections; Respiratory Tract Diseases; Bronchitis; Mycobacterium Infections; Bronchial Disease; Pulmonary Hemorrhage; Signs and Symptoms; Signs and Symptoms, Respiratory; Pathologic Processes; Mycosis; Hemorrhage; Lung Cancer; Pulmonary Embolism; Arteriovenous Fistula

Macrolide Therapy in Adults and Children with Non-Cystic Fibrosis Bronchiectasis: A Systematic Review and Meta-Analysis

PubMed Central

Tang, Yan; Gao, Yang; Lin, Zhi-ya; Lin, Zhi-min; Zhong, Nan-shan; Chen, Rong-chang

2014-01-01

Background A systematic review and meta-analysis was conducted to evaluate the efficacy and safety of macrolide therapy in adults and children with bronchiectasis. Methods We searched the PUBMED, EMBASE, CENTRAL databases to identify relevant studies. Two reviewers evaluated the studies and extracted data independently. The primary outcome was the number of bronchiectasis exacerbations. Secondary outcomes included exacerbation-related admissions, quality of life (QoL), spirometry, 6-minute walk test (6MWT) and adverse events. Results Nine eligible trials with 559 participants were included. Six were conducted on adults, and the remaining on children. Macrolide therapy significantly reduced the number of patients experiencing one or more exacerbation in adults [risk ratio (RR) = 0.59; 95% CI, 0.40 to 0.86; P = 0.006; I2 = 65%] and children [RR = 0.86; 95% CI, 0.75–0.99; P = 0.04; I2 = 0%], but not the number of patients with admissions for exacerbation. Macrolide therapy was also associated with reduced frequency of exacerbations in adults (RR = 0.42; 95% CI, 0.29 to 0.61; P<0.001; I2 = 64%) and children (RR = 0.50; 95% CI, 0.35 to 0.71; P<0.001). Pooled analyses suggested that spirometry, including FEV1 and FVC, were significantly improved in adults but not in children. Macrolide therapy improved the QoL (WMD, −6.56; 95% CI, −11.99 to −1.12; P = 0.02; I2 = 86%) but no significant difference in 6MWT (WMD, 4.15; 95% CI, −11.83 to 20.13; P = 0.61; I2 = 31%) and the overall adverse events (RR, 0.96; 95% CI, 0.82 to 1.13; P = 0.66; I2 = 0%) in adults. However, reports of diarrhea and abdominal discomforts were higher with macrolide therapy. Conclusions Macrolide maintenance therapy, both in adults and children, was effective and safe in reducing bronchiectasis exacerbations, but not the admissions for exacerbations. In addition, macrolide administration in adults was associated with improvement in QoL and spirometry, but not 6WMT. Future studies are warranted to verify the optimal populations and clarify its potential effects on antimicrobial resistance. PMID:24603554

Anti-bacterial antibody and T cell responses in bronchiectasis are differentially associated with lung colonization and disease.

PubMed

Jaat, Fathia G; Hasan, Sajidah F; Perry, Audrey; Cookson, Sharon; Murali, Santosh; Perry, John D; Lanyon, Clare V; De Soyza, Anthony; Todryk, Stephen M

2018-05-30

As a way to determine markers of infection or disease informing disease management, and to reveal disease-associated immune mechanisms, this study sought to measure antibody and T cell responses against key lung pathogens and to relate these to patients' microbial colonization status, exacerbation history and lung function, in Bronchiectasis (BR) and Chronic Obstructive Pulmonary Disease (COPD). One hundred nineteen patients with stable BR, 58 with COPD and 28 healthy volunteers were recruited and spirometry was performed. Bacterial lysates were used to measure specific antibody responses by ELISA and T cells by ELIspot. Cytokine secretion by lysate-stimulated T cells was measured by multiplex cytokine assay whilst activation phenotype was measured by flow cytometry. Typical colonization profiles were observed in BR and COPD, dominated by P.aeruginosa, H.influenzae, S.pneumoniae and M.catarrhalis. Colonization frequency was greater in BR, showing association with increased antibody responses against P.aeruginosa compared to COPD and HV, and with sensitivity of 73% and specificity of 95%. Interferon-gamma T cell responses against P.aeruginosa and S.pneumoniae were reduced in BR and COPD, whilst reactive T cells in BR had similar markers of homing and senescence compared to healthy volunteers. Exacerbation frequency in BR was associated with increased antibodies against P. aeruginosa, M.catarrhalis and S.maltophilia. T cell responses against H.influenzae showed positive correlation with FEV 1 % (r = 0.201, p = 0.033) and negative correlation with Bronchiectasis Severity Index (r = - 0.287, p = 0.0035). Our findings suggest a difference in antibody and T cell immunity in BR, with antibody being a marker of exposure and disease in BR for P.aeruginosa, M.catarrhalis and H.influenzae, and T cells a marker of reduced disease for H.influenzae.

Development and validation of a clinical risk score for predicting drug-resistant bacterial pneumonia in older Chinese patients.

PubMed

Ma, Hon Ming; Ip, Margaret; Woo, Jean; Hui, David S C

2014-05-01

Health care-associated pneumonia (HCAP) and drug-resistant bacterial pneumonia may not share identical risk factors. We have shown that bronchiectasis, recent hospitalization and severe pneumonia (confusion, blood urea level, respiratory rate, low blood pressure and 65 year old (CURB-65) score ≥ 3) were independent predictors of pneumonia caused by potentially drug-resistant (PDR) pathogens. This study aimed to develop and validate a clinical risk score for predicting drug-resistant bacterial pneumonia in older patients. We derived a risk score by assigning a weighting to each of these risk factors as follows: 14, bronchiectasis; 5, recent hospitalization; 2, severe pneumonia. A 0.5 point was defined for the presence of other risk factors for HCAP. We compared the areas under the receiver-operating characteristics curve (AUROC) of our risk score and the HCAP definition in predicting PDR pathogens in two cohorts of older patients hospitalized with non-nosocomial pneumonia. The derivation and validation cohorts consisted of 354 and 96 patients with bacterial pneumonia, respectively. PDR pathogens were isolated in 48 and 21 patients in the derivation and validation cohorts, respectively. The AUROCs of our risk score and the HCAP definition were 0.751 and 0.650, respectively, in the derivation cohort, and were 0.782 and 0.671, respectively, in the validation cohort. The differences between our risk score and the HCAP definition reached statistical significance. A score ≥ 2.5 had the best balance between sensitivity and specificity. Our risk score outperformed the HCAP definition to predict pneumonia caused by PDR pathogens. A history of bronchiectasis or recent hospitalization is the major indication of starting empirical broad-spectrum antibiotics. © 2014 Asian Pacific Society of Respirology.

The effect of N-acetylcysteine on biofilms: Implications for the treatment of respiratory tract infections.

PubMed

Blasi, Francesco; Page, Clive; Rossolini, Gian Maria; Pallecchi, Lucia; Matera, Maria Gabriella; Rogliani, Paola; Cazzola, Mario

2016-08-01

In airway infections, biofilm formation has been demonstrated to be responsible for both acute and chronic events, and constitutes a genuine challenge in clinical practice. Difficulty in eradicating biofilms with systemic antibiotics has led clinicians to consider the possible role of non-antibiotic therapy. The aim of this review is to examine current evidence for the use of N-acetylcysteine (NAC) in the treatment of biofilm-related respiratory infections. Electronic searches of PUBMED up to September 2015 were conducted, searching for 'biofilm', 'respiratory tract infection', 'N-acetylcysteine', 'cystic fibrosis', 'COPD', 'bronchiectasis', 'otitis', and 'bronchitis' in titles and abstracts. Studies included for review were primarily in English, but a few in Italian were also selected. Biofilm formation may be involved in many infections, including ventilator-associated pneumonia, cystic fibrosis, bronchiectasis, bronchitis, and upper respiratory airway infections. Many in vitro studies have demonstrated that NAC is effective in inhibiting biofilm formation, disrupting preformed biofilms (both initial and mature), and reducing bacterial viability in biofilms. There are fewer clinical studies on the use of NAC in disruption of biofilm formation, although there is some evidence that NAC alone or in combination with antibiotics can decrease the risk of exacerbations of chronic bronchitis, chronic obstructive pulmonary disease, and rhinosinusitis. However, the usefulness of NAC in the treatment of cystic fibrosis and bronchiectasis is still matter of debate. Most of the studies published to date have used oral or intramuscular NAC formulations. Evidence from in vitro studies indicates that NAC has good antibacterial properties and the ability to interfere with biofilm formation and disrupt biofilms. Results from clinical studies have provided some encouraging findings that need to be confirmed and expanded using other routes of administration of NAC such as inhalation. Copyright © 2016 Elsevier Ltd. All rights reserved.

Can mean platelet volume and neutrophil-to-lymphocyte ratio be biomarkers of acute exacerbation of bronchiectasis in children?

PubMed Central

Erdem, Semiha Bahceci; Karaman, Sait; Yazici, Selcuk; Can, Demet

2017-01-01

Introduction Bronchiectasis (BE) is a parenchymal lung disease evolving as a result of recurrent lung infections and chronic inflammation. Although it has been shown in adult studies that mean platelet volume (MPV) and neutrophil-to-lymphocyte ratio (NLR) can be used as biomarkers of airway inflammation, knowledge is limited in the paediatric age group. The aim of our study is to investigate the potential of MPV and NLR as biomarkers that may indicate acute exacerbations of non-cystic fibrosis BE in children. Material and methods Children with non-cystic fibrosis BE (n = 50), who were followed in the division of Paediatric Pulmonology of our hospital between June 2010 and July 2015, were involved in the present retrospective cross-sectional study. Haemogram values during acute exacerbations and non-exacerbation periods, and a control group were compared. Results In children with bronchiectasis, the average leukocyte count (p < 0.001), platelet count (p = 0.018), absolute neutrophil count (p < 0.001), and NLR (p < 0.001) were higher, as expected, when compared with the control group. NLR values, in the period of acute exacerbation were significantly higher than the values of both the non-exacerbation periods (p = 0.02) and the control group (p < 0.001). In contrast, MPV values in the period of acute exacerbation did not exhibit a significant difference from those of non-exacerbation periods (p = 0.530) and the control group (p = 0.103). Conclusions It was concluded that leukocyte count, platelet count, absolute neutrophil count, and NLR can be used to show chronic inflammation in BE, but only NLR and absolute neutrophil count can be used as biomarkers to show acute exacerbations. PMID:29472813

Chronic respiratory disease in the regional and remote population of the Northern Territory Top End: A perspective from the specialist respiratory outreach service.

PubMed

Kruavit, Anuk; Fox, Melissa; Pearson, Rebecca; Heraganahally, Subash

2017-10-01

To study the demographic, rates and types of chronic respiratory conditions in Indigenous and non-Indigenous patients in regional and remote communities of Northern Territory Top End, who were referred to the specialist respiratory outreach service. A retrospective, observational study over a 2 years period. Remote community health clinics within the Northern Territory Top End visited by the specialist respiratory outreach team. Only patients referred to respiratory specialist outreach team were included. There were 444 participants, with 210 males and 234 females. In total, 79.3% were Indigenous. The rates of chronic respiratory disease diagnoses and smoking status within the different communities and health district regions. Chronic obstructive pulmonary disease (COPD) was the most common primary respiratory condition in our cohort of patients (50.5%) followed by sleep apnoea in (14.2%), bronchiectasis in (11.6%), asthma (11%), interstitial lung disease (2.5%), nodule/cancer (1.8%) sarcoidosis (0.2%) and others (9.2%). COPD, smoking status and bronchiectasis was more frequently noted among the Indigenous patients compared to non-Indigenous patients (56.3% versus 28.3%, P < 0.001), (52.6% versus 25.0%, P < 0.001), and (12.5% versus 3.3%, P = 0.01) respectively. Obstructive sleep apnoea was more commonly diagnosed in non-Indigenous patients. Indigenous patients of the remote and rural communities of the Top End have significantly higher rates of smoking and COPD compared to non-Indigenous patients. Bronchiectasis is also more common among Indigenous patients. Further efforts are warranted to develop strategies to address the disparity and optimise the respiratory outreach service to this population. © 2017 National Rural Health Alliance Inc.

Bronchoarterial ratio in never-smokers adults: Implications for bronchial dilation definition.

PubMed

Diaz, Alejandro A; Young, Thomas P; Maselli, Diego J; Martinez, Carlos H; Maclean, Erick S; Yen, Andrew; Dass, Chandra; Simpson, Scott A; Lynch, David A; Kinney, Gregory L; Hokanson, John E; Washko, George R; San José Estépar, Raul

2017-01-01

Bronchiectasis manifests as recurrent respiratory infections and reduced lung function. Airway dilation, which is measured as the ratio of the diameters of the bronchial lumen (B) and adjacent pulmonary artery (A), is a defining radiological feature of bronchiectasis. A challenge to equating the bronchoarterial (BA) ratio to disease severity is that the diameters of airway and vessel in health are not established. We sought to explore the variability of BA ratio in never-smokers without pulmonary disease and its associations with lung function. Objective measurements of the BA ratio on volumetric computed tomography (CT) scans and pulmonary function data were collected in 106 never-smokers. The BA ratio was measured in the right upper lobe apical bronchus (RB1) and the right lower lobe basal posterior bronchus. The association between the BA ratio and forced expiratory volume in 1 s (FEV 1 ) was assessed using regression analysis. The BA ratio was 0.79 ± 0.16 and was smaller in more peripheral RB1 bronchi (P < 0.0001). The BA ratio was >1, a typical threshold for bronchiectasis, in 10 (8.5%) subjects. Subjects with a BA ratio >1 versus ≤1 had smaller artery diameters (P < 0.0001) but not significantly larger bronchial lumens. After adjusting for age, gender, race and height, the BA ratio was directly related to FEV 1 (P = 0.0007). In never-smokers, the BA ratio varies by airway generation and is associated with lung function. A BA ratio >1 is driven by small arteries. Using artery diameter as reference to define bronchial dilation seems inappropriate. © 2016 Asian Pacific Society of Respirology.

Polymicrobial airway bacterial communities in adult bronchiectasis patients

PubMed Central

2014-01-01

Background Chronic airway infection contributes to the underlying pathogenesis of non-cystic fibrosis bronchiectasis (NCFBr). In contrast to other chronic airway infections, associated with COPD and CF bronchiectasis, where polymicrobial communities have been implicated in lung damage due to the vicious circle of recurrent bacterial infections and inflammation, there is sparse information on the composition of bacterial communities in NCFBr. Seventy consecutive patients were recruited from an outpatient adult NCFBr clinic. Bacterial communities in sputum samples were analysed by culture and pyrosequencing approaches. Bacterial sequences were analysed using partial least square discrimination analyses to investigate trends in community composition and identify those taxa that contribute most to community variation. Results The lower airway in NCFBr is dominated by three bacterial taxa Pasteurellaceae, Streptococcaceae and Pseudomonadaceae. Moreover, the bacterial community is much more diverse than indicated by culture and contains significant numbers of other genera including anaerobic Prevotellaceae, Veillonellaceae and Actinomycetaceae. We found particular taxa are correlated with different clinical states, 27 taxa were associated with acute exacerbations, whereas 11 taxa correlated with stable clinical states. We were unable to demonstrate a significant effect of antibiotic therapy, gender, or lung function on the diversity of the bacterial community. However, presence of clinically significant culturable taxa; particularly Pseudomonas aeruginosa and Haemophilus influenzae correlated with a significant change in the diversity of the bacterial community in the lung. Conclusions We have demonstrated that acute exacerbations, the frequency of exacerbation and episodes of clinical stability are correlated, in some patients, with a significantly different bacterial community structure, that are associated with a presence of particular taxa in the NCFBr lung. Moreover, there appears to be an inverse relationship between the abundance of P. aeruginosa and that of of H. influenzae within the NCFBr lung bacterial community. This interaction requires further exploration. PMID:24886473

[Pulmonary infections in patients with rheumatoid arthritis].

PubMed

Takayanagi, Noboru; Tsuchiya, Yutaka; Tokunaga, Daidou; Miyahara, Yousuke; Yamaguchi, Shouzaburo; Saito, Hiroo; Ubukata, Mikio; Kurashima, Kazuyoshi; Yanagisawa, Tsutomu; Sugita, Yutaka

2007-06-01

We studied 149 rheumatoid arthritis (RA) patients (mean age 68.0 years; 68 men, 81 women) with pulmonary infections. The mean age at the onset of RA and the duration of RA was 57.2 +/- 15.2 years and 10.9 +/- 11.5 years, respectively. Pulmonary infections included nontuberculous mycobacteriosis in 59 patients (Mycobacterium avium complex infection, 50 cases : Mycobacterium kansasii infection, 4 cases; others, 5 cases), pneumonia in 46 patients, pulmonary tuberculosis in 28 patients, pulmonary aspergillosis in 12 patients, pulmonary cryptococcosis in 5 patients, Pneumocystis jiroveci pneumonia in 5 patients, lung abscess in 9 patients, exacerbation of bronchiectasis in 7 patients, and empyema in 4 patients. One hundred percent of patients with exacerbation of bronchiectasis, 91.7% of patients with pulmonary aspergillosis, 87% of patients with pneumonia, and 81.4% of patients with nontuberculous mycobacteriosis had underlying lung diseases. The pulmonary infections during therapy with steroids were pulmonary tuberculosis (78.6%), pneumonia (65.2%), and pulmonary aspergillosis (58.3%), while the pulmonary infections during methotrexate treatment were Pneumocystis jiroveci pneumonia (80%), pulmonary cryptococcosis (40%), and pulmonary tuberculosis (28.6%). Pulmonary infections in RA patients who were taking TNFalpha inhibitors included 1 patient each with nontuberculous mycobacteriosis, pneumonia, pulmonary tuberculosis, and Pneumocystis jiroveci pneumonia. Among the RA patients with lung abscess, malignancy was noted in 55.6%, and diabetes mellitus in 22.2%. Pseudomonas aeruginosa was the second-most-common cause of pneumonia and cause of all exacerbations of bronchiectasis. As well as immunosuppressive medications (steroids, methotrexate, TNFalpha inhibitors) and systemic comorbid diseases, underlying lung diseases could be one of the risk factor for pulmonary infections in patients with RA. The dominant risk factor for each pulmonary infection in patients with RA might be different.

Evaluation of pulmonary disease using static lung volumes in primary ciliary dyskinesia.

PubMed

Pifferi, Massimo; Bush, Andrew; Pioggia, Giovanni; Caramella, Davide; Tartarisco, Gennaro; Di Cicco, Maria; Zangani, Marta; Chinellato, Iolanda; Maggi, Fabrizio; Tezza, Giovanna; Macchia, Pierantonio; Boner, Attilio

2012-11-01

In primary ciliary dyskinesia (PCD) lung damage is usually evaluated by high-resolution CT (HRCT). To evaluate whether HRCT abnormalities and Pseudomonas aeruginosa infection were better predicted by spirometry or plethysmography. A cross-sectional study performed in consecutive patients with PCD who underwent sputum culture, spirometry, plethysmography and HRCT within 48 h. Principal component analysis and soft computing were used for data evaluation. Fifty patients (26 children) were studied. P aeruginosa infection was found in 40% of the patients and bronchiectasis in 88%. There was a correlation between infection with P aeruginosa and extent of bronchiectasis (p=0.009; r =0.367) and air-trapping (p=0.03; r =0.315). Moreover, there was an association between infection with P aeruginosa and residual volume (RV) values >150% (p=0.04) and RV/total lung capacity (TLC) ratio >140% (p=0.001), but not between infection with P aeruginosa and forced expiratory volume in 1 s (FEV(1))<80%, or forced expiratory flow between 25% and 75% of forced vital capacity (FVC) (FEF(25-75%))<70% or FEV(1)/FVC<70% (<80% in children). Severity of the total lung impairment on chest HRCT directly correlated with RV when expressed as per cent predicted (p=0.003; r =0.423), and RV/TLC (p<0.001; r =0.513) or when expressed as z scores (p=0.002, r =0.451 and p<0.001, r =0.536 respectively). Principal component analysis on plethysmographic but not on spirometry data allowed recognition of different severities of focal air trapping, atelectasis and extent of bronchiectasis. Plethysmography better predicts HRCT abnormalities than spirometry. Whether it might be a useful test to define populations of patients with PCD who should or should not have HRCT scans requires further longitudinal studies.

Effects of periodontal treatment on exacerbation frequency and lung function in patients with chronic periodontitis: study protocol of a 1-year randomized controlled trial.

PubMed

Romero, Sergio dos Santos; Pinto, Erika Horácio; Longo, Priscila Larcher; Dal Corso, Simone; Lanza, Fernanda Cordoba; Stelmach, Rafael; Rached, Samia Zahi; Lino-Dos-Santos-Franco, Adriana; Mayer, Marcia Pinto Alves; Bussadori, Sandra Kalil; Fernandes, Kristianne Porta Santos; Mesquita-Ferrari, Raquel Agnelli; Horliana, Anna Carolina Ratto Tempestini

2017-01-23

Chronic obstructive pulmonary disease (COPD) has been associated with periodontal disease (PD), and periodontal treatment (PT) has been connected to reduction of lung disease exacerbations. Bronchiectasis has many clinical similarities with COPD but, although it is also a chronic lung disease, to date it has not been studied with relation to PD. The aim of this study is to evaluate whether PT associated with photodynamic therapy (PDT) reduces the number of exacerbations, improves pulmonary function, periodontal clinical parameters and quality of life after 1 year of periodontal treatment follow-up. Bronchiectasis patients will undergo medical anamnesis and periodontal examination. Participants with periodontitis will be divided into two groups and PT will be performed as G1 control group (n = 32) - OHO (oral hygiene orientation) + supragingival treatment + simulation of using photodynamic therapy (PDT); G2 experimental (n = 32) - scaling and root planing + PDT + OHO. Lung function will be assessed both at baseline and after 1 year by spirometry, exacerbation history will be analyzed through clinical records monitoring. Three instruments for quality of life assessment will also be applied - Saint George's Respiratory Questionnaire and Impact Profile Analysis Oral health (OHIP-14). It is expected that periodontal treatment can improve the analyzed parameters after 1 year. Although only one study evaluates exacerbation in COPD after 1 year of PT, bronchiectasis has not been studied in the dentistry field to date. NCT02514226. Version #1. This study protocol receives grant from FAPESP (São Paulo Research Foundation) #2015/20535-1. First received: July 22, 2015, 1 st version. This protocol has been approved by the Research Ethics Committee of Nove de Julho University.

Eight-weekly intravenous antibiotics is beneficial in severe bronchiectasis.

PubMed

Mandal, P; Sidhu, M K; Donaldson, L S; Chalmers, J D; Smith, M P; Turnbull, K; Scott, J; Hill, A T

2013-01-01

The aim of our study was to assess the impact of 8-weekly intravenous (IV) antibiotics on exacerbation frequency and health-related quality of life in bronchiectasis. Patients were recruited prospectively from June 2008 to December 2010. Patients with recurrent exacerbations (five or more exacerbations per year) and subjectively reporting ill health between antibiotic courses were recruited. Eight-weekly IV antibiotics (for 14 days) were initiated. Patients were followed up for 1 year. Main outcome was reduction in exacerbation frequency and improvement in health-related quality of life (HRQoL) at 1 year after starting intravenous antibiotic therapy. Other outcomes recorded were forced expiratory volume in 1 s (FEV(1)), forced vital capacity (FVC), incremental shuttle walk test (ISWT), 24-h sputum volume, sputum microbiology, body mass index (BMI), markers of inflammation--white cell count (WCC), C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). In total, 19 patients were recruited. Mean age was 64.1 years and 52.6% were female. With 8-weekly antibiotics, there was a significant reduction in the number of exacerbations [mean (SE): 9.3 (0.5) in the year before vs. 8.0 (0.4) in the year after; P = 0.02]. In 63.2%, Leicester Cough Questionnaire (LCQ) improved by ≥1.3 U (P = 0.006)] and in 42.1% St. George's Respiratory Questionnaire (SGRQ) improved by ≥4 U (P = 0.03). Exercise capacity increased by 58.7 m (P = 0.004). There was no improvement in the other end points. Treatment with 8-weekly intravenous antibiotics in severe bronchiectasis reduced exacerbation frequency and improved exercise tolerance and health-related quality of life.

Effects of long-term azithromycin therapy on airway oxidative stress markers in non-cystic fibrosis bronchiectasis.

PubMed

Diego, Afredo De; Milara, Javier; Martinez-Moragón, Eva; Palop, Marta; León, Montse; Cortijo, Julio

2013-10-01

To explore the effect of long-term therapy with azithromycin in regards to airway oxidative stress markers in exhaled breath condensate (EBC) of adult patients with stable non-cystic fibrosis (CF) bronchiectasis. Open-label prospective study of 30 patients randomized to azithromycin 250 mg three times per week during 3 months (16 patients) or control (14 patients). Primary outcome were changes in nitric oxide, 8-isoprostane, pH, nitrites and nitrates in EBC. Secondary outcomes were changes in exacerbation rates, dyspnoea (Borg scale), sputum volume (cc), sputum colour (15-point scale), bacterial infection, health-related quality of life (St George's Respiratory Questionnaire), lung function and radiological extension. Azithromycin produced a significant decrease in sputum volume (8.9 (1.8) mL vs 2.1 (3.4) mL) and number of exacerbations (0.1 (0.6) vs 1.2 (0.9)). Dyspnoea (0.4 (0.1) vs 0.1 (0.2)) and health-related quality of life also improved after therapy. However, oxidative stress markers in EBC, systemic inflammatory markers as well as functional respiratory tests did not differ from the control group after therapy. A post-hoc analysis comparing patients infected or not with Pseudomonas aeruginosa revealed that these effects were more pronounced in infected patients. In this subgroup, treatment was followed by a significant reduction in sputum volume, number of exacerbations, dyspnoea and St George's Respiratory Questionnaire total score. Of all airway oxidative stress markers, only nitrates in EBC were reduced after therapy. Long-term azythromicin treatment has some clinical benefits in patients with non-CF stable bronchiectasis, but it does not affect airway oxidative stress markers. © 2013 The Authors. Respirology © 2013 Asian Pacific Society of Respirology.

A Randomized Controlled Trial of Atorvastatin in Patients With Bronchiectasis Infected With Pseudomonas Aeruginosa: A Proof of Concept Study.

PubMed

Bedi, Pallavi; Chalmers, James D; Graham, Catriona; Clarke, Andrea; Donaldson, Samantha; Doherty, Catherine; Govan, John R W; Davidson, Donald J; Rossi, Adriano G; Hill, Adam T

2017-08-01

There are no randomized controlled trials of statin therapy in patients with severe bronchiectasis who are chronically infected with Pseudomonas aeruginosa. Thirty-two patients chronically infected with P aeruginosa were recruited in this double-blind cross-over randomized controlled trial. Sixteen patients were recruited in each arm, were given atorvastatin 80 mg or placebo for 3 months followed by a washout period for 6 weeks, and then crossed over and administered the alternative therapy for 3 months. Twenty-seven patients completed the study. Atorvastatin did not significantly improve the primary end point of cough as measured by the Leicester Cough Questionnaire (mean difference, 1.92; 95% CI for difference, -0.57-4.41; P = .12). However, atorvastatin treatment resulted in an improved St. Georges Respiratory Questionnaire (-5.62 points; P = .016) and reduced serum levels of CXCL8 (P = .04), tumor necrosis factor (P = .01), and intercellular adhesion molecule 1 (P = .04). There was a trend toward improvement in serum C-reactive protein and serum neutrophil counts (P = .07 and P = .06, respectively). We demonstrated in vitro that atorvastatin 10 μM reduced formyl-methionyl-leucyl phenylalanine-induced upregulation of CD11b expression and changes in calcium flux, reflecting an ability to decrease neutrophil activation. We demonstrated that atorvastatin reduced systemic inflammation and improved quality of life in patients with bronchiectasis who were infected with P aeruginosa. These effects may be due to an ability of atorvastatin to modulate neutrophil activation. ClinicalTrials.gov; No.: NCT01299194; URL: www.clinicaltrials.gov. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

In vivo study of indomethacin in bronchiectasis: effect on neutrophil function and lung secretion.

PubMed

Llewellyn-Jones, C G; Johnson, M M; Mitchell, J L; Pye, A; Okafor, V C; Hill, S L; Stockley, R A

1995-09-01

Bronchiectasis is associated with sputum containing high levels of the proteolytic enzyme elastase, which is thought to be involved in the pathogenesis of the disease. Agents which inhibit neutrophil function and interfere with neutrophil elastase release may have a beneficial effect on the development and progression of such diseases. We have studied the effects of the nonsteroidal anti-inflammatory agent indomethacin on neutrophil function in nine patients with clinically stable bronchiectasis. All patients remained clinically stable during the study. We observed a significant reduction in peripheral neutrophil chemotaxis to 10 nmol.L-1 N-formyl-methionyl-leucyl-phenylalanine (FMLP) from a mean of 19.86 (SEM 1.35) to 8.46 (0.68) cells.field-1 after 4 weeks of therapy. There was also a significant reduction in fibronectin degradation both by resting and FMLP-stimulated neutrophils, from a mean of 1.90 (0.19) micrograms x 3 x 10(5) cells at the start of therapy to 0.87 (0.08) micrograms after 4 weeks, and from 3.17 (0.35) micrograms to 1.48 (0.05) micrograms, respectively. There was no effect on spontaneous or stimulated superoxide anion generation by neutrophils. Despite the marked changes in peripheral neutrophil function, no adverse effect was observed on viable bacterial load in the bronchial secretions. In addition, there was no difference in sputum albumin, elastase or myeloperoxidase levels, and only minor changes in the chemotactic activity of the sputum. These results suggest that nonsteroidal anti-inflammatory agents have a major effect on peripheral neutrophil function but do not appear to have an adverse effect on bacterial colonization of the airways.

The provision of generalist and specialist palliative care for patients with non-malignant respiratory disease in the North and Republic of Ireland: a qualitative study.

PubMed

Veigh, Clare Mc; Reid, Joanne; Larkin, Philip; Porter, Sam; Hudson, Peter

2017-07-11

Previous research and key guidelines have suggested potential models of palliative care for patients with COPD and interstitial lung disease. However, these recommendations are often not effectively implemented in clinical practice and are void of guidance regarding palliative care for patients with bronchiectasis, another form of non-malignant respiratory disease. The aim of this research was to explore generalist and specialist palliative care service provision for people with non-malignant respiratory disease in the North and Republic of Ireland. Qualitative study involving a convenience sample of 17 bereaved carers and 18 healthcare professionals recruited from 2 rural and 2 urban sites on the Island of Ireland. Data collection consisted of semi-structured interviews with carers of patients with COPD, interstitial lung disease or bronchiectasis who had died 3-18 months previously; and 4 focus groups with healthcare professionals. Data analysed using thematic analysis. Findings highlighted the lack of a clear model of holistic care delivery for patients with non-malignant respiratory disease and illuminated the varying levels of palliative care provision this client group experienced. Additionally, ambiguity amongst healthcare professionals regarding prognostication illuminated the importance of the provision of palliative care being based on patient need, not prognosis. This research developed a potential model of palliative care which may help healthcare professionals introduce palliative care, and specialist respiratory care, early in the disease trajectory of non-malignant respiratory disease, whilst also encouraging the involvement of specialist palliative care for complex symptom management. This research provides an important insight into a potential model of palliative care for people with non-malignant respiratory disease, inclusive of bronchiectasis. However, the feasibility of integrating this model into clinical practice requires further exploration.

Macrolide antibiotics for bronchiectasis.

PubMed

Kelly, Carol; Chalmers, James D; Crossingham, Iain; Relph, Nicola; Felix, Lambert M; Evans, David J; Milan, Stephen J; Spencer, Sally

2018-03-15

Bronchiectasis is a chronic respiratory disease characterised by abnormal and irreversible dilatation and distortion of the smaller airways. Bacterial colonisation of the damaged airways leads to chronic cough and sputum production, often with breathlessness and further structural damage to the airways. Long-term macrolide antibiotic therapy may suppress bacterial infection and reduce inflammation, leading to fewer exacerbations, fewer symptoms, improved lung function, and improved quality of life. Further evidence is required on the efficacy of macrolides in terms of specific bacterial eradication and the extent of antibiotic resistance. To determine the impact of macrolide antibiotics in the treatment of adults and children with bronchiectasis. We identified trials from the Cochrane Airways Trials Register, which contains studies identified through multiple electronic searches and handsearches of other sources. We also searched trial registries and reference lists of primary studies. We conducted all searches on 18 January 2018. We included randomised controlled trials (RCTs) of at least four weeks' duration that compared macrolide antibiotics with placebo or no intervention for the long-term management of stable bronchiectasis in adults or children with a diagnosis of bronchiectasis by bronchography, plain film chest radiograph, or high-resolution computed tomography. We excluded studies in which participants had received continuous or high-dose antibiotics immediately before enrolment or before a diagnosis of cystic fibrosis, sarcoidosis, or allergic bronchopulmonary aspergillosis. Our primary outcomes were exacerbation, hospitalisation, and serious adverse events. Two review authors independently screened the titles and abstracts of 103 records. We independently screened the full text of 40 study reports and included 15 trials from 30 reports. Two review authors independently extracted outcome data and assessed risk of bias for each study. We analysed dichotomous data as odds ratios (ORs) and continuous data as mean differences (MDs) or standardised mean differences (SMDs). We used standard methodological procedures as expected by Cochrane. We included 14 parallel-group RCTs and one cross-over RCT with interventions lasting from 8 weeks to 24 months. Of 11 adult studies with 690 participants, six used azithromycin, four roxithromycin, and one erythromycin. Four studies with 190 children used either azithromycin, clarithromycin, erythromycin, or roxithromycin.We included nine adult studies in our comparison between macrolides and placebo and two in our comparison with no intervention. We included one study with children in our comparison between macrolides and placebo and one in our comparison with no intervention.In adults, macrolides reduced exacerbation frequency to a greater extent than placebo (OR 0.34, 95% confidence interval (CI) 0.22 to 0.54; 341 participants; three studies; I 2 = 65%; moderate-quality evidence). This translates to a number needed to treat for an additional beneficial outcome of 4 (95% CI 3 to 8). Data show no differences in exacerbation frequency between use of macrolides (OR 0.31, 95% CI 0.08 to 1.15; 43 participants; one study; moderate-quality evidence) and no intervention. Macrolides were also associated with a significantly better quality of life compared with placebo (MD -8.90, 95% CI -13.13 to -4.67; 68 participants; one study; moderate-quality evidence). We found no evidence of a reduction in hospitalisations (OR 0.56, 95% CI 0.19 to 1.62; 151 participants; two studies; I 2 = 0%; low-quality evidence), in the number of participants with serious adverse events, including pneumonia, respiratory and non-respiratory infections, haemoptysis, and gastroenteritis (OR 0.49, 95% CI 0.20 to 1.23; 326 participants; three studies; I 2 = 0%; low-quality evidence), or in the number experiencing adverse events (OR 0.83, 95% CI 0.51 to 1.35; 435 participants; five studies; I 2 = 28%) in adults with macrolides compared with placebo.In children, there were no differences in exacerbation frequency (OR 0.40, 95% CI 0.11 to 1.41; 89 children; one study; low-quality evidence); hospitalisations (OR 0.28, 95% CI 0.07 to 1.11; 89 children; one study; low-quality evidence), serious adverse events, defined within the study as exacerbations of bronchiectasis or investigations related to bronchiectasis (OR 0.43, 95% CI 0.17 to 1.05; 89 children; one study; low-quality evidence), or adverse events (OR 0.78, 95% CI 0.33 to 1.83; 89 children; one study), in those receiving macrolides compared to placebo. The same study reported an increase in macrolide-resistant bacteria (OR 7.13, 95% CI 2.13 to 23.79; 89 children; one study), an increase in resistance to Streptococcus pneumoniae (OR 13.20, 95% CI 1.61 to 108.19; 89 children; one study), and an increase in resistance to Staphylococcus aureus (OR 4.16, 95% CI 1.06 to 16.32; 89 children; one study) with macrolides compared with placebo. Quality of life was not reported in the studies with children. Long-term macrolide therapy may reduce the frequency of exacerbations and improve quality of life, although supporting evidence is derived mainly from studies of azithromycin, rather than other macrolides, and predominantly among adults rather than children. However, macrolides should be used with caution, as limited data indicate an associated increase in microbial resistance. Macrolides are associated with increased risk of cardiovascular death and other serious adverse events in other populations, and available data cannot exclude a similar risk among patients with bronchiectasis.

Developing Optimal Parameters for Hyperpolarized Noble Gas and Inert Fluorinated Gas MRI of Lung Disorders

ClinicalTrials.gov

2018-06-21

Lung Transplant; Lung Resection; Lung Cancer; Asthma; Cystic Fibrosis; Chronic Obstructive Pulmonary Disease; Emphysema; Mesothelioma; Asbestosis; Pulmonary Embolism; Interstitial Lung Disease; Pulmonary Fibrosis; Bronchiectasis; Seasonal Allergies; Cold Virus; Lung Infection; Pulmonary Hypertension; Pulmonary Dysplasia; Obstructive Sleep Apnea

Pulmonary infection caused by Mycobacterium kansasii: findings on computed tomography of the chest*

PubMed Central

Mogami, Roberto; Goldenberg, Telma; de Marca, Patricia Gomes Cytrangulo; Mello, Fernanda Carvalho de Queiroz; Lopes, Agnaldo José

2016-01-01

Objective To describe the main tomography findings in patients diagnosed with pulmonary infection caused by Mycobacterium kansasii. Materials and Methods Retrospective study of computed tomography scans of 19 patients with pulmonary infection by M. kansasii. Results Of the 19 patients evaluated, 10 (52.6%) were male and 9 (47.4%) were female. The mean age of the patients was 58 years (range, 33-76 years). Computed tomography findings were as follows: architectural distortion, in 17 patients (89.5%); reticular opacities and bronchiectasis, in 16 (84.2%); cavities, in 14 (73.7%); centrilobular nodules, in 13 (68.4%); small consolidations, in 10 (52.6%); atelectasis and large consolidations, in 9 (47.4%); subpleural blebs and emphysema, in 6 (31.6%); and adenopathy, in 1 (5.3%). Conclusion There was a predominance of cavities, as well as of involvement of the small and large airways. The airway disease was characterized by bronchiectasis and bronchiolitis presenting as centrilobular nodules. PMID:27777472

Inhaled antibiotics in non-cystic fibrosis bronchiectasis: A meta-analysis.

PubMed

Xu, Li; Zhang, Fei; Du, Shuai; Yu, Qi; Chen, Lin; Long, Li-Hui; Li, Ya-Ming; Jia, Ai-Hua

2016-09-01

To evaluate the efficacy and safety of inhaled antibiotics for the treatment of non-cystic fibrosis bronchiectasis (NCFB). Pubmed, Cochrane library, Embase, Elsevier, OVID, Springerlink, Web of knowledge and NEJM were searched for randomized controlled trials (RCTs) on inhaled antibiotics in treatment of NCFB from inception until April 2015. Meta-analysis was conducted to assess the efficacy and safety of inhaled antibiotics in the treatment of NCFB. Twelve RCTs involving 1154 participants were included. They showed that inhaled antibiotics were more effective in reduction of sputum bacterial density, eradication of P. aeruginosa, prolonged time to exacerbation and reduction of new pathogens emergence with no significant difference in adverse events compared with control groups. However, we did not find significant benefits of inhaled antibiotics in reducing the risk of acute exacerbation, improving health-related quality of life and reduction of P. aeruginosa resistance. Moreover, inhaled antibiotics exerted a statistically significant reduction in FEV1%. Inhaled antibiotics may be an alternative pathway to inhibit airway inflammation with no more adverse events in patients with NCFB.

[Specific aspects and care of lung involvement in adults with cystic fibrosis].

PubMed

Pin, I; Grenet, D; Scheid, P; Domblides, P; Stern, M; Hubert, D

2000-08-01

Respiratory impairment is present in almost all adult cystic fibrosis patients and makes the prognosis. Viscous, infected and abundant secretions, inflammation and bronchial oedema, bronchoconstriction and respiratory muscle fatigue lead to airway obstruction, bronchiectasis and respiratory failure. The disease is preferentially located in the upper lobes. Exacerbations of the disease are due to bronchial infections and are often responsible for drops of the respiratory function. Regular spirometric surveillance is fundamental for the prognosis and the assessment of the effects of the treatment. Among adult patients chronic colonisation with mucoid and often multiresistant strains of Pseudomonas Aeruginosa are common. It is treated with i.v. high doses antibiotic courses and nebulized antibiotics between i.v. courses. Respiratory failure may require long term oxygen and non invasive mechanical ventilation. Systemic hypervascularization around the bronchiectasis may lead to moderate to severe hemoptysis, which may require embolization. Pneumothorax are associated with poor prognosis and are treated by pleural drainage and if failure by thoracoscopy.

Azithromycin for prevention of exacerbations in non-cystic fibrosis bronchiectasis (EMBRACE): a randomised, double-blind, placebo-controlled trial.

PubMed

Wong, Conroy; Jayaram, Lata; Karalus, Noel; Eaton, Tam; Tong, Cecilia; Hockey, Hans; Milne, David; Fergusson, Wendy; Tuffery, Christine; Sexton, Paul; Storey, Louanne; Ashton, Toni

2012-08-18

Azithromycin is a macrolide antibiotic with anti-inflammatory and immunomodulatory properties. We tested the hypothesis that azithromycin would decrease the frequency of exacerbations, increase lung function, and improve health-related quality of life in patients with non-cystic fibrosis bronchiectasis. We undertook a randomised, double-blind, placebo-controlled trial at three centres in New Zealand. Between Feb 12, 2008, and Oct 15, 2009, we enrolled patients who were 18 years or older, had had at least one pulmonary exacerbation requiring antibiotic treatment in the past year, and had a diagnosis of bronchiectasis defined by high-resolution CT scan. We randomly assigned patients to receive 500 mg azithromycin or placebo three times a week for 6 months in a 1:1 ratio, with a permuted block size of six and sequential assignment stratified by centre. Participants, research assistants, and investigators were masked to treatment allocation. The coprimary endpoints were rate of event-based exacerbations in the 6-month treatment period, change in forced expiratory volume in 1 s (FEV(1)) before bronchodilation, and change in total score on St George's respiratory questionnaire (SGRQ). Analyses were by intention to treat. This study is registered with the Australian New Zealand Clinical Trials Registry, number ACTRN12607000641493. 71 patients were in the azithromycin group and 70 in the placebo group. The rate of event-based exacerbations was 0·59 per patient in the azithromycin group and 1·57 per patient in the placebo group in the 6-month treatment period (rate ratio 0·38, 95% CI 0·26-0·54; p<0·0001). Prebronchodilator FEV(1) did not change from baseline in the azithromycin group and decreased by 0·04 L in the placebo group, but the difference was not significant (0·04 L, 95% CI -0·03 to 0·12; p=0·251). Additionally, change in SGRQ total score did not differ between the azithromycin (-5·17 units) and placebo groups (-1·92 units; difference -3·25, 95% CI -7·21 to 0·72; p=0·108). Azithromycin is a new option for prevention of exacerbations in patients with non-cystic fibrosis bronchiectasis with a history of at least one exacerbation in the past year. Health Research Council of New Zealand and Auckland District Health Board Charitable Trust. Copyright © 2012 Elsevier Ltd. All rights reserved.

Comparing accelerometer, pedometer and a questionnaire for measuring physical activity in bronchiectasis: a validity and feasibility study?

PubMed

O'Neill, B; McDonough, S M; Wilson, J J; Bradbury, I; Hayes, K; Kirk, A; Kent, L; Cosgrove, D; Bradley, J M; Tully, M A

2017-01-14

There are challenges for researchers and clinicians to select the most appropriate physical activity tool, and a balance between precision and feasibility is needed. Currently it is unclear which physical activity tool should be used to assess physical activity in Bronchiectasis. The aim of this research is to compare assessment methods (pedometer and IPAQ) to our criterion method (ActiGraph) for the measurement of physical activity dimensions in Bronchiectasis (BE), and to assess their feasibility and acceptability. Patients in this analysis were enrolled in a cross-sectional study. The ActiGraph and pedometer were worn for seven consecutive days and the IPAQ was completed for the same period. Statistical analyses were performed using SPSS 20 (IBM). Descriptive statistics were used; the percentage agreement between ActiGraph and the other measures were calculated using limits of agreement. Feedback about the feasibility of the activity monitors and the IPAQ was obtained. There were 55 (22 male) data sets available. For step count there was no significant difference between the ActiGraph and Pedometer, however, total physical activity time (mins) as recorded by the ActiGraph was significantly higher than the pedometer (mean ± SD, 232 (75) vs. 63 (32)). Levels of agreement between the two devices was very good for step count (97% agreement); and variation in the levels of agreement were within accepted limits of ±2 standard deviations from the mean value. IPAQ reported more bouted- moderate - vigorous physical activity (MVPA) [mean, SD; 167(170) vs 6(9) mins/day], and significantly less sedentary time than ActiGraph [mean, SD; 362(115) vs 634(76) vmins/day]. There were low levels of agreement between the two tools (57% sedentary behaviour; 0% MVPA 10+ ), with IPAQ under-reporting sedentary behaviour and over-reporting MVPA 10+ compared to ActiGraph. The monitors were found to be feasible and acceptable by participants and researchers; while the IPAQ was accepta ble to use, most patients required assistance to complete it. Accurate measurement of physical activity is feasible in BE and will be valuable for future trials of therapeutic interventions. ActiGraph or pedometer could be used to measure simple daily step counts, but ActiGraph was superior as it measured intensity of physical activity and was a more precise measure of time spent walking. The IPAQ does not appear to represent an accurate measure of physical activity in this population. Clinical Trials Registration Number NCT01569009 : Physical Activity in Bronchiectasis.

Volumetric capnography for the evaluation of pulmonary disease in adult patients with cystic fibrosis and noncystic fibrosis bronchiectasis.

PubMed

Veronez, L; Moreira, M M; Soares, S T P; Pereira, M C; Ribeiro, M A G O; Ribeiro, J D; Terzi, R G G; Martins, L C; Paschoal, I A

2010-06-01

This study was designed to use volumetric capnography to evaluate the breathing pattern and ventilation inhomogeneities in patients with chronic sputum production and bronchiectasis and to correlate the phase 3 slope of the capnographic curve to spirometric measurements. Twenty-four patients with cystic fibrosis (CF) and 21 patients with noncystic fibrosis idiopathic bronchiectasis (BC) were serially enrolled. The diagnosis of cystic fibrosis was based on the finding of at least two abnormal sweat chloride concentrations (iontophoresis sweat test). The diagnosis of bronchiectasis was made when the patient had a complaint of chronic sputum production and compatible findings at high-resolution computed tomography (HRCT) scan of the thorax. Spirometric tests and volumetric capnography were performed. The 114 subjects of the control group for capnographic variables were nonsmoker volunteers, who had no respiratory symptoms whatsoever and no past or present history of lung disease. Compared with controls, patients in CF group had lower SpO(2) (P < 0.0001), higher respiratory rates (RR) (P < 0.0001), smaller expiratory volumes normalized for weight (V(E)/kg) (P < 0.028), smaller expiratory times (Te) (P < 0.0001), and greater phase 3 Slopes normalized for tidal volume (P3Slp/V(E)) (P < 0.0001). Compared with controls, patients in the BC group had lower SpO(2) (P < 0.0001), higher RR (P < 0.004), smaller V(E)/kg (P < 0.04), smaller Te (P < 0.007), greater P3Slp/V(E) (P < 0.0001), and smaller VCO(2) (P < 0.0002). The pooled data from the two patient groups compared with controls showed that the patients had lower SpO(2) (P < 0.0001), higher RR (P < 0.0001), smaller V(E)/kg (P < 0.05), smaller Te (P < 0.0001), greater P3Slp/V(E) (P < 0.0001), and smaller VCO(2) (P < 0.0003). All of the capnographic and spirometric variables evaluated showed no significant differences between CF and BC patients. Spirometric data in this study reveals that the patients had obstructive defects with concomitant low vital capacities and both groups had very similar abnormalities. The capnographic variables in the patient group suggest a restrictive respiratory pattern (greater respiratory rates, smaller expiratory times and expiratory volumes, normal peak expiratory flows). Both groups of patients showed increased phase III slopes compared with controls, which probably indicates the presence of diffuse disease of small airways in both conditions leading to inhomogeneities of ventilation.

Adenocarcinoma of the Cervix Uteri and Endometrium Combined With the Kartagener Syndrome on FDG PET/CT.

PubMed

Zhang, Yin; Chen, Yue; Huang, Zhanwen; Zhou, Fan

2015-11-01

A 45-year-old woman with pathologically confirmed adenocarcinoma of the cervix uteri and endometrium underwent FDG PET/CT for staging. No metastasis was found. However, the images revealed bronchiectasis, sinusitis, and situs inversus totalis, which are the triad of Kartagener syndrome.

[Pulmonary non invasive infection by Scedosporium apiospermum].

PubMed

Cruz, Rodrigo; Barros, Manuel; Reyes, Mirtha

2015-08-01

We reported a case of non-invasive pulmonary infection by Scedosporium apiospermum in 67 years old female with bronchiectasis and caverns secondary to tuberculosis. Diagnosis was made with lung CT and bronchial lavage cultures. The patient was initially treated with itraconazole for six weeks without success and then voriconazole for 16 weeks, with good clinical response.

Serum antibodies to outer membrane proteins (OMPs) of Moraxella (Branhamella) catarrhalis in patients with bronchiectasis: identification of OMP B1 as an important antigen.

PubMed Central

Sethi, S; Hill, S L; Murphy, T F

1995-01-01

Moraxella (Branhamella) catarrhalis is a common cause of lower respiratory tract infections in adults and of otitis media in children. Little is known about the human immune response to this bacterium. In this study, immunoblot assays were performed to detect serum immunoglobulin G antibodies directed at purified outer membrane of M. catarrhalis. Twelve serum samples, two each from six patients with bronchiectasis who were persistently colonized with this organism, were tested with their homologous M. catarrhalis sputum isolates. In all the sera, the most prominent and consistent antibody response was to a minor 84-kDa outer membrane protein, OMP B1. Immunoblot adsorption assays show that these antibodies recognize surface exposed epitopes on OMP B1. Further analysis of human serum antibodies eluted from the surface of intact bacterial cells shows that these surface-exposed epitopes on OMP B1 are heterogeneous among strains of M. catarrhalis. OMP B1 is therefore an important OMP antigen on the surface of M. catarrhalis for the human immune response to infection by this bacterium. PMID:7890418

Inhaled Antibiotic Therapy in Chronic Respiratory Diseases

PubMed Central

Maselli, Diego J.; Keyt, Holly; Restrepo, Marcos I.

2017-01-01

The management of patients with chronic respiratory diseases affected by difficult to treat infections has become a challenge in clinical practice. Conditions such as cystic fibrosis (CF) and non-CF bronchiectasis require extensive treatment strategies to deal with multidrug resistant pathogens that include Pseudomonas aeruginosa, Methicillin-resistant Staphylococcus aureus, Burkholderia species and non-tuberculous Mycobacteria (NTM). These challenges prompted scientists to deliver antimicrobial agents through the pulmonary system by using inhaled, aerosolized or nebulized antibiotics. Subsequent research advances focused on the development of antibiotic agents able to achieve high tissue concentrations capable of reducing the bacterial load of difficult-to-treat organisms in hosts with chronic respiratory conditions. In this review, we focus on the evidence regarding the use of antibiotic therapies administered through the respiratory system via inhalation, nebulization or aerosolization, specifically in patients with chronic respiratory diseases that include CF, non-CF bronchiectasis and NTM. However, further research is required to address the potential benefits, mechanisms of action and applications of inhaled antibiotics for the management of difficult-to-treat infections in patients with chronic respiratory diseases. PMID:28509852

Inhaled Antibiotic Therapy in Chronic Respiratory Diseases.

PubMed

Maselli, Diego J; Keyt, Holly; Restrepo, Marcos I

2017-05-16

The management of patients with chronic respiratory diseases affected by difficult to treat infections has become a challenge in clinical practice. Conditions such as cystic fibrosis (CF) and non-CF bronchiectasis require extensive treatment strategies to deal with multidrug resistant pathogens that include Pseudomonas aeruginosa , Methicillin-resistant Staphylococcus aureus , Burkholderia species and non-tuberculous Mycobacteria (NTM). These challenges prompted scientists to deliver antimicrobial agents through the pulmonary system by using inhaled, aerosolized or nebulized antibiotics. Subsequent research advances focused on the development of antibiotic agents able to achieve high tissue concentrations capable of reducing the bacterial load of difficult-to-treat organisms in hosts with chronic respiratory conditions. In this review, we focus on the evidence regarding the use of antibiotic therapies administered through the respiratory system via inhalation, nebulization or aerosolization, specifically in patients with chronic respiratory diseases that include CF, non-CF bronchiectasis and NTM. However, further research is required to address the potential benefits, mechanisms of action and applications of inhaled antibiotics for the management of difficult-to-treat infections in patients with chronic respiratory diseases.

Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic Fibrosis Diagnosed following Newborn Screening

PubMed Central

Hall, Graham L.; Logie, Karla M.; Parsons, Faith; Schulzke, Sven M.; Nolan, Gary; Murray, Conor; Ranganathan, Sarath; Robinson, Phil; Sly, Peter D.; Stick, Stephen M.

2011-01-01

Background In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution. The primary objective of this analysis was to determine the relationships between ventilation distribution outcomes and the presence and extent of structural damage as assessed by chest CT in infants and young children with CF. Methods Data of infants and young children with CF diagnosed following newborn screening consecutively reviewed between August 2005 and December 2009 were analysed. Ventilation distribution (lung clearance index and the first and second moment ratios [LCI, M1/M0 and M2/M0, respectively]), chest CT and airway pathology from bronchoalveolar lavage were determined at diagnosis and then annually. The chest CT scans were evaluated for the presence or absence of bronchiectasis and air trapping. Results Matched lung function, chest CT and pathology outcomes were available in 49 infants (31 male) with bronchiectasis and air trapping present in 13 (27%) and 24 (49%) infants, respectively. The presence of bronchiectasis or air trapping was associated with increased M2/M0 but not LCI or M1/M0. There was a weak, but statistically significant association between the extent of air trapping and all ventilation distribution outcomes. Conclusion These findings suggest that in early CF lung disease there are weak associations between ventilation distribution and lung damage from chest CT. These finding are in contrast to those reported in older children. These findings suggest that assessments of LCI could not be used to replace a chest CT scan for the assessment of structural lung disease in the first two years of life. Further research in which both MBW and chest CT outcomes are obtained is required to assess the role of ventilation distribution in tracking the progression of lung damage in infants with CF. PMID:21886842

The Th17 Pathway in Cystic Fibrosis Lung Disease

PubMed Central

Tan, Hui-Leng; Regamey, Nicolas; Brown, Sarah; Bush, Andrew; Lloyd, Clare M.; Davies, Jane C.

2012-01-01

Rationale Cystic fibrosis (CF) is characterized by bronchoalveolar neutrophilia and submucosal lymphocytosis. We hypothesized that Th17 lymphocytes are part of this submucosal infiltrate. Objectives Quantification and phenotyping of the lymphocytic infiltrate in the bronchial submucosa of patients with CF (n=53, of which 20 were newly diagnosed), non-CF bronchiectasis (n = 17), and healthy control subjects (n = 13). Methods We measured IL-17 levels in bronchoalveolar lavage and CD4+, CD8+, and IL-17+ cell counts in endobronchial biopsies. Correlations were made with infection status and other inflammatory markers. Potential cellular sources of IL-17 were determined by double staining. Measurements and Main Results IL-17+ cell counts (median [interquartile range] cells/mm2) were significantly higher in patients with established CF (205 [115–551]) and non-CF bronchiectasis (245 [183–436]) than in control subjects (53 [12–82]) (P<0.01 for both). Patients with newly diagnosed CF had intermediate counts (171 [91–252]). IL-17–positive CD4+ T cells, γδT cells, natural killer T cells, and neutrophils were identified. Bronchoalveolar lavage IL-17 levels (pg/ml) were highest in established CF (14.6 [2.2–38.4]), low in newly diagnosed CF and control subjects (1.7 [1.7–1.74]; 1.7 [1.7–3]), and intermediate in non-CF bronchiectasis (9.1 [1.7–34] pg/ml) (Kruskal-Wallis P = 0.001). There was a significant correlation between IL-17 and neutrophil counts (P < 0.001, R = 0.6) as well as IL-4 (P < 0.001, R = 0.84). Conclusions Th17 lymphocytes are present in the airway submucosa in CF, even in a young, newly diagnosed group. Other IL-17+ cells include neutrophils, γδ T cells, and natural killer T cells. PMID:21474644

The SENSOR Study: Protocol for a Mixed-Methods Study of Self-Management Checks to Predict Exacerbations of Pseudomonas Aeruginosa in Patients with Long-Term Respiratory Conditions

PubMed Central

Roberts, Claire; Gunatilake, Samal; Storrar, Will; Elliott, Scott; Glaysher, Sharon; Green, Ben; Rule, Steven; Fogg, Carole; Dewey, Ann; Auton, Kevin A; Chauhan, Anoop J

2017-01-01

Background There are an estimated three million people in the United Kingdom with chronic obstructive pulmonary disease (COPD), and the incidence of bronchiectasis is estimated at around 0.1% but is more common in COPD and severe asthma. Both COPD and bronchiectasis are characterized by exacerbations in which bacteria play a central role. Pseudomonas aeruginosa is isolated from sputum samples from 4% to 15% of adults with COPD and is more likely to be isolated from patients with severe disease. Earlier detection of exacerbations may improve morbidity and mortality by expediting treatment. Aseptika Ltd has developed a system for patients to self-monitor important physiological measurements including levels of physical activity, peak flow, forced expiratory volume (FEV1), and biomarkers for P aeruginosa in sputum. Objective We aim to test this system in 20 participants with P aeruginosa colonization and 10 controls with Haemophilus influenzae. Methods We plan to recruit 30 adult participants with COPD or non-CF bronchiectasis who have cultured P aeruginosa or H influenzae during an exacerbation in the last 6 months. They must produce sputum on most days and should have been stable for 4 weeks prior to entry. Daily data collected will include symptoms, health care usage, medication, weight, FEV1, physical activity level, blood pressure, oxygen saturation, and temperature. Sputum and urine samples will be provided daily. These data will be analyzed to assess predictive value in detecting upcoming exacerbations. Qualitative data will be gathered through self-administered questionnaires and semistructured interviews to gather information on participant coping and their use of the technology involved. Results Recruitment has been completed and results from the study should be available at the end of 2017. Conclusions The SENSOR study aims to test a home-monitoring system in people with chronic airway infection and is currently underway. PMID:28526665

Short-term effects of three slow expiratory airway clearance techniques in patients with bronchiectasis: a randomised crossover trial.

PubMed

Herrero-Cortina, B; Vilaró, J; Martí, D; Torres, A; San Miguel-Pagola, M; Alcaraz, V; Polverino, E

2016-12-01

To compare the efficacy of three slow expiratory airway clearance techniques (ACTs). Randomised crossover trial. Tertiary hospital. Thirty-one outpatients with bronchiectasis and chronic sputum expectoration. Autogenic drainage (AD), slow expiration with glottis opened in lateral posture (ELTGOL), and temporary positive expiratory pressure (TPEP). Sputum expectoration during each session (primary endpoint) and in the 24-hour period after each session. Leicester Cough Questionnaire (LCQ) score and spirometry results were recorded at the beginning and after each week of treatment. Data were summarised as median difference [95% confidence interval (CI)]. Median (interquartile range) daily expectoration at baseline was 21.1 (15.3 to 35.6)g. During physiotherapy sessions, AD and ELTGOL expectorated more sputum than TPEP [AD vs TPEP 3.1g (95% CI 1.5 to 4.8); ELTGOL vs TPEP 3.6g (95% CI 2.8 to 7.1)], while overall expectoration in the 24-hour period after each session was similar for all techniques (P=0.8). Sputum clearance at 24hours post-intervention was lower than baseline assessment for all techniques [AD vs baseline -10.0g (95% CI -15.0 to -6.8); ELTGOL vs baseline -9.2g (95% CI -14.2 to -7.9); TPEP vs baseline -6.0g (95% CI -12.0 to -6.1)]. The LCQ score increased with all techniques (AD 0.5, 95% CI 0.1 to 0.5; ELTGOL 0.9, 95% CI 0.5 to 2.1; TPEP 0.4, 95% CI 0.1 to 1.2), being similar for all ACTs (P=0.6). No changes in lung function were observed. Slow expiratory ACTs enhance mucus clearance during treatment sessions, and reduce expectoration for the rest of the day in patients with bronchiectasis. NCT01854788. Copyright © 2015 Chartered Society of Physiotherapy. Published by Elsevier Ltd. All rights reserved.

Predicting high risk of exacerbations in bronchiectasis: the E-FACED score.

PubMed

Martinez-Garcia, M A; Athanazio, R A; Girón, R; Máiz-Carro, L; de la Rosa, D; Olveira, C; de Gracia, J; Vendrell, M; Prados-Sánchez, C; Gramblicka, G; Corso Pereira, M; Lundgren, F L; Fernandes De Figueiredo, M; Arancibia, F; Rached, S Z

2017-01-01

Although the FACED score has demonstrated a great prognostic capacity in bronchiectasis, it does not include the number or severity of exacerbations as a separate variable, which is important in the natural history of these patients. Construction and external validation of a new index, the E-FACED, to evaluate the predictive capacity of exacerbations and mortality. The new score was constructed on the basis of the complete cohort for the construction of the original FACED score, while the external validation was undertaken with six cohorts from three countries (Brazil, Argentina, and Chile). The main outcome was the number of annual exacerbations/hospitalizations, with all-cause and respiratory-related deaths as the secondary outcomes. A statistical evaluation comprised the relative weight and ideal cut-off point for the number or severity of the exacerbations and was incorporated into the FACED score (E-FACED). The results obtained after the application of FACED and E-FACED were compared in both the cohorts. A total of 1,470 patients with bronchiectasis (819 from the construction cohorts and 651 from the external validation cohorts) were followed up for 5 years after diagnosis. The best cut-off point was at least two exacerbations in the previous year (two additional points), meaning that the E-FACED has nine points of growing severity. E-FACED presented an excellent prognostic capacity for exacerbations (areas under the receiver operating characteristic curve: 0.82 for at least two exacerbations in 1 year and 0.87 for at least one hospitalization in 1 year) that was statistically better than that of the FACED score (0.72 and 0.78, P <0.05, respectively). The predictive capacities for all-cause and respiratory mortality were 0.87 and 0.86, respectively, with both being similar to those of the FACED. E-FACED score significantly increases the FACED capacity to predict future yearly exacerbations while maintaining the score's simplicity and prognostic capacity for death.

Long-term air humidification therapy is cost-effective for patients with moderate or severe chronic obstructive pulmonary disease or bronchiectasis.

PubMed

Milne, Richard J; Hockey, Hans; Rea, Harry

2014-06-01

To establish the cost-effectiveness of long-term humidification therapy (LTHT) added to usual care for patients with moderate or severe chronic obstructive pulmonary disease or bronchiectasis. Resource usage in a 12-month clinical trial of LTHT was estimated from hospital records, patient diaries, and the equipment supplier. Health state utility values were derived from the St. Georges Respiratory Questionnaire (SGRQ) total score. All patients who remained in the trial for 12 months and who had at least 90 days of diary records were included (87 of 108). Clinical costs were NZ $3973 (95% confidence interval [CI] $1614-$6332) for the control group and NZ $3331 (95% CI $948-$6920) for the intervention group. The mean health benefit per patient was -6.9 SGRQ units (95% CI -13.0 to -7.2; P < 0.05) or +0.0678 quality-adjusted life-years (95% CI 0.001-0.135). With the intervention costing NZ $2059 annually, the mean cost per quality-adjusted life-year was NZ $20,902 (US $18,907) and the bootstrap median was NZ $19,749 (2.5th percentile -$40,923, 97.5th percentile $221,275). At a willingness-to-pay (WTP) threshold of NZ $30,000, the probability of cost-effectiveness was 61%, ranging from 49% to 72% as the cost of LTHT was varied by ±30%. At a WTP of NZ $20,000, the probability was 49% (range 34%-61%). LTHT is moderately cost-effective for patients with moderate to severe chronic obstructive pulmonary disease or bronchiectasis at a WTP threshold that is acceptable for public funding of medicines in New Zealand. These findings must be interpreted with caution because of the modest size of the clinical study, necessary lack of blinding in the clinical trial, and uncertainty in estimating health state utility from the SQRQ. Copyright © 2014 International Society for Pharmacoeconomics and Outcomes Research (ISPOR). Published by Elsevier Inc. All rights reserved.

The Bronchiectasis Severity Index. An International Derivation and Validation Study

PubMed Central

Goeminne, Pieter; Aliberti, Stefano; McDonnell, Melissa J.; Lonni, Sara; Davidson, John; Poppelwell, Lucy; Salih, Waleed; Pesci, Alberto; Dupont, Lieven J.; Fardon, Thomas C.; De Soyza, Anthony; Hill, Adam T.

2014-01-01

Rationale: There are no risk stratification tools for morbidity and mortality in bronchiectasis. Identifying patients at risk of exacerbations, hospital admissions, and mortality is vital for future research. Objectives: This study describes the derivation and validation of the Bronchiectasis Severity Index (BSI). Methods: Derivation of the BSI used data from a prospective cohort study (Edinburgh, UK, 2008–2012) enrolling 608 patients. Cox proportional hazard regression was used to identify independent predictors of mortality and hospitalization over 4-year follow-up. The score was validated in independent cohorts from Dundee, UK (n = 218); Leuven, Belgium (n = 253); Monza, Italy (n = 105); and Newcastle, UK (n = 126). Measurements and Main Results: Independent predictors of future hospitalization were prior hospital admissions, Medical Research Council dyspnea score greater than or equal to 4, FEV1 < 30% predicted, Pseudomonas aeruginosa colonization, colonization with other pathogenic organisms, and three or more lobes involved on high-resolution computed tomography. Independent predictors of mortality were older age, low FEV1, lower body mass index, prior hospitalization, and three or more exacerbations in the year before the study. The derived BSI predicted mortality and hospitalization: area under the receiver operator characteristic curve (AUC) 0.80 (95% confidence interval, 0.74–0.86) for mortality and AUC 0.88 (95% confidence interval, 0.84–0.91) for hospitalization, respectively. There was a clear difference in exacerbation frequency and quality of life using the St. George’s Respiratory Questionnaire between patients classified as low, intermediate, and high risk by the score (P < 0.0001 for all comparisons). In the validation cohorts, the AUC for mortality ranged from 0.81 to 0.84 and for hospitalization from 0.80 to 0.88. Conclusions: The BSI is a useful clinical predictive tool that identifies patients at risk of future mortality, hospitalization, and exacerbations across healthcare systems. PMID:24328736

Predicting high risk of exacerbations in bronchiectasis: the E-FACED score

PubMed Central

Martinez-Garcia, MA; Athanazio, RA; Girón, R; Máiz-Carro, L; de la Rosa, D; Olveira, C; de Gracia, J; Vendrell, M; Prados-Sánchez, C; Gramblicka, G; Corso Pereira, M; Lundgren, FL; Fernandes De Figueiredo, M; Arancibia, F; Rached, SZ

2017-01-01

Background Although the FACED score has demonstrated a great prognostic capacity in bronchiectasis, it does not include the number or severity of exacerbations as a separate variable, which is important in the natural history of these patients. Objective Construction and external validation of a new index, the E-FACED, to evaluate the predictive capacity of exacerbations and mortality. Methods The new score was constructed on the basis of the complete cohort for the construction of the original FACED score, while the external validation was undertaken with six cohorts from three countries (Brazil, Argentina, and Chile). The main outcome was the number of annual exacerbations/hospitalizations, with all-cause and respiratory-related deaths as the secondary outcomes. A statistical evaluation comprised the relative weight and ideal cut-off point for the number or severity of the exacerbations and was incorporated into the FACED score (E-FACED). The results obtained after the application of FACED and E-FACED were compared in both the cohorts. Results A total of 1,470 patients with bronchiectasis (819 from the construction cohorts and 651 from the external validation cohorts) were followed up for 5 years after diagnosis. The best cut-off point was at least two exacerbations in the previous year (two additional points), meaning that the E-FACED has nine points of growing severity. E-FACED presented an excellent prognostic capacity for exacerbations (areas under the receiver operating characteristic curve: 0.82 for at least two exacerbations in 1 year and 0.87 for at least one hospitalization in 1 year) that was statistically better than that of the FACED score (0.72 and 0.78, P<0.05, respectively). The predictive capacities for all-cause and respiratory mortality were 0.87 and 0.86, respectively, with both being similar to those of the FACED. Conclusion E-FACED score significantly increases the FACED capacity to predict future yearly exacerbations while maintaining the score’s simplicity and prognostic capacity for death. PMID:28182132

Impact of cough across different chronic respiratory diseases: comparison of two cough-specific health-related quality of life questionnaires.

PubMed

Polley, Liam; Yaman, Nurman; Heaney, Liam; Cardwell, Chris; Murtagh, Eimear; Ramsey, John; MacMahon, Joseph; Costello, Richard W; McGarvey, Lorcan

2008-08-01

Cough is a prominent symptom across a range of common chronic respiratory diseases and impacts considerably on patient health status. We undertook a cross-sectional comparison of scores from two cough-specific health-related quality of life (HRQoL) questionnaires, the Leicester Cough Questionnaire (LCQ), and the Cough Quality of Life Questionnaire (CQLQ), together with a generic HRQoL measure, the EuroQol. Questionnaires were administered to and spirometry performed on 147 outpatients with chronic cough (n = 83), COPD (n = 18), asthma (n = 20), and bronchiectasis (n = 26). There was no significant difference in the LCQ and CQLQ total scores between groups (p = 0.24 and p = 0.26, respectively). Exploratory analyses of questionnaire subdomains revealed differences in psychosocial issues and functional impairment between the four groups (p = 0.01 and p = 0.05, respectively). CQLQ scores indicated that chronic coughers have more psychosocial issues than patients with bronchiectasis (p = 0.03) but less functional impairment than COPD patients (p = 0.04). There was a significant difference in generic health status across the four disease groups (p = 0.04), with poorest health status in COPD patients. A significant inverse correlation was observed between CQLQ and LCQ in each disease group (chronic cough r = - 0.56, p < 0.001; COPD r = - 0.49, p = 0.04; asthma r = - 0.94, p < 0.001; and bronchiectasis r = - 0.88, p < 0.001). There was no correlation between cough questionnaire scores and FEV(1) in any group, although a significant correlation between EuroQol visual analog scale component and FEV(1) (r = 0.639, p = 0.004) was observed in COPD patients. Cough adversely affects health status across a range of common respiratory diseases. The LCQ and CQLQ can each provide important additional information concerning the impact of cough.

The Rationale and Evidence for Use of Inhaled Antibiotics to Control Pseudomonas aeruginosa Infection in Non-cystic Fibrosis Bronchiectasis

PubMed Central

2018-01-01

Abstract Non-cystic fibrosis bronchiectasis (NCFBE) is a chronic inflammatory lung disease characterized by irreversible dilation of the bronchi, symptoms of persistent cough and expectoration, and recurrent infective exacerbations. The prevalence of NCFBE is on the increase in the United States and Europe, but no licensed therapies are currently available for its treatment. Although there are many similarities between NCFBE and cystic fibrosis (CF) in terms of respiratory symptoms, airway microbiology, and disease progression, there are key differences, for example, in response to treatment, suggesting differences in pathogenesis. This review discusses possible reasons underlying differences in response to inhaled antibiotics in people with CF and NCFBE. Pseudomonas aeruginosa infections are associated with the most severe forms of bronchiectasis. Suboptimal levels of antibiotics in the lung increase the mutation frequency of P. aeruginosa and lead to the development of mucoid strains characterized by formation of a protective polysaccharide biofilm. Mucoid strains of P. aeruginosa are associated with a chronic infection stage, requiring long-term antibiotic therapy. Inhaled antibiotics provide targeted delivery to the lung with minimal systemic toxicity and adverse events compared with oral/intravenous routes of administration, and they could be alternative treatment options to help address some of the treatment challenges in the management of severe cases of NCFBE. This review provides an overview of completed and ongoing trials that evaluated inhaled antibiotic therapy for NCFBE. Recently, several investigators conducted phase 3 randomized controlled trials with inhaled aztreonam and ciprofloxacin in patients with NCFBE. While the aztreonam trial results were not associated with significant clinical benefit in NCFBE, initial results reported from the inhaled ciprofloxacin (dry powder for inhalation and liposome-encapsulated/dual-release formulations) trials hold promise. A more targeted approach could identify specific populations of NCFBE patients who benefit from inhaled antibiotics. PMID:29077527

Clinical, Bronchographic, Radiological, and Physiological Observations in Ten Cases of Asbestosis

PubMed Central

Leathart, G. L.

1960-01-01

Ten cases of asbestosis (eight male, two female), aged 45-65 years have been kept under observation for periods of up to eight years. Bronchiectasis was demonstrated bronchographically in six cases. Clubbing of the fingers and coarse crepitations appeared to be signs of bronchiectasis rather than of uncomplicated asbestosis. It is suggested that the prevalence of bronchiectasis is higher than has been reported previously because the patients survived longer. The radiological findings are tabulated and compared with previous descriptions. In these subjects there was no relationship between radiological and clinical state. Nine patients eventually showed clinical deterioration and it often proceeded rapidly. The radiograph however, usually remained unaltered. Pulmonary function tests, including diffusing capacity, arterial blood analysis and estimation of mechanical properties of the lung, were carried out in these 10 cases, and in 11 asbestos workers (aged 35-64 years) without radiological abnormality. The steady state diffusing capacity for carbon monoxide (Dco) at rest was lower in asbestosis than in the control subjects. The pulmonary compliance was remarkably low in asbestosis and related fairly closely to the vital capacity. The maximum voluntary ventilation was also low and was related to increased pulmonary resistance but it cannot be said whether this is in the airways or in the lung tissue. Indirect evidence of inequalities of ventilation/perfusion ratio was obtained in most cases. There is no convincing evidence that pulmonary fibrosis occurs without radiological abnormality, but a defect of diffusion may occur. There is no test of pulmonary function which is diagnostic, but a low pulmonary compliance, especially if combined with a low diffusing capacity, is confirmatory. It is suggested that the demonstration of a progressive decline in vital capacity, or in diffusing capacity, may enable a diagnosis of asbestosis to be made before radiological abnormality has appeared, but this point has not been proved.

Impact of Long-Term Erythromycin Therapy on the Oropharyngeal Microbiome and Resistance Gene Reservoir in Non-Cystic Fibrosis Bronchiectasis.

PubMed

Choo, Jocelyn M; Abell, Guy C J; Thomson, Rachel; Morgan, Lucy; Waterer, Grant; Gordon, David L; Taylor, Steven L; Leong, Lex E X; Wesselingh, Steve L; Burr, Lucy D; Rogers, Geraint B

2018-04-25

Long-term macrolide therapy reduces rates of pulmonary exacerbation in bronchiectasis. However, little is known about the potential for macrolide therapy to alter the composition and function of the oropharyngeal commensal microbiota or to increase the carriage of transmissible antimicrobial resistance. We assessed the effect of long-term erythromycin on oropharyngeal microbiota composition and the carriage of transmissible macrolide resistance genes in 84 adults with bronchiectasis, enrolled in the Bronchiectasis and Low-dose Erythromycin Study (BLESS) 48-week placebo-controlled trial of twice-daily erythromycin ethylsuccinate (400 mg). Oropharyngeal microbiota composition and macrolide resistance gene carriage were determined by 16S rRNA gene amplicon sequencing and quantitative PCR, respectively. Long-term erythromycin treatment was associated with a significant increase in the relative abundance of oropharyngeal Haemophilus parainfluenzae ( P = 0.041) and with significant decreases in the relative abundances of Streptococcus pseudopneumoniae ( P = 0.024) and Actinomyces odontolyticus ( P = 0.027). Validation of the sequencing results by quantitative PCR confirmed a significant decrease in the abundance of Actinomyces spp. ( P = 0.046). Erythromycin treatment did not result in a significant increase in the number of subjects who carried erm (A), erm (B), erm (C), erm (F), mef (A/E), and msrA macrolide resistance genes. However, the abundance of erm (B) and mef (A/E) gene copies within carriers who had received erythromycin increased significantly ( P < 0.05). Our findings indicate that changes in oropharyngeal microbiota composition resulting from long-term erythromycin treatment are modest and are limited to a discrete group of taxa. Associated increases in levels of transmissible antibiotic resistance genes within the oropharyngeal microbiota highlight the potential for this microbial system to act as a reservoir for resistance. IMPORTANCE Recent demonstrations that long-term macrolide therapy can prevent exacerbations in chronic airways diseases have led to a dramatic increase in their use. However, little is known about the wider, potentially adverse impacts of these treatments. Substantial disruption of the upper airway commensal microbiota might reduce its contribution to host defense and local immune regulation, while increases in macrolide resistance carriage would represent a serious public health concern. Using samples from a randomized controlled trial, we show that low-dose erythromycin given over 48 weeks influences the composition of the oropharyngeal commensal microbiota. We report that macrolide therapy is associated with significant changes in the relative abundances of members of the Actinomyces genus and with significant increases in the carriage of transmissible macrolide resistance. Determining the clinical significance of these changes, relative to treatment benefit, now represents a research priority. © Crown copyright 2018.

Non cystic fibrosis bronchiectasis: A longitudinal retrospective observational cohort study of Pseudomonas persistence and resistance.

PubMed

McDonnell, Melissa J; Jary, Hannah R; Perry, Audrey; MacFarlane, James G; Hester, Katy L M; Small, Therese; Molyneux, Catherine; Perry, John D; Walton, Katherine E; De Soyza, Anthony

2015-06-01

The hallmark of non-cystic fibrosis bronchiectasis is recurrent bronchial infection, yet there are significant gaps in our understanding of pathogen persistence, resistance and exacerbation frequencies. Pseudomonas aeruginosa is a key pathogen thought to be a marker of disease severity and progression, yet little is known if the infection risk is seen in those with milder disease or if there is any potential for eradication. These data are important in determining risk stratification and follow up. A retrospective review of consecutive adult patients attending a specialist UK bronchiectasis clinic over a two-year recruitment period between July 2007 and June 2009 was performed. Analysis of our primary outcome, longitudinal microbiological status, was recorded based on routine clinical follow-up through to data capture point or date of death. Patients were stratified by lung function and infecting organism. 155 patients (mean (SD) age 62.2 (12.4) years; 60.1% female) were identified from clinic records with microbiological data for a median (IQR) follow up duration of 46 (35-62) months. Baseline mean FEV1% predicted was 60.6% (24.8) with mean exacerbation frequency of 4.42/year; 73.6% reported 3 or more exacerbations/year. Haemophilus influenzae was isolated in 90 (58.1%) patients and P. aeruginosa in 78 (50.3%) patients with persistent infection in 51 (56.7%) H. influenzae and 47 (60.3%) P. aeruginosa, respectively. Of the P. aeruginosa colonised patients, 16 (34%) became culture negative on follow-up with a mean of 5.2 negative sputum cultures/patient. P. aeruginosa was isolated from 5 out of 39 patients (12.8%) with minimal airflow limitation as compared to 18 out of 38 patients (47.4%) with severe airflow limitation. Although hospital admissions were significantly higher in the P. aeruginosa infected group (1.3 vs. 0.7 admissions per annum, p = 0.035), overall exacerbation rates were the same (4.6 vs. 4.3, p = 0.58). Independent predictors of P. aeruginosa colonisation were low FEV1% predicted (OR 2.46; 95% CI 1.27-4.77) and polymicrobial colonisation (OR 4.07; 95% CI 1.56-10.58). 17 (11%) patients were infected with multi-resistant strains; however, none were pan-resistant. P. aeruginosa is associated with greater persistent infection rates and more hospital admissions than H. influenzae. Exacerbation rates, however, were similar; therefore H. influenzae causes significant out-patient morbidity. P. aeruginosa infection occurs across all strata of lung function impairment but is infrequently multi-resistant in bronchiectasis. Careful microbiology follow up is required even in those with well-preserved lung function. Copyright © 2014 Elsevier Ltd. All rights reserved.

Posterior Circulation Stroke After Bronchial Artery Embolization. A Rare but Serious Complication

DOE Office of Scientific and Technical Information (OSTI.GOV)

Laborda, Alicia; Tejero, Carlos; Fredes, Arturo, E-mail: fredesarturo@gmail.com

Bronchial artery embolization (BAE) is the treatment of choice for massive hemoptysis with rare complications that generally are mild and transient. There are few references in the medical literature with acute cerebral embolization as a complication of BAE. We report a case of intracranial posterior territory infarctions as a complication BAE in a patient with hemoptysis due to bronchiectasis.

[Quality of life and adherence to nebulised antibiotic therapy using a new device in non-cystic fibrosis bronchiectasis].

PubMed

Gulini, Martina; Prados, Concepción; Pérez, Amparo; Romero, David; Feliz, Darwin; Gómez Carrera, Luis; Cabanillas, Juan José; Barbero, Javier; Alvarez-Sala, Rodolfo

2012-01-01

Analysis of psychological variables, quality of life, depression and anxiety, subjective perception of compliance, level of satisfaction with the electronic device, and objective verification) in order to study the therapeutic adherence, in non-cystic fibrosis bronchiectasis (NCFB) colonized by Pseudomonas aeruginosa. A cross-sectional descriptive study was conducted on 22 NCFB stable patients colonised by Pseudomonas aeruginosa (two groups of 11 subjects: GROUP 1, Pari-LC plus nebuliser device, and GROUP 2, with the I- neb device), both containing sodium colistemethate. Different variables, were obtained, such as, self-perceived health (St George questionnaire), state of mind (HADS scale), and subjective perception of therapeutic compliance and objective verification by using the device software. There were no significant differences in questionnaire results. The therapeutic support in GROUP 2, could be proved objectively (up to 60%) using specific software, and the excellent tolerability using a specific questionnaire created for the study. These types of aerosol devices demonstrate a high level of therapeutic support, that can be measured objectively and is different from what the patients subjectively indicates. Copyright © 2011 Elsevier España, S.L. All rights reserved.

Bilateral pneumothorax, lung cavitations, and pleural empyema in a cocaine addict.

PubMed

Solaini, Leonardo; Solini, Leonardo; Gourgiotis, Stavros; Salemis, Nikolaos S; Koukis, Ioannis

2008-12-01

A case of bilateral pneumothorax, lung cavitations, and pleural empyema in a cocaine user is described. The patient was treated by left tube thoracostomy and right lower lobectomy. The postoperative course was uneventful. Six months later, the patient remains asymptomatic. The pathology examination of the specimen revealed infected bronchiectasis, interstitial desquamative pneumonia, diffuse alveolar damage, subsegmental arterial thrombosis, and consequent areas of pulmonary infarction.

[Intrabronchial migratory cereal-ear, an unusual foreign body inhalation].

PubMed

Douira-Khomsi, W; Merghni, A; Louati, H; Ben Hassine, L; Khemakhem, R; Ould Mohamed Sghair, Y; Jlidi, S; Hamzaoui, M; Chaouachi, B; Bellagha, I

2009-09-01

Bronchial foreign body inhalation is a frequent and potentially serious accident in children. It can provoke long-term complications such as bronchiectasis and recurrent pulmonary infections. The foreign body is generally blocked in a bronchus and induces obstructive emphysema with superinfection. We report 2 cases of bronchial inhalation of a migrating cereal-ear, eliminated by parietal fistulization, in 9- and 11-year-old boys.

[Kartagener sindrome (primary ciliary dyskinesia). Report of a case and literature review].

PubMed

Pino Rivero, V; Pardo Romero, G; Iglesias González, R J; Rodríguez Carmona, M; del Castillo Beneyto, F

2007-01-01

Kartagener syndrome (a clinical variant of primary ciliary dyskinesia) is a recessive autossomical disease characterized by the triad of chronic sinusitis, bronchiectasis and situs inversus with dextrocardia. We report one case described in a 8 years old boy who besides presented a seromucous otitis and bronchitis of repetition. Finally we performed a short bibliographic review at respect of this uncommon pathology.

Clinical associations of Human T-Lymphotropic Virus type 1 infection in an indigenous Australian population.

PubMed

Einsiedel, Lloyd; Spelman, Tim; Goeman, Emma; Cassar, Olivier; Arundell, Mick; Gessain, Antoine

2014-01-01

In resource-poor areas, infectious diseases may be important causes of morbidity among individuals infected with the Human T-Lymphotropic Virus type 1 (HTLV-1). We report the clinical associations of HTLV-1 infection among socially disadvantaged Indigenous adults in central Australia. HTLV-1 serological results for Indigenous adults admitted 1(st) January 2000 to 31(st) December 2010 were obtained from the Alice Springs Hospital pathology database. Infections, comorbid conditions and HTLV-1 related diseases were identified using ICD-10 AM discharge morbidity codes. Relevant pathology and imaging results were reviewed. Disease associations, admission rates and risk factors for death were compared according to HTLV-1 serostatus. HTLV-1 western blots were positive for 531 (33.3%) of 1595 Indigenous adults tested. Clinical associations of HTLV-1 infection included bronchiectasis (adjusted Risk Ratio, 1.35; 95% CI, 1.14-1.60), blood stream infections (BSI) with enteric organisms (aRR, 1.36; 95% CI, 1.05-1.77) and admission with strongyloidiasis (aRR 1.38; 95% CI, 1.16-1.64). After adjusting for covariates, HTLV-1 infection remained associated with increased numbers of BSI episodes (adjusted negative binomial regression, coefficient, 0.21; 95% CI, 0.02-0.41) and increased admission numbers with strongyloidiasis (coefficient, 0.563; 95% CI, 0.17-0.95) and respiratory conditions including asthma (coefficient, 0.99; 95% CI, 0.27-1.7), lower respiratory tract infections (coefficient, 0.19; 95% CI, 0.04-0.34) and bronchiectasis (coefficient, 0.60; 95% CI, 0.02-1.18). Two patients were admitted with adult T-cell Leukemia/Lymphoma, four with probable HTLV-1 associated myelopathy and another with infective dermatitis. Independent predictors of mortality included BSI with enteric organisms (aRR 1.78; 95% CI, 1.15-2.74) and bronchiectasis (aRR 2.07; 95% CI, 1.45-2.98). HTLV-1 infection contributes to morbidity among socially disadvantaged Indigenous adults in central Australia. This is largely due to an increased risk of other infections and respiratory disease. The spectrum of HTLV-1 related diseases may vary according to the social circumstances of the affected population.

Validation of the Hong Kong Chinese version of the St. George Respiratory Questionnaire in patients with bronchiectasis.

PubMed

Chan, Shelley L; Chan-Yeung, Moira M; Ooi, Gaik C; Lam, Cindy L; Cheung, Tung F; Lam, Wah K; Tsang, Kenneth W

2002-12-01

To validate the Hong Kong Chinese version of the St. George Respiratory Questionnaire (SGRQ-HK) in patients with bronchiectasis. Outpatients (93 patients; 61 women; mean age [+/- SD], 59.0 +/- 14.2 years) were assessed at baseline by the SGRQ-HK, the Hong Kong Chinese version of the 36-item short form health survey (SF-36-HK), and the hospital anxiety and depression scale (HADS). Forty randomly selected patients also were reassessed at 2 weeks for repeatability. Seventy-two patients were further reassessed at 6 months for responsiveness. Cronbach alpha coefficients, which reflected internal consistency, were > 0.7 for all SGRQ-HK components except for symptoms (alpha = 0.59), and the intraclass correlation coefficients between baseline and the 2-week follow-up visits were between 0.80 and 0.94 (p > 0.05). SGRQ-HK component scores and total scores correlated with all the component scores of the SF-36-HK and the HADS (p < 0.02). SGRQ-HK component scores and total scores correlated with the scores of the SF-36-HK and the HADS, confirming the concurrent validity. All SGRQ-HK scores correlated negatively with FEV(1), FVC, and arterial oxygen saturation (p < 0.005), while the activity score correlated with the Karnofsky performance scale and the number of bronchiectatic lobes (p < 0.001). SGRQ-HK scores positively correlated with Borg scale scores, exacerbation frequency, and 24-h sputum volumes (p < 0.03). Patients with 24-h sputum volumes of > or = 10 mL or < 10 mL had significantly different SQRG-HK component scores and total scores (p < 0.002), although this sensitivity was not displayed by scores on the HADS or the SF-36-HK. Patients with 25% reductions in 24-h sputum volumes had significant improvements in SGRQ-HK activity scores, impact scores, and total scores (p < 0.02), but not in other quality-of-life measures or clinical parameters, indicating the responsiveness of the SGRQ-HK. The SGRQ-HK is a valid and sensitive instrument for determining quality of life in bronchiectasis patients.

Phase 3 randomized study of the efficacy and safety of inhaled dry powder mannitol for the symptomatic treatment of non-cystic fibrosis bronchiectasis.

PubMed

Bilton, Diana; Daviskas, Evangelia; Anderson, Sandra D; Kolbe, John; King, Gregory; Stirling, Rob G; Thompson, Bruce R; Milne, David; Charlton, Brett

2013-07-01

Inhaled dry powder mannitol enhanced mucus clearance and improved quality of life over 2 weeks in non-cystic fibrosis bronchiectasis. This study's objective was to investigate the efficacy and safety of dry powder mannitol over 12 weeks. Patients with bronchiectasis confirmed by high-resolution CT (HRCT) scan, aged 15 to 80 years, with FEV1≥50% predicted and ≥1 L participated in a randomized, placebo-controlled, double-blind study. Patients with a negative mannitol provocation test were randomized to inhale 320 mg mannitol (n=231) or placebo (n=112) bid for 12 weeks. To further assess safety, the same mannitol dose/frequency was administered to a patient subset in an open-label extension over 52 weeks. Primary end points were changes from baseline at 12 weeks in 24-h sputum weight and St. George's Respiratory Questionnaire (SGRQ) score. There was a significant difference of 4.3 g in terms of change in sputum weight over 12 weeks (95% CI, 1.64-7.00; P=.002) between mannitol and placebo; however, this was largely driven by a decrease in sputum weight in the placebo group. This was associated, in turn, with more antibiotic use in the placebo group (50 of 112 [45%]) than in the inhaled mannitol group (85 of 231 [37%]). There was no statistical difference between the groups (P=.304) in total SGRQ score (mannitol, -3.4 points [95% CI, -4.81 to -1.94] vs placebo, -2.1 points [95% CI, -4.12 to -0.09]). In a subgroup study (n=82), patients receiving mannitol showed less small airway mucus plugging on HRCT scan at 12 weeks compared with patients receiving placebo (P=.048). Compliance rates were high, and mannitol was well tolerated with adverse events similar to those of placebo. Because the difference in sputum weights appears to be associated with increased antibiotic use in the placebo group, a larger controlled study is now required to investigate the long-term mannitol effect on pulmonary exacerbations and antibiotic use. ClinicalTrials.gov; No.: NCT0027753; URL: www.clinicaltrials.gov.

Aspergillus vertebral osteomyelitis and epidural abscess.

PubMed

Tew, C W; Han, F C; Jureen, R; Tey, B H

2009-04-01

We present the first reported case of Aspergillus vertebral osteomyelitis and epidural abscess in Singapore in a 50-year-old man with post-tuberculous bronchiectasis. The patient presented with acute urinary retention and flaccid paraplegia. Despite surgical debridement and treatment with voriconazole, the patient developed multiorgan failure and died two weeks after presentation. Early diagnosis and prompt initiation of treatment are emphasised in the hope of improving the outcome of this aggressive condition.

Predictors of Acute Lung Injury

DTIC Science & Technology

2013-02-26

sputum samples (bronchiectasis, chronic bronchitis, cystic fibrosis , etc.). Earlier experiments in this laboratory demonstrated that inflammatory...interleukin 1 beta (IL-1β), IL-6, IL-8, IL-10, C-reactive protein, pentraxin 3 (PTX3), and activated complement 5 daily for 4 days. The diagnosis of ALI or...standard curve. The coefficient of variation for the repeated measures was 5% for these biomarker analyses. 3.2.2 ALI/ARDS Determination. Diagnosis

Inhaled antibiotics in the treatment of non-cystic fibrosis bronchiectasis: clinical and drug delivery perspectives.

PubMed

Sugianto, Tiffanie Daisy; Chan, Hak-Kim

2016-01-01

Non-cystic fibrosis bronchiectasis (NCFB) is a chronic, progressive, suppurative lung disease characterized by permanent dilatation of bronchial subdivisions, which further causes accumulation of sputum and bacterial infections. The advent of inhaled antibiotics over the past two decades has been expected to effectively attenuate the problem of chronic bacterial infections in CF and NCFB subjects with higher, local drug concentrations and minimal systemic side effects. This review summarizes and evaluates current clinical evidence of efficacy and adverse effects of inhaled antibiotics in NCFB, as well as ongoing preclinical and clinical studies, followed by a discussion of issues and challenges in clinical practice and drug delivery strategies, together with future research directions. The evidence base of the clinical efficacy of inhaled antibiotics in NCFB is limited and the degrees of reported clinical benefits have been modest and conflicting. Challenges surrounding inhaled antibiotics application and development include the lack of knowledge of disease factors and optimum management strategies, unreceptive lung pathophysiology and the lack of factors that support compliance and tolerability. Nonetheless, research continues to give birth to new clinical findings and novel formulations such as combination antibiotics and sustained-release formulations, which add great value to the development of efficacious, safe and convenient inhalable antibiotics of the future.

Immunomodulatory effects of macrolide antibiotics - part 2: advantages and disadvantages of long-term, low-dose macrolide therapy.

PubMed

Altenburg, J; de Graaff, C S; van der Werf, T S; Boersma, W G

2011-01-01

The available evidence for long-term, low-dose treatment with 14- and 15-membered ring macrolides in non-cystic fibrosis (CF) bronchiectasis, COPD, chronic sinusitis, and asthma is reviewed with special attention to possible adverse effects and the emergence of resistance during long-term macrolide treatment. Macrolide maintenance therapy has been proven to be of benefit in diffuse panbronchiolitis and CF, presumably due to an anti-inflammatory mechanism of action in addition to its direct antimicrobial effect. Solid evidence to justify this treatment regimen for non-CF bronchiectasis, asthma, or sinusitis is still lacking, although a beneficial effect of long-term macrolide therapy has been found in small clinical trials on these subjects. Data from randomized trials of long-term macrolide treatment in COPD are conflicting. A sufficiently long duration of treatment and the careful selection of patients appears to be crucial. Aside from its beneficial effects, possible side effects of macrolide treatment should be taken into account, the most important of these being gastrointestinal upset and cardiac arrhythmias. Development of macrolide resistance among respiratory pathogens is very common during long-term macrolide treatment. Whether this finding is clinically significant is a matter of debate. Copyright © 2010 S. Karger AG, Basel.

Gas exchanges in children with cystic fibrosis or primary ciliary dyskinesia: A retrospective study.

PubMed

Fuger, Marilyn; Aupiais, Camille; Thouvenin, Guillaume; Taytard, Jessica; Tamalet, Aline; Escudier, Estelle; Boizeau, Priscilla; Corvol, Harriet; Beydon, Nicole

2018-05-01

Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) both entail bronchiectasis and pulmonary impairment as measured using spirometry, during childhood. We aimed at looking whether blood gas exchanges progressed differently between CF and PCD children in a retrospective study of repeated measurements. Comparisons between groups (Wilcoxon-Mann-Whitney and Chi-squared tests) and a mixed linear model, adjusted for age, evaluated associations between diseases and PaO 2 , PaCO 2, or PaO 2- PaCO 2 ratio. Among 42 PCD and 73 CF children, 62% and 59% had respectively bronchiectasis (P = 0.75). Spirometry and blood gases were similar at inclusion (PaO 2 median [IQR] PCD -1.80 [-3.40; -0.40]; CF -1.80 [-4.20; 0.60] z-scores; P = 0.72). PaO 2 and PaO 2 -PaCO 2 ratio similarly and significantly decreased with age in both groups (P < 0.01) whereas PaCO 2 increased more in CF (P = 0.02) remaining within the range of normal (except for one child). To conclude, gas exchange characteristics, similarly initially impaired in PCD and CF children, tended to less deteriorate with time in PCD children who could benefit from an early diagnosis. Copyright © 2018 Elsevier B.V. All rights reserved.

The regional association between bronchiectasis and lung cancer in chest CT.

PubMed

Kim, Yeon Wook; Lee, Chang-Hoon; Jin, Kwang-Nam; Lee, Jung-Kyu; Heo, Eun Young; Park, Sung Soo; Chung, Hee Soon; Kim, Deog Kyeom

2016-11-15

Limited studies have examined the association between lung cancer and bronchiectasis (BE). This study evaluated the regional association between BE and lung cancer by analyzing the lobar location of lung cancer in patients with underlying BE. This clustered multi-level study enrolled patients who had underlying BE and were newly diagnosed with lung cancer between January 1, 2010 and May 30, 2013 in two referral hospitals in South Korea. By analyzing the presence of lung cancer and underlying BE as event variables at the level of lung lobes on chest computed tomography (CT), we evaluated the association of BE and lung cancer by the locations of the diseases. Eighty-one patients with BE and combined lung cancer were enrolled. Within 486 lung lobes of the patients, combined BE and lung cancer in the same lobe was found in 11 lobes (2.3 %). Using the general estimating equation assuming BE as a risk factor of lung cancer, the results indicated that the prevalence of lung cancer was significantly lower in the lobes with pre-existing BE (β = -1.09, p-value = 0.001). Regionally, pre-existing BE was associated with a lower risk of the occurrence of lung cancer in the same lobe.

Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases.

PubMed

Schmitt, Pierre; Dalar, Levent; Jouneau, Stéphane; Toublanc, Bénédicte; Camuset, Juliette; Chatte, Gérard; Cellerin, Laurent; Dutau, Hervé; Sanchez, Stéphane; Sauvage, Maxime; Vergnon, Jean-Michel; Dury, Sandra; Deslée, Gaetan; Lebargy, François

2016-01-01

Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by enlargement of the trachea and main bronchi and associated with recurrent respiratory tract infections. This multicenter, retrospective study was carried out to describe respiratory conditions associated with tracheobronchomegaly. Nine institutions involved in the 'Groupe d'Endoscopie de Langue Française' (GELF) participated in this study. A standard form was used to record patient characteristics, treatments and follow-up from medical charts. Seventeen patients, 53% male, aged 58 ± 18 years at diagnosis were included. Recurrent infections revealed MKS in 88% of cases. Main comorbid conditions were diffuse bronchiectasis in 88% of patients and tracheobronchomalacia in 67% of cases. The exacerbation rate was 1.5 exacerbations/patient/year. The main non-respiratory morbid condition was gastroesophageal reflux disease in 29% of cases. Interventional bronchoscopy was performed in seven patients (41%), consisting of laser (n = 2) and tracheal stenting (n = 5). Complications related to stents were observed in 80% of cases with a mean stent duration of 8 months. Four deaths, including three due to respiratory causes, occurred during follow-up. This is the largest series of MKS reported in the literature, showing that bronchiectasis and tracheobronchomalacia are the main associated morbid conditions that constitute a challenge for treatment. © 2016 S. Karger AG, Basel.

Crackle analysis for chest auscultation and comparison with high-resolution CT findings.

PubMed

Kawamura, Takeo; Matsumoto, Tsuneo; Tanaka, Nobuyuki; Kido, Shoji; Jiang, Zhongwei; Matsunaga, Naofumi

2003-01-01

The purpose of our study was to clarify the correlation between respiratory sounds and the high-resolution CT (HRCT) findings of lung diseases. Respiratory sounds were recorded using a stethoscope in 41 patients with crackles. All had undergone inspiratory and expiratory CT. Subjects included 18 patients with interstitial pneumonia and 23 without interstitial pneumonia. Two parameters, two-cycle duration (2CD) and initial deflection width (IDW) of the "crackle," were induced by time-expanded waveform analysis. Two radiologists independently assessed 11 HRCT findings. An evaluation was carried out to determine whether there was a significant difference in the two parameters between the presence and absence of each HRCT finding. The two parameters of crackles were significantly shorter in the interstitial pneumonia group than the non-interstitial pneumonia group. Ground-glass opacity, honeycombing, lung volume reduction, traction bronchiectasis, centrilobular nodules, emphysematous change, and attenuation and volume change between inspiratory and expiratory CT were correlated with one or two parameters in all patients, whereas the other three findings were not. Among the interstitial pneumonia group, traction bronchiectasis, emphysematous change, and attenuation and volume change between inspiratory and expiratory CT were significantly correlated with one or two parameters. Abnormal respiratory sounds were correlated with some HRCT findings.

Clinical and Genetic Aspects of Primary Ciliary Dyskinesia / Kartagener Syndrome

PubMed Central

Leigh, Margaret W.; Pittman, Jessica E.; Carson, Johnny L.; Ferkol, Thomas W.; Dell, Sharon D.; Davis, Stephanie D.; Knowles, Michael R.; Zariwala, Maimoona A.

2013-01-01

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder of motile cilia. Most of the disease-causing mutations identified to date involve the heavy (DNAH5) or intermediate (DNAI1) chain dynein genes in ciliary outer dynein arms, although a few mutations have been noted in other genes. Clinical molecular genetic testing for PCD is available for the most common mutations. The respiratory manifestations of PCD (chronic bronchitis leading to bronchiectasis, chronic rhino-sinusitis and chronic otitis media) reflect impaired mucociliary clearance owing to defective axonemal structure. Ciliary ultrastructural analysis in most patients (>80%) reveals defective dynein arms, although defects in other axonemal components have also been observed. Approximately 50% of PCD patients have laterality defects (including situs inversus totalis and, less commonly, heterotaxy and congenital heart disease), reflecting dysfunction of embryological nodal cilia. Male infertility is common and reflects defects in sperm tail axonemes. Most PCD patients have a history of neonatal respiratory distress, suggesting that motile cilia play a role in fluid clearance during the transition from a fetal to neonatal lung. Ciliopathies involving sensory cilia, including autosomal dominant or recessive polycystic kidney disease, Bardet-Biedl syndrome, and Alstrom syndrome, may have chronic respiratory symptoms and even bronchiectasis suggesting clinical overlap with PCD. PMID:19606528

Variations in the ultrastructure of human nasal cilia including abnormalities found in retinitis pigmentosa.

PubMed Central

Fox, B; Bull, T B; Arden, G B

1980-01-01

The electron microscopic structure of cilia from the inferior turbinate of the nose was studied in 12 adults, four with chronic sinusitis, one with allergic rhinitis, two with bronchiectasis, three with deviated nasal septum, and two normals. The changes are compared with those found in nasal cilia in 14 patients with retinitis pigmentosa. There were compound cilia in the seven cases with chronic sinusitis, allergic rhinitis, and bronchiectasis but, apart from this, the structure of the cilia was similar in all 12 cases. There were variations in the microtubular pattern in about 4% of cilia, dynein arms were not seen in 4%, and in the rest an average of 5-6 dynein arms were seen in each cilium. The orientation of the cilia was 0 to 90 degrees. In the retinitis pigmentosa patients there was a highly significant increase in cilial abnormalities. The establishment on a quantitative basis of the variations in normal structure of nasal cilila facilitated the recognition of an association between cilial abnormalities and retinitis pigmentosa and should help in the identification of associations that may exist between cilial abnormalities and other diseases. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 7 Fig. 8 PMID:7400333

Acute retinal necrosis as a novel complication of chickenpox in adults.

PubMed Central

Matsuo, T; Koyama, M; Matsuo, N

1990-01-01

Three patients in their 20s suffered from chickenpox while in an immunocompromised state: one in pregnancy, one during a long course of corticosteroid for severe nephrotic syndrome, and the third with repeated upper airway infection due to bronchiectasis. They developed acute retinal necrosis about three weeks after the onset of chickenpox. Since acute retinal necrosis threatens sight, this unusual complication of chickenpox in adults needs serious consideration. Images PMID:2378860

Effects of Techa River Radiation Contamination on the Reproductive Function of Residents

DTIC Science & Technology

2006-11-01

general somatic diseases such as diseases of the respiratory organs (bronchiectasis), diabetes , hepatic, and renal insufficiency. The method of choice...the diagnosis of CRS in some of the patients was infantilism , i.e., retard- ed and insufficient sexual development. As is indicated in a previous...legs and left forearm after a railway disaster. The two remaining women, twins born in 1944, were diagnosed with infantilism , which is evidently the

Clinical Associations of Human T-Lymphotropic Virus Type 1 Infection in an Indigenous Australian Population

PubMed Central

Einsiedel, Lloyd; Spelman, Tim; Goeman, Emma; Cassar, Olivier; Arundell, Mick; Gessain, Antoine

2014-01-01

Introduction In resource-poor areas, infectious diseases may be important causes of morbidity among individuals infected with the Human T-Lymphotropic Virus type 1 (HTLV-1). We report the clinical associations of HTLV-1 infection among socially disadvantaged Indigenous adults in central Australia. Methodology and Principal Findings HTLV-1 serological results for Indigenous adults admitted 1st January 2000 to 31st December 2010 were obtained from the Alice Springs Hospital pathology database. Infections, comorbid conditions and HTLV-1 related diseases were identified using ICD-10 AM discharge morbidity codes. Relevant pathology and imaging results were reviewed. Disease associations, admission rates and risk factors for death were compared according to HTLV-1 serostatus. HTLV-1 western blots were positive for 531 (33.3%) of 1595 Indigenous adults tested. Clinical associations of HTLV-1 infection included bronchiectasis (adjusted Risk Ratio, 1.35; 95% CI, 1.14–1.60), blood stream infections (BSI) with enteric organisms (aRR, 1.36; 95% CI, 1.05–1.77) and admission with strongyloidiasis (aRR 1.38; 95% CI, 1.16–1.64). After adjusting for covariates, HTLV-1 infection remained associated with increased numbers of BSI episodes (adjusted negative binomial regression, coefficient, 0.21; 95% CI, 0.02–0.41) and increased admission numbers with strongyloidiasis (coefficient, 0.563; 95% CI, 0.17–0.95) and respiratory conditions including asthma (coefficient, 0.99; 95% CI, 0.27–1.7), lower respiratory tract infections (coefficient, 0.19; 95% CI, 0.04–0.34) and bronchiectasis (coefficient, 0.60; 95% CI, 0.02–1.18). Two patients were admitted with adult T-cell Leukemia/Lymphoma, four with probable HTLV-1 associated myelopathy and another with infective dermatitis. Independent predictors of mortality included BSI with enteric organisms (aRR 1.78; 95% CI, 1.15–2.74) and bronchiectasis (aRR 2.07; 95% CI, 1.45–2.98). Conclusion HTLV-1 infection contributes to morbidity among socially disadvantaged Indigenous adults in central Australia. This is largely due to an increased risk of other infections and respiratory disease. The spectrum of HTLV-1 related diseases may vary according to the social circumstances of the affected population. PMID:24454973

Postexposure Application of Fas Receptor Small-Interfering RNA to Suppress Sulfur Mustard-Induced Apoptosis in Human Airway Epithelial Cells: Implication for a Therapeutic Approach

DTIC Science & Technology

2013-01-01

bronchiolitis, bronchopneumo- nia, chronic obstructive pulmonary disease, bronchiectasis, asthma, large airway narrowing, and pulmonary fibrosis ... pulmonary fibrosis , acute lung injury [ALI], acute respiratory distress syndrome, etc.) (Beheshti et al., 2006; Emmler et al., 2007; Kuwano, 2008...cytokine in bronchoalveolar lavage (BAL) fluid in patients with pulmonary fibrosis due to sulfur mustard gas inhalation. J In- terferon Cytokine Res 27:38

Elastolytic activity of sputum and its relation to purulence and to lung function in patients with bronchiectasis.

PubMed Central

Stockley, R A; Hill, S L; Morrison, H M; Starkie, C M

1984-01-01

Sputum samples from 34 patients with bronchiectasis were assessed subjectively and the results related to objective measurements of elastase activity and albumin content. The results suggest that the macroscopic appearance of the sample is related to the elastase content. 88.7% of the purulent samples but none of the mucoid samples showing elastase activity. The macroscopic appearance was also associated with changes in protein transudation into the secretions. The median sputum: serum albumin concentration ratio was 0.71 X 10(-2) (range 0.22-4.7) in the mucoid samples but was greater in purulent samples (p less than 0.005), with a median value of 1.52 X 10(-2) (range 0.55-12.72), suggesting that purulence in the stable state was associated with low grade pulmonary inflammation or epithelial damage or both. Abnormalities of air flow were found in 24 of the patients (70.6%) but there was a significantly higher ratio of residual volume to total lung capacity (p less than 0.025) in patients who regularly produced purulent sputum (mean (SD) RV/TLC 44.4% (9.0%] than in those with mucoid or mucopurulent secretions (38.0% (9.9%]. A similar difference was found between those who produced elastase positive secretions and those who produced elastase negative ones. PMID:6565423

Role of long term antibiotics in chronic respiratory diseases.

PubMed

Suresh Babu, K; Kastelik, J; Morjaria, J B

2013-06-01

Antibiotics are commonly used in the management of respiratory disorders such as cystic fibrosis (CF), non-CF bronchiectasis, asthma and COPD. In those conditions long-term antibiotics can be delivered as nebulised aerosols or administered orally. In CF, nebulised colomycin or tobramycin improve lung function, reduce number of exacerbations and improve quality of life (QoL). Oral antibiotics, such as macrolides, have acquired wide use not only as anti-microbial agents but also due to their anti-inflammatory and pro-kinetic properties. In CF, macrolides such as azithromycin have been shown to improve the lung function and reduce frequency of infective exacerbations. Similarly macrolides have been shown to have some benefits in COPD including reduction in a number of exacerbations. In asthma, macrolides have been reported to improve some subjective parameters, bronchial hyperresponsiveness and airway inflammation; however have no benefits on lung function or overall asthma control. Macrolides have also been used with beneficial effects in less common disorders such as diffuse panbronchiolitis or post-transplant bronchiolitis obliterans syndrome. In this review we describe our current knowledge the use of long-term antibiotics in conditions such as CF, non-CF bronchiectasis, asthma and COPD together with up-to-date clinical and scientific evidence to support our understanding of the use of antibiotics in those conditions. Copyright © 2013 Elsevier Ltd. All rights reserved.

Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome.

PubMed

Leigh, Margaret W; Pittman, Jessica E; Carson, Johnny L; Ferkol, Thomas W; Dell, Sharon D; Davis, Stephanie D; Knowles, Michael R; Zariwala, Maimoona A

2009-07-01

Primary ciliary dyskinesia is a genetically heterogeneous disorder of motile cilia. Most of the disease-causing mutations identified to date involve the heavy (dynein axonemal heavy chain 5) or intermediate(dynein axonemal intermediate chain 1) chain dynein genes in ciliary outer dynein arms, although a few mutations have been noted in other genes. Clinical molecular genetic testing for primary ciliary dyskinesia is available for the most common mutations. The respiratory manifestations of primary ciliary dyskinesia (chronic bronchitis leading to bronchiectasis, chronic rhino-sinusitis, and chronic otitis media)reflect impaired mucociliary clearance owing to defective axonemal structure. Ciliary ultrastructural analysis in most patients (>80%) reveals defective dynein arms, although defects in other axonemal components have also been observed. Approximately 50% of patients with primary ciliary dyskinesia have laterality defects (including situs inversus totalis and, less commonly, heterotaxy, and congenital heart disease),reflecting dysfunction of embryological nodal cilia. Male infertility is common and reflects defects in sperm tail axonemes. Most patients with primary ciliary dyskinesia have a history of neonatal respiratory distress, suggesting that motile cilia play a role in fluid clearance during the transition from a fetal to neonatal lung. Ciliopathies involving sensory cilia, including autosomal dominant or recessive polycystic kidney disease, Bardet-Biedl syndrome, and Alstrom syndrome, may have chronic respiratory symptoms and even bronchiectasis suggesting clinical overlap with primary ciliary dyskinesia.

Activities and sources of beta-lactamase in sputum from patients with bronchiectasis.

PubMed Central

Dragicevic, P; Hill, S L; Burnett, D; Merrikin, D; Stockley, R A

1989-01-01

beta-Lactamase activity was measured in secretions from patients with bronchiectasis. Of 28 sputum samples, 23 contained measurable amounts of activity; values were significantly higher (P less than 0.01) in purulent samples than in mucoid or mucopurulent samples. beta-Lactamase activity was usually present in saliva collected before and between sputum expectorations, although values for sputum were higher than for either group of saliva samples (P less than 0.025 and P less than 0.005, respectively). This difference suggests that at least part of sputum beta-lactamase activity originates in the bronchial tree. Detailed microbiological study of a further eight specimens (seven were beta-lactamase positive) led to the isolation of Haemophilus influenzae from six, although only two of these isolates were beta-lactamase positive. Several other beta-lactamase-producing organisms were also isolated, including Staphylococcus aureus (n = 3), Escherichia coli (n = 1), Proteus spp. (n = 1), and Bacteroides spp. (n = 3). Size-exclusion high-performance liquid chromatography of the sputum showed several peaks of beta-lactamase activity which usually coeluted in fractions similar to those of their beta-lactamase-positive isolates. Therefore, sources of sputum beta-lactamases are often bacteria not considered truly pathogenic or not isolated during routine bacteriological assessment. These observations should be considered when embarking on antimicrobial therapy in bronchiectatic patients and suggest that increased dosages of penicillins are indicated. PMID:2663911

Bronchiectasis: correlation of high-resolution CT findings with health-related quality of life.

PubMed

Eshed, I; Minski, I; Katz, R; Jones, P W; Priel, I E

2007-02-01

To evaluate the relationship between the severity of bronchiectatic diseases, as evident on high-resolution computed tomography (HRCT) and the patient's quality of life measured using the St George's Respiratory Questionnaire (SGRQ). Forty-six patients (25 women, 21 men, mean age: 63 years) with bronchiectatic disease as evident on recent HRCT examinations were recruited. Each patient completed the SGRQ and underwent respiratory function tests. HRCT findings were blindly and independently scored by two radiologists, using the modified Bhalla scoring system. The relationships between HRCT scores, SGRQ scores and pulmonary function tests were evaluated. The patients' total CT score did not correlate with the SGRQ scores. However, patients with more advanced disease on HRCT, significantly differed in their SGRQ scores from patients with milder bronchiectatic disease. A significant correlation was found between the CT scores for the middle and distal lung zones and the activity, impacts and total SGRQ scores. No correlation was found between CT scores and respiratory function test indices. However, a significant correlation was found between the SGRQ scores and most of the respiratory function test indices. A correlation between the severity of bronchiectatic disease as expressed in HRCT and the health-related quality of life exists in patients with a more severe bronchiectatic disease but not in patients with mild disease. Such correlation depends on the location of the bronchiectasis in the pulmonary tree.

The L441P mutation of cystic fibrosis transmembrane conductance regulator and its molecular pathogenic mechanisms in a Korean patient with cystic fibrosis.

PubMed

Gee, Heon Yung; Kim, Chang Keun; Kim, So Won; Lee, Ji Hyun; Kim, Jeong-Ho; Kim, Kyung Hwan; Lee, Min Goo

2010-01-01

Cystic fibrosis (CF) is an autosomal recessive disorder usually found in populations of white Caucasian descent. CF is caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene. A 5-yr-old Korean girl was admitted complaining of coughing and greenish sputum. Chest radiographs and computed tomographic (CT) scan revealed diffuse bronchiectasis in both lungs. The patient had chronic diarrhea and poor weight gain, and the abdominal pancreaticobiliary CT scan revealed atrophy of the pancreas. Finally, CF was confirmed by the repeated analysis of the quantitative pilocarpine iontophoresis test. The chloride concentration of sweat samples taken from both forearms of the pateint was an average of 88.7 mM/L (normal value <40 mM/L). After a comprehensive search for mutations in the CFTR gene, the patient was found to carry the non-synonymous L441P mutation in one allele. Molecular physiologic analysis of the L441P mutation of CFTR revealed that the L441P mutation completely abolished the CFTR Cl(-) channel activity by disrupting proper protein folding and membrane trafficking of CFTR protein. These results confirmed the pathogenicity of the L441P mutation of CFTR circulating in the Korean population. The possibility of CF should be suspected in patients with chronic bronchiectasis, although the frequency of CF is relatively rare in East Asia.

[Clinical and functional characteristics of patients prior to lung transplantation: report of experience at the Clínica Puerta de Hierro].

PubMed

Laporta, Rosalía; Ussetti, Piedad; Mora, Gema; López, Cristina; Gómez, David; de Pablo, Alicia; Lázaro, M Teresa; Carreño, M Cruz; Ferreiro, M José

2008-08-01

The time at which lung transplantation is indicated is determined by clinical and functional criteria that vary according to the particular disease. The aim of our study was to present the criteria according to which patients were placed on waiting lists for lung transplantation in our hospital. We analyzed retrospectively the clinical characteristics, lung function, heart function, and 6-minute walk test results of patients who had received a lung transplant in our hospital from January 2002 through September 2005. During the study period 100 lung transplants were performed. The mean age of the patients was 45 years (range, 15-67 years) and 57% were men. The diseases that most often led to a lung transplant were chronic obstructive pulmonary disease (COPD) (35%), pulmonary fibrosis (29%), and bronchiectasis (21%). Lung function values differed by disease: mean (SD) forced expiratory volume in 1 second (FEV1) was 20% (11%) and forced vital capacity (FVC) was 37% (15%) in patients with COPD; FEV1 was 41% (15%) and FVC, 40% (17%) in patients with pulmonary fibrosis; and FEV1 was 23% (7%) and FVC, 37% (10%) in patients with bronchiectasis. The patients who received lung transplants in our hospital were in advanced phases of their disease and met the inclusion criteria accepted by the various medical associations when they were placed on the waiting list.

Pathological Studies on the Protective Effect of a Macrolide Antibiotic, Roxithromycin, against Sulfur Mustard Inhalation Toxicity in a Rat Model

DTIC Science & Technology

2011-01-01

placed in a water-jacketed (37C) glass vapor generator custom fabri - cated by Atmar Glass (Kennett Square, PA). Spontaneously breathing rats were...bronchiectasis, and pul- monary fibrosis (Emad and Rezaian 1997). Macrolide antibio- tics have shown effectiveness in a variety of chronic respiratory diseases ...observed in clinical studies of chronic inflammatory airway diseases (e.g., DPB) after long-term treatment with low dose of macrolides (Rubin 2004). For

High-Resolution Computed Tomography and Pulmonary Function Findings of Occupational Arsenic Exposure in Workers.

PubMed

Ergün, Recai; Evcik, Ender; Ergün, Dilek; Ergan, Begüm; Özkan, Esin; Gündüz, Özge

2017-05-05

The number of studies where non-malignant pulmonary diseases are evaluated after occupational arsenic exposure is very few. To investigate the effects of occupational arsenic exposure on the lung by high-resolution computed tomography and pulmonary function tests. Retrospective cross-sectional study. In this study, 256 workers with suspected respiratory occupational arsenic exposure were included, with an average age of 32.9±7.8 years and an average of 3.5±2.7 working years. Hair and urinary arsenic levels were analysed. High-resolution computed tomography and pulmonary function tests were done. In workers with occupational arsenic exposure, high-resolution computed tomography showed 18.8% pulmonary involvement. In pulmonary involvement, pulmonary nodule was the most frequently seen lesion (64.5%). The other findings of pulmonary involvement were 18.8% diffuse interstitial lung disease, 12.5% bronchiectasis, and 27.1% bullae-emphysema. The mean age of patients with pulmonary involvement was higher and as they smoked more. The pulmonary involvement was 5.2 times higher in patients with skin lesions because of arsenic. Diffusing capacity of lung for carbon monoxide was significantly lower in patients with pulmonary involvement. Besides lung cancer, chronic occupational inhalation of arsenic exposure may cause non-malignant pulmonary findings such as bronchiectasis, pulmonary nodules and diffuse interstitial lung disease. So, in order to detect pulmonary involvement in the early stages, workers who experience occupational arsenic exposure should be followed by diffusion test and high-resolution computed tomography.

Investigation into the Individualized Treatment of Traditional Chinese Medicine through a Series of N-of-1 Trials

PubMed Central

Yang, Peilan; Wang, Jie; Wu, Yingen; Zi, Suna; Tang, Jie; Wang, Zhenwei

2018-01-01

Purpose To compare the efficacy of individualized herbal decoction with standard decoction for patients with stable bronchiectasis through N-of-1 trials. Methods We conducted a single center N-of-1 trials in 17 patients with stable bronchiectasis. Each N-of-1 trial contains three cycles. Each cycle is divided into two 4-week intervention including individualized decoction and fixed decoction (control). The primary outcome was patient self-reported symptoms scores on a 1–7 point Likert scale. Secondary outcomes were 24-hour sputum volume and CAT scores. Results Among 14 completed trials, five showed that the individualized decoction was statistically better than the control decoction on symptom scores (P < 0.05) but was not clinically significant. The group data of all the trials showed that individualized decoction was superior to control decoction on symptom scores (2.13 ± 0.58 versus 2.30 ± 0.65, P = 0.002, mean difference and 95% CI: 0.18 (0.10, 0.25)), 24 h sputum volume (P = 0.009), and CAT scores (9.69 ± 4.89 versus 11.64 ± 5.59, P = 0.013, mean difference and 95% CI: 1.95 (1.04, 2.86)) but not clinically significant. Conclusion Optimizing the combined analysis of individual and group data and the improvement of statistical models may make contribution in establishing a method of evaluating clinical efficacy in line with the characteristics of traditional Chinese medicine individual diagnosis and treatment. PMID:29552084

Fourier-based linear systems description of free-breathing pulmonary magnetic resonance imaging

NASA Astrophysics Data System (ADS)

Capaldi, D. P. I.; Svenningsen, S.; Cunningham, I. A.; Parraga, G.

2015-03-01

Fourier-decomposition of free-breathing pulmonary magnetic resonance imaging (FDMRI) was recently piloted as a way to provide rapid quantitative pulmonary maps of ventilation and perfusion without the use of exogenous contrast agents. This method exploits fast pulmonary MRI acquisition of free-breathing proton (1H) pulmonary images and non-rigid registration to compensate for changes in position and shape of the thorax associated with breathing. In this way, ventilation imaging using conventional MRI systems can be undertaken but there has been no systematic evaluation of fundamental image quality measurements based on linear systems theory. We investigated the performance of free-breathing pulmonary ventilation imaging using a Fourier-based linear system description of each operation required to generate FDMRI ventilation maps. Twelve subjects with chronic obstructive pulmonary disease (COPD) or bronchiectasis underwent pulmonary function tests and MRI. Non-rigid registration was used to co-register the temporal series of pulmonary images. Pulmonary voxel intensities were aligned along a time axis and discrete Fourier transforms were performed on the periodic signal intensity pattern to generate frequency spectra. We determined the signal-to-noise ratio (SNR) of the FDMRI ventilation maps using a conventional approach (SNRC) and using the Fourier-based description (SNRF). Mean SNR was 4.7 ± 1.3 for subjects with bronchiectasis and 3.4 ± 1.8, for COPD subjects (p>.05). SNRF was significantly different than SNRC (p<.01). SNRF was approximately 50% of SNRC suggesting that the linear system model well-estimates the current approach.

Chronic Hypersensitivity Pneumonitis With a Usual Interstitial Pneumonia-Like Pattern: Correlation Between Histopathologic and Clinical Findings.

PubMed

Chiba, Sahoko; Tsuchiya, Kimitake; Akashi, Takumi; Ishizuka, Masahiro; Okamoto, Tsukasa; Furusawa, Haruhiko; Tateishi, Tomoya; Kishino, Mitsuhiro; Miyazaki, Yasunari; Tateishi, Ukihide; Takemura, Tamiko; Inase, Naohiko

2016-06-01

Hypersensitivity pneumonitis (HP) is an interstitial lung disease caused by the inhalation of environmental antigens. The relationship between clinical, radiologic, and histopathologic findings of chronic HP remains unclear. Sixteen patients with proven chronic bird-related HP with a usual interstitial pneumonia-like pattern were analyzed retrospectively. Histopathologic findings were semiquantitatively assessed and compared with clinical and radiologic findings. We also evaluated the histopathologic findings affecting prognosis. The extent of centrilobular fibrosis was negatively correlated with Pao2 (r = -0.55, P = .03). The extent of bridging fibrosis was positively correlated with the ratio of maximal expiratory flow at 50% of forced vital capacity to that at 25% (r = 0.60, P = .02). Patients with a greater extent of fibroblastic foci (FF) had more radiologic reticulation (P = .01), honeycombing (P = .01), and traction bronchiectasis (P = .02), and had significantly shorter survival time (P = .01) than patients with a lesser extent of FF. Multivariate analysis showed that the extent of FF was a significant prognostic factor (hazard ratio, 2.36; 95% confidence interval, 1.02-5.48; P = .04). Our findings demonstrated that the extent of FF was significantly associated with reticulation, honeycombing, and traction bronchiectasis on high-resolution CT scanning. Moreover, the extent of FF could be a useful predictor of mortality in chronic HP with a usual interstitial pneumonia-like pattern. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

The chemotactic activity of sputum from patients with bronchiectasis.

PubMed

Mikami, M; Llewellyn-Jones, C G; Bayley, D; Hill, S L; Stockley, R A

1998-03-01

Persistent polymorphonuclear neutrophil (PMN) recruitment to airway is thought to be an important component of continuing inflammation and progression of chronic destructive lung diseases. Although chemoattractants are required for the PMN to migrate, the nature of the chemoattractants in the airways has not yet been clarified. We therefore investigated the contribution of interleukin-8 (IL-8) and leukotriene-B4 (LTB4) to the chemotactic activity of lung secretions by inhibiting their activity using a monoclonal antibody to IL-8 and an LTB4 receptor antagonist (LY293111 sodium). Fifty-nine sputum samples obtained from 19 patients with bronchiectasis were studied. In preliminary studies the chemotactic responses to IL-8 and LTB4 were found to be additive, and we were able to remove their contribution independently with the appropriate antibody and antagonist. The chemotactic activity of the secretions was related to the macroscopic appearance (mucoid, mucopurulent, and purulent), and this appeared to be related to an increase in IL-8 contribution. Chemotactic activity was reduced by antibiotic therapy and again that seemed to relate to a reduction in the IL-8 contribution. The contributions of LTB4 were similar among the three types of sputum in varying clinical states. These data suggest that LTB4 and IL-8 are important chemotactic factors in lung secretions from such patients, although IL-8 appears to play a more important role during acute exacerbations. These results may be useful in determining therapeutic strategies for chronic destructive lung diseases in the future.

Economic burden of non-cystic fibrosis bronchiectasis in the first year after diagnosis from a US health plan perspective.

PubMed

Joish, Vijay N; Spilsbury-Cantalupo, Monica; Operschall, Elisabeth; Luong, Ba; Boklage, Susan

2013-06-01

Recent estimates suggest the prevalence of non-cystic fibrosis bronchiectasis (NCFB) may be increasing in the US. The objective of this study was to determine the current economic burden of NCFB compared with non-NCFB controls in the first year after diagnosis within a commercially enrolled US population. A retrospective matched cross-sectional case control (1:3) study design was used. Data were derived from MarketScan(®) Commercial Claims and Encounters Database, which captures all patient-level demographic data and all medical and pharmacy claims during the period 1 January 2005 to 31 December 2009. NCFB patients were identified using ICD-9 codes 494.0 and 494.1. Individuals with medical claims for cystic fibrosis or chronic obstructive pulmonary disease were excluded. Incremental burden of NCFB was estimated for overall and respiratory-related expenditures using multivariate regression models which adjusted for baseline characteristics and healthcare resource utilization. All demographic characteristics and economic outcomes were ascertained in 12 months before (baseline period) and 12 months after (follow-up) index event, which was defined as the first bronchiectasis-related medical event. Non-parametric bootstrap technique was used to calculate the 95 % confidence limits for the adjusted estimate. All costs are inflation-adjusted to a baseline year of 2009 using the consumer price index. All statistical tests were conducted using SAS 9.2 and STATA 12.0. The study sample used for healthcare burden analyses had 9,146 cases and 27,438 matched controls. The majority of the sample was between the ages of 45-64 years old and 64 % were female. A greater proportion of cases than controls had an increase from baseline to follow-up in both total (49 vs 40 %) and respiratory-related costs (57 vs 25 %). The average increase in overall and respiratory-related costs compared with controls after adjusting for differences in baseline characteristics was US$2,319 (95 % CI 1,872-2,765) and US$1,607 (95 % CI 1,406-1,809), respectively. The primary driver for this increment was increase in outpatient visits of approximately 2 overall and 1.6 respiratory-related visits per patient per year, which translated to US$1,730 (95 % CI 1,332-2,127) and US$1,253 (95 % CI 1,097-1,408), respectively. This study found that the cost of managing NCFB in the first year within a commercially enrolled population may be burdensome. Compared with previously published estimates in the literature, the burden of NCFB may be also increasing.

[Molecular diagnosis of two Chinese cystic fibrosis children and literature review].

PubMed

Xu, B P; Wang, H; Zhao, Y H; Liu, J; Yao, Y; Feng, X L; Shen, K L

2016-05-01

To investigate the clinical manifestations and molecular features of cystic fibrosis in Chinese children. A retrospective analysis of two pediatric cystic fibrosis cases diagnosed by gene test in Beijing Children's Hospital, Capital Medical University from 2010 to 2015, and Chinese cystic fibrosis reported patients searched of"cystic fibrosis, Chinese"on Chinese databases (CNKI, Wanfang Data) and PubMed from 1975 to 2015.The clinical manifestations and molecular features were analyzed. One of the two newly diagnosed cystic fibrosis cases was a 10-year old girl who suffered from reccurent cough with expectoration and associated with cirrhosis.Sweat tests showed increased chloride twice with the lower level of 306.82 mmol/L.The other was an 8-month old boy with reccurent pneumonia from neonate, failure to thrive and fatty diarrhea.Two children had various degrees of bronchiectasis and massive sticky secretion on the bronchoscopy.They had no family history and their parents had no consanguineous marriage.CFTR mutations of c. 595C>T and c. 2290C>T were found in gene tests.On the database, twenty-one reports involving thirty-six Chinese patients (16 males and 20 females) were retrieved.Together with this group of 2 cases, a total of 38 cases were involved.The age at diagnosis was 4 months to 28 years with a median age of 10 years.All patients had reccurent respiratory infections, twenty-seven cases (71%) had malnutrition, fifteen (39%)had chronic diarrhea, and 16 cases (42%) had other digestive manifestations, including jaundice (4 cases), hepatomegaly (11 cases), ascites (2 cases) and pancreatic atrophy (3 cases). Five cases had a positive family history and six cases had a suspicious family history.Consanguineous marriage was found in three families.Sweat test revealed elevated chloride (52-327 mmol/L) in 28 cases.Eight of the 16 patients who performed pancreatic exocrine function examination showed pancreatic insufficiency.Eighteen of the 20 patients described the image manifestation of bronchiectasis.The age at death of 13 patients(34%) was 4 months to 25 years with a median of 11 years.Twenty-nine rare CFTR mutations were found in 25 tested patients, and c. 1766+ 5G>T(5 cases), c.2083dupG and c. 2684G>A (3 cases) held the top three mutation patterns. Respiratory symptoms and bronchiectasis are the main manifestations of Chinese cystic fibrosis patients, although some of them may be associated with malnutrition, digestive symptoms and pancreatic insufficiency. CFTR mutations are various and rare in Caucasians.

Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment.

PubMed

Khalil, Nasreen; O'Connor, Robert

2004-07-20

Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal pulmonary fibrotic lung disease. The diagnostic histological changes are called usual interstitial pneumonia and are characterized by histological temporal heterogeneity, whereby normal lung tissue is interspersed with interstitial fibrosis, honeycomb cysts and fibroblast foci. Pulmonary functions show restricted volumes and capacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomography demonstrates evidence of fibrosis and lung remodelling such as honeycomb cysts and traction bronchiectasis. There is no known effective treatment for IPF, but lung transplantation improves survival.

Value of allohaemagglutinins in the diagnosis of a polysaccharide antibody deficiency

PubMed Central

Schaballie, H; Vermeulen, F; Verbinnen, B; Frans, G; Vermeulen, E; Proesmans, M; De Vreese, K; Emonds, MP; De Boeck, K; Moens, L; Picard, C; Bossuyt, X; Meyts, I

2015-01-01

Polysaccharide antibody deficiency is characterized by a poor or absent antibody response after vaccination with an unconjugated pneumococcal polysaccharide vaccine. Allohaemagglutinins (AHA) are antibodies to A or B polysaccharide antigens on the red blood cells, and are often used as an additional or alternative measure to assess the polysaccharide antibody response. However, few studies have been conducted to establish the clinical significance of AHA. To investigate the value of AHA to diagnose a polysaccharide antibody deficiency, pneumococcal polysaccharide antibody titres and AHA were studied retrospectively in 180 subjects in whom both tests had been performed. Receiver operating characteristic curves for AHA versus the pneumococcal vaccine response as a marker for the anti-polysaccharide immune response revealed an area under the curve between 0·5 and 0·573. Sensitivity and specificity of AHA to detect a polysaccharide antibody deficiency, as diagnosed by vaccination response, were low (calculated for cut-off 1/4–1/32). In subjects with only low pneumococcal antibody response, the prevalence of bronchiectasis was significantly higher than in subjects with only low AHA (45·5 and 1·3%, respectively) or normal pneumococcal antibody response and AHA (2·4%). A logistic regression model showed that low pneumococcal antibody response but not AHA was associated with bronchiectasis (odds ratio 46·2). The results of this study do not support the routine use of AHA to assess the polysaccharide antibody response in patients with suspected immunodeficiency, but more studies are warranted to clarify the subject further. PMID:25516411

Risk factors for drug-resistant bacterial pneumonia in older patients hospitalized with pneumonia in a Chinese population.

PubMed

Ma, H M; Ip, Margaret; Woo, Jean; Hui, David S C; Lui, Grace C Y; Lee, Nelson L S; Chan, Paul K S; Rainer, T H

2013-09-01

The relationship between healthcare-associated pneumonia (HCAP) and resistant bacteria is unclear. The aim of this study was to identify the risk factors for pneumonia caused by drug-resistant bacteria (DRB). A prospective cohort study was conducted at a tertiary teaching hospital in Hong Kong. Consecutive older patients (aged ≥65 years) were hospitalized with pneumonia from January 2004 to June 2005. DRB comprised methicillin-resistant Staphylococcus aureus (MRSA), Pseudomonas aeruginosa, extended-spectrum β-lactamase (ESBL) producing Enterobacteriaceae and Acinetobacter baumannii. The entire cohort consisted of 1176 older patients. Of 472 (40.1%) patients with etiological diagnosis established, bacterial pneumonia was found in 354 (30.1%) cases. DRB were isolated in 48 patients: P. aeruginosa (41), MRSA (5) and ESBL producing enteric bacilli (3). Co-infection with P. aeruginosa and MRSA was found in one patient. The prevalence of DRB in culture-positive pneumonia was 20.1% (48/239). Patients with DRB were more likely to have limitation in activities of daily living, bronchiectasis, dementia, severe pneumonia, recent hospitalization and recent antibiotic use. Logistic regression revealed that bronchiectasis [relative risk (RR) 14.12, P = 0.002], recent hospitalization (RR 4.89, P < 0.001) and severe pneumonia (RR 2.42, P = 0.010) were independent predictors of drug-resistant bacterial pneumonia. Recent hospitalization is the only risk factor for HCAP which is shown to be associated with DRB. Nursing home residence is not a risk factor. The concept of HCAP may not be totally applicable in Hong Kong where the prevalence of drug-resistant pathogens in pneumonia is low.

Characteristic features of tacrolimus-induced lung disease in rheumatoid arthritis patients.

PubMed

Sasaki, Takanori; Nakamura, Wataru; Inokuma, Shigeko; Matsubara, Erika

2016-02-01

This paper aims to study the background and clinical characteristics of tacrolimus (TAC)-induced lung disease. A case of a rheumatoid arthritis (RA) patient who developed TAC-induced interstitial lung disease (TAC-ILD) is reported. The Japanese Pharmaceuticals and Medical Devices Agency (PMDA) website was searched for cases of TAC-ILD and its prevalence among all cases of TAC-related adverse events. As for cases of TAC-ILD, its underlying disease, preexisting lung diseases, and fatal outcome were also searched. Literature review of TAC-ILD cases was added. A 65-year-old female RA patient with preexisting bronchiectasis developed near-fatal TAC-ILD. Amelioration of RA, ground-glass opacities in the upper, anterior, and central lung fields, and decrease in peripheral blood lymphocyte count were the major findings in this patient. A search of the PMDA website revealed the following: the prevalence of TAC-ILD was 3 % of all cases of TAC-related adverse events, 56 out of 85 RA cases (66 %), and one out of 15 other cases had a preexisting lung disease; the prevalences of fatal outcome in RA and other cases were 24 and 38 %, respectively. A few cases in the literature had preexisting ILD and developed diffuse alveolar damage. In our case, preexisting bronchiectasis, arthritis remission, newly developed ground-glass opacities (GGOs) in the upper, anterior, and central lung fields, and decrease in peripheral blood lymphocyte count were the major findings. From the search of the PMDA website, about one fourth of the cases with TAC-related lung injury had a fatal outcome, and among RA patients, two thirds had preexisting lung diseases.

[Allergic bronchopulmonary aspergillosis. A report of a case and literature review].

PubMed

Meza Brítez, Ricardo L; del Río Navarro, Blanca E; Ochoa López, Georgina; Pietropaolo Cienfuegos, Dino; del Río Chivardi, Jaime M; Rosas Vargas, Miguel A

2008-01-01

Allergic bronchopulmonary aspergillosis is a world rare disease with a prevalence between 1 and 2%. It presents in moderate-severe asthma and cistic fibrosis patients. The diagnosis is made in the basis of Rossenberg and Greenberg criteria that can be essential or non essential. We present the case of a 3-year-old boy with allergic bronchopulmonary aspergillosis without bronchiectasies and with a good response to corticosteroids. His mother complained of two years of nasal obstruction, purulent rinorrea, nasal pruritus, sneezing, chronic cough and recurrent wheezing, twice to thrice a month. He also occasionally had vomits and diarrhea in relation with strawberries, banana, cow's milk and chocolate. We made the diagnosis of asthma, allergic rhinitis, sinusitis, and probably food allergy. We treated him with step approach of ICS according to GINA 2006, albuterol PRN, and elimination diet, with bad response. Laboratory exams: Blood white cells with eosinophilia (6%), total serum IgE: 1684 ng/L, aspergillus skin prick test: 4mm, serum IgG-Aspergillus fumigatus: 2.3 mcg/mL, serum IgE-Aspergillus fumigatus: negative, chest roentgenographic parahiliar and apical infiltrates, and chest computed tomography without bronchiectasies. We added prednisone to the treatment for four months, and we observed a very good response; he is now in treatment as mild persistent asthma with ICS low doses. ABPA must be suspected in patients with moderate-severe persistent asthma and a skin prick test positive to Aspergillus fumigatus regardless the age. The treatment with oral corticosteroids is the mainstream of management, and most of the patients have a good response, as we observed with this patient.

[Bronchiolitis with airflow obstruction in adults].

PubMed

Fournier, M; Marceau, A; Dauriat, G; Camuset, J; Groussard, O

2004-04-01

The purpose of this paper is twofold: to describe the clinical and anatomical characteristics of bronchiolitis associated with airflow obstruction in adults; to present through a clinical approach, a classification of the main aetiologies or pathological frames associated with that entity. The constrictive bronchiolitis type is the most frequently encountered. On clinical grounds, cough, crackles, and a progressive dyspnea develop usually within a few weeks. Radiological signs of bronchiolar abnormalities are best visualized on high resolution expiratory CT scan. The decrease in maximal airflows and oxygen tension is of limited amplitude and poorly reversible with bronchodilators. Diagnosis is easily performed when a causative event, or the clinical context, can be delineated: inhalation of toxic fumes, diffuse bronchiectasis, rheumatoid arthritis, lung or bone marrow transplantation. Delayed formation of bronchiectasis in the central airways is common. The treatment is not standardized; corticosteroids are usually prescribed as a first line therapy; the benefit of the addition of, or substitution with immunosuppressive drugs has not been adequately evaluated, but is, on the mean, of limited amplitude. Recent advances in the identification of inhaled agents toxic for the distal airways help in establishing appropriate measures of prevention. When the aetiology of the bronchiolitis cannot be suspected, extensive search of a causative agent should be performed, including microbial and mineral analysis of bronchoalveolar products. Negative results should lead to perform a surgical lung biopsy. The study of chronic rejection processes in animal models of lung transplantation, the identification of inhibitory factors of bronchiolar fibrogenesis, and the efficacy of some anti-cytokines on inflammatory processes could result in new therapeutic approaches.

Cardiac and Respiratory Disease in Aged Horses.

PubMed

Marr, Celia M

2016-08-01

Respiratory and cardiac diseases are common in older horses. Advancing age is a specific risk factor for cardiac murmurs and these are more likely in males and small horses. Airway inflammation is the most common respiratory diagnosis. Recurrent airway obstruction can lead to irreversible structural change and bronchiectasis; with chronic hypoxia, right heart dysfunction and failure can develop. Valvular heart disease most often affects the aortic and/or the mitral valve. Management of comorbidity is an essential element of the therapeutic approach to cardiac and respiratory disease in older equids. Copyright © 2016 Elsevier Inc. All rights reserved.

[A favourable outcome in yellow nail syndrome: role of respiratory physiotherapy].

PubMed

Fournier, C; Just, N; Leroy, S; Wallaert, B

2003-12-01

The yellow nail syndrome is a rare condition that is easily diagnosed but the nail manifestations are poorly understood. A 51 year old patient presented with a chronic cough. The diagnosis was based on the typical appearance of the nails. The patient had bilateral basal bronchiectasis. Daily physiotherapy with bronchial drainage lead to a progressive improvement in the respiratory symptoms without recourse to antibiotics. Surprisingly the abnormalities of the nails disappeared after 2 years treatment. This observation illustrates the possibility of spontaneous resolution of severe nail abnormalities during the course of the yellow nail syndrome.

Histopathological Analogies in Chronic Pulmonary Lesions between Cattle and Humans: Basis for an Alternative Animal Model

PubMed Central

Ramírez-Romero, Rafael; Nevárez-Garza, Alicia M.; Rodríguez-Tovar, Luis E.; Wong-González, Alfredo; Ledezma-Torres, Rogelio A.; Hernández-Vidal, Gustavo

2012-01-01

Most of the natural cases of pneumonia in feedlot cattle are characterized by a longer clinical course due to chronic lung lesions. Microscopically, these lesions include interstitial fibroplasia, bronchitis, bronchiectasis, bronchiolitis obliterans, and epithelial metaplasia of the airways. Herein, the aim was to review, under a medical perspective, the pathologic mechanisms operating in these chronic pneumonic lesions in calves. Based on the similarities of these changes to those reported in bronchiolitis obliterans/organising pneumonia (BO/OP) and chronic obstructive pulmonary disease (COPD) in human beings, calves are proposed as an alternative animal model. PMID:22629176

Dual antibiotics for bronchiectasis.

PubMed

Felix, Lambert M; Grundy, Seamus; Milan, Stephen J; Armstrong, Ross; Harrison, Haley; Lynes, Dave; Spencer, Sally

2018-06-11

Bronchiectasis is a chronic respiratory disease characterised by abnormal and irreversible dilatation of the smaller airways and associated with a mortality rate greater than twice that of the general population. Antibiotics serve as front-line therapy for managing bacterial load, but their use is weighed against the development of antibiotic resistance. Dual antibiotic therapy has the potential to suppress infection from multiple strains of bacteria, leading to more successful treatment of exacerbations, reduced symptoms, and improved quality of life. Further evidence is required on the efficacy of dual antibiotics in terms of management of exacerbations and extent of antibiotic resistance. To evaluate the effects of dual antibiotics in the treatment of adults and children with bronchiectasis. We identified studies from the Cochrane Airways Group Specialised Register (CAGR), which includes the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, the Cumulative Index to Nursing and Allied Health Literature (CINAHL), Allied and Complementary Medicine (AMED), and PsycINFO, as well as studies obtained by handsearching of journals/abstracts. We also searched the following trial registries: US National Institutes of Health Ongoing Trials Register, ClinicalTrials.gov, and the World Health Organization (WHO) International Clinical Trials Registry Platform. We imposed no restriction on language of publication. We conducted our search in October 2017. We searched for randomised controlled trials comparing dual antibiotics versus a single antibiotic for short-term (< 4 weeks) or long-term management of bronchiectasis diagnosed in adults and/or children by bronchography, plain film chest radiography, or high-resolution computed tomography. Primary outcomes included exacerbations, length of hospitalisation, and serious adverse events. Secondary outcomes were response rates, emergence of resistance to antibiotics, systemic markers of infection, sputum volume and purulence, measures of lung function, adverse events/effects, deaths, exercise capacity, and health-related quality of life. We did not apply outcome measures as selection criteria. Two review authors independently screened the titles and abstracts of 287 records, along with the full text of seven reports. Two studies met review inclusion criteria. Two review authors independently extracted outcome data and assessed risk of bias. We extracted data from only one study and conducted GRADE assessments for the following outcomes: successful treatment of exacerbation; response rates; and serious adverse events. Two randomised trials assessed the effectiveness of oral plus inhaled dual therapy versus oral monotherapy in a total of 118 adults with a mean age of 62.8 years. One multi-centre trial compared inhaled tobramycin plus oral ciprofloxacin versus ciprofloxacin alone, and one single-centre trial compared nebulised gentamicin plus systemic antibiotics versus a systemic antibiotic alone. Published papers did not report study funding sources.Effect estimates from one small study with 53 adults showed no evidence of treatment benefit with oral plus inhaled dual therapy for the following primary outcomes at the end of the study: successful management of exacerbation - cure at day 42 (odds ratio (OR) 0.66, 95% confidence interval (CI) 0.22 to 2.01; 53 participants; one study; very low-quality evidence); number of participants with Pseudomonas aeruginosa eradication at day 21 (OR 2.33, 95% CI 0.66 to 8.24; 53 participants; one study; very low-quality evidence); and serious adverse events (OR 0.48, 95% CI 0.08 to 2.87; 53 participants; one study; very low-quality evidence). Similarly, researchers provided no evidence of treatment benefit for the following secondary outcomes: clinical response rates - relapse at day 42 (OR 0.57, 95% CI 0.12 to 2.69; 53 participants; one study; very low-quality evidence); microbiological response rate at day 21 - eradicated (OR 2.40, 95% CI 0.67 to 8.65; 53 participants; one study; very low-quality evidence); and adverse events - incidence of wheeze (OR 5.75, 95% CI 1.55 to 21.33). Data show no evidence of benefit in terms of sputum volume, lung function, or antibiotic resistance. Outcomes from a second small study with 65 adults, available only as an abstract, were not included in the quantitative data synthesis. The included studies did not report our other primary outcomes: duration; frequency; and time to next exacerbation; nor our secondary outcomes: systemic markers of infection; exercise capacity; and quality of life. We did not identify any trials that included children. A small number of studies in adults have generated high-quality evidence that is insufficient to inform robust conclusions, and studies in children have provided no evidence. We identified only one dual-therapy combination of oral and inhaled antibiotics. Results from this single trial of 53 adults that we were able to include in the quantitative synthesis showed no evidence of treatment benefit with oral plus inhaled dual therapy in terms of successful treatment of exacerbations, serious adverse events, sputum volume, lung function, and antibiotic resistance. Further high-quality research is required to determine the efficacy and safety of other combinations of dual antibiotics for both adults and children with bronchiectasis, particularly in terms of antibiotic resistance.

Exhaled breath condensate MMP-9 levels in children with bronchiectasis.

PubMed

Karakoc, Gulbin Bingol; Inal, Ayfer; Yilmaz, Mustafa; Altintas, Derya Ufuk; Kendirli, Seval Guneser

2009-10-01

Bronchiectasis (BE) is still an important cause of chronic supurative respiratory diseases in developing countries. Neutrophil-derived proteases such as neutrophil elastase and matrix metalloproteases (MMPs) are implicated in causing airway damage in chronic pulmonary disease. In this study, we aimed to evaluate the MMP-9 and its natural tissue inhibitors of metalloproteinases (TIMP-1) levels utilizing the exhaled breath condensate (EBC) method and their relationship with radiological findings and pulmonary functions in children with BE.Thirty-eight children with BE and 12 healthy children were included: Group 1 (cystic fibrosis [CF] BE), Group 2 (non-CF BE), Group 3 (control group). High-resolution computerized tomography (HRCT) scores were calculated according to the anatomic extent of BE. Pulmonary function tests were performed, and MMP-9 and TIMP-1 levels in EBC were analyzed by ELISA.Exhaled breath condensate MMP-9 level was 48.9 +/- 26.8 ng/ml for Group 1, and for Group 2, 42.8 +/- 18.1 ng/ml; and for Group 3, 30 +/- 3.7 ng/ml. Although no statistically significant difference was found between the Groups 1 and 2, a significant difference was detected between these groups and controls. No statistically significant difference was found in TIMP-1 levels regarding all groups. EBC MMP-9 levels were inversely correlated with pulmonary functions test, and positively with HRCT scores and annual number of pulmonary infections.In conclusion, this study showed that EBC of children with both CF BE and non-CF BE contained higher levels of MMP-9 in comparison to controls. We suggest that EBC MMP-9 level may be a useful marker of airway injury in patients with BE however prospective studies are needed.

RESPIRE 2: a phase III placebo-controlled randomised trial of ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis.

PubMed

Aksamit, Timothy; De Soyza, Anthony; Bandel, Tiemo-Joerg; Criollo, Margarita; Elborn, J Stuart; Operschall, Elisabeth; Polverino, Eva; Roth, Katrin; Winthrop, Kevin L; Wilson, Robert

2018-01-01

We evaluated the efficacy and safety of ciprofloxacin dry powder for inhalation (DPI) in patients with non-cystic fibrosis bronchiectasis, two or more exacerbations in the previous year and predefined sputum bacteria.Patients were randomised 2:1 to twice-daily ciprofloxacin DPI 32.5 mg or placebo in 14- or 28-day on/off treatment cycles for 48 weeks. Primary end-points were time to first exacerbation and frequency of exacerbations. Enrolling countries and α level split (0.049 and 0.001 for 14- and 28-day cycles, respectively) differed from RESPIRE 1.Patients were randomised to ciprofloxacin DPI (14 days on/off (n=176) or 28 days on/off (n=171)) or placebo (14 days on/off (n=88) or 28 days on/off (n=86)). The exacerbation rate was low across treatment arms (mean±sd 0.6±0.9). Active treatment showed trends to prolonged time to first exacerbation (ciprofloxacin DPI 14 days on/off: hazard ratio 0.87, 95.1% CI 0.62-1.21; p=0.3965; ciprofloxacin DPI 28 days on/off: hazard ratio 0.71, 99.9% CI 0.39-1.27; p=0.0511) and reduced frequency of exacerbations (ciprofloxacin DPI 14 days on/off: incidence rate ratio 0.83, 95.1% CI 0.59-1.17; p=0.2862; ciprofloxacin DPI 28 days on/off: incidence rate ratio 0.55, 99.9% CI 0.30-1.02; p=0.0014), although neither achieved statistical significance. Ciprofloxacin DPI was well tolerated.Trends towards clinical benefit were seen with ciprofloxacin DPI, but primary end-points were not met. Copyright ©ERS 2018.

Clinical Features of Childhood Primary Ciliary Dyskinesia by Genotype and Ultrastructural Phenotype

PubMed Central

Ferkol, Thomas W.; Rosenfeld, Margaret; Lee, Hye-Seung; Dell, Sharon D.; Sagel, Scott D.; Milla, Carlos; Zariwala, Maimoona A.; Pittman, Jessica E.; Shapiro, Adam J.; Carson, Johnny L.; Krischer, Jeffrey P.; Hazucha, Milan J.; Cooper, Matthew L.; Knowles, Michael R.; Leigh, Margaret W.

2015-01-01

Rationale: The relationship between clinical phenotype of childhood primary ciliary dyskinesia (PCD) and ultrastructural defects and genotype is poorly defined. Objectives: To delineate clinical features of childhood PCD and their associations with ultrastructural defects and genotype. Methods: A total of 118 participants younger than 19 years old with PCD were evaluated prospectively at six centers in North America using standardized procedures for diagnostic testing, spirometry, chest computed tomography, respiratory cultures, and clinical phenotyping. Measurements and Main Results: Clinical features included neonatal respiratory distress (82%), chronic cough (99%), and chronic nasal congestion (97%). There were no differences in clinical features or respiratory pathogens in subjects with outer dynein arm (ODA) defects (ODA alone; n = 54) and ODA plus inner dynein arm (IDA) defects (ODA + IDA; n = 18) versus subjects with IDA and central apparatus defects with microtubular disorganization (IDA/CA/MTD; n = 40). Median FEV1 was worse in the IDA/CA/MTD group (72% predicted) versus the combined ODA groups (92% predicted; P = 0.003). Median body mass index was lower in the IDA/CA/MTD group (46th percentile) versus the ODA groups (70th percentile; P = 0.003). For all 118 subjects, median number of lobes with bronchiectasis was three and alveolar consolidation was two. However, the 5- to 11-year-old IDA/CA/MTD group had more lobes of bronchiectasis (median, 5; P = 0.0008) and consolidation (median, 3; P = 0.0001) compared with the ODA groups (median, 3 and 2, respectively). Similar findings were observed when limited to participants with biallelic mutations. Conclusions: Lung disease was heterogeneous across all ultrastructural and genotype groups, but worse in those with IDA/CA/MTD ultrastructural defects, most of whom had biallelic mutations in CCDC39 or CCDC40. PMID:25493340

Automated airway evaluation system for multi-slice computed tomography using airway lumen diameter, airway wall thickness and broncho-arterial ratio

NASA Astrophysics Data System (ADS)

Odry, Benjamin L.; Kiraly, Atilla P.; Novak, Carol L.; Naidich, David P.; Lerallut, Jean-Francois

2006-03-01

Pulmonary diseases such as bronchiectasis, asthma, and emphysema are characterized by abnormalities in airway dimensions. Multi-slice computed tomography (MSCT) has become one of the primary means to depict these abnormalities, as the availability of high-resolution near-isotropic data makes it possible to evaluate airways at oblique angles to the scanner plane. However, currently, clinical evaluation of airways is typically limited to subjective visual inspection only: systematic evaluation of the airways to take advantage of high-resolution data has not proved practical without automation. We present an automated method to quantitatively evaluate airway lumen diameter, wall thickness and broncho-arterial ratios. In addition, our method provides 3D visualization of these values, graphically illustrating the location and extent of disease. Our algorithm begins by automatic airway segmentation to extract paths to the distal airways, and to create a map of airway diameters. Normally, airway diameters decrease as paths progress distally; failure to taper indicates abnormal dilatation. Our approach monitors airway lumen diameters along each airway path in order to detect abnormal profiles, allowing even subtle degrees of pathologic dilatation to be identified. Our method also systematically computes the broncho-arterial ratio at every terminal branch of the tree model, as a ratio above 1 indicates potentially abnormal bronchial dilatation. Finally, the airway wall thickness is computed at corresponding locations. These measurements are used to highlight abnormal branches for closer inspection, and can be summed to compute a quantitative global score for the entire airway tree, allowing reproducible longitudinal assessment of disease severity. Preliminary tests on patients diagnosed with bronchiectasis demonstrated rapid identification of lack of tapering, which also was confirmed by corresponding demonstration of elevated broncho-arterial ratios.

A randomised controlled crossover trial of nurse practitioner versus doctor led outpatient care in a bronchiectasis clinic

PubMed Central

Sharples, L; Edmunds, J; Bilton, D; Hollingworth, W; Caine, N; Keogan, M; Exley, A

2002-01-01

Background: With the decrease in junior doctor hours, the advent of specialist registrars, and the availability of highly trained and experienced nursing personnel, the service needs of patients with chronic respiratory diseases attending routine outpatient clinics may be better provided by appropriately trained nurse practitioners. Methods: A randomised controlled crossover trial was used to compare nurse practitioner led care with doctor led care in a bronchiectasis outpatient clinic. Eighty patients were recruited and randomised to receive 1 year of nurse led care and 1 year of doctor led care in random order. Patients were followed up for 2 years to ensure patient safety and acceptability and to assess differences in lung function. Outcome measures were forced expiratory volume in 1 second (FEV1), 12 minute walk test, health related quality of life, and resource use. Results: The mean difference in FEV1 was 0.2% predicted (95% confidence interval –1.6 to 2.0%, p=0.83). There were no significant differences in the other clinical or health related quality of life measures. Nurse led care resulted in significantly increased resource use compared with doctor led care (mean difference £1497, 95% confidence interval £688 to £2674, p<0.001), a large part of which resulted from the number and duration of hospital admissions. The mean difference in resource use was greater in the first year (£2625) than in the second year (£411). Conclusions: Nurse practitioner led care for stable patients within a chronic chest clinic is safe and is as effective as doctor led care, but may use more resources. PMID:12149523

Presence of anxiety and depression in patients with bronchiectasis unrelated to cystic fibrosis.

PubMed

Girón Moreno, Rosa María; Fernandes Vasconcelos, Gilda; Cisneros, Carolina; Gómez-Punter, Rosa Mar; Segrelles Calvo, Gonzalo; Ancochea, Julio

2013-10-01

Patients with chronic bronchiectasis (BQ) may suffer from psychological disorders. The objective of this study was to assess the presence of anxiety and depression in patients from a specialised BQ Unit, using validated questionnaires. We included patients consecutively diagnosed with BQ (unrelated to cystic fibrosis) by high resolution computed tomography in the study. Patients were clinically stable in the previous three weeks and voluntarily completed the Beck Depression Inventory, State-Trait Anxiety Inventory and St. George's Respiratory Questionnaire, after signing the informed consent. They were classified according to their scores on the psychological screening questionnaires, and their results were compared with the clinical, radiological and functional parameters and Quality of Life. Seventy patients were included, 48 women and 22 men, with a mean age of 64.19years. Thirty-four percent (34%) of patients showed symptoms of depression, and around 55% had scores above the 50th percentile in trait and state anxiety. The amount of sputum was associated with trait anxiety. Bacterial colonization was related to anxiety (trait and state), especially Pseudomonas aeruginosa colonization. Female patients showed a higher risk of depression. There was no relationship between the Quality of Life scores and the established classifications of anxiety and depression. A high percentage of patients with BQ presented anxiety (trait and state) and depression. The daily sputum production and bacterial colonization (especially with P. aeruginosa) were the variables most related to anxiety; depression was more common in women. We believe that the presence of psychological disorders should be evaluated, especially in patients with this profile. Copyright © 2012 SEPAR. Published by Elsevier Espana. All rights reserved.

Novel loss-of-function variants in DIAPH1 associated with syndromic microcephaly, blindness, and early onset seizures.

PubMed

Al-Maawali, Almundher; Barry, Brenda J; Rajab, Anna; El-Quessny, Malak; Seman, Ann; Coury, Stephanie Newton; Barkovich, A James; Yang, Edward; Walsh, Christopher A; Mochida, Ganeshwaran H; Stoler, Joan M

2016-02-01

Exome sequencing identified homozygous loss-of-function variants in DIAPH1 (c.2769delT; p.F923fs and c.3145C>T; p.R1049X) in four affected individuals from two unrelated consanguineous families. The affected individuals in our report were diagnosed with postnatal microcephaly, early-onset epilepsy, severe vision impairment, and pulmonary symptoms including bronchiectasis and recurrent respiratory infections. A heterozygous DIAPH1 mutation was originally reported in one family with autosomal dominant deafness. Recently, however, a homozygous nonsense DIAPH1 mutation (c.2332C4T; p.Q778X) was reported in five siblings in a single family affected by microcephaly, blindness, early onset seizures, developmental delay, and bronchiectasis. The role of DIAPH1 was supported using parametric linkage analysis, RNA and protein studies in their patients' cell lines and further studies in human neural progenitors cells and a diap1 knockout mouse. In this report, the proband was initially brought to medical attention for profound metopic synostosis. Additional concerns arose when his head circumference did not increase after surgical release at 5 months of age and he was diagnosed with microcephaly and epilepsy at 6 months of age. Clinical exome analysis identified a homozygous DIAPH1 mutation. Another homozygous DIAPH1 mutation was identified in the research exome analysis of a second family with three siblings presenting with a similar phenotype. Importantly, no hearing impairment is reported in the homozygous affected individuals or in the heterozygous carrier parents in any of the families demonstrating the autosomal recessive microcephaly phenotype. These additional families provide further evidence of the likely causal relationship between DIAPH1 mutations and a neurodevelopmental disorder. © 2016 Wiley Periodicals, Inc.

Constrictive Bronchiolitis in Cystic Fibrosis Adolescents with Refractory Pulmonary Decline.

PubMed

Harris, William T; Boyd, J Todd; McPhail, Gary L; Brody, Alan S; Szczesniak, Rhonda D; Korbee, Leslie L; Baker, Michael L; Clancy, John P

2016-12-01

Refractory lung function decline in association with recurrent pulmonary exacerbations is a common, yet poorly explained finding in cystic fibrosis (CF). To investigate the histopathologic mechanisms of pulmonary deterioration during adolescence and early adulthood, we reviewed clinically-indicated lung biopsy specimens obtained during a period of persistent decline. To determine if peribronchiolar remodeling is prominent in lung biopsy specimens obtained in adolescents with CF refractory to conventional therapy. Six adolescents with CF (mean age, 16.2 y; mean FEV 1 , 52% predicted at biopsy) with significant pulmonary deterioration over 12-24 months (mean FEV 1 decline of 14% predicted/year) despite aggressive intervention underwent computed tomography imaging and ultimately lung biopsy to aid clinical management. In addition to routine clinical evaluation, histopathologic investigation included staining for transforming growth factor-β (TGF-β, a genetic modifier of CF lung disease), collagen deposition (a marker of fibrosis), elastin (to evaluate for bronchiectasis), and α-smooth muscle actin (to identify myofibroblasts). All computed tomography scans demonstrated a mix of bronchiectasis and hyperinflation that was variable across lung regions and within patients. Lung biopsy revealed significant peribronchiolar remodeling, particularly in patients with more advanced disease, with near complete obliteration of the peribronchiolar lumen (constrictive bronchiolitis). Myofibroblast differentiation (a TGF-β-dependent process) was prominent in specimens with significant airway remodeling. Constrictive bronchiolitis is widely present in the lung tissue of adolescents with CF with advanced disease and may contribute to impaired lung function that is refractory to conventional therapy (antibiotics, antiinflammatories, and mucolytics). TGF-β-dependent myofibroblast differentiation is prominent in areas of active fibrogenesis and may foster small airway remodeling in CF lung disease.

Computed tomography correlates with improvement with ivacaftor in cystic fibrosis patients with G551D mutation.

PubMed

Sheikh, Shahid I; Long, Frederick R; McCoy, Karen S; Johnson, Terri; Ryan-Wenger, Nancy A; Hayes, Don

2015-01-01

Ivacaftor corrects the cystic fibrosis transmembrane conductance regulator (CFTR) gating defect associated with G551D mutation and is quickly becoming an important treatment in patients with cystic fibrosis (CF) due to this genetic mutation. A single-center study was performed in CF patients receiving ivacaftor to evaluate the usefulness of high resolution computed tomography (HRCT) of the chest as a way to gauge response to ivacaftor therapy. Ten patients with CF were enrolled for at least one year before and after starting ivacaftor. At time of enrollment, mean age was 20.9 ± 10.8 (range 10-44) years. There were significant improvements from baseline to 6 months in mean %FVC (93 ± 16 to 99 ± 16) and %FEV1 (79 ± 26 to 87 ± 28) but reverted to baseline at one year. Mean sweat chloride levels decreased significantly from baseline to one year. Mean weight and BMI improved at 6 months. Weight continued to improve with stabilization of BMI at one year. Chest HRCT showed significant improvement at one year in mean modified Brody scores for bronchiectasis, mucous plugging, airway wall thickness, and total Brody scores. Elevated bronchiectasis and airway wall thickness scores correlated significantly with lower %FEV1, while higher airway wall thickness and mucus plugging scores correlated with more pulmonary exacerbations requiring IV and oral antibiotics respectively. Based on our findings, HRCT imaging is a useful tool in monitoring response to ivacaftor therapy that corrects the gating defect associated with the G551D-CFTR mutation. Copyright © 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Aberrant Left Inferior Bronchial Artery Originating from the Left Gastric Artery in a Patient with Acute Massive Hemoptysis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Jiang, Sen, E-mail: jasfly77@vip.163.com; Sun, Xi-Wen, E-mail: xwsun@citiz.net; Yu, Dong, E-mail: yudong_mail@126.com

Massive hemoptysis is a life-threatening condition, and the major source of bleeding in this condition is the bronchial circulation. Bronchial artery embolization is a safe and effective treatment for controlling hemoptysis. However, the sites of origin of the bronchial arteries (BAs) have numerous anatomical variations, which can result in a technical challenge to identify a bleeding artery. We present a rare case of a left inferior BA that originated from the left gastric artery in a patient with recurrent massive hemoptysis caused by bronchiectasis. The aberrant BA was embolized, and hemoptysis has been controlled for 8 months.

[Indication, type of resection and results of surgery in cases of lung tuberculosis. A historical and regional overview].

PubMed

Hillejan, L; Nemat, A; Marra, A; Stamatis, G

2002-06-01

Pulmonary tuberculosis has become a rare indication for surgical intervention in all industrial nations. Over a period of 10 years we overview 193 patients who were suffering this disease and underwent thoracotomy. Main indication (79.8 %) was pulmonary nodules, of unknown origin. In this cases wedge resection was performed. Expanded resectional techniques were necessary in cases of cavernes, superinfected bronchiectasis, bronchial stenosis, hemoptysis and destroyed lungs due to tuberculosis. Considering the heterogenous groups of patients, the perioperative morbidity (21.8 %) and mortality (0.5 %) has to be regarded in comparison to the data found in the literature.

Diagnosis of Adult Patients with Cystic Fibrosis.

PubMed

Nick, Jerry A; Nichols, David P

2016-03-01

The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis. Copyright © 2016 Elsevier Inc. All rights reserved.

Bronchopulmonary levage to remove pulmonary casts and plugs.

PubMed

Passy, V; Ermshar, C

1976-04-01

Therapy for such obstructive pulmonary lung diseases as alveolar proteinosis, pneumonia, asthma, bronchiectasis, and cystic fibrosis, ranges from medical therapy to inhalation therapy to direct bronchoscopy and irrigation. The basic pathophysiologic feature is the retention of mucous secretions and foreign material within the lungs and therapy directed to removal of these secretions. Bronchopulmonary lavage recently has been suggested as an effective tool toward resolution of these illnesses. The otolaryngologist, who is adept in endoscopy, is asked to perform bronchoscopic procedures with washings to aid in the alleviation of these obstructive pulmonary diseases. This paper deals with the effectiveness of massive bronchopulmonary lavage in the elimination of severe chronic obstructive pulmonary lung diseases.

High rhinovirus burden in lower airways of children with cystic fibrosis.

PubMed

Kieninger, Elisabeth; Singer, Florian; Tapparel, Caroline; Alves, Marco P; Latzin, Philipp; Tan, Hui-Leng; Bossley, Cara; Casaulta, Carmen; Bush, Andrew; Davies, Jane C; Kaiser, Laurent; Regamey, Nicolas

2013-03-01

Rhinovirus (RV)-induced pulmonary exacerbations are common in cystic fibrosis (CF) and have been associated with impaired virus clearance by the CF airway epithelium in vitro. Here, we assess in vivo the association of RV prevalence and load with antiviral defense mechanisms, airway inflammation, and lung function parameters in children with CF compared with a control group and children with other chronic respiratory diseases. RV presence and load were measured by real-time reverse transcription-polymerase chain reaction in BAL samples and were related to antiviral and inflammatory mediators measured in BAL and to clinical parameters. BAL samples were obtained from children with CF (n = 195), non-CF bronchiectasis (n = 40), or asthma (n = 29) and from a control group (n = 35) at a median (interquartile range [IQR]) age of 8.2 (4.0-11.7) years. RV was detected in 73 samples (24.4%). RV prevalence was similar among groups. RV load (median [IQR] x 10(3) copies/mL) was higher in children with CF (143.0 [13.1-1530.0]), especially during pulmonary exacerbations, compared with children with asthma (3.0 [1.3-25.8], P = .006) and the control group (0.5 [0.3-0.5], P < .001), but similar to patients with non-CF bronchiectasis (122.1 [2.7-4423.5], P = not significant). In children with CF, RV load was negatively associated with interferon (IFN)- b and IFN- l , IL-1ra levels, and FEV 1 , and positively with levels of the cytokines CXCL8 and CXCL10. RV load in CF BAL is high, especially during exacerbated lung disease. Impaired production of antiviral mediators may lead to the high RV burden in the lower airways of children with CF. Whether high RV load is a cause or a consequence of inflammation needs further investigation in longitudinal studies.

[Imaging origins and characteristics analysis of acute and chronic aspiration pneumonia].

PubMed

Wang, Kang; Li, Ming; Wang, Xiongbiao; Qin, Jianmin; Wang, Zhi; Zhao, Zehua; Qin, Le; Hua, Yanqing

2014-11-11

To discuss about the pathologic and imaging origins and characteristics of CT scaning and X-ray radiography for acute and chronic aspiration pneumonia. Imaging data from 30 patients with aspiration pneumonia were retrospectively analyzed, CT scaning was performed in 27 patients, which PMVR reconstruction was performed in 21 cases;3 exammed by X-ray with 2 used by esophagography. Opaque bodies were detected in trachea by CT scaning in 12 patients.7 patients in acute phase rapidly developed into acute respiratory distress syndrome(ARDS). CT signs of 30 patients with acute and chronic aspiration pneumonia included: centrilobular nodules were detected in 2 cases with acute phase, 4 cases with subacute phase and 4 cases with chronic phase; the imaging of ground glass opacity were detected in 9 cases with acute phase, 2 cases with subacute phase and 3 cases with chronic phase; the imaging of bronchiectasis was detected in 8 cases with chronic phase, which mucilage embolism was detected in 3 of 8 cases; the imaging of atelectasis was detected in 6 cases with chronic phase; the imaging of sheeted consolidation was detected in 5 cases with chronic phase, 8 case with acute phase; the imaging of interstitial fibrosis was detected in 3 cases with chronic phase. Lesions of inferior lobe of right lung were detected in 9 cases with chronic phase, 4 cases with subacute phase, 11 case with acute phase;lesions of inferior lobe of left lung were detected in 6 cases with chronic phase and 3 cases with subacute group, 11 case with acute phase. The imaging features of acute and chronic aspiration pneumonia overlap with GGO and centrilobular nodules in every group. While the imaging features of atelectasis, bronchiectasis or mucilage embolism are found in chronic phase. The chest CT scaning may accurately evaluate the dynamic change of aspiration pneumonia.

The RESPIRE trials: Two phase III, randomized, multicentre, placebo-controlled trials of Ciprofloxacin Dry Powder for Inhalation (Ciprofloxacin DPI) in non-cystic fibrosis bronchiectasis.

PubMed

Aksamit, Timothy; Bandel, Tiemo-Joerg; Criollo, Margarita; De Soyza, Anthony; Elborn, J Stuart; Operschall, Elisabeth; Polverino, Eva; Roth, Katrin; Winthrop, Kevin L; Wilson, Robert

2017-07-01

The primary goals of long-term disease management in non-cystic fibrosis bronchiectasis (NCFB) are to reduce the number of exacerbations, and improve quality of life. However, currently no therapies are licensed for this. Ciprofloxacin Dry Powder for Inhalation (Ciprofloxacin DPI) has potential to be the first long-term intermittent therapy approved to reduce exacerbations in NCFB patients. The RESPIRE programme consists of two international phase III prospective, parallel-group, randomized, double-blinded, multicentre, placebo-controlled trials of the same design. Adult patients with idiopathic or post-infectious NCFB, a history of ≥2 exacerbations in the previous 12months, and positive sputum culture for one of seven pre-specified pathogens, undergo stratified randomization 2:1 to receive twice-daily Ciprofloxacin DPI 32.5mg or placebo using a pocket-sized inhaler in one of two regimens: 28days on/off treatment or 14days on/off treatment. The treatment period is 48weeks plus an 8-week follow-up after the last dose. The primary efficacy endpoints are time to first exacerbation after treatment initiation and frequency of exacerbations using a stringent definition of exacerbation. Secondary endpoints, including frequency of events using different exacerbation definitions, microbiology, quality of life and lung function will also be evaluated. The RESPIRE trials will determine the efficacy and safety of Ciprofloxacin DPI. The strict entry criteria and stratified randomization, the inclusion of two treatment regimens and a stringent definition of exacerbation should clarify the patient population best positioned to benefit from long-term inhaled antibiotic therapy. Additionally RESPIRE will increase understanding of NCFB treatment and could lead to an important new therapy for sufferers. The RESPIRE trials are registered in ClinicalTrials.gov, ID number NCT01764841 (RESPIRE 1; date of registration January 8, 2013) and NCT02106832 (RESPIRE 2; date of registration April 4, 2014). Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

The relationship of SSRI and SNRI usage with interstitial lung disease and bronchiectasis in an elderly population: a case–control study

PubMed Central

Rosenberg, Ted; Lattimer, Rory; Montgomery, Patrick; Wiens, Christian; Levy, Liran

2017-01-01

Background The association between interstitial lung disease (ILD) and selective serotonin reuptake inhibitors and serotonin norepinephrine reuptake inhibitors (SSRI/SNRI) has been previously described in published case reports. However, its prevalence may be more common than expected. We examined the association between SSRI/SNRI usage and presence of ILD and or bronchiectasis (ILD/B) in an elderly population. Methods We conducted a retrospective case series and case–control study involving all 296 eligible elderly patients in one primary care geriatric practice in Victoria, BC, Canada. Cases required the presence of ILD/B on computed tomography (CT) or chest X-ray (CXR). Cases were excluded if they had other causes for ILD/B on CXR or CT such as exposure to known pneumotoxic drugs, metastatic cancer, rheumatoid lung disease, sarcoidosis, previous pulmonary tuberculosis, or pneumoconiosis. Data were abstracted from the patients’ medical record. The exposure variable was standardized cumulative person-month (p-m) dose of SSRI/SNRI. The study was approved by the Clinical Research Ethics Board of University of British Columbia with a waiver of informed consent. Results A total of 12 cases and 273 controls were identified. Their mean ages were 89.0 and 88.7 years, respectively (p=0.862). A total of 10/12 cases and 99/273 controls were exposed to SSRI/SNRI. The odds ratio was 8.79, 95% confidence interval 2.40–32.23 (p=0.001). The median p-m exposure to SSRI/SNRI was 110.0 months for cases and 29.5 for controls (p=0.003). Conclusion SSRIs and SNRIs were significantly associated with the risk of ILD/B in this elderly population. Because of their widespread usage, further studies should be done to validate these findings. Prescribers should cautiously monitor patients for development of insidious pulmonary symptoms when these drugs are used. PMID:29200837

[Study of quality of life in adults with common variable immunodeficiency by using the Questionnaire SF-36].

PubMed

López-Pérez, Patricia; Miranda-Novales, Guadalupe; Segura-Méndez, Nora Hilda; Del Rivero-Hernández, Leonel; Cambray-Gutiérrez, Cesar; Chávez-García, Aurora

2014-01-01

Quality of life is a multidimensional concept that includes physical, emotional and social components associated with the disease. The use of tools to assess the Quality of Life Health Related (HRQOL) has increased in recent decades. Common variable immunodeficiency (CVID) is the most commonly diagnosed primary immunodeficiency. To evaluate the quality of life in patients with CVID using the questionnaire SF -36. A descriptive cross-sectional survey included 23 patients diagnosed with CVID, belonging to the Immunodeficiency Clinic Service of Allergology and Clinical Immunology in CMN Siglo XXI, IMSS. The questionnaire SF- 36 validated in Spanish was applied. descriptive statistics with simple frequencies and percentages, inferential statistics: Fisher exact test and ANOVA to compare means. The study involved 23 patients, 14 women (60%) and 9 men (40%), mean age 38.6 ± 14.7 years. The highest score was obtained in 83% emotional role. Dimensions with further deterioration in both genders were: 54% general health, vitality 59% and physical performance 72%. No differences were found regarding gender. The only issue in which statistically significant differences were found in patients with more than 3 comorbidities was change in health status in the past year (p=0.007). Patients with severe comorbidities, such as haematologicaloncological (leukemias, lymphomas, neoplasms), and pulmonary (severe bronchiectasis) showed further deterioration in the aspects of physical performance 73% and 64% emotional role. 65% of patients reported an improvement in health status in 74% in the last year. Adult patients with CVID show deterioration in different dimensions, particularly in the areas of general health, vitality and physical performance. Patients with severe comorbidities such as leukemia, lymphomas, malignancies and severe bronchiectasis show further deterioration in some aspects of quality of life, especially in physical performance and emotional role. A higher number of comorbidities was significantly associated with a lower score in changing health. The questionnaire SF-36 is useful for evaluating the quality of life of our patients with CVID.

Quality of life in children with non-cystic-fibrosis bronchiectasis.

PubMed

Gokdemir, Yasemin; Hamzah, Ameer; Erdem, Ela; Cimsit, Cagatay; Ersu, Refika; Karakoc, Fazilet; Karadag, Bulent

2014-01-01

Non-cystic-fibrosis bronchiectasis (non-CF BE) continues to be a problem in developing countries and it is therefore important to examine and assess this disease. The aims of this prospective study were to evaluate the health-related quality of life (HRQOL) in non-CF BE children and also to assess the risk factors associated with HRQOL. Forty-two non-CF BE patients between the ages of 9 and 18 years were enrolled in the study. All recruited patients completed the generic Short-Form-36 (SF-36), the St. George's Respiratory Questionnaire (SGRQ) for disease-specific QOL scale and forms on socioeconomic status (SES). The extent and severity of CT abnormalities were evaluated by using the modified Bhalla scoring system. Association between HRQOL questionnaires and demographic variables, pulmonary function test, high-resolution CT scores and SES were evaluated. SF-36 and SGRQ subscales all correlated inversely with each other (SF-36 physical component summary with SGRQ symptoms score: r = -0.466, p = 0.001, activity score: r = -0.666, p = 0.000 and impact score: r = -0.667, p = 0.000. SF-36 mental component summary with SGRQ symptoms score: r = -0.396, p = 0.005, activity score: r = -0.533, p = 0.000 and impact score: r = -0.512, p = 0.000). There was an inverse correlation between SGRQ symptoms scores and the duration of regular follow-up (r = -0.3, p = 0.04). The symptoms subscale of SGRQ correlated positively with low values for pulmonary function testing (r = -0.417, p = 0.003) and frequent antibiotic requirements (r = 0.303, p = 0.035). Early diagnosis and regular follow-up of children with non-CF BE is important for improving their QOL. As expected, the severity and frequency of symptoms are inversely related to the pulmonary function and the QOL scores. A disease-specific questionnaire should be developed to monitor QOL in children with non-CF BE. © 2014 S. Karger AG, Basel

[Domiciliary noninvasive positive pressure ventilation in chronic alveolar hypoventilation].

PubMed

Casas, J P; Robles, A M; Pereyra, M A; Abbona, H L; López, A M

2000-01-01

Effectiveness of treatment with domiciliary nocturnal noninvasive positive pressure ventilation is analyzed in a group of patients with chronic alveolar hypoventilation of different etiologies. It was applied with two levels of pressure (BiPAP) via nasal mask. Criteria for evaluation were symptomatology and improvement in gas exchange. Data were analyzed by Student t tests. A total of 13 patients were included, mean age 55.7 range 20 to 76 years (5 male 8 female). Main diagnosis was tuberculosis in 6, four of them having had surgical procedure (thoracoplasty 2, frenicectomy 1 and neumonectomy 1), myopathy 3 (myasthenia gravis 1, muscular dystrophy 1 and diaphragmatic paralysis 1), obesity-hypoventilation syndrome 1, escoliosis 1, bronchiectasis 1 and cystic fibrosis 1. These last two patients were on waiting list for lung transplantation. At the moment of consultation, the symptoms were: dysnea 13/13 (100%), astenia 13/13 (100%), hypersomnolency 10/13 (77%), cephalea 9/13 (69%), leg edema 6/13 (46%), loss of memory 6/13 (46%). Regarding gas exchange, they showed hypoxemia and hypercapnia. Mean follow up was of 2.2 years (range 6 months to 4 years). Within the year, all 13 patients became less dyspneic. Astenia, hypersomnolency, cephalea, leg edema and memory loss disappeared. Improvement in gas exchange was: PaO2/FiO2 from 269 +/- 65.4 (basal) to 336.7 +/- 75.3 post-treatment (p = 0.0018). PaCO2 from 70.77 +/- 25.48 mmHg (basal) to 46.77 +/- 8.14 mmHg (p = 0.0013). Ventilatory support was discontinued en 5 patients: three because of pneumonia requiring intubation and conventional mechanical ventilation, two of them died and one is still with tracheostomy; One patient with bronchiectasis and one with cystic fibrosis were transplanted. The remaining eight patients are stable. In conclusion, chronic alveolar hypoventilation can be effectively treated with domiciliary nocturnal noninvasive ventilation. Long term improvement in symptomatology and arterial blood gases can be obtained without significant complications.

Middle lobe syndrome in children today.

PubMed

Romagnoli, Vittorio; Priftis, Kostas N; de Benedictis, Fernando M

2014-06-01

Middle lobe syndrome in children is a distinct clinical and radiographic entity that has been well described in the pediatric literature. However, issues regarding its etiology, clinical presentation, and management continue to puzzle the clinical practitioner. Pathophysiologically, there are two forms of middle lobe syndrome, namely obstructive and nonobstructive. Middle lobe syndrome may present as symptomatic or asymptomatic, as persistent or recurrent atelectasis, or as pneumonitis or bronchiectasis of the middle lobe and/or lingula. A lower threshold of performing a chest radiograph is warranted in children with persistent or recurrent nonspecific respiratory symptoms, particularly if there is clinical deterioration, in order to detect middle lobe syndrome and to initiate a further diagnostic and therapeutic workup. Copyright © 2014 Elsevier Ltd. All rights reserved.

[Infection due to Rothia mucilaginosa. A respiratory pathogen?].

PubMed

Ramos, José M; Mateo, Ignacio; Vidal, Inmaculada; Rosillo, Eva M; Merino, Esperanza; Portilla, Joaquín

2014-05-01

To describe the spectrum of infections caused by Rothia mucilaginosa. Retrospective study of 20 cases diagnosed with R. mucilaginosa from 2009 to 2012. Pulmonary infection was the most frequent clinical presentation (n=14, 70%): bronchiectasis infected (10), followed by pleural empyema (2), pneumonia (1) and acute bronchitis (1). Two episodes were of gastrointestinal origin: cholangitis secondary to biliary drainage and secondary peritonitis. Two episodes included bacteremia in patients with hematological malignancy. One patient had a surgical wound infection with bacteremia, and another had a bacteremic urinary tract infection in a patient with nephrostomy. R. mucilaginosa may be responsible for infections of the lower respiratory tract in predisposed patients. Copyright © 2013 Elsevier España, S.L. y Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica. All rights reserved.

Rothia mucilaginosa pneumonia in an immunocompetent patient.

PubMed

Baeza Martínez, Carlos; Zamora Molina, Lucia; García Sevila, Raquel; Gil Carbonell, Joan; Ramos Rincon, José Manuel; Martín Serrano, Concepción

2014-11-01

Rothia mucilaginosa is a gram-postive coccus that occurs as part of the normal flora of the oropharynx and upper respiratory tract. Lower respiratory tract infections caused by this organism are rare and usually occur in immunocompromised patients. This is the case of an immunocompetent 47-year-old woman with right upper lobe pneumonia in which R.mucilaginosa was isolated in sputum and bronchial aspirate. Infections caused by this agent in the last four years in our hospital were reviewed. The most common predisposing factor was COPD with bronchiectasis. R.mucilaginosa was identified as the causative agent for pneumonia in only two cases, of which one was our case and the other was a patient with lung cancer. Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.

Description of a new rare alpha-1 antitrypsin mutation in Naples (Italy): PI*M S-Napoli

PubMed Central

Mosella, Marco; Accardo, Mariasofia; Molino, Antonio; Maniscalco, Mauro; Zamparelli, Alessandro Sanduzzi

2018-01-01

Alpha-1 antitrypsin deficiency is a rare and often underdiagnosed hereditary disorder, which mainly affects the Caucasian population. We report a case of a noncystic fibrosis bronchiectasis patient in the absence of emphysema associated with low serum alpha-1-antitrypsin (AAT) level, in the absence of the most common defective alleles associated with AAT deficiency (PI*S and PI*Z) but with a new mutation in heterozygosis. This mutation is characterized by the substitution in the coding region of exon 3, of a guanine (G) for a thymine (T), generating the replacement of a glutamine (Gln) by a histidine (His) in codon 212 (cod 212 GlnCAG > HisCAT), corresponds to a new S allelic variant. This mutation, never identified before, is called S-Napoli. PMID:29387258

Radiological signs associated with pulmonary multi-drug resistant tuberculosis: an analysis of published evidences.

PubMed

Wáng, Yì Xiáng J; Chung, Myung Jin; Skrahin, Aliaksandr; Rosenthal, Alex; Gabrielian, Andrei; Tartakovsky, Michael

2018-03-01

Despite that confirmative diagnosis of pulmonary drug-sensitive tuberculosis (DS-TB) and multidrug resistant tuberculosis (MDR-TB) is determined by microbiological testing, early suspicions of MDR-TB by chest imaging are highly desirable in order to guide diagnostic process. We aim to perform an analysis of currently available literatures on radiological signs associated with pulmonary MDR-TB. A literature search was performed using PubMed on January 29, 2018. The search words combination was "((extensive* drug resistant tuberculosis) OR (multidrug-resistant tuberculosis)) AND (CT or radiograph or imaging or X-ray or computed tomography)". We analyzed English language articles reported sufficient information of radiological signs of DS-TB vs. MDR-TB. Seventeen articles were found to be sufficiently relevant and included for analysis. The reported pulmonary MDR-TB cases were grouped into four categories: (I) previously treated (or 'secondary', or 'acquired') MDR-TB in HIV negative (-) adults; (II) new (or 'primary') MDR-TB in HIV(-) adults; (III) MDR-TB in HIV positive (+) adults; and (IV) MDR-TB in child patients. The common radiological findings of pulmonary MDR-TB included centrilobular small nodules, branching linear and nodular opacities (tree-in-bud sign), patchy or lobular areas of consolidation, cavitation, and bronchiectasis. While overall MDR-TB cases tended to have more extensive disease, more likely to be bilateral, to have pleural involvement, to have bronchiectasis, and to have lung volume loss; these signs alone were not sufficient for differential diagnosis of MDR-TB. Current literatures suggest that the radiological sign which may offer good specificity for pulmonary MDR-TB diagnosis, though maybe at the cost of low sensitivity, would be thick-walled multiple cavities, particularly if the cavity number is ≥3. For adult HIV(-) patients, new MDR-TB appear to show similar prevalence of cavity lesion, which was estimated to be around 70%, compared with previously treated MDR-TB. Thick-walled multiple cavity lesions present the most promising radiological sign for MDR-TB diagnosis. For future studies cavity lesion characteristics should be quantified in details.

Outcome of domiciliary nasal intermittent positive pressure ventilation in restrictive and obstructive disorders.

PubMed Central

Simonds, A. K.; Elliott, M. W.

1995-01-01

BACKGROUND--Nasal intermittent positive pressure ventilation (NIPPV) is a new technique which has rapidly supplanted other non-invasive methods of ventilation over the last 5-10 years. Data on its effectiveness are limited. METHODS--The outcome of long term domiciliary NIPPV has been analysed in 180 patients with hypercapnic respiratory failure predominantly due to chest wall restriction, neuromuscular disorders, or chronic obstructive lung disease. One hundred and thirty eight patients were started on NIPPV electively, and 42 following an acute hypercapnic exacerbation. Outcome measures were survival (five year probability of continuing NIPPV), pulmonary function, and health status. A crossover study from negative pressure ventilation to NIPPV was carried out in a subgroup of patients. RESULTS--Five year acturial probability of continuing NIPPV for individuals with early onset scoliosis (n = 47), previous poliomyelitis (n = 30), following tuberculous lung disease (n = 20), general neuromuscular disorders (n = 29), and chronic obstructive pulmonary disease (n = 33) was 79% (95% CI 66 to 92), 100%, 94% (95% CI 83 to 100), 81% (95% CI 61 to 100), 43% (95% CI 6 to 80), respectively. Most of the patients with bronchiectasis died within two years. One year after starting NIPPV electively the mean (SD) PaO2 compared with the pretreatment value was +1.8 (1.9) kPa, mean PaCO2 -1.4 (1.3) kPa in patients with extrapulmonary restrictive disorders, and PaO2 +0.8 (1.0) kPa, PaCO2 -0.9 (0.8) kPa in patients with obstructive lung disease. Arterial blood gas tensions improved in patients transferred from negative pressure ventilation to NIPPV. Health status was ranked highest in patients with early onset scoliosis, previous poliomyelitis, and following tuberculous lung disease. In the group as a whole health perception was comparable to outpatients with other chronic disorders. CONCLUSIONS--The long term outcome of domiciliary NIPPV in patients with chronic respiratory failure due to scoliosis, previous poliomyelitis, and chest wall and pulmonary disease secondary to tuberculosis is encouraging. The results of NIPPV in patients with COPD and progressive neuromuscular disorders show benefit in some subgroups. The outcome in end stage bronchiectasis is poor. Images PMID:7638799

Outcome of domiciliary nasal intermittent positive pressure ventilation in restrictive and obstructive disorders.

PubMed

Simonds, A K; Elliott, M W

1995-06-01

Nasal intermittent positive pressure ventilation (NIPPV) is a new technique which has rapidly supplanted other non-invasive methods of ventilation over the last 5-10 years. Data on its effectiveness are limited. The outcome of long term domiciliary NIPPV has been analysed in 180 patients with hypercapnic respiratory failure predominantly due to chest wall restriction, neuromuscular disorders, or chronic obstructive lung disease. One hundred and thirty eight patients were started on NIPPV electively, and 42 following an acute hypercapnic exacerbation. Outcome measures were survival (five year probability of continuing NIPPV), pulmonary function, and health status. A crossover study from negative pressure ventilation to NIPPV was carried out in a subgroup of patients. Five year acturial probability of continuing NIPPV for individuals with early onset scoliosis (n = 47), previous poliomyelitis (n = 30), following tuberculous lung disease (n = 20), general neuromuscular disorders (n = 29), and chronic obstructive pulmonary disease (n = 33) was 79% (95% CI 66 to 92), 100%, 94% (95% CI 83 to 100), 81% (95% CI 61 to 100), 43% (95% CI 6 to 80), respectively. Most of the patients with bronchiectasis died within two years. One year after starting NIPPV electively the mean (SD) PaO2 compared with the pretreatment value was +1.8 (1.9) kPa, mean PaCO2 -1.4 (1.3) kPa in patients with extrapulmonary restrictive disorders, and PaO2 +0.8 (1.0) kPa, PaCO2 -0.9 (0.8) kPa in patients with obstructive lung disease. Arterial blood gas tensions improved in patients transferred from negative pressure ventilation to NIPPV. Health status was ranked highest in patients with early onset scoliosis, previous poliomyelitis, and following tuberculous lung disease. In the group as a whole health perception was comparable to outpatients with other chronic disorders. The long term outcome of domiciliary NIPPV in patients with chronic respiratory failure due to scoliosis, previous poliomyelitis, and chest wall and pulmonary disease secondary to tuberculosis is encouraging. The results of NIPPV in patients with COPD and progressive neuromuscular disorders show benefit in some subgroups. The outcome in end stage bronchiectasis is poor.

Spectrum of high resolution computed tomography findings in occupational lung disease: experience in a tertiary care institute.

PubMed

Bhawna, Satija; Ojha, U C; Kumar, Sanyal; Gupta, Rajiv; Gothi, Dipti; Pal, R S

2013-01-01

To study the spectrum of high resolution computed tomography (HRCT) findings in occupational lung disease in industrial workers and to assess the utility of International classification of HRCT for occupational and environmental respiratory diseases (ICHOERD). Retrospective analysis of radiological data (radiographs and computed tomography chest scans) gathered over a period of 3 years (January 2010- December 2012) of industrial workers in an organised sector who presented with respiratory complaints. The HRCT findings were evaluated using ICHOERD. There were 5 females and 114 males in the study, with a mean age of 49 years. These workers were exposed to different harmful agents including silica, asbestos, cotton dust, metal dust, iron oxide, organic dust, rubber fumes, plastic fumes, acid fumes, and oil fumes. There were 10 smokers in the study. The radiograph of chest was normal in 53 patients. 46% of these normal patients (21.8% of total) demonstrated positive findings on HRCT. When the radiograph was abnormal, HRCT provided more accurate information and excluded the other diagnosis. The HRCT findings were appropriately described using the ICHOERD. Bronchiectasis was the most common finding (44.5%) with mild central cylindrical bronchiectasis as the most common pattern. Pleural thickening was seen in 41 patients (34.5%). Enlarged hilar or mediastinal lymphnodes were seen in 10 patients (8.4%) with egg-shell calcification in 1 patient exposed to silica. Bronchogenic carcinoma was seen in 1 patient exposed to asbestos. Occupational lung disease is a common work related condition in industrial workers even in the organized sector. Though chest radiograph is the primary diagnostic tool, HRCT is the undisputed Gold Standard for evaluation of these patients. Despite the disadvantage of radiation exposure, low dose CT may serve as an important tool for screening and surveillance. The ICHOERD is a powerful and reliable tool not only for diagnosis, but also for quantitative and analytical measurement of disease, thereby contributing to assessing the medical epidemiology of lung disease. It should always be used while evaluating HRCT of a patient with occupational lung disease.

Burkholderia cepacia complex in cystic fibrosis in a Brazilian reference center.

PubMed

Dentini, Priscila; Marson, Fernando Augusto Lima; Bonadia, Luciana Cardoso; Bertuzzo, Carmen Sílvia; Ribeiro, Antônio Fernando; Levy, Carlos Emílio; Ribeiro, José Dirceu

2017-12-01

The Burkholderia cepacia complex (BCC) can cause a severe decline in lung function in cystic fibrosis (CF). Our objective was to determine the BCC prevalence and to evaluate its clinical impact on CF. Clinical and laboratory variables were determined for CF patients with BCC (Group-A = 50 patients) and without BCC (Group-B = 134 patients). The microorganisms were identified by biochemical tests, the Vitek2 ® Compact test, recA-PCR and recA-nested-PCR with species-specific primers and DNA sequencing. The patients were evaluated by the Shwachman-Kulczycki score (SKCS), Bhalla score (BS), spirometry and body mass index (BMI). The BCC prevalence was 22.5%. The most common species were Burkholderia multivorans (30%), Burkholderia cepacia (24%), Burkholderia cenocepacia IIIA (10%), B. cenocepacia IIIB (2%) and Burkholderia vietnamiensis (2%). There was difference between the groups in nutritional status (p = 0.02) and general activity (p = 0.026). There was difference in total BS points (p = 0.04) and the following parameters: bronchiectasis severity (p = 0.007), peribronchial thickening (p = 0.013), bronchiectasis extent (p = 0.01) and general aspects of the affected bronchial zone (p = 0.02). The respiratory disorder classifications were as follows: obstructive-4.8% (Group-A) and 23.8% (Group-B); restrictive-9.5% (Group-A and Group-B); obstructive + restrictive-19% (Group-A) and 1.6% (Group-B); and obstructive + restrictive with a decreased forced expiratory flow-47.6% (Group-A) and 30.2% (Group-B) (p = 0.02). Nutritional status was a minor contributing factor to weight, height and BMI in the Group-A (p = 0.02). The BCC prevalence, particularly the prevalence of B. multivorans, was higher in this study. The SKCS, BS, spirometry and nutritional status results showed that BCC has a negative impact on clinical status. Phenotypic methods are useful for the identification of presumptive BCC. The Vitek2 ® Compact test showed accuracy in BCC identification. PCR, nested-PCR, and recA sequencing showed specificity in BCC species identification.

SINUSITIS, BRONCHIECTASIS, AND FLATUS IN A SUMATRAN ORANGUTAN (PONGO ABELII): COULD THIS BE CYSTIC FIBROSIS?

PubMed

Stringer, Elizabeth; Cossaboon, Cindy; Han, Sushan; Taylor-Cousar, Jennifer L

2016-03-01

A 31-yr-old male Sumatran orangutan (Pongo abelii) presented with 14 yr of chronic purulent nasal drainage and cough with intermittent exacerbation of symptoms requiring systemic antibiotic treatment. He was diagnosed with a cystic fibrosis (CF)-like condition. Evaluation consisted of bronchoscopy with bronchoalveolar lavage, culture, and computed tomography scanning of the sinuses and chest. Although the presence of low fecal elastase activity increased the suspicion for a diagnosis of CF, pilocarpine iontophoresis with sweat collection and analysis ("sweat testing") was inconclusive. Medical management included twice-daily nebulization with bronchodilators and alternating month inhaled antibiotics, pancreatic enzyme replacement therapy, and simethicone as needed. Sinopulmonary and gastrointestinal symptoms improved substantially with treatment. Several years later, the animal died acutely of colonic volvulus. Necropsy and histopathology confirmed CF-like lung disease with chronic air sacculitis.

Primary Sjögren's syndrome and bronchiolitis. An unusual association.

PubMed

Medina-Paz, Lourdes; Che-Morales, José Luis; Alonso Moshe Barrera-Pérez, Hirian; Cortes-Telles, Arturo

2017-03-03

Sjögren's syndrome (SS) is a systemic autoimmune disease that mainly affects the exocrine glands. It has been reported that the association between SS and respiratory involvement occurs in 11% and 45% of cases, and can be the initial manifestation of the disease. The diagnosis is based on the integration of clinical, immunological and histopathological criteria. This paper examines a 58-year-old man with SS, severe dyspnea and recurrent exacerbations associated with bronchiolitis-bronchiectasis. The absence of anti-Ro and anti-La antibodies associated with severe bronchiolitis is a clinical presentation that is poorly characterized in this group of patients; moreover, how to treat them remains unclear. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

Respiratory disease caused by synthetic fibres: a new occupational disease.

PubMed Central

Pimentel, J C; Avila, R; Lourenço, A G

1975-01-01

Seven patients exposed to the inhalation of synthetic fibres presented with various bronchopulmonary diseases, such as asthma, extrinsic allergic alveolitis, chronic bronchitis with bronchiectasis, spontaneous pneumothorax, and chronic pneumonia. The histological features are described and an attempt has been made to set up immunological techniques for the diagnosis. A series of histochemical techniques, based on textile chemistry, are proposed for the identification of the inclusions found in bronchopulmonary lesions. The results of the experimental production of the disease in guinea-pigs by the inhalation of synthetic fibre dusts are presented. The prognosis of these cases is good in the acute or recently established cases but is poor when widespread and irreversible fibrosis has set in. The authors consider that pulmonary disease due to inhaled particles is probably set off by an individual factor, possibly immunological. Images PMID:1179318

Foreign bodies in the larynx and tracheobronchial tree in children. A review of 225 cases.

PubMed

Rothmann, B F; Boeckman, C R

1980-01-01

In a study of 225 patients with foreign bodies in the larynx, trachea and bronchi, 77% were 36 months of age or less. The male-female ratio was 2:1. Food or food derivatives were the causative agent in 70% of the cases, with 38% due to a portion of nut. The foreign body involved the right and left bronchus with equal frequency. A choking episode followed by an audible wheeze (55%) was the most common presenting complaint. Obstructive emphysema was demonstrated in 60% and was best demonstrated by inspiration-expiration chest roentgenograms or fluoroscopy. A radio-opaque object was seen in 13%. Two hundred ten foreign objects (93%) were removed by endoscopy. Four patients required pulmonary resection for bronchiectasis and in three patients bronchotomy was performed. Five patients expelled the foreign body spontaneously, two patients were transferred to another hospital, and one foreign body was not recovered. There was no mortality.

Pediatric common variable immunodeficiency: immunologic and phenotypic associations with switched memory B cells.

PubMed

Yong, Pierre L; Orange, Jordan S; Sullivan, Kathleen E

2010-08-01

Recent studies suggest that patients with common variable immunodeficiency (CVID) and low numbers of switched memory B cells have lower IgG levels and higher rates of autoimmune disease, splenomegaly, and granulomatous disease; however, no prior literature has focused exclusively on pediatric cases. We examined the relationship between switched memory B cells and clinical and immunologic manifestations of CVID in a pediatric population. Forty-five patients were evaluated. Patients were categorized as Group I (<5 switched memory B cells/ml, n = 24) or Group II (> or =5 switched memory B cells/mL, n = 21). CD3(+) T-cell counts and CD19(+) B-cell levels were lower among Group I patients. Only those in Group I had meningitis, sepsis, bronchiectasis, granulomatous lung disease, autoimmune cytopenias, or hematologic malignancies. Segregation of pediatric patients into high risk (Group I) and average risk (Group II) may assist in targeting surveillance appropriately.

How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old.

PubMed

Prados, C; Lerín, M; Cabanillas, J J; Gómez-Carrera, L; Álvarez-Sala, R

2017-01-01

To specify the prevalence of patients diagnosed with CF at age of ≥60 year-old and to analyze their characteristics. Observational study of CF patients which were diagnosed at age ≥60 year-old. The analyzed variables were: age, sex, nationality, lung function parameters, conditions present at diagnosis, microbiological characteristics and genetic findings. eight patients were included. 7 patients were female (87.5%) with a mean age of 70.6 years (median 71.5 years, range 60-78 years). The most important findings were: sweat test >60 mEq/l; heterozygotes F508del; bronchiectasis in CT; methicillin-sensitive Staphylococcus aureus (50%) in sputum. The most patients presented a normal or mild obstructive lung function. CF must also be considered a disease diagnosed in adulthood, incorporating the sweat test within the usual techniques of differential diagnosis in patients with different diseases associated with CF, because genetic counselling is esencial.

Silicosis Caused by Chronic Inhalation of Snail Shell Powder

PubMed Central

Jung, Jae Woo; Lee, Byung Ook; Lee, Jae Hee; Park, Sung Woon; Kim, Bo Min; Choi, Jae Chol; Shin, Jong Wook; Park, In Won; Choi, Byoung Whui

2012-01-01

A 70-yr-old woman visited our hospital for shortness of breath. Chest CT showed ground glass opacity and traction bronchiectasis at right middle, lower lobe and left lingular division. Video-assisted thoracic surgical biopsy at right lower lobe and pathologic examination revealed mixed dust pneumoconiosis. Polarized optical microscopy showed lung lesions were consisted of silica and carbon materials. She was a housewife and never been exposed to silica dusts occupationally. She has taken freshwater snails as a health-promoting food for 40 yr and ground shell powder was piled up on her backyard where she spent day-time. Energy dispersive X-ray spectroscopy of snail shell and scanning electron microscopy with energy dispersive x-ray spectroscopy of lung lesion revealed that silica occupies important portion. Herein, we report the first known case of silicosis due to chronic inhalation of shell powder of freshwater snail. PMID:22219621

What is the importance of classifying Aspergillus disease in cystic fibrosis patients?

PubMed

Jones, Andrew M; Horsley, Alex; Denning, David W

2014-08-01

Aspergillus species are commonly isolated from lower respiratory tract samples of patients with cystic fibrosis (CF) and markers of immunological sensation to Aspergillus are frequently encountered in this group of patients; however, the contribution of Aspergillus to CF lung disease outside of the typical complications of ABPA and aspergilloma formation remains largely unclear. Patients with CF show discretely different responses to Aspergillus, though the underlying reasons for this variation are unknown. Recent work has begun to allow us to categorize patient responses to Aspergillus based upon molecular markers of infection and immune sensitization. Aspergillus sensitization and/or airway infection is associated with worse FEV1, in CF and other patients (asthma, chronic obstructive pulmonary disease, bronchiectasis). Classification of different clinical phenotypes of Aspergillus will enable future studies to determine the natural history of different manifestations of Aspergillus disease and evaluate the effects of intervention with antifungal therapy.

Spectrum of Clinical Diseases Caused By Disorders of Primary Cilia

PubMed Central

Aygun, Meral Gunay-; Hildebrandt, Friedhelm

2011-01-01

The ciliopathies are a category of diseases caused by disruption of the physiological functions of cilia. Ciliary dysfunction results in a broad range of phenotypes, including renal, hepatic, and pancreatic cyst formation; situs abnormalities; retinal degeneration; anosmia; cerebellar or other brain anomalies; postaxial polydactyly; bronchiectasis; and infertility. The specific clinical features are dictated by the subtype, structure, distribution, and function of the affected cilia. This review highlights the clinical variability caused by dysfunction of motile and nonmotile primary cilia and emphasizes the genetic heterogeneity and phenotypic overlap that are characteristics of these disorders. There is a need for additional research to understand the shared and unique functions of motile and nonmotile cilia and the pathophysiology resulting from mutations in cilia, basal bodies, or centrosomes. Increased understanding of ciliary biology will improve the diagnosis and management of primary ciliary dyskinesia, syndromic ciliopathies, and cilia-related cystic diseases. PMID:21926397

High-resolution computed tomography findings of acute respiratory distress syndrome, acute interstitial pneumonia, and acute exacerbation of idiopathic pulmonary fibrosis.

PubMed

Ichikado, Kazuya

2014-02-01

Diffuse alveolar damage (DAD) is the pathologic feature of rapidly progressive lung diseases, including acute respiratory distress syndrome, acute interstitial pneumonia, and acute exacerbation of idiopathic pulmonary fibrosis. The clinical significance and limitation of high-resolution computed tomography (HRCT) findings in these diseases were reviewed. The HRCT findings correlate well with pathologic phases (exudative, proliferative, and fibrotic) of DAD, although it cannot detect early exudative phase. Traction bronchiolectasis or bronchiectasis within areas of increased attenuation on HRCT scan is a sign of progression from the exudative to the proliferative and fibrotic phase of DAD. Extensive abnormalities seen on HRCT scans, which are indicative of fibroproliferative changes, were independently predictive of poor prognosis in patients with clinically early acute respiratory distress syndrome, acute interstitial pneumonia, and acute exacerbation of idiopathic pulmonary fibrosis. © 2013 Published by Elsevier Inc.

[Small airway diseases and immune deficiency].

PubMed

Burgel, P-R; Bergeron, A; Knoop, C; Dusser, D

2016-02-01

Innate or acquired immune deficiency may show respiratory manifestations, often characterized by small airway involvement. The purpose of this article is to provide an overview of small airway disease across the major causes of immune deficiency. In patients with common variable immune deficiency, recurrent lower airway infections may lead to bronchiolitis and bronchiectasis. Follicular and/or granulomatous bronchiolitis of unknown origin may also occur. Bronchiolitis obliterans is the leading cause of death after the first year in patients with lung transplantation. Bronchiolitis obliterans also occurs in patients with allogeneic haematopoietic stem cell transplantation, especially in the context of systemic graft-versus-host disease. Small airway diseases have different clinical expression and pathophysiology across various causes of immune deficiency. A better understanding of small airways disease pathogenesis in these settings may lead to the development of novel targeted therapies. Copyright © 2015 SPLF. Published by Elsevier Masson SAS. All rights reserved.

X-linked agammaglobulinemia in northern Thailand.

PubMed

Trakultivakorn, Muthita; Ochs, Hans D

2006-03-01

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by a failure to generate immunoglobulins of all isotypes due to the absence of mature B cells and plasma cells, secondary to mutations in the Bruton's tyrosine kinase (Btk) gene. We report six patients with XLA, confirmed by mutation analysis, from northern Thailand. The mean age of onset was 2.5 years and the mean age at diagnosis was 7.3 years. All patients had a history of otitis media, pneumonia and arthritis at the time of diagnosis, five patients had developed bronchiectasis and 3 patients septicemia. Other infections reported included sinusitis (5/6), pericarditis (1/6), meningitis (1/6) and pyoderma (1/6). Haemophilus influenzae, Streptococcus pneumoniae, Pseudomonas aeruginosa and Staphylococcus aureus were isolated on multiple occasions. One patient died of sepsis at the age of 16 years. These observations demonstrate that early diagnosis and treatment can improve prognosis and quality of life.

Risk Factors for Chronic Cough Among 14,669 Individuals From the General Population.

PubMed

Çolak, Yunus; Nordestgaard, Børge G; Laursen, Lars C; Afzal, Shoaib; Lange, Peter; Dahl, Morten

2017-09-01

Risk factors for chronic cough in the general population have not been described systematically. We identified and ranked chronic cough risk factors at the individual and community level using data from 14,669 individuals from the Copenhagen General Population Study. Severity of chronic cough was assessed using the Leicester Cough Questionnaire (LCQ). We ranked chronic cough risk factors based on magnitude of age-adjusted ORs at the individual level and of the population attributable risks (PARs) at the community level. Prevalence of chronic cough in the general population was 4% overall and 3% in never smokers, 4% in former smokers, and 8% in current smokers. Median score of the LCQ was 5.8 (25th-75th percentile, 5.0-6.3) for physical domain, 5.6 (25th-75th percentile, 4.6-6.3) for psychologic domain, 6.3 (25th-75th percentile, 5.5-6.8) for social domain, and 17.3 (25th- 75th percentile, 15.4-18.9) in total. At the level of the individual, age-adjusted ORs for the three top-ranked risk factors were 5.0 (95% CI, 1.4-18) for bronchiectasis, 2.6 (95% CI, 1.7-3.9) for asthma and 2.3 (95% CI, 1.5-3.4) for gastroesophageal reflux disease in never smokers, 7.1 (95% CI, 2.6-20) for bronchiectasis, 3.1 (95% CI, 2.2-4.4) for asthma and 2.2 (95% CI, 1.5-3.2) for occupational exposure to dust/fumes in former smokers, and 1.9 (95% CI, 1.3-2.9) for airflow limitation in current smokers. At the level of the community, the three top-ranked risk factors were female sex (PAR, 19%), asthma (PAR, 10%), and gastroesophageal reflux disease (PAR, 8%) in never smokers; abdominal obesity (PAR, 20%), low income (PAR, 20%), and asthma (PAR, 13%) in former smokers; and airflow limitation (PAR, 23%) in current smokers. Risk factors for chronic cough differ at the level of the individual and community, and by smoking status. Strategies to prevent and treat modifiable chronic cough risk factors should be tailored accordingly. Copyright © 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Causes of chronic productive cough: An approach to management.

PubMed

Martin, Matthew J; Harrison, Tim W

2015-09-01

A chronic 'productive' or 'wet' cough is a common presenting complaint for patients attending the adult respiratory clinic. Most reviews and guidelines suggest that the causes of a productive cough are the same as those of a non-productive cough and as such the same diagnostic pathway should be followed. We suggest a different diagnostic approach for patients with a productive cough, focussing on the conditions that are the most likely causes of this problem. This review is intended to briefly summarise the epidemiology, clinical features, pathophysiology and treatment of a number of conditions which are often associated with chronic productive cough to aid decision making when encountering a patient with this often distressing symptom. The conditions discussed include bronchiectasis, chronic bronchitis, asthma, eosinophilic bronchitis and immunodeficiency. We also propose an adult version of the paediatric diagnosis of protracted bacterial bronchitis (PBB) in patients with idiopathic chronic productive cough who appear to respond well to low dose macrolide therapy. Copyright © 2015 Elsevier Ltd. All rights reserved.

Halitosis

PubMed Central

2014-01-01

Introduction Halitosis can be caused by oral disease or by respiratory tract conditions such as sinusitis, tonsillitis, and bronchiectasis, but an estimated 40% of affected individuals have no underlying organic disease. Methods and outcomes We conducted a systematic review and aimed to answer the following clinical question: What are the effects of treatments in people with physiological halitosis? We searched: Medline, Embase, The Cochrane Library, and other important databases up to July 2013 (Clinical evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found 11 studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review, we present information relating to the effectiveness and safety of the following interventions: artificial saliva; cleaning, brushing, or scraping the tongue; regular use of mouthwash; sugar-free chewing gums; and zinc toothpastes. PMID:25234037

Diagnosis and management of primary ciliary dyskinesia.

PubMed

Lucas, Jane S; Burgess, Andrea; Mitchison, Hannah M; Moya, Eduardo; Williamson, Michael; Hogg, Claire

2014-09-01

Primary ciliary dyskinesia (PCD) is an inherited autosomal-recessive disorder of motile cilia characterised by chronic lung disease, rhinosinusitis, hearing impairment and subfertility. Nasal symptoms and respiratory distress usually start soon after birth, and by adulthood bronchiectasis is invariable. Organ laterality defects, usually situs inversus, occur in ∼50% of cases. The estimated prevalence of PCD is up to ∼1 per 10,000 births, but it is more common in populations where consanguinity is common. This review examines who to refer for diagnostic testing. It describes the limitations surrounding diagnosis using currently available techniques and considers whether recent advances to genotype patients with PCD will lead to genetic testing and screening to aid diagnosis in the near future. It discusses the challenges of monitoring and treating respiratory and ENT disease in children with PCD. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

[Norwegian scabies, a rare diagnosis in Tunisia].

PubMed

Mebazaa, Amel; Bedday, Bouraoui; Trabelsi, Sabiha; Denguezli, Mohamed; Ben Said, Moncef; Belajouza, Colandane; Nouira, Rafiaa

2006-10-01

Norwegian scabies is a particular form of scabies, rarely reported in Tunisia. It habitually occurs in immunocompromised hosts, with prolonged immunosuppressive therapy, congenital immune disease, HIV-1 infection or neoplasm. We report an observation of norwegian scabies, occuring in a woman with prolounged systemic corticosteroid therapy. A 65-year-old woman, with a long history of obstructive bronchoneumopathy and bronchiectasis, treated with systemic corticosteroids for 5 years, was referred with a mild pruriginous and scaly erythroderma. This dermatosis developed within 5 months, with fever and impairing of health. Skin parasitological exams showed a massive infestation by sarcoptes. Histologic findings revealed multiple sarcoptes scabiei and eggs in the stratum corneum. The patient was treated with benzyl benzoate during 48 hours. She died rapidly with acute respiratory distress. basing on this observation of norwegian scabies occuring in an old immunocompromised woman, we put the accent on this particular form of scabies, often misappreciated, and incite to practice skin and nail parasitological exams in case of mild pruriginous, chronic scaly erythroderma. developing in immunocompromised patients.

Sex differences in chronic cor pulmonale in delhi.

PubMed

Padmavati, S; Arora, R

1976-10-01

Chronic cor pulmonale is more prevalent in northern India than in the south. It is equally common in men and in women and accounts for 20% of all admissions for heart disorder in Delhi. In a study of 766 patients (239 men and 527 women) carried out over a 15-year period there were some striking sex differences. Some 75% of men and 10% of women smoked. The women came from the poorest class and all of them cooked from an early age over smoky and primitive fireplaces in ill-ventilated huts, while only 7% of the men cooked their own food. Chronic bronchitis and bronchiectasis were the commonest associated lung disorders in both sexes. The women developed heart failure 10-15 years earlier and showed more severe congestive failure with larger hearts and greater derangement of pulmonary function. It is concluded that the cause of chronic cor pulmonale in women in Delhi was damage to the lungs from exposure to smoky cooking fuels from girlhood onwards, followed by repeated chest infections.

[Respiratory disease registries in Spain: fundamentals and organization].

PubMed

Lara, Beatriz; Morales, Pilar; Blanco, Ignacio; Vendrell, Montserrat; de Gracia Roldán, Javier; Monreal, Manel; Orriols, Ramón; Isidro, Isabel; Abú-Shams, Khalil; Escribano, Pilar; Villena, Victoria; Rodrigo, Teresa; Vidal Plà, Rafael; García-Yuste, Mariano; Miravitlles, Marc

2011-08-01

This present paper describes the general characteristics, objectives and organizational aspects of the respiratory disease registries in Spain with the aim to report their activities and increase their diffusion. The document compiles information on the following registries: the Spanish Registry of Patients with Alpha-1 Antitrypsin Deficiency, Spanish Registry of Bronchiectasis, International Registry of Thromboembolic Disease, Spanish Registry of Occupational Diseases, Spanish Registry of Pulmonary Artery Hypertension, Registry of Pleural Mesothelioma, Spanish Registry of Tuberculosis and Spanish Multi-center Study of Neuroendocrine Pulmonary Tumors. Our paper provides information on each of the registries cited. Each registry has compiled specific clinical information providing data in real situations, and completes the results obtained from clinical assays. Said information has been published both in national as well as international publications and has lead to the creation of various guidelines. Therefore, the activities of the professionals involved in the registries have spread the knowledge about the diseases studied, promoting the exchange of information among workgroups. Copyright © 2010 SEPAR. Published by Elsevier Espana. All rights reserved.

CT-Definable Subtypes of Chronic Obstructive Pulmonary Disease: A Statement of the Fleischner Society

PubMed Central

Austin, John H. M.; Hogg, James C.; Grenier, Philippe A.; Kauczor, Hans-Ulrich; Bankier, Alexander A.; Barr, R. Graham; Colby, Thomas V.; Galvin, Jeffrey R.; Gevenois, Pierre Alain; Coxson, Harvey O.; Hoffman, Eric A.; Newell, John D.; Pistolesi, Massimo; Silverman, Edwin K.; Crapo, James D.

2015-01-01

The purpose of this statement is to describe and define the phenotypic abnormalities that can be identified on visual and quantitative evaluation of computed tomographic (CT) images in subjects with chronic obstructive pulmonary disease (COPD), with the goal of contributing to a personalized approach to the treatment of patients with COPD. Quantitative CT is useful for identifying and sequentially evaluating the extent of emphysematous lung destruction, changes in airway walls, and expiratory air trapping. However, visual assessment of CT scans remains important to describe patterns of altered lung structure in COPD. The classification system proposed and illustrated in this article provides a structured approach to visual and quantitative assessment of COPD. Emphysema is classified as centrilobular (subclassified as trace, mild, moderate, confluent, and advanced destructive emphysema), panlobular, and paraseptal (subclassified as mild or substantial). Additional important visual features include airway wall thickening, inflammatory small airways disease, tracheal abnormalities, interstitial lung abnormalities, pulmonary arterial enlargement, and bronchiectasis. © RSNA, 2015 PMID:25961632

The Lung Immune Response to Nontypeable Haemophilus influenzae (Lung Immunity to NTHi)

PubMed Central

King, Paul T.; Sharma, Roleen

2015-01-01

Haemophilus influenzae is divided into typeable or nontypeable strains based on the presence or absence of a polysaccharide capsule. The typeable strains (such as type b) are an important cause of systemic infection, whilst the nontypeable strains (designated as NTHi) are predominantly respiratory mucosal pathogens. NTHi is present as part of the normal microbiome in the nasopharynx, from where it may spread down to the lower respiratory tract. In this context it is no longer a commensal and becomes an important respiratory pathogen associated with a range of common conditions including bronchitis, bronchiectasis, pneumonia, and particularly chronic obstructive pulmonary disease. NTHi induces a strong inflammatory response in the respiratory tract with activation of immune responses, which often fail to clear the bacteria from the lung. This results in recurrent/persistent infection and chronic inflammation with consequent lung pathology. This review will summarise the current literature about the lung immune response to nontypeable Haemophilus influenzae, a topic that has important implications for patient management. PMID:26114124

Update on Pediatric Cough.

PubMed

Kantar, Ahmad

2016-02-01

Despite the high prevalence of cough in children, the topic has been poorly researched. Although pediatricians recognize that chronic cough in children is different from that in adults, this difference seems less recognizable to other health professionals. During childhood, the respiratory tract and nervous system undergo a series of anatomical and physiological maturation processes that influence the cough reflex. Additionally, immunological responses undergo developmental and memorial processes that make infection and congenital abnormalities the overwhelming cause of cough in children. The lack of comprehensive clinical data regarding chronic cough in children has initially required pediatricians to adopt an adult approach to the problem. In the last 10 years, however, research has led to the reconsideration of the etiology of chronic cough in children. Currently, attention has focused on protracted bacterial bronchitis as a major cause of chronic cough in preschool-aged children and as a possible precursor of bronchiectasis. New research horizons are emerging for both the treatment and prevention of particular causes of chronic cough in children.

Management of male infertility due to congenital bilateral absence of vas deferens should not ignore the diagnosis of cystic fibrosis.

PubMed

Grzegorczyk, V; Rives, N; Sibert, L; Dominique, S; Macé, B

2012-10-01

Microsurgical or percutaneous epididymal sperm aspiration and intracytoplasmic sperm injection (ICSI) are proposed to overcome male infertility due to congenital bilateral absence of vas deferens (CBAVD). CBAVD has been associated with mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and consequently, genetic counselling has to be addressed before beginning ICSI procedure. However, management of male infertility due to CBAVD should not ignore a mild form of cystic fibrosis. We describe the case of cystic fibrosis late diagnosis performed in a 49-year-old infertile men with CBAVD. CFTR molecular testing detected two mutations F508del and A455E corresponding to a cystic fibrosis genotype. Pneumological evaluation revealed a severe obstructive respiratory disease, bronchiectasis and high sweat chloride levels. Symptoms consistent with a cystic fibrosis have to be identified in infertile men with CBAVD before beginning assisted reproductive procedures. © 2012 Blackwell Verlag GmbH.

Cytokine profile of nasal and middle ear polyps in a patient with Woakes' syndrome and eosinophilic otitis media.

PubMed

De Loof, Marie; De Leenheer, Els; Holtappels, Gabriële; Bachert, Claus

2016-05-03

Woakes' syndrome is characterised by severe recurrent nasal polyps in early childhood with broadening of the nose, nasal dyscrinia, frontal sinus aplasia and bronchiectasis. Eosinophilic otitis media (EOM) is an intractable condition that can cause gradual or sudden deterioration of hearing and is associated with comorbid asthma. For these reasons, both diseases must be recognised and treated appropriately. We present a case of a 20-year-old man with Woakes' syndrome complicated by EOM, with polyps in both middle ears; this condition has so far not been described as an entity of eosinophilic disease in the literature. We have, from reviewing the literature, learned that this is the first time that results of tissue examination on nasal polyposis with respect to tissue IgE, eosinophil-cationic protein, interleukin (IL)-17 and IL-5 in a patient with Woakes' syndrome and EOM, has been reported and discussed. 2016 BMJ Publishing Group Ltd.

[Application of selection criteria in sequential double lung transplantation].

PubMed

Borro, J M; Tarazona, V; Vicente, R; Cafarena, J M; Ramos, F; Sales, G; Galán, G; Lozano, C; Morant, P; Calvo, V; Morcillo, A; París, F

1999-03-01

Since the first sequential double lung transplant was performed in 1986, such procedures have been increasing in number and the criteria used as indications for this type of surgery have broadened. Our aim was to reflect on the application of selection criteria and to describe the anesthetic and surgical techniques and postoperative follow-up of 72 patients who underwent this type of transplant surgery between March 1993 and December 1998. Actuarial survival five years after surgery was 74.4%. Among patients requiring transplantation after septic disease, actuarial survival was 90.8% for cystic fibrosis and 88.2% for bronchiectasis. Of the preoperative risk factors analyzed (prior surgery, pachypleuritis, multiresistant germs, poor nutrition, mechanical ventilation and corticoid therapy), only prior treatment with high doses of corticoids proved significant. Eleven patients have been diagnosed of bronchiolitis obliterans, four have died and only two continue to experience difficulties in daily living. The high survival rate and the restriction-free life after recovery lead us to consider sequential double lung transplantation to be the treatment of choice for all pulmonary diseases.

Halitosis

PubMed Central

2008-01-01

Introduction Halitosis can be caused by oral disease, or by respiratory tract conditions such as sinusitis, tonsillitis, and bronchiectasis, but an estimated 40% of affected individuals have no underlying organic disease. Methods and outcomes We conducted a systematic review and aimed to answer the following clinical question: What are the effects of treatments in people with physiological halitosis? We searched: Medline, Embase, The Cochrane Library, and other important databases up to July 2008 (Clinical evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found five systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review we present information relating to the effectiveness and safety of the following interventions: artificial saliva; cleaning, brushing, or scraping the tongue; diet modification; regular or single use of mouthwash; sugar-free chewing gums; and zinc toothpastes. PMID:19445739

Mycobacterium simiae: clinical, radiological and microbiological characteristics in 97 patients.

PubMed

Coolen-Allou, Nathalie; Touron, Thomas; Belmonte, Olivier; Gazaille, Virgile; Andre, Michel; Allyn, Jérôme; Picot, Sandrine; Payet, Annabelle; Veziris, Nicolas

2018-05-14

Mycobacterium simiae is a rare species of slow-growing nontuberculous mycobacteria (NTM).From 2002 to 2017, we conducted a retrospective study that included all patients with NTM-positive respiratory samples detected in two university hospitals of the French overseas department of Reunion Island. We recorded the prevalence of M. simiae in this cohort, as well as the clinical, radiological, and microbiological features of patients with at least one sample positive for M. simiae In our cohort, 97 patients (15.1%) were positive for M. simiae Twenty-one patients (21.6%) met the American Thoracic Society (ATS) criteria for infection. M. simiae infection was associated with bronchiectasis, micronodular lesions and weight loss. Antibiotic susceptibility testing was performed in 60 patients and the isolates were found to have low susceptibility to antibiotics, except for amikacin, fluoroquinolones, and clarithromycin. Treatment failed in 4 of the 8 patients treated for M. simiae infection.Here, we describe a specific cluster corresponding to a large cohort of patients with M. simiae, a rare nontuberculous mycobacteria associated with low pathogenicity and a poor susceptibility to antibiotics. Copyright © 2018 American Society for Microbiology.

The diversity of the effects of sulfur mustard gas inhalation on respiratory system 10 years after a single, heavy exposure: analysis of 197 cases.

PubMed

Emad, A; Rezaian, G R

1997-09-01

To find out the late pulmonary sequelae of sulfur mustard gas inhalation in 197 veterans, 10 years after their exposure. Cross-sectional clinical study. University hospital. One hundred ninety-seven veterans with a single, heavy exposure to sulfur mustard gas in 1986 and 86 nonexposed veterans as their control group. Pulmonary function tests, carbon monoxide diffusion capacity, bronchoscopy, and high-resolution CT of the chest were performed in all patients. Transbronchial lung biopsy was done in 24 suspected cases of pulmonary fibrosis. Asthma was diagnosed in 21 (10.65%), chronic bronchitis in 116 (58.88%), bronchiectasis in 17 (8.62%), airway narrowing due to searing or granulation tissue in 19 (9.64%), and pulmonary fibrosis in 24 (12.18%) cases. None of these were found among the control group except for a single case of chronic bronchitis. Although the respiratory symptoms of an acute sulfur mustard gas inhalation are usually transient and nonspecific, it can lead to the development of a series of chronic destructive pulmonary sequelae in such cases.

Inhaled antibiotics for lower respiratory tract infections: focus on ciprofloxacin.

PubMed

Serisier, D J

2012-05-01

The administration of antibiotics by the inhaled route offers an appealing and logical approach to treating infectious respiratory conditions. Studies in the cystic fibrosis (CF) population have established the efficacy of this therapeutic concept and inhaled antibiotic therapy is now one of the pillars of management in CF. There are now a number of new inhaled antibiotic formulations that have shown impressive preliminary evidence for efficacy in CF and are commencing phase III efficacy studies. Translation of this paradigm into the non-CF bronchiectasis population has proven difficult thus far, apparently due to problems with tolerability of inhaled formulations. Inhaled versions of ciprofloxacin have shown good tolerability and microbiological efficacy in preliminary studies, suggesting that effective inhaled antibiotics are finally on the horizon for this previously neglected patient population. The increased use of long-term inhaled antibiotics for a wider range of non-CF indications presents risks to the broader community of greater antimicrobial resistance development that must be carefully weighed against any demonstrated benefits. Copyright 2012 Prous Science, S.A.U. or its licensors. All rights reserved.

Respiratory morbidity among street sweepers working at Hanumannagar Zone of Nagpur Municipal Corporation, Maharashtra.

PubMed

Yogesh, Sabde D; Zodpey, Sanjay P

2008-01-01

Due to the occupational exposure street sweepers are very much vulnerable to develop the chronic diseases of respiratory system. Therefore this study was undertaken to find out the proportion of chronic respiratory morbidity among the street sweepers and the role of various associated risk factors. The study included two groups: study group i.e. street sweepers and comparison group (Class IV workers working in the office buildings). Various risk factors studied were age, sex, socioeconomic status, length of service, smoking habit, type of house, area of residence, cooking fuel and pets. Proportion of chronic respiratory morbidity (chronic bronchitis, asthma and bronchiectasis) was higher (8.1%) among street sweepers compared to comparison group (2.1%), the difference being statistically significant. Unconditional multivariate logistic regression revealed that risk of having chronic respiratory morbidity among street sweepers was 4.24 (95% CI of OR = 1.24 to 14.50) times higher than that in the comparison group and the risk increased significantly with increasing length of service (OR = 1.75, 95 % CI = 1.09 to 2.81).

What is chronic cough in children?

PubMed Central

Ioan, Iulia; Poussel, Mathias; Coutier, Laurianne; Plevkova, Jana; Poliacek, Ivan; Bolser, Donald C.; Davenport, Paul W.; Derelle, Jocelyne; Hanacek, Jan; Tatar, Milos; Marchal, François; Schweitzer, Cyril; Fontana, Giovanni; Varechova, Silvia

2014-01-01

The cough reflex is modulated throughout growth and development. Cough—but not expiration reflex—appears to be absent at birth, but increases with maturation. Thus, acute cough is the most frequent respiratory symptom during the first few years of life. Later on, the pubertal development seems to play a significant role in changing of the cough threshold during childhood and adolescence resulting in sex-related differences in cough reflex sensitivity in adulthood. Asthma is the major cause of chronic cough in children. Prolonged acute cough is usually related to the long-lasting effects of a previous viral airway infection or to the particular entity called protracted bacterial bronchitis. Cough pointers and type may orient toward specific etiologies, such as barking cough in croup or tracheomalacia, paroxystic whooping cough in Pertussis. Cough is productive in protracted bacterial bronchitis, sinusitis or bronchiectasis. Cough is usually associated with wheeze or dyspnea on exertion in asthma; however, it may be the sole symptom in cough variant asthma. Thus, pediatric cough has particularities differentiating it from adult cough, so the approach and management should be developmentally specific. PMID:25221517

Lung disease in indigenous children.

PubMed

Chang, A B; Brown, N; Toombs, M; Marsh, R L; Redding, G J

2014-12-01

Children in indigenous populations have substantially higher respiratory morbidity than non-indigenous children. Indigenous children have more frequent respiratory infections that are, more severe and, associated with long-term sequelae. Post-infectious sequelae such as chronic suppurative lung disease and bronchiectasis are especially prevalent among indigenous groups and have lifelong impact on lung function. Also, although estimates of asthma prevalence among indigenous children are similar to non-indigenous groups the morbidity of asthma is higher in indigenous children. To reduce the morbidity of respiratory illness, best-practice medicine is essential in addition to improving socio-economic factors, (eg household crowding), tobacco smoke exposure, and access to health care and illness prevention programs that likely contribute to these issues. Although each indigenous group may have unique health beliefs and interfaces with modern health care, a culturally sensitive and community-based comprehensive care system of preventive and long term care can improve outcomes for all these conditions. This article focuses on common respiratory conditions encountered by indigenous children living in affluent countries where data is available. Crown Copyright © 2014. Published by Elsevier Ltd. All rights reserved.

Childhood allergic bronchopulmonary aspergillosis presenting as a middle lobe syndrome.

PubMed

Shah, Ashok; Gera, Kamal; Panjabi, Chandramani

2016-01-01

Allergic bronchopulmonary aspergillosis (ABPA) is infrequently documented in children with asthma. Although collapse is not uncommon, middle lobe syndrome (MLS) as a presentation of ABPA is rather a rarity. A 9-year-old female child with asthma presented with increase in intensity of symptoms along with a right midzone patchy consolidation on a chest radiograph. In addition, an ill-defined opacity abutting the right cardiac border with loss of cardiac silhouette was noted. A right lateral view confirmed a MLS, which was further corroborated by high resolution computed tomography. Central bronchiectasis was also observed, which prompted a work-up for ABPA. The child met 7/8 major diagnostic criteria for ABPA. She was then initiated on oral prednisolone that resulted in a marked clinical improvement within a fortnight. Radiological clearance occurred at 3 months with inflation of the middle lobe. ABPA presenting with MLS in a child is yet to be reported. A high index of suspicion is required to establish the diagnosis of ABPA in a child presenting with MLS. This would obviate the invasive investigations usually done to ascertain the cause of MLS.

Co-morbidities in severe asthma: Clinical impact and management.

PubMed

Porsbjerg, Celeste; Menzies-Gow, Andrew

2017-05-01

Patients with severe asthma represent a minority of the total asthma population, but carry a majority of the morbidity and healthcare costs. Achieving better asthma control in this group of patients is therefore of key importance. Systematic assessment of patients with possible severe asthma to identify treatment barriers and triggers of asthma symptoms, including co-morbidities, improves asthma control and reduces healthcare costs and is recommended by international guidelines on management of severe asthma. This review provides the clinician with an overview of the prevalence and clinical impact of the most common co-morbidities in severe asthma, including chronic rhinosinusitis, nasal polyposis, allergic rhinitis, dysfunctional breathing, vocal cord dysfunction, anxiety and depression, obesity, obstructive sleep apnoea syndrome (OSAS), gastroesophageal reflux disease (GERD), bronchiectasis, allergic bronchopulmonary aspergillosis (ABPA) and eosinophilic granulomatous with polyangiitis (EGPA). Furthermore, the review offers a summary of recommended diagnostic and management approaches for each co-morbidity. Finally, the review links co-morbid conditions to specific phenotypes of severe asthma, in order to guide the clinician on which co-morbidities to look for in specific patients. © 2017 Asian Pacific Society of Respirology.

Lung transplantation: overall approach regarding its major aspects

PubMed Central

de Camargo, Priscila Cilene León Bueno; Teixeira, Ricardo Henrique de Oliveira Braga; Carraro, Rafael Medeiros; Campos, Silvia Vidal; Afonso, José Eduardo; Costa, André Nathan; Fernandes, Lucas Matos; Abdalla, Luis Gustavo; Samano, Marcos Naoyuki; Pêgo-Fernandes, Paulo Manuel

2015-01-01

ABSTRACT Lung transplantation is a well-established treatment for patients with advanced lung disease. The evaluation of a candidate for transplantation is a complex task and involves a multidisciplinary team that follows the patient beyond the postoperative period. Currently, the mean time on the waiting list for lung transplantation in the state of São Paulo, Brazil, is approximately 18 months. For Brazil as a whole, data from the Brazilian Organ Transplant Association show that, in 2014, there were 67 lung transplants and 204 patients on the waiting list for lung transplantation. Lung transplantation is most often indicated in cases of COPD, cystic fibrosis, interstitial lung disease, non-cystic fibrosis bronchiectasis, and pulmonary hypertension. This comprehensive review aimed to address the major aspects of lung transplantation: indications, contraindications, evaluation of transplant candidates, evaluation of donor candidates, management of transplant recipients, and major complications. To that end, we based our research on the International Society for Heart and Lung Transplantation guidelines and on the protocols used by our Lung Transplant Group in the city of São Paulo, Brazil. PMID:26785965

Primary ciliary dyskinesia.

PubMed

Lobo, L J; Zariwala, M A; Noone, P G

2014-09-01

Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder of cilia structure and function, leading to chronic infections of the respiratory tract, fertility problems and disorders of organ laterality. Making a definitive diagnosis is challenging, utilizing characteristic phenotypes, ciliary functional and ultra-structural defects in addition to newer screening tools such as nasal nitric oxide and genetic testing. There are 21 known PCD causing genes and in the future, comprehensive genetic testing may help diagnosis young infants prior to developing symptoms thus improving survival. Therapy includes surveillance of pulmonary function and microbiology in addition to, airway clearance, antibiotics and early referral to bronchiectasis centers. Standardized care at specialized centers using a multidisciplinary approach is likely to improve outcomes. In conjunction with the PCD foundation and lead investigators and clinicians are developing a network of PCD clinical centers to coordinate the effort in North America and Europe. As the network grows, care and knowledge will undoubtedly improve. © The Author 2014. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Opportunities to diagnose chronic obstructive pulmonary disease in routine care in the UK: a retrospective study of a clinical cohort.

PubMed

Jones, Rupert C M; Price, David; Ryan, Dermot; Sims, Erika J; von Ziegenweidt, Julie; Mascarenhas, Laurence; Burden, Anne; Halpin, David M G; Winter, Robert; Hill, Sue; Kearney, Matt; Holton, Kevin; Moger, Anne; Freeman, Daryl; Chisholm, Alison; Bateman, Eric D

2014-04-01

Patterns of health-care use and comorbidities present in patients in the period before diagnosis of chronic obstructive pulmonary disease (COPD) are unknown. We investigated these factors to inform future case-finding strategies. We did a retrospective analysis of a clinical cohort in the UK with data from Jan 1, 1990 to Dec 31, 2009 (General Practice Research Database and Optimum Patient Care Research Database). We assessed patients aged 40 years or older who had an electronically coded diagnosis of COPD in their primary care records and had a minimum of 3 years of continuous practice data for COPD (2 years before diagnosis up to a maximum of 20 years, and 1 year after diagnosis) and at least two prescriptions for COPD since diagnosis. We identified missed opportunites to diagnose COPD from routinely collected patient data by reviewing patterns of health-care use and comorbidities present before diagnosis. We assessed patterns of health-care use in terms of lower respiratory consultations (infective and non-infective), lower respiratory consultations with a course of antibiotics or oral steroids, and chest radiography. If these events did not lead to a diagnosis of COPD, they were deemed to be missed opportunities. This study is registered with ClinicalTrials.gov, number NCT01655667. We assessed data for 38,859 patients. Opportunities for diagnosis were missed in 32,900 (85%) of 38,859 patients in the 5 years immediately preceding diagnosis of COPD; in 12,856 (58%) of 22,286 in the 6-10 years before diagnosis, in 3943 (42%) of 9351 in the 11-15 years before diagnosis; and in 95 (8%) of 1167 in the 16-20 years before diagnosis. Between 1990 and 2009, we noted decreases in the age at diagnosis (0·05 years of age per year, 95% CI 0·03-0·07) and yearly frequency of lower respiratory prescribing consultations (rate ratio 0·982 opportunities per year, 95% CI 0·979-0·985). Prevalence of all comorbidities present at COPD diagnosis increased except for asthma and bronchiectasis, which decreased between 1990 and 2007, from 281 (33·4%) of 842 patients to 451 of 1465 (30·8%) for asthma, and from 53 of 842 (6·3%) to 53 of 1465 (3·6%) for bronchiectasis. In the 2 years before diagnosis, of 6897 patients who had had a chest radiography, only 2296 (33%) also had spirometry. Opportunities to diagnose COPD at an earlier stage are being missed, and could be improved by case-finding in patients with lower respiratory tract symptoms and concordant long-term comorbidities. UK Department of Health, Research in Real Life. Copyright © 2014 Elsevier Ltd. All rights reserved.

Evaluation of plasma, erythrocytes, and bronchoalveolar lavage fluid antioxidant defense system in sulfur mustard-injured patients.

PubMed

Jafari, Mahvash; Ghanei, Mostafa

2010-03-01

Sulfur mustard (SM) is a strong alkylating agent that causes acute and chronic effects on different organs following exposure. Main late respiratory complications are chronic obstructive pulmonary disease, bronchiectasis, asthma, and bronchiolitis obliterans. It seems that oxidative stress plays a major role in pathogenesis of diseases. This study was undertaken to evaluate the long-term effect of SM on plasma, erythrocytes, and brochoalveolar lavage fluid antioxidant defense system in SM-injured patients. Brochoalveolar lavage fluid, plasma, and erythrocyte samples were taken from 54 patients in the case group exposed to SM and 25 controls with chronic respiratory disease without a history of exposure to SM. Superoxide dismutase, catalase, and glutathione peroxidase activities in lavage fluid, plasma, and erythrocytes were significantly higher in case group. The increased glutathione S-transferase activity in lavage fluid was associated with a depletion of glutathione and an increase of malondialdehyde levels. There was no significant change observed in glutathione reductase activity. The data suggest that oxidative damage might have an important role for patients exposed to SM. SM may induce an oxidative stress response by depleting the antioxidant defense systems and increasing lipid peroxidation in lung cells.

Antisynthetase syndrome: Analysis of 11 cases.

PubMed

Zamarrón-de Lucas, Ester; Gómez Carrera, Luis; Bonilla, Gema; Petit, Dessiree; Mangas, Alberto; Álvarez-Sala, Rodolfo

2017-02-23

Antisynthetase syndrome (ASS) is characterised by a series of clinical manifestations such as myositis, fever, mechanic's hands and diffuse interstitial lung disease (ILD), all associated with positivity to antisynthetase antibodies. The presence of ILD will be that, to a great extent it will mark the response to treatment and prognosis. Eleven cases of patients with ASS and pulmonary involvement in monitoring at a Pulmonary monographic consult in a third level hospital consult are described. Nine patients presented positivity to anti-Jo antibody and 2 to anti-PL12. Four patients' HRCT pattern showed NSIP, four UIP, one COP and 2 ground-glass opacity. A percentage of 73 were accompanied by bronchiectasis and bronchiolectasis and 27% honeycombing. Functional exploration was mainly affected by DLCO with up to 45% of the positive walking test. Corticodependence is highlighted, often requiring immunosuppressive treatment both chronically and in exacerbations. All patients maintain good prognosis so far. Patients with interstitial lung disease should have at least a determination of antisynthetase antibodies in order to identify this disease, better prognosis than other interstitial diseases such as idiopathic pulmonary fibrosis. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

Magnetomotive optical coherence elastography for relating lung structure and function in cystic fibrosis

NASA Astrophysics Data System (ADS)

Chhetri, Raghav K.; Carpenter, Jerome; Superfine, Richard; Randell, Scott H.; Oldenburg, Amy L.

2010-02-01

Cystic fibrosis (CF) is a genetic defect in the cystic fibrosis transmembrane conductance regulator protein and is the most common life-limiting genetic condition affecting the Caucasian population. It is an autosomal recessive, monogenic inherited disorder characterized by failure of airway host defense against bacterial infection, which results in bronchiectasis, the breakdown of airway wall extracellular matrix (ECM). In this study, we show that the in vitro models consisting of human tracheo-bronchial-epithelial (hBE) cells grown on porous supports with embedded magnetic nanoparticles (MNPs) at an air-liquid interface are suitable for long term, non-invasive assessment of ECM remodeling using magnetomotive optical coherence elastography (MMOCE). The morphology of ex vivo CF and normal lung tissues using OCT and correlative study with histology is also examined. We also demonstrate a quantitative measure of normal and CF airway elasticity using MMOCE. The improved understanding of pathologic changes in CF lung structure and function and the novel method of longitudinal in vitro ECM assessment demonstrated in this study may lead to new in vivo imaging and elastography methods to monitor disease progression and treatment in cystic fibrosis.

HIV-related chronic lung disease in adolescents: are we prepared for the future?

PubMed

Goussard, Pierre; Gie, Robert P

2017-12-01

Chronic lung diseases (CLD) are the most chronic disease occurring in adolescents living with human immunodeficiency virus (ALHIV). In ALHIV who received antiretroviral therapy (ART) late in childhood, bronchiectasis, bronchiolitis obliterans and interstitial pneumonitis are common. In adolescents who received ART early in life the spectrum of CLD has changed with asthma and chronic obstructive pulmonary disease being common. Areas covered: The aim of this paper was to review CLD in ALHIV. We conducted a literature review of electronic databases focusing on CLD that were common prior to the introduction of ART (1996-2004), the present situation where ART is widely available (2005 to 2016), and articles which aided us speculating on the impact of HIV-related CLD in adolescents transitioning to adult HIV-clinics. Amongst the approximately 2.1 million adolescents living with HIV, CLD commonly occurs. Awareness of the CLD amongst ALHIV needs to be raised to ensure that disease appropriate treatment is available to these vulnerable adolescents. Expert commentary: As adolescents' transition from pediatric HIV-clinics to adult HIV-clinics the evidence shows that adolescents might not receive optimal care if adult pulmonologists are not aware of the CLD that commonly occur in ALHIV.

Chest CT findings in patients with dysphagia and aspiration: a systematic review

PubMed Central

Scheeren, Betina; Gomes, Erissandra; Alves, Giordano; Marchiori, Edson; Hochhegger, Bruno

2017-01-01

ABSTRACT The objective of this systematic review was to characterize chest CT findings in patients with dysphagia and pulmonary aspiration, identifying the characteristics and the methods used. The studies were selected from among those indexed in the Brazilian Virtual Library of Health, LILACS, Indice Bibliográfico Español de Ciencias de la Salud, Medline, Cochrane Library, SciELO, and PubMed databases. The search was carried out between June and July of 2016. Five articles were included and reviewed, all of them carried out in the last five years, published in English, and coming from different countries. The sample size in the selected studies ranged from 43 to 56 patients, with a predominance of adult and elderly subjects. The tomographic findings in patients with dysphagia-related aspiration were varied, including bronchiectasis, bronchial wall thickening, pulmonary nodules, consolidations, pleural effusion, ground-glass attenuation, atelectasis, septal thickening, fibrosis, and air trapping. Evidence suggests that chest CT findings in patients with aspiration are diverse. In this review, it was not possible to establish a consensus that could characterize a pattern of pulmonary aspiration in patients with dysphagia, further studies of the topic being needed. PMID:28767772

Chest CT findings in patients with dysphagia and aspiration: a systematic review.

PubMed

Scheeren, Betina; Gomes, Erissandra; Alves, Giordano; Marchiori, Edson; Hochhegger, Bruno

2017-01-01

The objective of this systematic review was to characterize chest CT findings in patients with dysphagia and pulmonary aspiration, identifying the characteristics and the methods used. The studies were selected from among those indexed in the Brazilian Virtual Library of Health, LILACS, Indice Bibliográfico Español de Ciencias de la Salud, Medline, Cochrane Library, SciELO, and PubMed databases. The search was carried out between June and July of 2016. Five articles were included and reviewed, all of them carried out in the last five years, published in English, and coming from different countries. The sample size in the selected studies ranged from 43 to 56 patients, with a predominance of adult and elderly subjects. The tomographic findings in patients with dysphagia-related aspiration were varied, including bronchiectasis, bronchial wall thickening, pulmonary nodules, consolidations, pleural effusion, ground-glass attenuation, atelectasis, septal thickening, fibrosis, and air trapping. Evidence suggests that chest CT findings in patients with aspiration are diverse. In this review, it was not possible to establish a consensus that could characterize a pattern of pulmonary aspiration in patients with dysphagia, further studies of the topic being needed.

Diagnostic dilemma: aspergillosis.

PubMed

Kothari, K; Singh, V; Sharma, R; Khandelwal, R

2000-04-01

Two cases of varied forms of Aspergillosis are reported who were being diagnosed and treated on different lines. One case, who was treated on lines of allergic bronchitis, had very high total eosinophil count and, fleeting pulmonary infiltrates over a period of 5 years along with history of cough, fever and weight loss. Aspergillus fumigatus was grown on sputum culture. On the background of a long standing history of bronchial asthma with evidence of peripheral as well as central eosinophilia, fleeting pulmonary infiltrates and A. fumigatus grown on sputum culture, we kept the diagnosis of Allergic Bronchopulmonary aspergillosis (ABPA) and put the patient on steroids and Itraconazole. Patient showed good response to therapy. Another case, a 50 year old male, presented to us with clinical picture of subacute myelitis. Being a known case of ABPA and on steroid therapy for long duration, we kept the diagnosis of invasive aspergillosis. Growth of Aspergillus fumigatus on sputum culture on three occasions and MR imaging of spine further supported our view. Aspergillosis of the lung do not have characteristic clinico-radiological features of permit the diagnosis and should be considered in the differential diagnosis of tuberculosis, pneumonia, bronchiectasis, lung abscess and bronchial asthma.

Induced tolerance to nebulized colistin after severe reaction to the drug.

PubMed

Domínguez-Ortega, J; Manteiga, E; Abad-Schilling, C; Juretzcke, M A; Sánchez-Rubio, J; Kindelan, C

2007-01-01

Daily nebulized colistin therapy has been used as maintenance therapy for patients with chronic Pseudomonas aeruginosa infection and in treatment protocols aimed at eradicating early P aeruginosa infection. Colistin-induced nephrotoxicity and mild neurotoxic effects have been described but hypersensitivity reactions are rare. However, bronchial constriction has been reported associated with the inhalation of the antibiotic. We report the case of a 63-year-old man who had been diagnosed with bronchiectasis and bronchopleural fistula and who developed severe bronchospasm when using nebulized colistin. A skin prick test (80 mg/mL) with colistin was performed and was negative. An intradermal test was not performed due to its possible irritant effect. As our patient suffered from a tobramycin-resistant P aeruginosa infection, we started a procedure to induce tolerance to 80 mg colistin (8 mg, 16 mg, 24 mg, 32 mg, 40 mg, 80 mg) nebulized in 30-minutes-intervals. No changes in forced expiratory volume in 1 second values were observed and the patient continues on treatment twice daily after the tolerance induction with no new episodes of bronchospasm. We report the first successful procedure to induce tolerance to colistin after escalating doses of inhaled colistin.

Pulmonary changes on HRCT scans in nonsmoking females with COPD due to wood smoke exposure *

PubMed Central

Moreira, Maria Auxiliadora Carmo; Barbosa, Maria Alves; de Queiroz, Maria Conceição de Castro Antonelli Monteiro; Teixeira, Kim Ir Sen Santos; Torres, Pedro Paulo Teixeira e Silva; de Santana, Pedro José; Montadon, Marcelo Eustáquio; Jardim, José Roberto

2013-01-01

OBJECTIVE: To identify and characterize alterations seen on HRCT scans in nonsmoking females with COPD due to wood smoke exposure. METHODS: We evaluated 42 nonsmoking females diagnosed with wood smoke-related COPD and 31 nonsmoking controls with no history of wood smoke exposure or pulmonary disease. The participants completed a questionnaire regarding demographic data, symptoms, and environmental exposure. All of the participants underwent spirometry and HRCT of the chest. The COPD and control groups were adjusted for age (23 patients each). RESULTS: Most of the patients in the study group were diagnosed with mild to moderate COPD (83.3%). The most common findings on HRCT scans in the COPD group were bronchial wall thickening, bronchiectasis, mosaic perfusion pattern, parenchymal bands, tree-in-bud pattern, and laminar atelectasis (p < 0.001 vs. the control group for all). The alterations were generally mild and not extensive. There was a positive association between bronchial wall thickening and hour-years of wood smoke exposure. Centrilobular emphysema was uncommon, and its occurrence did not differ between the groups (p = 0.232). CONCLUSIONS: Wood smoke exposure causes predominantly bronchial changes, which can be detected by HRCT, even in patients with mild COPD. PMID:23670500

Spectroscopic, structural and drug docking studies of carbocysteine

NASA Astrophysics Data System (ADS)

Manivannan, M.; Rajeshwaran, K.; Govindhan, R.; Karthikeyan, B.

2017-09-01

Carbocysteine or carbocisteine having the empirical formula C5H9NO4S,is one of the most therapeutically prescribed expectorant, sold under the brand name viz., Mucodyne (UK and India), Rhinathiol and Mucolite. In pediatric respiratory pathology, it can relieve the symptoms of obstructive pulmonary disease (COPD) and bronchiectasis. On the consideration of its extensive pharmaceutical usage and medicinal value, we have investigated its chemical structure and composition by employing various spectral techniques like 1H, 13C NMR, FT-IR,Raman, UV-Visible spectroscopy and powder X-ray diffraction method. Density Functional Theoretical (DFT) studies on its electronic structure is also carried out. Drug docking studies were carried out to ascertain the nature of molecular interaction with the biological protein system. Furthermore theoretical Raman spectrum of this molecule has been computed and compared with the experimental Raman spectrum. The forbidden energy gap between its frontier molecular orbitals, viz., HOMO-LUMO is calculated and correlated with its observed λmax value. Atomic orbitals which are mainly contributes to the frontier molecular orbitals were identified. Molecular electrostatic potential diagram has been mapped to explain its chemical activity. Based on the results, a suitable mechanism of its protein binding mode and drug action has been discussed.

Arsenic exposure and its impact on health in Chile.

PubMed

Ferreccio, Catterina; Sancha, Ana María

2006-06-01

The problem of arsenic in Chile was reviewed. In Chile, the population is exposed to arsenic naturally via drinking-water and by air pollution resulted from mining activities. The sources of arsenic were identified to estimate the exposure of population to arsenic through air, water, and food. Health effects, particularly early effects, observed in children and adults, such as vascular diseases (premature cardiac infarct), respiratory illnesses (bronchiectasis), and skin lesions have been described. Chronic effects, such as lung and bladder cancers, were reported 20 years after peak exposure and persisted 27 years after mitigation measures for removing arsenic from drinking surface water were initiated. Although the effects of arsenic are similar in different ethnic and cultural groups (e.g. Japanese, Chinese, Indian, Bangladeshi, American, and Taiwanese), variations could be explained by age at exposure, the dose received, smoking, and nutrition. Since health effects were observed at arsenic levels of 50 microg/L in drinking-water, it is advised that Chile follows the World Health Organization's recommendation of 10 microg/L. The Chilean experience in removal of arsenic suggests that it is feasible to reach this level using the conventional coagulation process.

The global burden of respiratory infections in indigenous children and adults: A review.

PubMed

Basnayake, Thilini L; Morgan, Lucy C; Chang, Anne B

2017-11-01

This review article focuses on common lower respiratory infections (LRIs) in indigenous populations in both developed and developing countries, where data is available. Indigenous populations across the world share some commonalities including poorer health and socio-economic disadvantage compared with their non-indigenous counterparts. Generally, acute and chronic respiratory infections are more frequent and more severe in both indigenous children and adults, often resulting in substantial consequences including higher rates of bronchiectasis and poorer outcomes for patients with chronic obstructive pulmonary disease (COPD). Risk factors for the development of respiratory infections require recognition and action. These risk factors include but are not limited to socio-economic factors (e.g. education, household crowding and nutrition), environmental factors (e.g. smoke exposure and poor access to health care) and biological factors. Risk mitigation strategies should be delivered in a culturally appropriate manner and targeted to educate both individuals and communities at risk. Improving the morbidity and mortality of respiratory infections in indigenous people requires provision of best practice care and awareness of the scope of the problem by healthcare practitioners, governing bodies and policy makers. © 2017 Asian Pacific Society of Respirology.

Pulmonary aspergilloma: A rare differential diagnosis to lung cancer after positive FDG PET scan

PubMed Central

Spycher, Franziska; Kocher, Gregor J.; Gugger, Mathias; Geiser, Thomas; Ott, Sebastian R.

2014-01-01

Early diagnosis and treatment of lung cancer, one of the leading causes of cancer-related death, is important to improve morbidity and mortality. Therefore any suspect solitary pulmonary nodule should prompt the pursuit for a definitive histological diagnosis. We describe the case of a 55-years-old male ex-smoker, who was admitted to our hospital due to recurrent hemoptysis and dry cough. A CT scan showed an irregular nodule of increasing size (28 mm in diameter) in the left lower lobe (LLL). A whole body PET-CT scan (643 MBq F-18 FDG i.v.) was performed and confirmed an avid FDG uptake of the nodule in the LLL, highly suspicious of lung cancer, without any evidence of lymphogenic or hematogenic metastasis. Bronchoscopy was not diagnostic and due to severe adhesions after prior chest trauma and the central location of the nodule, a lobectomy of the LLL was performed. Surprisingly, histology showed a simple aspergilloma located in a circumscribed bronchiectasis with no evidence of malignancy. This is a report of an informative example of an aspergilloma, which presented with symptoms and radiological features of malignant lung cancer. PMID:26029524

Sarcoidosis and multiple myeloma: Concurrent presentation of an unusual association

PubMed Central

Nair, Vidya; Prajapat, Deepak; Talwar, Deepak

2016-01-01

Literature on concurrent association of sarcoidosis with lymphoproliferative malignancies other than lymphoma e.g. multiple myeloma is meager. The rarity of the situation prompted us to report this patient who was a 51-year-old woman with a 2-years history of breathlessness, cough with expectoration, chest pain and backache. Initial evaluation revealed mild anemia, increased alkaline phosphatase with chest skiagram showing both lower zone non homogenous opacities with calcified hilar lymph nodes. CECT chest showed mediastinal with bilateral hilar lymphadenopathy, parenchymal fibrosis, traction bronchiectasis, ground glass opacities, septal and peribronchovascular thickening affecting mid and lower lung zones bilaterally. MRI Dorsolumbar spine was suggestive of marrow infiltrative disorder. EBUS FNA of intrathoracic nodes, EBB and TBLB confirmed sarcoidosis. PET CT revealed hyper metabolic activity in lung, multiple lymph nodes and lytic bone lesions. Serum protein electrophoresis and immunofixation revealed a monoclonal paraprotein, immunoglobulin IgG kappa type. Bone marrow biopsy revealed an increase in plasma cells (15%), but no granulomas. Diagnosis of Indolent or multiple myeloma with sarcoidosis was established. 12 cases of sarcoidosis and multiple myeloma have been reported in literature, and mostly preceding the onset of multiple myeloma by many years, in our case both were diagnosed concurrently. PMID:26933313

[USE OF QuantiFERON-TB Gold in Tube AND T-SPOT.TB FOR DIAGNOSING PATIENTS WITH SUSPECTED PULMONARY TUBERCULOSIS].

PubMed

Okimoto, Niro; Kurihara, Takeyuki; Miyashita, Naoyuki

2016-04-01

We analyzed the use of QFT-TB Gold in Tube and T-SPOT.TB in diagnosing patients with suspected pulmonary tuberculosis. We evaluated 122 patients with suspected pulmonary tuberculosis (where chest X-ray showed consolidation or. tumor shadow in predilection sites of pulmonary tuberculosis and through contact investigation). QFT-TB Gold and T-SPOT.TB were performed for all the patients. The positive response rate and history of pulmonary tuberculosis in patients who showed positive results for the tests were evaluated. Ninteen patients showed positive results for QFT-TB Gold, and 9, for T-SPOT.TB. Four patients showed positive results for QFT-TB Gold, and 3, for T-SPOT.TB in 4 patients with active tuberculosis. The patients without active tuberculosis whose IGRAs were positive (old pulmonary tuberculosis, Mycobacterium avium cmplex, pneumonia, lung cancer, pulmonary sequestration, bronchiectasis) had a past history of pulmonary tuberculosis. The positive result rate of QFT?-TB Gold was higher than that of T-SPOT.TB in the subjects with suspected pulmonary tuberculosis. We think that QFT-TB Gold reflected the past history of pulmonary tuberculosis.

Neutrophil formyl-peptide receptors. Relationship to peptide-induced responses and emphysema.

PubMed

Stockley, R A; Grant, R A; Llewellyn-Jones, C G; Hill, S L; Burnett, D

1994-02-01

A reproducible assay was established to assess the number of formyl-peptide receptors expressed on the surface of human polymorphonuclear leukocytes (PMN). Using this assay the number of receptors was shown to demonstrate wide within- and between-subject variability. However, the receptor numbers were related to the chemotactic response (r = 0.572) and degranulation response (r = 0.512) to the peptide formyl-methionyl-leucyl-phenylalanine. Subsequent studies showed increased receptor numbers on PMN from patients with emphysema (median, 459 x 10(3)/cell; range, 207 to 1,080) as compared with age-matched control subjects (median, 288; range, 168 to 519; p < 0.02), which may explain the increased chemotactic response of the PMN to formyl peptides. This difference was not observed in patients with bronchiectasis, suggesting that the increased receptor number is a feature of emphysema. Furthermore, the increase was largely a feature of smokers with emphysema (median, 463; range, 362 to 1,080), whereas age-matched smokers without emphysema had lower numbers of receptors (p < 0.001; median, 332; range, 243 to 411). This observation suggests a mechanism that may explain the susceptibility of some smokers to the development of emphysema.

Effects of radiation therapy on the lung: radiologic appearances and differential diagnosis.

PubMed

Choi, Yo Won; Munden, Reginald F; Erasmus, Jeremy J; Park, Kyung Joo; Chung, Woo Kyung; Jeon, Seok Chol; Park, Choong-Ki

2004-01-01

Radiation-induced lung disease (RILD) due to radiation therapy is common. Radiologic manifestations are usually confined to the lung tissue within the radiation port and are dependent on the interval after completion of treatment. In the acute phase, RILD typically manifests as ground-glass opacity or attenuation or as consolidation; in the late phase, it typically manifests as traction bronchiectasis, volume loss, and scarring. However, the use of oblique beam angles and the development of newer irradiation techniques such as three-dimensional conformal radiation therapy can result in an unusual distribution of these findings. Awareness of the atypical manifestations of RILD can be useful in preventing confusion with infection, recurrent malignancy, lymphangitic carcinomatosis, and radiation-induced tumors. In addition, knowledge of radiologic findings that are outside the expected pattern for RILD can be useful in diagnosis of infection or recurrent malignancy. Such findings include the late appearance or enlargement of a pleural effusion; development of consolidation, a mass, or cavitation; and occlusion of bronchi within an area of radiation-induced fibrosis. A comprehensive understanding of the full spectrum of these manifestations is important to facilitate diagnosis and management in cancer patients treated with radiation therapy. Copyright RSNA, 2004

Cartilage-hair hypoplasia with normal height in childhood-4 patients with a unique genotype.

PubMed

Klemetti, P; Valta, H; Kostjukovits, S; Taskinen, M; Toiviainen-Salo, S; Mäkitie, O

2017-08-01

The manifestations of cartilage-hair hypoplasia (CHH), a metaphyseal chondrodysplasia caused by RMRP mutations, include short stature, hypoplastic hair, immunodeficiency and increased risk of malignancies. Clinical features show significant variability. We report a patient with normal height until age 12.5 years (-1.6 SDS at 11 years) who was diagnosed with CHH at 14 years. RMRP sequencing revealed compound heterozygosity for g.70A>G mutation and a 10-nucleotide duplication at position -13 (TACTCTGTGA). Through the Finnish Skeletal Dysplasia Register, we identified 3 additional patients with identical genotype. Two of them also showed unusually mild growth failure (height SDS -1.6 at 14 years and -3.0 at 12 years, respectively). Three of the 4 patients suffered from recurrent infections; 1 developed progressive bronchiectasis and another died from aggressive lymphoma. Our findings expand the phenotypic variability in CHH to include normal childhood height. The milder growth retardation related to this particular genotype was not associated with less severe extra-skeletal manifestations, emphasizing the need for careful follow-up also in CHH patients with mild-skeletal manifestations. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

[Clinical studies on SM-4300].

PubMed

Sawae, Y; Nagafuchi, S

1985-09-01

Use of SM-4300, which is a newly developed human immunoglobulin preparation for intravenous administration, has clinically been evaluated in the patients with severe or intractable bacterial infections. Of total 13 cases of the admitted patients at the 1st department of internal medicine, faculty of medicine, Kyushu university, 10-pneumonia case were associated with blood diseases like acute myelocytic leukemia (AML) and multiple myeloma (MM), and in addition, with other underlying diseases like lung cancer and bronchiectasis, 1 was prosthetic valve endocarditis, 1 cholecystitis associated with pericarditis and 1 fever of undetermined origin (FUO). SM-4300 of 5 g single bolus or 3 daily doses of 2.5 g per day were infused with chemotherapy drugs preceedingly administered for more than 3 days and the results were evaluated; good in 4, fair 4, poor 2 and unknown 3, and the efficacy rate was 40%. Bacteriologically, the results were decreased in 1, persisted 1 and the majority was unknown. Observed were no side reactions nor the changes in clinical examination variables incurred by this drug. It is therefore considered that SM-4300 is of use for the treatment of intractable bacterial infections when used with antibiotics.

Weapons of mass destruction, WMD.

PubMed

Vogel, H

2007-08-01

Since the invasion into Iraq in 2003, weapons of mass destruction (WMD), have come to general notice; they include today chemical, biological, and atomic/nuclear weapons, (CW, BW, and AW). Radiological findings shall be described. X-ray findings of victims of WMD are described. From CW, own observations are reported. Examples of (possible) X-ray findings of victims of BW are described. AW may induce radiation disease. Exposure to sulfur-lost induces severe bronchitis; if the radiograph shows pulmonary infiltrations, the prognosis is bad; a late consequence maybe bronchiectasis. BW can be based on bacteria, virus or toxins. An approach of the X-ray findings for BW victims is based on the assumption that the disease induced by BW has the same (or a similar) clinic and radiology as that induced by the original microorganism or by the unchanged toxism. This approximation may have its limits, if the germ or toxin has been modified. In survivors of AW, the radiology is probably that of victims of thermal radiation and blast. WMD seem to be a real or a possible threat. They can be used in war, in terrorist attacks, in crime, and in action of secret services. In case that WMD are employed, X-ray diagnostic will be used to evaluate the prognosis (triage) and the risk of infection.

Sperm-Associated Antigen–17 Gene Is Essential for Motile Cilia Function and Neonatal Survival

PubMed Central

Teves, Maria Eugenia; Zhang, Zhibing; Costanzo, Richard M.; Henderson, Scott C.; Corwin, Frank D.; Zweit, Jamal; Sundaresan, Gobalakrishnan; Subler, Mark; Salloum, Fadi N.; Rubin, Bruce K.

2013-01-01

Primary ciliary dyskinesia (PCD), resulting from defects in cilia assembly or motility, is caused by mutations in a number of genes encoding axonemal proteins. PCD phenotypes are variable, and include recurrent respiratory tract infections, bronchiectasis, hydrocephaly, situs inversus, and male infertility. We generated knockout mice for the sperm-associated antigen–17 (Spag17) gene, which encodes a central pair (CP) protein present in the axonemes of cells with “9 + 2” motile cilia or flagella. The targeting of Spag17 resulted in a severe phenotype characterized by immotile nasal and tracheal cilia, reduced clearance of nasal mucus, profound respiratory distress associated with lung fluid accumulation and disruption of the alveolar epithelium, cerebral ventricular expansion consistent with emerging hydrocephalus, failure to suckle, and neonatal demise within 12 hours of birth. Ultrastructural analysis revealed the loss of one CP microtubule in approximately one quarter of tracheal cilia axonemes, an absence of a C1 microtubule projection, and other less frequent CP structural abnormalities. SPAG6 and SPAG16 (CP proteins that interact with SPAG17) were increased in tracheal tissue from SPAG17-deficient mice. We conclude that Spag17 plays a critical role in the function and structure of motile cilia, and that neonatal lethality is likely explained by impaired airway mucociliary clearance. PMID:23418344

Interaction between mycobacteria and mucus on a human respiratory tissue organ culture model with an air interface.

PubMed

Middleton, A M; Chadwick, M V; Nicholson, A G; Wilson, R; Thornton, D J; Kirkham, S; Sheehan, J K

2004-01-01

Mycobacteria adhere specifically to extracellular matrix (ECM) and mucus with a fibrous, but not globular, appearance, in organ cultures of human respiratory mucosa examined by scanning electron microscopy. Previously, light microscopy sections made of tissue infected for 7 days demonstrated mycobacteria associated with mucus on the organ culture surface, and within submucosal glands in areas of damaged epithelium. The authors have now investigated the interactions between Mycobacterium avium complex (MAC), Mycobacterium tuberculosis (MTB), and Mycobacterium smegmatis (MS) and mucus by preincubating bacteria with purified mucins MUC5AC and MUC5B prior to inoculation onto the organ culture mucosal surface. They have also measured mucin production by the organ culture after mycobacterial infection. Mucus did not cause clumping of mycobacteria. There was a significant (P=.03) increase in the amount of fibrous mucus, but not globular mucus, observed on tissue inoculated with mucins compared to controls. The number of bacteria adhering to ECM was markedly reduced after incubation with mucins, which could indicate a protective effect. Mycobacterial infection did not increase mucin production by the organ culture. Mycobacterial adherence to mucins may play a role in the pathogenicity of mycobacteria in diseases such as cystic fibrosis, bronchiectasis, and chronic obstructive pulmonary disease (COPD), in which there are changes in mucus composition and clearance.

Respiratory Disease Related Mortality and Morbidity on an Island of Greece Exposed to Perlite and Bentonite Mining Dust

PubMed Central

Sampatakakis, Stefanos; Linos, Athena; Papadimitriou, Eleni; Petralias, Athanasios; Dalma, Archontoula; Papasaranti, Eirini Saranti; Christoforidou, Eleni; Stoltidis, Melina

2013-01-01

A morbidity and mortality study took place, focused on Milos Island, where perlite and bentonite mining sites are located. Official data concerning number and cause of deaths, regarding specific respiratory diseases and the total of respiratory diseases, for both Milos Island and the Cyclades Prefecture were used. Standardized Mortality Ratios (SMRs) were computed, adjusted specifically for age, gender and calendar year. Tests of linear trend were performed. By means of a predefined questionnaire, the morbidity rates of specific respiratory diseases in Milos, were compared to those of the municipality of Oinofita, an industrial region. Chi-square analysis was used and the confounding factors of age, gender and smoking were taken into account, by estimating binary logistic regression models. The SMRs for Pneumonia and Chronic Obstructive Pulmonary Disease (COPD) were found elevated for both genders, although they did not reach statistical significance. For the total of respiratory diseases, a statistically significant SMR was identified regarding the decade 1989–1998. The morbidity study revealed elevated and statistically significant Odds Ratios (ORs), associated with allergic rhinitis, pneumonia, COPD and bronchiectasis. An elevated OR was also identified for asthma. After controlling for age, gender and smoking, the ORs were statistically significant and towards the same direction. PMID:24129114

Freezing the Bioactive Conformation to Boost Potency: The Identification of BAY 85-8501, a Selective and Potent Inhibitor of Human Neutrophil Elastase for Pulmonary Diseases

PubMed Central

von Nussbaum, Franz; Li, Volkhart M-J; Allerheiligen, Swen; Anlauf, Sonja; Bärfacker, Lars; Bechem, Martin; Delbeck, Martina; Fitzgerald, Mary F; Gerisch, Michael; Gielen-Haertwig, Heike; Haning, Helmut; Karthaus, Dagmar; Lang, Dieter; Lustig, Klemens; Meibom, Daniel; Mittendorf, Joachim; Rosentreter, Ulrich; Schäfer, Martina; Schäfer, Stefan; Schamberger, Jens; Telan, Leila A; Tersteegen, Adrian

2015-01-01

Human neutrophil elastase (HNE) is a key protease for matrix degradation. High HNE activity is observed in inflammatory diseases. Accordingly, HNE is a potential target for the treatment of pulmonary diseases such as chronic obstructive pulmonary disease (COPD), acute lung injury (ALI), acute respiratory distress syndrome (ARDS), bronchiectasis (BE), and pulmonary hypertension (PH). HNE inhibitors should reestablish the protease–anti-protease balance. By means of medicinal chemistry a novel dihydropyrimidinone lead-structure class was identified. Further chemical optimization yielded orally active compounds with favorable pharmacokinetics such as the chemical probe BAY-678. While maintaining outstanding target selectivity, picomolar potency was achieved by locking the bioactive conformation of these inhibitors with a strategically positioned methyl sulfone substituent. An induced-fit binding mode allowed tight interactions with the S2 and S1 pockets of HNE. BAY 85-8501 ((4S)-4-[4-cyano-2-(methylsulfonyl)phenyl]-3,6-dimethyl-2-oxo-1-[3-(trifluoromethyl)phenyl]-1,2,3,4-tetrahydropyrimidine-5-carbonitrile) was shown to be efficacious in a rodent animal model related to ALI. BAY 85-8501 is currently being tested in clinical studies for the treatment of pulmonary diseases. PMID:26083237

Fungal infection by Mucorales order in lung transplantation: 4 case reports.

PubMed

Neto, F M F D; Camargo, P C L B; Costa, A N; Teixeira, R H O B; Carraro, R M; Afonso, J E; Campos, S V; Samano, M N; Fernandes, L M; Abdalla, L G; Pêgo-Fernandes, P M

2014-01-01

Mucorales is a fungus that causes systemic, highly lethal infections in immunocompromised patients. The overall mortality of pulmonary mucormycosis can reach 95%. This work is a review of medical records of 200 lung transplant recipients between the years of 2003 and 2013, in order to identify the prevalence of Mucorales in the Lung Transplantation service of Heart Institute (InCor), Hospital das Clínicas da Universidade de São Paulo, Brazil, by culture results from bronchoalveolar lavage and necropsy findings. We report 4 cases found at this analyses: 3 in patients with cystic fibrosis and 1 in a patient with bronchiectasis due to Kartagener syndrome. There were 2 unfavorable outcomes related to the presence of Mucorales, 1 by reduction of immunosuppression, another by invasive infection. Another patient died from renal and septic complications from another etiology. One patient was diagnosed at autopsy just 5 days after lung transplantation, with the Mucor inside the pulmonary vein with a precise, well-defined involvement only of donor's segment, leading to previous colonization hypothesis. There are few case reports of Mucorales infection in lung transplantation in the literature. Surveillance for the presence of Mucor can lead to timely fungal treatment and reduce morbidity and mortality in the immunocompromised patients, especially lung transplant recipients. Copyright © 2014 Elsevier Inc. All rights reserved.

Respiratory disease related mortality and morbidity on an island of Greece exposed to perlite and bentonite mining dust.

PubMed

Sampatakakis, Stefanos; Linos, Athena; Papadimitriou, Eleni; Petralias, Athanasios; Dalma, Archontoula; Papasaranti, Eirini Saranti; Christoforidou, Eleni; Stoltidis, Melina

2013-10-14

A morbidity and mortality study took place, focused on Milos Island, where perlite and bentonite mining sites are located. Official data concerning number and cause of deaths, regarding specific respiratory diseases and the total of respiratory diseases, for both Milos Island and the Cyclades Prefecture were used. Standardized Mortality Ratios (SMRs) were computed, adjusted specifically for age, gender and calendar year. Tests of linear trend were performed. By means of a predefined questionnaire, the morbidity rates of specific respiratory diseases in Milos, were compared to those of the municipality of Oinofita, an industrial region. Chi-square analysis was used and the confounding factors of age, gender and smoking were taken into account, by estimating binary logistic regression models. The SMRs for Pneumonia and Chronic Obstructive Pulmonary Disease (COPD) were found elevated for both genders, although they did not reach statistical significance. For the total of respiratory diseases, a statistically significant SMR was identified regarding the decade 1989-1998. The morbidity study revealed elevated and statistically significant Odds Ratios (ORs), associated with allergic rhinitis, pneumonia, COPD and bronchiectasis. An elevated OR was also identified for asthma. After controlling for age, gender and smoking, the ORs were statistically significant and towards the same direction.

Host-microbe interactions in distal airways: relevance to chronic airway diseases.

PubMed

Martin, Clémence; Burgel, Pierre-Régis; Lepage, Patricia; Andréjak, Claire; de Blic, Jacques; Bourdin, Arnaud; Brouard, Jacques; Chanez, Pascal; Dalphin, Jean-Charles; Deslée, Gaetan; Deschildre, Antoine; Gosset, Philippe; Touqui, Lhousseine; Dusser, Daniel

2015-03-01

This article is the summary of a workshop, which took place in November 2013, on the roles of microorganisms in chronic respiratory diseases. Until recently, it was assumed that lower airways were sterile in healthy individuals. However, it has long been acknowledged that microorganisms could be identified in distal airway secretions from patients with various respiratory diseases, including cystic fibrosis (CF) and non-CF bronchiectasis, chronic obstructive pulmonary disease, asthma and other chronic airway diseases (e.g. post-transplantation bronchiolitis obliterans). These microorganisms were sometimes considered as infectious agents that triggered host immune responses and contributed to disease onset and/or progression; alternatively, microorganisms were often considered as colonisers, which were considered unlikely to play roles in disease pathophysiology. These concepts were developed at a time when the identification of microorganisms relied on culture-based methods. Importantly, the majority of microorganisms cannot be cultured using conventional methods, and the use of novel culture-independent methods that rely on the identification of microorganism genomes has revealed that healthy distal airways display a complex flora called the airway microbiota. The present article reviews some aspects of current literature on host-microbe (mostly bacteria and viruses) interactions in healthy and diseased airways, with a special focus on distal airways. Copyright ©ERS 2015.

Phenotypic presentation of chronic cough in children

PubMed Central

2017-01-01

Chronic cough in children is increasingly defined as a cough that lasts more than four weeks. It is recognized as a different entity than cough in adults. As a result, the diagnostic approach and management of chronic cough in children are no longer extrapolated from adult guidelines. These differences are attributed to the various characteristics of the respiratory tract, immunological system and nervous system in children. Specific paediatric guidelines and algorithms for chronic cough are now widely applied. Post-infectious cough, asthma, bronchiectasis, malacia and protracted bacterial bronchitis (PBB) appear to be the major causes of cough in young children. By adolescence, the causes of cough are more likely to be similar to those in adults, namely, gastroesophageal reflux, asthma, and upper airway syndrome. In a primary setting, it is essential to investigate the underlying disease entity that initiates and sustains chronic cough. The use of cough management protocols or algorithms improves clinical outcomes and should differ depending on the associated characteristics of the cough and the child’s clinical history. Performing a thorough history and physical examination is crucial to starting an individualised approach. A correct interpretation of the phenotypic presentation can be translated into guidance for workup. This approach will be helpful for adequate management without the risk of inappropriate investigations or inadequate treatment. PMID:28523136

How I treat warts, hypogammaglobulinemia, infections, and myelokathexis syndrome.

PubMed

Badolato, Raffaele; Donadieu, Jean

2017-12-07

Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a genetic disease characterized by neutropenia, lymphopenia, susceptibility to infections, and myelokathexis, which describes degenerative changes of mature neutrophils and hyperplasia of bone marrow myeloid cells. Some patients present with hypogammaglobulinemia and/or refractory warts of skin and genitalia. Congenital cardiac defects constitute uncommon manifestations of the disease. The disorder, which is inherited as an autosomal dominant trait, is caused by heterozygous mutations of the chemokine receptor CXCR4. These mutations lead to an increased sensitivity of neutrophils and lymphocytes to the unique ligand CXCL12 and to an increased accumulation of mature neutrophils in the bone marrow. Despite greatly improved knowledge of the disease, therapeutic choices are insufficient to prevent some of the disease outcomes, such as development of bronchiectasis, anogenital dysplasia, or invasive cancer. The available therapeutic measures aimed at preventing the risk for infection in WHIM patients are discussed. We critically evaluate the diagnostic criteria of WHIM syndrome, particularly when WHIM syndrome should be suspected in patients with congenital neutropenia and lymphopenia despite the absence of hypogammaglobulinemia and/or warts. Finally, we discuss recent results of trials evaluating plerixafor, a selective antagonist of CXCR4, as a mechanism-oriented strategy for treatment of WHIM patients. © 2017 by The American Society of Hematology.

Apparent X-linked primary ciliary dyskinesia associated with retinitis pigmentosa and a hearing loss.

PubMed

Krawczyński, Maciej R; Dmeńska, Hanna; Witt, Michał

2004-01-01

Three brothers, one 10-year-old and a pair of 14-year-old dizygotic twins--expressed the classical, early-onset retinitis pigmentosa (RP) with typical ophthalmoscopic findings, night blindness, visual field constricted to 10 degrees and flat ERG response. All three brothers were also diagnosed with primary ciliary dyskinesia (PCD) and had recurrent respiratory infections, chronic sinusitis and bronchiectasis. In all of them, resection of the middle lobe of the right lung was performed. A similar clinical picture of coexisting RP and PCD was noted in the brother of the probands' mother. All probands displayed situs solitus. Consistent with the X-linked mode of RP inheritance, there were also three obligatory female carriers of the disorder in this family: the mother of the affected boys, her mother and a daughter of her brother. In all of them, retinitis pigmentosa "sine pigmento" was found with milder but clinically significant symptoms (mild night blindness, visual field constricted to 30 degrees, and scotopic and photopic ERG responses reduced to 30-60%). No extraocular symptoms were detected in any of the heterozygous female carriers. This family presents an example of two rare phenomena: X-linked dominant retinitis pigmentosa (with milder expression in females) and a rare combination of RP with recurrent respiratory infections due to PCD.

Clinical and Immunological Features of Common Variable Immunodeficiency in China

PubMed Central

Lin, Lian-Jun; Wang, Yu-Chuan; Liu, Xin-Min

2015-01-01

Background: Common variable immunodeficiency (CVID) is one of the most common symptomatic primary immunodeficiency syndromes. The purpose of this article was to broaden our knowledge about CVID for better diagnosis and treatment. Methods: Clinical and immunological features of 40 Chinese patients with CVID were analyzed retrospectively. Results: The median age at onset was 11-year-old (range 4–51 years). The median age at diagnosis was 14.5-year-old (range 5–66 years). The average time of delay in diagnosis was 5.3 years (range 1–41 years). The most common main complaint was fever due to infections (35 cases, 87.5%). Pneumonia (28 cases, 70%) was the most common type of infections. Bronchiectasis was present in 6 patients (15%). Autoimmune disease was detected in 6 cases of CVID, and malignancy in 2 cases. The median total serum levels of IgG, IgA, and IgM at diagnosis were 1.07 g/L, 0.07 g/L, and 0.28 g/L, respectively. The percentages of CD3−/CD10+ B-cells were 1%–3.14%. Conclusions: Infection is the most frequent presentation of CVID. Patients with unexplainable infections should receive further examination including serum immunoglobulin (Ig) and lymphocyte subset analysis. Regular and sufficient substitution with Ig is recommended. PMID:25635425

Osimertinib-induced interstitial lung disease in a patient with non-small cell lung cancer pretreated with nivolumab: A case report.

PubMed

Takakuwa, Osamu; Oguri, Tetsuya; Uemura, Takehiro; Sone, Kazuki; Fukuda, Satoshi; Okayama, Minami; Kanemitsu, Yoshihiro; Ohkubo, Hirotsugu; Takemura, Masaya; Ito, Yutaka; Maeno, Ken; Niimi, Akio

2017-09-01

Osimertinib (AZD9291) is a third-generation epidermal growth factor receptor (EGFR) tyrosine kinase inhibitor approved for EGFR-T790M-positive non-small cell lung cancer. A high incidence of interstitial lung disease (ILD) during combination treatment with osimertinib and anti-programmed cell death-ligand 1 (PD-L1) inhibitor has been reported. The current study presents a case of ILD development during osimertinib treatment following nivolumab (an anti-PD-1 antibody) treatment. The 59-year-old female was diagnosed with stage IV lung adenocarcinoma harboring a deletion in exon 19 of the EGFR gene. Following nivolumab as a sixth-line treatment, an EGFR-T790M-encoding mutation in EGFR exon 20 was identified by re-biopsy. Osimertinib was therefore initiated as a seventh-line treatment. A partial response was subsequently noted; however, 63 days after initiation of the treatment the patient presented with dyspnea with decreased oxygenation in the absence of fever and sputum. A computed tomography scan revealed the emergence of ground-glass opacities with bronchiectasis in both lungs, and a diagnosis of ILD due to osimertinib was made. Following steroid pulse therapy with discontinuation of osimertinib, the patient's chest findings and respiratory condition improved. Therefore, it is considered that anti-PD-1 therapies may be associated with a risk of ILD during subsequent osimertinib treatment.

Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry.

PubMed

Hajjar, Joud; Guffey, Danielle; Minard, Charles G; Orange, Jordan S

2017-02-01

Patients with primary immunodeficiency (PID) often report fatigue, yet this symptom has not been studied in PID. Fatigue affects 6-7.5% of healthy adults. The goal of this study is to estimate the prevalence of fatigue in patients with PID and investigate its associated factors. We analyzed 2537 PID patients registered in USIDNET to determine responses to the field "fatigue" in the core registry form. Demographics, immune phenotypes, and comorbid conditions were compared between fatigued and non-fatigued patients to identify relevant associations and potential drivers. A focused analysis was performed for patients with predominantly antibody deficiency disorders (PADs). Fatigue was reported in 25.9% (95% CI 23.7-28.3) of PAD patients, compared to 6.4% (95% CI 4.9-8.2) of non-PAD. Patients with common variable immunodeficiency (CVID) had the highest prevalence of fatigue (p < 0.001) among all PID diagnoses. Other factors that were associated with a higher rate of fatigue among PAD patients included female sex, higher BMI, depression, bronchiectasis, and autoimmunity. Additionally, fatigued PAD patients had lower absolute lymphocyte, CD3, CD4, and CD8 counts compared to non-fatigued patients. Our findings suggest that fatigue is overrepresented in PAD patients. Prospective studies to estimate prevalence, risk factors, and fatigue etiology in PID are warranted, so therapeutic interventions can be considered.

[Scedosporium apiospermum disseminated infection in a single lung transplant recipient].

PubMed

Solé, Amparo

2011-01-01

Scedosporium spp. are filamentous fungi, and the 2 most important species are Scedosporium prolificans and Scedosporium apiospermum. S. apiospermum accounts for approximately 25% of non-Aspergillus filamentous fungi infections in organ transplant recipients. Scedosporium can colonize the sinuses and airways of lung recipients with underlying pulmonary diseases, such as bronchiectasis or cystic fibrosis before transplant, and develop invasive disease after lung transplantation. In fact, invasive diseases caused by S. apiospermum have been reported only rarely, in single lung transplant recipients and cystic fibrosis transplant patients. The treatment of scedosporiasis is complicated due to the difficulty in early diagnosis together with inherent resistance to amphotericin B. A case of disseminated S. apiospermum infection after single lung transplant in a patient with pulmonary fibrosis is reported. Leg mycetoma was the initial sign of this disseminated infection. In this case report, current treatment options are discussed, and a review of the literature of previously published cases of lung transplants is made. One conclusion based on this case is the risk of emergent molds related to antifungal prophylaxis. In addition, colonization by Scedosporium in transplant recipients should not be ignored, and target prophylaxis or suppressive therapy should be considered in all those cases with residual lesions in native lung or chronic rejection in transplanted lungs. Copyright © 2011 Revista Iberoamericana de Micología. Published by Elsevier Espana. All rights reserved.

Stimulation effect of wide type CFTR chloride channel by the naturally occurring flavonoid tangeretin.

PubMed

Jiang, Yu; Yu, Bo; Wang, Xue; Sui, Yujie; Zhang, Yaofang; Yang, Shuang; Yang, Hong; Ma, Tonghui

2014-12-01

Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated chloride channel expressed in the apical membrane of serous epithelial cells. Both deficiency and overactivation of CFTR may cause fluid and salt secretion related diseases. In the present study, we identified tangeretin from Pericarpium Citri Reticulatae Viride as a CFTR activator using high-throughput screening based on FRT cell-based fluorescence assay. The activation effect of tangeretin on CFTR chloride channel and the possible underlying mechanisms were investigated. Fluorescence quenching tests showed that tangeretin dose- and time-dependently activated CFTR chloride channel, the activity had rapid and reversible characteristics and the activation effect could be completely reversed by the CFTR specific blocker CFTRinh-172. Primary mechanism studies indicated that the activation effect of tangeretin on CFTR chloride channel was FSK dependent as well as had additional effect with FSK and IBMX suggesting that tangeretin activates CFTR by direct interacting with the protein. Ex-vivo tests revealed that tangeretin could accelerate the speed of the submucosal gland fluid secretion. Short-circuit current measurement demonstrated that tangeretin activated rat colonic mucosa chloride current. Thus, CFTR Cl(-) channel is a molecular target of natural compound tangeretin. Tangeretin may have potential use for the treatment of CFTR-related diseases like cystic fibrosis, bronchiectasis and habitual constipation. Copyright © 2014 Elsevier B.V. All rights reserved.

Advanced sclerosis of the chest wall skin secondary to chronic graft-versus-host disease: a case with severe restrictive lung defect.

PubMed

Ödek, Çağlar; Kendirli, Tanil; İleri, Talia; Yaman, Ayhan; Fatih Çakmakli, Hasan; Ince, Elif; İnce, Erdal; Ertem, Mehmet

2014-10-01

Pulmonary chronic graft-versus-host disease (cGvHD) is one of the most common causes of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (aHSCT). Herein, we describe a patient with severe restrictive lung defect secondary to cGvHD. A 21-year-old male patient was admitted to our pediatric intensive care unit (PICU) with pneumonia and respiratory distress. He had a history of aHSCT for chronic myelogeneous leukemia at the age of 17 years. Six months after undergoing aHSCT, he had developed cGvHD involving skin, mouth, eye, lung, liver, and gastrointestinal tract. At the time of PICU admission he had respiratory distress and required ventilation support. Thorax high-resolution computed tomography was consistent with bronchiolitis obliterans. Although bronchiolitis obliterans is an obstructive lung defect, a restrictive pattern became prominent in the clinical course because of the sclerotic chest wall skin. The activity of cGvHD kept increasing despite the therapy and we lost the patient because of severe respiratory distress and massive hemoptysis secondary to bronchiectasis. In conclusion, pulmonary cGvHD can present with restrictive changes related with the advanced sclerosis of the chest wall skin. Performing a fasciotomy or a scar revision for the rigid chest wall in selected patients may improve the patients ventilation.

Evaluation of chronic arsenic poisoning due to consumption of contaminated ground water in West Bengal, India.

PubMed

Ghosh, Asutosh

2013-08-01

Chronic arsenic poisoning is an important public health problem and most notable in West Bengal and Bangladesh. In this study different systemic manifestations in chronic arsenic poisoning were evaluated. A nonrandomized, controlled, cross-sectional, observational study was carried out in Arsenic Clinic, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, over a period of 1 year 4 months. Seventy-three cases diagnosed clinically, consuming water containing arsenic ≥50 μg/L and having hair and nail arsenic level >0.6 μg/L, were included. Special investigations included routine parameters and organ-specific tests. Arsenic levels in the drinking water, hair, and nail were measured in all. Twenty-five nonsmoker healthy controls were evaluated. Murshidabad and districts adjacent to Kolkata, West Bengal, were mostly affected. Middle-aged males were the common sufferers. Skin involvement was the commonest manifestation (100%), followed by hepatomegaly [23 (31.5%)] with or without transaminitis [7 (9.58%)]/portal hypertension [9 (12.33%)]. Restrictive abnormality in spirometry [11 (15.06%)], bronchiectasis [4 (5.47%)], interstitial fibrosis [2 (2.73%)], bronchogenic carcinoma [2 (2.73%)], oromucosal plaque [7 (9.58%)], nail hypertrophy [10 (13.69%)], alopecia [8 (10.95%)], neuropathy [5 (6.84%)], and Electrocardiography abnormalities [5 (6.84%)] were also observed. Mucocutaneous and nail lesions, hepatomegaly, and restrictive change in spirometry were the common and significant findings. Other manifestations were characteristic but insignificant.

Interstitial lung disease in an adult with Fanconi anemia: Clues to the pathogenesis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Rubinstein, W.S.; Wenger, S.L.; Hoffman, R.M.

1997-03-31

We have studied a 38-year-old man with a prior diagnosis of Holt-Oram syndrome, who presented with diabetes mellitus. He had recently taken prednisone for idiopathic interstitial lung disease and trimethoprim-sulfamethoxazole for sinusitis. Thrombocytopenia progressed to pancytopenia. The patient had skeletal, cardiac, renal, cutaneous, endocrine, hepatic, neurologic, and hematologic manifestations of Fanconi anemia (FA). Chest radiographs showed increased interstitial markings at age 25, dyspnea began in his late 20s, and he stopped smoking at age 32. At age 38, computerized tomography showed bilateral upper lobe fibrosis, lower lobe honeycombing, and bronchiectasis. Pulmonary function tests, compromised at age 29, showed a moderatelymore » severe obstructive and restrictive pattern by age 38. Serum alpha-1 antitrypsin level was 224 (normal 85-213) mg/dL and PI phenotype was M1. Karyotype was 46,X-Y with a marked increase in chromosome aberrations induced in vitro by diepoxybutane. The early onset and degree of pulmonary disease in this patient cannot be fully explained by environmental or known genetic causes. The International Fanconi Anemia Registry (IFAR) contains no example of a similar pulmonary presentation. Gene-environment (ecogenetic) interactions in FA seem evident in the final phenotype. The pathogenic mechanism of lung involvement in FA may relate to oxidative injury and cytokine anomalies. 49 refs., 2 figs., 1 tab.« less

Respiratory infections in patients with cystic fibrosis undergoing lung transplantation.

PubMed

Lobo, Leonard J; Noone, Peadar G

2014-01-01

Cystic fibrosis is an inherited disease characterised by chronic respiratory infections associated with bronchiectasis. Lung transplantation has helped to extend the lives of patients with cystic fibrosis who have advanced lung disease. However, persistent, recurrent, and newly acquired infections can be problematic. Classic cystic fibrosis-associated organisms, such as Staphylococcus aureus and Pseudomonas aeruginosa, are generally manageable post-transplantation, and are associated with favourable outcomes. Burkholderia cenocepacia poses particular challenges, although other Burkholderia species are less problematic. Despite concerns about non-tuberculous mycobacteria, especially Mycobacterium abscessus, post-transplantation survival has not been definitively shown to be less than average in patients with these infections. Fungal species can be prevalent before and after transplantation and are associated with high morbidity, so should be treated aggressively. Appropriate viral screening and antiviral prophylaxis are necessary to prevent infection with and reactivation of Epstein-Barr virus and cytomegalovirus and their associated complications. Awareness of drug pharmacokinetics and interactions in cystic fibrosis is crucial to prevent toxic effects and subtherapeutic or supratherapeutic drug dosing. With the large range of potential infectious organisms in patients with cystic fibrosis, infection control in hospital and outpatient settings is important. Despite its complexity, lung transplantation in the cystic fibrosis population is safe, with good outcomes if the clinician is aware of all the potential pathogens and remains vigilant by means of surveillance and proactive treatment. Copyright © 2014 Elsevier Ltd. All rights reserved.

Long-term effects of mustard gas on respiratory system of Iranian veterans after Iraq-Iran war: a review.

PubMed

Razavi, Seyed-Mansour; Ghanei, Mostafa; Salamati, Payman; Safiabadi, Mehdi

2013-01-01

To review long-term respiratory effects of mustard gas on Iranian veterans having undergone Iraq-Iran war. Electronic databases of Scopus, Medline, ISI, IranMedex, and Irandoc sites were searched. We accepted articles published in scientific journals as a quality criterion.The main pathogenic factors are free radical mediators. Prevalence of pulmonary involvement is approximately 42.5%. The most common complaints are cough and dyspnea. Major respiratory complications are chronic obstructive pulmonary disease, bronchiectasis, and asthma. Spirometry results can reveal restrictive and obstructive pulmonary disease. Plain chest X-ray does not help in about 50% of lung diseases. High-resolution CT of the lung is the best modality for diagnostic assessment of parenchymal lung and bronchi. There is no definite curative treatment for mustard lung. The effective treatment regimens consist of oxygen administration, use of vaporized moist air, respiratory physiotherapy, administration of mucolytic agents, bronchodilators, corticosteroids, and long-acting beta-2 agonists, antioxidants, surfactant, magnesium ions, therapeutic bronchoscopy, laser therapy, placement of respiratory stents, early tracheostomy in laryngospasm, and ultimately lung transplantation. High-resolution CT of the lung is the most accurate modality for the evaluation of the lung parenchyma and bronchi. The treatment efficacy of patients exposed to mustard gas depends on patient conditions (acute or chronic, upper or lower respiratory tract involvement). There are various treatment protocols, but unfortunately none of them is definitely curable.

Domiciliary humidification improves lung mucociliary clearance in patients with bronchiectasis.

PubMed

Hasani, A; Chapman, T H; McCool, D; Smith, R E; Dilworth, J P; Agnew, J E

2008-01-01

Inspired air humidification has been reported to show some benefit in bronchiectatic patients. We have investigated the possibility that one effect might be to enhance mucociliary clearance. Such enhancement might, if it occurs, help to lessen the risks of recurrent infective episodes. Using a radioaerosol technique, we measured lung mucociliary clearance before and after 7 days of domiciliary humidification. Patients inhaled high flow saturated air at 37 degrees C via a patient-operated humidification nasal inhalation system for 3 h per day. We assessed tracheobronchial mucociliary clearance from the retention of (99m)Tc-labelled polystyrene tracer particles monitored for 6 h, with a follow-up 24-h reading. Ten out of 14 initially recruited patients (age 37-75 years; seven females) completed the study (two withdrew after their initial screening and two prior to the initial clearance test). Seven patients studied were non-smokers; three were ex-smokers (1-9 pack-years). Initial tracer radioaerosol distribution was closely similar between pre- and post-treatment. Following humidification, lung mucociliary clearance significantly improved, the area under the tracheobronchial retention curve decreased from 319 +/- 50 to 271 +/- 46%h (p < 0.07). Warm air humidification treatment improved lung mucociliary clearance in our bronchiectatic patients. Given this finding plus increasing laboratory and clinical interest in humidification mechanisms and effects, we believe further clinical trials of humidification therapy are desirable, coupled with analysis of humidification effects on mucus properties and transport.

Changes in neuroendocrine elements in bronchial mucosa in chronic lung disease in adults.

PubMed

Pilmane, M; Luts, A; Sundler, F

1995-05-01

It is not clear whether there is any association between metaplasia of the bronchial epithelium and changes in the distribution of neuroendocrine cells. This study examined, by immunohistological techniques, the distribution of neuroendocrine cells and juxtamucoscal nerve fibres in bronchial biopsies showing metaplastic changes. Bronchial biopsies from 12 subjects with epithelial metaplasia associated with bronchiectasis and diffuse pulmonary fibrosis were examined by conventional light microscopy and immunohistological techniques for protein gene product 9.5 (PGP), chromogranin A and B (CAB), serotonin, vasoactive intestinal peptide (VIP), substance P (SP), calcitonin gene-related peptide (CGRP), calcitonin (CT), and gastrin releasing peptide (GRP). Regions of non-metaplastic epithelium contained numerous PGP and serotonin immunoreactive cells. Sub-populations of these cells displayed CAB, CGRP, CT, and GRP immunoreactivity. Metaplastic epithelium contained only a few weakly stained PGP, serotonin, CAB, GRP, CT and CGRP immunoreactive cells in six cases. Metaplastic epithelium was characterised by a high number of CAB-containing cells in six cases and in these biopsies prominent PGP-containing nerve bundles were seen in the subepithelial layer beneath the metaplastic epithelium. The distribution patterns of neuroendocrine cells and neuronal elements vary between areas of normal and metaplastic epithelium and within areas of metaplastic epithelium. Neuronal hyperplasia was associated with an increase in the number of CAB-containing cells within the metaplastic epithelium.

Changes in neuroendocrine elements in bronchial mucosa in chronic lung disease in adults.

PubMed Central

Pilmane, M.; Luts, A.; Sundler, F.

1995-01-01

BACKGROUND--It is not clear whether there is any association between metaplasia of the bronchial epithelium and changes in the distribution of neuroendocrine cells. This study examined, by immunohistological techniques, the distribution of neuroendocrine cells and juxtamucoscal nerve fibres in bronchial biopsies showing metaplastic changes. METHODS--Bronchial biopsies from 12 subjects with epithelial metaplasia associated with bronchiectasis and diffuse pulmonary fibrosis were examined by conventional light microscopy and immunohistological techniques for protein gene product 9.5 (PGP), chromogranin A and B (CAB), serotonin, vasoactive intestinal peptide (VIP), substance P (SP), calcitonin gene-related peptide (CGRP), calcitonin (CT), and gastrin releasing peptide (GRP). RESULTS--Regions of non-metaplastic epithelium contained numerous PGP and serotonin immunoreactive cells. Sub-populations of these cells displayed CAB, CGRP, CT, and GRP immunoreactivity. Metaplastic epithelium contained only a few weakly stained PGP, serotonin, CAB, GRP, CT and CGRP immunoreactive cells in six cases. Metaplastic epithelium was characterised by a high number of CAB-containing cells in six cases and in these biopsies prominent PGP-containing nerve bundles were seen in the subepithelial layer beneath the metaplastic epithelium. CONCLUSIONS--The distribution patterns of neuroendocrine cells and neuronal elements vary between areas of normal and metaplastic epithelium and within areas of metaplastic epithelium. Neuronal hyperplasia was associated with an increase in the number of CAB-containing cells within the metaplastic epithelium. Images PMID:7541167

Primary vs. Secondary Antibody Deficiency: Clinical Features and Infection Outcomes of Immunoglobulin Replacement

PubMed Central

Duraisingham, Sai S.; Buckland, Matthew; Dempster, John; Lorenzo, Lorena; Grigoriadou, Sofia; Longhurst, Hilary J.

2014-01-01

Secondary antibody deficiency can occur as a result of haematological malignancies or certain medications, but not much is known about the clinical and immunological features of this group of patients as a whole. Here we describe a cohort of 167 patients with primary or secondary antibody deficiencies on immunoglobulin (Ig)-replacement treatment. The demographics, causes of immunodeficiency, diagnostic delay, clinical and laboratory features, and infection frequency were analysed retrospectively. Chemotherapy for B cell lymphoma and the use of Rituximab, corticosteroids or immunosuppressive medications were the most common causes of secondary antibody deficiency in this cohort. There was no difference in diagnostic delay or bronchiectasis between primary and secondary antibody deficiency patients, and both groups experienced disorders associated with immune dysregulation. Secondary antibody deficiency patients had similar baseline levels of serum IgG, but higher IgM and IgA, and a higher frequency of switched memory B cells than primary antibody deficiency patients. Serious and non-serious infections before and after Ig-replacement were also compared in both groups. Although secondary antibody deficiency patients had more serious infections before initiation of Ig-replacement, treatment resulted in a significant reduction of serious and non-serious infections in both primary and secondary antibody deficiency patients. Patients with secondary antibody deficiency experience similar delays in diagnosis as primary antibody deficiency patients and can also benefit from immunoglobulin-replacement treatment. PMID:24971644

Cystic fibrosis: a clinical view.

PubMed

Castellani, Carlo; Assael, Baroukh M

2017-01-01

Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude. Classical treatment includes pancreatic enzyme replacement, respiratory physiotherapy, mucolitics, and aggressive antibiotic therapy. A significant proportion of patients with severe symptoms still requires lung or, less frequently, liver transplantation. The great number of mutations and their diverse effects on the CFTR protein account only partially for CF clinical variability, and modifier genes have a role in modulating the clinical expression of the disease. Despite the increasing understanding of CFTR functioning, several aspects of CF need still to be clarified, e.g., the worse outcome in females, the risk of malignancies, the pathophysiology, and best treatment of comorbidities, such as CF-related diabetes or CF-related bone disorder. Research is focusing on new drugs restoring CFTR function, some already available and with good clinical impact, others showing promising preliminary results that need to be confirmed in phase III clinical trials.

Interaction between Pseudomonas aeruginosa and host defenses in cystic fibrosis.

PubMed

Marshall, B C; Carroll, K C

1991-03-01

The major causes of morbidity and mortality in cystic fibrosis are chronic pulmonary obstruction and infection. Mucoid Pseudomonas aeruginosa is the primary pathogen in up to 90% of these patients. Once Pseudomonas organisms colonize the airways, they are virtually never eradicated. No defect in systemic host defense has been elucidated, however, several mechanisms contribute to the breakdown in host defenses that allow persistence of this organism in the endobronchial space. These mechanisms involve both bacterial adaptation to an unfavorable host environment and impaired host response. P aeruginosa adapts to the host by expressing excessive mucoid exopolysaccharide and a less virulent form of lipopolysaccharide. These features make it less likely to cause systemic infection, yet still enable it to resist local host defenses. Mucociliary clearance becomes impaired due to abnormal viscoelastic properties of sputum, squamous metaplasia of the respiratory epithelium, and bronchiectasis. Despite a brisk antibody response to a variety of Pseudomonas antigens, several defects in antibody-mediated opsonophagocytosis have been identified. These include (1) development of antibody isotypes that are suboptimal at promoting phagocytosis, (2) formation of immune complexes that inhibit phagocytosis, and (3) proteolytic fragmentation of immunoglobulins in the endobronchial space. Complement-mediated opsonophagocytosis is also compromised by proteolytic cleavage of complement receptors from the cell surface of neutrophils and complement opsonins from the surface of Pseudomonas. The resultant chronic inflammation and infection lead to eventual obliteration of the airways.

Respiratory infection rates differ between geographically distant paediatric cystic fibrosis cohorts

PubMed Central

Hart, Emily; Turkovic, Lidija; Padros-Goossens, Marc; Stick, Stephen M.; Ranganathan, Sarath C.

2016-01-01

Respiratory infections are a major cause of pulmonary decline in children with cystic fibrosis (CF). We compared the prevalence of infection in early life at geographically distant CF treatment centres participating in the same surveillance programme in Australia. Lower airway microbiology, inflammation and structural lung disease at annual review were evaluated for 260 children 0–8 years old with CF at 1032 visits to CF treatment centres in Melbourne or Perth. Melbourne patients were more likely to be culture-positive for common respiratory pathogens at all age groups (odds ratio (OR) 1.85, 95% CI 1.33–2.58). Subjects <2 years old in Melbourne were also more likely to have neutrophil elastase present (OR 3.11, 95% CI 1.62–5.95). Bronchiectasis (OR 2.02, 95% CI 1.21–3.38) and air trapping (OR 2.53, 95% CI 1.42–4.51) in subjects 2–5 years old was more common in Melbourne subjects. The severity of structural lung disease was also worse in Melbourne patients >5 years old. Patients at both centres had a similar rate of hospitalisations and prescribed antibiotics. No procedural differences were identified that could explain the disparity between pathogen prevalence. Geographical differences in early acquisition of infection may contribute to variability in outcomes between CF centres. PMID:27957481

Diaphragmatic Paralysis: A Critical Review of its Use as a Therapeutic Measure in Respiratory Disease

PubMed Central

Campbell, A. J.

1934-01-01

Diaphragmatic paralysis first suggested as a therapeutic measure in lung disease by Steurtz (1911), who did simple phrenicotomy. Felix (1922) showed in 25% of cases this was ineffective owing to the presence of an accessory phrenic, and suggested phrenic exairesis, i.e. complete evulsion of the phrenic nerve. Goetze (1922) suggested radical phrenicotomy, i.e. division of the phrenic and excision of the nerve to the subclavius. Effects of diaphragmatic paralysis.—The diaphragm rises to the full expiratory position (4-8 cm.). Paradoxical movement (Kienböch's phenomenon) on affected side. Muscle atrophies. Collapse of the lung produced, affecting base and apex also. Lung volume reduced by ⅙th to ⅓rd. Physical signs.—Indrawing of the epigastrium. Thoracic breathing. Litten's sign absent. Less resistance to abdominal palpation on affected side. Diminished resonance at border of sternum and at base. Deficient inspiratory murmur at base. Radiography.—Paradoxical movement. Bittorf's test. Indications.—(A) Pulmonary tuberculosis. I. As the sole therapeutic measure. (1) In cases where pneumothorax has failed. (2) For relief of symptoms such as: (a) hæmoptysis; (b) cough; (c) tachycardia (d) nausea and vomiting; (e) pain; (f) hiccup. II. Combined with pneumothorax. (a) For basal adhesions; (b) alternative to bilateral pneumothorax; (c) to lengthen interval between refills; (d) at conclusion of pneumothorax treatment. III. Combined with thoracoplasty. (B) Other diseases. Unresolved pneumonia, fibrosis of the lung, bronchiectasis, abscess of the lung, hydatid disease. PMID:19989972

Diaphragmatic Paralysis: A Critical Review of its Use as a Therapeutic Measure in Respiratory Disease: (Section of Medicine).

PubMed

Campbell, A J

1934-10-01

Diaphragmatic paralysis first suggested as a therapeutic measure in lung disease by Steurtz (1911), who did simple phrenicotomy. Felix (1922) showed in 25% of cases this was ineffective owing to the presence of an accessory phrenic, and suggested phrenic exairesis, i.e. complete evulsion of the phrenic nerve. Goetze (1922) suggested radical phrenicotomy, i.e. division of the phrenic and excision of the nerve to the subclavius.Effects of diaphragmatic paralysis.-The diaphragm rises to the full expiratory position (4-8 cm.). Paradoxical movement (Kienböch's phenomenon) on affected side. Muscle atrophies. Collapse of the lung produced, affecting base and apex also. Lung volume reduced by (1/6)th to (1/3)rd.Physical signs.-Indrawing of the epigastrium. Thoracic breathing. Litten's sign absent. Less resistance to abdominal palpation on affected side. Diminished resonance at border of sternum and at base. Deficient inspiratory murmur at base.Radiography.-Paradoxical movement. Bittorf's test.Indications.-(A) Pulmonary tuberculosis.I. As the sole therapeutic measure.(1) In cases where pneumothorax has failed.(2) For relief of symptoms such as: (a) haemoptysis; (b) cough; (c) tachycardia (d) nausea and vomiting; (e) pain; (f) hiccup.II. Combined with pneumothorax.(a) For basal adhesions; (b) alternative to bilateral pneumothorax; (c) to lengthen interval between refills; (d) at conclusion of pneumothorax treatment.III. Combined with thoracoplasty.(B) Other diseases.Unresolved pneumonia, fibrosis of the lung, bronchiectasis, abscess of the lung, hydatid disease.

CFTR gene mutations in isolated chronic obstructive pulmonary disease

DOE Office of Scientific and Technical Information (OSTI.GOV)

Pignatti, P.F.; Bombien, C.; Marigo, C.

1994-09-01

In order to identify a possible hereditary predisposition to the development of chronic obstructive pulmonary disease (COPD), we have looked for the presence of cystic fibrosis transmembrane regulator (CFTR) gene DNA sequence modifications in 28 unrelated patients with no signs of cystic fibrosis. The known mutations in Italian CF patients, as well as the most frequent worldwide CF mutations, were investigated. In addition, a denaturing gradient gel electrophoresis analysis of about half of the coding sequence of the gene in 56 chromosomes from the patients and in 102 chromosomes from control individuals affected by other pulmonary diseases and from normalmore » controls was performed. Nine different CFTR gene mutations and polymorphisms were found in seven patients, a highly significant increase over controls. Two of the patients were compound heterozygotes. Two frequent CF mutations were detected: deletion F508 and R117H; two rare CF mutations: R1066C and 3667ins4; and five CF sequence variants: R75Q (which was also described as a disease-causing mutation in male sterility cases due to the absence of the vasa deferentia), G576A, 2736 A{r_arrow}G, L997F, and 3271+18C{r_arrow}T. Seven (78%) of the mutations are localized in transmembrane domains. Six (86%) of the patients with defined mutations and polymorphisms had bronchiectasis. These results indicate that CFTR gene mutations and sequence alterations may be involved in the etiopathogenesis of some cases of COPD.« less

Pulmonary manifestations of ankylosing spondylitis.

PubMed

Kanathur, Naveen; Lee-Chiong, Teofilo

2010-09-01

Ankylosing spondylitis, a chronic multisystem inflammatory disorder, can present with articular and extra-articular features. It can affect the tracheobronchial tree and the lung parenchyma, and respiratory complications include chest wall restriction, apical fibrobullous disease with or without secondary pulmonary superinfection, spontaneous pneumothorax, and obstructive sleep apnea. Ankylosing spondylitis is a common cause of pulmonary apical fibrocystic disease; early involvement may be unilateral or asymmetrical, but most cases eventually consist of bilateral apical fibrobullous lesions, many of which are progressive with coalescence of the nodules, formation of cysts and cavities, fibrosis, and bronchiectasis. Mycobacterial or fungal superinfection of the upper lobe cysts and cavities occurs commonly. Aspergillus fumigatus is the most common pathogen isolated, followed by various species of mycobacteria. Prognosis of patients with fibrobullous apical lesions is mainly determined by the presence, extent, and severity of superinfection. Pulmonary function test results are nonspecific and generally parallel the severity of parenchymal involvement. A restrictive ventilatory impairment can develop in patients with ankylosing spondylitis because of either fusion of the costovertebral joints and ankylosis of the thoracic spine or anterior chest wall involvement. Chest radiographic findings may mirror the severity of clinical involvement. Pulmonary parenchymal disease is typically progressive, and cyst formation, cavitation, and fibrosis are seen in advanced cases. No treatment has been shown to alter the clinical course of apical fibrobullous disease. Although several antiinflammatory agents, such as infliximab, etanercept, and adalimumab, are being used to treat ankylosing spondylitis, their effects on pulmonary manifestations are unclear.

Allergic Bronchopulmonary Aspergillosis: A Perplexing Clinical Entity

PubMed Central

Panjabi, Chandramani

2016-01-01

In susceptible individuals, inhalation of Aspergillus spores can affect the respiratory tract in many ways. These spores get trapped in the viscid sputum of asthmatic subjects which triggers a cascade of inflammatory reactions that can result in Aspergillus-induced asthma, allergic bronchopulmonary aspergillosis (ABPA), and allergic Aspergillus sinusitis (AAS). An immunologically mediated disease, ABPA, occurs predominantly in patients with asthma and cystic fibrosis (CF). A set of criteria, which is still evolving, is required for diagnosis. Imaging plays a compelling role in the diagnosis and monitoring of the disease. Demonstration of central bronchiectasis with normal tapering bronchi is still considered pathognomonic in patients without CF. Elevated serum IgE levels and Aspergillus-specific IgE and/or IgG are also vital for the diagnosis. Mucoid impaction occurring in the paranasal sinuses results in AAS, which also requires a set of diagnostic criteria. Demonstration of fungal elements in sinus material is the hallmark of AAS. In spite of similar histopathologic features, co-existence of ABPA and AAS is still uncommon. Oral corticosteroids continue to be the mainstay of management of allergic aspergillosis. Antifungal agents play an adjunctive role in ABPA as they help reduce the fungal load. Saprophytic colonization in cavitary ABPA may lead to aspergilloma formation, which could increase the severity of the disease. The presence of ABPA, AAS, and aspergilloma in the same patient has also been documented. All patients with Aspergillus-sensitized asthma must be screened for ABPA, and AAS should always be looked for. PMID:27126721

Chronic Obstructive Pulmonary Disease: official diagnosis and treatment guidelines of the Czech Pneumological and Phthisiological Society; a novel phenotypic approach to COPD with patient-oriented care.

PubMed

Koblizek, Vladimir; Chlumsky, Jan; Zindr, Vladimir; Neumannova, Katerina; Zatloukal, Jakub; Zak, Jaroslav; Sedlak, Vratislav; Kocianova, Jana; Zatloukal, Jaromir; Hejduk, Karel; Pracharova, Sarka

2013-06-01

COPD is a global concern. Currently, several sets of guidelines, statements and strategies to managing COPD exist around the world. The Czech Pneumological and Phthisiological Society (CPPS) has commissioned an Expert group to draft recommended guidelines for the management of stable COPD. Subsequent revisions were further discussed at the National Consensus Conference (NCC). Reviewers' comments contributed to the establishment of the document's final version. The hallmark of the novel approach to COPD is the integrated evaluation of the patient's lung functions, symptoms, exacerbations and identifications of clinical phenotype(s). The CPPS defines 6 clinically relevant phenotypes: frequent exacerbator, COPD-asthma overlap, COPD-bronchiectasis overlap, emphysematic phenotype, bronchitic phenotype and pulmonary cachexia phenotype. Treatment recommendations can be divided into four steps. 1(st) step = Risk exposure elimination: reduction of smoking and environmental tobacco smoke (ETS), decrease of home and occupational exposure risks. 2(nd) step = Standard treatment: inhaled bronchodilators, regular physical activity, pulmonary rehabilitation, education, inhalation training, comorbidity treatment, vaccination. 3(rd) step = Phenotype-specific therapy: PDE4i, ICS+LABA, LVRS, BVR, AAT augmentation, physiotherapy, mucolytic, ABT. 4(th) step = Care for respiratory insufficiency and terminal COPD: LTOT, lung transplantation, high intensity-NIV and palliative care. Optimal treatment of COPD patients requires an individualised, multidisciplinary approach to the patient's symptoms, clinical phenotypes, needs and wishes. The new Czech COPD guideline reflects and covers these requirements.

Australian epidemic strain pseudomonas (AES-1) declines further in a cohort segregated cystic fibrosis clinic.

PubMed

Griffiths, Amanda L; Wurzel, Danielle F; Robinson, Phil J; Carzino, Rosemary; Massie, John

2012-01-01

To evaluate changes in prevalence of an epidemic strain of Pseudomonas aeruginosa (AES-1, Australian epidemic strain, type 1) in a paediatric cystic fibrosis (CF) centre practising cohort segregation, to describe the patients' clinical characteristics at acquisition and observe mortality rates. Cohort segregation was introduced in our paediatric CF clinic January 2000. The prevalence of AES-1 was analysed in 1999, 2002 and 2007. Age at acquisition, lung function, presence of bronchiectasis, hospitalisations, prior P. aeruginosa infection and mortality rates were collected. AES-1 infection was determined by pulse-field-gel-electrophoresis (PFGE) on airway specimen cultures taken three monthly. The prevalence of AES-1 declined from 21% in 1999 to 14% in 2002 (risk difference 7% (95% CI 1,13) p=0.0256) and to 6% in 2007 (risk difference 8% (95% CI 3,13) p=0.0018). New acquisitions after the introduction of cohort segregation were uncommon (10 by 2002 and another 7 by 2007) with a declining incidence of 3.3 cases/year (1999 to 2002) compared to 1.4 cases/year (2002 to 2007). Twenty-two of 32 (69%) deaths between 1999 and 2007 occurred in patients infected with AES-1. Cohort segregation has been associated with reductions in the prevalence of AES-1 in our CF clinic. Mortality was higher in patients infected with AES-1 than other organisms. Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Utility of serum Aspergillus-galactomannan antigen to evaluate the risk of severe acute exacerbation in chronic obstructive pulmonary disease

PubMed Central

Yoshimura, Katsuhiro; Inoue, Yusuke; Nishimoto, Koji; Karayama, Masato; Furuhashi, Kazuki; Enomoto, Noriyuki; Nakamura, Yutaro; Inui, Naoki; Yokomura, Koushi; Imokawa, Shiro; Suda, Takafumi

2018-01-01

Background Recent studies have shown that the microbiome, namely Aspergillus species, play a previously unrecognized role in both stable and exacerbated chronic obstructive pulmonary disease (COPD). Galactomannan is a major component of the Aspergillus cell wall that has been widely used as a diagnostic marker. Objectives To explore whether serum levels of Aspergillus-galactomannan antigen could be used to evaluate the risk of severe acute exacerbation of COPD (AE-COPD). Methods We measured the Aspergillus-galactomannan antigen levels of 191 patients with stable COPD, and examined its clinical relevance including AE-COPD. Results There were 77 (40.3%) patients who were positive for serum Aspergillus-galactomannan antigen (≥0.5). High Aspergillus-galactomannan antigen level (≥0.7) was associated with older age and presence of bronchiectasis and cysts on computed tomography images. Compared to patients with low Aspergillus-galactomannan antigen level (<0.7), patients with high Aspergillus-galactomannan antigen level had significantly higher incidence of severe AE-COPD (P = 0.0039, Gray’s test) and respiratory-related mortality (P = 0.0176, log-rank test). Multivariate analysis showed that high Aspergillus-galactomannan antigen level was independently associated with severe AE-COPD (hazard ratio, 2.162; 95% confidence interval, 1.267−3.692; P = 0.005). Conclusion Serum Aspergillus-galactomannan antigen was detected in patients with COPD, and elevated serum Aspergillus-galactomannan antigen was associated with severe AE-COPD. PMID:29870550

Alpha-1-Antitrypsin Deficiency in Serbian Adults with Lung Diseases

PubMed Central

Stankovic, Marija; Divac-Rankov, Aleksandra; Petrovic-Stanojevic, Natasa; Mitic-Milikic, Marija; Nagorni-Obradovic, Ljudmila; Radojkovic, Dragica

2012-01-01

Aim: Alpha-1-antitrypsin (A1AT) is the main inhibitor of neutrophil elastase, and severe alpha-1-antitrypsin deficiency (A1ATD) is a genetic risk factor for early-onset emphysema. Despite the relatively high prevalence of A1ATD, this condition is frequently underdiagnosed. Our aim was to determine the distribution of the A1ATD phenotypes/alleles in patients with lung diseases as well as in the Serbian population. Methods: The study included the adults with chronic obstructive pulmonary disease (COPD) (n=348), asthma (n=71), and bronchiectasis (n=35); the control was 1435 healthy blood donors. The A1ATD variants were identified by isoelectric focusing or polymerase chain reaction-mediated site-directed mutagenesis. Results: PiMZ heterozygotes, PiZZ homozygotes, and Z allele carriers are associated with significantly higher risk of developing COPD than healthy individuals (odds ratios 3.43, 42.42, and 5.49 respectively). The calculated prevalence of PiZZ, PiMZ, and PiSZ was higher in patients with COPD (1:202, 1:8, and 1:1243) than in the Serbian population (1:5519, 1:38, and 1:5519). Conclusion: The high prevalence of A1ATD phenotypes/allele in our population has confirmed the necessity of screening for A1ATD in patients with COPD. On the other hand, on the basis of the estimated number of those with A1ATD among the COPD patients, it is possible to assess the diagnostic efficiency of A1ATD in the Serbian population. PMID:22971141

Autoinflammatory disease in the lung.

PubMed

Scambler, Thomas; Holbrook, Jonathan; Savic, Sinisa; McDermott, Michael F; Peckham, Daniel

2018-04-19

Ascertaining the dominant cell type driving an immunological disease is essential to understanding the causal pathology and, therefore, selecting or developing an effective treatment. Classifying immunological diseases in this way has led to successful treatment regimens for many monogenic diseases; however, when the dominant cell type is unclear and there is no obvious causal genetic mutation, then identifying the correct disease classification and appropriate therapy can be challenging. In this review we focus on pulmonary immunological diseases where an innate immune signature has been identified as a predominant aspect of the immunopathology. We describe the molecular pathology of 'autoinflammatory diseases of the lung' and propose that small molecule and biological therapies, including recombinant interleukin-1 receptor antagonist, that target key innate immune pathways, are likely be beneficial in the control of pulmonary and systemic inflammation in these conditions. In addition, the successful use of macrolide antibiotics to treat lung infections in these conditions further confirms that the innate immune system is the key conductor of inflammation in these pulmonary diseases, as there is a strong body of evidence that macrolides are able to modulate the NLRP3 inflammasome and interleukin-1β and interleukin-18 secretion, both of which are central players in the innate immune response. Throughout this review we highlight the published evidence of autoinflammatory disease in chronic obstructive pulmonary disease, bronchiectasis, cystic fibrosis and rheumatoid lung disease and suggest that the fundamental pathology of these diseases places them towards the autoinflammatory pole of the immunological disease continuum. © 2018 John Wiley & Sons Ltd.

Utility of the inspiratory phase in high-resolution computed tomography evaluations of pediatric patients with bronchiolitis obliterans after allogeneic bone marrow transplant: reducing patient radiation exposure

PubMed Central

Togni Filho, Paulo Henrique; Casagrande, João Luiz Marin; Lederman, Henrique Manoel

2017-01-01

Objective To evaluate the utility of the inspiratory phase in high-resolution computed tomography (HRCT) of the chest for the diagnosis of post-bone marrow transplantation bronchiolitis obliterans. Materials and Methods This was a retrospective, observational, cross-sectional study. We selected patients of either gender who underwent bone marrow transplantation and chest HRCT between March 1, 2002 and December 12, 2014. Ages ranged from 3 months to 20.7 years. We included all examinations in which the HRCT was performed appropriately. The examinations were read by two radiologists, one with extensive experience in pediatric radiology and another in the third year of residency, who determined the presence or absence of the following imaging features: air trapping, bronchiectasis, alveolar opacities, nodules, and atelectasis. Results A total of 222 examinations were evaluated (mean, 5.4 ± 4.5 examinations per patient). The expiratory phase findings were comparable to those obtained in the inspiratory phase, except in one patient, in whom a small uncharacteristic nodule was identified only in the inspiratory phase. Air trapping was identified in a larger number of scans in the expiratory phase than in the inspiratory phase, as was atelectasis, although the difference was statistically significant only for air trapping. Conclusion In children being evaluated for post-bone marrow transplantation bronchiolitis obliterans, the inspiratory phase can be excluded from the chest HRCT protocol, thus reducing by half the radiation exposure in this population. PMID:28428651

Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry

PubMed Central

Guffey, Danielle; Minard, Charles G.; Orange, Jordan S.

2017-01-01

Introduction Patients with primary immunodeficiency (PID) often report fatigue, yet this symptom has not been studied in PID. Fatigue affects 6–7.5% of healthy adults. The goal of this study is to estimate the prevalence of fatigue in patients with PID and investigate its associated factors. Methods We analyzed 2537 PID patients registered in USIDNET to determine responses to the field “fatigue” in the core registry form. Demographics, immune phenotypes, and comorbid conditions were compared between fatigued and non-fatigued patients to identify relevant associations and potential drivers. A focused analysis was performed for patients with predominantly antibody deficiency disorders (PADs). Results Fatigue was reported in 25.9%(95% CI 23.7–28.3) of PAD patients, compared to 6.4% (95% CI 4.9–8.2) of non-PAD. Patients with common variable immunodeficiency (CVID) had the highest prevalence of fatigue (p < 0.001) among all PID diagnoses. Other factors that were associated with a higher rate of fatigue among PAD patients included female sex, higher BMI, depression, bronchiectasis, and autoimmunity. Additionally, fatigued PAD patients had lower absolute lymphocyte, CD3, CD4, and CD8 counts compared to non-fatigued patients. Conclusion Our findings suggest that fatigue is overrepresented in PAD patients. Prospective studies to estimate prevalence, risk factors, and fatigue etiology in PID are warranted, so therapeutic interventions can be considered. PMID:28124237

Causes of recurrent pneumonia in children in a general hospital.

PubMed

Hoving, M F Paulien; Brand, Paul L P

2013-03-01

Because the few previous studies on underlying causes of recurrent pneumonia in children have come from tertiary care referral centres where selection bias may be important, the aim of this study was to examine underlying causes of recurrent pneumonia in children in a general hospital. We performed a retrospective chart review in a general hospital of 62 children with recurrent pneumonia over a 7.5 years period. In 19 patients (30.6%), no cause was identified, commonly because favourable natural history obviated the need for a full and invasive diagnostic work-up. Other underlying causes included recurrent aspiration in 16 patients (25.7%), lung disease (airway stenosis, bronchiectasis, middle lobe syndrome or tracheooesophageal fistula) in 10 patients (16.1%) and immune deficiency in 10 patients (16.1%). In contrast to previous studies, asthma was never diagnosed as an underlying cause, but diagnostic confusion between asthma (or recurrent upper respiratory tract infections) and recurrent pneumonia was common. The cause of recurrent pneumonia in children remains elusive in almost a third of patients, partly because the favourable natural history consistent with immune system maturation eliminates the need for further diagnostic procedures. Asthma is more likely a differential diagnostic consideration than an underlying cause of recurrent pneumonia in children. A standardised diagnostic guideline is needed to improve knowledge on causes of recurrent pneumonia in children. © 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

Characterization of primary cilia in human airway smooth muscle cells.

PubMed

Wu, Jun; Du, Hui; Wang, Xiangling; Mei, Changlin; Sieck, Gary C; Qian, Qi

2009-08-01

Considerable evidence indicates a key role for primary cilia of mammalian cells in mechanochemical sensing. Dysfunctions of primary cilia have been linked to the pathogenesis of several human diseases. However, cilia-related research has been limited to a few cell and tissue types; to our knowledge, no literature exists on primary cilia in airway smooth muscle (ASM). The aim of this study was to characterize primary cilia in human ASM. Primary cilia of human bronchial smooth muscle cells (HBSMCs) were examined using immunofluorescence confocal microscopy, and scanning and transmission electron microscopy. HBSMC migration and injury repair were examined by scratch-wound and epidermal growth factor (EGF)-induced migration assays. Cross-sectional images of normal human bronchi revealed that primary cilia of HBSMCs within each ASM bundle aggregated at the same horizontal level, forming a "cilium layer." Individual cilia of HBSMCs projected into extracellular matrix and exhibited varying degrees of deflection. Mechanochemical sensing molecules, polycystins, and alpha2-, alpha5-, and beta1-integrins were enriched in cilia, as was EGF receptor, known to activate jointly with integrins during cell migration. Migration assays demonstrated a ciliary contribution to HBSMC migration and wound repair. The primary cilia of ASM cells exert a role in sensing and transducing extracellular mechanochemical signals and in ASM injury repair. Defects in ASM ciliary function could potentially affect airway wall maintenance and/or remodeling, possibly relating to the genesis of bronchiectasis in autosomal dominant polycystic kidney disease, a disease of ciliopathy.

Endovascular treatment of ectopic bronchial artery aneurysm with brachiocephalic artery stent placement and coil embolization

PubMed Central

Di, Xiao; Ji, Dong-Hua; Chen, Yu; Liu, Chang-Wei; Liu, Bao; Yang, Juan

2016-01-01

Abstract Background: Bronchial artery aneurysm (BAA) is an uncommon but potentially life-threatening disease, and multiple BAAs are even rarer. Clinically, the tortuous and short neck of a BAA may present significant challenges for invasive intervention. Methods: This report describes the detailed process of diagnosis and treatment and includes a literature review of the etiology, clinical presentation, and therapeutic management of BAA. Results: A rare case of multiple BAAs, with one having an inflow artery arising from the brachiocephalic trunk, was referred to our hospital. The patient was successfully treated with coil embolization and brachiocephalic artery stent placement. In addition, we conducted a literature review involving 63 cases of BAA. BAA was most commonly associated with bronchiectasis and was located predominantly in the mediastinum. There was no significant difference between the diameters of the ruptured aneurysms and those of the nonruptured aneurysms (P = 0.115). Transcatheter arterial embolization was the most commonly adopted technique to treat BAA, while thoracic aortic endovascular repair was selected if the neck between the aneurysm and the aorta was short. Subgroup analysis suggested that patients with > 1 BAA were significantly more likely to be female than male (χ2 test, P = 0.034). Conclusion: Transcatheter coil embolization combined with stent placement could be a reasonable treatment option for BAAs with a tortuous and short neck. According to our literature review, patients with multiple BAAs display distinctive clinical characteristics compared with patients with a single BAA. PMID:27583854

Endobronchial Valves for Endoscopic Lung Volume Reduction: Best Practice Recommendations from Expert Panel on Endoscopic Lung Volume Reduction.

PubMed

Slebos, Dirk-Jan; Shah, Pallav L; Herth, Felix J F; Valipour, Arschang

2017-01-01

Endoscopic lung volume reduction (ELVR) is being adopted as a treatment option for carefully selected patients suffering from severe emphysema. ELVR with the one-way endobronchial Zephyr valves (EBV) has been demonstrated to improve pulmonary function, exercise capacity, and quality of life in patients with both heterogeneous and homogenous emphysema without collateral ventilation. In this "expert best practices" review, we will highlight the practical aspects of this therapy. Key selection criteria for ELVR are hyperinflation with a residual volume >175% of predicted, forced expiratory volume <50% of predicted, and a 6-min walking distance >100 m. Patients with repeated infectious complications, severe bronchiectasis, and those with unstable cardiovascular comorbidities should be excluded from EBV treatment. The procedure may be performed with either conscious sedation or general anesthesia and positive pressure mechanical ventilation using a flexible endotracheal tube or a rigid bronchoscope. Chartis and EBV placement should be performed in 1 procedure when possible. The sequence of valve placement should be orchestrated to avoid obstruction and delivery of subsequent valves. If atelectasis has not occurred by 1 month after procedure, evaluate valve position on CT and consider replacing the valves that are not optimally positioned. Pneumothorax is a common complication and typically occurs in the first 2 days following treatment. A management algorithm for pneumothorax has been previously published. Long-term sequelae from EBV therapy do occur but are easily manageable. © 2016 The Author(s) Published by S. Karger AG, Basel.

Treatment of complex airway lesions after lung transplantation with self-expandable nitinol stents: early experience.

PubMed

Anile, M; Venuta, F; Diso, D; Liparulo, V; Ricella, C; De Giacomo, T; Pugliese, F; Rolla, M; Quattrucci, S; Pecoraro, Y; Rendina, E A; Coloni, G F

2010-05-01

Airway complications (AC) are considered a serious cause of morbidity after lung transplantation (LT). Mechanical dilatation, laser vaporization, and silicone stent placement usually solve it. However, the use of self-expandable metallic stents (SENS) may be indicated in selected cases. Ten lung transplant recipients with AC were treated with SENS. Six patients underwent LT for cystic fibrosis, 2 for idiopathic pulmonary fibrosis, 1 for bronchiectasis, and 1 for emphysema. All patients received at least 1 treatment attempt with dilatation and silicone stent placement. The indications for SENS placement were the presence of a tortuous airway axis with stenosis and malacia of the right main bronchus in 5 patients; a long stenosis of the main and intermediate right bronchus involving the upper lobe orifice in 3 patients; or malacia that could not be stabilized with silicone stents in 3 cases. In 1 patient the procedure was bilateral. Functional improvement was immediate with a mean forced expiratory volume at 1 second (FEV(1)) gain of 35%. No stent dislocation was observed. Symptoms did not occur again in 5 patients with previous recurrent episodes of pneumonia. One stenosis, which was due to the ingrowth of granulation tissue occurred at 6 months after the procedure, was successfully treated with mechanical dilatation and laser vaporization. The deployment of SENS in a selected group of patients with AC after LT was easy, safe, and effective. Copyright (c) 2010 Elsevier Inc. All rights reserved.

[1990-1996: the experience of the La Fe Lung Transplant Group (Valencia)].

PubMed

Borro Maté, J M; Morales Marín, P; Lozano Ruiz, C; Tarrazona Hervás, V; Galán Gil, G; Calvo Medina, V; Morant Guillén, P; Ramos Briones, F; Vicente Guillén, R; Paris Romeu, F

1997-10-01

Objective to review the experience of the lung transplantation unit at Hospital La Fe (Valencia). Between February 1990 and March 1996 we performed 40 lung transplants. The following causes were most common: cystic fibrosis (9 cases), emphysema (8), pulmonary fibrosis (8) and bronchiectasis (7). Types of intervention were 27 double lung transplants (25 sequential and 9 blocked), 9 single lung transplants, and 4 heart-lung transplants. We then reviewed the 36 single and double lung transplants. The main exclusion criteria were age over 65 years, malignant disease, kidney or liver disease, severe or non reversible central nervous system disease, and drug addiction. Prior surgery, mechanical ventilation and the presence of Aspergillus were considered lower-order contraindications. Mean patient age was 37.7 years (14-59). Six patients were colonized by Aspergillus before transplantation. Five had undergone earlier surgery and two were mechanically ventilated before the transplant. The most common complication was respiratory infection, which was present in 6 of the 7 patients who died. Other complications in order of frequency were dehiscence and/or bronchial stenosis, corticoid myopathy and postoperative bleeding. The actuarial survival rate of single and double lung transplants was 67.85 after 3 years, and 87.5% in patients with cystic fibrosis. Lung transplantation is a well-established procedure that is gradually being extended to treat more conditions. The main obstacle is the scarcity of donors. The main challenge at present is bronchiolitis obliterans.

Prevention of infection in children and adolescents with primary immunodeficiency disorders.

PubMed

Papadopoulou-Alataki, Efimia; Hassan, Amel; Davies, E Graham

2012-12-01

Primary Immunodeficiency diseases (PIDs) are a heterogenous group of inherited disorders that may involve one or multiple components of the immune system. PIDs are uncommon, chronic and severe disorders, in which patients cannot mount a sufficiently protective immune response, leading to an increased susceptibility to infections. This review addresses the current practices for the prevention of infection in children and adolescents with PIDs, particular covering immunisations and antimicrobial prophylaxis. Over recent years, there have been major advances in molecular and cellular understanding in the field of PIDs. Many different disorders are recognised with variable spectra of infection susceptibility depending on the particular aspects of the immune response that are affected. Immunoglobulin prophylaxis is the mainstay of treatment for PIDs and provides passive protection. Prophylactic antimicrobials are efficacious in children and adolescents with predominant defects in primary T cell immunodeficiency diseases and phagocytic disorders, and also with predominant defects in antibody production. Prophylactic antibiotics are suggested for patients with antibody deficiency diseases if recurrent infections exceed three per year, if severe infections occur despite adequate immunoglobulin replacement and in hypogammaglobulinaemic patients who have bronchiectasis. Certain immunisations are effective in antibody deficiencies, T cell deficiencies, complement deficiencies and phagocytic disorders. There are remarkably few published data relating to clinical management aimed at preventing infectious complications in children and adolescents with PIDs. The cornerstones of the prevention of infection in most PID patients are: antimicrobial prophylaxis, appropriate vaccination, immunoglobulin replacement, for the more severe cases, and regular ongoing follow-up.

Chronic health effects of sulphur mustard exposure with special reference to Iranian veterans

PubMed Central

Balali-Mood, M; Mousavi, SH; Balali-Mood, B

2008-01-01

The widespread use of sulphur mustard (SM) as an incapacitating chemical warfare agent in the past century has proved its long-lasting toxic effects. It may also be used as a chemical terrorist agent. Therefore, all health professionals should have sufficient knowledge and be prepared for any such chemical attack. SM exerts direct toxic effects on the eyes, skin, and respiratory tissue, with subsequent systemic action on the nervous, immunological, haematological, digestive, and reproductive systems. SM is an alkylating agent that affects DNA synthesis, and, thus, delayed complications have been seen since the First World War. Cases of malignancies in the target organs, particularly in haematopoietic, respiratory, and digestive systems, have been reported. Important delayed respiratory complications include chronic bronchitis, bronchiectasis, frequent bronchopneumonia, and pulmonary fibrosis, all of which tend to deteriorate with time. Severe dry skin, delayed keratitis, and reduction of natural killer cells with subsequent increased risk of infections and malignancies are also among the most distressing long-term consequences of SM intoxication. However, despite a lot of research over the past decades on Iranian veterans, there are still major gaps in the SM literature. Immunological and neurological dysfunction, as well as the relationship between SM exposure and mutagenicity, carcinogenicity, and teratogenicity are important fields that require further studies, particularly on Iranian veterans with chronic health effects of SM poisoning. There is also a paucity of information on the medical management of acute and delayed toxic effects of SM poisoning—a subject that greatly challenges health care specialists. PMID:22460216

Iatrogenic osteoporosis, bilateral HIP osteonecrosis, and secondary adrenal suppression in an HIV-infected man receiving inhaled corticosteroids and ritonavir-boosted highly active antiretroviral therapy.

PubMed

Kaviani, Nargess; Bukberg, Phillip; Manessis, Anastasios; Yen, Vincent; Young, Iven

2011-01-01

To report the first case of severe osteoporosis associated with a vertebral pathologic fracture and osteonecrosis of femoral heads in an HIV-infected man receiving inhaled corticosteroids and ritonavir-boosted antiretroviral therapy. We describe an HIV-infected man with severe osteoporosis, bilateral hip osteonecrosis, and secondary adrenal suppression, including detailed clinical, laboratory, and radiographic data, and review the related literature. A 60-year-old man with a 15-year history of HIV infection and a medical history of long-standing bronchiectasis treated with inhaled corticosteroids and hypogonadism treated with testosterone was referred to the endocrinology clinic after experiencing an osteoporotic vertebral fracture. He was taking ritonavir-boosted antiretroviral therapy. Osteonecrosis of both hips was also diagnosed, which required total hip replacement therapy. Laboratory evaluation revealed adrenal insufficiency due to increased effect of exogenous inhaled steroids and no other secondary causes of osteoporosis. A bone densitometry study showed osteoporosis of both hips and the lumbar spine. He was treated with intravenous pamidronate. During treatment, he developed bilateral femoral fractures after minor trauma. Given the potential for increased serum levels of inhaled corticosteroids in patients taking ritonavir-boosted highly active antiretroviral therapy, attention must be paid to the risk of bone loss in HIV-infected patients taking inhaled corticosteroids. Prescribing calcium and vitamin D supplementation and considering early osteoporosis screening are reasonable measures for this patient population. Interaction between inhaled corticosteroids and ritonavir may increase risk of hypothalamus-pituitary-adrenal axis suppression.

Loss-of-Function Mutations in a Human Gene Related to Chlamydomonas reinhardtii Dynein IC78 Result in Primary Ciliary Dyskinesia

PubMed Central

Pennarun, Gaëlle; Escudier, Estelle; Chapelin, Catherine; Bridoux, Anne-Marie; Cacheux, Valère; Roger, Gilles; Clément, Annick; Goossens, Michel; Amselem, Serge; Duriez, Bénédicte

1999-01-01

Summary Primary ciliary dyskinesia (PCD) is a group of heterogeneous disorders of unknown origin, usually inherited as an autosomal recessive trait. Its phenotype is characterized by axonemal abnormalities of respiratory cilia and sperm tails leading to bronchiectasis and sinusitis, which are sometimes associated with situs inversus (Kartagener syndrome) and male sterility. The main ciliary defect in PCD is an absence of dynein arms. We have isolated the first gene involved in PCD, using a candidate-gene approach developed on the basis of documented abnormalities of immotile strains of Chlamydomonas reinhardtii, which carry axonemal ultrastructural defects reminiscent of PCD. Taking advantage of the evolutionary conservation of genes encoding axonemal proteins, we have isolated a human sequence (DNAI1) related to IC78, a C. reinhardtii gene encoding a dynein intermediate chain in which mutations are associated with the absence of outer dynein arms. DNAI1 is highly expressed in trachea and testis and is composed of 20 exons located at 9p13-p21. Two loss-of-function mutations of DNAI1 have been identified in a patient with PCD characterized by immotile respiratory cilia lacking outer dynein arms. In addition, we excluded linkage between this gene and similar PCD phenotypes in five other affected families, providing a clear demonstration of locus heterogeneity. These data reveal the critical role of DNAI1 in the development of human axonemal structures and open up new means for identification of additional genes involved in related developmental defects. PMID:10577904

[Anesthesia in single and bilateral sequential lung transplantation. Lung Transplantation Group].

PubMed

Della Rocca, G; Coccia, C; Pugliese, F; Pompei, L; Ruberto, F; Costa, M G; Venuta, F; Rendina, E A; De Giacomo, T; Pietropaoli, P; Gasparetto, A

2000-04-01

Anesthesia for lung transplantation: intraoperative complications and long term results. 52 patients were scheduled for 16 single lung transplantations (SLT) (9 fibrosis and 7 emphysema) and 36 bilateral sequential lung transplantations (DLT) (4 bronchiectasis, 6 emphysema, 3 fibrosis, 22 cystic fibrosis and 1 pulmonary hypertension). Anesthesia was induced with propofol or midazolam, and fentanyl or alfentanil. As muscle relaxant vecuronium bromide was used. Anesthesia was maintained with isoflurane, fentanyl in boluses or sufentanil continuous infusion in O2 100%. Prostaglandin E1 (20-300 ng/kg/min), inhaled nitric oxide (10-40 ppm), dobutamine (5-15 mcg/kg/min), norepinephrine (0.05-3 mcg/kg/min) and ephedrine (5-10 mg per bolus) were used for hemodynamic management. In 2 patients inhaled areosolized prostacyclin were administered. Mean pulmonary arterial pressure (mPA) and pulmonary vascular resistance (PVRI) increased after pulmonary artery clamping during first lung (mPA: 3347 nel DLT, 3643 nel SLT; PVRI; 375488 nel DLT, 377420 nel SLT) and second lung implantation (mPA: 3746; PVRI: 263553) and decreased after reperfusion of the first (mPA: 4737 nel DLT, 4329 nel SLT; PVRI: 488263 nel DLT, 420233 nel SLT) and the second lung (mPA: 4629; PVRI: 553260). Only in 9 cases (7 DLT and 2 SLT) C-P bypass was used. With a strong drug support with pulmonary vasodilators, positive inotropic and systemic vasoconstrictor drugs, in most patients we transplanted C-P bypass can be avoided. Intraoperative deaths were not observed. Two years actuarial survival is 65% for DLT and 60% for SLT.

Primary ciliary dyskinesia. Recent advances in diagnostics, genetics, and characterization of clinical disease.

PubMed

Knowles, Michael R; Daniels, Leigh Anne; Davis, Stephanie D; Zariwala, Maimoona A; Leigh, Margaret W

2013-10-15

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous recessive disorder of motile cilia that leads to oto-sino-pulmonary diseases and organ laterality defects in approximately 50% of cases. The estimated incidence of PCD is approximately 1 per 15,000 births, but the prevalence of PCD is difficult to determine, primarily because of limitations in diagnostic methods that focus on testing ciliary ultrastructure and function. Diagnostic capabilities have recently benefitted from (1) documentation of low nasal nitric oxide production in PCD and (2) discovery of biallelic mutations in multiple PCD-causing genes. The use of these complementary diagnostic approaches shows that at least 30% of patients with PCD have normal ciliary ultrastructure. More accurate identification of patients with PCD has also allowed definition of a strong clinical phenotype, which includes neonatal respiratory distress in >80% of cases, daily nasal congestion and wet cough starting soon after birth, and early development of recurrent/chronic middle-ear and sinus disease. Recent studies, using advanced imaging and pulmonary physiologic assessments, clearly demonstrate early onset of lung disease in PCD, with abnormal air flow mechanics by age 6-8 years that is similar to cystic fibrosis, and age-dependent onset of bronchiectasis. The treatment of PCD is not standardized, and there are no validated PCD-specific therapies. Most patients with PCD receive suboptimal management, which should include airway clearance, regular surveillance of pulmonary function and respiratory microbiology, and use of antibiotics targeted to pathogens. The PCD Foundation is developing a network of clinical centers, which should improve diagnosis and management of PCD.

Prospective evaluation of respiratory exacerbations in children with cystic fibrosis from newborn screening to 5 years of age.

PubMed

Byrnes, Catherine Ann; Vidmar, Suzanna; Cheney, Joyce L; Carlin, John B; Armstrong, David S; Cooper, Peter J; Grimwood, Keith; Moodie, Marj; Robertson, Colin F; Rosenfeld, Margaret; Tiddens, Harm A; Wainwright, Claire E

2013-07-01

Newborn screening allows novel treatments for cystic fibrosis (CF) to be trialled in early childhood before irreversible lung injury occurs. As respiratory exacerbations are a potential trial outcome variable, we determined their rate, duration and clinical features in preschool children with CF; and whether they were associated with growth, lung structure and function at age 5 years. Respiratory exacerbations were recorded prospectively in Australasian CF Bronchoalveolar Lavage trial subjects from enrolment after newborn screening to age 5 years, when all participants underwent clinical assessment, chest CT scans and spirometry. 168 children (88 boys) experienced 2080 exacerbations, at an average rate of 3.66 exacerbations per person-year; 80.1% were community managed and 19.9% required hospital admission. There was an average increase in exacerbation rate of 9% (95% CI 4% to 14%; p<0.001) per year of age. Exacerbation rate differed by site (p<0.001) and was 26% lower (95% CI 12% to 38%) in children receiving 12 months of prophylactic antibiotics. The rate of exacerbations in the first 2 years was associated with reduced forced expiratory volume in 1 s z scores. Ever having a hospital-managed exacerbation was associated with bronchiectasis (OR 2.67, 95% CI 1.13 to 6.31) in chest CT scans, and lower weight z scores at 5 years of age (coefficient -0.39, 95% CI -0.74 to -0.05). Respiratory exacerbations in young children are markers for progressive CF lung disease and are potential trial outcome measures for novel treatments in this age group.

Chronic obstructive pulmonary disease: knowing what we mean, meaning what we say.

PubMed

Joshi, J M

2008-01-01

Chronic obstructive pulmonary disease (COPD) is defined in several different ways using different criteria based on symptoms, physiological impairment and pathological abnormalities. While some use COPD to mean smoking related chronic airway disease, others include all disorders causing chronic airway obstruction. When COPD is used as a broad descriptive term, specific disorders that cause chronic airway obstruction remain under-diagnosed and the prevalence estimates vary considerably. The lack of agreement over the precise terminology and classification of COPD has resulted in widespread confusion. Terminology includes definition, diagnostic criteria, and a system for staging severity. Recently, COPD is defined more clearly and diagnosed using precise criteria that include tobacco smoking greater than 10 pack years, symptoms and airway obstruction on spirometry. A multi-dimensional severity grading system, the BODE (body mass index, obstruction, dyspnoea, and exercise tolerance) index has been designed to assess the respiratory and systemic expressions of COPD. This review proposes that the broad group of chronic disorders of the airways (with or without airway obstruction) be called chronic airway disease (CAD). The term COPD should be used exclusively for tobacco smoking related chronic airway disease. Chronic airway obstruction or obstructive lung disease may be used to define those conditions with airways obstruction caused by factors other than tobacco smoking. The aetiology may be appended to the label, for example, chronic airway obstruction/obstructive lung disease associated with bronchiectasis, chronic airway obstruction/obstructive lung disease associated with obliterative bronchiolitis or chronic airway obstruction/obstructive lung disease due to biomass fuel/occupational exposure.

Cystic fibrosis.

PubMed

Elborn, J Stuart

2016-11-19

Cystic fibrosis is a common life-limiting autosomal recessive genetic disorder, with highest prevalence in Europe, North America, and Australia. The disease is caused by mutation of a gene that encodes a chloride-conducting transmembrane channel called the cystic fibrosis transmembrane conductance regulator (CFTR), which regulates anion transport and mucociliary clearance in the airways. Functional failure of CFTR results in mucus retention and chronic infection and subsequently in local airway inflammation that is harmful to the lungs. CFTR dysfunction mainly affects epithelial cells, although there is evidence of a role in immune cells. Cystic fibrosis affects several body systems, and morbidity and mortality is mostly caused by bronchiectasis, small airways obstruction, and progressive respiratory impairment. Important comorbidities caused by epithelial cell dysfunction occur in the pancreas (malabsorption), liver (biliary cirrhosis), sweat glands (heat shock), and vas deferens (infertility). The development and delivery of drugs that improve the clearance of mucus from the lungs and treat the consequent infection, in combination with correction of pancreatic insufficiency and undernutrition by multidisciplinary teams, have resulted in remarkable improvements in quality of life and clinical outcomes in patients with cystic fibrosis, with median life expectancy now older than 40 years. Innovative and transformational therapies that target the basic defect in cystic fibrosis have recently been developed and are effective in improving lung function and reducing pulmonary exacerbations. Further small molecule and gene-based therapies are being developed to restore CFTR function; these therapies promise to be disease modifying and to improve the lives of people with cystic fibrosis. Copyright © 2016 Elsevier Ltd. All rights reserved.

Incidental Finding of Bronchopulmonary Sequestration in a 64-Year-Old Female.

PubMed

Tunsupon, Pichapong; Arshad, Ayesha; Patel, Sumit; Mador, M Jeffery

2017-01-01

Bronchopulmonary sequestration is a congenital abnormality of the primitive foregut. In adults, the typical age at presentation is 20-25 years. A 64-year-old female was referred for evaluation of an 8 × 6-cm right lower lobe cystic lesion. Her medical history was significant for recurrent right lower lobe pneumonia requiring multiple hospitalizations. Her physical examination was significant for crackles at the right lung base. Computed tomography (CT) of the chest with contrast showed cystic changes with thickened septation of the medial segment of the right lower lobe lacking distinct visceral pleura and with arterial supply from the anomalous branch of the thoracic aorta arising near the celiac trunk. Pulmonary angiography confirmed the diagnosis of intralobar pulmonary sequestration. The patient underwent celiac endovascular coil embolization of the anomalous artery to lessen the risk of hemorrhage prior to video-assisted thoracoscopic surgery (VATS) resection of the right lower lobe. She recovered well and was discharged home 1 week after VATS lobectomy. Follow-up CT of the chest 2 months later showed normal postsurgical changes related to right lower lobe lobectomy. The patient remained asymptomatic and resumed her daily activities. Pulmonary sequestration can present with recurrent pneumonia in late adulthood. Physicians must review any previous imaging studies of the chest to identify the structural abnormality and be cognizant of differential diagnoses such as infected cystic bronchiectasis, bronchogenic cyst, congenital diaphragmatic hernia, or cystic adenomatoid malformation that can occur in conjunction with bronchopulmonary sequestration. Pulmonary angiogram is the gold standard to confirm the diagnosis of bronchopulmonary sequestration. Surgical resection is the standard of care.

[Lung transplantation in Reunion Island, current situation from January 2005 till May 2015].

PubMed

Simon, C; Allou, N; Schlossmacher, P; Gendry, T; Delay, L; Gazaille, V

2017-04-01

Lung transplantation (LT) is a therapeutic option for patients with terminal respiratory failure and high risk of mortality in two years. Until now, this activity is not performed in Reunion Island. The candidate potential are thus directed to the metropolitan reference centres causing logistics and financials constraints. This work presents a current situation of the pulmonary transplantation in Reunion Island. This retrospective study includes patients from Reunion Island with respiratory insufficiency who have been transferred to metropolitan centres to apply to LT. The selection was made from January, 2005 till May, 2015. Twenty-nine patients included, aged from 14 to 64 years, were transferred to metropolitan France: 13 patients with cystic fibrosis, 13 patients with pulmonary fibrosis, 1 patients with bronchiectasis, 1 patient with chronic obstructive pulmonary disease (COPD) and 1 patient with pulmonary arterial hypertension. Fifteen patients underwent LT (4 live in Reunion Island, 5 live in metropolitan France and 6 are dead), 1 patient is alive on waiting list, 3 died on the waiting list, 7 patients were refused for transplantation and 3 patients are lost to follow-up. The number of patients transferred for LT increases over 10 years with a maximal incidence in 2013 of 7 repatriated patients including 3 transplanted patients. LT could be performed in half of our patients with possible come back and follow-up on Reunion Island. Indications follow the current trends except an under representation of COPD. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Longitudinal profiling of the lung microbiome in the AERIS study demonstrates repeatability of bacterial and eosinophilic COPD exacerbations

PubMed Central

Lambert, Christophe; Clarke, Stuart C; Kim, Viktoriya L; Magid-Slav, Michal; Miller, Bruce E; Patel, Ruchi; Sathe, Ganesh; Simola, Daniel F; Sung, Ruby; Tal-Singer, Ruth; Tuck, Andrew C; Van Horn, Stephanie; Weynants, Vincent; Williams, Nicholas P; Devaster, Jeanne-Marie; Wilkinson, Tom M A

2018-01-01

Background Alterations in the composition of the lung microbiome associated with adverse clinical outcomes, known as dysbiosis, have been implicated with disease severity and exacerbations in COPD. Objective To characterise longitudinal changes in the lung microbiome in the AERIS study (Acute Exacerbation and Respiratory InfectionS in COPD) and their relationship with associated COPD outcomes. Methods We surveyed 584 sputum samples from 101 patients with COPD to analyse the lung microbiome at both stable and exacerbation time points over 1 year using high-throughput sequencing of the 16S ribosomal RNA gene. We incorporated additional lung microbiology, blood markers and in-depth clinical assessments to classify COPD phenotypes. Results The stability of the lung microbiome over time was more likely to be decreased in exacerbations and within individuals with higher exacerbation frequencies. Analysis of exacerbation phenotypes using a Markov chain model revealed that bacterial and eosinophilic exacerbations were more likely to be repeated in subsequent exacerbations within a subject, whereas viral exacerbations were not more likely to be repeated. We also confirmed the association of bacterial genera, including Haemophilus and Moraxella, with disease severity, exacerbation events and bronchiectasis. Conclusions Subtypes of COPD have distinct bacterial compositions and stabilities over time. Some exacerbation subtypes have non-random probabilities of repeating those subtypes in the future. This study provides insights pertaining to the identification of bacterial targets in the lung and biomarkers to classify COPD subtypes and to determine appropriate treatments for the patient. Trial registration number Results, NCT01360398. PMID:29386298

Tracheomalacia is associated with lower FEV1 and Pseudomonas acquisition in children with CF

PubMed Central

Fischer, Anthony J.; Singh, Sachinkumar B.; Adam, Ryan J.; Stoltz, David A.; Baranano, Christopher F.; Kao, Simon; Weinberger, Miles M.; McCray, Paul B.; Starner, Timothy D.

2016-01-01

BACKGROUND Tracheomalacia (TM) occurs in approximately 1 in 2,100 children. Because the trachea develops abnormally in animal models of cystic fibrosis (CF), we hypothesized this may also occur in children with CF, increasing their risk of TM. PURPOSE To examine the prevalence and clinical consequences of TM in children with CF. METHODS We studied children with CF born between 1995 and 2012. TM was defined as dynamic collapse of the trachea, and the severity was recorded as described in the chart. The effect of TM on patient outcomes, including FEV1, CT changes, and acquisition of CF pathogens, was assessed using a longitudinal patient dataset. RESULTS 89% of children with CF had at least one bronchoscopy (n = 97/109). 15% of these children had TM described in any bronchoscopy report (n= 15/97). Of the patients with TM, 8 had meconium ileus (p = 0.003) and all were pancreatic insufficient. Pseudomonas aeruginosa infection occurred 1.3 years earlier among children with TM (p = 0.01). Starting FEV1 values by age 8 were diminished by over 18% of predicted for patients with TM. Life-threatening episodes of airway obstruction occurred in 3 of 15 patients with CF and TM, including one leading to death. Gender, prematurity, and hepatic disease were not associated with TM. No difference was observed in the frequency of bronchiectasis. CONCLUSIONS TM is significantly more common in infants and children with CF than in the general population and is associated with airway obstruction and earlier Pseudomonas acquisition. PMID:24166775

Idiopathic pulmonary fibrosis: evolving concepts.

PubMed

Ryu, Jay H; Moua, Teng; Daniels, Craig E; Hartman, Thomas E; Yi, Eunhee S; Utz, James P; Limper, Andrew H

2014-08-01

Idiopathic pulmonary fibrosis (IPF) occurs predominantly in middle-aged and older adults and accounts for 20% to 30% of interstitial lung diseases. It is usually progressive, resulting in respiratory failure and death. Diagnostic criteria for IPF have evolved over the years, and IPF is currently defined as a disease characterized by the histopathologic pattern of usual interstitial pneumonia occurring in the absence of an identifiable cause of lung injury. Understanding of the pathogenesis of IPF has shifted away from chronic inflammation and toward dysregulated fibroproliferative repair in response to alveolar epithelial injury. Idiopathic pulmonary fibrosis is likely a heterogeneous disorder caused by various interactions between genetic components and environmental exposures. High-resolution computed tomography can be diagnostic in the presence of typical findings such as bilateral reticular opacities associated with traction bronchiectasis/bronchiolectasis in a predominantly basal and subpleural distribution, along with subpleural honeycombing. In other circumstances, a surgical lung biopsy may be needed. The clinical course of IPF can be unpredictable and may be punctuated by acute deteriorations (acute exacerbation). Although progress continues in unraveling the mechanisms of IPF, effective therapy has remained elusive. Thus, clinicians and patients need to reach informed decisions regarding management options including lung transplant. The findings in this review were based on a literature search of PubMed using the search terms idiopathic pulmonary fibrosis and usual interstitial pneumonia, limited to human studies in the English language published from January 1, 2000, through December 31, 2013, and supplemented by key references published before the year 2000. Copyright © 2014 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

Primary Ciliary Dyskinesia. Recent Advances in Diagnostics, Genetics, and Characterization of Clinical Disease

PubMed Central

Daniels, Leigh Anne; Davis, Stephanie D.; Zariwala, Maimoona A.; Leigh, Margaret W.

2013-01-01

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous recessive disorder of motile cilia that leads to oto-sino-pulmonary diseases and organ laterality defects in approximately 50% of cases. The estimated incidence of PCD is approximately 1 per 15,000 births, but the prevalence of PCD is difficult to determine, primarily because of limitations in diagnostic methods that focus on testing ciliary ultrastructure and function. Diagnostic capabilities have recently benefitted from (1) documentation of low nasal nitric oxide production in PCD and (2) discovery of biallelic mutations in multiple PCD-causing genes. The use of these complementary diagnostic approaches shows that at least 30% of patients with PCD have normal ciliary ultrastructure. More accurate identification of patients with PCD has also allowed definition of a strong clinical phenotype, which includes neonatal respiratory distress in >80% of cases, daily nasal congestion and wet cough starting soon after birth, and early development of recurrent/chronic middle-ear and sinus disease. Recent studies, using advanced imaging and pulmonary physiologic assessments, clearly demonstrate early onset of lung disease in PCD, with abnormal air flow mechanics by age 6–8 years that is similar to cystic fibrosis, and age-dependent onset of bronchiectasis. The treatment of PCD is not standardized, and there are no validated PCD-specific therapies. Most patients with PCD receive suboptimal management, which should include airway clearance, regular surveillance of pulmonary function and respiratory microbiology, and use of antibiotics targeted to pathogens. The PCD Foundation is developing a network of clinical centers, which should improve diagnosis and management of PCD. PMID:23796196

A rare case of respiratory disorders associated with two autosomal recessive diseases and male infertility.

PubMed

Mendeluk, Gabriela Ruth; Costa, Sergio López; Scigliano, Sergio; Menga, Guillermo; Demiceu, Sergio; Palaoro, Luis Alberto

2013-01-01

The study of nasal ciliary beat frequency (CBF) and ultrastructure may contribute to the understanding of pathognomonic cases of male infertility associated with defects in sperm motility. This study was designed to report a particular case of male infertility, characterized by the association of two respiratory autosomal recessive genetic diseases (alpha-1-antitrypsin deficiency [AAT-D] and primary ciliary dyskinesia [PCD]). A 39-year-old patient with complete sperm immotility, AAT-D, and bronchiectasis was studied in the Laboratory of Male Fertility, the Department of Urology, the Respiratory Center of a Pediatric Hospital, and in the Department of Clinical Medicine of a Rehabilitation Respiratory Hospital. Family history, physical examination, hormonal analysis, microbial assays, semen analysis, nasal ciliary function, and structure study by digital high-speed video photography and transmission electron microscopy are described. A noninvasive nasal biopsy to retrieve ciliated epithelium lining the inferior surface of the inferior nasal turbinates was performed and CBF was determined. Beat pattern was slightly curved and rigid, not wide, and metacronic in all the observed fields analyzed. CBF was 8.2 Hz in average (reference value, 10-15 Hz) Ultrastructural assay revealed absence of the inner dynein arms in 97% of the cilia observed. The final infertility accurate diagnosis was achieved by the study of nasal CBF and ultrastructure contributing to the patient health management and genetic counseling while deciding fatherhood. Beyond this particular case, the present report may open a new field of studies in male infertility, mainly in cases of asthenozoospermia.

A rare case of respiratory disorders associated with two autosomal recessive diseases and male infertility

PubMed Central

Costa, Sergio López; Scigliano, Sergio; Menga, Guillermo; Demiceu, Sergio; Palaoro, Luis Alberto

2013-01-01

The study of nasal ciliary beat frequency (CBF) and ultrastructure may contribute to the understanding of pathognomonic cases of male infertility associated with defects in sperm motility. This study was designed to report a particular case of male infertility, characterized by the association of two respiratory autosomal recessive genetic diseases (alpha-1-antitrypsin deficiency [AAT-D] and primary ciliary dyskinesia [PCD]). A 39-year-old patient with complete sperm immotility, AAT-D, and bronchiectasis was studied in the Laboratory of Male Fertility, the Department of Urology, the Respiratory Center of a Pediatric Hospital, and in the Department of Clinical Medicine of a Rehabilitation Respiratory Hospital. Family history, physical examination, hormonal analysis, microbial assays, semen analysis, nasal ciliary function, and structure study by digital high-speed video photography and transmission electron microscopy are described. A noninvasive nasal biopsy to retrieve ciliated epithelium lining the inferior surface of the inferior nasal turbinates was performed and CBF was determined. Beat pattern was slightly curved and rigid, not wide, and metacronic in all the observed fields analyzed. CBF was 8.2 Hz in average (reference value, 10–15 Hz) Ultrastructural assay revealed absence of the inner dynein arms in 97% of the cilia observed. The final infertility accurate diagnosis was achieved by the study of nasal CBF and ultrastructure contributing to the patient health management and genetic counseling while deciding fatherhood. Beyond this particular case, the present report may open a new field of studies in male infertility, mainly in cases of asthenozoospermia. PMID:23772318

Retrospective evaluation of thoracic computed tomography findings in dogs naturally infected by Angiostrongylus vasorum.

PubMed

Coia, Mark E; Hammond, Gawain; Chan, Daniel; Drees, Randi; Walker, David; Murtagh, Kevin; Stone, Janine; Bexfield, Nicholas; Reeve, Lizzie; Helm, Jenny

2017-09-01

Angiostrongylus vasorum (A. vasorum) is an important emerging disease of canidae. Cardiorespiratory signs are common in affected dogs, therefore thoracic imaging is critical for diagnosing and monitoring disease. Descriptions of thoracic computed tomography (CT) findings in dogs naturally infected with A. vasorum are currently lacking. Aims of this multicenter, retrospective study were to describe thoracic CT findings in a group of dogs with confirmed disease, determine whether any changes were consistent among dogs, and propose standardized terms for describing thoracic CT findings. Nine UK-based referral centers' clinical and imaging databases were searched for dogs that had a confirmed diagnosis of A. vasorum, and had undergone thoracic CT examination. Eighteen dogs, from seven of the centers, fulfilled the inclusion criteria. The lung lobes were divided into the following three zones and the CT changes described in each: pleural (zone 1), subpleural (zone 2), and peribronchovascular (zone 3). The predominent abnormality was increased lung attenuation due to poorly defined ground-glass opacity or consolidation. There were regions of mosaic attenuation due to peripheral bronchiectasis. Nine/18 (50%) dogs showed hyperattenuating nodules of varying sizes with ill-defined margins. The distribution always affected zones 1 and 2 with varied involvement of zone 3; this resulted in clear delineation between zones 2 and 3. Tracheobronchial lymphadenomegaly was frequently noted. Findings were nonspecific and there was considerable overlap with other pulmonary conditions. However, authors recommend that A. vasorum be considered a likely differential diagnosis for dogs with a predominantly peripheral distribution of lung changes. © 2017 American College of Veterinary Radiology.

Respiratory problems in children with esophageal atresia and tracheoesophageal fistula.

PubMed

Porcaro, Federica; Valfré, Laura; Aufiero, Lelia Rotondi; Dall'Oglio, Luigi; De Angelis, Paola; Villani, Alberto; Bagolan, Pietro; Bottero, Sergio; Cutrera, Renato

2017-09-05

Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient's needs and harmonize the care. A retrospective data collection was performed on 105 children with EA and TEF followed at Department of Pediatric Medicine of Bambino Gesù Children's Hospital (Rome, Italy) between 2010 and 2015. 69/105 (66%) children reported lower respiratory symptoms with a mean age onset of 2.2 ± 2.5 years and only 63/69 (91%) performed specialist assessment at Respiratory Unit. Recurrent pneumonia (33%) and wheezing (31%) were the most reported symptoms. The first respiratory evaluation was performed after surgically correction of gastroesophageal reflux (GER) at mean age of 3.9 ± 4.2 years. Twenty nine patients have undergone to chest CT with contrast enhancement detecting localized atelectasis (41%), residual tracheal diverticulum (34%), bronchiectasis (31%), tracheal vascular compression (21%), tracheomalacia (17%) and esophageal diverticulum (14%). Fifty three patients have undergone to airways endoscopy detecting tracheomalacia (66%), residual tracheal diverticulum (26%), recurrent tracheoesophageal fistula (19%) and vocal cord paralysis (11%). Our study confirms that respiratory symptoms often complicate EA and TEF; their persistence despite medical and surgical treatment of GER means that other etiological hypothesis must be examined and that a complete respiratory diagnostic work up must be considered.

Sweat test practice in pediatric pulmonology after introduction of cystic fibrosis newborn screening.

PubMed

Grimaldi, Céline; Brémont, François; Berlioz-Baudoin, Michèle; Brouard, Jacques; Corvol, Harriet; Couderc, Laure; Lezmi, Guillaume; Pin, Isabelle; Petit, Isabelle; Reix, Philippe; Remus, Natacha; Schweitzer, Cyril; Thumerelle, Caroline; Dubus, Jean-Christophe

2015-12-01

The influence of the generalization of cystic fibrosis newborn screening (CFNBS) in France on sweat test (ST) prescription is unknown. In this French retrospective, descriptive, and multicenter study, we studied the indications, number, methods, and results of STs prescribed by a pediatric pulmonologist in children who had a negative CFNBS and an ST for respiratory symptoms in 2012. We included 502 children with 523 STs, performed with four different methods. The main indication was asthma (71.3 %), then chronic cough (52.4 %), atypical lower airway infections (42.2 %), and bronchiectasis (7 %). Four children had a diagnosis of CF (0.8 %), all presenting with chronic productive cough and recurrent respiratory infections. Asthma is the most frequent indication of ST in our cohort. Because of the systematic CFNBS in France, some prescriptions should be avoided, particularly in case of severe or moderate asthma with no other associated symptom. Moreover, methods of STs often do not follow the guidelines and need standardization. • Newborn screening (NBS) has become the most frequent circumstance of the diagnosis of cystic fibrosis (CF) in France after its generalization. • The prescription of sweat test (ST) in children with respiratory symptoms who already had a negative NBS has not been studied. • In children with a negative CF NBS referred to a university hospital for respiratory diseases, despite important variations of ST methods, only 4 children among 502 have been diagnosed as CF. • Despite recommendations, ST prescription should be avoided in children with moderate to severe asthma and no other associated symptom.

Assessing idiopathic pulmonary fibrosis (IPF) with bronchoscopic OCT (Conference Presentation)

NASA Astrophysics Data System (ADS)

Hariri, Lida P.; Adams, David C.; Colby, Thomas V.; Tager, Andrew M.; Suter, Melissa J.

2016-03-01

Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal form of fibrotic lung disease, with a significantly worse prognosis than other forms of pulmonary fibrosis (3-year survival rate of 50%). Distinguishing IPF from other fibrotic diseases is essential to patient care because it stratifies prognosis and therapeutic decision-making. However, making the diagnosis often requires invasive, high-risk surgical procedures to look for microscopic features not seen on chest CT, such as characteristic cystic honeycombing in the peripheral lung. Optical coherence tomography (OCT) provides rapid 3D visualization of large tissue volumes with microscopic resolutions well beyond the capabilities of CT. We aim to determine whether bronchoscopic OCT can provide a low-risk, non-surgical method for IPF diagnosis. We have developed bronchoscopic OCT catheters that access the peripheral lung and conducted in vivo peripheral lung imaging in patients, including those with pulmonary fibrosis. We also conducted bronchoscopic OCT in ex vivo lung from pulmonary fibrosis patients, including IPF, to determine if OCT could successfully visualize features of IPF through the peripheral airways. Our results demonstrate that OCT is able to visualize characteristic features of IPF through the airway, including microscopic honeycombing (< 1 mm diameter) not visible by CT, dense peripheral fibrosis, and spatial disease heterogeneity. We also found that OCT has potential to distinguish mimickers of IPF honeycombing, such as traction bronchiectasis and emphysema, from true honeycombing. These findings support the potential of bronchoscopic OCT as a minimally-invasive method for in vivo IPF diagnosis. However, future clinical studies are needed to validate these findings.

Clinical characterization of children with resistant airflow obstruction, a multicenter study.

PubMed

Krishnan, Sankaran; Dozor, Allen J; Bacharier, Leonard; Lang, Jason E; Irvin, Charles G; Kaminsky, David; Farber, Harold J; Gerald, Lynn; Brown, Mark; Holbrook, Janet T; Wise, Robert A; Ryu, Julie; Bose, Sonali; Yasin, Razan; Saams, Joy; Henderson, Robert J; Teague, William G

2018-05-17

To characterize a cohort of children with airflow limitation resistant to bronchodilator (BD) therapy. Pulmonary function tests performed in children 6-17 years of age at 15 centers in a clinical research consortium were screened for resistant airflow limitation, defined as a post-BD FEV 1 and/or an FEV 1 /FVC less than the lower limits of normal. Demographic and clinical data were analyzed for associations with pulmonary function. 582 children were identified. Median age was 13 years (IQR: 11, 16), 60% were males; 62% were Caucasian, 28% were African-American; 19% were obese; 32% were born prematurely and 21% exposed to second hand smoke. Pulmonary diagnoses included asthma (93%), prior significant pneumonia (28%), and bronchiectasis (5%). 65% reported allergic rhinitis, and 11% chronic sinusitis. Subjects without a history of asthma had significantly lower post-BD FEV 1 % predicted (p = 0.008). Subjects without allergic rhinitis had lower post-BD FEV 1 % predicted (p = 0.003). Children with allergic rhinitis, male sex, obesity and Black race had better pulmonary function post-BD. There was lower pulmonary function in children after age 11 years without a history of allergic rhinitis, as compared to those with a history of allergic rhinitis. The most prevalent diagnosis in children with BD-resistant airflow limitation is asthma. Allergic rhinitis and premature birth are common co-morbidities. Children without a history of asthma, as well as those with asthma but no allergic rhinitis, had lower pulmonary function. Children with BD-resistant airflow limitation may represent a sub-group of children with persistent obstruction and high risk for life-long airway disease.

In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.

PubMed

McGarry, Meghan E; Illek, Beate; Ly, Ngoc P; Zlock, Lorna; Olshansky, Sabrina; Moreno, Courtney; Finkbeiner, Walter E; Nielson, Dennis W

2017-04-01

Ivacaftor, a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, decreases sweat chloride concentration, and improves pulmonary function in 6% of cystic fibrosis (CF) patients with specific CFTR mutations. Ivacaftor increases chloride transport in many other CFTR mutations in non-human cells, if CFTR is in the epithelium. Some CF patients have CFTR in the epithelium with residual CFTR function. The effect of ivacaftor in these patients is unknown. This was a series of randomized, crossover N-of-1 trials of ivacaftor and placebo in CF patients ≥8 years old with potential residual CFTR function (intermediate sweat chloride concentration, pancreatic sufficient, or mild bronchiectasis on chest CT). Human nasal epithelium (HNE) was obtained via nasal brushing and cultured. Sweat chloride concentration change was the in vivo outcome. Chloride current change in HNE cultures with ivacaftor was the in vitro outcome. Three subjects had decreased sweat chloride concentration (-14.8 to -40.8 mmol/L, P < 0.01). Two subjects had unchanged sweat chloride concentration. Two subjects had increased sweat chloride concentration (+23.8 and +27.3 mmol/L, P < 0.001); both were heterozygous for A455E and pancreatic sufficient. Only subjects with decreased sweat chloride concentration had increased chloride current in HNE cultures. Some CF patients with residual CFTR function have decreased sweat chloride concentration with ivacaftor. Increased chloride current in HNE cultures among subjects with decreased sweat chloride concentrations may predict clinical response to ivacaftor. Ivacaftor can increase sweat chloride concentration in certain mutations with unclear clinical effect. Pediatr Pulmonol. 2017;52:472-479. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

Genetic mannose binding lectin deficiency is associated with airway microbiota diversity and reduced exacerbation frequency in COPD.

PubMed

Dicker, Alison J; Crichton, Megan L; Cassidy, Andrew J; Brady, Gill; Hapca, Adrian; Tavendale, Roger; Einarsson, Gisli G; Furrie, Elizabeth; Elborn, J Stuart; Schembri, Stuart; Marshall, Sara E; Palmer, Colin N A; Chalmers, James D

2018-06-01

In cystic fibrosis and bronchiectasis, genetic mannose binding lectin (MBL) deficiency is associated with increased exacerbations and earlier mortality; associations in COPD are less clear. Preclinical data suggest MBL interferes with phagocytosis of Haemophilus influenzae , a key COPD pathogen. We investigated whether MBL deficiency impacted on clinical outcomes or microbiota composition in COPD. Patients with COPD (n=1796) underwent MBL genotyping; linkage to health records identified exacerbations, lung function decline and mortality. A nested subcohort of 141 patients, followed for up to 6 months, was studied to test if MBL deficiency was associated with altered sputum microbiota, through 16S rRNA PCR and sequencing, or airway inflammation during stable and exacerbated COPD. Patients with MBL deficiency with COPD were significantly less likely to have severe exacerbations (incidence rate ratio (IRR) 0.66, 95% CI 0.48 to 0.90, p=0.009), or to have moderate or severe exacerbations (IRR 0.77, 95% CI 0.60 to 0.99, p=0.047). MBL deficiency did not affect rate of FEV 1 decline or mortality. In the subcohort, patients with MBL deficiency had a more diverse lung microbiota (p=0.008), and were less likely to be colonised with Haemophilus spp. There were lower levels of airway inflammation in patients with MBL deficiency. Patients with MBL deficient genotype with COPD have a lower risk of exacerbations and a more diverse lung microbiota. This is the first study to identify a genetic association with the lung microbiota in COPD. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Post-infectious bronchiolitis obliterans in children: CT features that predict responsiveness to pulse methylprednisolone.

PubMed

Yoon, H M; Lee, J S; Hwang, J-Y; Cho, Y A; Yoon, H-K; Yu, J; Hong, S-J; Yoon, C H

2015-05-01

Intravenous pulse methylprednisolone therapy (IPMT) is an important treatment option for post-infectious obliterative bronchiolitis (OB), although it must be used carefully and only in selected patients because of its drawbacks. This study evaluated whether CT and clinical features of children with post-infectious OB can predict their responsiveness to IPMT. We searched the medical records for patients (less than 18 years of age) who were diagnosed with post-infectious OB between January 2000 and December 2011. 17 children who received IPMT were included in this study. All underwent chest CT before and after IPMT. The radiological features seen on pre-treatment CT were recorded. The air-trapping area percentages on pre- and post-treatment CT images were determined. The nine patients who exhibited decreased air trapping on post-treatment CT scans relative to pre-treatment scans were classed as responders. The patient ages and time from initial pneumonia to IPMT were recorded. All responders and only four non-responders had thickened bronchial walls before treatment (p = 0.029). The two groups did not differ significantly in terms of bronchiolitis, bronchiectasis or the extent of air trapping, although the responders had a significantly shorter median interval between initial pneumonia and IPMT (4 vs 50 months; p = 0.005) and were significantly younger (median, 2.0 vs 7.5 years; p = 0.048). Immediate IPMT may improve the degree of air trapping in children with post-infectious OB if they show a thickened bronchial wall on CT. Children with post-infectious OB may respond favourably to IPMT when pre-treatment CT indicates bronchial-wall thickening.

Nocturnal cough in children with stable cystic fibrosis.

PubMed

van der Giessen, Lianne; Loeve, Martine; de Jongste, Johan; Hop, Wim; Tiddens, Harm

2009-09-01

To date no studies have been published on nocturnal cough frequency in children with stable CF. Aim of the study was to assess nocturnal cough frequency in children with CF. In addition nocturnal cough frequency was correlated with parameters of disease severity. During two nights cough was recorded with a digital audio recorder in 25 patients (mean age 13 years; range 6-19) with clinically stable CF. In addition oxygen saturation was measured. The day following the recording spirometry was carried out. CT scores were obtained from the most recent routine CT scan. Cough was expressed in cough seconds (csec) and in cough seconds per hour (csec/hr). Data shown are median values and interquartile range (IQR). First night: 8 csec (IQR 3-52); 0.9 csec/hr (IQR 0.3-6.1) Second night: 6 csec (IQR 2-32); 0.6 csec/hr (IQR 0.1-3.4). Csec in the 1st night did not correlate significantly with csec in the 2nd night. Only for the 2nd night a strong correlation was found between csec/hr and the FEV1%pred (r(s) = -0.75, P < 0.001) and FEF(75) %pred (r(s) = -0.71, P < 0.001). Bronchiectasis score correlated borderline with the mean csec/hr of both nights (r(s) = 0.39, P = 0.08). During both nights cough was significantly higher in the first hour of sleep (P < or = 0.04). Frequency of nocturnal coughing in children with CF was higher than that described for normal children. Nocturnal cough tended to be more severe in children with more advanced CF lung disease. Nocturnal cough was more severe in the first hour of sleep and varied from night-to-night. Copyright 2009 Wiley-Liss, Inc.

Nocturnal oxygen saturation in children with stable cystic fibrosis.

PubMed

van der Giessen, Lianne; Bakker, Marije; Joosten, Koen; Hop, Wim; Tiddens, Harm

2012-11-01

Hypoxemia during sleep is a common finding in Cystic Fibrosis (CF) patients with more advanced lung disease. Nocturnal hypoxemia is associated with frequent awakenings and poor sleep quality. For children with CF, data of nocturnal oxygen saturation are sparse. To assess the oxygen saturation profile during sleep in 25 clinically stable children with CF lung disease and to correlate these data with spirometry, cough frequency, sleep quality, and CT-scan scores. During two nights cough was recorded with a digital audio recorder in 25 clinically stable CF patients. In addition oxygen saturation was measured. The day following the recording spirometry was carried out. CT scores were obtained from the most recent routine CT scan. Twenty-two patients were included in the study. Mean age (range) was 13 (6-18) years. Spirometry was FVC% 84 (range 52-114), FEV(1) % 77 (range 43-115), and FEF(75) % 50 (range 12-112). The mean SO(2) was 95.6% for the first and 96.2% for the second night. Mean SO(2) between the two nights correlated strongly (r(s) = 0.84, P < 0.001). Positive correlation was observed between mean SO(2) of the two nights (mean × SO(2)) and FVC, FEV(1) and FEF(75). Correlations were found between mean × SO(2) and the total CT score (r(s) = -0.45, P = 0.05) and the bronchiectasis subscore (r(s) = -0.48, P = 0.03). Nocturnal oxygen saturation in children with stable CF is lower than that in healthy children, and is correlated with lung function parameters and CT scores. Monitoring oxygen saturation during one night is sufficient to get a representative recording. Copyright © 2012 Wiley Periodicals, Inc.

Mortality in perinatally HIV-infected young people in England following transition to adult care: an HIV Young Persons Network (HYPNet) audit.

PubMed

Fish, R; Judd, A; Jungmann, E; O'Leary, C; Foster, C

2014-04-01

Mortality in young people with perinatally acquired HIV infection (PHIV) following transfer to adult care has not been characterized in the UK. We conducted a multicentre audit to establish the number of deaths and associated factors. Fourteen adult clinics caring for infected young people reported deaths to 30 September 2011 on a proforma. Deaths were matched to the Collaborative HIV Paediatric Study, a clinical database of HIV-infected children in the UK/Ireland, to describe clinical characteristics in paediatric care of those who died post-transition. Eleven deaths were reported from 14 clinics which cared for 248 adults with PHIV. For the 11 deaths, the median age at transfer to adult care was 17 years (range 15-21 years), and at death was 21 years (range 17-24 years). Causes of death were suicide (two patients), advanced HIV disease (seven patients) and bronchiectasis (one patient), with one cause missing. At death, the median CD4 count was 27 cells/μL (range 0-630 cells/μL); five patients were on antiretroviral therapy (ART) but only two had a viral load < 50 HIV-1 RNA copies/mL. Nine had poor adherence when in paediatric care, continuing into adult care despite multidisciplinary support. Eight had ART resistance, although all had potentially suppressive regimens available. Nine had mental health diagnoses. Our findings highlight the complex medical and psychosocial issues faced by some adults with PHIV, with nine of the 11 deaths in our study being associated with poor adherence and advanced HIV disease. Novel adherence interventions and mental health support are required for this vulnerable cohort. © 2013 British HIV Association.

Primary antibody deficiencies at Queen Rania Children Hospital in Jordan: single center experience.

PubMed

Habahbeh, Zeyad M; Abu-Shukair, Mohammad E; Almutereen, Mohammad A; Alzyoud, Raed M; Wahadneh, Adel M

2014-03-01

Primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of B cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. This retrospective study aimed at description and analysis of demographic, clinical, immunological features and complications of subjects diagnosed with primary antibody deficiency at a referral center in Jordan. The medical records of pediatric patients who were diagnosed as primary antibody deficiency (PAD) during the period from January 2006 to June 2013 were reviewed. Patients were diagnosed as PADs based on the Pan-American Group for Immunodeficiency (PAGID) and the European Society for Immunodeficiency (ESID) diagnostic criteria. A total number of 53 patients with PAD were identified; 37(70%) males and 16(30%) females, 16(30%) patients with congenital agammaglobulinemia, 16(30%) patients with common variable immunodeficiency, 4(7.5%) patients with IgG subclass deficiency, 10(19%) cases with transient hypogammaglobulinemia of infancy and 7(13.5%) patients as undefined PAD. The most common infection among patients was pneumonia (62%); followed by suppurative otitis media in 49% of patients. Cytopenia was the most noted autoimmune association and was found at prevalence of 22 %, other autoimmune associations (17%) including inflammatory arthritis, discoid lupus, inflammatory bowel disease, vasculitis and celiac disease. The prevalence of long-term complications was 58%, the most frequent ones were; stunted growth in 13%, bronchiectasis and lymphoproliferation in 11% for each. Our results indicated that congenital agammaglobulinemia and common variable immunodeficiency are the most frequent primary antibody deficiency in our patients. The awareness of families, general population as well as primary health physicians is crucial in the establishment of early diagnosis and prompt commencement of appropriate therapy for PADs.

Surgical risk factors associated with lung transplantation.

PubMed

Paradela, M; González, D; Parente, I; Fernández, R; De La Torre, M M; Delgado, M; García, J A; Fieira, E; Bonhome, C; Maté, J M B

2009-01-01

Despite years of experience with lung transplantation, perioperative morbidity rates remain high. The objective of this study was to analyze our series of lung transplant recipients, seeking to identify possible intra- and postoperative risk factors associated with mortality. We performed a descriptive, retrospective study of 224 consecutive patients undergoing lung transplantation over a period of 112 months; we excluded retransplant procedures. We gathered details of the surgical procedure and postoperative period in the recovery unit. Univariate analysis using the chi-square test identified variables associated with the incidence of mortality. From 1999 to 2008, we performed 224 lung transplants, including 66% in men and 34% in women. Their overall mean age was 49.9 +/- 13.5 years. The conditions that led to transplantation were pulmonary fibrosis (38.4%); chronic obstructive pulmonary disease emphysema (29%); cystic fibrosis (10.7%); bronchiectasis (8.9%); pulmonary hypertension (3.1%); and other diseases (9.9%). A total of 124 (55.4%) patients underwent single and 100 (44.6%) received sequential bilateral lung transplantations. Surgical risk factors were identified in 51.3% of the cases, the most frequent being hemorrhage (25.3%), followed by severe pulmonary hypertension (14.7%) and cardiopulmonary bypass (12.1%). Greater perioperative mortality was detected among patients with surgical risk factors, namely, significantly related to cardiopulmonary bypass, pulmonary hypertension, and air leak. A higher frequency of surgical risk factors was observed among patients with bilateral lung transplantations and longer procedures, but they were not associated with greater perioperative mortality. Reoperation was necessary in 16 patients (7.2%), mainly owing to bleeding, it was not significantly related to mortality risk. The incidence of surgical risk factors in lung transplantation was high, especially in bilateral lung transplantations and prolonged procedures. Postoperative bleeding requiring reoperation was not frequent and not associated with increased preoperative mortality in our series.

Clinical characteristics and risk factors of pulmonary hypertension associated with chronic respiratory diseases: a retrospective study.

PubMed

Chen, Yonghua; Liu, Chunli; Lu, Wenju; Li, Mengxi; Hadadi, Cyrus; Wang, Elizabeth Wenqian; Yang, Kai; Lai, Ning; Huang, Junyi; Li, Shiyue; Zhong, Nanshan; Zhang, Nuofu; Wang, Jian

2016-03-01

Chronic respiratory disease-associated pulmonary hypertension (PH) is an important subtype of PH, which lacks clinical epidemiological data in China. Six hundred and ninety three patients hospitalized from 2010 to 2013 were classified by echocardiography according to pulmonary arterial systolic pressure (PASP): mild (36≤ PASP <50 mmHg); moderate (50≤ PASP <70 mmHg) and severe (PASP ≥70 mmHg). Dyspnea (93.51%) was the most common symptom. Hemoptysis observed in the severe group (6.42%) was significantly higher than the other two groups (P<0.05). COPD (78.35%), lung bullae (44.16%), tuberculosis (including obsolete pulmonary tuberculosis) (38.82%), and bronchiectasis (30.45%) were frequently present. Mild group occupied the highest proportion (84.7%) in COPD, while severe group occupied the highest proportion (19.3%) in pulmonary embolism (P<0.01). Age, partial pressure of oxygen (PaO2), hematocrit (HCT), partial pressure of carbon dioxide (PaCO2), increase of N-terminal pro brain natriuretic peptide (NT-proBNP) and right ventricular (RV) diameter (>20 mm) were associated with moderate-to-severe PH, while RV [odds ratio (OR) =3.53, 95% CI, 2.17-5.74], NT-proBNP (OR=2.44, 95% CI, 1.51-3.95), HCT (OR=1.03, 95% CI, 1.00-1.07) and PaCO2 (OR=1.01, 95% CI, 1.00-1.03) were independent risk factors. PH related to respiratory diseases is mostly mild to moderate, and the severity is associated with the category of respiratory disease. Increased HCT can be an independent risk factor for PH related to chronic respiratory diseases.

Chronic respiratory disease, inhaled corticosteroids and risk of non-tuberculous mycobacteriosis.

PubMed

Andréjak, Claire; Nielsen, Rikke; Thomsen, Vibeke Ø; Duhaut, Pierre; Sørensen, Henrik Toft; Thomsen, Reimar Wernich

2013-03-01

Chronic respiratory disease and inhaled corticosteroid (ICS) therapy for chronic obstructive pulmonary disease (COPD) increase the risk of pneumonia. Few data are available on the association of these risk factors with non-tuberculous mycobacterial (NTM) pulmonary disease. This study examined chronic respiratory diseases and ICS use as risk factors in a population-based case-control study encompassing all adults in Denmark with microbiologically confirmed NTM pulmonary disease between 1997 and 2008. The study included 10 matched population controls per case. Conditional logistic regression was used to compute adjusted ORs for NTM pulmonary disease with regard to chronic respiratory disease history. Overall, chronic respiratory disease was associated with a 16.5-fold (95% CI 12.2 to 22.2) increased risk of NTM pulmonary disease. The adjusted OR for NTM disease was 15.7 (95% CI 11.4 to 21.5) for COPD, 7.8 (95% CI 5.2 to 11.6) for asthma, 9.8 (95% CI 2.03 to 52.8) for pneumoconiosis, 187.5 (95% CI 24.8 to 1417.4) for bronchiectasis, and 178.3 (95% CI 55.4 to 574.3) for tuberculosis history. ORs were 29.1 (95% CI 13.3 to 63.8) for patients with COPD on current ICS therapy and 7.6 (95% CI 3.4 to 16.8) for patients with COPD who had never received ICS therapy. Among patients with COPD, ORs increased according to ICS dose, from 28.1 for low-dose intake to 47.5 for high-dose intake (more than 800 μg/day). The OR was higher for fluticasone than for budesonide. Chronic respiratory disease, particularly COPD treated with ICS therapy, is a strong risk factor for NTM pulmonary disease.

Prospective evaluation of respiratory exacerbations in children with cystic fibrosis from newborn screening to 5 years of age

PubMed Central

Byrnes, Catherine Ann; Vidmar, Suzanna; Cheney, Joyce L; Carlin, John B; Armstrong, David S; Cooper, Peter J; Grimwood, Keith; Moodie, Marj; Robertson, Colin F; Rosenfeld, Margaret; Tiddens, Harm A; Wainwright, Claire E

2013-01-01

Background Newborn screening allows novel treatments for cystic fibrosis (CF) to be trialled in early childhood before irreversible lung injury occurs. As respiratory exacerbations are a potential trial outcome variable, we determined their rate, duration and clinical features in preschool children with CF; and whether they were associated with growth, lung structure and function at age 5 years. Methods Respiratory exacerbations were recorded prospectively in Australasian CF Bronchoalveolar Lavage trial subjects from enrolment after newborn screening to age 5 years, when all participants underwent clinical assessment, chest CT scans and spirometry. Results 168 children (88 boys) experienced 2080 exacerbations, at an average rate of 3.66 exacerbations per person-year; 80.1% were community managed and 19.9% required hospital admission. There was an average increase in exacerbation rate of 9% (95% CI 4% to 14%; p<0.001) per year of age. Exacerbation rate differed by site (p<0.001) and was 26% lower (95% CI 12% to 38%) in children receiving 12 months of prophylactic antibiotics. The rate of exacerbations in the first 2 years was associated with reduced forced expiratory volume in 1 s z scores. Ever having a hospital-managed exacerbation was associated with bronchiectasis (OR 2.67, 95% CI 1.13 to 6.31) in chest CT scans, and lower weight z scores at 5 years of age (coefficient −0.39, 95% CI −0.74 to −0.05). Conclusions Respiratory exacerbations in young children are markers for progressive CF lung disease and are potential trial outcome measures for novel treatments in this age group. PMID:23345574

Is sweat chloride predictive of severity of cystic fibrosis lung disease assessed by chest computed tomography?

PubMed

Caudri, Daan; Zitter, David; Bronsveld, Inez; Tiddens, Harm

2017-09-01

Cystic Fibrosis (CF) lung disease is characterized by a marked heterogeneity. Sweat chloride-level is a functional marker of the CF Transmembrane Regulator (CFTR) protein and could be an important predictor of later disease severity. In this retrospective analysis children from the Rotterdam CF clinic with available sweat chloride level at diagnosis and at least one routine spirometry-controlled volumetric chest CT scan in follow-up were included. CT scans were scored using the CF-CT scoring system (% of maximum). Associations between sweat chloride-levels and CF-CT scores were calculated using linear regression models, adjusting for age at sweat test and age at follow-up. Because structural lung damage develops over the course of many years, effect modification by the age at follow-up CT-scan was tested for by age-stratification. In 59 children (30 male) sweat chloride was measured at diagnosis (median age 0.5 years, range 0-13) and later chest CT performed (median age 14 years, range 6-18). Sweat chloride was associated with significantly higher CT-CT total score, bronchiectasis score, and mucus plugging score. Stratification for age at follow-up in tertiles showed this association remained only in the oldest age group (range 15-18 years). In that subgroup associations were found with all but one of the CF-CT subscores, as well as with all tested lung functions parameters. Sweat chloride-level is a significant predictor of CF lung disease severity as determined by chest CT and lung function. This association could only be demonstrated in children with follow-up to age 15 years and above. © 2017 Wiley Periodicals, Inc.

Key considerations on nebulization of antimicrobial agents to mechanically ventilated patients.

PubMed

Rello, J; Rouby, J J; Sole-Lleonart, C; Chastre, J; Blot, S; Luyt, C E; Riera, J; Vos, M C; Monsel, A; Dhanani, J; Roberts, J A

2017-09-01

Nebulized antibiotics have an established role in patients with cystic fibrosis or bronchiectasis. Their potential benefit to treat respiratory infections in mechanically ventilated patients is receiving increasing interest. In this consensus statement of the European Society of Clinical Microbiology and Infectious Diseases, the body of evidence of the therapeutic utility of aerosolized antibiotics in mechanically ventilated patients was reviewed and resulted in the following recommendations: Vibrating-mesh nebulizers should be preferred to jet or ultrasonic nebulizers. To decrease turbulence and limit circuit and tracheobronchial deposition, we recommend: (a) the use of specifically designed respiratory circuits avoiding sharp angles and characterized by smooth inner surfaces, (b) the use of specific ventilator settings during nebulization including use of a volume controlled mode using constant inspiratory flow, tidal volume 8 mL/kg, respiratory frequency 12 to 15 bpm, inspiratory:expiratory ratio 50%, inspiratory pause 20% and positive end-expiratory pressure 5 to 10 cm H 2 O and (c) the administration of a short-acting sedative agent if coordination between the patient and the ventilator is not obtained, to avoid patient's flow triggering and episodes of peak decelerating inspiratory flow. A filter should be inserted on the expiratory limb to protect the ventilator flow device and changed between each nebulization to avoid expiratory flow obstruction. A heat and moisture exchanger and/or conventional heated humidifier should be stopped during the nebulization period to avoid a massive loss of aerosolized particles through trapping and condensation. If these technical requirements are not followed, there is a high risk of treatment failure and adverse events in mechanically ventilated patients receiving nebulized antibiotics for pneumonia. Copyright © 2017 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Use of nebulized antimicrobials for the treatment of respiratory infections in invasively mechanically ventilated adults: a position paper from the European Society of Clinical Microbiology and Infectious Diseases.

PubMed

Rello, J; Solé-Lleonart, C; Rouby, J-J; Chastre, J; Blot, S; Poulakou, G; Luyt, C-E; Riera, J; Palmer, L B; Pereira, J M; Felton, T; Dhanani, J; Bassetti, M; Welte, T; Roberts, J A

2017-09-01

With an established role in cystic fibrosis and bronchiectasis, nebulized antibiotics are increasingly being used to treat respiratory infections in critically ill invasively mechanically ventilated adult patients. Although there is limited evidence describing their efficacy and safety, in an era when there is a need for new strategies to enhance antibiotic effectiveness because of a shortage of new agents and increases in antibiotic resistance, the potential of nebulization of antibiotics to optimize therapy is considered of high interest, particularly in patients infected with multidrug-resistant pathogens. This Position Paper of the European Society of Clinical Microbiology and Infectious Diseases provides recommendations based on the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology regarding the use of nebulized antibiotics in invasively mechanically ventilated adults, based on a systematic review and meta-analysis of the existing literature (last search July 2016). Overall, the panel recommends avoiding the use of nebulized antibiotics in clinical practice, due to a weak level of evidence of their efficacy and the high potential for underestimated risks of adverse events (particularly, respiratory complications). Higher-quality evidence is urgently needed to inform clinical practice. Priorities of future research are detailed in the second part of the Position Paper as guidance for researchers in this field. In particular, the panel identified an urgent need for randomized clinical trials of nebulized antibiotic therapy as part of a substitution approach to treatment of pneumonia due to multidrug-resistant pathogens. Copyright © 2017 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Health conditions in rural areas with high livestock density: Analysis of seven consecutive years.

PubMed

van Dijk, Christel E; Zock, Jan-Paul; Baliatsas, Christos; Smit, Lidwien A M; Borlée, Floor; Spreeuwenberg, Peter; Heederik, Dick; Yzermans, C Joris

2017-03-01

Previous studies investigating health conditions of individuals living near livestock farms generally assessed short time windows. We aimed to take time-specific differences into account and to compare the prevalence of various health conditions over seven consecutive years. The sample consisted of 156,690 individuals registered in 33 general practices in a (rural) area with a high livestock density and 101,015 patients from 23 practices in other (control) areas in the Netherlands. Prevalence of health conditions were assessed using 2007-2013 electronic health record (EHR) data. Two methods were employed to assess exposure: 1) Comparisons between the study and control areas in relation to health problems, 2) Use of individual estimates of livestock exposure (in the study area) based on Geographic Information System (GIS) data. A higher prevalence of chronic bronchitis/bronchiectasis, lower respiratory tract infections and vertiginous syndrome and lower prevalence of respiratory symptoms and emphysema/COPD was found in the study area compared with the control area. A shorter distance to the nearest farm was associated with a lower prevalence of upper respiratory tract infections, respiratory symptoms, asthma, COPD/emphysema, allergic rhinitis, depression, eczema, vertiginous syndrome, dizziness and gastrointestinal infections. Especially exposure to cattle was associated with less health conditions. Living within 500m of mink farms was associated with increased chronic enteritis/ulcerative colitis. Livestock-related exposures did not seem to be an environmental risk factor for the occurrence of health conditions. Nevertheless, lower respiratory tract infections, chronic bronchitis and vertiginous syndrome were more common in the area with a high livestock density. The association between exposure to minks and chronic enteritis/ulcerative colitis remains to be elucidated. Copyright © 2016 Elsevier Ltd. All rights reserved.

The impact of smoke exposure on the clinical phenotype of alpha-1 antitrypsin deficiency in Ireland: exploiting a national registry to understand a rare disease.

PubMed

O'Brien, M Emmet; Pennycooke, Kevin; Carroll, Tomás P; Shum, Jonathan; Fee, Laura T; O'Connor, Catherine; Logan, P Mark; Reeves, Emer P; McElvaney, Noel G

2015-05-01

Individuals with Alpha-1 antitrypsin deficiency (AATD) have mutations in the SERPINA1 gene causing genetic susceptibility to early onset lung and liver disease that may result in premature death. Environmental interactions have a significant impact in determining the disease phenotype and outcome in AATD. The aim of this study was to assess the impact of smoke exposure on the clinical phenotype of AATD in Ireland. Clinical demographics and available thoracic computerised tomography (CT) imaging were detected from 139 PiZZ individuals identified from the Irish National AATD Registry. Clinical information was collected by questionnaire. Data was analysed to assess AATD disease severity and evaluate predictors of clinical phenotype. Questionnaires were collected from 107/139 (77%) and thoracic CT evaluation was available in 72/107 (67.2%). 74% of respondents had severe Chronic Obstructive Pulmonary Disease (COPD) (GOLD stage C or D). Cigarette smoking was the greatest predictor of impairment in FEV1 and DLCO (%predicted) and the extent of emphysema correlated most significantly with DLCO. Interestingly the rate of FEV1 decline was similar in ex-smokers when compared to never-smokers. Passive smoke exposure in childhood resulted in a greater total pack-year smoking history. Radiological evidence of bronchiectasis was a common finding and associated with increasing age. The Irish National AATD Registry facilitates clinical and basic science research of this condition in Ireland. This study illustrates the detrimental effect of smoke exposure on the clinical phenotype of AATD in Ireland and the benefit of immediate smoking cessation at any stage of lung disease.

Chronic suppurative lung disease in a developing country: impact on child and parent.

PubMed

Nathan, Anna Marie; Muthusamy, Ananthan; Thavagnanam, Surendran; Hashim, Azfawahiza; de Bruyne, Jessie

2014-05-01

To investigate the impact of chronic suppurative lung disease (CSLD) on growth and lung function in the child as well as quality of life of the child and parent. Cross-sectional study in 60 children with CSLD, bronchiectasis (including cystic fibrosis) and bronchiolitis obliterans. Thirty-five parents were interviewed while the remaining patients' data were collated from medical notes. Anthropometric measurements at first diagnosis and at interview were compared. The most recent lung function was also collected. The Parent Cough-Specific Quality of Life (PC-QOL) and the Depression, Anxiety and Stress (DASS21) questionnaires were administered to parents. The median (range) age at diagnosis was 1.3 (0.2-11) years. The median (IQR) duration between anthropometric measurements was 35 (15, 59) months. Children with cystic fibrosis (CF) had improvements both in weight and BMI, whereas children with non-CF CSLD had no improvements in any growth parameter. Seventy-eight percent of children who performed spirometry had values <80% of normal predicted value. PC-QOL scores were low. Frequent exacerbations (more than twice in the past 6 months) and cough (more than 2 days/week) were not associated with significantly lower PC-QOL scores. Seventy-seven percent of interviewed parents had abnormal DASS21 scores with 54% being stressed and 51% being depressed. Mental health was better in parents of children with CF. CSLD had a negative impact on growth, lung function, and quality of life. Children with CF had a better outcome in growth as well as better parental mental health compared to children with other etiologies. Pediatr Pulmonol. 2014; 49:435-440. © 2013 Wiley Periodicals, Inc. © 2014 Wiley Periodicals, Inc.

Clinical spectrum and features of activated phosphoinositide 3-kinase δ syndrome: A large patient cohort study.

PubMed

Coulter, Tanya I; Chandra, Anita; Bacon, Chris M; Babar, Judith; Curtis, James; Screaton, Nick; Goodlad, John R; Farmer, George; Steele, Cathal Laurence; Leahy, Timothy Ronan; Doffinger, Rainer; Baxendale, Helen; Bernatoniene, Jolanta; Edgar, J David M; Longhurst, Hilary J; Ehl, Stephan; Speckmann, Carsten; Grimbacher, Bodo; Sediva, Anna; Milota, Tomas; Faust, Saul N; Williams, Anthony P; Hayman, Grant; Kucuk, Zeynep Yesim; Hague, Rosie; French, Paul; Brooker, Richard; Forsyth, Peter; Herriot, Richard; Cancrini, Caterina; Palma, Paolo; Ariganello, Paola; Conlon, Niall; Feighery, Conleth; Gavin, Patrick J; Jones, Alison; Imai, Kohsuke; Ibrahim, Mohammad A A; Markelj, Gašper; Abinun, Mario; Rieux-Laucat, Frédéric; Latour, Sylvain; Pellier, Isabelle; Fischer, Alain; Touzot, Fabien; Casanova, Jean-Laurent; Durandy, Anne; Burns, Siobhan O; Savic, Sinisa; Kumararatne, D S; Moshous, Despina; Kracker, Sven; Vanhaesebroeck, Bart; Okkenhaug, Klaus; Picard, Capucine; Nejentsev, Sergey; Condliffe, Alison M; Cant, Andrew James

2017-02-01

Activated phosphoinositide 3-kinase δ syndrome (APDS) is a recently described combined immunodeficiency resulting from gain-of-function mutations in PIK3CD, the gene encoding the catalytic subunit of phosphoinositide 3-kinase δ (PI3Kδ). We sought to review the clinical, immunologic, histopathologic, and radiologic features of APDS in a large genetically defined international cohort. We applied a clinical questionnaire and performed review of medical notes, radiology, histopathology, and laboratory investigations of 53 patients with APDS. Recurrent sinopulmonary infections (98%) and nonneoplastic lymphoproliferation (75%) were common, often from childhood. Other significant complications included herpesvirus infections (49%), autoinflammatory disease (34%), and lymphoma (13%). Unexpectedly, neurodevelopmental delay occurred in 19% of the cohort, suggesting a role for PI3Kδ in the central nervous system; consistent with this, PI3Kδ is broadly expressed in the developing murine central nervous system. Thoracic imaging revealed high rates of mosaic attenuation (90%) and bronchiectasis (60%). Increased IgM levels (78%), IgG deficiency (43%), and CD4 lymphopenia (84%) were significant immunologic features. No immunologic marker reliably predicted clinical severity, which ranged from asymptomatic to death in early childhood. The majority of patients received immunoglobulin replacement and antibiotic prophylaxis, and 5 patients underwent hematopoietic stem cell transplantation. Five patients died from complications of APDS. APDS is a combined immunodeficiency with multiple clinical manifestations, many with incomplete penetrance and others with variable expressivity. The severity of complications in some patients supports consideration of hematopoietic stem cell transplantation for severe childhood disease. Clinical trials of selective PI3Kδ inhibitors offer new prospects for APDS treatment. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

Disease Evolution and Response to Rapamycin in Activated Phosphoinositide 3-Kinase δ Syndrome: The European Society for Immunodeficiencies-Activated Phosphoinositide 3-Kinase δ Syndrome Registry

PubMed Central

Maccari, Maria Elena; Abolhassani, Hassan; Aghamohammadi, Asghar; Aiuti, Alessandro; Aleinikova, Olga; Bangs, Catherine; Baris, Safa; Barzaghi, Federica; Baxendale, Helen; Buckland, Matthew; Burns, Siobhan O.; Cancrini, Caterina; Cant, Andrew; Cathébras, Pascal; Cavazzana, Marina; Chandra, Anita; Conti, Francesca; Coulter, Tanya; Devlin, Lisa A.; Edgar, J. David M.; Faust, Saul; Fischer, Alain; Garcia-Prat, Marina; Hammarström, Lennart; Heeg, Maximilian; Jolles, Stephen; Karakoc-Aydiner, Elif; Kindle, Gerhard; Kiykim, Ayca; Kumararatne, Dinakantha; Grimbacher, Bodo; Longhurst, Hilary; Mahlaoui, Nizar; Milota, Tomas; Moreira, Fernando; Moshous, Despina; Mukhina, Anna; Neth, Olaf; Neven, Benedicte; Nieters, Alexandra; Olbrich, Peter; Ozen, Ahmet; Schmid, Jana Pachlopnik; Picard, Capucine; Prader, Seraina; Rae, William; Reichenbach, Janine; Rusch, Stephan; Savic, Sinisa; Scarselli, Alessia; Scheible, Raphael; Sediva, Anna; Sharapova, Svetlana O.; Shcherbina, Anna; Slatter, Mary; Soler-Palacin, Pere; Stanislas, Aurelie; Suarez, Felipe; Tucci, Francesca; Uhlmann, Annette; van Montfrans, Joris; Warnatz, Klaus; Williams, Anthony Peter; Wood, Phil; Kracker, Sven; Condliffe, Alison Mary; Ehl, Stephan

2018-01-01

Activated phosphoinositide 3-kinase (PI3K) δ Syndrome (APDS), caused by autosomal dominant mutations in PIK3CD (APDS1) or PIK3R1 (APDS2), is a heterogeneous primary immunodeficiency. While initial cohort-descriptions summarized the spectrum of clinical and immunological manifestations, questions about long-term disease evolution and response to therapy remain. The prospective European Society for Immunodeficiencies (ESID)-APDS registry aims to characterize the disease course, identify outcome predictors, and evaluate treatment responses. So far, 77 patients have been recruited (51 APDS1, 26 APDS2). Analysis of disease evolution in the first 68 patients pinpoints the early occurrence of recurrent respiratory infections followed by chronic lymphoproliferation, gastrointestinal manifestations, and cytopenias. Although most manifestations occur by age 15, adult-onset and asymptomatic courses were documented. Bronchiectasis was observed in 24/40 APDS1 patients who received a CT-scan compared with 4/15 APDS2 patients. By age 20, half of the patients had received at least one immunosuppressant, but 2–3 lines of immunosuppressive therapy were not unusual before age 10. Response to rapamycin was rated by physician visual analog scale as good in 10, moderate in 9, and poor in 7. Lymphoproliferation showed the best response (8 complete, 11 partial, 6 no remission), while bowel inflammation (3 complete, 3 partial, 9 no remission) and cytopenia (3 complete, 2 partial, 9 no remission) responded less well. Hence, non-lymphoproliferative manifestations should be a key target for novel therapies. This report from the ESID-APDS registry provides comprehensive baseline documentation for a growing cohort that will be followed prospectively to establish prognostic factors and identify patients for treatment studies. PMID:29599784

Disease Evolution and Response to Rapamycin in Activated Phosphoinositide 3-Kinase δ Syndrome: The European Society for Immunodeficiencies-Activated Phosphoinositide 3-Kinase δ Syndrome Registry.

PubMed

Maccari, Maria Elena; Abolhassani, Hassan; Aghamohammadi, Asghar; Aiuti, Alessandro; Aleinikova, Olga; Bangs, Catherine; Baris, Safa; Barzaghi, Federica; Baxendale, Helen; Buckland, Matthew; Burns, Siobhan O; Cancrini, Caterina; Cant, Andrew; Cathébras, Pascal; Cavazzana, Marina; Chandra, Anita; Conti, Francesca; Coulter, Tanya; Devlin, Lisa A; Edgar, J David M; Faust, Saul; Fischer, Alain; Garcia-Prat, Marina; Hammarström, Lennart; Heeg, Maximilian; Jolles, Stephen; Karakoc-Aydiner, Elif; Kindle, Gerhard; Kiykim, Ayca; Kumararatne, Dinakantha; Grimbacher, Bodo; Longhurst, Hilary; Mahlaoui, Nizar; Milota, Tomas; Moreira, Fernando; Moshous, Despina; Mukhina, Anna; Neth, Olaf; Neven, Benedicte; Nieters, Alexandra; Olbrich, Peter; Ozen, Ahmet; Schmid, Jana Pachlopnik; Picard, Capucine; Prader, Seraina; Rae, William; Reichenbach, Janine; Rusch, Stephan; Savic, Sinisa; Scarselli, Alessia; Scheible, Raphael; Sediva, Anna; Sharapova, Svetlana O; Shcherbina, Anna; Slatter, Mary; Soler-Palacin, Pere; Stanislas, Aurelie; Suarez, Felipe; Tucci, Francesca; Uhlmann, Annette; van Montfrans, Joris; Warnatz, Klaus; Williams, Anthony Peter; Wood, Phil; Kracker, Sven; Condliffe, Alison Mary; Ehl, Stephan

2018-01-01

Activated phosphoinositide 3-kinase (PI3K) δ Syndrome (APDS), caused by autosomal dominant mutations in PIK3CD (APDS1) or PIK3R1 (APDS2), is a heterogeneous primary immunodeficiency. While initial cohort-descriptions summarized the spectrum of clinical and immunological manifestations, questions about long-term disease evolution and response to therapy remain. The prospective European Society for Immunodeficiencies (ESID)-APDS registry aims to characterize the disease course, identify outcome predictors, and evaluate treatment responses. So far, 77 patients have been recruited (51 APDS1, 26 APDS2). Analysis of disease evolution in the first 68 patients pinpoints the early occurrence of recurrent respiratory infections followed by chronic lymphoproliferation, gastrointestinal manifestations, and cytopenias. Although most manifestations occur by age 15, adult-onset and asymptomatic courses were documented. Bronchiectasis was observed in 24/40 APDS1 patients who received a CT-scan compared with 4/15 APDS2 patients. By age 20, half of the patients had received at least one immunosuppressant, but 2-3 lines of immunosuppressive therapy were not unusual before age 10. Response to rapamycin was rated by physician visual analog scale as good in 10, moderate in 9, and poor in 7. Lymphoproliferation showed the best response (8 complete, 11 partial, 6 no remission), while bowel inflammation (3 complete, 3 partial, 9 no remission) and cytopenia (3 complete, 2 partial, 9 no remission) responded less well. Hence, non-lymphoproliferative manifestations should be a key target for novel therapies. This report from the ESID-APDS registry provides comprehensive baseline documentation for a growing cohort that will be followed prospectively to establish prognostic factors and identify patients for treatment studies.

Air pollution and emergency department visits for respiratory diseases: A multi-city case crossover study.

PubMed

Szyszkowicz, Mieczysław; Kousha, Termeh; Castner, Jessica; Dales, Robert

2018-05-01

Increasing evidence suggests that ambient air pollution is a major risk factor for both acute and chronic respiratory disease exacerbations and emergencies. The objective of this study was to determine the association between ambient air pollutants and emergency department (ED) visits for respiratory conditions in nine districts across the province of Ontario in Canada. Health, air pollutant (PM 2.5 , NO 2 , O 3 , and SO 2 ), and meteorological data were retrieved from April 2004 to December 2011. Respiratory diseases were categorized as: chronic obstructive pulmonary disease (COPD, including bronchiectasis) and acute upper respiratory diseases. A case-crossover design was used to test the associations between ED visits and ambient air pollutants, stratified by sex and season. For COPD among males, positive results were observed for NO 2 with lags of 3-6 days, for PM 2.5 with lags 1-8, and for SO 2 with lags of 4-8 days. For COPD among females, positive results were observed for O 3 with lags 2-4 days, and for SO 2 among lags of 3-6 days. For upper respiratory disease emergencies among males, positive results were observed for NO 2 (lags 5-8 days), for O 3 , (lags 0-6 days), PM 2.5 (all lags), and SO 2 (lag 8), and among females, positive results were observed for NO 2 for lag 8 days, for O 3 , PM 2.5 among all lags. Our study provides evidence of the associations between short-term exposure to air pollution and increased risk of ED visits for upper and lower respiratory diseases in an environment where air pollutant concentrations are relatively low. Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.

The association between gender and pediatric respiratory morbidity.

PubMed

Ben-Shmuel, Atar; Sheiner, Eyal; Wainstock, Tamar; Landau, Daniella; Vaknin, Flear; Walfisch, Asnat

2018-06-26

To evaluate the association between newborn gender and the risk for later pediatric respiratory morbidity. A population based cohort analysis was performed by comparing the risk of long-term respiratory morbidity (until 18 years of age) according to gender. Respiratory morbidity included hospitalizations involving pneumonia, asthma, bronchitis, bronchiolitis, upper respiratory tract infection (URTI), influenza, and bronchiectasis. Deliveries occurred between the years 1991 and 2014 in a tertiary medical center. Kaplan-Meier survival curves were constructed to compare cumulative respiratory morbidity. A Cox proportional hazards model controlled for confounders. During the study period 240 953 newborns met the inclusion criteria. Among them, 118 113 were females (49.0%) and 122 840 were males (51.0%). During the 18 years of follow-up, 13 719 (5.7%) different newborns were hospitalized with respiratory related morbidity. Males had significantly higher rates of respiratory morbidity as compared with females (6.4% vs 4.9% respectively, P < 0.001, OR 1.32, 95% CI 1.28-1.37). Specifically, pneumonia, bronchitis, asthma, bronchiolitis, and URTI were significantly more common in males. Males exhibited higher total cumulative respiratory morbidity (log rank P < 0.001), as well as higher cumulative morbidity in several sub-categories. These sub-categories included pneumonia, bronchitis, asthma, bronchiolitis, and URTI (P < 0.05 in all). The Cox regression model demonstrated male gender to be an independent risk factor for pediatric respiratory morbidity while adjusting for gestational age, birthweight, and other confounders (HR 1.29, 95% CI 1.25-1.34, P < 0.001). Males are at an increased risk for pediatric respiratory morbidity, independent of obstetrical characteristics such as gestational age and birthweight. © 2018 Wiley Periodicals, Inc.

Comparison of chest CT findings in nontuberculous mycobacterial diseases vs. Mycobacterium tuberculosis lung disease in HIV-negative patients with cavities.

PubMed

Kim, Cherry; Park, So Hee; Oh, Sang Young; Kim, Sung-Soo; Jo, Kyung-Wook; Shim, Tae Sun; Kim, Mi Young

2017-01-01

This article focuses on the differences between CT findings of HIV-negative patients who have cavities with nontuberculous mycobacteria (NTM) disease and those with Mycobacterium tuberculosis infections (TB). We retrospectively reviewed 128 NTM disease patients (79 males and 49 females) with cavities in chest CT, matched for age and gender with 128 TB patients in the same period. Sputum cultures of all patients were positive for pathogens. Two independent chest radiologists evaluated the characteristics of the largest cavity and related factors. Interobserver agreement was excellent (κ value, 0.853-0.938). Cavity walls in NTM disease were significantly thinner (6.9±4 mm vs 10.9±6 mm, P<0.001) and more even (the ratio of thickness, 2.6±1 vs 3.7±2, P<0.001) than those in TB. The thickening of adjacent pleura next to the cavity was also significantly thicker in NTM than TB (P<0.001). However, in the multivariate analysis, thickening of adjacent pleura was the only significant factor among the representative cavity findings (Odds ratio [OR], 6.49; P<0.001). In addition, ill-defined tree-in-bud nodules (OR, 8.82; P<0.001), number of non-cavitary nodules (≥10mm) (OR, 0.72; P = 0.003), and bronchiectasis in the RUL (OR, 5.3; P = 0.002) were significantly associated ancillary findings with NTM disease in the multivariate analysis. The major cavities in NTM disease generally have thinner and more even walls than those in TB. When cavities are associated with adjacent pleural thickening, ill-defined satellite tree-in-bud nodules, or fewer non-cavitary nodules ≥10 mm, these CT findings are highly suggestive of NTM disease rather than TB.

HRCT findings of collagen vascular disease-related interstitial pneumonia (CVD-IP): a comparative study among individual underlying diseases.

PubMed

Tanaka, N; Kunihiro, Y; Kubo, M; Kawano, R; Oishi, K; Ueda, K; Gondo, T

2018-05-29

To identify characteristic high-resolution computed tomography (CT) findings for individual collagen vascular disease (CVD)-related interstitial pneumonias (IPs). The HRCT findings of 187 patients with CVD, including 55 patients with rheumatoid arthritis (RA), 50 with systemic sclerosis (SSc), 46 with polymyositis/dermatomyositis (PM/DM), 15 with mixed connective tissue disease, 11 with primary Sjögren's syndrome, and 10 with systemic lupus erythematosus, were evaluated. Lung parenchymal abnormalities were compared among CVDs using χ 2 test, Kruskal-Wallis test, and multiple logistic regression analysis. A CT-pathology correlation was performed in 23 patients. In RA-IP, honeycombing was identified as the significant indicator based on multiple logistic regression analyses. Traction bronchiectasis (81.8%) was further identified as the most frequent finding based on χ 2 test. In SSc IP, lymph node enlargement and oesophageal dilatation were identified as the indicators based on multiple logistic regression analyses, and ground-glass opacity (GGO) was the most extensive based on Kruskal-Wallis test, which reflects the higher frequency of the pathological nonspecific interstitial pneumonia (NSIP) pattern present in the CT-pathology correlation. In PM/DM IP, airspace consolidation and the absence of honeycombing were identified as the indicators based on multiple logistic regression analyses, and predominance of consolidation over GGO (32.6%) and predominant subpleural distribution of GGO/consolidation (41.3%) were further identified as the most frequent findings based on χ 2 test, which reflects the higher frequency of the pathological NSIP and/or the organising pneumonia patterns present in the CT-pathology correlation. Several characteristic high-resolution CT findings with utility for estimating underlying CVD were identified. Copyright © 2018 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

Should lung transplantation be performed for patients on mechanical respiratory support? The US experience.

PubMed

Mason, David P; Thuita, Lucy; Nowicki, Edward R; Murthy, Sudish C; Pettersson, Gösta B; Blackstone, Eugene H

2010-03-01

The study objectives were to (1) compare survival after lung transplantation in patients requiring pretransplant mechanical ventilation or extracorporeal membrane oxygenation with that of patients not requiring mechanical support and (2) identify risk factors for mortality. Data were obtained from the United Network for Organ Sharing for lung transplantation from October 1987 to January 2008. A total of 15,934 primary transplants were performed: 586 in patients on mechanical ventilation and 51 in patients on extracorporeal membrane oxygenation. Differences between nonsupport patients and those on mechanical ventilation or extracorporeal membrane oxygenation support were expressed as 2 propensity scores for use in comparing risk-adjusted survival. Unadjusted survival at 1, 6, 12, and 24 months was 83%, 67%, 62%, and 57% for mechanical ventilation, respectively; 72%, 53%, 50%, and 45% for extracorporeal membrane oxygenation, respectively; and 93%, 85%, 79%, and 70% for unsupported patients, respectively (P < .0001). Recipients on mechanical ventilation were younger, had lower forced vital capacity, and had diagnoses other than emphysema. Recipients on extracorporeal membrane oxygenation were also younger, had higher body mass index, and had diagnoses other than cystic fibrosis/bronchiectasis. Once these variables, transplant year, and propensity for mechanical support were accounted for, survival remained worse after lung transplantation for patients on mechanical ventilation and extracorporeal membrane oxygenation. Although survival after lung transplantation is markedly worse when preoperative mechanical support is necessary, it is not dismal. Thus, additional risk factors for mortality should be considered when selecting patients for lung transplantation to maximize survival. Reduced survival for this high-risk population raises the important issue of balancing maximal individual patient survival against benefit to the maximum number of patients. Copyright 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

Long-term health effects of chemical warfare agents on children following a single heavy exposure.

PubMed

Talabani, J M; Ali, A I; Kadir, A M; Rashid, R; Samin, F; Greenwood, D; Hay, Awm

2017-01-01

In the 1980s, villages in the Kurdistan region of Iraq were exposed to chemical weapons (CWs), which killed and injured thousands of civilians. There has been no clinical assessment of the long-term effects of CWs exposure on those injured. We report the first such evaluation of CW effects on long-term health of children. Patients from the CW-exposed areas were interviewed to assess previous and current clinical history and underwent clinical examination. The status of organs known to be targets of CWs, including skin, eyes, respiratory and neuromuscular systems, was assessed. Children of similar age and social background, but with no history of CW exposure, were selected as a control population. Results showed that 70% of children in the CWs group had chronic health problems in contrast to 3.3% in the unexposed group ( p < 0.0001). Fifty-five per cent of the CW-exposed group had long-term visual impairment but none in the unexposed population. Thirty-six per cent of the CW-exposed group had chronic dermatological conditions compared with 0.8% of the unexposed group ( p < 0.0001), 31% of the CWs group had neurological sequelae compared with 0.4% of the unexposed group ( p < 0.0001) and 51% of the CWs group had long-term respiratory problems compared with 1.5% of the unexposed group ( p < 0.0001). Respiratory complaints including asthma, chronic bronchitis and bronchiectasis were particularly common. Our study suggests that CWs used were probably a combination of sulphur mustard and organophosphate nerve agents. Results also indicate that the prevalence of acute and chronic health problems following exposure to CW agents appear to be higher in children compared with reported data in adults.

Autosomal Dominant STAT3 Deficiency and Hyper-IgE Syndrome Molecular, Cellular, and Clinical Features From a French National Survey

PubMed Central

Chandesris, Marie-Olivia; Melki, Isabelle; Natividad, Angels; Puel, Anne; Fieschi, Claire; Yun, Ling; Thumerelle, Caroline; Oksenhendler, Eric; Boutboul, David; Thomas, Caroline; Hoarau, Cyrille; Lebranchu, Yvon; Stephan, Jean-Louis; Cazorla, Celine; Aladjidi, Nathalie; Micheau, Marguerite; Tron, Fran[cedil]cois; Baruchel, Andre; Barlogis, Vincent; Palenzuela, Gilles; Mathey, Catherine; Dominique, Stephane; Body, Gerard; Munzer, Martine; Fouyssac, Fanny; Jaussaud, Rolland; Bader-Meunier, Brigitte; Mahlaoui, Nizar; Blanche, Stephane; Debre, Marianne; Le Bourgeois, Muriel; Gandemer, Virginie; Lambert, Nathalie; Grandin, Virginie; Ndaga, Stephanie; Jacques, Corinne; Harre, Chantal; Forveille, Monique; Alyanakian, Marie-Alexandra; Durandy, Anne; Bodemer, Christine; Suarez, Felipe; Hermine, Olivier; Lortholary, Olivier; Casanova, Jean-Laurent; Fischer, Alain; Picard, Capucine

2013-01-01

Autosomal dominant deficiency of signal transducer and activator of transcription 3 (STAT3) is the main genetic etiology of hyper-immunoglobulin (Ig) E syndrome. We documented the molecular, cellular, and clinical features of 60 patients with heterozygous STAT3 mutations from 47 kindreds followed in France. We identified 11 known and 13 new mutations of STAT3. Low levels of interleukin (IL)-6-dependent phosphorylation and nuclear translocation (or accumulation) of STAT3 were observed in Epstein-Barr virus-transformed B lymphocytes (EBV-B cells) from all STAT3-deficient patients tested. The immunologic phenotype was characterized by high serum IgE levels (96% of the patients), memory B-cell lymphopenia (94.5%), and hypereosinophilia (80%). A low proportion of IL-17A-producing circulating T cells was found in 14 of the 15 patients tested. Mucocutaneous infections were the most frequent, typically caused by Staphylococcus aureus (all patients) and Candida albicans (85%). Up to 90% of the patients had pneumonia, mostly caused by Staph. aureus (31%) or Streptococcus pneumoniae (30%). Recurrent pneumonia was associated with secondary bronchiectasis and pneumatocele (67%), as well as secondary aspergillosis (22%). Up to 92% of the patients had dermatitis and connective tissue abnormalities, with facial dysmorphism (95%), retention of decidual teeth (65%), osteopenia (50%), and hyperextensibility (50%). Four patients developed non-Hodgkin lymphoma. The clinical outcome was favorable, with 56 patients, including 43 adults, still alive at the end of study (mean age, 21 yr; range, 1 mo to 46 yr). Only 4 patients died, 3 from severe bacterial infection (aged 1, 15, and 29 yr, respectively). Antibiotic prophylaxis (90% of patients), antifungal prophylaxis (50%), and IgG infusions (53%) improved patient health, as demonstrated by the large decrease in pneumonia recurrence. Overall, the prognosis of STAT3 deficiency may be considered good, provided that multiple prophylactic measures, including IgG infusions, are implemented. PMID:22751495

Chronic Obstructive Pulmonary Disease: Lobe-based Visual Assessment of Volumetric CT by Using Standard Images—Comparison with Quantitative CT and Pulmonary Function Test in the COPDGene Study

PubMed Central

Kim, Song Soo; Lee, Ho Yun; Nevrekar, Dipti V.; Forssen, Anna V.; Crapo, James D.; Schroeder, Joyce D.; Lynch, David A.

2013-01-01

Purpose: To provide a new detailed visual assessment scheme of computed tomography (CT) for chronic obstructive pulmonary disease (COPD) by using standard reference images and to compare this visual assessment method with quantitative CT and several physiologic parameters. Materials and Methods: This research was approved by the institutional review board of each institution. CT images of 200 participants in the COPDGene study were evaluated. Four thoracic radiologists performed independent, lobar analysis of volumetric CT images for type (centrilobular, panlobular, and mixed) and extent (on a six-point scale) of emphysema, the presence of bronchiectasis, airway wall thickening, and tracheal abnormalities. Standard images for each finding, generated by two radiologists, were used for reference. The extent of emphysema, airway wall thickening, and luminal area were quantified at the lobar level by using commercial software. Spearman rank test and simple and multiple regression analyses were performed to compare the results of visual assessment with physiologic and quantitative parameters. Results: The type of emphysema, determined by four readers, showed good agreement (κ = 0.63). The extent of the emphysema in each lobe showed good agreement (mean weighted κ = 0.70) and correlated with findings at quantitative CT (r = 0.75), forced expiratory volume in 1 second (FEV1) (r = −0.68), FEV1/forced vital capacity (FVC) ratio (r = −0.74) (P < .001). Agreement for airway wall thickening was fair (mean κ = 0.41), and the number of lobes with thickened bronchial walls correlated with FEV1 (r = −0.60) and FEV1/FVC ratio (r = −0.60) (P < .001). Conclusion: Visual assessment of emphysema and airways disease in individuals with COPD can provide reproducible, physiologically substantial information that may complement that provided by quantitative CT assessment. © RSNA, 2012 Supplemental material: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.12120385/-/DC1 PMID:23220894

The lung mycobiome: an emerging field of the human respiratory microbiome

PubMed Central

Nguyen, Linh D. N.; Viscogliosi, Eric; Delhaes, Laurence

2015-01-01

The lung microbiome, which is believed to be stable or at least transient in healthy people, is now considered as a poly-microorganism component contributing to disease pathogenesis. Most research studies on the respiratory microbiome have focused on bacteria and their impact on lung health, but there is evidence that other non-bacterial organisms, comprising the viruses (virome) and fungi (mycobiome), are also likely to play an important role in healthy people as well as in patients. In the last few years, the lung mycobiome (previously named the fungal microbiota or microbiome) has drawn closer attention. There is growing evidence that the lung mycobiome has a significant impact on clinical outcome of chronic respiratory diseases (CRD) such as asthma, chronic obstructive pulmonary disease, cystic fibrosis, and bronchiectasis. Thanks to advances in culture independent methods, especially next generation sequencing, a number of fungi not detected by culture methods have been molecularly identified in human lungs. It has been shown that the structure and diversity of the lung mycobiome vary in different populations (healthy and different diseased individuals) which could play a role in CRD. Moreover, the link between lung mycobiome and different biomes of other body sites, especially the gut, has also been unraveled. By interacting with the bacteriome and/or virome, the respiratory mycobiome appears to be a cofactor in inflammation and in the host immune response, and therefore may contribute to the decline of the lung function and the disease progression. In this review, we report the recent limited explorations of the human respiratory mycobiome, and discuss the mycobiome’s connections with other local microbial communities, as well as the relationships with the different biomes of other body sites. These studies suggest several outlooks for this understudied emerging field, which will certainly call for a renewal of our understanding of pulmonary diseases. PMID:25762987

Chronic obstructive pulmonary disease: quantitative and visual ventilation pattern analysis at xenon ventilation CT performed by using a dual-energy technique.

PubMed

Park, Eun-Ah; Goo, Jin Mo; Park, Sang Joon; Lee, Hyun Ju; Lee, Chang Hyun; Park, Chang Min; Yoo, Chul-Gyu; Kim, Jong Hyo

2010-09-01

To evaluate the potential of xenon ventilation computed tomography (CT) in the quantitative and visual analysis of chronic obstructive pulmonary disease (COPD). This study was approved by the institutional review board. After informed consent was obtained, 32 patients with COPD underwent CT performed before the administration of xenon, two-phase xenon ventilation CT with wash-in (WI) and wash-out (WO) periods, and pulmonary function testing (PFT). For quantitative analysis, results of PFT were compared with attenuation parameters from prexenon images and xenon parameters from xenon-enhanced images in the following three areas at each phase: whole lung, lung with normal attenuation, and low-attenuating lung (LAL). For visual analysis, ventilation patterns were categorized according to the pattern of xenon attenuation in the area of structural abnormalities compared with that in the normal-looking background on a per-lobe basis: pattern A consisted of isoattenuation or high attenuation in the WI period and isoattenuation in the WO period; pattern B, isoattenuation or high attenuation in the WI period and high attenuation in the WO period; pattern C, low attenuation in both the WI and WO periods; and pattern D, low attenuation in the WI period and isoattenuation or high attenuation in the WO period. Among various attenuation and xenon parameters, xenon parameters of the LAL in the WO period showed the best inverse correlation with results of PFT (P < .0001). At visual analysis, while emphysema (which affected 99 lobes) commonly showed pattern A or B, airway diseases such as obstructive bronchiolitis (n = 5) and bronchiectasis (n = 2) and areas with a mucus plug (n = 1) or centrilobular nodules (n = 5) showed pattern D or C. WI and WO xenon ventilation CT is feasible for the simultaneous regional evaluation of structural and ventilation abnormalities both quantitatively and qualitatively in patients with COPD. (c) RSNA, 2010.

Simple image-based no-wash method for quantitative detection of surface expressed CFTR

PubMed Central

Larsen, Mads Breum; Hu, Jennifer; Frizzell, Raymond A.; Watkins, Simon C.

2016-01-01

Cystic fibrosis (CF) is the most common lethal genetic disease among Caucasians. It is caused by mutations in the CF Transmembrane Conductance Regulator (CFTR) gene, which encodes an apical membrane anion channel that is required for regulating the volume and composition of epithelial secretions. The most common CFTR mutation, present on at least one allele in >90% of CF patients, deletes phenylalanine at position 508 (F508del), which causes the protein to misfold. Endoplasmic reticulum (ER) quality control elicits the degradation of mutant CFTR, compromising its trafficking to the epithelial cell apical membrane. The absence of functional CFTR leads to depletion of airway surface liquid, impaired clearance of mucus and bacteria from the lung, and predisposes to recurrent infections. Ultimately, respiratory failure results from inflammation and bronchiectasis. Although high throughput screening has identified small molecules that can restore the anion transport function of F508del CFTR, they correct less than 15% of WT CFTR activity, yielding insufficient clinical benefit. To date, most primary CF drug discovery assays have employed measurements of CFTR’s anion transport function, a method that depends on the recruitment of a functional CFTR to the cell surface, involves multiple wash steps, and relies on a signal that saturates rapidly. Screening efforts have also included assays for detection of extracellularly HA-tagged or HRP-tagged CFTR, which require multiple washing steps. We have recently developed tools and cell lines that report the correction of mutant CFTR trafficking by currently available small molecules, and have extended this assay to the 96-well format. This new and simple no-wash assay of F508del CFTR at the cell surface may permit the discovery of more efficacious drugs, and hopefully thereby prevent the catastrophic effects of this disease. In addition, the modular design of this platform should make it useful for other diseases where loss-of-function results from folding and/or trafficking defects in membrane proteins. PMID:26361332

The impact of slice-reduced computed tomography on histogram-based densitometry assessment of lung fibrosis in patients with systemic sclerosis.

PubMed

Nguyen-Kim, Thi Dan Linh; Maurer, Britta; Suliman, Yossra A; Morsbach, Fabian; Distler, Oliver; Frauenfelder, Thomas

2018-04-01

To evaluate usability of slice-reduced sequential computed tomography (CT) compared to standard high-resolution CT (HRCT) in patients with systemic sclerosis (SSc) for qualitative and quantitative assessment of interstitial lung disease (ILD) with respect to (I) detection of lung parenchymal abnormalities, (II) qualitative and semiquantitative visual assessment, (III) quantification of ILD by histograms and (IV) accuracy for the 20%-cut off discrimination. From standard chest HRCT of 60 SSc patients sequential 9-slice-computed tomography (reduced HRCT) was retrospectively reconstructed. ILD was assessed by visual scoring and quantitative histogram parameters. Results from standard and reduced HRCT were compared using non-parametric tests and analysed by univariate linear regression analyses. With respect to the detection of parenchymal abnormalities, only the detection of intrapulmonary bronchiectasis was significantly lower in reduced HRCT compared to standard HRCT (P=0.039). No differences were found comparing visual scores for fibrosis severity and extension from standard and reduced HRCT (P=0.051-0.073). All scores correlated significantly (P<0.001) to histogram parameters derived from both, standard and reduced HRCT. Significant higher values of kurtosis and skewness for reduced HRCT were found (both P<0.001). In contrast to standard HRCT histogram parameters from reduced HRCT showed significant discrimination at cut-off 20% fibrosis (sensitivity 88% kurtosis and skewness; specificity 81% kurtosis and 86% skewness; cut-off kurtosis ≤26, cut-off skewness ≤4; both P<0.001). Reduced HRCT is a robust method to assess lung fibrosis in SSc with minimal radiation dose with no difference in scoring assessment of lung fibrosis severity and extension in comparison to standard HRCT. In contrast to standard HRCT histogram parameters derived from the approach of reduced HRCT could discriminate at a threshold of 20% lung fibrosis with high sensitivity and specificity. Hence it might be used to detect early disease progression of lung fibrosis in context of monitoring and treatment of SSc patients.

Higher HTLV-1c proviral loads are associated with blood stream infections in an Indigenous Australian population.

PubMed

Einsiedel, Lloyd; Cassar, Olivier; Spelman, Tim; Joseph, Sheela; Gessain, Antoine

2016-05-01

An association between blood stream infections (BSI) and HTLV-1 seropositivity in Indigenous Australians might result from HTLV-1 mediated inflammation and parasite coinfections that provide portals of entry for bacteria. To determine whether BSI risk increases with HTLV-1c proviral load (PVL) and to identify the pathogens responsible in the context of HTLV-1 related conditions. Indigenous adults admitted to Alice Springs Hospital, central Australia, were recruited as cases or controls according to whether they had a BSI. Clinical data were extracted from case notes and the hospital pathology database. HTLV-1 serology and PVL assays were then performed and risk factors for BSI were determined for HTLV-1 infected subjects. Risk factors were compared between 44 cases and 30 controls who were HTLV-1 Western blot positive. In a multivariable model, high HTLV-1c PVL was predictive of BSI during the study period (OR, 9.10; 95% CI, 2.40-34.4). Median (IQR) HTLV-1c PVL (copies per 100 PBL) was 39-fold higher for patients recording any BSI (0.116 (0.009, 0.277)) relative to those who had no BSI (0.003 (0.000, 0.067))(p=0.0007). Mean (SD) PVL for subjects with no BSI (n=27), 1 BSI (n=37) and ≥2 BSI (n=10) during the study period were 0.120 (0.301), 0.271 (0.622) and 0.500 (0.654) copies per 100 PBL, respectively (p=0.0007). Five BSI were associated with possible complications of HTLV-1; strongyloidiasis (3), infective dermatitis (1), HTLV-1 associated bronchiectasis (1). Higher HTLV-1c PVL predicts BSI risk; however; most BSI were not associated with recognised complications of HTLV-1 infection. Crown Copyright © 2016. Published by Elsevier B.V. All rights reserved.

Survival of patients with chronic respiratory failure on long-term oxygen therapy and or non-invasive ventilation at home.

PubMed

Cano, Noël J; Pichard, Claude; Court-Fortune, Isabelle; Costes, Frédéric; Cynober, Luc; Gérard-Boncompain, Michèle; Molano, Luis Carlos; Cuvelier, Antoine; Laaban, Jean-Pierre; Melchior, Jean-Claude; Raphaël, Jean-Claude; France, Julie; Lloret, Thomas; Roth, Hubert; Pison, Christophe

2015-08-01

Chronic respiratory failure (CRF) is the common fate of respiratory diseases where systemic effects contribute to outcomes. In a prospective cohort of home-treated patients with CRF, we looked for predictors of long-term survival including respiratory, nutritional and inflammatory dimensions. 637 stable outpatients with CRF, 397 men, 68 ± 11 years, on long-term oxygen therapy and/or non-invasive ventilation from 21 chest clinics were enrolled and followed over 53 ± 31 months. CRF resulted from Chronic Obstructive Pulmonary Disease (COPD) in 48.5%, restrictive disorders 32%, mixed (obstructive and restrictive patterns) respiratory failure 13.5%, bronchiectasis 6%. Demographic characteristics, smoking habits, underlying respiratory diseases, forced expiratory volume in one second (FEV1), forced vital capacity (FVC), arterial blood gases, 6-min walking distance (6MWD), hemoglobin, body mass index (BMI), serum albumin, transthyretin, C-reactive protein (CRP), history of respiratory assistance, antibiotic and oral corticosteroid use during the previous year were recorded. 322 deaths occurred during the follow-up. One-, five- and 8-year actuarial survival was 89%, 56% and 47%. By Cox univariate analysis, age, respiratory disease, PaO2, PaCO2, FEV1/FVC, BMI, 6MWD, activity score, type and length of home respiratory assistance, smoking habits, oral corticosteroid and antibiotic uses, albumin, transthyretin, hemoglobin and CRP levels were associated with survival. Multivariate analysis identified eight independent markers of survival: age, FEV1/FVC, PaO2, PaCO2, 6MWD, BMI, serum transthyretin, CRP ≥ 5 mg/l. In CRF, whatever the underlying diseases, besides the levels of obstructive ventilatory defect and gas exchange failure, 6MWD, BMI, serum transthyretin and CRP ≥ 5 mg/l predicted long-term survival identifying potential targets for nutritional rehabilitation. Copyright © 2014 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.

Pulmonary manifestations of anti-ARS antibody positive interstitial pneumonia--with or without PM/DM.

PubMed

Takato, Hazuki; Waseda, Yuko; Watanabe, Satoshi; Inuzuka, Kanako; Katayama, Nobuyuki; Ichikawa, Yukari; Yasui, Masahide; Fujimura, Masaki

2013-01-01

Autoantibodies against aminoacyl-tRNA synthetases (ARS) have been found to be highly specific for polymyositis and dermatomyositis (PM/DM) and to correlate strongly with complicating interstitial pneumonia (IP). The aim of the present study was to compare the clinical presentations of anti-ARS antibody-positive IP patients with or without manifestations of PM/DM. We retrospectively examined 36 IP patients with anti-ARS antibodies. Sixteen patients presented with and 20 without the features of PM/DM. They were divided into PM/DM-IP and idiopathic-IP (IIP) groups. Clinical symptoms, findings on physical examination, laboratory data, pulmonary function, computed tomography (CT), and bronchoalveolar lavage fluid (BALF) cell counts were compared. Skin findings, myalgia, and elevation of serum creatinine kinase were found in the PM/DM-IP group. Features common to both groups included: volume loss in lower bilateral lobes; ground-glass opacities, reticular shadows and traction bronchiectasis on chest CT; high percentage of lymphocytes (IIP: 44.0% ± 21.0% (mean ± SD), PM/DM-IP: 50.5% ± 23.5%) and low CD4/8 ratios (IIP: 0.36 ± 0.34, PM/DM-IP: 0.44 ± 0.42) in BALF; decreased pulmonary function, including percentage of predicted vital capacity (VC) (IIP: 80.1% ± 15.4%, PM/DM-IP: 73.6% ± 16.4%), residual volume (RV) (IIP: 70.7% ± 21.7%, PM/DM-IP: 71.5% ± 17.1%), total lung capacity (TLC) (IIP: 73.4% ± 13.6%, PM/DM-IP: 71.6% ± 13.0%), and diffusing capacity DLco (IIP: 57.5% ± 26.7%, PM/DM-IP: 46.4% ± 10.3%). Both groups achieved good responses to initial corticosteroid or immunosuppressant therapy. Patients with anti-ARS antibody-positive IP have common pulmonary manifestations regardless of the presence of PM/DM. Copyright © 2012. Published by Elsevier Ltd.

Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype.

PubMed

Knowles, Michael R; Ostrowski, Lawrence E; Leigh, Margaret W; Sears, Patrick R; Davis, Stephanie D; Wolf, Whitney E; Hazucha, Milan J; Carson, Johnny L; Olivier, Kenneth N; Sagel, Scott D; Rosenfeld, Margaret; Ferkol, Thomas W; Dell, Sharon D; Milla, Carlos E; Randell, Scott H; Yin, Weining; Sannuti, Aruna; Metjian, Hilda M; Noone, Peadar G; Noone, Peter J; Olson, Christina A; Patrone, Michael V; Dang, Hong; Lee, Hye-Seung; Hurd, Toby W; Gee, Heon Yung; Otto, Edgar A; Halbritter, Jan; Kohl, Stefan; Kircher, Martin; Krischer, Jeffrey; Bamshad, Michael J; Nickerson, Deborah A; Hildebrandt, Friedhelm; Shendure, Jay; Zariwala, Maimoona A

2014-03-15

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous recessive disorder of motile cilia, but the genetic cause is not defined for all patients with PCD. To identify disease-causing mutations in novel genes, we performed exome sequencing, follow-up characterization, mutation scanning, and genotype-phenotype studies in patients with PCD. Whole-exome sequencing was performed using NimbleGen capture and Illumina HiSeq sequencing. Sanger-based sequencing was used for mutation scanning, validation, and segregation analysis. We performed exome sequencing on an affected sib-pair with normal ultrastructure in more than 85% of cilia. A homozygous splice-site mutation was detected in RSPH1 in both siblings; parents were carriers. Screening RSPH1 in 413 unrelated probands, including 325 with PCD and 88 with idiopathic bronchiectasis, revealed biallelic loss-of-function mutations in nine additional probands. Five affected siblings of probands in RSPH1 families harbored the familial mutations. The 16 individuals with RSPH1 mutations had some features of PCD; however, nasal nitric oxide levels were higher than in patients with PCD with other gene mutations (98.3 vs. 20.7 nl/min; P < 0.0003). Additionally, individuals with RSPH1 mutations had a lower prevalence (8 of 16) of neonatal respiratory distress, and later onset of daily wet cough than typical for PCD, and better lung function (FEV1), compared with 75 age- and sex-matched PCD cases (73.0 vs. 61.8, FEV1 % predicted; P = 0.043). Cilia from individuals with RSPH1 mutations had normal beat frequency (6.1 ± Hz at 25°C), but an abnormal, circular beat pattern. The milder clinical disease and higher nasal nitric oxide in individuals with biallelic mutations in RSPH1 provides evidence of a unique genotype-phenotype relationship in PCD, and suggests that mutations in RSPH1 may be associated with residual ciliary function.

The characteristics of patients with pulmonary Mycobacterium avium-intracellulare complex disease diagnosed by bronchial lavage culture compared to those diagnosed by sputum culture.

PubMed

Maekawa, Koichi; Naka, Megumi; Shuto, Saki; Harada, Yuka; Ikegami, Yumiko

2017-09-01

The utility of bronchoscopy for the diagnosis of pulmonary Mycobacterium avium-intracellulare complex (MAC) disease has been reported; however, which patients require bronchoscopy remains unclear. Our objective was to identify the characteristics of the patients in whom bronchoscopy is needed for the diagnosis of MAC disease. Fifty-four patients with pulmonary MAC disease were divided into two groups according to established diagnostic criteria: 39 patients were diagnosed by sputum culture and 15 patients were diagnosed by bronchial lavage culture. We analysed the differences in demographic and clinical characteristics as well as microbiological and radiological data between the two groups. There were no significant differences in age, sex, smoking status, MAC species, underlying diseases, or steroid use. Significantly more patients diagnosed by sputum culture than bronchial lavage culture had a positive sputum smear for acid-fast bacilli (79.5% vs. 0.0%, respectively; p < 0.001) and any symptoms (75.3% vs. 46.2%, respectively; p = 0.0059). No significant differences were found in the prevalence of each computed tomography finding, including nodules, air-space disease, bronchiectasis, and cavities. However, more patients diagnosed by sputum culture than bronchial lavage culture had abnormalities in the left upper division (48.7% vs. 13.3%, respectively; p = 0.017) and higher numbers of affected lobes (4.3 ± 1.4 vs. 3.3 ± 1.6, respectively; p = 0.034). If patients suspected of having pulmonary MAC disease have a negative sputum smear, no symptoms, no abnormal findings in the left upper division, or fewer affected lobes on computed tomography, bronchoscopy might be needed for the diagnosis. Copyright © 2017 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Functional mechanism of lung mosaic CT attenuation: assessment with deep-inspiration breath-hold perfusion SPECT-CT fusion imaging and non-breath-hold Technegas SPECT.

PubMed

Suga, K; Yasuhiko, K; Iwanaga, H; Tokuda, O; Matsunaga, N

2009-01-01

The functional mechanism of lung mosaic computed tomography attenuation (MCA) in pulmonary vascular disease (PVD) and obstructive airway disease (OAD) has not yet been fully clarified. To clarify the mechanism of MCA in these diseases by assessing the relationship between regional lung function and CT attenuation change at MCA sites with the use of automated deep-inspiratory breath-hold (DIBrH) perfusion single-photon emission computed tomography (SPECT)-CT fusion images and non-breath-hold Technegas SPECT. Subjects were 42 PVD patients (31 pulmonary thromboembolism, four primary/two secondary pulmonary hypertension, and five Takayasu arteritis), 12 OAD patients (five acute asthma, four obliterative bronchiolitis, and three bronchiectasis), and 12 normal controls, all of whom had MCA on DIBrH CT. The relationship between regional lung function and CT attenuation change at the lung slices with MCA was assessed using DIBrH perfusion SPECT-CT fusion images and non-breath-hold Technegas SPECT. The severity of perfusion defects with or without MCA was quantified by regions-of-interest analysis. On DIBrH CT and perfusion SPECT, in contrast to no noticeable CT attenuation abnormality and fairly uniform perfusion in controls, 60 MCA and 274 perfusion defects in PVD patients, and 18 MCA and 61 defects in OAD patients were identified, with a total of 77 ventilation defects on Technegas SPECT in all patients. SPECT-CT correlation showed that, throughout the 78 MCA sites of all patients, lung perfusion was persistently decreased at low CT attenuation and preserved at intervening high CT attenuation, while lung ventilation was poorly correlated with CT attenuation change. The radioactivity ratios of reduced perfusion and the intervening preserved perfusion at the 78 perfusion defects with MCA were significantly lower than those at the remaining 257 defects without MCA (P<0.0001). Although further validation is required, our results indicate that heterogeneous pulmonary arterial perfusion may be a dominant mechanism of MCA in PVD and OAD.

Characteristics and self-rated health of overlap syndrome.

PubMed

Chung, Jung Wha; Kong, Kyoung Ae; Lee, Jin Hwa; Lee, Seok Jeong; Ryu, Yon Ju; Chang, Jung Hyun

2014-01-01

Overlap syndrome shares features of both asthma and chronic obstructive pulmonary disease (COPD). The aim of this study was to investigate characteristics of overlap syndrome and their effect on self-rated health (SRH). We analyzed data from the Fourth Korea National Health and Nutrition Examination Survey of 2007-2009. Subjects with acceptable spirometry and available wheezing history were included. Subjects were classified into four groups based on forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) results and the presence or absence of self-reported wheezing for the previous 12 months: 1) COPD group, defined as having FEV1/FVC <0.7 without self-reported wheezing history; 2) asthma group, defined as having self-reported wheezing history without FEV1/FVC <0.7; 3) overlap syndrome group, having both FEV1/FVC <0.7 and wheezing history; and 4) non-obstructive disease (NOD) group, having neither FEV1/FVC <0.7 nor self-reported wheezing. SRH was categorized as better or lower based on responses to a questionnaire. From a total 9,104 subjects, 700 were assigned to the COPD group, 560 to the asthma group, 210 to the overlap syndrome group, and 7,634 to the NOD group. Compared to the other groups, subjects in the overlap syndrome group were more likely to have low lung function, a high proportion of smokers, low socioeconomic status, short education duration, lower SRH, and past diagnosis of pulmonary tuberculosis or bronchiectasis. Multiple logistic regression analysis revealed that both overlap syndrome and asthma groups were independently associated with lower SRH after adjustment for age, sex, socioeconomic status, education level, smoking status, comorbidities, and lung function. Female, old age, low education level, low economic status, smoker and other comorbidities were also associated with lower SRH. Overlap syndrome was accompanied by high morbidity and was associated with lower SRH, which needs more appropriate care.

Historical perspective on effects and treatment of sulfur mustard injuries.

PubMed

Graham, John S; Schoneboom, Bruce A

2013-12-05

Sulfur mustard (2,2'-dichlorodiethyl sulfide; SM) is a potent vesicating chemical warfare agent that poses a continuing threat to both military and civilian populations. Significant SM injuries can take several months to heal, necessitate lengthy hospitalizations, and result in long-term complications affecting the skin, eyes, and lungs. This report summarizes initial and ongoing (chronic) clinical findings from SM casualties from the Iran-Iraq War (1980-1988), with an emphasis on cutaneous injury. In addition, we describe the cutaneous manifestations and treatment of several men recently and accidentally exposed to SM in the United States. Common, chronic cutaneous problems being reported in the Iranian casualties include pruritis (the primary complaint), burning, pain, redness, desquamation, hyperpigmentation, hypopigmentation, erythematous papular rash, xerosis, multiple cherry angiomas, atrophy, dermal scarring, hypertrophy, and sensitivity to mechanical injury with recurrent blistering and ulceration. Chronic ocular problems include keratitis, photophobia, persistent tearing, sensation of foreign body, corneal thinning and ulceration, vasculitis of the cornea and conjunctiva, and limbal stem cell deficiency. Chronic pulmonary problems include decreases in lung function, bronchitis with hyper-reactive airways, bronchiolitis, bronchiectasis, stenosis of the trachea and other large airways, emphysema, pulmonary fibrosis, decreased total lung capacity, and increased incidences of lung cancer, pulmonary infections, and tuberculosis. There are currently no standardized or optimized methods of casualty management; current treatment strategy consists of symptomatic management and is designed to relieve symptoms, prevent infections, and promote healing. New strategies are needed to provide for optimal and rapid healing, with the goals of (a) returning damaged tissue to optimal appearance and normal function in the shortest period of time, and (b) ameliorating chronic effects. Further experimental research and clinical trials will be needed to prevent or mitigate the acute clinical effects of SM exposure and to reduce or eliminate the long-term manifestations. Published by Elsevier Ireland Ltd.

Respiratory risks from household air pollution in low and middle income countries

PubMed Central

Gordon, Stephen B; Bruce, Nigel G; Grigg, Jonathan; Hibberd, Patricia L; Kurmi, Om P; Lam, Kin-bong Hubert; Mortimer, Kevin; Asante, Kwaku Poku; Balakrishnan, Kalpana; Balmes, John; Bar-Zeev, Naor; Bates, Michael N; Breysse, Patrick N; Buist, Sonia; Chen, Zhengming; Havens, Deborah; Jack, Darby; Jindal, Surinder; Kan, Haidong; Mehta, Sumi; Moschovis, Peter; Naeher, Luke; Patel, Archana; Perez-Padilla, Rogelio; Pope, Daniel; Rylance, Jamie; Semple, Sean; Martin, William J

2016-01-01

A third of the world’s population uses solid fuel derived from plant material (biomass) or coal for cooking, heating, or lighting. These fuels are smoky, often used in an open fire or simple stove with incomplete combustion, and result in a large amount of household air pollution when smoke is poorly vented. Air pollution is the biggest environmental cause of death worldwide, with household air pollution accounting for about 3·5–4 million deaths every year. Women and children living in severe poverty have the greatest exposures to household air pollution. In this Commission, we review evidence for the association between household air pollution and respiratory infections, respiratory tract cancers, and chronic lung diseases. Respiratory infections (comprising both upper and lower respiratory tract infections with viruses, bacteria, and mycobacteria) have all been associated with exposure to household air pollution. Respiratory tract cancers, including both nasopharyngeal cancer and lung cancer, are strongly associated with pollution from coal burning and further data are needed about other solid fuels. Chronic lung diseases, including chronic obstructive pulmonary disease and bronchiectasis in women, are associated with solid fuel use for cooking, and the damaging effects of exposure to household air pollution in early life on lung development are yet to be fully described. We also review appropriate ways to measure exposure to household air pollution, as well as study design issues and potential effective interventions to prevent these disease burdens. Measurement of household air pollution needs individual, rather than fixed in place, monitoring because exposure varies by age, gender, location, and household role. Women and children are particularly susceptible to the toxic effects of pollution and are exposed to the highest concentrations. Interventions should target these high-risk groups and be of sufficient quality to make the air clean. To make clean energy available to all people is the long-term goal, with an intermediate solution being to make available energy that is clean enough to have a health impact. PMID:25193349

Diagnosis of Cystic Fibrosis in Nonscreened Populations.

PubMed

Sosnay, Patrick R; White, Terry B; Farrell, Philip M; Ren, Clement L; Derichs, Nico; Howenstine, Michelle S; Nick, Jerry A; De Boeck, Kris

2017-02-01

Although the majority of cases of cystic fibrosis (CF) are now diagnosed through newborn screening, there is still a need to standardize the diagnostic criteria for those diagnosed outside of the neonatal period. This is because newborn screening started relatively recently, it is not performed everywhere, and even for individuals who were screened, there is the possibility of a false negative. To limit irreversible organ pathology, a timely diagnosis of CF and institution of CF therapies can greatly benefit these patients. Experts on CF diagnosis were convened at the 2015 CF Foundation Diagnosis Consensus Conference. The participants reviewed and discussed published works and instructive cases of CF diagnosis in individuals presenting with signs, symptoms, or a family history of CF. Through a modified Delphi methodology, several consensus statements were agreed upon. These consensus statements were updates of prior CF diagnosis conferences and recommendations. CF diagnosis in individuals outside of newborn screening relies on the clinical evidence and on evidence of CF transmembrane conductance regulator (CFTR) dysfunction. Clinical evidence can include typical organ pathologies seen in CF such as bronchiectasis or pancreatic insufficiency but often represent a broad range of severity including mild cases. CFTR dysfunction can be demonstrated using sweat chloride testing, CFTR molecular genetic analysis, or CFTR physiologic tests. On the basis of the large number of patients with bona fide CF currently followed in registries with sweat chloride levels between 30 and 40 mmol/L, the threshold considered "intermediate" was lowered from 40 mmol/L in the prior diagnostic guidelines to 30 mmol/L. The CF diagnosis was also discussed in the context of CFTR-related disorders in which CFTR dysfunction may be present, but the individual does not meet criteria for CF. CF diagnosis remains a rare but important condition that can be diagnosed when characteristic clinical features are seen in an individual with demonstrated CFTR dysfunction. Copyright © 2016 Elsevier Inc. All rights reserved.

Modeling breath-enhanced jet nebulizers to estimate pulmonary drug deposition.

PubMed

Wee, Wallace B; Leung, Kitty; Coates, Allan L

2013-12-01

Predictable delivery of aerosol medication for a given patient and drug-device combination is crucial, both for therapeutic effect and to avoid toxicity. The gold standard for measuring pulmonary drug deposition (PDD) is gamma scintigraphy. However, these techniques expose patients to radiation, are complicated, and are relevant for only one patient and drug-device combination, making them less available. Alternatively, in vitro experiments have been used as a surrogate to estimate in vivo performance, but this is time-consuming and has few "in vitro to in vivo" correlations for therapeutics delivered by inhalation. An alternative method for determining inhaled mass and PDD is proposed by deriving and validating a mathematical model, for the individual breathing patterns of normal subjects and drug-device operating parameters. This model was evaluated for patients with cystic fibrosis (CF). This study is comprised of three stages: mathematical model derivation, in vitro testing, and in vivo validation. The model was derived from an idealized patient's respiration cycle and the steady-state operating characteristics of a drug-device combination. The model was tested under in vitro dynamic conditions that varied tidal volume, inspiration-to-expiration time, and breaths per minute. This approach was then extended to incorporate additional physiological parameters (dead space, aerodynamic particle size distribution) and validated against in vivo nuclear medicine data in predicting PDD in both normal subjects and those with CF. The model shows strong agreement with in vitro testing. In vivo testing with normal subjects yielded good agreement, but less agreement for patients with chronic obstructive lung disease and bronchiectasis from CF. The mathematical model was successful in accommodating a wide range of breathing patterns and drug-device combinations. Furthermore, the model has demonstrated its effectiveness in predicting the amount of aerosol delivered to "normal" subjects. However, challenges remain in predicting deposition in obstructive lung disease.

Longitudinal evaluation of bronchopulmonary disease in children with cystic fibrosis.

PubMed

Farrell, Philip M; Li, Zhanhai; Kosorok, Michael R; Laxova, Anita; Green, Christopher G; Collins, Jannette; Lai, Hui-Chuan; Makholm, Linda M; Rock, Michael J; Splaingard, Mark L

2003-09-01

Children with cystic fibrosis (CF) develop bronchopulmonary disease at variable ages. Determining the epidemiology of chronic lung disease and quantifying its severity, however, have been difficult in infants and young children. As part of the Wisconsin CF Neonatal Screening Project, we were presented with an ideal opportunity to assess longitudinally the evolution of symptoms, signs, and quantitative measures of CF respiratory disease. After newborn screening test results led to early recognition, 64 patients diagnosed at a median age of 6.71 weeks were enrolled and studied systematically at a median age of 11.3 years to obtain clinical information, chest radiographs, and pulmonary function tests. Our observations revealed that a frequent cough by history is evident by 10.5 months of age in half the patients. Quantitative chest radiology (CXR scoring) demonstrated that potentially irreversible abnormalities are present in half the children by 2 years. The severity of Wisconsin and Brasfield CXR scores increased in association with respiratory infections. Longitudinal progression of Wisconsin CXR scores was related to age (P < 0.001), pancreatic insufficiency (P = 0.005), and respiratory secretion cultures positive for Staphylococus aureas (P = 0.039). In contrast, serial spirometry showed limited sensitivity, as did lung volume determinations; neither was satisfactory as repeated measures with acceptable quality control until after 7 years of age. Time to event analyses revealed that half the patients had % predicted FEF(25-75) and FEV(1)/FVC values greater than 80% until 10.7 and 9.9 years, respectively. We conclude that of the methods evaluated, quantitative chest radiology is currently the best procedure for frequent assessment of bronchopulmonary disease in CF, and that radiographic progression is evident in approximately 85% of patients by 5 years of age. Our results also suggest that bronchiectasis and other radiographic evidence of chronic infection are apparent prior to airways obstruction in young CF patients. Copyright 2003 Wiley-Liss, Inc.

[Assessment of complications in patients with lung transplantation with high resolution computerized tomography].

PubMed

Macori, F; Iacari, V; Falchetto Osti, M; Potente, G; Anaveri, G

1998-01-01

High Resolution Computed Tomography (HRCT) has been used by many authors to study the early complications of lung transplantation. Bronchoscopy, transbronchial biopsy and the clinical parameters are the tools of choice to diagnose such complications; HRCT showed excellent sensitivity (100%) and good specificity (93%) especially in detecting bronchial stenoses. We report the preliminary results of HRCT in detecting early/late complications in lung transplant recipients. Sixteen lung transplant recipients (5 single and 11 double transplants) were examined with HRCT at the Servizio Speciale Diagnostica V of "La Sapienza" University (Rome, Italy). The CT findings were compared with the results of bronchoscopy and respiratory function tests. The patients (8 men and 8 women; age range: 18-57 years, mean: 37.5) had cystic fibrosis (9), emphysema (3), alpha-1-antitrypsin deficiency (1), idiopathic pulmonary fibrosis (2), and bronchiectasis (1). During the follow-up, one patient died of pulmonary edema. CT findings were normal in 3 patients and mild pleural effusion was seen in 2. The other HRCT findings were: bronchial stenosis in 5 cases (which was bilateral in 1) and bronchial dehiscence in 1 patient; four cases of infection (1 CMV, 1 aspecific bacterial pneumonia, 1 Chlamydia psittacea and 1 Aspergillosis) and one of brochiolitis obliterans. A patient was treated for acute and one for chronic rejection. A CMV infection involved only the native lung in a patient. CT is easy to perform and a repeatable and well-tolerated tool with high sensitivity (100%) and good specificity (93%) in the early diagnosis of complications, particularly bronchial stenoses, which complications are often missed at bronchoscopy or clinically silent. CT should be always performed before bronchoscopy because it can provide valuable information for bronchoscopy targeting. In agreement with other authors we consider HRCT a very useful tool in the early diagnosis of the complications following lung transplantation.

Comorbidities in adults with asthma: Population-based cross-sectional analysis of 1.4 million adults in Scotland.

PubMed

Weatherburn, C J; Guthrie, B; Mercer, S W; Morales, D R

2017-10-01

Comorbidity in people with asthma can significantly increase asthma morbidity and lower adherence to asthma guidelines. The objective of this study was to comprehensively measure the prevalence of physical and mental health comorbidities in adults with asthma using a large nationally representative population. Cross-sectional analysis of routine primary care electronic medical records for 1 424 378 adults in the UK, examining the prevalence of 39 comorbidities in people with and without asthma, before and after adjustment for age, sex, social deprivation and smoking status using logistic regression. Of 39 comorbidities measured, 36 (92%) were significantly more common in adults with asthma; 62.6% of adults with asthma had ≥1 comorbidity vs 46.2% of those without, and 16.3% had ≥4 comorbidities vs 8.7% of those without. Comorbidities with the largest absolute increase in prevalence in adults with asthma were as follows: chronic obstructive pulmonary disease (COPD) (13.4% vs 3.1%), depression (17.3% vs 9.1%), painful conditions (15.4% vs 8.4%) and dyspepsia (10.9% vs 5.2%). Comorbidities with the largest relative difference in adults with asthma compared to those without were as follows: COPD (adjusted odds ratio [aOR] 5.65, 95% CI 5.52-5.79), bronchiectasis (aOR 4.65, 95% CI 4.26-5.08), eczema/psoriasis (aOR 3.30, 95% CI 3.14-3.48), dyspepsia (aOR 2.20, 95% CI 2.15-2.25) and chronic sinusitis (aOR 2.12, 95% CI 1.99-2.26). Depression and anxiety were more common in adults with asthma (aOR 1.60, 95% CI 1.57-1.63, and aOR 1.53, 95% CI 1.48-1.57, respectively). Physical and mental health comorbidities are the norm in adults with asthma. Appropriate recognition and management should form part of routine asthma care. © 2017 John Wiley & Sons Ltd.

Pneumothorax associated with nontuberculous mycobacteria

PubMed Central

Ueyama, M; Asakura, Takanori; Morimoto, Kozo; Namkoong, Ho; Matsuda, Shuichi; Osawa, Takeshi; Ishii, Makoto; Hasegawa, Naoki; Kurashima, Atsuyuki; Goto, Hajime

2016-01-01

Abstract The incidence of nontuberculous mycobacterial pulmonary disease (NTMPD) is increasing worldwide. Secondary spontaneous pneumothorax occurs as a complication of underlying lung disease and is associated with higher morbidity, mortality, and recurrence than primary spontaneous pneumothorax. We here investigated the clinical features and long-term outcomes of pneumothorax associated with NTMPD. We conducted a retrospective study on consecutive adult patients with pneumothorax associated with NTMPD at Fukujuji Hospital and Keio University Hospital from January 1992 to December 2013. We reviewed the medical records of 69 such patients to obtain clinical characteristics, radiological findings, and long-term outcomes, including pneumothorax recurrence and mortality. The median age of the patients was 68 years; 34 patients were women. The median body mass index was 16.8 kg/m2. Underlying pulmonary diseases mainly included chronic obstructive pulmonary disease and pulmonary tuberculosis. On computed tomography, nodules and bronchiectasis were observed in 46 (98%) and 45 (96%) patients, respectively. Consolidation, pleural thickening, interlobular septal thickening, and cavities were most common, and observed in 40 (85%), 40 (85%), 37 (79%), and 36 (77%) patients, respectively. Regarding pneumothorax treatment outcomes, complete and incomplete lung expansion were observed in 49 patients (71%) and 15 patients (22%), respectively. The survival rate after pneumothorax was 48% at 5 years. By the end of the follow-up, 33 patients had died, and the median survival was 4.4 years with a median follow-up period of 1.7 years. The rate of absence of recurrence after the first pneumothorax was 59% at 3 years. By the end of the follow-up, 18 patients had experienced pneumothorax recurrence. Furthermore, 12/18 patients (66%) with recurrent pneumothorax died during the study period. Twenty-three patients (70%) died because of NTMPD progression. Low body mass index (BMI) was a negative prognostic factor for pneumothorax associated with NTMPD in multivariate analysis (HR 0.79, 95% CI 0.64−0.96; P = 0.018) Patients with pneumothorax associated with NTMPD have advanced disease, a high rate of pneumothorax recurrence, and poor prognosis, regardless of the pneumothorax treatment used. Further improvements in early diagnosis of NTMPD and appropriate management in both NTMPD and NTMPD-associated pneumothorax are needed. PMID:27442650

Pneumothorax associated with nontuberculous mycobacteria: A retrospective study of 69 patients.

PubMed

Ueyama, Masako; Asakura, Takanori; Morimoto, Kozo; Namkoong, Ho; Matsuda, Shuichi; Osawa, Takeshi; Ishii, Makoto; Hasegawa, Naoki; Kurashima, Atsuyuki; Goto, Hajime

2016-07-01

The incidence of nontuberculous mycobacterial pulmonary disease (NTMPD) is increasing worldwide. Secondary spontaneous pneumothorax occurs as a complication of underlying lung disease and is associated with higher morbidity, mortality, and recurrence than primary spontaneous pneumothorax. We here investigated the clinical features and long-term outcomes of pneumothorax associated with NTMPD.We conducted a retrospective study on consecutive adult patients with pneumothorax associated with NTMPD at Fukujuji Hospital and Keio University Hospital from January 1992 to December 2013. We reviewed the medical records of 69 such patients to obtain clinical characteristics, radiological findings, and long-term outcomes, including pneumothorax recurrence and mortality.The median age of the patients was 68 years; 34 patients were women. The median body mass index was 16.8 kg/m. Underlying pulmonary diseases mainly included chronic obstructive pulmonary disease and pulmonary tuberculosis. On computed tomography, nodules and bronchiectasis were observed in 46 (98%) and 45 (96%) patients, respectively. Consolidation, pleural thickening, interlobular septal thickening, and cavities were most common, and observed in 40 (85%), 40 (85%), 37 (79%), and 36 (77%) patients, respectively. Regarding pneumothorax treatment outcomes, complete and incomplete lung expansion were observed in 49 patients (71%) and 15 patients (22%), respectively. The survival rate after pneumothorax was 48% at 5 years. By the end of the follow-up, 33 patients had died, and the median survival was 4.4 years with a median follow-up period of 1.7 years. The rate of absence of recurrence after the first pneumothorax was 59% at 3 years. By the end of the follow-up, 18 patients had experienced pneumothorax recurrence. Furthermore, 12/18 patients (66%) with recurrent pneumothorax died during the study period. Twenty-three patients (70%) died because of NTMPD progression. Low body mass index (BMI) was a negative prognostic factor for pneumothorax associated with NTMPD in multivariate analysis (HR 0.79, 95% CI 0.64-0.96; P = 0.018)Patients with pneumothorax associated with NTMPD have advanced disease, a high rate of pneumothorax recurrence, and poor prognosis, regardless of the pneumothorax treatment used. Further improvements in early diagnosis of NTMPD and appropriate management in both NTMPD and NTMPD-associated pneumothorax are needed.

Profile of renal AA amyloidosis in older and younger individuals: a single-centre experience.

PubMed

Erdogmus, Siyar; Kendi Celebi, Zeynep; Akturk, Serkan; Kumru, Gizem; Duman, Neval; Ates, Kenan; Erturk, Sehsuvar; Nergizoglu, Gokhan; Kutlay, Sim; Sengul, Sule; Keven, Kenan

2018-05-18

In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis. This is a retrospective study involving 53 patients who were diagnosed with AA amyloidosis by kidney biopsy from 2006 to 2016. In all patients, kidney biopsies were performed due to asymptomatic proteinuria, nephrotic syndrome and/or renal insufficiency. The patients were separated into two groups on the basis of age (group I: ≥60 years and group II: <60 years). Outcomes of patients in terms of the requirement of renal replacement therapy and mortality were recorded. In patients with group I, the causes of AA amyloidosis were as follows: FMF 16 (50%), bronchiectasis 7 (23%), chronic osteomyelitis 2 (6%), inflammatory bowel disease 2 (6%), rheumatoid arthritis 2 (6%), ankylosing spondylitis 1 (3%) and unknown aetiology 2 (6%). The underlying disorders of AA amyloidosis in group II patients were as follows: FMF 17 (81%), Behcet's disease 1 (5%) and unknown aetiology 3 (14%). No statistically significant differences were detected between two groups with regard to systolic and diastolic blood pressures, albumin, proteinuria and lipids. The combination of chronic kidney disease and nephrotic syndrome was the most common clinical presentation in group I (73%) and group II (43%) (p = .05). Compared to the group II, estimated glomerular filtration rate was significantly lower in group I at the time of kidney biopsy (p = .003). At 12-month follow-up, 61% of the group I and 33% of the group II developed end-stage kidney disease requiring dialysis, while 11% of the group I died. Our results indicated that renal AA amyloidosis is a rare disease in advanced age patients. At baseline and follow-up period, advanced age patients had worse kidney disease and outcomes.