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Sample records for pulmonary disease consistent

  1. Chronic obstructive pulmonary disease

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/000091.htm Chronic obstructive pulmonary disease To use the sharing features on this page, please enable JavaScript. Chronic obstructive pulmonary disease (COPD) is a common lung disease. Having COPD ...

  2. Pulmonary vascular diseases.

    PubMed

    Mélot, C; Naeije, R

    2011-04-01

    Diseases of the pulmonary vasculature are a cause of increased pulmonary vascular resistance (PVR) in pulmonary embolism, chronic thromboembolic pulmonary hypertension (CTEPH), and pulmonary arterial hypertension or decreased PVR in pulmonary arteriovenous malformations on hereditary hemorrhagic telangiectasia, portal hypertension, or cavopulmonary anastomosis. All these conditions are associated with a decrease in both arterial PO2 and PCO2. Gas exchange in pulmonary vascular diseases with increased PVR is characterized by a shift of ventilation and perfusion to high ventilation-perfusion ratios, a mild to moderate increase in perfusion to low ventilation-perfusion ratios, and an increased physiologic dead space. Hypoxemia in these patients is essentially explained by altered ventilation-perfusion matching amplified by a decreased mixed venous PO2 caused by a low cardiac output. Hypocapnia is accounted for by hyperventilation, which is essentially related to an increased chemosensitivity. A cardiac shunt on a patent foramen ovale may be a cause of severe hypoxemia in a proportion of patients with pulmonary hypertension and an increase in right atrial pressure. Gas exchange in pulmonary arteriovenous malformations is characterized by variable degree of pulmonary shunting and/or diffusion-perfusion imbalance. Hypocapnia is caused by an increased ventilation in relation to an increased pulmonary blood flow with direct peripheral chemoreceptor stimulation by shunted mixed venous blood flow. PMID:23737196

  3. Pulmonary rehabilitation in chronic obstructive pulmonary disease.

    PubMed

    Saey, D; Bernard, S; Gagnon, P; Laviolette, L; Soicher, J; Maltais, F; Esgagne, P; Coats, V; Devost, A-A

    2009-06-01

    Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality worldwide and an important worldwide cause of disability and handicap. Centered around exercise training, pulmonary rehabilitation is a global, multidisciplinary, individualized and comprehensive approach acting on the patient as a whole and not only on the pulmonary component of the disease. Pulmonary rehabilitation is now well recognized as an effective and key intervention in the management of several respiratory diseases particularly in COPD. Modern and effective pulmonary rehabilitation programs are global, multidisciplinary, individualized and use comprehensive approach acting on the patient as a whole and not only on the pulmonary component of the disease. In the last two decades interest for pulmonary rehabilitation is on the rise and a growing literature including several guidelines is now available. This review addresses the recent developments in the broad area of pulmonary rehabilitation as well as new methods to consider in the development of future and better programs. Modern literature for rationale, physiopathological basis, structure, exercise training as well challenges for pulmonary rehabilitation programs are addressed. Among the main challenges of pulmonary rehabilitation, efforts have to be devoted to improve accessibility to early rehabilitation strategies, not only to patients with COPD but to those with other chronic respiratory diseases. PMID:19776711

  4. Chronic Obstructive Pulmonary Disease (COPD)

    MedlinePlus

    Chronic Obstructive Pulmonary Disease (COPD) Chronic Obstructive Pulmonary Disease (COPD) is a preventable and treatable disease that makes it difficult to empty air out of the lungs. This difficulty in ...

  5. Chronic Obstructive Pulmonary Disease.

    PubMed

    Hattab, Yousef; Alhassan, Sulaiman; Balaan, Marvin; Lega, Mark; Singh, Anil C

    2016-01-01

    Chronic obstructive pulmonary disease (COPD) is a chronic smoking-related lung disease associated with significant mortality and morbidity. It carries an enormous economic burden on the health care system. This results in a significant social impact on affected patients and their families. In this article, we review COPD in general, critical care management of patients presenting with acute exacerbation of COPD, and methods of prevention. PMID:26919673

  6. [Nontuberculous mycobacterial pulmonary disease].

    PubMed

    Ringshausen, F C; Rademacher, J

    2016-02-01

    Nontuberculous mycobacteria (NTM) are a group of biologically diverse, ubiquitous and naturally multi-drug resistant bacteria with facultative pathogenicity. Recent data suggest that their clinical significance is increasing worldwide and that susceptible individuals may be at risk for infection via contaminated surfaces and aerosols. These individuals often have a predisposition for chronic respiratory diseases, e. g. bronchiectasis, chronic obstructive pulmonary disease (COPD) and cystic fibrosis and these conditions frequently share the same unspecific signs and symptoms with NTM pulmonary disease (NTM-PD). As a consequence, the diagnosis of NTM-PD, which is established based on clinical, radiological and microbiological criteria, is often delayed. Treating NTM-PD is more demanding than treating pulmonary tuberculosis as therapy is generally more tedious, toxic and expensive as well as being prone to failure. Patient and pathogen-specific factors guide the choice of an appropriate antimicrobial combination regimen, which should comply with national and international recommendations. Adverse events are common, should be anticipated and closely monitored. If infections with infrequently encountered mycobacterial species and severe or refractory disease occur, an interdisciplinary approach should be used, involving infectious disease specialists, experienced thoracic surgeons and referral to an NTM specialist center. PMID:26810111

  7. Drug-induced pulmonary disease

    MedlinePlus

    ... improve. Some drug-induced lung diseases, such as pulmonary fibrosis, may never go away. ... Complications that may develop include: Diffuse interstitial pulmonary fibrosis Hypoxemia (low blood oxygen) Respiratory failure

  8. Pulmonary veno-occlusive disease

    MedlinePlus

    ... occlusive disease is an extremely rare disease that causes high blood pressure in the lung arteries ( pulmonary hypertension ). ... In most cases, the cause of pulmonary veno-occlusive disease is ... arteries directly connected to the right side of the heart. The ...

  9. Chronic obstructive pulmonary disease.

    PubMed

    Barnes, Peter J; Burney, Peter G J; Silverman, Edwin K; Celli, Bartolome R; Vestbo, Jørgen; Wedzicha, Jadwiga A; Wouters, Emiel F M

    2015-01-01

    Chronic obstructive pulmonary disease (COPD) is a common disease with high global morbidity and mortality. COPD is characterized by poorly reversible airway obstruction, which is confirmed by spirometry, and includes obstruction of the small airways (chronic obstructive bronchiolitis) and emphysema, which lead to air trapping and shortness of breath in response to physical exertion. The most common risk factor for the development of COPD is cigarette smoking, but other environmental factors, such as exposure to indoor air pollutants - especially in developing countries - might influence COPD risk. Not all smokers develop COPD and the reasons for disease susceptibility in these individuals have not been fully elucidated. Although the mechanisms underlying COPD remain poorly understood, the disease is associated with chronic inflammation that is usually corticosteroid resistant. In addition, COPD involves accelerated ageing of the lungs and an abnormal repair mechanism that might be driven by oxidative stress. Acute exacerbations, which are mainly triggered by viral or bacterial infections, are important as they are linked to a poor prognosis. The mainstay of the management of stable disease is the use of inhaled long-acting bronchodilators, whereas corticosteroids are beneficial primarily in patients who have coexisting features of asthma, such as eosinophilic inflammation and more reversibility of airway obstruction. Apart from smoking cessation, no treatments reduce disease progression. More research is needed to better understand disease mechanisms and to develop new treatments that reduce disease activity and progression. PMID:27189863

  10. Cavitary Pulmonary Disease

    PubMed Central

    Gadkowski, L. Beth; Stout, Jason E.

    2008-01-01

    Summary: A pulmonary cavity is a gas-filled area of the lung in the center of a nodule or area of consolidation and may be clinically observed by use of plain chest radiography or computed tomography. Cavities are present in a wide variety of infectious and noninfectious processes. This review discusses the differential diagnosis of pathological processes associated with lung cavities, focusing on infections associated with lung cavities. The goal is to provide the clinician and clinical microbiologist with an overview of the diseases most commonly associated with lung cavities, with attention to the epidemiology and clinical characteristics of the host. PMID:18400799

  11. Pulmonary veno-occlusive disease

    MedlinePlus

    ... disorder. Alternative Names Pulmonary vaso-occlusive disease Images Respiratory system References Channick RN, Rubin LJ. Pulmonary hypertension. In: Mason RJ, Broaddus VC, Martin TR, et al., eds. Murray and Nadel's Textbook of Respiratory Medicine . 5th ed. Philadelphia, PA: Elsevier Saunders; 2010: ...

  12. Diseases of Pulmonary Surfactant Homeostasis

    PubMed Central

    Whitsett, Jeffrey A.; Wert, Susan E.; Weaver, Timothy E.

    2015-01-01

    Advances in physiology and biochemistry have provided fundamental insights into the role of pulmonary surfactant in the pathogenesis and treatment of preterm infants with respiratory distress syndrome. Identification of the surfactant proteins, lipid transporters, and transcriptional networks regulating their expression has provided the tools and insights needed to discern the molecular and cellular processes regulating the production and function of pulmonary surfactant prior to and after birth. Mutations in genes regulating surfactant homeostasis have been associated with severe lung disease in neonates and older infants. Biophysical and transgenic mouse models have provided insight into the mechanisms underlying surfactant protein and alveolar homeostasis. These studies have provided the framework for understanding the structure and function of pulmonary surfactant, which has informed understanding of the pathogenesis of diverse pulmonary disorders previously considered idiopathic. This review considers the pulmonary surfactant system and the genetic causes of acute and chronic lung disease caused by disruption of alveolar homeostasis. PMID:25621661

  13. Diseases of pulmonary surfactant homeostasis.

    PubMed

    Whitsett, Jeffrey A; Wert, Susan E; Weaver, Timothy E

    2015-01-01

    Advances in physiology and biochemistry have provided fundamental insights into the role of pulmonary surfactant in the pathogenesis and treatment of preterm infants with respiratory distress syndrome. Identification of the surfactant proteins, lipid transporters, and transcriptional networks regulating their expression has provided the tools and insights needed to discern the molecular and cellular processes regulating the production and function of pulmonary surfactant prior to and after birth. Mutations in genes regulating surfactant homeostasis have been associated with severe lung disease in neonates and older infants. Biophysical and transgenic mouse models have provided insight into the mechanisms underlying surfactant protein and alveolar homeostasis. These studies have provided the framework for understanding the structure and function of pulmonary surfactant, which has informed understanding of the pathogenesis of diverse pulmonary disorders previously considered idiopathic. This review considers the pulmonary surfactant system and the genetic causes of acute and chronic lung disease caused by disruption of alveolar homeostasis. PMID:25621661

  14. Pulmonary veno-occlusive disease.

    PubMed

    Montani, David; Lau, Edmund M; Dorfmüller, Peter; Girerd, Barbara; Jaïs, Xavier; Savale, Laurent; Perros, Frederic; Nossent, Esther; Garcia, Gilles; Parent, Florence; Fadel, Elie; Soubrier, Florent; Sitbon, Olivier; Simonneau, Gérald; Humbert, Marc

    2016-05-01

    Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH) characterised by preferential remodelling of the pulmonary venules. In the current PH classification, PVOD and pulmonary capillary haemangiomatosis (PCH) are considered to be a common entity and represent varied expressions of the same disease. The recent discovery of biallelic mutations in the EIF2AK4 gene as the cause of heritable PVOD/PCH represents a major milestone in our understanding of the molecular pathogenesis of PVOD. Although PVOD and pulmonary arterial hypertension (PAH) share a similar clinical presentation, with features of severe precapillary PH, it is important to differentiate these two conditions as PVOD carries a worse prognosis and life-threatening pulmonary oedema may occur following the initiation of PAH therapy. An accurate diagnosis of PVOD based on noninvasive investigations is possible utilising oxygen parameters, low diffusing capacity for carbon monoxide and characteristic signs on high-resolution computed tomography of the chest. No evidence-based medical therapy exists for PVOD at present and lung transplantation remains the preferred definitive therapy for eligible patients. PMID:27009171

  15. Celiac disease with pulmonary haemosiderosis and cardiomyopathy.

    PubMed

    Işikay, Sedat; Yilmaz, Kutluhan; Kilinç, Metin

    2012-01-01

    Celiac disease or pulmonary haemosiderosis can be associated with several distinguished conditions. Pulmonary haemosiderosis is a rare, severe and fatal disease characterised by recurrent episodes of alveolar haemorrhage, haemoptysis and anaemia. Association of pulmonary haemosiderosis and celiac disease is extremely rare. We describe a case of celiac disease presented with dilated cardiomyopathy and pulmonary haemosiderosis without gastrointestinal symptoms of celiac disease. In addition, vitamin A deficiency was detected. This case suggests that celiac disease should be considered in patients with cardiomyopathy and/or pulmonary haemosiderosis regardless of the intestinal symptoms of celiac disease. PMID:23169927

  16. Pulmonary complications of hepatic diseases

    PubMed Central

    Surani, Salim R; Mendez, Yamely; Anjum, Humayun; Varon, Joseph

    2016-01-01

    Severe chronic liver disease (CLD) may result from portal hypertension, hepatocellular failure or the combination of both. Some of these patients may develop pulmonary complications independent from any pulmonary pathology that they may have. Among them the hepatopulmonary syndrome (HPS), portopulmonary hypertension (PPH) and hepatic hydrothorax (HH) are described in detail in this literature review. HPS is encountered in approximately 15% to 30% of the patients and its presence is associated with increase in mortality and also requires liver transplantation in many cases. PPH has been reported among 4%-8% of the patient with CLD who have undergone liver transplantation. The HH is another entity, which has the prevalence rate of 5% to 6% and is associated in the absence of cardiopulmonary disease. These clinical syndromes occur in similar pathophysiologic environments. Most treatment modalities work as temporizing measures. The ultimate treatment of choice is liver transplant. This clinical review provides basic concepts; pathophysiology and clinical presentation that will allow the clinician to better understand these potentially life-threatening complications. This article will review up-to-date information on the pathophysiology, clinical features and the treatment of the pulmonary complications among liver disease patients. PMID:27468192

  17. Chronic obstructive pulmonary disease.

    PubMed

    Brusasco, Vito; Martinez, Fernando

    2014-01-01

    COPD is characterized by airflow limitation that is not fully reversible. The morphological basis for airflow obstruction results from a varying combination of obstructive changes in peripheral conducting airways and destructive changes in respiratory bronchioles, alveolar ducts, and alveoli. A reduction of vascularity within the alveolar septa has been reported in emphysema. Typical physiological changes reflect these structural abnormalities. Spirometry documents airflow obstruction when the FEV1/FVC ratio is reduced below the lower limit of normality, although in early disease stages FEV1 and airway conductance are not affected. Current guidelines recommend testing for bronchoreversibility at least once and the postbronchodilator FEV1/FVC be used for COPD diagnosis; the nature of bronchodilator response remains controversial, however. One major functional consequence of altered lung mechanics is lung hyperinflation. FRC may increase as a result of static or dynamic mechanisms, or both. The link between dynamic lung hyperinflation and expiratory flow limitation during tidal breathing has been demonstrated. Hyperinflation may increase the load on inspiratory muscles, with resulting length adaptation of diaphragm. Reduction of exercise tolerance is frequently noted, with compelling evidence that breathlessness and altered lung mechanics play a major role. Lung function measurements have been traditionally used as prognostic indices and to monitor disease progression; FEV1 has been most widely used. An increase in FVC is also considered as proof of bronchodilatation. Decades of work has provided insight into the histological, functional, and biological features of COPD. This has provided a clearer understanding of important pathobiological processes and has provided additional therapeutic options. PMID:24692133

  18. [Tobacco and morphology: pulmonary diseases].

    PubMed

    Carvalho, Lina

    2007-01-01

    Tobacco is implicated in multisystemic carcinogenesis through more than fifty identified carcinogenic metabolites that produce mutations responsible for alterations in cell cycle, immune response and endocrine regulation. Is one of nine risk factors identified in one third of cancer deaths together with obesity, sedentary, alcohol consumption, sexual promiscuity, drug addiction, and open and closed air contamination. Answering for cardiovascular diseases as the first cause of death in civilized world, tobacco is also pointed as the major factor implicated in the development of COPD (chronic obstructive pulmonary disease), RB-ILD (respiratory bronchiolitis and interstitial lung disease), DIP (desquamative interstitial pneumonia), bronchiolitis and bronchiolocentric interstitial fibrosis, Langerhans cells histiocytosis, eosinophilic pneumonia, sarcoidosis, epidermoid metaplasia in respiratory epithelium and lung cancer. The chronic tobacco induced inflammatory state is the basis for the acquisition of genetic alterations dependent on the tobacco contaminants. PMID:17632677

  19. Non-infectious Pulmonary Diseases and HIV.

    PubMed

    Triplette, M; Crothers, K; Attia, E F

    2016-06-01

    Pulmonary complications remain among the most frequent causes of morbidity and mortality for individuals with HIV despite the advent of antiretroviral therapy (ART) and improvement in its efficacy and availability. The prevalence of non-infectious pulmonary diseases is rising in this population, reflecting both an increase in smoking and the independent risk associated with HIV. The unique mechanisms of pulmonary disease in these patients remain poorly understood, and direct effects of HIV, genetic predisposition, inflammatory pathways, and co-infections have all been implicated. Lung cancer, chronic obstructive pulmonary disease (COPD), and pulmonary hypertension are the most prevalent non-infectious pulmonary diseases in persons with HIV, and the risk of each of these diseases is higher among HIV-infected (HIV+) persons than in the general population. This review discusses the latest advances in the literature on these important complications of HIV infection. PMID:27121734

  20. Pulmonary disease caused by Mycobacterium malmoense.

    PubMed

    Alberts, W M; Chandler, K W; Solomon, D A; Goldman, A L

    1987-06-01

    Mycobacterium malmoense was isolated from pulmonary material from 4 patients. Two patients had repeatedly positive smears and cultures along with roentgenographic progression of pulmonary disease in the absence of another pathogen. These 2 patients therefore meet the criteria for diagnosis of pulmonary mycobacteriosis. Isolation of the organism may represent colonization in a third patient, and M. malmoense has been isolated from a fourth patient on 2 occasions. It is not yet definite, however, that the pulmonary process is due to mycobacterial disease. Although uncommon, pulmonary disease caused by this organism has been reported from Europe. Only 1 prior case of pulmonary disease caused by M. malmoense, however, has been reported in the United States. PMID:3592410

  1. Pulmonary manifestations revealing Rosai-Dorfman disease.

    PubMed

    Goupil de Bouillé, J; de Muret, A; Diot, E; Dumont, P; Plantier, L; Diot, P; Marchand-Adam, Sylvain

    2015-01-01

    Rosai-Dorfman disease is a rare non-Langerhans cell histiocytosis, mainly involving cervical nodes. We present the case of a patient with a pulmonary form of Rosai-Dorfman disease without peripheral or intra-thoracic lymph nodes, characterized by the presence of pulmonary nodules and cysts associated with bilateral pleural effusions. PMID:26422575

  2. Pulmonary Hypertension in Parenchymal Lung Disease

    PubMed Central

    Tsangaris, Iraklis; Tsaknis, Georgios; Anthi, Anastasia; Orfanos, Stylianos E.

    2012-01-01

    Idiopathic pulmonary arterial hypertension (IPAH) has been extensively investigated, although it represents a less common form of the pulmonary hypertension (PH) family, as shown by international registries. Interestingly, in types of PH that are encountered in parenchymal lung diseases such as interstitial lung diseases (ILDs), chronic obstructive pulmonary disease (COPD), and many other diffuse parenchymal lung diseases, some of which are very common, the available data is limited. In this paper, we try to browse in the latest available data regarding the occurrence, pathogenesis, and treatment of PH in chronic parenchymal lung diseases. PMID:23094153

  3. Systemic and Pulmonary Vascular Remodelling in Chronic Obstructive Pulmonary Disease

    PubMed Central

    Muñoz-Esquerre, Mariana; López-Sánchez, Marta; Escobar, Ignacio; Huertas, Daniel; Penín, Rosa; Molina-Molina, María; Manresa, Frederic; Dorca, Jordi; Santos, Salud

    2016-01-01

    Background Chronic Obstructive Pulmonary Disease (COPD) is associated with subclinical systemic atherosclerosis and pulmonary vascular remodelling characterized by intimal hyperplasia and luminal narrowing. We aimed to determine differences in the intimal thickening of systemic and pulmonary arteries in COPD subjects and smokers. Secondary aims include comparisons with a non-smokers group; determining the clinical variables associated with systemic and pulmonary intimal thickening, and the correlations between systemic and pulmonary remodelling changes. Methods All consecutive subjects undergoing lung resection were included and divided into 3 groups: 1) COPD, 2) smokers, and 3) non-smokers. Sections of the 5th intercostal artery and muscular pulmonary arteries were measured by histo-morphometry. Four parameters of intimal thickening were evaluated: 1) percentage of intimal area (%IA), 2) percentage of luminal narrowing, 3) intimal thickness index, and 4) intima-to-media ratio. Results In the adjusted analysis, the systemic arteries of COPD subjects showed greater intimal thickening (%IA) than those of smokers (15.6±1.5% vs. 14.2±1.6%, p = 0.038). In the pulmonary arteries, significant differences were observed for %IA between the 2 groups (37.3±2.2% vs. 29.3±2.3%, p = 0.016). Among clinical factors, metabolic syndrome, gender and COPD status were associated with the systemic intimal thickening, while only COPD status was associated with pulmonary intimal thickening. A correlation between the %IA of the systemic and pulmonary arteries was observed (Spearman’s rho = 0.46, p = 0.008). Conclusions Greater intimal thickening in systemic and pulmonary arteries is observed in COPD patients than in smokers. There is a correlation between systemic and pulmonary vascular remodelling in the overall population. PMID:27046203

  4. Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease.

    PubMed

    Chaisson, Neal F; Dodson, Mark W; Elliott, Charles Gregory

    2016-09-01

    This article provides an overview of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH), two disorders that challenge clinicians, radiologists, and pathologists because they often mimic pulmonary arterial hypertension (PAH). The article reviews the features that differentiate PVOD and PCH from PAH. The article also describes the overlap of PVOD and PCH, highlighted by recent reports of families diagnosed with PVOD or PCH caused by EIF2AK4 mutations. In addition, the article outlines current approaches to the diagnosis and treatment of PVOD and PCH. PMID:27514598

  5. Pregnancy and chronic progressive pulmonary disease.

    PubMed

    Wexler, Isaiah D; Johannesson, Marie; Edenborough, Frank P; Sufian, Beth S; Kerem, Eitan

    2007-02-15

    Progressive pulmonary disease may preclude the option of pregnancy for a number of women in their child-bearing years due to the severity of the disease. For a subset of women with chronic lung disease including cystic fibrosis, pregnancy is possible, but can have a devastating effect both on the prospective mother and fetus. The potential hazards of pregnancy in cystic fibrosis or other progressive pulmonary diseases may trigger a moral conflict between physician and patient. The female patient may argue that her autonomy cannot be circumscribed and that the physician is obliged to assist her reproductive efforts. The physician can counter that his/her participation in potentially harmful interventions is not consistent with professional norms requiring adherence to the principles of beneficence and nonmaleficence. Whenever possible, the ethical conflict between physician and patient should be resolved before initiation of pregnancy. We propose that this best be done through structured negotiations between physician and patient with the goal of constructing an ethical framework for reducing the moral tension between the two. Steps in the negotiating process include defining the therapeutic alliance, information exchange, dialog, and deliberation. As part of the information exchange, it is important to discuss alternatives to pregnancy such as adoption and surrogacy, especially when there are strong contraindications to pregnancy. If negotiations reach a satisfactory conclusion for both sides, there should be a well-delineated consensual agreement to commence the pregnancy with the full support of the medical team. PMID:17110647

  6. The Impact of Immunosenescence on Pulmonary Disease

    PubMed Central

    Murray, Michelle A.; Chotirmall, Sanjay H.

    2015-01-01

    The global population is aging with significant gains in life expectancy particularly in the developed world. Consequently, greater focus on understanding the processes that underlie physiological aging has occurred. Key facets of advancing age include genomic instability, telomere shortening, epigenetic changes, and declines in immune function termed immunosenescence. Immunosenescence and its associated chronic low grade systemic “inflamm-aging” contribute to the development and progression of pulmonary disease in older individuals. These physiological processes predispose to pulmonary infection and confer specific and unique clinical phenotypes observed in chronic respiratory disease including late-onset asthma, chronic obstructive pulmonary disease, and pulmonary fibrosis. Emerging concepts of the gut and airway microbiome further complicate the interrelationship between host and microorganism particularly from an immunological perspective and especially so in the setting of immunosenescence. This review focuses on our current understanding of the aging process, immunosenescence, and how it can potentially impact on various pulmonary diseases and the human microbiome. PMID:26199462

  7. Pulmonary disease in gestational trophoblastic neoplasms.

    PubMed Central

    McNair, O. M.; Polk, O. D.

    1992-01-01

    Gestational trophoblastic neoplasms can present as pulmonary nodules without significant disease of the reproductive organs. This article describes a case of metastatic gestational trophoblastic disease to the lungs. This entity must be considered in the differential diagnosis in any female of reproductive age who presents with multiple pulmonary nodules. Thoracotomy has a limited role in the initial evaluation of patients with this disease. However, it may be needed in patients who have evidence of persistent pulmonary disease, despite appropriate therapy. Images Figure 1 Figure 2 Figure 3 PMID:1324326

  8. Ciliate communities consistently associated with coral diseases

    NASA Astrophysics Data System (ADS)

    Sweet, M. J.; Séré, M. G.

    2016-07-01

    Incidences of coral disease are increasing. Most studies which focus on diseases in these organisms routinely assess variations in bacterial associates. However, other microorganism groups such as viruses, fungi and protozoa are only recently starting to receive attention. This study aimed at assessing the diversity of ciliates associated with coral diseases over a wide geographical range. Here we show that a wide variety of ciliates are associated with all nine coral diseases assessed. Many of these ciliates such as Trochilia petrani and Glauconema trihymene feed on the bacteria which are likely colonizing the bare skeleton exposed by the advancing disease lesion or the necrotic tissue itself. Others such as Pseudokeronopsis and Licnophora macfarlandi are common predators of other protozoans and will be attracted by the increase in other ciliate species to the lesion interface. However, a few ciliate species (namely Varistrombidium kielum, Philaster lucinda, Philaster guamense, a Euplotes sp., a Trachelotractus sp. and a Condylostoma sp.) appear to harbor symbiotic algae, potentially from the coral themselves, a result which may indicate that they play some role in the disease pathology at the very least. Although, from this study alone we are not able to discern what roles any of these ciliates play in disease causation, the consistent presence of such communities with disease lesion interfaces warrants further investigation.

  9. Pulmonary Complications of Sickle Cell Disease

    PubMed Central

    Miller, Andrew C.

    2012-01-01

    Sickle cell disease (SCD) is a common monogenetic disorder with high associated morbidity and mortality. The pulmonary complications of SCD are of particular importance, as acute chest syndrome and pulmonary hypertension have the highest associated mortality rates within this population. This article reviews the pathophysiology, diagnosis, and treatment of clinically significant pulmonary manifestations of SCD, including acute chest syndrome, asthma, and pulmonary hypertension in adult and pediatric patients. Clinicians should be vigilant in screening and treating such comorbidities to improve patient outcomes. PMID:22447965

  10. Update on pulmonary hypertension complicating chronic obstructive pulmonary disease

    PubMed Central

    Jyothula, Soma; Safdar, Zeenat

    2009-01-01

    Pulmonary hypertension (PH) is the hemodynamic manifestation of various pathological processes that result in elevated pulmonary artery pressures (PAP). The National Institutes of Health Registry defined pulmonary arterial hypertension as the mean PAP of more than 25 mm Hg with a pulmonary capillary wedge pressure or left atrial pressure equal to or less than 15 mm Hg. This definition remains the currently accepted definition of PH that is used to define PH related to multiple clinical conditions including chronic obstructive pulmonary disease (COPD). The estimated US prevalence of COPD by the National Health Survey in 2002 in people aged >25 was 12.1 million. There is a lack of large population-based studies in COPD to document the correct prevalence of PH and outcome. The major cause of PH in COPD is hypoxemia leading to vascular remodeling. Echocardiogram is the initial screening tool of choice for PH. This simple noninvasive test can provide an estimate of right ventricular systolic and right atrial pressures. Right heart catheterization remains the gold standard to diagnose PH. It provides accurate measurement of mean PAP and pulmonary capillary wedge pressure. Oxygen therapy remains the cornerstone therapeutic for hypoxemia in COPD patients. Anecdotal reports suggest utility of PDE5-inhibitors and prostacyclin to treat COPD-related PH. Large randomized clinical trials are needed before the use of these drugs can be recommended. PMID:19802350

  11. [Hydatid disease diagnosed following a pulmonary embolism].

    PubMed

    Menassa-Moussa, L; Braidy, C; Riachy, M; Tabet, G; Smayra, T; Haddad-Zebouni, S; Ghossain, M; Aoun, N

    2009-11-01

    Hydatidosis is a parasitic disease found worldwide, particularly in Mediterranean countries, caused by Echinococcus granulosis infection. Humans are an intermediate and accidental host in the cycle of this parasite. The hydatid pulmonary arterial embolism is extremely rare, usually arising in the heart or the liver. We report a case of hydatid pulmonary embolism explored with multidetector scanner and MRI, and confirmed at pathology of the operative specimen. To our knowledge, this is the first case of inaugural hydatid pulmonary arterial embolism found on CT scan establishing the diagnosis of the disease in a patient who had no other location of hydatid cyst. PMID:19615835

  12. Pulmonary complications of sickle cell disease.

    PubMed

    Khoury, Ruby A; Musallam, Khaled M; Mroueh, Salman; Abboud, Miguel R

    2011-01-01

    The pulmonary complications of sickle cell disease are a major cause of morbidity and mortality in affected patients. The acute chest syndrome (ACS) is a leading cause of death in patients with sickle cell disease and has a multifactorial etiology. Hydroxyurea (HU), stem cell transplantation (SCT) and chronic transfusions are known to prevent the recurrence of ACS. Careful management of patients admitted for pain crises and surgery including use of incentive spirometry is critical in preventing this complication. Pulmonary hypertension is well known to be associated with sickle cell disease and patients with pulmonary hypertension have increased mortality. Asthma is also commonly seen in patients with sickle cell disease and is associated with a more complicated course. Chronic lung disease develops in a significant proportion of patients with sickle cell disease. PMID:21973051

  13. Pulmonary veno-occlusive disease presenting with thrombosis of pulmonary arteries.

    PubMed Central

    Katz, D. S.; Scalzetti, E. M.; Katzenstein, A. L.; Kohman, L. J.

    1995-01-01

    Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension. An unusual case presenting with thrombosis of the right pulmonary artery and serological evidence of autoimmunity is reported. Images PMID:7638821

  14. Pulmonary Veno-Occlusive Disease: A Newly Recognized Cause of Severe Pulmonary Hypertension in Dogs.

    PubMed

    Williams, K; Andrie, K; Cartoceti, A; French, S; Goldsmith, D; Jennings, S; Priestnall, S L; Wilson, D; Jutkowitz, A

    2016-07-01

    Pulmonary hypertension is a well-known though poorly characterized disease in veterinary medicine. In humans, pulmonary veno-occlusive disease (PVOD) is a rare cause of severe pulmonary hypertension with a mean survival time of 2 years without lung transplantation. Eleven adult dogs (5 males, 6 females; median age 10.5 years, representing various breeds) were examined following the development of severe respiratory signs. Lungs of affected animals were evaluated morphologically and with immunohistochemistry for alpha smooth muscle actin, desmin, CD31, CD3, CD20, and CD204. All dogs had pulmonary lesions consistent with PVOD, consisting of occlusive remodeling of small- to medium-sized pulmonary veins, foci of pulmonary capillary hemangiomatosis (PCH), and accumulation of hemosiderophages; 6 of 11 dogs had substantial pulmonary arterial medial and intimal thickening. Ultrastructural examination and immunohistochemistry showed that smooth muscle cells contributed to the venous occlusion. Increased expression of CD31 was evident in regions of PCH indicating increased numbers of endothelial cells in these foci. Spindle cells strongly expressing alpha smooth muscle actin and desmin co-localized with foci of PCH; similar cells were present but less intensely labeled elsewhere in non-PCH alveoli. B cells and macrophages, detected by immunohistochemistry, were not co-localized with the venous lesions of canine PVOD; small numbers of CD3-positive T cells were occasionally in and around the wall of remodeled veins. These findings indicate a condition in dogs with clinically severe respiratory disease and pathologic features resembling human PVOD, including foci of pulmonary venous remodeling and PCH. PMID:26926086

  15. Gene polymorphisms and chronic obstructive pulmonary disease

    PubMed Central

    Wu, Xiaodan; Yuan, Bowei; López, Elena; Bai, Chunxue; Wang, Xiangdong

    2014-01-01

    The genetic component was suggested to contribute to the development of chronic obstructive pulmonary disease (COPD), a major and growing public health burden. The present review aims to characterize the evidence that gene polymorphisms contribute to the aetiology of COPD and related traits, and explore the potential relationship between certain gene polymorphisms and COPD susceptibility, severity, lung function, phenotypes, or drug effects, even though limited results from related studies lacked consistency. Most of these studies were association studies, rather than confirmatory studies. More large-sized and strictly controlled studies are needed to prove the relationship between gene polymorphisms and the reviewed traits. More importantly, prospective confirmatory studies beyond initial association studies will be necessary to evaluate true relationships between gene polymorphisms and COPD and help individualized treatment for patients with COPD. PMID:24256364

  16. The genetics of chronic obstructive pulmonary disease

    PubMed Central

    Wood, Alice M; Stockley, Robert A

    2006-01-01

    Chronic obstructive pulmonary disease (COPD) is a heterogeneous disease caused by the interaction of genetic susceptibility and environmental influences. There is increasing evidence that genes link to disease pathogenesis and heterogeneity by causing variation in protease anti-protease systems, defence against oxidative stress and inflammation. The main methods of genomic research for complex disease traits are described, together with the genes implicated in COPD thus far, their roles in disease causation and the future for this area of investigation. PMID:17054776

  17. Late pulmonary toxicity after treatment for Hodgkin's disease.

    PubMed

    Villani, F; De Maria, P; Bonfante, V; Viviani, S; Laffranchi, A; Dell'oca, I; Dirusso, A; Zanini, M

    1997-01-01

    The combination of mediastinal radiotherapy (RT) with chemotherapy (CT) including bleomycin is associated with an increased risk of pulmonary toxicity. The aim of the present investigation was to evaluate late pulmonary effects of RT plus CT consisting of the ABVD regimen in patients suffering from early stage Hodgkin's disease. For this purpose pulmonary function was serially evaluated before, at the end and at least 1 year after therapy in 32 patients (median age 28 years) with Hodgkin's disease stages IA,B-IIA. Treatment consisted of four cycles of ABVD chemotherapy followed by mediastinal irradiation at the median dose of 36 Gy (range 30.6-43.2). At the end of treatment, resting mean pulmonary function tests showed a significant decline of forced expiratory volume in 1 second (FEV1), forced expiratory flow at 25-75%, (FEF25-75%), total lung capacity (TLC), vital capacity (VC) and carbon monoxide diffusing capacity (DLCO). The decline of TLC, VC and DLCO, indicative of a pulmonary defect of restrictive type, persisted 1 year from the end of therapy. Only seven patients developed symptoms of cough and mild shortness of breath with effort. These data confirm that RT combined with short term ABVD result in pulmonary dysfunction that does not seem to have clinical significance. PMID:9494599

  18. Nontuberculous mycobacterial pulmonary disease mimicking lung cancer

    PubMed Central

    Hong, Su Jin; Kim, Tae Jung; Lee, Jae-Ho; Park, Jeong-Soo

    2016-01-01

    Abstract To describe the features and clinical implications of computed tomography (CT), positron emission tomography (PET), and percutaneous needle aspiration biopsy (PCNB) in pulmonary nontuberculous mycobacterial (NTM) disease manifesting as a solitary nodule, mass, or mass-like consolidation mimicking malignancy. Among a cohort of 388 patients with NTM pulmonary disease, 14 patients with clinically and radiologically suspected lung cancer were included in our study. Two chest radiologists evaluated CT features, including lesion type (nodule, mass, or mass-like consolidation), morphologic features (margin, degree of enhancement, calcification), and presence of accompanying findings suggestive of NTM pulmonary disease (bronchiectasis with clustered centrilobular nodules or upper-lobe cavitary lesions) by consensus. Diagnostic procedures for microbiologic diagnosis of NTM disease and clinical outcome were reviewed. Incidence of NTM pulmonary disease presenting as solitary nodule/mass (n = 8) or mass-like consolidation (n = 6) was 3.6% (14 of 388). Most lesions were detected incidentally during routine health check-up or evaluation of other disease (11 of 14, 79%). Lesions typically showed poor contrast-enhancement (9 of 12) and internal calcification (6 of 14). No lesions had CT features suggestive of NTM pulmonary disease. All 4 lesions for which PET/CT imaging was performed showed strong fluorodeoxyglucose uptake simulating malignant lesions (mean, 4.9; range, 3.6–7.8). PCNB revealed mycobacterial histology in 6 of 11 specimens and positive culture results were obtained for 7 of 7 specimens. NTM pulmonary disease may present as a solitary nodule, mass, or mass-like consolidation mimicking malignancy. CT features and PCNB are important to diagnose NTM disease mimicking lung cancer to avoid unnecessary surgery. PMID:27367996

  19. [Pulmonary manifestations of connective tissue diseases].

    PubMed

    Rehbock, B

    2015-03-01

    Systemic autoimmune diseases are responsible for about 25% of all deaths due to interstitial lung disease; therefore, an early identification of patients with pulmonary manifestation changes the management. Detection, differential diagnostic classification and staging of the pneumological pattern of findings are largely based on high-resolution computed tomography (HR-CT). The main differential diagnostic challenges are interstitial manifestations which present with radiological-histopathological phenotypes of interstitial pneumonia. The most common form of interstitial pulmonary reaction form of connective tissue diseases is the nonspecific interstitial pneumonia (NSIP) pattern. In rheumatoid arthritis, a usual interstitial pneumonia (UIP) pattern is dominant. Uncharacteristic reactions of airways and pleura can be the leading symptom or present as accompanying findings. A serious complication is pulmonary hypertension. Drug-induced lung lesions can present with similar HR-CT morphology as connective tissue diseases and can only be differentiated in the temporal and clinical context. PMID:25693496

  20. Pulmonary nuclear medicine: Techniques in diagnosis of lung disease

    SciTech Connect

    Atkins, H.L.

    1984-01-01

    This book presents papers on the application of nuclear medicine to the diagnosis of lung diseases. Topics considered include lung physiology and anatomy, radiopharmaceuticals in pulmonary medicine, pulmonary embolism, obstructive pulmonary disease, diffuse infiltrative lung disease, pneumoconioses, tumor localization scans in primary lung tumors, the interactions of heart diseases and lung diseases on radionuclide tests of lung anatomy and function, radionuclide imaging in pediatric lung diseases, and future possibilities in pulmonary nuclear medicine.

  1. Chronic Obstructive Pulmonary Disease Prevalence and Mortality

    EPA Science Inventory

    This indicator describes data on chronic pulmonary disease (COPD) prevalence and deaths across the U.S. for the time periods 1997-2009 and 1979-2007, respectively. COPD, also known as chronic lung disease, may be partly caused or exacerbated by environmental exposures such as ...

  2. Clinical Utility of Echocardiography for the Diagnosis and Management of Pulmonary Vascular Disease in Young Children With Chronic Lung Disease

    PubMed Central

    Mourani, Peter M.; Sontag, Marci K.; Younoszai, Adel; Ivy, D. Dunbar; Abman, Steven H.

    2011-01-01

    hypertension in young children with chronic lung disease; however, estimates of systolic pulmonary artery pressure were not obtained consistently and were not reliable for determining the severity of pulmonary hypertension. PMID:18245423

  3. Pulmonary manifestations of gastroesophageal reflux disease

    PubMed Central

    Gaude, Gajanan S.

    2009-01-01

    Gastroesophageal reflux disease (GERD) may cause, trigger or exacerbate many pulmonary diseases. The physiological link between GERD and pulmonary disease has been extensively studied in chronic cough and asthma. A primary care physician often encounters patients with extra esophageal manifestations of GERD in the absence of heartburn. Patients may present with symptoms involving the pulmonary system; noncardiac chest pain; and ear, nose and throat disorders. Local irritation in the esophagus can cause symptoms that vary from indigestion, like chest discomfort and abdominal pain, to coughing and wheezing. If the gastric acid reaches the back of the throat, it may cause a bitter taste in the mouth and/or aspiration of the gastric acid into the lungs. The acid can cause throat irritation, postnasal drip and hoarseness, as well as recurrent cough, chest congestion and lung inflammation leading to asthma and/or bronchitis/ pneumonia. This clinical review examines the potential pathophysiological mechanisms of pulmonary manifestations of GERD. It also reviews relevant clinical information concerning GERD-related chronic cough and asthma. Finally, a potential management strategy for GERD in pulmonary patients is discussed. PMID:19641641

  4. [Pulmonary complications in adult sickle cell disease].

    PubMed

    Maître, B; Mekontso-Dessap, A; Habibi, A; Bachir, D; Parent, F; Godeau, B; Galacteros, F

    2011-02-01

    Sickle cell disease is an autosomal genetic condition which represents the most frequent genetic disease in Île-de-France and Caribbean islands. The main clinical manifestations can be divided into infectious disease, hemolytic anemia and vaso-occlusive events. Pulmonary complications represent 20 to 30% of mortality due to sickle cell and can be divided into acute and chronic events. Acute chest syndrome (ACS) is an acute lung injury often preceded by a vaso-occlusive crisis and triggered by different factors including: hypoventilation, pulmonary infectious disease and vascular occlusions. These occlusions can be secondary to fat embolism, thrombosis or sickling. Treatment is mainly supportive combining oxygen supplementation adequate hydration analgesia and sedation. Exchange transfusion may be indicated in severe forms of ACS, characterized by a right ventricular dysfunction and acute respiratory failure. Pulmonary hypertension is the most serious chronic complication. Its frequency is estimated at 6% in adult patients and is more often described in patients with venous ulcers and higher levels of chronic hemolysis. Prognosis is poor with 12.5% of patients dying in the first two years following diagnosis irrespective of the actual pulmonary artery pressure level. There are currently limited data on the effects of any treatment modality. Other respiratory complications such as sleep disorders and nocturnal hypoxemia, infiltrative lung disease and exertional dyspnea are described and should be considered. PMID:21402228

  5. Pulmonary hypertension complicating connective tissue disease.

    PubMed

    Lynch, Joseph P; Belperio, John A; Saggar, Rajeev; Fishbein, Michael C; Saggar, Rajan

    2013-10-01

    Pulmonary hypertension (PH) may complicate connective tissue disease (CTD), particularly systemic sclerosis (SSc, scleroderma), and markedly increases mortality. More than 70% of cases of PH complicating CTD occur in SSc, which is the major focus of this article. Pulmonary complications (i.e., interstitial lung disease [ILD] and PH) are the leading causes of scleroderma-related deaths. "Isolated" PH (i.e., without ILD) complicates SSc in 7.5 to 20% of cases; secondary PH may also occur in patients with SSc-associated ILD. Several clinical markers and specific autoantibody profiles have been associated with PH in SSc. The role of PH-specific therapy is controversial, as prognosis and responsiveness to therapy are worse in SSc-associated PH compared with idiopathic pulmonary arterial hypertension. We discuss medical therapies for CTD-associated PH and the role of lung transplantation for patients failing medical therapy. PMID:24037627

  6. Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome.

    PubMed

    Krieger, Eric V; Leary, Peter J; Opotowsky, Alexander R

    2015-11-01

    Patients with adult congenital heart disease have an increased risk of developing pulmonary hypertension. There are several mechanisms of pulmonary hypertension in patients with adult congenital heart disease, and understanding them requires a systematic approach to define the patient's hemodynamics and physiology. This article reviews the updated classification of pulmonary hypertension in patients with adult congenital heart disease with a focus on pathophysiology, diagnostics, and the evaluation of pulmonary hypertension in special adult congenital heart disease populations. PMID:26471823

  7. Pulmonary disease following intravesical BCG treatment.

    PubMed Central

    Kesten, S; Title, L; Mullen, B; Grossman, R

    1990-01-01

    Bacillus Calmette-Guérin (BCG) is an attenuated strain of Mycobacterium bovis that has been used in the treatment of malignant disease for over 20 years and for the treatment of bladder cancer since 1976. Major complications of this treatment are infrequent. We report two cases of systemic illness with pulmonary manifestations after treatment with intravesical BCG. Images PMID:2218978

  8. Pulmonary manifestations of inflammatory bowel disease

    PubMed Central

    Majewski, Sebastian

    2015-01-01

    Bronchopulmonary signs and symptoms are examples of variable extraintestinal manifestations of the inflammatory bowel diseases (IBD). These complications of Crohn's disease (CD) and ulcerative colitis (UC) seem to be underrecognized by both pulmonary physicians and gastroenterologists. The objective of the present review was to gather and summarize information on this particular matter, on the basis of available up-to-date literature. Tracheobronchial involvement is the most prevalent respiratory presentation, whereas IBD-related interstitial lung disease is less frequent. Latent and asymptomatic pulmonary involvement is not unusual. Differential diagnosis should always consider infections (mainly tuberculosis) and drug-induced lung pathology. The common link between intestinal disease and lung pathology is unknown, but many hypotheses have been proposed. It is speculated that environmental pollution, common immunological mechanisms and predisposing genetic factors may play a role. PMID:26788078

  9. Pulmonary manifestations of inflammatory bowel disease.

    PubMed

    Majewski, Sebastian; Piotrowski, Wojciech

    2015-12-10

    Bronchopulmonary signs and symptoms are examples of variable extraintestinal manifestations of the inflammatory bowel diseases (IBD). These complications of Crohn's disease (CD) and ulcerative colitis (UC) seem to be underrecognized by both pulmonary physicians and gastroenterologists. The objective of the present review was to gather and summarize information on this particular matter, on the basis of available up-to-date literature. Tracheobronchial involvement is the most prevalent respiratory presentation, whereas IBD-related interstitial lung disease is less frequent. Latent and asymptomatic pulmonary involvement is not unusual. Differential diagnosis should always consider infections (mainly tuberculosis) and drug-induced lung pathology. The common link between intestinal disease and lung pathology is unknown, but many hypotheses have been proposed. It is speculated that environmental pollution, common immunological mechanisms and predisposing genetic factors may play a role. PMID:26788078

  10. Inapparent pulmonary vascular disease in an ex-heroin user

    SciTech Connect

    Antonelli Incalzi, R.; Ludovico Maini, C.; Giuliano Bonetti, M.; Campioni, P.; Pistelli, R.; Fuso, L.

    1986-04-01

    A severe pulmonary vascular derangement, usually reported in drug addicts, was diagnosed in a 28-year-old asymptomatic ex-heroin user by means of fortuitously performed pulmonary perfusion imaging. Neither physical findings nor pulmonary function tests, aroused suspicion of the diagnosis. A search for asymptomatic pulmonary vascular disease probably should be undertaken in drug addicts.

  11. Chronic obstructive pulmonary disease: clinical integrative physiology.

    PubMed

    O'Donnell, Denis E; Laveneziana, Pierantonio; Webb, Katherine; Neder, J Alberto

    2014-03-01

    Peripheral airway dysfunction, inhomogeneous ventilation distribution, gas trapping, and impaired pulmonary gas exchange are variably present in all stages of chronic obstructive pulmonary disease (COPD). This article provides a cogent physiologic explanation for the relentless progression of activity-related dyspnea and exercise intolerance that all too commonly characterizes COPD. The spectrum of physiologic derangements that exist in smokers with mild airway obstruction and a history compatible with COPD is examined. Also explored are the perceptual and physiologic consequences of progressive erosion of the resting inspiratory capacity. Finally, emerging information on the role of cardiocirculatory impairment in contributing to exercise intolerance in patients with varying degrees of airway obstruction is reviewed. PMID:24507837

  12. [Pulmonary involvement in connective tissue disease].

    PubMed

    Bartosiewicz, Małgorzata

    2016-01-01

    The connective tissue diseases are a variable group of autoimmune mediated disorders characterized by multiorgan damage. Pulmonary complications are common, usually occur after the onset of joint symptoms, but can also be initially presenting complaint. The respiratory system may be involved in all its component: airways, vessels, parenchyma, pleura and respiratory muscles. Lung involvement is an increasing cause of morbidity and mortality in the connective tissue diseases. Clinical course is highly variable - can range from mild to rapidly progressive, some processes are reversible, while others are irreversible. Thus, the identification of reversible disease , and separately progressive disease, are important clinical issues. The frequency, clinical presentation, prognosis and responce to therapy are different, depending on the pattern of involvement as well as on specyfic diagnostic method used to identify it. High- resolution computed tompography plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease. In this article, pulmonary lung involvement accompanying most frequently apperaing connective tissue diseases - rheumatoid arthritis, systemic sclerosis, lupus erythematosus, polymyositis/dermatomyositis, Sjögrens syndrome and mixed connective tissue disaese are reviewed. PMID:27421127

  13. Meandering right pulmonary vein associated with severe and progressive "idiopathic-like" pulmonary hypertensive vascular disease.

    PubMed

    Cuenca, Sofia; Bret, Montserrat; Del Cerro, Maria Jesus

    2016-03-01

    Congenital anomalies of the pulmonary veins are rare. Meandering right pulmonary vein, considered a part of the Scimitar syndrome spectrum, is often an incidental finding during chest imaging. We present the case of a 4-year-old girl diagnosed with meandering pulmonary vein, who developed pulmonary hypertensive disease with an aggressive course, in spite of absence of hypoxia or elevated pulmonary wedge pressure. PMID:26374461

  14. Chronic Pulmonary Complications of Sickle Cell Disease.

    PubMed

    Mehari, Alem; Klings, Elizabeth S

    2016-05-01

    Sickle cell disease (SCD), the most common genetic hemolytic anemia worldwide, affects 250,000 births annually. In the United States, SCD affects approximately 100,000 individuals, most of African descent. Hemoglobin S (HbS) results from a glutamate-to-valine mutation of the sixth codon of the β-hemoglobin allele; the homozygous genotype (HbSS) is associated with the most prevalent and severe form of the disease. Other SCD genotypes include HbSC, composed of one HbS allele and one HbC (glutamate-to-lysine mutation) allele; and HbS-β-thalassemia(0) or HbS-β-thalassemia(+), composed of one HbS allele and one β-thalassemia allele with absent or reduced β-chain production, respectively. Despite advances in care, median survival remains in the fifth decade, due in large part to chronic complications of the disease. Chronic pulmonary complications in SCD are major contributors to this early mortality. Although our understanding of these conditions has improved much over the past 10 to 15 years, there remains no specific treatment for pulmonary complications of SCD. It is unclear whether conventional treatment regimens directed at non-SCD populations have equivalent efficacy in patients with SCD. This represents a critical research need. In this review, the authors review the state-of-the-art understanding of the following pulmonary complications of SCD: (1) pulmonary hypertension; (2) venous thromboembolic disease; (3) sleep-disordered breathing; (4) asthma and recurrent wheezing; and (5) pulmonary function abnormalities. This review highlights the advances as well as the knowledge gaps in this field to update clinicians and other health care providers and to garner research interest from the medical community. PMID:26836905

  15. Pulmonary Physiology of Chronic Obstructive Pulmonary Disease, Cystic Fibrosis, and Alpha-1 Antitrypsin Deficiency.

    PubMed

    Stockley, James A; Stockley, Robert A

    2016-04-01

    Cystic fibrosis is predominantly an airway disease with marked bronchiectatic changes associated with inflammation, chronic colonization, and progressive airflow obstruction. The condition can be identified in childhood and monitored with detectable airway changes early in life while conventional spirometry remains in the normal range. Alpha-1 antitrypsin deficiency can also be detected early in life through blood spot and genetic testing and leads (in some) to the development of airflow obstruction and a predominant emphysema phenotype with bronchiectatic changes in about 30%. Early detection also allows the natural history of the pulmonary physiological changes to be determined. Chronic obstructive pulmonary disease is usually detected late in the disease process when significant damage has occurred. The condition consists of varying combinations of airway disease, bronchiectasis, colonization, and emphysema. Lessons learned from the physiological evolution of airway disease in cystic fibrosis and the emphysema of alpha-1 antitrypsin deficiency provide strategies to enable early detection of chronic obstructive pulmonary disease in general and its phenotypes. PMID:27115945

  16. Osteoporosis in chronic obstructive pulmonary disease.

    PubMed

    Sarkar, Malay; Bhardwaj, Rajeev; Madabhavi, Irappa; Khatana, Jasmin

    2015-01-01

    Chronic obstructive pulmonary disease (COPD) is a lifestyle-related chronic inflammatory pulmonary disease associated with significant morbidity and mortality worldwide. COPD is associated with various comorbidities found in all stages of COPD. The comorbidities have significant impact in terms of morbidity, mortality, and economic burden in COPD. Management of comorbidities should be incorporated into the comprehensive management of COPD as this will also have an effect on the outcome in COPD patients. Various comorbidities reported in COPD include cardiovascular disease, skeletal muscle dysfunction, anemia, metabolic syndrome, and osteoporosis. Osteoporosis is a significant comorbidity in COPD patients. Various risk factors, such as tobacco smoking, systemic inflammation, vitamin D deficiency, and the use of oral or inhaled corticosteroids (ICSs) are responsible for its occurrence in patients with COPD. This review will focus on the prevalence, pathogenesis, risk factors, diagnosis, and treatment of osteoporosis in COPD patients. PMID:25788838

  17. Osteoporosis in Chronic Obstructive Pulmonary Disease

    PubMed Central

    Sarkar, Malay; Bhardwaj, Rajeev; Madabhavi, Irappa; Khatana, Jasmin

    2015-01-01

    Chronic obstructive pulmonary disease (COPD) is a lifestyle-related chronic inflammatory pulmonary disease associated with significant morbidity and mortality worldwide. COPD is associated with various comorbidities found in all stages of COPD. The comorbidities have significant impact in terms of morbidity, mortality, and economic burden in COPD. Management of comorbidities should be incorporated into the comprehensive management of COPD as this will also have an effect on the outcome in COPD patients. Various comorbidities reported in COPD include cardiovascular disease, skeletal muscle dysfunction, anemia, metabolic syndrome, and osteoporosis. Osteoporosis is a significant comorbidity in COPD patients. Various risk factors, such as tobacco smoking, systemic inflammation, vitamin D deficiency, and the use of oral or inhaled corticosteroids (ICSs) are responsible for its occurrence in patients with COPD. This review will focus on the prevalence, pathogenesis, risk factors, diagnosis, and treatment of osteoporosis in COPD patients. PMID:25788838

  18. Pathogenesis of chronic obstructive pulmonary disease

    PubMed Central

    Tuder, Rubin M.; Petrache, Irina

    2012-01-01

    The current epidemic of chronic obstructive pulmonary disease (COPD) has produced a worldwide health care burden, approaching that imposed by transmittable infectious diseases. COPD is a multidimensional disease, with varied intermediate and clinical phenotypes. This Review discusses the pathogenesis of COPD, with particular focus on emphysema, based on the concept that pulmonary injury involves stages of initiation (by exposure to cigarette smoke, pollutants, and infectious agents), progression, and consolidation. Tissue damage entails complex interactions among oxidative stress, inflammation, extracellular matrix proteolysis, and apoptotic and autophagic cell death. Lung damage by cigarette smoke ultimately leads to self-propagating processes, resulting in macromolecular and structural alterations — features similar to those seen in aging. PMID:22850885

  19. [Pulmonary obstructive chronic disease and physical exercise].

    PubMed

    António, Carla; Gonçalves, Ana Paula; Tavares, Alcina

    2010-01-01

    Chronic Obstructive Pulmonary Disease (COPD) is a disease that can be prevented and treated, with a pulmonary component and with significant systemic effects that contribute to the severity of clinical manifestations. COPD causes a number of changes, including those which lead to exercise tolerance limitation and to a progressive deterioration of life quality of the patients. Respiratory rehabilitation (RR) represents a key part of the treatment. The benefits of RR are independent of sex, age and disease severity. At the end of the program, the patient should have acquired a life style as independent and healthy as possible. With this article the authors intend to review the benefits of physical exercise in rehabilitation of patients with COPD and the different types of training used in the respiratory rehabilitation program established for each patient. PMID:20700562

  20. Management of pulmonary arterial hypertension associated with congenital heart disease.

    PubMed

    Togănel, Rodica; Benedek, I; Suteu, Carmen; Blesneac, Cristina

    2007-01-01

    Congenital heart diseases are the most common congenital malformations and account for about eight cases per 1000 births and are often associated with pulmonary arterial hypertension. Increased shear stress and the excess flow through the pulmonary vascular bed due to a systemic-to-pulmonary shunt lead to the development of pulmonary vascular disease and an increase in pulmonary vascular resistance. Without surgical repair approximately 30% of patients develop pulmonary vascular disease. Eisenmenger syndrome represents the extreme end of pulmonary arterial hypertension with congenital heart disease. We summarized the current therapeutic options for pulmonary arterial hypertension; conventional treatments including calcium channel blockers, anticoagulation, digitalis, diuretics, and new treatment: prostacyclin, bosentan, sildenafil, ambrisentan. Preliminary data of new therapies are encouraging with disease significantly improved natural history, but there is need for more evidence-based data. PMID:18333354

  1. Pulmonary hypertension in chronic lung diseases.

    PubMed

    Seeger, Werner; Adir, Yochai; Barberà, Joan Albert; Champion, Hunter; Coghlan, John Gerard; Cottin, Vincent; De Marco, Teresa; Galiè, Nazzareno; Ghio, Stefano; Gibbs, Simon; Martinez, Fernando J; Semigran, Marc J; Simonneau, Gerald; Wells, Athol U; Vachiéry, Jean-Luc

    2013-12-24

    Chronic obstructive lung disease (COPD) and diffuse parenchymal lung diseases (DPLD), including idiopathic pulmonary fibrosis (IPF) and sarcoidosis, are associated with a high incidence of pulmonary hypertension (PH), which is linked with exercise limitation and a worse prognosis. Patients with combined pulmonary fibrosis and emphysema (CPFE) are particularly prone to the development of PH. Echocardiography and right heart catheterization are the principal modalities for the diagnosis of COPD and DPLD. For discrimination between group 1 PH patients with concomitant respiratory abnormalities and group 3 PH patients (PH caused by lung disease), patients should be transferred to a center with expertise in both PH and lung diseases for comprehensive evaluation. The task force encompassing the authors of this article provided criteria for this discrimination and suggested using the following definitions for group 3 patients, as exemplified for COPD, IPF, and CPFE: COPD/IPF/CPFE without PH (mean pulmonary artery pressure [mPAP] <25 mm Hg); COPD/IPF/CPFE with PH (mPAP ≥25 mm Hg); PH-COPD, PH-IPF, and PH-CPFE); COPD/IPF/CPFE with severe PH (mPAP ≥35 mm Hg or mPAP ≥25 mm Hg with low cardiac index [CI <2.0 l/min/m(2)]; severe PH-COPD, severe PH-IPF, and severe PH-CPFE). The "severe PH group" includes only a minority of chronic lung disease patients who are suspected of having strong general vascular abnormalities (remodeling) accompanying the parenchymal disease and with evidence of an exhausted circulatory reserve rather than an exhausted ventilatory reserve underlying the limitation of exercise capacity. Exertional dyspnea disproportionate to pulmonary function tests, low carbon monoxide diffusion capacity, and rapid decline of arterial oxygenation upon exercise are typical clinical features of this subgroup with poor prognosis. Studies evaluating the effect of pulmonary arterial hypertension drugs currently not approved for group 3 PH patients should focus on

  2. Non-congenital heart disease associated pediatric pulmonary arterial hypertension.

    PubMed

    Ivy, D D; Feinstein, J A; Humpl, T; Rosenzweig, E B

    2009-12-01

    Recognition of causes of pulmonary hypertension other than congenital heart disease is increasing in children. Diagnosis and treatment of any underlying cause of pulmonary hypertension is crucial for optimal management of pulmonary hypertension. This article discusses the available knowledge regarding several disorders associated with pulmonary hypertension in children: idiopathic pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, hemoglobinopathies, hepatopulmonary syndrome, portopulmonary hypertension and HIV. Three classes of drugs have been extensively studied for the treatment of IPAH in adults: prostanoids (epoprostenol, treprostinil, iloprost, beraprost), endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan), and phosphodiesterase inhibitors (Sildenafil, tadalafil). These medications have been used in treatment of children with pulmonary arterial hypertension, although randomized clinical trial data is lacking. As pulmonary vasodilator therapy in certain diseases may be associated with adverse outcomes, further study of these medications is needed before widespread use is encouraged. PMID:21852894

  3. Non-congenital heart disease associated pediatric pulmonary arterial hypertension

    PubMed Central

    Ivy, D. D.; Feinstein, J. A.; Humpl, T.; Rosenzweig, E. B.

    2011-01-01

    Recognition of causes of pulmonary hypertension other than congenital heart disease is increasing in children. Diagnosis and treatment of any underlying cause of pulmonary hypertension is crucial for optimal management of pulmonary hypertension. This article discusses the available knowledge regarding several disorders associated with pulmonary hypertension in children: idiopathic pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, hemoglobinopathies, hepatopulmonary syndrome, portopulmonary hypertension and HIV. Three classes of drugs have been extensively studied for the treatment of IPAH in adults: prostanoids (epoprostenol, treprostinil, iloprost, beraprost), endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan), and phosphodiesterase inhibitors (Sildenafil, tadalafil). These medications have been used in treatment of children with pulmonary arterial hypertension, although randomized clinical trial data is lacking. As pulmonary vasodilator therapy in certain diseases may be associated with adverse outcomes, further study of these medications is needed before widespread use is encouraged. PMID:21852894

  4. Invasive pulmonary aspergillosis in patients with chronic obstructive pulmonary disease

    PubMed Central

    2013-01-01

    Background Invasive pulmonary aspergillosis (IPA) is an infection often occurring in neutropenic patients and has high mortality rates. In recent years, it has been reported that the incidence of IPA has also increased in patients with chronic obstructive pulmonary disease (COPD). The purpose of this study is to investigate the clinical and demographic characteristics and treatment responses of IPA in patients with COPD. Methods Seventy-one patients with a positive culture of Aspergillus from lower respiratory tract samples were examined retrospectively. Eleven (15.4%) of these patients, affected with grade 3 or 4 COPD, had IPA. Results Aspergillus hyphae were detected in lung biopsy in three (27.3%) out of 11 patients and defined as proven IPA; a pathological sample was not taken in the other eight (72.7%) patients, and these were defined as probable IPA. Aspergillus isolates were identified as six cases of Aspergillusfumigatus and three of Aspergillusniger in nine patients, while two isolates were not identified at species level. While five patients required intensive care unit admission, four of them received mechanical ventilation. The most common finding on chest X-ray and computed tomography (CT) (respectively 63.6%, 72.7%) was infiltration. Amphotericin B was the initial drug of choice in all patients and five patients were discharged with oral voriconazole after amphotericin B therapy. Six patients (54.5%) died before treatment was completed. Conclusions IPA should be taken into account in the differential diagnosis particularly in patients with severe and very severe COPD presenting with dyspnea exacerbation, poor clinical status, and a new pulmonary infiltrate under treatment with broad-spectrum antibiotics and steroids. PMID:24135224

  5. Pulmonary hemodynamic profile in chronic obstructive pulmonary disease

    PubMed Central

    Portillo, Karina; Torralba, Yolanda; Blanco, Isabel; Burgos, Felip; Rodriguez-Roisin, Roberto; Rios, Jose; Roca, Josep; Barberà, Joan A

    2015-01-01

    Introduction Few data are available in regards to the prevalence of pulmonary hypertension (PH) in the broad spectrum of COPD. This study was aimed at assessing the prevalence of PH in a cohort of COPD patients across the severity of airflow limitation, and reporting the hemodynamic characteristics at rest and during exercise. Methods We performed a retrospective analysis on COPD patients who underwent right-heart catheterization in our center with measurements obtained at rest (n=139) and during exercise (n=85). PH was defined as mean pulmonary artery pressure (mPAP) ≥25 mmHg and pulmonary capillary wedge pressure <15 mmHg. Exercise-induced PH (EIPH) was defined by a ratio of ΔmPAP/Δcardiac output >3. Results PH was present in 25 patients (18%). According to the Global Initiative for Chronic Obstructive Lung Disease (GOLD) classification, PH prevalence in GOLD 2 was 7% (3 patients); 25% (14 patients) in GOLD 3; and 22% (8 patients) in GOLD 4. Severe PH (mPAP ≥35 mmHg) was identified in four patients (2.8%). Arterial partial oxygen pressure was the outcome most strongly associated with PH (r=−0.29, P<0.001). EIPH was observed in 60 patients (71%) and had a similar prevalence in both GOLD 2 and 3, and was present in all GOLD 4 patients. Patients with PH had lower cardiac index during exercise than patients without PH (5.0±1.2 versus 6.7±1.4 L/min/m2, respectively; P=0.001). Conclusion PH has a similar prevalence in COPD patients with severe and very-severe airflow limitation, being associated with the presence of arterial hypoxemia. In contrast, EIPH is highly prevalent, even in moderate COPD, and might contribute to limiting exercise tolerance. PMID:26203238

  6. [Nutritional abnormalities in chronic obstructive pulmonary disease].

    PubMed

    Gea, Joaquim; Martínez-Llorens, Juana; Barreiro, Esther

    2014-07-22

    Nutritional abnormalities are associated with chronic obstructive pulmonary disease with a frequency ranging from 2 to 50%, depending on the geographical area and the study design. Diagnostic tools include anthropometry, bioelectrical impedance, dual energy radioabsortiometry and deuterium dilution, being the body mass and the lean mass indices the most frequently used parameters. While the most important consequences of nutritional abnormalities are muscle dysfunction and exercise limitation, factors implicated include an imbalance between caloric intake and consumption, and between anabolic and catabolic hormones, inflammation, tobacco smoking, poor physical activity, hypoxemia, some drugs and aging/comorbidities. The most important molecular mechanism for malnutrition associated with chronic obstructive pulmonary disease appears to be the mismatching between protein synthesis and breakdown. Among the therapeutic measures proposed for these nutritional abnormalities are improvements in lifestyle and nutritional support, although the use of anabolic drugs (such as secretagogues of the growth hormone) offers a new therapeutic strategy. PMID:24054776

  7. Pulmonary Vascular Complications of Liver Disease

    PubMed Central

    Fritz, Jason S.; Fallon, Michael B.

    2013-01-01

    Hepatopulmonary syndrome and portopulmonary hypertension are two pulmonary vascular complications of liver disease. The pathophysiology underlying each disorder is distinct, but patients with either condition may be limited by dyspnea. A careful evaluation of concomitant symptoms, the physical examination, pulmonary function testing and arterial blood gas analysis, and echocardiographic, imaging, and hemodynamic studies is crucial to establishing (and distinguishing) these diagnoses. Our understanding of the pathobiology, natural history, and treatment of these disorders has advanced considerably over the past decade; however, the presence of either still increases the risk of morbidity and mortality in patients with underlying liver disease. There is no effective medical treatment for hepatopulmonary syndrome. Although liver transplantation can resolve hepatopulmonary syndrome, there appears to be worse survival even with transplantation. Liver transplantation poses a very high risk of death in those with significant portopulmonary hypertension, where targeted medical therapies may improve functional status and allow successful transplantation in a small number of select patients. PMID:23155142

  8. Comorbidity in chronic obstructive pulmonary disease.

    PubMed

    Negewo, Netsanet A; McDonald, Vanessa M; Gibson, Peter G

    2015-11-01

    Patients with chronic obstructive pulmonary diseases (COPD) often experience comorbid conditions. The most common comorbidities that have been associated with COPD include cardiovascular diseases, lung cancer, metabolic disorder, osteoporosis, anxiety and depression, skeletal muscle dysfunction, cachexia, gastrointestinal diseases, and other respiratory conditions. Not only are comorbidities common but they also considerably influence disease prognosis and patients׳ health status, and are associated with poor clinical outcomes. However, perusal of literature indicates that little has been done so far to effectively assess, manage, and treat comorbidities in patients with COPD. The aim of this review is to comprehensively narrate the comorbid conditions that often coexist with COPD, along with their reported prevalence and their significant impacts in the disease management of COPD. A perspective on integrated disease management approaches for COPD is also discussed. PMID:26521102

  9. Pulmonary hypertension in hemolytic disorders: pulmonary vascular disease: the global perspective.

    PubMed

    Machado, Roberto F; Gladwin, Mark T

    2010-06-01

    The inherited hemoglobin disorders sickle cell disease and thalassemia are the most common monogenetic disorders worldwide. Pulmonary hypertension is one of the leading causes of morbidity and mortality in adult patients with sickle cell disease and thalassemia, and hemolytic disorders are potentially among the most common causes of pulmonary hypertension. The pathogenesis of pulmonary hypertension in hemolytic disorders is likely multifactorial, including hemolysis, impaired nitric oxide (NO) bioavailability, chronic hypoxemia, chronic thromboembolic disease, chronic liver disease, and asplenia. In contrast to patients with traditional forms of pulmonary arterial hypertension, patients with hemolytic disorders have a mild-to-moderate degree of elevation in mean pulmonary pressures, with mild elevations in pulmonary vascular resistance. The hemodynamic etiology of pulmonary hypertension in these patients is multifactorial and includes pulmonary arterial hypertension, pulmonary venous hypertension, and pulmonary hypertension secondary to a hyperdynamic state. Currently, there are limited data on the effects of any specific treatment modality for pulmonary hypertension in patients with hemolytic disorders. It is likely that maximization of treatment of the primary hemoglobinopathy in all patients and treatment with selective pulmonary vasodilators and antiproliferative agents in patients with pulmonary arterial hypertension would be beneficial. However, there is still a major need for large multinational trials of novel therapies for this patient population. PMID:20522578

  10. Pulmonary disease in patients with hematologic malignancies.

    PubMed

    Poletti, Venerino; Trisolini, Rocco; Tura, Sante

    2002-03-01

    Patients with hematologic neoplasms frequently experience pulmonary disease. The possibility of a malignant involvement of the lung parenchyma is a well recognized and not unusual event, secondary spread due to lymphoproliferative disorders being the most common situation. Furthermore, the development and the advances in treatment options such as hematopoietic stem cell transplantation, radiation therapy and/or combined drug regimen use have significantly widened the spectrum of non-neoplastic pulmonary complications that can crop up in these patients. Infections, drug/radiation-induced toxicity, and graft-versus-host disease (GVHD)-related complications account by now for most pulmonary problems in hematologic patients and represent a difficult challenge both in diagnostic and in therapeutic terms for the clinician. The aim of this review is to highlight the clinicopathologic spectrum of lung diseases which can occur in the setting of hematologic malignancies. A particular emphasis is devoted to the diagnostic approach, high-resolution computed tomography (HRCT) assuming a key role since different patterns of CT abnormalities are associated with a different yield of the available diagnostic tools and may help in narrowing the differential diagnosis. PMID:12002382

  11. Mycobacterium kansasii pulmonary diseases in Korea.

    PubMed

    Yim, Jae-Joon; Park, Young-Kil; Lew, Woo Jin; Bai, Gill-Han; Han, Sung Koo; Shim, Young-Soo

    2005-12-01

    Mycobacterium kansasii is one of the most common cause of pulmonary diseases due to nontuberculous mycobacteria. We investigated the changing in the number of isolation of M. kansasii and the clinical characteristics of M. kansasii pulmonary disease in Korea. Through searching the database of the Korean Institute of Tuberculosis, we identified the cases of isolated M. kansasii from 1992 to 2002. The number of M. kansasii isolation had increased from once in 1992 to 62 in 2002. Fifteen patients with M. kansasii pulmonary disease were identified during the period January 1997 to December 2002. Twelve patients (80%) were male and fourteen (93%) were from highly industrialized areas. The most common symptom was a cough. Seven patients (47%) had a cavitary lesion and right upper lobe was most commonly involved. Patients responded well to isoniazid and rifampicin based regimens both bacteriologically and radiographically. In conclusion, M. kansasii isolation has increased, especially in highly industrialized areas, as well as other nontuberculous mycobacteria in Korea. PMID:16361804

  12. Hypoxic pulmonary hypertension in chronic lung diseases: novel vasoconstrictor pathways.

    PubMed

    Rowan, Simon C; Keane, Michael P; Gaine, Seán; McLoughlin, Paul

    2016-03-01

    Pulmonary hypertension is a well recognised complication of chronic hypoxic lung diseases, which are among the most common causes of death and disability worldwide. Development of pulmonary hypertension independently predicts reduced life expectancy. In chronic obstructive pulmonary disease, long-term oxygen therapy ameliorates pulmonary hypertension and greatly improves survival, although the correction of alveolar hypoxia and pulmonary hypertension is only partial. Advances in understanding of the regulation of vascular smooth muscle tone show that chronic vasoconstriction plays a more important part in the pathogenesis of hypoxic pulmonary hypertension than previously thought, and that structural vascular changes contribute less. Trials of existing vasodilators show that pulmonary hypertension can be ameliorated and systemic oxygen delivery improved in carefully selected patients, although systemic hypotensive effects limit the doses used. Vasoconstrictor pathways that are selective for the pulmonary circulation can be blocked to reduce hypoxic pulmonary hypertension without causing systemic hypotension, and thus provide potential targets for novel therapeutic strategies. PMID:26895650

  13. [Pulmonary diseases among returning travelers].

    PubMed

    Barras, G; Michel, Y; Wagner, N; Loutan, L

    2012-05-01

    Respiratory tract infections are a frequent cause of travelers' health problems. Tropical diseases are relatively rare compared to common respiratory infections. Nevertheless, due to their potential gravity, they must be systematically considered. The differential diagnosis of tropical pathogens is wide. A targeted travel history taking into account specific epidemiological, geographical and behavioral risk factors helps focusing biological exams to identify the causative diagnosis. The chest radiography remains very helpful in this context; however, more specific exams, such as serologies are often necessary to reach a final diagnosis. PMID:22662629

  14. Pulmonary Hypertension and Congenital Heart Disease.

    PubMed

    Roth, Todd S; Aboulhosn, Jamil A

    2016-08-01

    Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is a frequent complication in adults with congenital heart disease. Regardless of etiology, the optimal treatment strategy for this difficult population is challenging. The new frontier of targeted PAH therapies has demonstrated improved functional capacity in the various phenotypes of PAH-CHD, with work currently in progress scrutinizing outcomes. In those who fail conventional medical therapy, heart and heart-lung (block) transplantation become the final therapeutic options, with the role of ventricular assist devices and the total artificial heart still under investigation in this group. PMID:27443136

  15. [Chronic obstructive pulmonary disease and pneumonia].

    PubMed

    Huerta, Arturo; Domingo, Rebeca; Soler, Néstor

    2010-01-01

    Chronic obstructive pulmonary disease (COPD) is a chronic disease causing increasing healthcare costs worldwide. Another respiratory disease causing high costs and morbidity is community-acquired pneumonia (CAP). Because of the constant growth in the population with both diseases (CAP and COPD), analyzing their clinical characteristics is important. Several cellular factors are known to contribute to differences in clinical expression: some lead to COPD exacerbations while others lead to symptoms of pneumonia. The use of new biomarkers (procalcitonin, pro-adrenomedullin and copeptin) help to distinguish among these clinical pictures. To decrease morbidity and mortality, clinical guidelines on antibiotic therapy must be followed and this therapy should be prescribed to patients with CAP and COPD. There are also prevention measures such as the pneumococcal vaccine whose role in the prevention of pneumococcal CAP should be further studied. The present review aims to elucidate some of the above-mentioned issues. PMID:20620690

  16. Osteoporosis Associated with Chronic Obstructive Pulmonary Disease

    PubMed Central

    Watanabe, Reiko; Inoue, Daisuke

    2016-01-01

    Recent epidemiological studies have revealed that osteoporosis is closely associated with common chronic diseases including diabetes, hypertension, chronic kidney disorders, and chronic obstructive pulmonary disease (COPD). COPD is a chronic inflammatory airway disease but now well known to be associated with various systemic comorbidities including osteoporosis. Osteoporosis and osteoporotic fractures are extremely common in COPD patients, which have significant impacts on their quality of life (QOL), activities of daily life (ADL), respiratory function, and possibly their prognosis. COPD-associated osteoporosis is however extremely under-recognized, hence undertreated. Recent studies have suggested that both decreased bone mineral density (BMD) and impaired bone quality compromise bone strength causing fractures in COPD. In COPD patients, various general clinical risk factors for osteoporosis are present including smoking, older age, low body weight, and physical inactivity. In addition, disease-related risk factors such as decreased pulmonary function, inflammation, glucocorticoid use and vitamin D deficiency/insufficiency have been linked to the development of osteoporosis in COPD. Increased awareness of osteoporosis in COPD, especially that of high prevalence of vertebral fractures is called upon among general physicians as well as pulmonologists. Routine screening for osteoporosis and risk assessment of fractures will enable physicians to diagnose COPD patients with comorbid osteoporosis at an early stage. Timely prevention of developing osteoporosis together with appropriate treatment of established osteoporosis may improve QOL and ADL of the COPD patients, preserve their lung function and eventually result in better prognosis in these patients. PMID:27622174

  17. Osteoporosis Associated with Chronic Obstructive Pulmonary Disease.

    PubMed

    Okazaki, Ryo; Watanabe, Reiko; Inoue, Daisuke

    2016-08-01

    Recent epidemiological studies have revealed that osteoporosis is closely associated with common chronic diseases including diabetes, hypertension, chronic kidney disorders, and chronic obstructive pulmonary disease (COPD). COPD is a chronic inflammatory airway disease but now well known to be associated with various systemic comorbidities including osteoporosis. Osteoporosis and osteoporotic fractures are extremely common in COPD patients, which have significant impacts on their quality of life (QOL), activities of daily life (ADL), respiratory function, and possibly their prognosis. COPD-associated osteoporosis is however extremely under-recognized, hence undertreated. Recent studies have suggested that both decreased bone mineral density (BMD) and impaired bone quality compromise bone strength causing fractures in COPD. In COPD patients, various general clinical risk factors for osteoporosis are present including smoking, older age, low body weight, and physical inactivity. In addition, disease-related risk factors such as decreased pulmonary function, inflammation, glucocorticoid use and vitamin D deficiency/insufficiency have been linked to the development of osteoporosis in COPD. Increased awareness of osteoporosis in COPD, especially that of high prevalence of vertebral fractures is called upon among general physicians as well as pulmonologists. Routine screening for osteoporosis and risk assessment of fractures will enable physicians to diagnose COPD patients with comorbid osteoporosis at an early stage. Timely prevention of developing osteoporosis together with appropriate treatment of established osteoporosis may improve QOL and ADL of the COPD patients, preserve their lung function and eventually result in better prognosis in these patients. PMID:27622174

  18. Dust events, pulmonary diseases and immune system

    PubMed Central

    Esmaeil, Nafiseh; Gharagozloo, Marjan; Rezaei, Abbas; Grunig, Gabriele

    2014-01-01

    Incidences of sand storms have increased in recent years and there is evidence that these dusts can move across long distances. Sand dusts have different adverse effects on health, but one of the most important of them is pulmonary disease. After inhalation of dust, many dust particles are moved to the airways. Dust particles can be sensed by airways epithelial cells, activate macrophages, dendritic cells and innate immune cells and then initiate responses in various populations of specific immune cells such as T helper cells subsets (Th1, Th2, Th17), T cytotoxic cells and B cells. Initiation of inflammatory immune responses, activation of immune cells and releases of many cytokines, chemokines and other inflammatory molecules, have variable pathologic affects on lung in different respiratory diseases. Unfortunately control of desert dusts is more difficult than control of air pollution. For prevention and treatment of respiratory diseases that are caused by desert dusts, researchers need well-designed epidemiological studies, combined with analysis of the precise composition of sand dusts, and the precise mechanisms of the immune responses. Recognizing the exact cellular and molecular immune mechanisms would be very useful to find new approaches for treatment of desert dust associated pulmonary diseases. PMID:24660118

  19. Asbestos-related pulmonary disease in boilermakers

    SciTech Connect

    Demers, R.Y.; Neale, A.V.; Robins, T.; Herman, S.C. )

    1990-01-01

    Boilermakers are skilled building tradesmen who construct, repair, and dismantle boilers. The present study reports on the evaluation of members of a Michigan boilermaker's union for the presence of signs and symptoms of chronic pulmonary disease. Study variables included standardized evaluations of chest x-ray findings, pulmonary function testing, physical examination, and respiratory symptoms. An overall participation rate of 69% was achieved. A non-participant survey identified no significant differences between participants and non-participants in dyspnea, cough, age, or smoking history. Among participants with greater than 20 years experience in the trade, the mean FVC was 91% of predicted; the FEV1 was 86% of predicted; 25% showed at least a 1/0 profusion of interstitial markings on chest x-ray; 30% had bilateral pleural abnormalities; and 52% had audible inspiratory rales on physical examination. Forced vital capacity and forced expiratory volume at one second both decreased with years in the trade. Chest x-ray findings of interstitial fibrosis and pleural plaques were related to ten or more years in the trade, as were respiratory symptoms of pulmonary rales, wheeze, and dyspnea.

  20. Prevalence of Nontuberculous Mycobacterial Pulmonary Disease, Germany, 2009–2014

    PubMed Central

    Wagner, Dirk; de Roux, Andrés; Diel, Roland; Hohmann, David; Hickstein, Lennart; Welte, Tobias; Rademacher, Jessica

    2016-01-01

    We analyzed routine statutory health insurance claim data to determine prevalence of nontuberculous mycobacterial pulmonary disease in Germany. Documented prevalence rates of this nonnotifiable disease increased from 2.3 to 3.3 cases/100,000 population from 2009 to 2014. Prevalence showed a strong association with advanced age and chronic obstructive pulmonary disease. PMID:27191473

  1. Prevalence of Nontuberculous Mycobacterial Pulmonary Disease, Germany, 2009-2014.

    PubMed

    Ringshausen, Felix C; Wagner, Dirk; de Roux, Andrés; Diel, Roland; Hohmann, David; Hickstein, Lennart; Welte, Tobias; Rademacher, Jessica

    2016-06-01

    We analyzed routine statutory health insurance claim data to determine prevalence of nontuberculous mycobacterial pulmonary disease in Germany. Documented prevalence rates of this nonnotifiable disease increased from 2.3 to 3.3 cases/100,000 population from 2009 to 2014. Prevalence showed a strong association with advanced age and chronic obstructive pulmonary disease. PMID:27191473

  2. Chronic obstructive pulmonary disease and left ventricle.

    PubMed

    Portillo, Karina; Abad-Capa, Jorge; Ruiz-Manzano, Juan

    2015-05-01

    Several studies have shown that the interaction between chronic obstructive pulmonary disease (COPD) and cardiovascular comorbidity is complex and bidirectional, since each of these diseases complicates the prognosis of the other. Recent advances in imaging technology have led to better characterization of cardiac chambers and allowed the relationship between certain cardiac function parameters and COPD clinical and functional variables to be explored. Although cardiac abnormalities in COPD have been mainly associated with the right ventricle, several studies have reported that the left ventricle may also be affected in this disease. A better understanding of the mechanisms involved and their clinical implications will establish diagnostic and therapeutic strategies for patients with both these conditions. PMID:24816034

  3. Early origins of chronic obstructive pulmonary disease.

    PubMed

    Narang, Indra; Bush, Andrew

    2012-04-01

    Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality worldwide and a significant challenge for adult physicians. However, there is a misconception that COPD is a disease of only adult smokers. There is a growing body of evidence to support the hypothesis that chronic respiratory diseases such as COPD have their origins in early life. In particular, adverse maternal factors will interact with the environment in a susceptible host promoting altered lung growth and development antenatally and in early childhood. Subsequent lung injury and further gene-environment interactions may result in permanent lung injury manifest by airway obstruction predisposing to COPD. This review will discuss the currently available data regarding risk factors in early life and their role in determining the COPD phenotype. PMID:22265926

  4. Pathophysiology and treatment of pulmonary hypertension in sickle cell disease.

    PubMed

    Gordeuk, Victor R; Castro, Oswaldo L; Machado, Roberto F

    2016-02-18

    Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric oxide deficiency state and vasculopathy consequent to intravascular hemolysis, (2) chronic pulmonary thromboembolism, or (3) upregulated hypoxic responses secondary to anemia, low O2 saturation, and microvascular obstruction. The remainder have postcapillary pulmonary hypertension secondary to left ventricular dysfunction. Although the pulmonary artery pressure in SCD patients with pulmonary hypertension is only moderately elevated, they have a markedly higher risk of death than patients without pulmonary hypertension. Guidelines for diagnosis and management of SCD-related pulmonary hypertension were published recently by the American Thoracic Society. Management of adults with sickle-related pulmonary hypertension is based on anticoagulation for those with thromboembolism; oxygen therapy for those with low oxygen saturation; treatment of left ventricular failure in those with postcapillary pulmonary hypertension; and hydroxyurea or transfusions to raise the hemoglobin concentration, reduce hemolysis, and prevent vaso-occlusive events that cause additional increases in pulmonary pressure. Randomized trials have not identified drugs to lower pulmonary pressure in SCD patients with precapillary pulmonary hypertension. Patients with hemodynamics of pulmonary arterial hypertension should be referred to specialized centers and considered for treatments known to be effective in other forms of pulmonary arterial hypertension. There have been reports that some of these treatments

  5. Improving Outcomes for Pulmonary Vascular Disease

    PubMed Central

    Robbins, Ivan M.; Blaisdell, Carol J.; Abman, Steven H.

    2012-01-01

    Recognizing the importance of improving lung health through lung disease research, the National Heart, Lung, and Blood Institute (NHLBI) convened a workshop of multidisciplinary experts for the following purpose: (1) to review the current scientific knowledge underlying the basis for treatment of adults and children with pulmonary vascular diseases (PVDs); (2) to identify gaps, barriers, and emerging scientific opportunities in translational PVD research and the means to capitalize on these opportunities; (3) to prioritize new research directions that would be expected to affect the clinical course of PVDs; and (4) to make recommendations to the NHLBI on how to fill identified gaps in adult and pediatric PVD clinical research. Workshop participants reviewed experiences from previous PVD clinical trials and ongoing clinical research networks with other lung disorders, including acute respiratory distress syndrome, chronic obstructive lung disease, and idiopathic pulmonary fibrosis, as well. Bioinformatics experts discussed strategies for applying cutting-edge health information technology to clinical studies. Participants in the workshop considered approaches in the following broad concept areas: (1) improved phenotyping to identify potential subjects for appropriate PVD clinical studies; (2) identification of potential new end points for assessing key outcomes and developing better-designed PVD clinical trials; and (3) the establishment of priorities for specific clinical research needed to advance care of patients with various subsets of PVDs from childhood through adulthood. This report provides a summary of the objectives and recommendations to the NHLBI concentrating on clinical research efforts that are needed to better diagnose and treat PVDs. PMID:22335936

  6. Evolving Concepts of Pulmonary Hypertension Secondary to Left Heart Disease.

    PubMed

    Ramu, Bhavadharini; Thenappan, Thenappan

    2016-04-01

    Pulmonary hypertension associated with left heart disease is the most common form of pulmonary hypertension. Although its pathophysiology remains incompletely understood, it is now well recognized that the presence of pulmonary hypertension is associated with a worse prognosis. Right ventricular failure has independent and additive prognostic value over pulmonary hypertension for adverse outcomes in left heart disease. Recently, several new terminologies have been introduced to better define and characterize the nature and severity of pulmonary hypertension. Several new treatment options including the use of pulmonary arterial hypertension specific therapies are being considered, but there is lack of evidence. Here, we review the recent advances in this field and summarize the diagnostic and therapeutic modalities of use in the management of pulmonary hypertension associated with left heart disease. PMID:26886381

  7. [Pathogenesis of chronic obstructive pulmonary disease].

    PubMed

    Vogelmeier, C; Koczulla, R; Fehrenbach, H; Bals, R

    2006-09-01

    It is currently believed that the most important factor in the pathogenesis of chronic obstructive pulmonary disease (COPD) is inflammation of the small airways caused by inhaled particles and gases. In this context, a disturbance of the physiological balance between proteases and antiproteases develops that may cause lung emphysema. Moreover, oxidative stress seems to be important, as it may enhance the inflammatory reaction. The development of emphysema may also involve a loss of alveolar cells by apoptosis. Finally, several studies have indicated that a systemic inflammation is induced by COPD that may be of relevance to the development of systemic components that are observed in COPD patients. PMID:16845536

  8. Combined pulmonary fibrosis and emphysema: effect of pulmonary rehabilitation in comparison with chronic obstructive pulmonary disease

    PubMed Central

    Tomioka, Hiromi; Mamesaya, Nobuaki; Yamashita, Shyuji; Kida, Yoko; Kaneko, Masahiro; Sakai, Hideki

    2016-01-01

    Objective To evaluate the effectiveness of short-term comprehensive inpatient pulmonary rehabilitation for patients with combined pulmonary fibrosis and emphysema (CPFE), and to compare responses with those of patients with chronic obstructive pulmonary disease (COPD) who underwent an identical programme. Design Retrospective analysis of several outcome measures. Setting Pulmonary ward at a 358-bed community teaching hospital. Methods 3-week inpatient pulmonary rehabilitation programme assessed by pulmonary function tests, 6 min walk test and health-related quality of life (HRQL) using the Short Form-36 (SF-36). Results 17 patients with CPFE and 49 patients with COPD were referred to and completed the programme between March 2007 and February 2015. Age, sex, smoking status, body mass index and the Medical Research Council dyspnoea grade were comparable between groups. In the CPFE group, improvement from the start of the programme to the programme end was observed in forced expiratory volume in 1 s (FEV1) (from 1.7±0.4 to 1.8±0.4, p=0.034); however, there was no significant improvement in the 6 min walk test (distance, SpO2 nadir and Borg scale on exercise). With regard to HRQL, improvement was observed in physical function (p=0.015) whereas deterioration was observed in social functioning (p=0.044). In the COPD group, significant improvement was observed after the programme in the FEV1, 6 min walk test and 4 of the 8 SF-36 subscales. There was a significant difference in changes in the 6 min walk distance: −16.6±58.4 in CPFE versus 30.2±55.6 in COPD (p=0.009). In 2 domains, there was a significant difference in SF-36 scores between groups: Δvitality, −6.3±22.4 in CPFE versus 11.3±21.1 in COPD, p=0.009; and Δsocial functioning, −18.8±34.2 in CPFE versus 5.3±35.9 in COPD, p=0.027. Conclusion Patients with COPD derived greater benefits than those with CPFE, from the relatively short periods of inpatient pulmonary rehabilitation. PMID:26933506

  9. Respiratory Conditions Update: Chronic Obstructive Pulmonary Disease.

    PubMed

    Karel, Daphne J

    2016-09-01

    Chronic obstructive pulmonary disease (COPD) is defined as persistent airflow limitation due to irritant-induced chronic inflammation. A postbronchodilator forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) ratio of 0.7 or less is diagnostic in a patient with dyspnea, chronic cough or sputum production, and a history of irritant exposure. Tobacco smoking is the most significant etiology, and smoking cessation is the only intervention shown to slow disease progression. Long-acting beta2-agonists and long-acting muscarinic antagonists are first-line treatments for patients with persistently symptomatic COPD with an FEV1 of 80% or less of predicted. When COPD is uncontrolled with a long-acting bronchodilator, combination therapy with a long-acting muscarinic antagonist-long-acting beta2-agonist or long-acting beta2-agonist-inhaled corticosteroid should be prescribed. Patients with COPD and reduced exercise tolerance should undergo pulmonary rehabilitation and be evaluated for supplemental oxygen therapy. Other treatment options for persistently symptomatic COPD include inhaler triple therapy (ie, long-acting muscarinic antagonist, long-acting beta2-agonist, inhaled corticosteroid), phosphodiesterase type 4 inhibitors, oxygen, and surgical interventions. PMID:27576232

  10. Pulmonary hypertension associated with lung diseases and hypoxemia.

    PubMed

    Cuttica, Michael J

    2016-05-01

    Pulmonary hypertension that develops in the setting of underlying lung diseases such as COPD or idiopathic pulmonary fibrosis (IPF) is associated with decreased functional status, worsening hypoxemia and quality of life, and increased mortality. This complication of lung disease is complex in its origin and carries a unique set of diagnostic and therapeutic issues. This review attempts to provide an overview of mechanisms associated with the onset of pulmonary hypertension in COPD and IPF, touches on appropriate evaluation, and reviews the state of knowledge on treating pulmonary hypertension related to underlying lung disease. PMID:27086030

  11. Treatment of stable chronic obstructive pulmonary disease.

    PubMed

    Rennard, Stephen I

    Chronic obstructive pulmonary disease (COPD) is a readily diagnosable disorder that responds to treatment. Smoking cessation can reduce symptoms and prevent progression of disease. Bronchodilator therapy is key in improvement of lung function. Three classes of bronchodilators-beta agonists, anticholinergics, and theophylline-are available and can be used individually or in combination. Inhaled glucocorticoids can also improve airflow and can be combined with bronchodilators. Inhaled glucocorticoids, in addition, might reduce exacerbation frequency and severity as might some bronchodilators. Effective use of pharmacotherapy in COPD needs integration with a rehabilitation programme and successful treatment of co-morbidities, including depression and anxiety. Treatment for stable COPD can improve the function and quality of life of many patients, could reduce admissions to hospital, and has been suggested to improve survival. PMID:15337408

  12. [Chronic obstructive pulmonary disease : new pharmacotherapeutic options].

    PubMed

    Greulich, T; Koczulla, A R; Vogelmeier, C

    2012-11-01

    Data about the clinical presentation of chronic obstructive pulmonary disease (COPD) have resulted in a new classification of the disease. The degree of airflow limitation has been amended by symptoms and exacerbation rate. The standard pharmacotherapy of stable COPD is in transition, as fixed combinations of long acting beta agonists and long acting anticholinergics are in the late stages of clinical development. On this background inhaled corticosteroids will need to be re-evaluated. Roflumilast is a recently approved therapeutic option that primarily diminishes exacerbation frequency in patients with chronic bronchitis and severe airflow obstruction (FEV(1) < 50%). In COPD patients with acute exacerbations procalcitonin levels can be used to guide antibiotic therapy. Comparable clinical outcomes can be achieved while using significantly less amounts of antibiotics. PMID:22955248

  13. Integrative Genomics of Chronic Obstructive Pulmonary Disease

    PubMed Central

    Hobbs, Brian D.; Hersh, Craig P.

    2014-01-01

    Chronic obstructive pulmonary disease (COPD) is a complex disease with both environmental and genetic determinants, the most important of which is cigarette smoking. There is marked heterogeneity in the development of COPD among persons with similar cigarette smoking histories, which is likely partially explained by genetic variation. Genomic approaches such as genomewide association studies and gene expression studies have been used to discover genes and molecular pathways involved in COPD pathogenesis; however, these “first generation” omics studies have limitations. Integrative genomic studies are emerging which can combine genomic datasets to further examine the molecular underpinnings of COPD. Future research in COPD genetics will likely use network-based approaches to integrate multiple genomic data types in order to model the complex molecular interactions involved in COPD pathogenesis. This article reviews the genomic research to date and offers a vision for the future of integrative genomic research in COPD. PMID:25078622

  14. Pharmacological treatment of chronic obstructive pulmonary disease

    PubMed Central

    Montuschi, Paolo

    2006-01-01

    None of the drugs currently available for chronic obstructive pulmonary disease (COPD) are able to reduce the progressive decline in lung function which is the hallmark of this disease. Smoking cessation is the only intervention that has proved effective. The current pharmacological treatment of COPD is symptomatic and is mainly based on bronchodilators, such as selective β2-adrenergic agonists (short- and long-acting), anticholinergics, theophylline, or a combination of these drugs. Glucocorticoids are not generally recommended for patients with stable mild to moderate COPD due to their lack of efficacy, side effects, and high costs. However, glucocorticoids are recommended for severe COPD and frequent exacerbations of COPD. New pharmacological strategies for COPD need to be developed because the current treatment is inadequate. PMID:18044097

  15. Chronic Obstructive Pulmonary Disease: An Overview

    PubMed Central

    Devine, John F.

    2008-01-01

    Chronic obstructive pulmonary disease is a growing healthcare problem that is expected to worsen as the population ages and the worldwide use of tobacco products increases. Smoking cessation is the only effective means of prevention. Employers are in a unique position to help employees stop smoking. During the long asymptomatic phase, lung function nevertheless continues to decline; therefore, many patients seek medical attention only when they are at an advanced stage or when they have experienced an acute exacerbation. To help preserve patients' quality of life and reduce healthcare costs related to this chronic disease, clinicians need to accurately diagnose the condition and appropriately manage patients through the long course of their illness. This article discusses the current approach to patient management. PMID:25126252

  16. Chronic obstructive pulmonary disease: an overview.

    PubMed

    Duncan, Deborah

    As chronic obstructive pulmonary disease (COPD) is one of the major causes of worldwide mortality, it is important to prevent, diagnose and manage it. COPD creates a huge burden on the NHS and has a significant impact on patients. This is a problem with the increase in morbidity and mortality rates. In primary care there is a lack of knowledge, under-use of quality-assured spirometry and under-diagnosis in about half of all cases. To be able to effectively diagnose, assess and manage COPD, health professionals must understand the physiology and aetiology of the disease. COPD is similar to asthma in its presentation and physiology but management of the condition can differ. The authors therefore looked at the similarities between the two conditions and what tests one can use to make a diagnosis of COPD. PMID:27081728

  17. Recent updates in chronic obstructive pulmonary disease.

    PubMed

    Garvey, Christine

    2016-01-01

    Chronic obstructive pulmonary disease (COPD), characterized by chronic airways inflammation and progressive airflow limitation, is a common, preventable and treatable disease. Worldwide, COPD is a major cause of morbidity and mortality; smoking tobacco is the most important risk factor. This translational review of recent updates in COPD care for the primary care audience, includes recommendations from the 2015 Global Initiative for chronic obstructive lung disease (GOLD) report on diagnosis, pharmacological and non-pharmacological treatment, prevalence of comorbidities, management of exacerbations and the asthma and COPD overlap syndrome, with a focus on the importance and benefit of physical activity and exercise in COPD patients. Exacerbations and comorbidities contribute to the overall severity of COPD in individual patients. Management of exacerbations includes reducing the impact of the current exacerbation and preventing development of subsequent episodes. Healthcare professionals need to be alert to comorbidities, such as cardiovascular disease, anxiety/depression, lung cancer, infections and diabetes, which are common in COPD patients and can have a significant impact on HRQoL and prognosis. Pulmonary rehabilitation is recommended by a number of guidelines for all symptomatic COPD patients, regardless of severity, and involves exercise training, patient education, nutritional advice and psychosocial support. At all stages of COPD, regular physical activity and exercise can aid symptom control, improve HRQoL, reduce rates of hospitalization, and improve morbidity and respiratory mortality. Healthcare professionals play a pivotal role in improving HRQoL and health-related outcomes in COPD patients to meet their specific needs and in providing appropriate diagnosis, management and advice on smoking cessation. PMID:26560514

  18. [Pulmonary hemorrhage associated with celiac disease].

    PubMed

    Testa, María Eugenia; Maffey, Alberto; Colom, Alejandro; Agüero, Luis; Rogé, Ignacio; Andrewartha, María Sol; Teper, Alejandro

    2012-08-01

    Idiopathic pulmonary hemosiderosis is a severe and potentially fatal disease characterized by recurrent episodes of alveolar hemorrhage, hemoptysis, and anemia. His association with celiac disease, described as Lane- Hamilton syndrome, could be due to the fact that both entities share a common pathogenic immune pathway. We report two patients of 13 years who consulted for hemoptysis and severe anemia that had not responded to immunosuppressive treatment with pulses of methyl prednisolone, oral meprednisone and hydroxychloroquine. Although both children highlight the absence of gastrointestinal symptoms at the time of consultation, the dosage of anti-endomysial and anti-transglutaminase antibodies was positive and biopsy confirmed the presence of intestinal enteropathy. It is emphasized that in patients with diffuse alveolar hemorrhage, even in the absence of gastrointestinal symptoms, the concomitant presence of celiac disease should be evaluated. If celiac disease is present, the incorporation of a gluten-free diet helps to control the symptoms, allows reducing the immunosuppressive treatment and improves the clinical course of both entities. PMID:22859336

  19. Peptides in Bronchoalveolar Lavage in Chronic Obstructive Pulmonary Disease

    PubMed Central

    Wendt, Chris H.; Nelsestuen, Gary; Harvey, Stephen; Gulcev, Makedonka; Stone, Matthew; Reilly, Cavan

    2016-01-01

    Background Chronic Obstructive Pulmonary Disease (COPD) is a heterogeneous disease with a significant public health burden. Currently there is no biomarker that identifies those at risk of developing COPD, progression of disease or disease phenotypes. We performed metabolomic profiling of bronchoalveolar lavage fluid (BALF) from COPD patients to determine if metabolites correlated with clinical measurements such as lung function, functional status and degree of emphysema. Methods Metabolomic components of BALF from 59 subjects with COPD and 20 healthy controls were separated by reversed-phase UPLC and analyzed by ESI-ToF mass spectrometry. We used univariate analysis and multiple regression models to investigate associations between metabolomic features and various clinical variables, such as lung function, functional status as measured by the St. George Respiratory Quotient Score and emphysema as measured by the CT density mask score. Results We identified over 3900 features by mass spectrometry, many consistent with peptides. Subjects with severe COPD had increased concentration of peptides compared to controls (p < 9.526e-05). The peptide concentration correlated with spirometry, specifically pulmonary function tests associated with airflow obstruction. There was no correlation with CT density, i.e. emphysema, or functional status. Conclusions Metabolomic profiling of BALF in COPD patients demonstrated a significant increase in peptides compared to healthy controls that associated strongly to lung function, but not emphysema or functional status. PMID:27227774

  20. [Anemias in chronic obstructive pulmonary disease].

    PubMed

    Budnevsky, A V; Esaulenko, I E; Ovsyannikov, E S; Zhusina, Yu G

    2016-01-01

    According to different studies, anemia occurs in 8--33% of patients with chronic obstructive pulmonary disease (COPD). The paper describes the most important various causes of anemia in COPD, such as systemic inflammation and endocrine disorders, the use of some medications (theophylline, angiotensin-converting enzyme inhibitors), frequent COPD exacerbations, and long-term oxygen therapy. Lower hemoglobin levels in COPD patients are accompanied by increased shortness of breath, reduced exercise tolerance, and lower quality of life. Furthermore, some investigations have shown that anemia is an independent predictor of death in patients with COPD. In spite of the fact that anemia may be successfully in these patients, the evidence suggesting the importance of its impact on the prognosis of COPD is limited. PMID:27191018

  1. Pulmonary arterial hypertension in connective tissue diseases.

    PubMed

    Goldberg, Avram

    2010-01-01

    Pulmonary arterial hypertension (PAH) is an entity that is known to complicate connective tissue diseases (CTD). PAH in CTD is a very important diagnosis which greatly affects treatment and prognosis. The most commonly affected CTD is scleroderma, although lupus, inflammatory myopathies such as poly and dermatomyositis, and mixed CTD are also associated with PAH. The manifestations of PAH have both similarities and differences when occurring in the setting of CTD as compared with idiopathic PAH. These differences are most notable in scleroderma. In this section we will discuss the features of PAH as they appear in CTDs, and in particular, scleroderma. The focus of this article is an approach to the diagnosis and treatment of PAH in CTD, and how this setting might differ from idiopathic and other forms of PAH. PMID:20160534

  2. The pathology of chronic obstructive pulmonary disease.

    PubMed

    Hogg, James C; Timens, Wim

    2009-01-01

    The pathogenesis of chronic obstructive pulmonary disease (COPD) is based on the innate and adaptive inflammatory immune response to the inhalation of toxic particles and gases. Although tobacco smoking is the primary cause of this inhalation injury, many other environmental and occupational exposures contribute to the pathology of COPD. The immune inflammatory changes associated with COPD are linked to a tissue-repair and -remodeling process that increases mucus production and causes emphysematous destruction of the gas-exchanging surface of the lung. The common form of emphysema observed in smokers begins in the respiratory bronchioles near the thickened and narrowed small bronchioles that become the major site of obstruction in COPD. The mechanism(s) that allow small airways to thicken in such close proximity to lung tissue undergoing emphysematous destruction remains a puzzle that needs to be solved. PMID:18954287

  3. Skeletal Implications of Chronic Obstructive Pulmonary Disease.

    PubMed

    Misof, Barbara M; Moreira, Carolina A; Klaushofer, Klaus; Roschger, Paul

    2016-04-01

    Chronic obstructive pulmonary disease (COPD) is associated with numerous comorbidities, among which osteoporosis is of high significance. Low bone mass and the occurrence of fragility fractures is a common finding in patients with COPD. Typical risk factors related directly or indirectly to these skeletal complications include systemic inflammation, tobacco smoking, vitamin D deficiency, and treatment with oral or inhaled corticosteroids. In particular, treatment with glucocorticoids appears to be a strong contributor to bone changes in COPD, but does not fully account for all skeletal complications. Additional to the effects of COPD on bone mass, there is evidence for COPD-related changes in bone microstructure and material properties. This review summarizes the clinical outcomes of low bone mass and increased fracture risk, and reports on recent observations in bone tissue and material in COPD patients. PMID:26861899

  4. Rare lung diseases II: Pulmonary alveolar proteinosis

    PubMed Central

    Juvet, Stephen C; Hwang, David; Waddell, Thomas K; Downey, Gregory P

    2008-01-01

    The present article is the second in a series on rare lung diseases. It focuses on pulmonary alveolar proteinosis (PAP), a disorder in which lipoproteinaceous material accumulates in the alveolar space. PAP was first described in 1958, and for many years the nature of the material accumulating in the lungs was unknown. Major insights into PAP have been made in the past decade, and these have led to the notion that PAP is an autoimmume disorder in which autoantibodies interfere with signalling through the granulocyte-macrophage colony-stimulating factor receptor, leading to macrophage and neutrophil dysfunction. This has spurred new therapeutic approaches to this disorder. The discussion of PAP will begin with a case report, then will highlight the classification of PAP and review recent insights into the pathogenesis of PAP. The approach to therapy and the prognosis of PAP will also be discussed. PMID:18551202

  5. Palliative care in chronic obstructive pulmonary disease.

    PubMed

    Lilly, Evan J; Senderovich, Helen

    2016-10-01

    Chronic obstructive pulmonary disease (COPD) is the only major worldwide cause of mortality that is currently increasing in prevalence. Furthermore, COPD is incurable, and the only therapy that has been shown to increase survival is oxygen therapy in selected patients. Compared to patients with cancer, patients with COPD experience similar levels of pain, breathlessness, fatigue, depression, and anxiety and have a worse quality of life but have comparatively little access to palliative care. When these patients do receive palliative care, they tend to be referred later than patients with cancer. Many disease, patient-, and provider-related factors contribute to this phenomenon, including COPD's unpredictable course, misperceptions of palliative care among patients and physicians, and lack of advance care planning discussions outside of crisis situations. A new paradigm for palliative care would introduce palliative treatments alongside, rather than at the exclusion of disease-modifying interventions. This integrated approach would circumvent the issue of difficult prognostication in COPD, as any patient would receive individualized palliative interventions from the time of diagnosis. These points will be covered in this review, which discusses the challenges in providing palliative care to COPD patients, the strategies to mitigate the challenges, management of common symptoms, and the evidence for integrated palliative care models as well as some suggestions for future development. PMID:27481751

  6. Chronic Obstructive Pulmonary Disease in the elderly.

    PubMed

    Incalzi, Raffaele Antonelli; Scarlata, Simone; Pennazza, Giorgio; Santonico, Marco; Pedone, Claudio

    2014-04-01

    The prevalence of Chronic Obstructive Pulmonary Disease (COPD) dramatically increases with age, and COPD complicated by chronic respiratory failure may be considered a geriatric condition. Unfortunately, most cases remain undiagnosed because of atypical clinical presentation and difficulty with current respiratory function diagnostic standards. Accordingly, the disease is under-recognized and undertreated. This is expected to impact noticeably the health status of unrecognized COPD patients because a timely therapy could mitigate the distinctive and important effects of COPD on the health status. Comorbidity also plays a pivotal role in conditioning both the health status and the therapy of COPD besides having major prognostic implication. Several problems affect the overall quality of the therapy for the elderly with COPD, and current guidelines as well as results from pharmacological trials only to some extent apply to this patient. Finally, physicians of different specialties care for the elderly COPD patient: physician's specialty largely determines the kind of approach. In conclusion, COPD, in itself a complex disease, becomes difficult to identify and to manage in the elderly. Interdisciplinary efforts are desirable to provide the practicing physician with a multidisciplinary guide to the identification and treatment of COPD. PMID:24183233

  7. Pulmonary disease caused by nontuberculous mycobacteria.

    PubMed

    Weiss, Curtis H; Glassroth, Jeffrey

    2012-12-01

    The propensity of various nontuberculous mycobacteria to cause lung disease varies widely and is conditioned by host factors; infection is believed to occur from environmental sources. Nontuberculous mycobacteria pulmonary disease (PNTM) is increasing worldwide and Mycobacterium avium complex is the most common cause. PNTM usually occurs in one of three prototypical forms: hypersensitivity pneumonitis, cavitary tuberculosis-like disease or nodular bronchiectasis. PNTM has been linked in some patients to genetic variants of the cystic fibrosis transmembrane conductance regulator gene and a distinct patient phenotype. Interactions between PNTM and other comorbidities are also increasingly appreciated. Guidelines for diagnosis, emphasizing chest imaging and microbiology, have been published; speciation using molecular techniques is critical for accuracy and for treatment decisions. Clinical trials are lacking to inform treatment for many species and experience with M. avium complex and several others species serves as a guide instead. Use of multiple drugs for a period of at least 12 months following sputum conversion is the norm for most species. In vitro drug susceptibility results for many drugs may not correlate with clinical outcomes and such testing should be done on a selective basis. PMID:23234447

  8. Two case reports of Castleman disease with pulmonary involvement.

    PubMed

    Zhang, H; Zhang, J-Q; Zhong, X-N; Liu, G-N; Li, M-H; Bai, J; He, Z-Y; Deng, J-M

    2015-03-01

    Castleman disease (CD) is a rare reactive lymphoproliferative disorder, first identified in 1954. We recently had the opportunity to analyse the characteristics of two variations of CD with pulmonary involvement. Case 1 had localised retroperitoneal hyaline vascular type CD, while Case 2 was diagnosed as multicentric plasma cell type CD. Both patients had pulmonary symptoms and signs, including cough, dyspnoea, hypoxaemia and ventilatory dysfunction; however, they had different physiological manifestations of their pulmonary abnormalities. PMID:25686148

  9. Genetics of chronic obstructive pulmonary disease.

    PubMed

    Silverman, E K

    2001-01-01

    The marked variability in the development of chronic obstructive pulmonary disease (COPD) in response to cigarette smoking has been known for decades, but severe alpha 1-antitrypsin deficiency (PI Z) remains the only proven genetic risk factor for COPD. With cigarette smoking, PI Z subjects tend to develop more severe pulmonary impairment at an earlier age than non-smoking PI Z individuals. However, PI Z individuals exhibit wide variability in pulmonary function impairment, even among individuals with similar smoking histories. Therefore, other genes and environmental exposures are also likely involved. The role of heterozygosity for the Z allele as a risk factor for COPD remains controversial, but accumulating evidence suggests that at least some PI MZ individuals are at increased risk of developing airflow obstruction. In individuals without alpha 1-antitrypsin deficiency, familial aggregation of COPD has been reported in several studies. To study novel genetic determinants of COPD, our research group enrolled 44 severe, early-onset COPD probands (FEV1 < 40%, age < 53 yrs, non-PI Z) and 266 of their relatives. A marked female predominance was noted among the early-onset COPD probands. In addition, increased risk to current or ex-smoking first-degree relatives of early-onset COPD probands for reduced FEV1, chronic bronchitis and spirometric bronchodilator responsiveness has been demonstrated. These data strongly support the genetic basis for the development of COPD and the potential for gene-by-environment interaction. A variety of studies have examined candidate gene loci with association studies, comparing the distribution of variants in genes hypothesized to be involved in the development of COPD in COPD patients and control subjects. For most genetic loci which have been tested, there have been inconsistent results. Genetic heterogeneity could contribute to difficulty in replicating associations between studies. In addition, case-control association studies

  10. Clinical Analysis of Pulmonary Nocardiosis in Patients With Autoimmune Disease.

    PubMed

    Li, Shan; Song, Xin yu; Zhao, Yu yue; Xu, Kai; Bi, Ya Lan; Huang, Hui; Xu, Zuo jun

    2015-09-01

    Nocardiosis is an opportunistic infection that most commonly involves the lung; however, only a few case reports of autoimmune disease complicated by pulmonary nocardiosis exist in the literature. We conducted a retrospective analysis of 24 cases of both autoimmune disease and pulmonary nocardiosis at the Peking Union Medical College Hospital between 1990 and 2012. Fifty-two cases were hospitalized with nocardiosis, 24 of whom had at least 1 autoimmune disease before the diagnosis of pulmonary nocardiosis. The cohort patients consisted of 5 men and 19 women, with a mean age of 44.2 years. All were negative for human immunodeficiency virus. All but 1 patient had received immunosuppressants, including corticosteroids, cyclophosphamide, azathioprine, methotrexate, or hydroxychloroquine. Fever (87.5%), cough (83.3%), and sputum (79.2%) were the most common clinical manifestations. Ten cases were accompanied by subcutaneous nodules and/or cutaneous abscesses, and 4 had brain abscess. Half of them were lymphocytopenic. Thirteen of the 16 cases who underwent lymphocyte subtype analysis had decreased CD4+ T-cell counts. Nineteen cases had decreased serum albumin levels. Nocardia was isolated from sputum (13/24), bronchoalveolar lavage fluid (4/6), lung tissue (5/6), pleural effusions (3/5), skin or cutaneous pus (7/10), and brain tissue (1/1). The most common imaging findings were air-space opacities (83.3%), followed by nodules (62.5%), cavitations (45.8%), and masses (37.5%). Five were administered co-trimoxazole only, and the others were treated with 2 or more antibiotics. All 5 cases with skin abscesses and 2 of the 4 cases with brain abscesses were treated by surgical incision and drainage. None underwent thoracic surgery. Corticosteroid dosages were decreased in all cases, and cytotoxic agents were discontinued in some cases. Twenty-two cases recovered, and 2 died. Pulmonary nocardiosis associated with an underlying autoimmune disease showed a female predominance and

  11. Clinical Analysis of Pulmonary Nocardiosis in Patients With Autoimmune Disease

    PubMed Central

    Li, Shan; Song, Xin yu; Zhao, Yu yue; Xu, Kai; Bi, Ya lan; Huang, Hui; Xu, Zuo jun

    2015-01-01

    Abstract Nocardiosis is an opportunistic infection that most commonly involves the lung; however, only a few case reports of autoimmune disease complicated by pulmonary nocardiosis exist in the literature. We conducted a retrospective analysis of 24 cases of both autoimmune disease and pulmonary nocardiosis at the Peking Union Medical College Hospital between 1990 and 2012. Fifty-two cases were hospitalized with nocardiosis, 24 of whom had at least 1 autoimmune disease before the diagnosis of pulmonary nocardiosis. The cohort patients consisted of 5 men and 19 women, with a mean age of 44.2 years. All were negative for human immunodeficiency virus. All but 1 patient had received immunosuppressants, including corticosteroids, cyclophosphamide, azathioprine, methotrexate, or hydroxychloroquine. Fever (87.5%), cough (83.3%), and sputum (79.2%) were the most common clinical manifestations. Ten cases were accompanied by subcutaneous nodules and/or cutaneous abscesses, and 4 had brain abscess. Half of them were lymphocytopenic. Thirteen of the 16 cases who underwent lymphocyte subtype analysis had decreased CD4+ T-cell counts. Nineteen cases had decreased serum albumin levels. Nocardia was isolated from sputum (13/24), bronchoalveolar lavage fluid (4/6), lung tissue (5/6), pleural effusions (3/5), skin or cutaneous pus (7/10), and brain tissue (1/1). The most common imaging findings were air-space opacities (83.3%), followed by nodules (62.5%), cavitations (45.8%), and masses (37.5%). Five were administered co-trimoxazole only, and the others were treated with 2 or more antibiotics. All 5 cases with skin abscesses and 2 of the 4 cases with brain abscesses were treated by surgical incision and drainage. None underwent thoracic surgery. Corticosteroid dosages were decreased in all cases, and cytotoxic agents were discontinued in some cases. Twenty-two cases recovered, and 2 died. Pulmonary nocardiosis associated with an underlying autoimmune disease showed a female

  12. Chronic obstructive pulmonary disease and oxidative stress.

    PubMed

    Domej, W; Földes-Papp, Z; Flögel, E; Haditsch, B

    2006-04-01

    The respiratory tract as the main entrance for various inhalative substances has great potential to generate reactive species directly or indirectly in excess. Thus, heavy smokers are at high risk for development, impairment and failed response to treatment of chronic obstructive pulmonary disease (COPD). The article is an update regarding the influence of reactive oxygen (ROS) and nitrogen (RNS) species on COPD; however, we do not intend to describe ROS and RNS actions on the entire lung tissue. Here, we focus on the airways, because in human most of the described effects of ROS and RNS species are measured on respiratory epithelial cells obtained by bronchoscopy. ROS and RNS species are physiological compounds in cells and risk factors for several respiratory diseases. In general, both kinds of species are thermodynamically stabile, but their reaction behaviors in cellular environments are very different. For example, the life times of the superoxide anion radical range from micro/milliseconds up to minutes and even hours in in-vitro model systems. Oxidative stress by cigarette smoke was investigated in detail by the authors of this article. In addition, original studies by the authors on the amount of fine particulate matter and trace elements in lung biopsies after defined inhalation indicate a distortion of the equilibrium between oxidants and antioxidants. We also try to present some modern views with respect to genomic medicine for future therapeutic perspectives, although this is an upcoming sector of COPD therapy. PMID:16724946

  13. [Autoimmunity in pathogenesis of chronic obstructive pulmonary disease].

    PubMed

    Urboniene, Daiva; Sakalauskas, Raimundas; Sitkauskiene, Brigita

    2005-01-01

    For years, smoking induced inflammatory reaction, comprised mainly of neutrophils and macrophages, has been accepted to be the major component in pathogenesis of chronic obstructive pulmonary disease. New developments in molecular and cell biology have provided scientists with new knowledge and understanding of inflammatory processes in lung. Recent reports have underlined the role of autoimmunity and T lymphocytes as a potential important factor, which takes place in the pathogenesis of chronic obstructive pulmonary disease. This article reviews potential mechanism of T cell mediated immune response in chronic obstructive pulmonary disease. PMID:15827384

  14. Connective tissue disease-associated pulmonary arterial hypertension.

    PubMed

    Sung, Yon K; Chung, Lorinda

    2015-05-01

    Pulmonary arterial hypertension (PAH) is characterized by vascular remodeling of pulmonary arterioles that leads to increased pulmonary vascular resistance, right heart failure, and death. It is associated with connective tissue diseases, including systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease. PAH is characterized by dyspnea on exertion and fatigue. Syncopal events suggest severe disease. Patients may present with signs of right heart failure. One- and 3-year survival rates are approximately 81% and 52%, respectively. Given the high prevalence and mortality, algorithms for screening are currently under investigation and will hopefully lead to earlier diagnosis and improved survival. PMID:25836644

  15. Pulmonary Arterial Hypertension Associated with Congenital Heart Disease and Eisenmenger Syndrome: Current Practice in Pediatrics

    PubMed Central

    Frank, David B.; Hanna, Brian D.

    2015-01-01

    Pulmonary arterial hypertension (PAH) is an uncommon but serious disease characterized by severe pulmonary vascular disease and significant morbidity and mortality. PAH associated with congenital heart disease (APAH-CHD) is one etiology of PAH that has innate characteristics delineating it from other forms of PAH. The patient with APAH-CHD presents with unique challenges consisting of not only pulmonary vascular disease but also the complexity of the cardiac lesion. Eisenmenger syndrome (ES) represents the severe end of the spectrum for disease in APAH-CHD. Over time, systemic-to-pulmonary shunting through cardiac defects increases pulmonary vascular resistance to levels significant enough to reverse shunting across the defect. Historically, ES patients have been reported to have better outcomes than IPAH despite similarities in pulmonary vascular disease. However, recent studies are challenging this notion. Nonetheless, APAH-CHD survival has improved with the advent of modern PAH targeted therapies. New therapeutic options have allowed us to reconsider the dogma of inoperability in APAH-CHD patients with unrepaired defects. Certainly advances have been made, however, investigators must continue to advance the field through controlled clinical trials in both adult and pediatric APAH-CHD patients. PMID:25604592

  16. The evolving spectrum of pulmonary disease in responders to the World Trade Center tragedy.

    PubMed

    Guidotti, Tee L; Prezant, David; de la Hoz, Rafael E; Miller, Albert

    2011-09-01

    On September 11, 2001, events at the World Trade Center (WTC) exposed residents ofNew York City to WTC dust and products of combustion and pyrolysis. The majority ofWTC-exposed fire department rescue workers experienced a substantial decline in airflowover the first 12 months post-9/11, in addition to the normal age-related declinethat affected all responders, followed by a persistent plateau in pulmonary function inthe 6 years thereafter. The spectrum of the resulting pulmonary diseases consists ofchronic inflammation, characterized by airflow obstruction, and expressing itself indifferent ways in large and small airways. These conditions include irritant-inducedasthma, non-specific chronic bronchitis, aggravated pre-existing obstructive lung disease(asthma or COPD), and bronchiolitis. Conditions concomitant with airwaysobstruction, particularly chronic rhinosinusitis and upper airway disease, and gastroesophagealreflux, have been prominent in this population. Less common have beenreports of sarcoidosis or interstitial pulmonary fibrosis. Pulmonary fibrosis and bronchiolitisare generally characterized by long latency, relatively slow progression, and asilent period with respect to pulmonary function during its evolution. For these reasons,the incidence of these outcomes may be underestimated and may increase overtime. The spectrum of chronic obstructive airways disease is broad in this populationand may importantly include involvement at the bronchiolar level, manifested as smallairways disease. Protocols that go beyond conventional screening pulmonary functiontesting and imaging may be necessary to identify these diseases in order to understandthe underlying pathologic processes so that treatment can be most effective. PMID:23236631

  17. Pulmonary artery denervation for treatment of a patient with pulmonary hypertension secondary to left heart disease

    PubMed Central

    2016-01-01

    Abstract Pulmonary hypertension (PH) predicts poor outcome in patients with left heart disease. A 62-year-old man was referred for heart failure associated with ischemic cardiomyopathy. He received a diagnosis of combined postcapillary and precapillary PH secondary to left heart disease on the basis of hemodynamic parameters. After the pulmonary artery denervation procedure was performed, hemodynamic parameters were markedly improved, which resulted in a significant increase in functional capacity. PMID:27252851

  18. [Chronic obstructive pulmonary disease: The golden decade. Implications for the diagnosis, prevention and treatment of chronic obstructive pulmonary disease].

    PubMed

    López-Giraldo, Alejandra; Rodríguez-Roisin, Robert; Agustí, Alvar

    2015-06-01

    Chronic obstructive pulmonary disease (COPD) is a complex and heterogeneous illness, which causes an important socio-economic burden. The last decade has witnessed significant advances in the understanding and knowledge of COPD with a paradigm shift in both the assessment and management of the disease. The article here reviews these changes with a particular focus on the last revision (2013) of the Global Strategy for the Diagnosis, Management, and Prevention of chronic obstructive pulmonary disease. PMID:24820902

  19. Group III Pulmonary Hypertension: Pulmonary Hypertension Associated with Lung Disease: Epidemiology, Pathophysiology, and Treatments.

    PubMed

    Klinger, James R

    2016-08-01

    Pulmonary hypertension (PH) associated with chronic lung disease (WHO group 3) is the second leading cause of PH and is associated with increased morbidity and mortality. Elevation of pulmonary arterial pressure (PAP) is usually moderate and correlates with severity of lung disease. In a small minority, PAP may approach that seen in WHO group 1 pulmonary arterial hypertension (PAH). Current medications for treating PAH have not shown benefit in controlled trials of group 3 PH and their routine use is discouraged. Patients with severe group 3 PH should be considered for referral to expert centers or entry into clinical trials. PMID:27443138

  20. Chronic obstructive pulmonary disease and cerebrovascular disease: A comprehensive review.

    PubMed

    Lahousse, Lies; Tiemeier, Henning; Ikram, M Arfan; Brusselle, Guy G

    2015-11-01

    Along with the aging population, the public health burden of cerebrovascular disease is increasing. Cerebral small vessel disease and accumulation of brain pathology associate with cognitive decline and can lead to clinical outcomes, such as stroke and dementia. Chronic Obstructive Pulmonary Disease (COPD) is a common respiratory disease among elderly. The quality of life and prognosis of patients with COPD is greatly determined by the presence of comorbidities including stroke and cognitive impairment. Despite the clinical relevance of cerebral small vessel disease, stroke and (vascular) cognitive impairment in patients with COPD, literature is scarce and underlying mechanisms are unknown. The aim of the present review is therefore to summarize current scientific knowledge, to provide a better understanding of the interplay between COPD and the aging brain and to define remaining knowledge gaps. This narrative review article 1) overviews the epidemiology of cerebral small vessel disease, stroke and cognitive impairment in patients with COPD; 2) discusses potential underlying mechanisms including aging, smoking, systemic inflammation, vasculopathy, hypoxia and genetic susceptibility; and 3) highlights areas requiring further research. PMID:26342840

  1. Pulmonary megakaryocytes: "missing link" between cardiovascular and respiratory disease?

    PubMed Central

    Sharma, G K; Talbot, I C

    1986-01-01

    Pulmonary megakaryocytes were quantitated in a series of 30 consecutive hospital necropsies using a two stage immunoperoxidase stain for factor VIII related antigen. In all 30 cases they were found with a mean density of 14.65 megakaryocytes/cm2 in lung sections of 5 micron in thickness. The maximum concentration of intrapulmonary megakaryocytes was consistently found to be in the central zone of the right upper lobe. Less than 22% of the observed cells possessed abundant cytoplasm, the rest appearing as effete, naked, and seminaked nuclei. The mean megakaryocyte count was found to be increased in association with both respiratory pathology (positive smoking history and impaired lung function) and cardiovascular disease states--shock; thromboembolism; myocardial infarction; and severe atheroma in the abdominal aorta, the coronary circulation, and the circle of Willis. Pulmonary megakaryocytes probably embolise from bone marrow. This may reflect stimulated thrombopoiesis, caused by increased platelet consumption in association with atherosclerotic disease, but it cannot be taken to confirm that the lung is the principal site of platelet production. Images PMID:3531243

  2. Pulmonary artery aneurysms in Behçet's disease.

    PubMed

    Yilmaz, Sema; Cimen, Kadriye Akar

    2010-08-01

    Behçet's disease is the most common cause of pulmonary artery aneurysms. Pulmonary artery aneurysms are rare, but they are life-threatening because of their high tendency to rupture. However, there is also a chance that the aneurysms may completely resolve with immunosuppressive therapy. A 30-year-old man was admitted with chest pain, painful oral and genital ulcers, skin rash, weakness, and intermittent hemoptysis. He had a history of Behçet's disease for 6 years. The chest radiography showed a round opacity superior part of hilus on right. The helical thoracic computed tomography (CT) angiography demonstrated pulmonary aneurysms associated with Behçet's disease. The patient was successfully treated with colchicine, corticosteroids, and cyclophosphamide. A discussion about pulmonary artery aneurysms associated with Behçet's disease is provided in this case. PMID:19693504

  3. Chronic Bronchitis and Chronic Obstructive Pulmonary Disease

    PubMed Central

    Criner, Gerard J.

    2013-01-01

    Chronic bronchitis (CB) is a common but variable phenomenon in chronic obstructive pulmonary disease (COPD). It has numerous clinical consequences, including an accelerated decline in lung function, greater risk of the development of airflow obstruction in smokers, a predisposition to lower respiratory tract infection, higher exacerbation frequency, and worse overall mortality. CB is caused by overproduction and hypersecretion of mucus by goblet cells, which leads to worsening airflow obstruction by luminal obstruction of small airways, epithelial remodeling, and alteration of airway surface tension predisposing to collapse. Despite its clinical sequelae, little is known about the pathophysiology of CB and goblet cell hyperplasia in COPD, and treatment options are limited. In addition, it is becoming increasingly apparent that in the classic COPD spectrum, with emphysema on one end and CB on the other, most patients lie somewhere in the middle. It is known now that many patients with severe emphysema can develop CB, and small airway pathology has been linked to worse clinical outcomes, such as increased mortality and lesser improvement in lung function after lung volume reduction surgery. However, in recent years, a greater understanding of the importance of CB as a phenotype to identify patients with a beneficial response to therapy has been described. Herein we review the epidemiology of CB, the evidence behind its clinical consequences, the current understanding of the pathophysiology of goblet cell hyperplasia in COPD, and current therapies for CB. PMID:23204254

  4. Nutrition in chronic obstructive pulmonary disease.

    PubMed

    Schols, A M

    2000-03-01

    Weight loss is a frequently occurring complication in patients with chronic obstructive pulmonary disease (COPD) and is a determining factor of functional capacity, health status, and mortality. Weight loss in COPD is a consequence of increased energy requirements unbalanced by dietary intake. Both metabolic and mechanical inefficiency contribute to the elevated energy expenditure. A disbalance between protein synthesis and protein breakdown may cause a disproportionate depletion of fat-free mass in some patients. Nutritional support is indicated for depleted patients with COPD because it provides not only supportive care, but direct intervention through improvement in respiratory and peripheral skeletal muscle function and in exercise performance. A combination of oral nutritional supplements and exercise or anabolic stimulus appears to be the best treatment approach to obtaining significant functional improvement. Patients responding to this treatment even demonstrated a decreased mortality. Poor response was related to the effects of systemic inflammation on dietary intake and catabolism. The effectiveness of anticatabolic modulation requires further investigation. PMID:10741769

  5. The resistance-compliance product of the pulmonary circulation varies in health and pulmonary vascular disease.

    PubMed

    Hadinnapola, Charaka; Li, Qiuju; Su, Li; Pepke-Zaba, Joanna; Toshner, Mark

    2015-04-01

    Pulmonary vascular resistance (PVR) is traditionally used to describe pulmonary hemodynamic characteristics. However, it does not take into account pulmonary artery compliance (Ca) or pulsatile flow. The product of PVR and Ca is known as RC time. Previous studies assert that the PVR-Ca relationship is fixed and RC time is constant between health and disease states. We hypothesized that RC time was not constant in health and pulmonary vascular disease. Right heart catheterizations performed in Papworth Hospital over a 6 year period were analyzed. Subjects were divided into those with normal pulmonary hemodynamics (NPH group; n = 156) and pulmonary arterial hypertension (PAH group; n = 717). RC time and the right ventricle (RV) oscillatory power fraction were calculated. RC time for the NPH group (0.47 ± 0.13 sec) is significantly lower than the PAH group (0.56 ± 0.16 sec; P < 0.0001). The RV oscillatory power fraction is lower in the NPH group (P < 0.0001). RC time correlates inversely with the RV oscillatory power fraction in each group. We conclude, there is an inverse relationship between PVR and Ca, however, this relationship is not always fixed. Consequently, RC time is significantly lower in health compared to disease with elevated pulmonary artery pressures. PAH leads to a decrease in cardiac efficiency. PMID:25902784

  6. Anaesthesia for patient with chronic obstructive pulmonary disease

    PubMed Central

    Duggappa, Devika Rani; Rao, G Venkateswara; Kannan, Sudheesh

    2015-01-01

    The chronic obstructive pulmonary disease has become a disease of public health importance. Among the various risk factors, smoking remains the main culprit. In addition to airway obstruction, the presence of intrinsic positive end expiratory pressure, respiratory muscle dysfunction contributes to the symptoms of the patient. Perioperative management of these patients includes identification of modifiable risk factors and their optimisation. Use of regional anaesthesia alone or in combination with general anaesthesia improves pulmonary functions and reduces the incidence of post-operative pulmonary complications. PMID:26556916

  7. Cognitive Impairment in Chronic Obstructive Pulmonary Disease

    PubMed Central

    Crişan, Alexandru F.; Oancea, Cristian; Timar, Bogdan; Fira-Mladinescu, Ovidiu; Crişan, Alexandru; Tudorache, Voicu

    2014-01-01

    Background/Purpose Chronic obstructive pulmonary disease (COPD), especially in severe forms, is commonly associated with multiple cognitive problems. Montreal Cognitive Assessment test (MoCA) is used to detect cognitive impairment evaluating several areas: visuospatial, memory, attention and fluency. Our study aim was to evaluate the impact of stable COPD and exacerbation (AECOPD) phases on cognitive status using MoCA questionnaire. Methods We enrolled 39 patients (pts), smokers with COPD group D (30 stable and 9 in AECOPD) and 13 healthy subjects (control group), having similar level of education and no significant differences regarding the anthropometric measurements. We analyzed the differences in MoCA score between these three groups and also the correlation between this score and inflammatory markers. Results Patients with AECOPD had a significant (p<0.001) decreased MoCA score (14.6±3.4) compared to stable COPD (20.2±2.4) and controls (24.2±5.8). The differences between groups were more accentuated for the language abstraction and attention (p<0.001) and delayed recall and orientation (p<0.001) sub-topics. No significant variance of score was observed between groups regarding visuospatial and naming score (p = 0.095). The MoCA score was significantly correlated with forced expiratory volume (r = 0.28) and reverse correlated with C-reactive protein (CRP) (r = −0.57), fibrinogen (r = −0.58), erythrocyte sedimentation rate (ESR) (r = −0.55) and with the partial pressure of CO2 (r = −0.47). Conclusions According to this study, COPD significantly decreases the cognitive status in advanced and acute stages of the disease. PMID:25033379

  8. Pulmonary arterial remodeling in chronic obstructive pulmonary disease is lobe dependent.

    PubMed

    Wrobel, Jeremy P; McLean, Catriona A; Thompson, Bruce R; Stuart-Andrews, Christopher R; Paul, Eldho; Snell, Gregory I; Williams, Trevor J

    2013-09-01

    Abstract Pulmonary arterial remodeling has been demonstrated in patients with severe chronic obstructive pulmonary disease (COPD), but it is not known whether lobar heterogeneity of remodeling occurs. Furthermore, the relationship between pulmonary hypertension (PH) and pulmonary arterial remodeling in COPD has not been established. Muscular pulmonary arterial remodeling in arteries 0.10-0.25 mm in diameter was assessed in COPD-explanted lungs and autopsy controls. Remodeling was quantified as the percentage wall thickness to vessel diameter (%WT) using digital image analysis. Repeat measures mixed-effects remodeling for %WT was performed according to lobar origin (upper and lower), muscular pulmonary arterial size (small, medium, and large), and echocardiography-based pulmonary arterial pressure (no PH, mild PH, and moderate-to-severe PH). Lobar perfusion and emphysema indices were determined from ventilation-perfusion and computed tomography scans, respectively. Overall, %WT was greater in 42 subjects with COPD than in 5 control subjects ([Formula: see text]). Within the COPD group, %WT was greater in the upper lobes ([Formula: see text]) and in the small muscular pulmonary arteries ([Formula: see text]). Lobar differences were most pronounced in medium and large arteries. Lobar emphysema index was not associated with arterial remodeling. However, there was a significant positive relationship between the lobar perfusion index and pulmonary arterial remodeling ([Formula: see text]). The presence of PH on echocardiography showed only a trend to a small effect on lower lobe remodeling. The pattern of pulmonary arterial remodeling in COPD is complicated and lobe dependent. Differences in regional blood flow partially account for the lobar heterogeneity of pulmonary arterial remodeling in COPD. PMID:24618551

  9. Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease.

    PubMed

    Whitsett, Jeffrey A; Wert, Susan E; Weaver, Timothy E

    2010-01-01

    The alveolar region of the lung creates an extensive epithelial surface that mediates the transfer of oxygen and carbon dioxide required for respiration after birth. Maintenance of pulmonary function depends on the function of type II epithelial cells that synthesize and secrete pulmonary surfactant lipids and proteins, reducing the collapsing forces created at the air-liquid interface in the alveoli. Genetic and acquired disorders associated with the surfactant system cause both acute and chronic lung disease. Mutations in the ABCA3, SFTPA, SFTPB, SFTPC, SCL34A2, and TERT genes disrupt type II cell function and/or surfactant homeostasis, causing neonatal respiratory failure and chronic interstitial lung disease. Defects in GM-CSF receptor function disrupt surfactant clearance, causing pulmonary alveolar proteinosis. Abnormalities in the surfactant system and disruption of type II cell homeostasis underlie the pathogenesis of pulmonary disorders previously considered idiopathic, providing the basis for improved diagnosis and therapies of these rare lung diseases. PMID:19824815

  10. Alveolar Surfactant Homeostasis and the Pathogenesis of Pulmonary Disease

    PubMed Central

    Whitsett, Jeffrey A.; Wert, Susan E.; Weaver, Timothy E.

    2014-01-01

    The alveolar region of the lung creates an extensive epithelial surface that mediates the transfer of oxygen and carbon dioxide required for respiration after birth. Maintenance of pulmonary function depends on the function of type II epithelial cells that synthesize and secrete pulmonary surfactant lipids and proteins, reducing the collapsing forces created at the air-liquid interface in the alveoli. Genetic and acquired disorders associated with the surfactant system cause both acute and chronic lung disease. Mutations in the ABCA3, SFTPA, SFTPB, SFTPC, SCL34A2, and TERT genes disrupt type II cell function and/or surfactant homeostasis, causing neonatal respiratory failure and chronic interstitial lung disease. Defects in GM-CSF receptor function disrupt surfactant clearance, causing pulmonary alveolar proteinosis. Abnormalities in the surfactant system and disruption of type II cell homeostasis underlie the pathogenesis of pulmonary disorders previously considered idiopathic, providing the basis for improved diagnosis and therapies of these rare lung diseases. PMID:19824815

  11. Pulmonary arterial hypertension associated with congenital heart disease. Personal perspectives.

    PubMed

    Nakanishi, Toshio

    2015-01-01

    The management of patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) has changed dramatically with the development of targeted therapy with selective pulmonary vasodilators. The number of adult Japanese patients with PAH associated with CHD is increasing. It is important to develop evidence-based guidelines for the management of these patients, and to achieve this, a register of adult Japanese patients with PAH associated with CHD should be established. At the World Symposium in Nice, France, in 2013, the consensus was reached that patients with a pulmonary resistance of < 4 Wood Units (WU)·m(2) have operable disease, and patients with a pulmonary resistance of > 8 WU·m(2) have inoperable disease. However, these criteria are conservative. Some patients with a pulmonary resistance of > 8 WU·m(2) and a good response to a pulmonary vasodilator test have operable disease and a favorable clinical course long after repair of CHD. The criteria determining operability in patients with PAH associated with CHD in the era of pulmonary vasodilators should be established using data obtained from patient registers and/or multicenter studies. The optimal management of Eisenmenger syndrome should also be established using data obtained from patient registers. Prospective studies should be conducted to determine the life expectancy of patients with Eisenmenger syndrome in the era of targeted therapy. A relatively mild increase in pulmonary resistance may result in failure of a Fontan circulation. The effects of pulmonary vasodilators on the long-term prognosis of patients who have undergone the Fontan operation are still unclear. PMID:25787791

  12. [Acute pulmonary histoplasmosis as an imported disease].

    PubMed

    van Crevel, R; van der Ven, A J; Meis, J F; Kullberg, B J

    1997-06-21

    A previously healthy 44-year-old male traveller presented with a dry cough, fever and an abnormal chest X-ray after a stay in Guatemala, where he had explored bat caves. Acute pulmonary histoplasmosis was diagnosed after culture of Histoplasma capsulatum from bronchial washings. A favourable response was seen upon treatment with itraconazole for six weeks. Acute pulmonary histoplasmosis should be considered in a healthy traveller returning with fever from the USA or subtropical areas. PMID:9380167

  13. Postpartum Acute Pulmonary Oedema with Sub clinical Rheumatic Heart Disease.

    PubMed

    R, Padmaja; Gande, Sri Krishna Padma Challa Rao

    2015-02-01

    Acute dyspnea with pulmonary oedema in postpartum is uncommon but life-threatening event. Contributing factors for pulmonary oedema include, administration of tocolytics, underlying cardiac disease, iatrogenic fluid overload and preeclampsia acounting 0.08% of pregnancies. Pulmonary embolism, amniotic fluid embolism, pneumonia, aspiration and pulmonary oedema are some of the potentially devastating conditions that should be considered by the attending physician. Here, we report a case of postpartum acute pulmonary oedema referred to causality after an emergency caesarean section in a private hospital. No matter what the underlying pathology, prompt administration and appropriate resuscitation is always the first priority. Only after the patient has been stabilized attention must be turned to diagnosis and specific treatment. A diagnosis of severe Mitral Stenosis, probably of rheumatic origin was made after stabilizing the patient. PMID:25859501

  14. Smoking Cessation in Chronic Obstructive Pulmonary Disease.

    PubMed

    Tashkin, Donald P

    2015-08-01

    Smoking cessation is the most effective strategy for slowing down the progression of chronic obstructive pulmonary disease (COPD) and reducing mortality in the approximately 50% of patients with diagnosed COPD who continue to smoke. While behavioral interventions (including simple advice) have modest efficacy in improving smoking quit rates, the combination of counseling and pharmacotherapy is more effective than either alone. When combined with even brief counseling, nicotine replacement therapy (NRT), bupropion SR, and varenicline have all been shown to be effective in promoting smoking cessation and sustained abstinence in smokers with COPD to a degree comparable to that observed in the general smoking population. However, the recidivism rate is high after initial quitting so that at the end of 1 year, approximately 80% or more of patients are still smoking. Thus, new approaches to smoking cessation are needed. One approach is to combine different pharmacotherapies, for example, nicotine patch plus rapidly acting NRT (e.g., gum or nasal spray) and/or bupropion or even varenicline plus either NRT or bupropion, in a stepwise approach over a varying duration depending on the severity of nicotine dependence and nicotine withdrawal symptoms during the quit attempt, as proposed in the American College of Chest Physicians Tobacco Dependence Took Kit. Electronic (e)-cigarettes, which deliver vaporized nicotine without most of the noxious components in the smoke from burning tobacco cigarettes, also has potential efficacy as a smoking cessation aid, but their efficacy and safety as either substitutes for regular cigarettes or smoking cessation aids require additional study. This task is complicated because e-cigarettes are currently unregulated and hundreds of different brands are currently available. PMID:26238637

  15. Pulmonary rehabilitation in chronic respiratory diseases--from goals to outcomes.

    PubMed

    Pesut, Dragica; Ciobanu, Laura; Nagorni-Obradovic, Ljudmila

    2008-01-01

    This paper is a review of current approaches on pulmonary rehabilitation (PR) in chronic pulmonary diseases. Pulmonary rehabilitation is the most accepted method of non-pharmacological treatment in patients with chronic obstructive pulmonary disease (COPD), bronchial asthma, bronchiectasis, cystic fibrosis, interstitial lung disease, neuromuscular degenerative disease and post-tuberculosis lung sequelae. Throughout its components, especially oxygen therapy, it is the most important intervention in chronic respiratory failure in order to improve exercise tolerance, lung function and self-management. Enrolling patients in pulmonary rehabilitation programmes is a consistent help to a better control of their illness and a step forward to the international standards of treating COPD and non-COPD chronic respiratory diseases. It is evidence-based that PR is effective in reducing dyspnoea, improving health-related quality of life, reducing the number of hospital days and the utilisation of costly healthcare resources; there are also psychosocial benefits from comprehensive PR programmes in patients with COPD. PR is currently considered as effective in patients with COPD and in some patients with chronic respiratory diseases other than COPD. PMID:18822868

  16. Blunted Hypoxic Pulmonary Vasoconstriction in Experimental Neonatal Chronic Lung Disease

    PubMed Central

    Rey-Parra, Gloria Juliana; Archer, Stephen L.; Bland, Richard D.; Albertine, Kurt H.; Carlton, David P.; Cho, Soo-Chul; Kirby, Beth; Haromy, Al; Eaton, Farah; Wu, Xichen; Thébaud, Bernard

    2008-01-01

    Rationale: Neonatal chronic lung disease (CLD), caused by prolonged mechanical ventilation (MV) with O2-rich gas, is the most common cause of long-term hospitalization and recurrent respiratory illness in extremely premature infants. Recurrent episodes of hypoxemia and associated ventilator adjustments often lead to worsening CLD. The mechanism that causes these hypoxemic episodes is unknown. Hypoxic pulmonary vasoconstriction (HPV), which is partially controlled by O2-sensitive voltage-gated potassium (Kv) channels, is an important adaptive response to local hypoxia that helps to match perfusion and ventilation in the lung. Objectives: To test the hypothesis that chronic lung injury (CLI) impairs HPV. Methods: We studied preterm lambs that had MV with O2-rich gas for 3 weeks and newborn rats that breathed 95%-O2 for 2 weeks, both of which resulted in airspace enlargement and pulmonary vascular changes consistent with CLD. Measurements and Main Results: HPV was attenuated in preterm lambs with CLI after 2 weeks of MV and in newborn rats with CLI after 2 weeks of hyperoxia. HPV and constriction to the Kv1.x-specific inhibitor, correolide, were preferentially blunted in excised distal pulmonary arteries (dPAs) from hyperoxic rats, whose dPAs exhibited decreased Kv1.5 and Kv2.1 mRNA and K+ current. Intrapulmonary gene transfer of Kv1.5, encoding the ion channel that is thought to trigger HPV, increased O2-sensitive K+ current in cultured smooth muscle cells from rat dPAs, and restored HPV in hyperoxic rats. Conclusions: Reduced expression/activity of O2-sensitive Kv channels in dPAs contributes to blunted HPV observed in neonatal CLD. PMID:18511704

  17. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension due to lung diseases and/or hypoxia

    PubMed Central

    Sakkijha, Husam; Idrees, Majdy M.

    2014-01-01

    Chronic lung diseases are common causes of pulmonary hypertension. It ranks second after the left heart disease. Both obstructive and restrictive lung diseases are know to cause pulmonary hypertension. The pathophysiology of the disease is complex, and includes factors affecting the blood vessels, airways, and lung parenchyma. Hypoxia and the inhalation of toxic materials are another contributing factors. Recent guidelines have further clarified the association between pulmonary hypertension and chronic lung disease and made general guidelines concerning the diagnosis and management. In this article, we will provide a detailed revision about the new classification and give general recommendations about the management of pulmonary hypertension in chronic lung diseases. PMID:25076998

  18. Pulmonary CT and MRI phenotypes that help explain chronic pulmonary obstruction disease pathophysiology and outcomes.

    PubMed

    Hoffman, Eric A; Lynch, David A; Barr, R Graham; van Beek, Edwin J R; Parraga, Grace

    2016-03-01

    Pulmonary x-ray computed tomographic (CT) and magnetic resonance imaging (MRI) research and development has been motivated, in part, by the quest to subphenotype common chronic lung diseases such as chronic obstructive pulmonary disease (COPD). For thoracic CT and MRI, the main COPD research tools, disease biomarkers are being validated that go beyond anatomy and structure to include pulmonary functional measurements such as regional ventilation, perfusion, and inflammation. In addition, there has also been a drive to improve spatial and contrast resolution while at the same time reducing or eliminating radiation exposure. Therefore, this review focuses on our evolving understanding of patient-relevant and clinically important COPD endpoints and how current and emerging MRI and CT tools and measurements may be exploited for their identification, quantification, and utilization. Since reviews of the imaging physics of pulmonary CT and MRI and reviews of other COPD imaging methods were previously published and well-summarized, we focus on the current clinical challenges in COPD and the potential of newly emerging MR and CT imaging measurements to address them. Here we summarize MRI and CT imaging methods and their clinical translation for generating reproducible and sensitive measurements of COPD related to pulmonary ventilation and perfusion as well as parenchyma morphology. The key clinical problems in COPD provide an important framework in which pulmonary imaging needs to rapidly move in order to address the staggering burden, costs, as well as the mortality and morbidity associated with COPD. PMID:26199216

  19. Comorbidity in chronic obstructive pulmonary disease. Related to disease severity?

    PubMed Central

    Echave-Sustaeta, Jose M; Comeche Casanova, Lorena; Cosio, Borja G; Soler-Cataluña, Juan Jose; Garcia-Lujan, Ricardo; Ribera, Xavier

    2014-01-01

    Background and objective Several diseases commonly co-exist with chronic obstructive pulmonary disease (COPD), especially in elderly patients. This study aimed to investigate whether there is an association between COPD severity and the frequency of comorbidities in stable COPD patients. Patients and methods In this multicenter, cross-sectional study, patients with spirometric diagnosis of COPD attended to by internal medicine departments throughout Spain were consecutively recruited by 225 internal medicine specialists. The severity of airflow obstruction was graded using the Global Initiative for Chronic Obstructive Lung Disease (GOLD) and data on demographics, smoking history, comorbidities, and dyspnea were collected. The Charlson comorbidity score was calculated. Results Eight hundred and sixty-six patients were analyzed: male 93%, mean age 69.8 (standard deviation [SD] 9.7) years and forced vital capacity in 1 second 42.1 (SD 17.7)%. Even, the mean (SD) Charlson score was 2.2 (2.2) for stage I, 2.3 (1.5) for stage II, 2.5 (1.6) for stage III, and 2.7 (1.8) for stage IV (P=0.013 between stage I and IV groups), independent predictors of Charlson score in the multivariate analysis were age, smoking history (pack-years), the hemoglobin level, and dyspnea, but not GOLD stage. Conclusion COPD patients attended to in internal medicine departments show high scores of comorbidity. However, GOLD stage was not an independent predictor of comorbidity. PMID:25429213

  20. [Pulmonary hypertension associated with congenital heart disease and Eisenmenger syndrome].

    PubMed

    Calderón-Colmenero, Juan; Sandoval Zárate, Julio; Beltrán Gámez, Miguel

    2015-01-01

    Pulmonary arterial hypertension is a common complication of congenital heart disease (CHD). Congenital cardiopathies are the most frequent congenital malformations. The prevalence in our country remains unknown, based on birthrate, it is calculated that 12,000 to 16,000 infants in our country have some cardiac malformation. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodeling and endothelial dysfunction secondary to an imbalance in vasoactive mediators which promotes vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apotosis and fibrosis. The progressive rise in pulmonary vascular resistance and increased pressures in the right heart provocated reversal of the shunt may arise with the development of Eisenmenger' syndrome the most advanced form de Pulmonary arterial hypertension associated with congenital heart disease. The prevalence of Pulmonary arterial hypertension associated with CHD has fallen in developed countries in recent years that is not yet achieved in developing countries therefore diagnosed late as lack of hospital infrastructure and human resources for the care of patients with CHD. With the development of targeted medical treatments for pulmonary arterial hypertension, the concept of a combined medical and interventional/surgical approach for patients with Pulmonary arterial hypertension associated with CHD is a reality. We need to know the pathophysiological factors involved as well as a careful evaluation to determine the best therapeutic strategy. PMID:25650280

  1. A translational preclinical model of interstitial pulmonary fibrosis and pulmonary hypertension: mechanistic pathways driving disease pathophysiology

    PubMed Central

    Jarman, Elizabeth R.; Khambata, Valerie S.; Yun Ye, Li; Cheung, Kenneth; Thomas, Matthew; Duggan, Nicholas; Jarai, Gabor

    2014-01-01

    Abstract Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease, in which a decline in patient prognosis is frequently associated with the onset of pulmonary hypertension (PH). Animal models exhibiting principle pathophysiological features of IPF and PH could provide greater insight into mechanistic pathways underlying disease progression and a means for evaluating novel therapeutic approaches for intervention. Here, we describe an in vivo disease model, in which animals develop progressive interstitial pulmonary fibrosis and associated PH, as defined by the presence of fibrotic foci adjacent to areas of alveolar injury and remodeling of the pulmonary vasculature. Associated changes in physiological parameters included a decline in lung function and increase in mean pulmonary arterial pressure (mPAP) >25 mmHg. The early fibrotic pathology is associated with a profibrogenic microenvironment, elevated levels of the matrix metalloproteases, MMP‐2, MMP‐7, and MMP‐12, TIMP‐1, the chemoattractant and mitogen, PDGF‐β, and the chemokines CCL2 and CXCL12, that are associated with the recruitment of macrophages, mast cells, and fibrocytes. Principle mechanistic pathways associated with disease pathogenesis are upregulated in the lungs and pulmonary arteries, with sustained increases in gene transcripts for the profibrotic mediator TGF‐β1 and components of the TGF‐β signaling pathway; PAI‐1, Nox‐4, and HIF‐1α. Therapeutic treatment with the ALK‐5/TGF‐β RI inhibitor SB‐525334 reversed established pulmonary fibrosis and associated vascular remodeling, leading to normalization in clinically translatable physiological parameters including lung function and hemodynamic measurements of mPAP. These studies highlight the application of this model in validating potential approaches for targeting common mechanistic pathways driving disease pathogenesis. PMID:25214520

  2. [Pulmonary surfactant homeostasis associated genetic abnormalities and lung diseases].

    PubMed

    Jiang, Xiaojing; Sun, Xiuzhu; Du, Weihua; Hao, Haisheng; Zhao, Xueming; Wang, Dong; Zhu, Huabin; Liu, Yan

    2016-08-10

    Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis. This paper has provided a review for the functions and processes of pulmonary surfactant metabolism, as well as the connection between disorders of surfactant homeostasis genes and lung diseases. PMID:27455022

  3. Giant high-pressure pulmonary artery aneurysm in an elderly patient with chronic obstructive pulmonary disease.

    PubMed

    Morais, Sandra A; Oliveira, Hugo M; de Almeida, José R; Eiras, Eduardo; Silva, Ana Catarina; Gavina, Cristina

    2016-03-01

    The authors report the case of a 74-year-old man, with a history of chronic obstructive pulmonary disease (COPD), GOLD grade 3, stable for the past two decades, who was admitted to our center with severe right heart failure. The chest radiograph showed moderate heart enlargement mainly of the right atrium and pulmonary artery, similar to previous chest radiographs in the previous 20 years. The transthoracic echocardiogram showed a pulmonary artery aneurysm (PAA), dilatation of the right chambers with pulmonary artery systolic pressure of 52 mmHg, and preserved right ventricular systolic function. A thoracic computed tomography scan confirmed the presence of a giant PAA 72 mm in diameter. The patient was started on high-dose diuretics, with significant clinical improvement. After optimization of medical therapy right heart catheterization was carried out with the patient in optimal clinical condition, which revealed mild precapillary pulmonary hypertension with a mean pulmonary artery pressure of 26 mmHg. On the basis of the clinical and imaging findings a stable, giant, high-pressure, PAA was diagnosed secondary to pulmonary hypertension induced by COPD, with a 20-year follow-up without need for surgical repair, which helped in our decision to maintain medical surveillance. The recent onset of heart failure is explained by the unfavorable evolution of COPD. This case may change the attitude expressed in previous studies favoring the choice of an invasive approach to treat giant high-pressure PAAs, instead supporting the maintenance of medical treatment. PMID:26922398

  4. Pulmonary hypertension during acute respiratory diseases in infants

    PubMed Central

    Bardi-Peti, Luiza; Ciofu, Eugen Pascal

    2010-01-01

    ABSTRACT Objectives:The study was undertaken to assess whether previously healthy infants with acute respiratory diseases develop elevated pulmonary artery pressures and to identify which type of disease is associated with pulmonary hypertension. Material and Methods:We performed 2D and Doppler echocardiography in 137 infants, aged between 1 and 12 month, from November 2007 to December 2009. 75 infants had acute respiratory diseases (49 bronchiolitis, 16 interstitial pneumonia, 3 bronchopneumonia, 6 episodic wheezing, 1 lobar pneumonia) and 62 were in the control group. We excluded children with congenital heart diseases and other conditions associated with pulmonary hypertension. The method of time to peak velocity corrected for heart rate was used to estimate pulmonary arterial pressure (PAP). We analysed 2 age-subgroups: 1-2 months and 2-12 months. A Student’s t-test for independent samples was used to compare the mean values of variables. Outcomes:Increased mean pulmonary pressures (>25mmHg) were measured in 18 infants with respiratory diseases, with the next distribution: 14 bronchiolitis, 2 bronchopneumonia, 1 episodic wheezing, 1 interstitial pneumonia. The values were categorized as mild-moderate pulmonary hypertension. Mean PAP were significantly increased in subjects with clinically bronchoobstructive disease (bronchiolitis, episodic wheezing, bronchopneumonia) vs. control (p=0.05 in first age-subgroup and<0.001 in second age-subgroup). In infants with bronchoobstructive disease hospitalization was significantly longer in patients with pulmonary hypertension vs. normal PAP (p= 0.04 in first age-subgroup and 0.005 in second age-subgroup). In patients with bronchoobstructive diseases, mean PAPm and PAPs were significantly increased in subjects with a moderate/severe episode of wheezing at admission vs. a mild episode (p=0.02). Mean PAPm and PAPs were increased in subjects with interstitial pneumonia vs. control, but without statistic significance

  5. Different approaches in the treatment of obstructive pulmonary diseases.

    PubMed

    Kabir, Eva Rahman; Morshed, Nabila

    2015-10-01

    Advances in drug formulation, inhalation device design and disease management are generating new opportunities for patients suffering from obstructive pulmonary diseases. This article provides a comprehensive review of the different promising pulmonary drug delivery technologies in the treatment of obstructive pulmonary diseases, particularly with regard to the treatment of asthma and chronic pulmonary diseases (COPD), which are increasing day by day due to increasing environmental pollution and its harmful and toxic contaminants. In the recent years, a better knowledge has been gained regarding the mechanism of action of glucocorticoids and how they suppress the chronic inflammation. New etiology has been brought into light regarding the inactivity of glucocorticoids in some patients having asthma and COPDs even though the inflammatory genes are triggered by similar molecules in both the diseases. This new knowledge has given a new platform to improve glucocorticoids and their resistance also how other combination therapy can be used for these diseases. It has also led to the quest for improving and developing other alternatives such as anti-leukotriene agents, muscarinic inhibitors, combination therapy, as well as biologic immune-modulators in the treatment of the different pulmonary diseases. Several new combinations of glucocorticoids are available in the global market for the use in pulmonary diseases especially asthma although their availability fluctuates between continents. There has been several studies done regarding the variation of effectiveness of the different inhaled glucocorticoids and hence it is important to take into consideration the different delivery systems and the methods which are used to treat the patients. PMID:26172082

  6. [Chronic obstructive pulmonary disease and cardiovascular diseases--'cardiopulmonary continuum'].

    PubMed

    Batura-Gabryel, Halina; Grabicki, Marcin

    2014-01-01

    Chronic obstructive pulmonary disease (COPD) is characterised by persistent airflow limitation and extrapulmonary comorbidities, which contribute to the overall severity. Some risk factors, with tobacco smoking as the most serious one, lead to a chronic, systemic inflammation that plays the main role in the pathogenesis of COPD and comorbidities, including cardiovascular diseases (CVD). The course of COPD is diverse; it depends on pathologies in the respiratory system and on other organ dysfunctions. CVDs are the most commonly recognised comorbidities in COPD patients. The severity and natural course of COPD, as well as quality of the patient's life, are influenced by them. CVDs are frequently the reason for hospitalisation and may lead to death. They are also an important prognostic factor. Comorbidities may prolong exacerbation of COPD. On the other hand, COPD is an independent risk factor of CVD. The prevalence of COPD is high in patients suffering from coronary artery disease, and airflow limitation is a major risk factor for chronic heart failure. These complex interactions between heart and lung can be denoted as 'cardiopulmonary continuum'. These dependencies are not recognised in detail. Currently research is being done, which attempts to explain these complicated relations. For many years COPD and CVD were not connected. Today it is known that patients suffering from COPD must be provided comprehensive care. It is necessary to monitor the risk of CVD and their influence on the COPD course. Careful and proper treatment of all diseases is essential. An interdisciplinary team with good cooperation should prepare a plan of COPD treatment with simultaneous therapy of comorbidities. PMID:25339571

  7. IDENTIFICATION AND CHARACTERIZATION OF DISEASE USING PULMONARY FUNCTION TESTS

    EPA Science Inventory

    Abstract
    Pulmonary function testing is used routinely in human medicine to objectively define functional deficits in individuals with respiratory disease. Despite the fact that respiratory disease is a common problem in veterinary medicine, evaluation of the small animal pa...

  8. Pulmonary rehabilitation for respiratory disorders other than chronic obstructive pulmonary disease.

    PubMed

    Rochester, Carolyn L; Fairburn, Carl; Crouch, Rebecca H

    2014-06-01

    Pulmonary rehabilitation (PR) is an important therapeutic intervention that should no longer be considered suitable only for patients with chronic obstructive pulmonary disease (COPD). A strong rationale exists for providing PR to persons with a broad range of respiratory disorders other than COPD. Evidence shows that PR for these patients is feasible, safe and effective. A disease-relevant approach should be undertaken, based on individual patients' needs. Further research is needed to better understand the optimal program content, duration and outcomes measures, to enable diverse patients to achieve maximal benefits of PR. PMID:24874132

  9. Pulmonary thromboembolic disease. Clinical management of acute and chronic disease.

    PubMed

    Torbicki, Adam

    2010-07-01

    Pulmonary thromboembolism falls between the areas of pulmonology and cardiology, internal medicine and intensive care, radiology and nuclear medicine, and hematology and cardiothoracic surgery. Depending on their clinical background, physicians faced with a patient with a pulmonary thromboembolism may speak different languages and adopt different treatment approaches. Now, however, there is an opportunity to end the Tower of Babel surrounding pulmonary thromboembolism. There is a growing acknowledgement that the key clinical problems in both acute pulmonary embolism and chronic thromboembolic pulmonary hypertension are linked to right ventricular pressure overload and right ventricular failure. As a result, cardiologists and cardiac intensive care specialists are taking an increasing interest in understanding and combating these conditions. The European Society of Cardiology was the first to elaborate comprehensive clinical practice guidelines for pulmonary thromboembolism and chronic thromboembolic pulmonary hypertension. The task forces involved in producing these guidelines included radiologists, pulmonologists, hematologists, intensive care physicians and surgeons, which ensured that the final document was universally acceptable. The aim of this article was to provide an overview of the epidemiology, risk factors, diagnosis, treatment, prognosis and prevention of acute pulmonary thromboembolism and chronic thromboembolic pulmonary hypertension, while taking into account European Society of Cardiology guidelines and incorporating new evidence where necessary. PMID:20609317

  10. Chronic thromboembolic pulmonary hypertension: a distinct disease entity.

    PubMed

    Lang, Irene

    2015-06-01

    Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct subtype of pulmonary hypertension (PH). One disease hypothesis is that CTEPH results from the non-resolution of venous thromboembolism. CTEPH is characterised by the presence of obstructive fibrotic thromboembolic material in the major pulmonary vessels, with concomitant microvascular arteriopathy, resulting in progressive PH. The clinical presentation of CTEPH is similar to pulmonary arterial hypertension with nonspecific symptoms, but it is distinguished from pulmonary arterial hypertension by the presence of mismatched segmental defects on the ventilation/perfusion scan. The exact prevalence and incidence of CTEPH are unknown, but are thought to have been underestimated in the past. CTEPH is unique among the subgroups of PH in that it is potentially curable with pulmonary endarterectomy, a surgical intervention intended to remove the occlusive material from the pulmonary vasculature. However, in some patients the obstructions are technically inaccessible or the risk/benefit ratios are unfavourable, making the condition inoperable. It is thought that the involvement of the smaller, more distal vessels is a target for medical treatment. Untreated, CTEPH may result in right heart failure and death. The pathophysiological mechanisms which cause CTEPH are complex and have not yet been fully elucidated. PMID:26028636

  11. Asthma and chronic obstructive pulmonary disease overlap: asthmatic chronic obstructive pulmonary disease or chronic obstructive asthma?

    PubMed

    Slats, Annelies; Taube, Christian

    2016-02-01

    Asthma and chronic obstructive pulmonary disease (COPD) are different disease entities. They are both clinical diagnoses, with diagnostic tools to discriminate between one another. However, especially in older patients (>55 years) it seems more difficult to differentiate between asthma and COPD. This has led to the definition of a new phenotype called asthma COPD overlap syndrome (ACOS). However, our understanding of ACOS is at a very preliminary stage, as most research has involved subjects with existing diagnoses of asthma or COPD from studies with different definitions for ACOS. This has led to different and sometimes opposing results between studies on several features of ACOS, also depending on the comparison with COPD alone, asthma alone or both, which are summarized in this review.We suggest not using the term ACOS for a patient with features of both asthma and COPD, but to describe a patient with chronic obstructive airway disease as completely as possible, with regard to characteristics that determine treatment response (e.g. eosinophilic inflammation) and prognosis (such as smoking status, exacerbation rate, fixed airflow limitation, hyperresponsiveness, comorbidities). This will provide a far more clinically relevant diagnosis, and would aid in research on treatment in more homogenous groups of patients with chronic airways obstruction. More research is certainly needed to develop more evidence-based definitions for this patient group and to evaluate biomarkers, which will help to further classify these patients, treat them more adequately and unravel the underlying pathophysiological mechanism. PMID:26596632

  12. Pulmonary function after transverse or midline incision in patients with obstructive pulmonary disease.

    PubMed

    Becquemin, J P; Piquet, J; Becquemin, M H; Melliere, D; Harf, A

    1985-01-01

    Atelectasis and bronchopneumonia occur frequently in patients undergoing aorto-iliac reconstructive surgery. Transverse (T) incisions in upper abdominal surgery are thought to be followed by fewer pulmonary complications than midline incisions (M) but reports remain controversial. We studied the incidence of postoperative pulmonary complications and lung dysfunction after T and M incisions for aorto-iliac surgery in 13 patients with chronic obstructive pulmonary disease (COPD) and 13 control patients with normal lungs (C). For all subjects, we evaluated (1) postoperative clinical or radiological pulmonary events; (2) preoperatively and on postoperative days 2 (D2), 5 (D5), 9 (D9) and 12 (D12) - the forced expiratory volume in 1 s (FEV1), vital capacity (VC), alveolar-arterial oxygen difference (AaPO2), and (3) convenience for the surgeon. Operatively, aortic exposure was excellent with both incisions. Bronchopneumonia occurred only after M in five patients (1 C, 4 COPD). In contrast with the control patients in whom no difference was found between T and M incisions, the FEV1 of COPD patients was significantly less impaired with T than with M incisions (p less than 0.005 on D2 and p less than 0.05 on D5). VC decreased similarly with both incisions on D2 but on D5 the improvement was less with M (p less than 0.005). Changes in AaPO2 were more marked on D2 and D5 for the COPD patients with M incisions. We conclude that (1) in patients with chronic obstructive pulmonary disease, laparotomy with a transverse incision was associated with better postoperative lung function and fewer pulmonary complications; (2) in patients without pulmonary disease, midline and transverse incisions were equivalent. PMID:2933436

  13. Pulmonary hypertension in children and adolescents with sickle cell disease.

    PubMed

    Onyekwere, O C; Campbell, A; Teshome, M; Onyeagoro, S; Sylvan, C; Akintilo, A; Hutchinson, S; Ensing, G; Gaskin, P; Kato, G; Rana, S; Kwagyan, J; Gordeuk, V; Williams, J; Castro, O

    2008-03-01

    The prevalence of pulmonary hypertension (PHTN) in the pediatric sickle cell disease (SCD) population is not known despite its high prevalence in adult patients. Our hypothesis was that increased pulmonary artery pressures (PAPs) would be found in SCD children and adolescents, especially those with a history of pulmonary complications: acute chest syndrome, obstructive sleep apnea, asthma, and reactive airway disease. Fifty-two SCD children, 23 of whom had underlying pulmonary disease, were screened for PHTN, which was defined as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/s. Twenty-four (46.15%) SCD patients had increased PAP (i.e., TRV > or =2.5 m/s), and 6 (11.5%) had significant PHTN (i.e., TRV > or =3.0 m/s). Pulmonary disease was marginally associated with PHTN (odds ratio 2.80 and confidence interval 0.88 to 8.86; p = 0.0795). As in adult SCD patients with PHTN, this complication was correlated with the degree of hemolysis as manifested by significantly higher lactate dehydrogenase and bilirubin, lower hemoglobin and hematocrit levels, and a strong association with Hb-SS phenotype. However, after statistical adjustment for age and sex, increased serum LDH was not associated with the development of PHTN. Further studies are needed to clarify the prevalence and mechanisms of PHTN in pediatric and adolescent patients with SCD. PMID:17680298

  14. Pulmonary vascular disease in a rabbit a high altitude

    NASA Astrophysics Data System (ADS)

    Heath, Donald; Williams, David; Rios-Datenz, Jaime; Gosney, John

    1990-03-01

    A male weanling rabbit of the New Zealand White strain, born and living at an altitude of 3800 m in La Paz, Bolivia, developed right ventricular hypertrophy. This was found to be associated with growth of vascular smooth muscle cells in the intima of pulmonary arterioles, and contrasted with muscularization of the walls of pulmonary arterioles, without extension into the intima, found in a healthy, high-altitude control rabbit of the same strain. A low-altitude control showed no such muscularization. It is concluded that alveolar hypoxia, acting directly or through an intermediate agent, is a growth factor for vascular smooth muscle cells in pulmonary arterioles. This is the first report of pulmonary vascular disease due to high altitude in rabbits.

  15. Pulmonary vascular resistance in children with congenital heart disease.

    PubMed Central

    Davies, N J; Shinebourne, E A; Scallan, M J; Sopwith, T A; Denison, D M

    1984-01-01

    Pulmonary and systemic blood flow and pulmonary vascular resistance were measured in 21 children with congenital heart disease. Blood flow was calculated by the direct Fick method, using measurements of metabolic gas exchange obtained by remote respiratory mass spectrometry. The observations showed that the administration of oxygen caused an appreciable fall in pulmonary vascular resistance in 16 of the 21 children studied and that this fall would not have been appreciated from a study of pulmonary arterial pressure alone as it was masked by a corresponding rise in blood flow. In 10 of 14 children, in whom superior vena caval blood was also sampled, the rise in flow was largely due to an increase in intracardiac left to right shunt. It was accompanied by widening of the alveolar-arterial oxygen gradient, perhaps due to imperfect gas equilibration within the lung. PMID:6515594

  16. Cardiovascular risk, chronic obstructive pulmonary disease and pulmonary rehabilitation: Can we learn from cardiac rehabilitation?

    PubMed

    Triest, Filip Jj; Singh, Sally J; Vanfleteren, Lowie Egw

    2016-08-01

    Patients with chronic obstructive pulmonary disease (COPD) who participate in pulmonary rehabilitation (PR) often have concomitant cardiovascular disease (CVD), which is a frequently undiagnosed and undertreated comorbidity. CVD contributes to the burden of the disease and is associated with an increased risk for hospitalizations and mortality. Optimizing the diagnosis and management of cardiovascular risk and disease should be considered as part of the holistic approach of PR. In addition, we need to consider similarities and differences in cardiac and PR programs, in order to improve personalized care in patients with both diseases. The current review addresses the burden of CVD in COPD patients who participate in PR, how CVD and its risk factors affect PR and should be managed during PR, and extends on what we can learn of the organization of cardiac rehabilitation programs. PMID:27081189

  17. Gemcitabine-Induced Pulmonary Toxicity: A Case Report of Pulmonary Veno-Occlusive Disease

    PubMed Central

    Turco, Célia; Jary, Marine; Kim, Stefano; Moltenis, Mélanie; Degano, Bruno; Manzoni, Philippe; Nguyen, Thierry; Genet, Bruno; Rabier, Marie-Blanche Valnet; Heyd, Bruno; Borg, Christophe

    2015-01-01

    INTRODUCTION Gemcitabine is a chemotherapeutic agent frequently used by for the treatment of several malignancies both in the adjuvant and metastatic setting. Although myelosuppression is the most adverse event of this therapy, gemcitabine might induce severe pulmonary toxicities. We describe a case of pulmonary veno-occlusive disease (PVOD) related to gemcitabine. CASE PRESENTATION The patient was an 83-year-old man with a metastatic pancreatic cancer who was treated by gemcitabine as first-line therapy. He was in good health and received no other chemotherapy. A dose of 1000 mg/m2 of gemcitabine was administered over a 30-minute intravenous infusion on days 1, 8, and 15 of a 28-day cycle. After a period of 6 months, a complete response was observed. Nevertheless, the patient developed a severe dyspnea, with arterial hypoxemia and very low lung diffusion for carbon monoxide. A CT scan showed diffuse ground glass opacities with septal lines, bilateral pleural effusion, and lymph node enlargement. On echocardiography, there was a suspicion of pulmonary hypertension with elevated systolic pulmonary artery pressure and normal left ventricular pressures. Right heart catheterization confirmed pulmonary hypertension and normal pulmonary artery occlusion pressure. Diagnosis of PVOD was made, and a gemcitabine-induced toxicity was suspected. A symptomatic treatment was started. At last follow-up, patient was in functional class I with near-normal of CT scan, arterial blood gases, and echocardiography. A gemcitabine-induced PVOD is the more likely diagnosis. PMID:26380562

  18. Treatment of pulmonary diseases with Holmium:YAG laser

    NASA Astrophysics Data System (ADS)

    Zhang, Mei-Jue; Zhu, Jing; Zhang, Hui-Guo; Wang, Fu-Juan; Ke, Lin; Ma, Wei; Luo, Qun-Hua; Zhang, Yue-E.

    1998-11-01

    We report 5 cases of pulmonary disease treated with Holmium:YAG laser through fibrous bronchoscope. 1 inflammatory granuloma was cured after three times of treatment. Compared with conventional methods such as electrocautery and microwave treatment, laser has the merit of good hemostasis effect and quick recovery of the operation area. The other 4 patients who were suffered late lung cancer received 3-7 times of palliative treatment. After the treatment, the tumor tissues become smaller variably, and tact were unobstructed, symptoms of tract- obstructed obviously alleviated. We think that laser treatment has some practical significance in alleviating tract blocking of pulmonary diseases of late stage, and therefore raise the life quality.

  19. The cytokine network in asthma and chronic obstructive pulmonary disease

    PubMed Central

    Barnes, Peter J.

    2008-01-01

    Asthma and chronic obstructive pulmonary disease (COPD) are very common inflammatory diseases of the airways. They both cause airway narrowing and are increasing in incidence throughout the world, imposing enormous burdens on health care. Cytokines play a key role in orchestrating the chronic inflammation and structural changes of the respiratory tract in both asthma and COPD and have become important targets for the development of new therapeutic strategies in these diseases. PMID:18982161

  20. Acute effects of riociguat in borderline or manifest pulmonary hypertension associated with chronic obstructive pulmonary disease

    PubMed Central

    Staehler, Gerd; Grünig, Ekkehard; Halank, Michael; Mitrovic, Veselin; Unger, Sigrun; Mueck, Wolfgang; Frey, Reiner; Grimminger, Friedrich; Schermuly, Ralph T.; Behr, Juergen

    2015-01-01

    Abstract Riociguat is the first oral soluble guanylate cyclase stimulator shown to improve pulmonary hemodynamics in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (PH). This pilot study assessed the impact of a single dose of riociguat on hemodynamics, gas exchange, and lung function in patients with PH associated with chronic obstructive pulmonary disease (COPD). Adults with COPD-associated borderline or manifest PH (pulmonary vascular resistance > 270 dyn·s·cm−5, mean pulmonary artery pressure ≥ 23 mmHg, ratio of forced expiratory volume in 1 second [FEV1] to forced vital capacity < 70%, and partial pressure of oxygen and carbon dioxide in arterial blood > 50 and ≤ 55 mmHg, respectively) received riociguat 1 or 2.5 mg during right heart catheterization. Twenty-two patients completed the study (11 men, 11 women, aged 56–82 years; 1-mg group: n = 10 [mean FEV1: 43.1%]; 2.5-mg group: n = 12 [mean FEV1: 41.2%]). Riociguat caused significant improvements (P < 0.01) from baseline in mean pulmonary artery pressure (1 mg: −3.60 mmHg [−11.44%]; 2.5 mg: −4.83 mmHg [−14.76%]) and pulmonary vascular resistance (1 mg: −58.32 dyn·s·cm−5 [−15.35%]; 2.5 mg: −123.8 dyn·s·cm−5 [−32.96%]). No relevant changes in lung function or gas exchange were observed. Single doses of riociguat were well tolerated and showed promising hemodynamic effects without untoward effects on gas exchange or lung function in patients with COPD-associated PH. Placebo-controlled studies of chronic treatment with riociguat are warranted. PMID:26064454

  1. Multidisciplinary Care of the Patient with Chronic Obstructive Pulmonary Disease

    PubMed Central

    Kuzma, Anne Marie; Meli, Yvonne; Meldrum, Catherine; Jellen, Patricia; Butler-Lebair, Marianne; Koczen-Doyle, Debra; Rising, Peter; Stavrolakes, Kim; Brogan, Frances

    2008-01-01

    The National Emphysema Treatment Trial used a multidisciplinary team approach to implement the maximum medical care protocol, including adjustment of medications and outpatient pulmonary rehabilitation for all patients and nutritional and psychological counseling as needed. This article discusses the benefits of such an approach in the care of the patient with chronic obstructive pulmonary disease. Team member roles complement each other and contribute to the goal of providing the highest-quality medical care. The primary focus of the team is to reinforce the medical plan and to provide patient education and support. This article reviews the elements of the initial patient assessment and the functional and nutritional assessment. Patient education focuses on medication use, recognition and management of chronic obstructive pulmonary disease exacerbation symptoms, smoking cessation, advance directives, and travel. PMID:18453373

  2. Current readings: Percutaneous ablation for pulmonary metastatic disease.

    PubMed

    Quirk, Matthew T; Pomykala, Kelsey L; Suh, Robert D

    2014-01-01

    Percutaneous image-guided ablation is a technique for maintaining local control of metastatic lung lesions that may, in selected patients, confer a survival benefit over no treatment or systemic therapy alone. Although the currently accepted treatment for oligometastatic pulmonary disease is surgical resection, the existing body of literature, including the recent investigations reviewed within this article, supports a role for percutaneous ablation as an important and relatively safe therapeutic option for nonsurgical and in carefully selected surgical patients, conferring survival benefits competitive with surgical metastasectomy. Continued clinical investigations are needed to further understand the nuances of thermal technologies and applications to treat lung primary and secondary pulmonary malignancy, directly compare available therapeutic options and further define the role of percutaneous image-guided ablation in the treatment of pulmonary metastatic disease. PMID:25527018

  3. Accuracy and Internal Consistency of Cardiac Magnetic Resonance Imaging in Measuring Branch Pulmonary Artery Flows in Patients With Conotruncal Anomalies and Branch Pulmonary Artery Stents.

    PubMed

    Harris, Matthew A; Avitabile, Catherine M; Fu, Gregory L; Kim, Daniel W; Kim, Timothy S; Gillespie, Matthew J; Keller, Marc S; Fogel, Mark A; Whitehead, Kevin K

    2016-04-01

    Clinicians use branch pulmonary artery (BPA) blood flow distribution to help determine the need for intervention. Although phase-contrast magnetic resonance (PCMR) flow measurements are accurate, this has never been shown in the vicinity of a BPA ferromagnetic stent (FS) which produces significant susceptibility artifact. We retrospectively reviewed 49 consecutive PCMR studies performed from 2005 to 2012 on patients with repaired conotruncal anomalies and either left (n = 29) or right PA (n = 20) stents. Three methods of measuring the stented BPA flow were compared: (1) main PA (MPA) minus nonstented BPA, (2) direct PCMR of stented BPA away from the artifact, and (3) pulmonary venous flows (ipsilateral to stented BPA and derived pulmonary blood flow ratio from bilateral pulmonary venous flows). Internal consistency was tested with the Student t test, linear regression, Bland-Altman analysis, and intraclass correlation (ICC). The mean age was 11.7 ± 6.9 years with 5.8 ± 4.2 years between stent placement and CMR. There was good agreement without significant difference between MPA-derived stented BPA flow (method 1) and direct PCMR of stented BPA (method 2; 41 ± 19% vs 39 ± 19%, p = 0.59; R(2) = 0.84, p <0.001; ICC = 0.96). There was also good agreement between methods 1 and 2 compared to pulmonary venous flows, with the highest correlation occurring between method 2 and ipsilateral pulmonary venous flow (R(2) = 0.90, p <0.001; ICC = 0.97 for MPA-derived-stented BPA flow; R(2) = 0.94, p <0.001; ICC = 0.98 for direct PCMR of stented BPA). Eleven of the 49 patients (22%) underwent interventional catheterization after PCMR. In conclusion, in the vicinity of a BPA FS, accurate measurement of the net fractional pulmonary blood flow ratio is feasible. PCMR adjacent to the stent and ipsilateral pulmonary venous flows provide the most internally consistent data. These data underscore PCMR's utility in managing patients with implanted FS. PMID:26993977

  4. Pulmonary nocardiosis in Chronic Obstructive Pulmonary Disease: A new clinical challenge

    PubMed Central

    Castellana, Giorgio; Grimaldi, Anna; Castellana, Marco; Farina, Claudio; Castellana, Giuseppe

    2016-01-01

    Pulmonary nocardiosis (PN) is a rare but severe disease caused by Nocardia spp. Despite the traditional description as opportunistic infection, case reports and case series of pulmonary nocardiosis have recently been reported in immunocompetent patients too, in particular among people with chronic pulmonary diseases such as advanced Chronic Obstructive Pulmonary Disease (COPD). PN is characterized by non-specific symptoms and radiological findings; bacteriological culture can be difficult. For the reasons above, diagnosis of PN is challenging, sometimes resulting in a misdiagnosis of tuberculosis. We report an interesting case of PN in a 75-year-old male with COPD. He complained a 3-months history of fatigue, evening rise in body temperature, night sweats, unexplained weight loss of 5 kg, worsening dyspnea, cough and mucopurulent sputum. The chest X-ray showed multiple nodules with cavitations bilaterally in the apical and subclavian regions. Nocardia cyriacigeorgica with 100% identity was identified in three sputum samples. Since the patient has never undergone a systemic and/or inhaled steroid therapy, and has no respiratory failure and comorbidities entailing immunodepression, it is conceivable that, in this immunocompetent patient, the COPD could represent an isolated risk factor for PN. Risk factors, clinical presentations, radiographic findings, differential diagnosis and review of the literature of PN cases in COPD, pointing out the similarities and differences, are also described. PMID:27144111

  5. Pulmonary Rehabilitation and Physical Activity in Patients with Chronic Obstructive Pulmonary Disease.

    PubMed

    Spruit, Martijn A; Pitta, Fabio; McAuley, Edward; ZuWallack, Richard L; Nici, Linda

    2015-10-15

    Physical inactivity is common in patients with chronic obstructive pulmonary disease (COPD) compared with age-matched healthy individuals or patients with other chronic diseases. Physical inactivity independently predicts poor outcomes across several aspects of this disease, but it is (at least in principle) treatable in patients with COPD. Pulmonary rehabilitation has arguably the greatest positive effect of any current therapy on exercise capacity in COPD; as such, gains in this area should facilitate increases in physical activity. Furthermore, because pulmonary rehabilitation also emphasizes behavior change through collaborative self-management, it may aid in the translation of increased exercise capacity to greater participation in activities involving physical activity. Both increased exercise capacity and adaptive behavior change are necessary to achieve significant and lasting increases in physical activity in patients with COPD. Unfortunately, it is readily assumed that this translation occurs naturally. This concise clinical review will focus on the effects of a comprehensive pulmonary rehabilitation program on physical activity in patients with COPD. Changing physical activity behavior in patients with COPD needs an interdisciplinary approach, bringing together respiratory medicine, rehabilitation sciences, social sciences, and behavioral sciences. PMID:26161676

  6. Epidemiology of Pulmonary Hypertension in Left Heart Disease.

    PubMed

    Guha, Ashrith; Amione-Guerra, Javier; Park, Myung H

    2016-01-01

    Pulmonary hypertension (PH) in the setting of left side heart disease is associated with adverse outcomes. The exact prevalence of PH in the different pathologies that affect the left ventricle, however, is difficult to access with the current literature. The lack of a standard definition of PH in older studies, the different modalities to assess pulmonary artery pressures and the varying disease severity, all account for the great variability in the reported prevalence of PH. PH can accompany heart failure (HF) with reduced (HFrEF) or preserved ejection fraction (HFpEF) as well as mitral and aortic valve disease; in any of these instances it is important to recognize whether the elevation of pulmonary pressures is driven by elevated left ventricular pressures only (isolated post-capillary PH) or if there is an accompanying remodeling component in the pulmonary arterioles (combined post-capillary and pre-capillary PH). The objective of this review is to describe the definitions, prevalence and the risk factors associated with the development of PH in the setting of HFrEF, HFpEF and valvular heart disease. PMID:27402130

  7. [Telemedicine for heart failure, diabetes and chronic obstructive pulmonary disease].

    PubMed

    Christiansen, St; Klötzer, J-P

    2016-03-01

    It is generally believed that telemedicine has a tremendous potential to improve health care. This publication reviews the current knowledge available on telemedical concepts for heart failure, diabetes and chronic pulmonary diseases, discusses existing difficulties, and suggests how such concepts could best be used in the near future. PMID:27111954

  8. Evaluation of Continuing Medical Education for Chronic Obstructive Pulmonary Diseases.

    ERIC Educational Resources Information Center

    Li Wang, Virginia; And Others

    1979-01-01

    A continuing medical education program is discussed that addresses chronic obstructive pulmonary disease and that links primary care physicians to a source of needed clinical knowledge at a relatively low cost. The educational methods, evaluation design, diagnosis of educational needs, selection of program content and behavioral outcomes are…

  9. Development of a Measure of Attitude toward Pulmonary Disease Prevention.

    ERIC Educational Resources Information Center

    McGaghie, William C.; And Others

    1993-01-01

    Systematic scale-development procedures, reliability analyses on 2,852 medical students (3 samples), and factor analysis were used to develop and refine a scale reflecting attitudes about pulmonary disease prevention. Development and verification samples included 110 and 2,691 students, respectively. The scale is promising for health education and…

  10. Update on pulmonary disease due to non-tuberculous mycobacteria.

    PubMed

    Stout, Jason E; Koh, Won-Jung; Yew, Wing Wai

    2016-04-01

    Non-tuberculous mycobacteria (NTM) are emerging worldwide as significant causes of chronic pulmonary infection, posing a number of challenges for both clinicians and researchers. While a number of studies worldwide have described an increasing prevalence of NTM pulmonary disease over time, population-based data are relatively sparse and subject to ascertainment bias. Furthermore, the disease is geographically heterogeneous. While some species are commonly implicated worldwide (Mycobacterium avium complex, Mycobacterium abscessus), others (e.g., Mycobacterium malmoense, Mycobacterium xenopi) are regionally important. Thoracic computed tomography, microbiological testing with identification to the species level, and local epidemiology must all be taken into account to accurately diagnose NTM pulmonary disease. A diagnosis of NTM pulmonary disease does not necessarily imply that treatment is required; a patient-centered approach is essential. When treatment is required, multidrug therapy based on appropriate susceptibility testing for the species in question should be used. New diagnostic and therapeutic modalities are needed to optimize the management of these complicated infections. PMID:26976549

  11. Is tuberculosis a lymphatic disease with a pulmonary portal

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Tuberculosis (TB) is commonly viewed as a pulmonary disease, in which infection, persistence, induction of pathology and bacterial expulsion all occur in the lungs. In this model, enlarged lymph nodes represent reactive adenitis and spread of organisms to extrapulmonary sites results in a non-transm...

  12. High-altitude disorders: pulmonary hypertension: pulmonary vascular disease: the global perspective.

    PubMed

    Pasha, M A Qadar; Newman, John H

    2010-06-01

    Globally, it is estimated that > 140 million people live at a high altitude (HA), defined as > 2,500 m (8,200 ft), and that countless others sojourn to the mountains for work, travel, and sport. The distribution of exposure to HA is worldwide, including 35 million in the Andes and > 80 million in Asia, including China and central Asia. HA stress primarily is due to the hypoxia of low atmospheric pressure, but dry air, intense solar radiation, extreme cold, and exercise contribute to acute and chronic disorders. The acute disorders are acute mountain sickness (also known as soroche), HA cerebral edema, and HA pulmonary edema (HAPE). Of these, HAPE is highly correlated with acute pulmonary hypertension. The first chronic syndrome described in HA dwellers in Peru was chronic mountain sickness (Monge disease), which has a large component of relative hypoventilation and secondary erythrocytosis. The prevalence of chronic mountain sickness in HA dwellers ranges from 1.2% in native Tibetans to 5.6% in Chinese Han; 6% to 8% in male residents of La Paz, Bolivia; and 15.6% in the Andes. Subacute mountain sickness is an exaggerated pulmonary hypertensive response to HA hypoxia occurring over months, most often in infants and very young children. Chronic pulmonary hypertension with heart failure but without hypoventilation is seen in Asia. Not only does HA pulmonary hypertension exact health consequences for the millions affected, but also the mechanisms of disease relate to pulmonary hypertension associated with multiple other disorders. Genetic understanding of these disorders is in its infancy. PMID:20522576

  13. Stress failure of pulmonary capillaries: role in lung and heart disease

    NASA Technical Reports Server (NTRS)

    West, J. B.; Mathieu-Costello, O.

    1992-01-01

    Pulmonary capillaries have extremely thin walls to allow rapid exchange of respiratory gases across them. Recently it has been shown that the wall stresses become very large when the capillary pressure is raised, and in anaesthetised rabbits, ultrastructural damage to the walls is seen at pressures of 40 mm Hg and above. The changes include breaks in the capillary endothelial layer, alveolar epithelial layer, and sometimes all layers of the wall. The strength of the thin part of the capillary wall can be attributed to the type IV collagen in the extracellular matrix. Stress failure of pulmonary capillaries results in a high-permeability form of oedema, or even frank haemorrhage, and is apparently the mechanism of neurogenic pulmonary oedema and high-altitude pulmonary oedema. It also explains the exercise-induced pulmonary haemorrhage that occurs in all racehorses. Several features of mitral stenosis are consistent with stress failure. Overinflation of the lung also leads to stress failure, a common cause of increased capillary permeability in the intensive care environment. Stress failure also occurs if the type IV collagen of the capillary wall is weakened by autoantibodies as in Goodpasture's syndrome. Neutrophil elastase degrades type IV collagen and this may be the starting point of the breakdown of alveolar walls that is characteristic of emphysema. Stress failure of pulmonary capillaries is a hitherto overlooked and potentially important factor in lung and heart disease.

  14. Mortality from lung cancer and chronic obstructive pulmonary disease in New Mexico, 1958-82.

    PubMed Central

    Samet, J M; Wiggins, C L; Key, C R; Becker, T M

    1988-01-01

    We examined mortality from lung cancer and from chronic obstructive pulmonary disease in Hispanic White, Other White, and Native American residents of New Mexico during the period 1958-82. Age-specific mortality was calculated by combining death certificate data with population estimates based on the 1960, 1970, and 1980 censuses that were adjusted for inconsistencies in the designation of race and ethnicity. In Other Whites, age-adjusted mortality rates from lung cancer and from chronic obstructive pulmonary disease increased progressively in males and females. Mortality rates for both diseases also increased in Hispanics during the study period, but the most recent rates for Hispanics were well below those for Other Whites. Age-specific mortality rates for lung cancer declined for more recently born Hispanic women at older ages. In Native Americans, rates for both diseases were low throughout the study period and did not show consistent temporal trends. PMID:3407816

  15. Early chronic obstructive pulmonary disease: definition, assessment, and prevention

    PubMed Central

    Rennard, Stephen I; Drummond, M Bradley

    2016-01-01

    Chronic obstructive pulmonary disease (COPD) is the third leading cause of death worldwide. COPD, however, is a heterogeneous collection of diseases with differing causes, pathogenic mechanisms, and physiological effects. Therefore a comprehensive approach to COPD prevention will need to address the complexity of COPD. Advances in the understanding of the natural history of COPD and the development of strategies to assess COPD in its early stages make prevention a reasonable, if ambitious, goal. PMID:25943942

  16. Early chronic obstructive pulmonary disease: definition, assessment, and prevention.

    PubMed

    Rennard, Stephen I; Drummond, M Bradley

    2015-05-01

    Chronic obstructive pulmonary disease (COPD) is the third leading cause of death worldwide. COPD, however, is a heterogeneous collection of diseases with differing causes, pathogenic mechanisms, and physiological effects. Therefore a comprehensive approach to COPD prevention will need to address the complexity of COPD. Advances in the understanding of the natural history of COPD and the development of strategies to assess COPD in its early stages make prevention a reasonable, if ambitious, goal. PMID:25943942

  17. Pulmonary disease management system with distributed wearable sensors.

    PubMed

    Fu, Yongji; Ayyagari, Deepak; Colquitt, Nhedti

    2009-01-01

    A pulmonary disease management system with on-body and near-body sensors is introduced in this presentation. The system is wearable for continuous ambulatory monitoring. Distributed sensor data is transferred through a wireless body area network (BAN) to a central controller for real time analysis. Physiological and environmental parameters are monitored and analyzed using prevailing clinical guidelines for self-management of environmentally-linked pulmonary ailments. The system provides patients with reminders, warnings, and instructions to reduce emergency room and physician visits, and improve clinical outcomes. PMID:19963731

  18. Emerging roles for transthoracic ultrasonography in pulmonary diseases

    PubMed Central

    Sartori, Sergio; Tombesi, Paola

    2010-01-01

    As a result of many advantages such as the absence of radiation exposure, non-invasiveness, low cost, safety, and ready availability, transthoracic ultrasonography (TUS) represents an emerging and useful technique in the management of pleural and pulmonary diseases. In this second part of a comprehensive review that deals with the role of TUS in pleuropulmonary pathology, the normal findings, sonographic artifacts and morphology of the most important and frequent pulmonary diseases are described. In particular, the usefulness of TUS in diagnosing or raising suspicion of pneumonia, pulmonary embolism, atelectasis, diffuse parenchymal diseases, adult and newborn respiratory distress syndrome, lung cancer and lung metastases are discussed, as well as its role in guidance for diagnostic and therapeutic interventional procedures. Moreover, the preliminary data about the role of contrast enhanced ultrasonography in the study of pulmonary pleural-based lesions are also reported. Finally, the limits of TUS when compared with chest computed tomography are described, highlighting the inability of TUS to depict lesions that are not in contact with the pleura or are located under bony structures, poor visualization of the mediastinum, and the need for very experienced examiners to obtain reliable results. PMID:21160632

  19. Pulmonary Hypertension and Right Heart Dysfunction in Chronic Lung Disease

    PubMed Central

    Zangiabadi, Amirmasoud; De Pasquale, Carmine G.; Sajkov, Dimitar

    2014-01-01

    Group 3 pulmonary hypertension (PH) is a common complication of chronic lung disease (CLD), including chronic obstructive pulmonary disease (COPD), interstitial lung disease, and sleep-disordered breathing. Development of PH is associated with poor prognosis and may progress to right heart failure, however, in the majority of the patients with CLD, PH is mild to moderate and only a small number of patients develop severe PH. The pathophysiology of PH in CLD is multifactorial and includes hypoxic pulmonary vasoconstriction, pulmonary vascular remodeling, small vessel destruction, and fibrosis. The effects of PH on the right ventricle (RV) range between early RV remodeling, hypertrophy, dilatation, and eventual failure with associated increased mortality. The golden standard for diagnosis of PH is right heart catheterization, however, evidence of PH can be appreciated on clinical examination, serology, radiological imaging, and Doppler echocardiography. Treatment of PH in CLD focuses on management of the underlying lung disorder and hypoxia. There is, however, limited evidence to suggest that PH-specific vasodilators such as phosphodiesterase-type 5 inhibitors, endothelin receptor antagonists, and prostanoids may have a role in the treatment of patients with CLD and moderate-to-severe PH. PMID:25165714

  20. Pulmonary epithelial permeability in hyaline-membrane disease

    SciTech Connect

    Jefferies, A.L.; Coates, G.; O'Brodovich, H.

    1984-10-25

    Neonatal hyaline-membrane disease is complicated by pulmonary edema, yet left atrial pressures are normal. Alveolar-capillary-membrane permeability may therefore be increased. To assess pulmonary epithelial permeability, we measured the pulmonary clearance and half-life of aerosolized /sup 99m/Tc-diethylenetriamine pentacetate (/sup 99m/Tc-DTPA) on 31 occasions in 15 intubated premature infants with hyaline-membrane disease. Three infants with respiratory failure due to other diseases were studied on four occasions. All studies of infants with hyaline-membrane disease that were performed in the first 72 hours of life demonstrated a biphasic clearance curve with a rapid-phase half-life of 1.6 +/- 0.6 minutes (mean +/- S.D.). As these infants recovered, the curve became monophasic with a half-life of 56.0 +/- 32.1 minutes. Two infants remained dependent on oxygen and ventilator support and had persistent biphasic curves with a rapid-phase half-life of 1.5 +/- 0.7 minutes. All infants without hyaline-membrane disease had monophasic curves with a half-life of 65.4 +/- 33.6 minutes. Using a similar technique, we observed that newborn lambs and piglets have a monophasic pulmonary clearance of /sup 99m/Tc-DTPA (114 +/- 59 minutes in lambs and 52.5 +/- 16.3 minutes in piglets). We conclude that the lungs of neonates with hyaline-membrane disease are abnormally permeable to small solutes and that this abnormality persists in infants with subsequent chronic lung disease.

  1. Animal models of chronic obstructive pulmonary disease.

    PubMed

    Pérez-Rial, Sandra; Girón-Martínez, Álvaro; Peces-Barba, Germán

    2015-03-01

    Animal models of disease have always been welcomed by the scientific community because they provide an approach to the investigation of certain aspects of the disease in question. Animal models of COPD cannot reproduce the heterogeneity of the disease and usually only manage to represent the disease in its milder stages. Moreover, airflow obstruction, the variable that determines patient diagnosis, not always taken into account in the models. For this reason, models have focused on the development of emphysema, easily detectable by lung morphometry, and have disregarded other components of the disease, such as airway injury or associated vascular changes. Continuous, long-term exposure to cigarette smoke is considered the main risk factor for this disease, justifying the fact that the cigarette smoke exposure model is the most widely used. Some variations on this basic model, related to exposure time, the association of other inducers or inhibitors, exacerbations or the use of transgenic animals to facilitate the identification of pathogenic pathways have been developed. Some variations or heterogeneity of this disease, then, can be reproduced and models can be designed for resolving researchers' questions on disease identification or treatment responses. PMID:25201221

  2. The Emerging Importance of Autophagy in Pulmonary Diseases

    PubMed Central

    Mizumura, Kenji; Cloonan, Suzanne M.; Haspel, Jeffrey A.

    2012-01-01

    Important cellular processes such as inflammation, apoptosis, differentiation, and proliferation confer critical roles in the pathogenesis of human diseases. In the past decade, an emerging process named “autophagy” has generated intense interest in both biomedical research and clinical medicine. Autophagy is a regulated cellular pathway for the turnover of organelles and proteins by lysosomal-dependent processing. Although autophagy was once considered a bulk degradation event, research shows that autophagy selectively degrades specific proteins, organelles, and invading bacteria, a process termed “selective autophagy.” It is increasingly clear that autophagy is directly relevant to clinical disease, including pulmonary disease. This review outlines the principal components of the autophagic process and discusses the importance of autophagy and autophagic proteins in pulmonary diseases from COPD, α1-antitrypsin deficiency, pulmonary hypertension, acute lung injury, and cystic fibrosis to respiratory infection and sepsis. Finally, we examine the dual nature of autophagy in the lung, which has both protective and deleterious effects resulting from adaptive and maladaptive responses, and the challenge this duality poses for designing autophagy-based diagnostic and therapeutic targets in lung disease. PMID:23131937

  3. Right or Left: The Role of Nanoparticles in Pulmonary Diseases

    PubMed Central

    Lu, Xuefei; Zhu, Tao; Chen, Chunying; Liu, Ying

    2014-01-01

    Due to the rapid development of the nanotechnology industry in the last decade, nanoparticles (NPs) are omnipresent in our everyday life today. Many nanomaterials have been engineered for medical purposes. These purposes include therapy for pulmonary diseases. On other hand, people are endeavoring to develop nanomaterials for improvement or replacement of traditional therapies. On the other hand, nanoparticles, as foreign material in human bodies, are reported to have potential adverse effects on the lung, including oxidase stress, inflammation, fibrosis and genotoxicity. Further, these damages could induce pulmonary diseases and even injuries in other tissues. It seems that nanoparticles may exert two-sided effects. Toxic effects of nanomaterials should be considered when their use is developed for therapies. Hence this review will attempt to summarize the two-side roles of nanoparticles in both therapies for pulmonary diseases and initiation of lung diseases and even secondary diseases caused by lung injuries. Determinants of these effects such as physicochemical properties of nanoparticles will also be discussed. PMID:25268624

  4. [Cellular senescence and pulmonary disease: COPD as an example].

    PubMed

    Boyer, L; Savale, L; Boczkowski, J; Adnot, S

    2014-12-01

    The biological mechanisms of aging, and more specifically cellular senescence, are increasingly a subject of research. Cellular senescence may be a common determinant of many age-related diseases, including some chronic lung diseases such as chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis. Many arguments suggest that these diseases are associated with premature senescence of lung cells, which may be involved in the pathophysiology of respiratory alterations. Furthermore, these diseases are associated with systemic manifestations, such as bone loss, muscle wasting and atherosclerosis, which impact on symptoms and prognosis. Whether these alterations are related to a common pathogenic mechanism or develop independently in patients with COPD remains an open question. In this review, we will focus on cellular senescence and COPD. Two concepts will be discussed: (1) the role of cell senescence in the pathophysiology of lung destruction, vascular remodeling and inflammation in COPD, (2) the possible link between the pulmonary and systemic manifestations of COPD which could reflect a general process of accelerated aging. PMID:25496787

  5. [Duration of effect of pulmonary rehabilitation in elderly patients with chronic obstructive pulmonary disease].

    PubMed

    Sudo, E; Ohga, E; Matsuse, T; Teramoto, S; Nagase, T; Katayama, H; Tanaka, M; Kikuchi, N; Kakurai, S; Fukuchi, Y; Ouchi, Y

    1997-09-01

    Pulmonary rehabilitation has been reported to be effective in patients with chronic obstructive pulmonary disease (COPD). To investigate the effect of a moderate period of pulmonary rehabilitation combined with inspiratory muscle training (IMT) in elderly patients with COPD, we instituted pulmonary rehabilitation for 60 weeks in five elderly patients (age: 65.4 +/- 3.7 SE years). IMT was performed for 15 min twice daily using a pressure threshold device. The inspiratory threshold was set at 15% of the maximal inspiratory pressure (PImax) of each individual. Maximal peak flow significantly increased from 3.0 +/- 0.6 (L/s) at baseline to 4.1 +/- 0.7 (L/s) after 12 weeks (p < 0.02) and was maintained at that level for 60 weeks. PImax increased significantly from 49.9 +/- 6.4 cmH2O at baseline to 67.3 +/- 3.0 cmH2O after 12 weeks (p < 0.05) and to 72.8 +/- 3.5 cmH2O (p < 0.02) after 60 weeks of rehabilitation. The 10-minute walking distance increased from 666.6 +/- 55.0 to 764.0 +/- 49.9 m (p < 0.05) after 12 weeks and was maintained at the same level. We conclude that pulmonary rehabilitation combined with IMT improves pulmonary function and inspiratory muscle strength in elderly patients with COPD between for 12 weeks after commencement, but occurs that no significant increase on lung function or 10-minute walking distance from 12 to 60 weeks. Respiratory muscle strength may further increase after 60 weeks. PMID:9430986

  6. Chronic Obstructive Pulmonary Disease-Evolving Concepts in Treatment: Advances in Pulmonary Rehabilitation.

    PubMed

    Nici, Linda; ZuWallack, Richard

    2015-08-01

    Over the past three decades, pulmonary rehabilitation has risen to the stature as a gold standard for the treatment of chronic obstructive pulmonary disease (COPD). This rise is owing to both the development of science explaining mechanisms underlying its effectiveness and the demonstration of its substantial benefits across multiple outcome areas of importance to patients. Arguably, pulmonary rehabilitation provides the greatest improvements of any therapy in the areas of dyspnea-relief, exercise performance, and functional and health status. Emerging science also indicates that it reduces subsequent health care utilization and-when administered in the perihospital period-mortality risk. These beneficial effects are realized despite the fact that pulmonary rehabilitation has virtually no direct effect on lung function in COPD. Instead, this comprehensive, patient-centered intervention reduces the negative effects from systemic morbidity (such as muscle wasting) and comorbidity (such as depression and anxiety) that frequently accompany COPD. Two major components of pulmonary rehabilitation are exercise training and behavioral interventions. An example of the latter is a collaborative action plan for the early recognition and prompt treatment of the COPD exacerbation. Innovation in pulmonary rehabilitation includes (1) expanding its applicability, such as demonstrating effectiveness in the non-COPD respiratory patient, in milder COPD, in the periexacerbation period, and its provision in the home and community settings; (2) improving its process, such as refining the self-management and behavioral interventions, and the promotion of physical activity in the home and community settings; and (3) promoting its accessibility, such as exploring its potential usefulness in nontraditional settings (the home and community) and developing technology to assist in its implementation. PMID:26238642

  7. IREB2 and GALC Are Associated with Pulmonary Artery Enlargement in Chronic Obstructive Pulmonary Disease

    PubMed Central

    Lee, Jin Hwa; Cho, Michael H.; Hersh, Craig P.; McDonald, Merry-Lynn N.; Wells, J. Michael; Dransfield, Mark T.; Bowler, Russell P.; Lynch, David A.; Lomas, David A.; Crapo, James D.

    2015-01-01

    Pulmonary hypertension is associated with advanced chronic obstructive pulmonary disease (COPD), although pulmonary vascular changes occur early in the course of the disease. Pulmonary artery (PA) enlargement (PAE) measured by computed tomography correlates with pulmonary hypertension and COPD exacerbation frequency. Genome-wide association studies of PAE in subjects with COPD have not been reported. To investigate whether genetic variants are associated with PAE within subjects with COPD, we investigated data from current and former smokers from the COPDGene Study and the Evaluation of COPD Longitudinally to Identify Predictive Surrogate Endpoints study. The ratio of the diameter of the PA to the diameter of the aorta (A) was measured using computed tomography. PAE was defined as PA/A greater than 1. A genome-wide association study for COPD with PAE was performed using subjects with COPD without PAE (PA/A ≤ 1) as a control group. A secondary analysis used smokers with normal spirometry as a control group. Genotyping was performed on Illumina platforms. The results were summarized using fixed-effect meta-analysis. Both meta-analyses revealed a genome-wide significant locus on chromosome 15q25.1 in IREB2 (COPD with versus without PAE, rs7181486; odds ratio [OR] = 1.32; P = 2.10 × 10−8; versus smoking control subjects, rs2009746; OR = 1.42; P = 1.32 × 10−9). PAE was also associated with a region on 14q31.3 near the GALC gene (rs7140285; OR = 1.55; P = 3.75 × 10−8). Genetic variants near IREB2 and GALC likely contribute to genetic susceptibility to PAE associated with COPD. This study provides evidence for genetic heterogeneity associated with a clinically important COPD vascular subtype. PMID:25101718

  8. IREB2 and GALC are associated with pulmonary artery enlargement in chronic obstructive pulmonary disease.

    PubMed

    Lee, Jin Hwa; Cho, Michael H; Hersh, Craig P; McDonald, Merry-Lynn N; Wells, J Michael; Dransfield, Mark T; Bowler, Russell P; Lynch, David A; Lomas, David A; Crapo, James D; Silverman, Edwin K

    2015-03-01

    Pulmonary hypertension is associated with advanced chronic obstructive pulmonary disease (COPD), although pulmonary vascular changes occur early in the course of the disease. Pulmonary artery (PA) enlargement (PAE) measured by computed tomography correlates with pulmonary hypertension and COPD exacerbation frequency. Genome-wide association studies of PAE in subjects with COPD have not been reported. To investigate whether genetic variants are associated with PAE within subjects with COPD, we investigated data from current and former smokers from the COPDGene Study and the Evaluation of COPD Longitudinally to Identify Predictive Surrogate Endpoints study. The ratio of the diameter of the PA to the diameter of the aorta (A) was measured using computed tomography. PAE was defined as PA/A greater than 1. A genome-wide association study for COPD with PAE was performed using subjects with COPD without PAE (PA/A ≤ 1) as a control group. A secondary analysis used smokers with normal spirometry as a control group. Genotyping was performed on Illumina platforms. The results were summarized using fixed-effect meta-analysis. Both meta-analyses revealed a genome-wide significant locus on chromosome 15q25.1 in IREB2 (COPD with versus without PAE, rs7181486; odds ratio [OR] = 1.32; P = 2.10 × 10(-8); versus smoking control subjects, rs2009746; OR = 1.42; P = 1.32 × 10(-9)). PAE was also associated with a region on 14q31.3 near the GALC gene (rs7140285; OR = 1.55; P = 3.75 × 10(-8)). Genetic variants near IREB2 and GALC likely contribute to genetic susceptibility to PAE associated with COPD. This study provides evidence for genetic heterogeneity associated with a clinically important COPD vascular subtype. PMID:25101718

  9. Chronic obstructive pulmonary disease - adults - discharge

    MedlinePlus

    ... visit when they're all better. Save Your Energy at Home Place items you use often in ... or the skin around your fingernails are blue Alternative Names COPD - adults - discharge; Chronic obstructive airways disease - ...

  10. Underrecognized comorbidities of chronic obstructive pulmonary disease

    PubMed Central

    Miłkowska-Dymanowska, Joanna; Białas, Adam J; Zalewska-Janowska, Anna; Górski, Paweł; Piotrowski, Wojciech J

    2015-01-01

    COPD is associated with different comorbid diseases, and their frequency increases with age. Comorbidities severely impact costs of health care, intensity of symptoms, quality of life and, most importantly, may contribute to life span shortening. Some comorbidities are well acknowledged and established in doctors’ awareness. However, both everyday practice and literature searches provide evidence of other, less recognized diseases, which are frequently associated with COPD. We call them underrecognized comorbidities, and the reason why this is so may be related to their relatively low clinical significance, inefficient literature data, or data ambiguity. In this review, we describe rhinosinusitis, skin abnormalities, eye diseases, different endocrinological disorders, and gastroesophageal reflux disease. Possible links to COPD pathogenesis have been discussed, if the data were available. PMID:26203239

  11. Vasculopathy and pulmonary hypertension in sickle cell disease

    PubMed Central

    Potoka, Karin P.

    2014-01-01

    Sickle cell disease (SCD) is an autosomal recessive disorder in the gene encoding the β-chain of hemoglobin. Deoxygenation causes the mutant hemoglobin S to polymerize, resulting in rigid, adherent red blood cells that are entrapped in the microcirculation and hemolyze. Cardinal features include severe painful crises and episodic acute lung injury, called acute chest syndrome. This population, with age, develops chronic organ injury, such as chronic kidney disease and pulmonary hypertension. A major risk factor for developing chronic organ injury is hemolytic anemia, which releases red blood cell contents into the circulation. Cell free plasma hemoglobin, heme, and arginase 1 disrupt endothelial function, drive oxidative and inflammatory stress, and have recently been referred to as erythrocyte damage-associated molecular pattern molecules (eDAMPs). Studies suggest that in addition to effects of cell free plasma hemoglobin on scavenging nitric oxide (NO) and generating reactive oxygen species (ROS), heme released from plasma hemoglobin can bind to the toll-like receptor 4 to activate the innate immune system. Persistent intravascular hemolysis over decades leads to chronic vasculopathy, with ∼10% of patients developing pulmonary hypertension. Progressive obstruction of small pulmonary arterioles, increase in pulmonary vascular resistance, decreased cardiac output, and eventual right heart failure causes death in many patients with this complication. This review provides an overview of the pathobiology of hemolysis-mediated endothelial dysfunction and eDAMPs and a summary of our present understanding of diagnosis and management of pulmonary hypertension in sickle cell disease, including a review of recent American Thoracic Society (ATS) consensus guidelines for risk stratification and management. PMID:25398989

  12. Forced Expiratory Capnography and Chronic Obstructive Pulmonary Disease (COPD)

    PubMed Central

    Brown, Robert H.; Brooker, Allison; Wise, Robert A.; Reynolds, Curt; Loccioni, Claudio; Russo, Adolfo; Risby, Terence H.

    2013-01-01

    This report proposes a potentially sensitive and simple physiological method to detect early changes and to follow disease progression in obstructive pulmonary disease (COPD) based upon the usual pulmonary function test. Pulmonary function testing is a simple, although relatively insensitive, method to detect and follow COPD. As a proof-of-concept, we have examined the slope of the plateau for carbon dioxide during forced expiratory capnography in healthy (n=10) and COPD subjects (n=10). We compared the change in the rate of exhalation of carbon dioxide over time as a marker of heterogeneous ventilation of the lung. All subjects underwent pulmonary function testing, body-plethysmography, and forced exhalation capnography. The subjects with COPD also underwent high-resolution computed tomography of the chest. Regression lines were fitted to the slopes of the forced exhalation capnogram curves. There was no difference in the mean levels of exhaled carbon dioxide between the COPD and the healthy groups (p>0.48). We found a significant difference in the mean slope of the forced exhalation capnogram for the COPD subjects compared to the healthy subjects (p=0.01). Most important, for the COPD subjects, there was a significant positive correlation between the slope of the forced exhaled capnogram and a defined radiodensity measurement of the lung by high-resolution computed tomography (r2=0.49, p=0.02). The slope of the forced exhalation capnogram may be a simple way to determine physiological changes in the lungs in patients with COPD that are not obtainable with standard pulmonary function tests. Forced exhalation capnography would be of great clinical benefit if it can identify early disease changes and at-risk individuals. PMID:23445906

  13. Vasculopathy and pulmonary hypertension in sickle cell disease.

    PubMed

    Potoka, Karin P; Gladwin, Mark T

    2015-02-15

    Sickle cell disease (SCD) is an autosomal recessive disorder in the gene encoding the β-chain of hemoglobin. Deoxygenation causes the mutant hemoglobin S to polymerize, resulting in rigid, adherent red blood cells that are entrapped in the microcirculation and hemolyze. Cardinal features include severe painful crises and episodic acute lung injury, called acute chest syndrome. This population, with age, develops chronic organ injury, such as chronic kidney disease and pulmonary hypertension. A major risk factor for developing chronic organ injury is hemolytic anemia, which releases red blood cell contents into the circulation. Cell free plasma hemoglobin, heme, and arginase 1 disrupt endothelial function, drive oxidative and inflammatory stress, and have recently been referred to as erythrocyte damage-associated molecular pattern molecules (eDAMPs). Studies suggest that in addition to effects of cell free plasma hemoglobin on scavenging nitric oxide (NO) and generating reactive oxygen species (ROS), heme released from plasma hemoglobin can bind to the toll-like receptor 4 to activate the innate immune system. Persistent intravascular hemolysis over decades leads to chronic vasculopathy, with ∼10% of patients developing pulmonary hypertension. Progressive obstruction of small pulmonary arterioles, increase in pulmonary vascular resistance, decreased cardiac output, and eventual right heart failure causes death in many patients with this complication. This review provides an overview of the pathobiology of hemolysis-mediated endothelial dysfunction and eDAMPs and a summary of our present understanding of diagnosis and management of pulmonary hypertension in sickle cell disease, including a review of recent American Thoracic Society (ATS) consensus guidelines for risk stratification and management. PMID:25398989

  14. A case of pulmonary Langerhans cell histiocytosis in a young woman with coeliac disease.

    PubMed

    Mastruzzo, Claudio; Vancheri, Carlo; Li Mura, Dario; Poletti, Venerino; Failla, Marco; Crimi, Nunzio

    2007-06-01

    Pulmonary Langerhans cells histiocytosis (pulmonary LCH) is an idiopathic unusual lung disease and its association with other systemic diseases has been rarely observed. Here, we describe a young non-smoking woman with concomitant pulmonary LCH and coeliac disease that, despite therapy, suddenly deteriorated. To the best of the authors' knowledge, this is the first report in the medical literature describing an association of coeliac disease with pulmonary LCH. Considering the concomitant occurrence of both diseases in our patient and the severe course of pulmonary LCH observed, we hypothesise that coeliac disease and pulmonary LCH might be related by a common disturbance in immunity and the onset and/or the course of pulmonary LCH could be influenced or markedly worsened by the presence of coeliac disease. PMID:17629812

  15. Mechanics and Function of the Pulmonary Vasculature: Implications for Pulmonary Vascular Disease and Right Ventricular Function

    PubMed Central

    Lammers, Steven; Scott, Devon; Hunter, Kendall; Tan, Wei; Shandas, Robin; Stenmark, Kurt R.

    2012-01-01

    The relationship between cardiac function and the afterload against which the heart muscle must work to circulate blood throughout the pulmonary circulation is defined by a complex interaction between many coupled system parameters. These parameters range broadly and incorporate system effects originating primarily from three distinct locations: input power from the heart, hydraulic impedance from the large conduit pulmonary arteries, and hydraulic resistance from the more distal microcirculation. These organ systems are not independent, but rather, form a coupled system in which a change to any individual parameter affects all other system parameters. The result is a highly nonlinear system which requires not only detailed study of each specific component and the effect of disease on their specific function, but also requires study of the interconnected relationship between the microcirculation, the conduit arteries, and the heart in response to age and disease. Here, we investigate systems-level changes associated with pulmonary hypertensive disease progression in an effort to better understand this coupled relationship. PMID:23487595

  16. [Costs of occupational asthma and chronic obstructive pulmonary disease].

    PubMed

    Pałczyński, Cezary

    2004-05-01

    Many published data indicate that a relevant proportion of cases of asthma and chronic obstructive pulmonary disease (COPD) are caused by occupational exposure. That phenomenon results in many negative economic consequences, including, apart from considerable medical costs, also costs owing to unemployment and claiming for compensation, which are frequent in patients suffering from that illnesses. Hence, it is probable that increasing prevalence of those diseases may result in higher economical costs. The effectiveness of primary and secondary prevention in reduction of costs concerning occupational asthma and COPD has been proved. Only effective prophylaxis may decrease individual and public costs related to both diseases. PMID:15524015

  17. Asthma and other pulmonary diseases in former elite athletes.

    PubMed Central

    Kujala, U. M.; Sarna, S.; Kaprio, J.; Koskenvuo, M.

    1996-01-01

    BACKGROUND: The prevalence of asthma is rising and there are recent reports of increasing asthma rates among top level skiers and runners in the Nordic countries. METHODS: The lifetime occurrence of pulmonary diseases (asthma, chronic bronchitis, emphysema) and current bronchitis symptoms was compared in former elite male athletes (n = 1282) who represented Finland between 1920 and 1965 at least once in international competitions and controls (n = 777) who, at the age of 20, were classified as healthy and who responded to a questionnaire in 1985. The presence of disease and symptoms was identified from the questionnaire and, in the case of asthma, also from a nationwide reimbursable medication register. The death certificates of the subjects of our original cohort who died between 1936 and 1985 were also investigated to determine the cause of death. RESULTS: The occurrence of the pulmonary diseases was associated with age, smoking habits, occupational group, and a history of exposure to chemicals. After adjusting for these variables, athletes who participated in mixed sports (odds ratio (OR) 0.46, 95% confidence interval (CI) 0.23 to 0.92) and power sports (OR 0.43, 95% CI 0.21 to 0.87) had lower odds ratios for emphysema, and endurance sports athletes had a lower odds ratio for the presence of at least one pulmonary disease (OR 0.53, 95% CI 0.28 to 0.98) when compared with controls. Athletes also tended to have fewer reimbursable medications for asthma and fewer current symptoms for chronic bronchitis. Between 1936 and 1985 two controls but none of the athletes died of asthma. CONCLUSIONS: The lifetime occurrence of asthma or other pulmonary diseases is not increased in former elite athletes, and exercise alone, even in a cold environment, did not appear to increase the prevalence of asthma, at least up to the mid 1980s. PMID:8779133

  18. Chronic Obstructive Pulmonary Disease: Evidence for an Autoimmune Component

    PubMed Central

    Stefanska, Anna M; Walsh, Patrick T

    2009-01-01

    Chronic obstructive pulmonary disease (COPD) is characterized by an irreversible limitation on pulmonary airflow associated with chronic inflammation and mucous hypersecretion (chronic bronchitis) and/or the pathological destruction of alveolar airspaces leading to emphysema. COPD, predominantly as a result of tobacco smoke exposure, represents the fourth leading cause of mortality worldwide and its prevalence is increasing. Despite this, much of the basic mechanisms which contribute to disease progression remain to be elucidated and current therapeutic approaches are, for the most part, based upon alleviating patient symptoms (bronchodilators) as opposed to treating the underlying pathological mechanisms triggered in response to cigarette smoke exposure. The classic disease paradigm suggests that an imbalance of pulmonary matrix proteases versus anti-proteases underlies the tissue destruction and inflammation associated with COPD. However, there is a growing appreciation of the complex and multifaceted nature of the pathological mechanisms associated with disease progression. Recently, there has been mounting evidence indicating that COPD patients exhibit many of the characteristics of a classical autoimmune response. We will discuss current evidence in support of this paradigm and outline how future therapeutic approaches may be tailored to address this. PMID:19403056

  19. Pulmonary function tests and impulse oscillometry in severe chronic obstructive pulmonary disease patients’ offspring

    PubMed Central

    Amra, Babak; Borougeni, Victoria Beigi; Golshan, Mohammad; Soltaninejad, Forogh

    2015-01-01

    Background: Several studies have showed an increased prevalence of airflow obstruction in first degree relatives of individuals with chronic obstructive pulmonary disease (COPD). Considering no specific research had evaluated airway resistance in offspring of patients with severe COPD, we utilized a spirometry and a impulse oscillometry (IO) to evaluate this population. Materials and Methods: In this case control study, from November 2011 to July 2012, we consecutively evaluated 54 offsprings of severe COPD patients (case group) admitted in the pulmonary ward, affiliated to the Isfahan University of Medical Sciences and control group. Pulmonary function tests and the IO were obtained for both groups. Student's t-test was used for inter-group comparisons, and P values below 0.05 were taken as significant. Results: Abnormal increased airway resistance was seen in cases in comparison with controls (R5 Hz [46.29%, P = 0.01], R25 Hz [42.59%, P < 0.001]). Also, considering the spirometry, case group had pulmonary function parameters less than control group (forced vital capacity [FVC]; P = 0.02, forced expiratory volume in 1st s; P < 0.001, forced expiratory flow (FEF) 25-75; P < 0.001, FEF 25-75/FVC; P < 0.001) but they were in normal range. Conclusion: This study demonstrated increased airway resistance among the severe COPD offsprings. The IO may be a sensitive tool for detection of high risk subjects in families with COPD. PMID:26622261

  20. Development of the chronic obstructive pulmonary disease activity rating scale: reliability, validity and factorial structure.

    PubMed

    Morimoto, Michiko; Takai, Kenichi; Nakajima, Kazuo; Kagawa, Koujiro

    2003-03-01

    The purpose of the present study was to develop the Chronic Obstructive Pulmonary Disease (COPD) Activity Rating Scale (CARS) to measure life-related activity in patients with COPD, and to confirm its reliability and constructive validity in a factorial structure model. The subjects consisted of 114 patients with COPD. An 88-item life-related activity list, generated previously from a literature review, was administered. The secondary structural model consisted of four factors with 12 items. The results of the confirmatory factor analysis by structural equation modeling showed the fit criteria to be statistically significant. The internal consistency of the 12 items was highly reliable (Cronbach's alpha = 0.924). The CARS score was correlated with pulmonary function tests, breathlessness, and the health-related quality of life (QOL) scales in Pearson correlation coefficient. The results suggest that the COPD Activity Rating Scale is a valid scale for the assessment of life-related activity in patients with COPD. PMID:12603718

  1. Glycopyrronium bromide for the treatment of chronic obstructive pulmonary disease.

    PubMed

    Riario-Sforza, Gian Galeazzo; Ridolo, Erminia; Riario-Sforza, Edoardo; Incorvaia, Cristoforo

    2015-02-01

    Glycopyrronium bromide is a new long-acting muscarinic antagonist to be used once-daily, which is approved as a bronchodilator for the symptomatic maintenance treatment of adult patients with chronic obstructive pulmonary disease (COPD). In the Glycopyrronium bromide in chronic Obstructive pulmonary disease airWays trials, treatment with inhaled glycopyrronium bromide at 50 μg once daily achieved a significantly better lung function than placebo, as measured by the trough forced expiratory volume in 1 s in patients with moderate-to-severe COPD. The lung function improvement was maintained for up to 52 weeks. Other improved indexes were dyspnea scores, health status, exacerbation rates and time of exercise endurance. Studies comparing the efficacy of glycopyrronium versus tiotropium bromide found substantial equivalence of the two drugs. Glycopyrronium was generally well tolerated. These data add inhaled glycopyrronium bromide to the treatment of patients with moderate to severe COPD as an effective once-daily LAMA. PMID:25547422

  2. Chronic Ethanol Exposure: Pathogenesis of Pulmonary Disease and Dysfunction

    PubMed Central

    Traphagen, Nicole; Tian, Zhi; Allen-Gipson, Diane

    2015-01-01

    Ethanol (EtOH) is the world’s most commonly used drug, and has been widely recognized as a risk factor for developing lung disorders. Chronic EtOH exposure affects all of the organ systems in the body and increases the risk of developing pulmonary diseases such as acute lung injury and pneumonia, while exacerbating the symptoms and resulting in increased mortality in many other lung disorders. EtOH and its metabolites inhibit the immune response of alveolar macrophages (AMs), increase airway leakage, produce damaging reactive oxygen species (ROS), and disrupt the balance of antioxidants/oxidants within the lungs. In this article, we review the role of EtOH exposure in the pathogenesis and progression of pulmonary disease. PMID:26492278

  3. Chronic obstructive pulmonary disease secondary to household air pollution.

    PubMed

    Assad, Nour A; Balmes, John; Mehta, Sumi; Cheema, Umar; Sood, Akshay

    2015-06-01

    Approximately 3 billion people around the world cook and heat their homes using solid fuels in open fires and rudimentary stoves, resulting in household air pollution. Household air pollution secondary to indoor combustion of solid fuel is associated with multiple chronic obstructive pulmonary disease (COPD) outcomes. The exposure is associated with both chronic bronchitis and emphysema phenotypes of COPD as well as a distinct form of obstructive airway disease called bronchial anthracofibrosis. COPD from household air pollution differs from COPD from tobacco smoke with respect to its disproportionately greater bronchial involvement, lesser emphysematous change, greater impact on quality of life, and possibly greater oxygen desaturation and pulmonary hypertensive changes. Interventions that decrease exposure to biomass smoke may decrease the risk for incident COPD and attenuate the longitudinal decline in lung function, but more data on exposure-response relationships from well-designed longitudinal studies are needed. PMID:26024348

  4. Homozygosity for moyamoya disease risk allele leads to moyamoya disease with extracranial systemic and pulmonary vasculopathy.

    PubMed

    Fukushima, Hiroyuki; Takenouchi, Toshiki; Kosaki, Kenjiro

    2016-09-01

    Moyamoya disease is characterized by diffuse distal intracranial stenosis. Recently, RNF213 has been identified as a susceptibility gene in the development of this condition. Pulmonary hypertension is a rare progressive vasculopathy with an unknown etiology. The co-occurrence of pulmonary hypertension and Moyamoya disease has been described in four patients; however, whether this co-occurrence represents a chance association or a common vascular pathology has remained unknown. Here, we report two unrelated male patients who presented during their childhood with dyspnea on exertion. Systemic vascular imaging studies revealed the presence of pulmonary hypertension and Moyamoya disease in both patients. Medical exome sequencing revealed that both patients had a homozygous mutation for p.Arg4810Lys in RNF213. We suggest that homozygosity in RNF213 may lead to a novel entity involving the brain and lung. Interestingly, when present in a heterozygous state, this mutation causes a classic cerebral vascular disease, Moyamoya disease. In the homozygous state, the exact same mutation led to Moyamoya disease with extracranial systemic vasculopathy in at least two patients. From a clinical standpoint, cerebrovascular or pulmonary vascular investigations may be warranted in patients with pulmonary hypertension or Moyamoya disease, respectively. © 2016 Wiley Periodicals, Inc. PMID:27375007

  5. Pulmonary administration of phosphoinositide 3-kinase inhibitor is a curative treatment for chronic obstructive pulmonary disease by alveolar regeneration.

    PubMed

    Horiguchi, Michiko; Oiso, Yuki; Sakai, Hitomi; Motomura, Tomoki; Yamashita, Chikamasa

    2015-09-10

    Chronic obstructive pulmonary disease (COPD) is an intractable pulmonary disease, causing widespread and irreversible alveoli collapse. The discovery of a low-molecular-weight compound that induces regeneration of pulmonary alveoli is of utmost urgency to cure intractable pulmonary diseases such as COPD. However, a practically useful compound for regenerating pulmonary alveoli is yet to be reported. Previously, we have elucidated that Akt phosphorylation is involved in a differentiation-inducing molecular mechanism of human alveolar epithelial stem cells, which play a role in regenerating pulmonary alveoli. In the present study, we directed our attention to phosphoinositide 3-kinase (PI3K)-Akt signaling and examined whether PI3K inhibitors display the pulmonary alveolus regeneration. Three PI3K inhibitors with different PI3K subtype specificities (Wortmannin, AS605240, PIK-75 hydrochloride) were tested for the differentiation-inducing effect on human alveolar epithelial stem cells, and Wortmannin demonstrated the most potent differentiation-inducing activity. We evaluated Akt phosphorylation in pulmonary tissues of an elastase-induced murine COPD model and found that Akt phosphorylation in the pulmonary tissue was enhanced in the murine COPD model compared with normal mice. Then, the alveolus-repairing effect of pulmonary administration of Wortmannin to murine COPD model was evaluated using X-ray CT analysis and hematoxylin-eosin staining. As a result, alveolar damages were repaired in the Wortmannin-administered group to a similar level of normal mice. Furthermore, pulmonary administration of Wortmannin induced a significant recovery of the respiratory function, compared to the control group. These results indicate that Wortmannin is capable of inducing differentiation of human alveolar epithelial stem cells and represents a promising drug candidate for curative treatment of pulmonary alveolar destruction in COPD. PMID:26160307

  6. Pulmonary Epithelioid Hemangioendothelioma in a Patient with Crohn's Disease

    PubMed Central

    Ramchandar, Nanda; Wojtczak, Henry A.

    2015-01-01

    Pulmonary epithelioid hemangioendothelioma (PEH) is a rare neoplasm, largely unresponsive to chemotherapeutic medications, and with varied prognosis. Imaging on computerized tomography may demonstrate perivascular nodules, but diagnosis is ultimately made on biopsy with immunohistochemical analysis. Here we describe a case of PEH in a 14-year-old male with Crohn's disease, which, to our knowledge, has not previously been described in the literature. PMID:25815232

  7. Pulmonary Function and the Risk of Functional Limitation in Chronic Obstructive Pulmonary Disease

    PubMed Central

    Eisner, Mark D.; Iribarren, Carlos; Yelin, Edward H.; Sidney, Stephen; Katz, Patricia P.; Ackerson, Lynn; Lathon, Phenius; Tolstykh, Irina; Omachi, Theodore; Byl, Nancy; Blanc, Paul D.

    2008-01-01

    The authors’ objective was to analyze the impact of respiratory impairment on the risk of physical functional limitations among adults with chronic obstructive pulmonary disease (COPD). They hypothesized that greater pulmonary function decrement would result in a broad array of physical functional limitations involving organ systems remote from the lung, a key step in the pathway leading to overall disability. The authors used baseline data from the Function, Living, Outcomes, and Work (FLOW) study, a prospective cohort study of adults with COPD recruited from northern California in 2005–2007. They studied the impact of pulmonary function impairment on the risk of functional limitations using validated measures: lower extremity function (Short Physical Performance Battery), submaximal exercise performance (6-Minute Walk Test), standing balance (Functional Reach Test), skeletal muscle strength (manual muscle testing with dynamometry), and self-reported functional limitation (standardized item battery). Multiple variable analysis was used to control for confounding by age, sex, race, height, educational attainment, and cigarette smoking. Greater pulmonary function impairment, as evidenced by lower forced expiratory volume in 1 second (FEV1), was associated with poorer Short Physical Performance Battery scores and less distance walked during the 6-Minute Walk Test. Lower forced expiratory volume in 1 second was also associated with weaker muscle strength and with a greater risk of self-reported functional limitation (p < 0.05). In conclusion, pulmonary function impairment is associated with multiple manifestations of physical functional limitation among COPD patients. Longitudinal follow-up can delineate the impact of these functional limitations on the prospective risk of disability, guiding preventive strategies that could attenuate the disablement process. PMID:18343879

  8. Pulmonary Involvement in Niemann-Pick Disease: A State-of-the-Art Review.

    PubMed

    von Ranke, Felipe Mussi; Pereira Freitas, Heloisa Maria; Mançano, Alexandre Dias; Rodrigues, Rosana Souza; Hochhegger, Bruno; Escuissato, Dante; Araujo Neto, Cesar Augusto; da Silva, Thiago Krieger Bento; Marchiori, Edson

    2016-08-01

    Niemann-Pick disease is a rare autosomal recessive lysosomal storage disease with three subtypes. Types A and B result from a deficiency of acid sphingomyelinase activity, associated with the accumulation of lipid-laden macrophages (so-called Niemann-Pick cells) in various tissues, especially the liver and spleen. Type A is a fatal neurodegenerative disorder of infancy. Type B Niemann-Pick disease is a less severe form with milder neurological involvement, characterized by hepatosplenomegaly, hyperlipidemia, and pulmonary involvement; most patients live into adulthood. Type C Niemann-Pick disease is a complex lipid storage disorder caused by defects in cholesterol trafficking, resulting in a clinical presentation dominated by neurological involvement. Pulmonary involvement occurs in all three types of Niemann-Pick disease, but most frequently in type B. Respiratory manifestations range from a lack of symptoms to respiratory failure. Progression of respiratory disease is slow, but inexorable, due to the accumulation of Niemann-Pick cells in the alveolar septa, bronchial walls, and pleura, potentially leading to a progressively worsening restrictive pattern on pulmonary function testing. Bronchoalveolar lavage has important diagnostic value because it shows the presence of characteristic Niemann-Pick cells. Radiographic findings consist of a reticular or reticulonodular pattern and, eventually, honeycombing, involving mainly the lower lung zones. The most common changes identified by high-resolution computed tomography are ground-glass opacities, mild smooth interlobular septal thickening, and intralobular lines. The aim of this review is to describe the main clinical, imaging, and pathological aspects of Niemann-Pick disease, with a focus on pulmonary involvement. PMID:27164983

  9. Noninvasive mechanical ventilation in chronic obstructive pulmonary disease and in acute cardiogenic pulmonary edema.

    PubMed

    Rialp Cervera, G; del Castillo Blanco, A; Pérez Aizcorreta, O; Parra Morais, L

    2014-03-01

    Noninvasive ventilation (NIV) with conventional therapy improves the outcome of patients with acute respiratory failure due to hypercapnic decompensation of chronic obstructive pulmonary disease (COPD) or acute cardiogenic pulmonary edema (ACPE). This review summarizes the main effects of NIV in these pathologies. In COPD, NIV improves gas exchange and symptoms, reducing the need for endotracheal intubation, hospital mortality and hospital stay compared with conventional oxygen therapy. NIV may also avoid reintubation and may decrease the length of invasive mechanical ventilation. In ACPE, NIV accelerates the remission of symptoms and the normalization of blood gas parameters, reduces the need for endotracheal intubation, and is associated with a trend towards lesser mortality, without increasing the incidence of myocardial infarction. The ventilation modality used in ACPE does not affect the patient prognosis. PMID:23158869

  10. Pulmonary vascular disease in mice xenografted with human BM progenitors from patients with pulmonary arterial hypertension

    PubMed Central

    Farha, Samar; Lichtin, Alan; Graham, Brian; George, Deepa; Aldred, Micheala; Hazen, Stanley L.; Loyd, James; Tuder, Rubin

    2012-01-01

    Hematopoietic myeloid progenitors released into the circulation are able to promote vascular remodeling through endothelium activation and injury. Endothelial injury is central to the development of pulmonary arterial hypertension (PAH), a proliferative vasculopathy of the pulmonary circulation, but the origin of vascular injury is unknown. In the present study, mice transplanted with BM-derived CD133+ progenitor cells from patients with PAH, but not from healthy controls, exhibited morbidity and/or death due to features of PAH: in situ thrombi and endothelial injury, angioproliferative remodeling, and right ventricular hypertrophy and failure. Myeloid progenitors from patients with heritable and/or idiopathic PAH all produced disease in xenografted mice. Analyses of hematopoietic transcription factors and colony formation revealed underlying abnormalities of progenitors that skewed differentiation toward the myeloid-erythroid lineage. The results of the present study suggest a causal role for hematopoietic stem cell abnormalities in vascular injury, right ventricular hypertrophy, and morbidity associated with PAH. PMID:22745307

  11. Pulmonary hypertension in chronic obstructive and interstitial lung diseases.

    PubMed

    Andersen, Charlotte U; Mellemkjær, Søren; Nielsen-Kudsk, Jens Erik; Bendstrup, Elisabeth; Hilberg, Ole; Simonsen, Ulf

    2013-10-01

    The purpose of the present review is to summarize the current knowledge on PH in relation to COPD and ILD from a clinical perspective with emphasis on diagnosis, biomarkers, prevalence, impact, treatment, and practical implications. PH in COPD and ILD is associated with a poor prognosis, and is considered one of the most frequent types of PH. However, the prevalence of PH among patients with COPD and ILD is not clear. The diagnosis of PH in chronic lung disease is often established by echocardiographic screening, but definitive diagnosis requires right heart catheterization, which is not systematically performed in clinical practice. Given the large number of patients with chronic lung disease, biomarkers to preclude or increase suspicion of PH are needed. NT-proBNP may be used as a rule-out test, but biomarkers with a high specificity for PH are still required. It is not known whether specific treatment with existent drugs effective in pulmonary arterial hypertension (PAH) is beneficial in lung disease related PH. Studies investigating existing PAH drugs in animal models of lung disease related PH have indicated a positive effect, and so have case reports and open label studies. However, treatment with systemically administered pulmonary vasodilators implies the risk of worsening the ventilation-perfusion mismatch in patients with lung disease. Inhaled vasodilators may be better suited for PH in lung disease, but new treatment modalities are also required. PMID:23849967

  12. Deciphering the genetic and modular connections between coronary heart disease, idiopathic pulmonary arterial hypertension and pulmonary heart disease

    PubMed Central

    YUAN, YE; ZHANG, YINGYING; ZHANG, XIAOXU; YU, YANAN; LI, BING; WANG, PENGQIAN; LI, HAIXIA; ZHAO, YIJUN; SHEN, CHUNTI; WANG, ZHONG

    2016-01-01

    Coronary heart disease (CHD), idiopathic pulmonary arterial hypertension (IPAH) and pulmonary heart disease (PHD) are circulatory system diseases that may simultaneously emerge in a patient and they are often treated together in clinical practice. However, the molecular mechanisms connecting these three diseases remain unclear. In order to determine the multidimensional characteristic correlations between these three diseases based on genomic networks to aid in medical decision-making, genes from the Online Mendelian Inheritance in Man database were obtained, and applied network construction and modularized analysis were conducted. Functional enrichment analysis was conducted to explore the associations between overlapping genes, modules and pathways. A total of 29 overlapping genes and 3 common modules were identifed for the 3 diseases. Glycosphingolipid biosynthesis and the arachidonic acid metabolism are common pathways, and the biosynthetic process is suggested to be the major function involved in the three diseases. The current study reported, to the best of our knowledge for the first time, the role of glycosphingolipid biosynthesis in IPAH and PHD. The present study provided an improved understanding of the pathological mechanisms underlying CHD, IPAH and PHD. The overlapping genes, modules and pathways suggest novel areas for further research, and drug targets. The observations of the current study additionally suggest that drug indications can be broadened because of the presence of common targets. PMID:27221156

  13. Deciphering the genetic and modular connections between coronary heart disease, idiopathic pulmonary arterial hypertension and pulmonary heart disease.

    PubMed

    Yuan, Ye; Zhang, Yingying; Zhang, Xiaoxu; Yu, Yanan; Li, Bing; Wang, Pengqian; Li, Haixia; Zhao, Yijun; Shen, Chunti; Wang, Zhong

    2016-07-01

    Coronary heart disease (CHD), idiopathic pulmonary arterial hypertension (IPAH) and pulmonary heart disease (PHD) are circulatory system diseases that may simultaneously emerge in a patient and they are often treated together in clinical practice. However, the molecular mechanisms connecting these three diseases remain unclear. In order to determine the multidimensional characteristic correlations between these three diseases based on genomic networks to aid in medical decision-making, genes from the Online Mendelian Inheritance in Man database were obtained, and applied network construction and modularized analysis were conducted. Functional enrichment analysis was conducted to explore the associations between overlapping genes, modules and pathways. A total of 29 overlapping genes and 3 common modules were identifed for the 3 diseases. Glycosphingolipid biosynthesis and the arachidonic acid metabolism are common pathways, and the biosynthetic process is suggested to be the major function involved in the three diseases. The current study reported, to the best of our knowledge for the first time, the role of glycosphingolipid biosynthesis in IPAH and PHD. The present study provided an improved understanding of the pathological mechanisms underlying CHD, IPAH and PHD. The overlapping genes, modules and pathways suggest novel areas for further research, and drug targets. The observations of the current study additionally suggest that drug indications can be broadened because of the presence of common targets. PMID:27221156

  14. Connective tissue disease-related pulmonary arterial hypertension.

    PubMed

    Thakkar, Vivek; Lau, Edmund M T

    2016-02-01

    Over the past two decades, there have been several advances in the assessment and management of connective tissue disease-related pulmonary arterial hypertension (CTD-PAH) that improved outcomes of the treatment of this lethal disease, and this will be the focus of this study. Systemic sclerosis is the leading cause of CTD-PAH, followed by systemic lupus erythematosus, mixed connective tissue disease, idiopathic inflammatory myositis, rheumatoid arthritis, and Sjogren's syndrome. Clinical registries have been invaluable in informing about the burden of disease, risk and prognostic factors, and temporal trends with respect to treatment and outcome in CTD-PAH. The major advances have centered on improved disease classification and diagnostic criteria, screening and early diagnosis, the emergence of evidence-based therapies including combination goal-orientated treatment strategies, and the establishment of centers with expertise in PAH. PMID:27421214

  15. Gas exchange in disease: asthma, chronic obstructive pulmonary disease, cystic fibrosis, and interstitial lung disease.

    PubMed

    Young, Iven H; Bye, Peter T P

    2011-04-01

    Ventilation-perfusion (VA/Q) inequality is the underlying abnormality determining hypoxemia and hypercapnia in lung diseases. Hypoxemia in asthma is characterized by the presence of low VA/Q units, which persist despite improvement in airway function after an attack. This hypoxemia is generally attenuated by compensatory redistribution of blood flow mediated by hypoxic vasoconstriction and changes in cardiac output, however, mediator release and bronchodilator therapy may cause deterioration. Patients with chronic obstructive pulmonary disease have more complex patterns of VA/Q inequality, which appear more fixed, and changes in blood flow and ventilation have less benefit in improving gas exchange efficiency. The inability of ventilation to match increasing cardiac output limits exercise capacity as the disease progresses. Deteriorating hypoxemia during exacerbations reflects the falling mixed venous oxygen tension from increased respiratory muscle activity, which is not compensated by any redistribution of VA/Q ratios. Shunt is not a feature of any of these diseases. Patients with cystic fibrosis (CF) have no substantial shunt when managed according to modern treatment regimens. Interstitial lung diseases demonstrate impaired oxygen diffusion across the alveolar-capillary barrier, particularly during exercise, although VA/Q inequality still accounts for most of the gas exchange abnormality. Hypoxemia may limit exercise capacity in these diseases and in CF. Persistent hypercapnic respiratory failure is a feature of advancing chronic obstructive pulmonary disease and CF, closely associated with sleep disordered breathing, which is not a prominent feature of the other diseases. Better understanding of the mechanisms of hypercapnic respiratory failure, and of the detailed mechanisms controlling the distribution of ventilation and blood flow in the lung, are high priorities for future research. PMID:23737199

  16. Treatment of pulmonary arterial hypertension in connective tissue disease.

    PubMed

    Grünig, Ekkehard

    2012-05-28

    Pulmonary arterial hypertension (PAH) is a group of distinct disorders that includes idiopathic PAH (IPAH), familial PAH and PAH associated with other conditions (APAH) such as connective tissue disease (CTD-APAH) or congenital heart disease. PAH is characterized by increased pulmonary arterial pressure and pulmonary vascular resistance. If left untreated, PAH can lead to right heart failure and premature death. CTD-APAH represents an important clinical subgroup of APAH that has a higher risk of death than IPAH. The European treatment guidelines advocate the use of PAH-targeted therapies including bosentan, ambrisentan, sildenafil, inhaled iloprost, intravenous epoprostenol (I-A recommendations), tadalafil or treprostinil (I-B recommendations) for patients in WHO functional class II-III. Not all randomized clinical studies of the approved PAH-targeted therapies have included patients with CTD-APAH. The purpose of this review is to describe the clinical characteristics of CTD-APAH and discuss the approved pharmacological treatments, with a focus on data specific to this subgroup where possible. PMID:22621693

  17. Connective tissue disease-associated pulmonary arterial hypertension

    PubMed Central

    Howard, Luke S.

    2015-01-01

    Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sclerosis to PAH-specific therapy appears to be worse than in other forms of PAH. Survival in systemic sclerosis-associated PAH is inferior to that observed in idiopathic PAH. Potential reasons for this include differences in age, the nature of the underlying pulmonary vasculopathy and the ability of the right ventricle to cope with increased afterload between patients with systemic sclerosis-associated PAH and idiopathic PAH, while coexisting cardiac and pulmonary disease is common in systemic sclerosis-associated PAH. Other forms of connective tissue-associated PAH have been less well studied, however PAH associated with systemic lupus erythematosus (SLE) has a better prognosis than systemic sclerosis-associated PAH and likely responds to immunosuppression. PMID:25705389

  18. Pulmonary manifestations in adult patients with chronic granulomatous disease.

    PubMed

    Salvator, Hélène; Mahlaoui, Nizar; Catherinot, Emilie; Rivaud, Elisabeth; Pilmis, Benoit; Borie, Raphael; Crestani, Bruno; Tcherakian, Colas; Suarez, Felipe; Dunogue, Bertrand; Gougerot-Pocidalo, Marie-Anne; Hurtado-Nedelec, Margarita; Dreyfus, Jean-François; Durieu, Isabelle; Fouyssac, Fanny; Hermine, Olivier; Lortholary, Olivier; Fischer, Alain; Couderc, Louis-Jean

    2015-06-01

    Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by failure of superoxide production in phagocytic cells. The disease is characterised by recurrent infections and inflammatory events, frequently affecting the lungs. Improvement of life expectancy now allows most patients to reach adulthood. We aimed to describe the pattern of pulmonary manifestations occurring during adulthood in CGD patients. This was a retrospective study of the French national cohort of adult patients (≥16 years old) with CGD. Medical data were obtained for 67 adult patients. Pulmonary manifestations affected two-thirds of adult patients. Their incidence was significantly higher than in childhood (mean annual rate 0.22 versus 0.07, p=0.01). Infectious risk persisted despite anti-infectious prophylaxis. Invasive fungal infections were frequent (0.11 per year per patient) and asymptomatic in 37% of the cases. They often required lung biopsy for diagnosis (10 out of 30). Noninfectious respiratory events concerned 28% of adult patients, frequently associated with a concomitant fungal infection (40%). They were more frequent in patients with the X-linked form of CGD. Immune-modulator therapies were required in most cases (70%). Respiratory manifestations are major complications of CGD in adulthood. Noninfectious pulmonary manifestations are as deleterious as infectious pneumonia. A specific respiratory monitoring is necessary. PMID:25614174

  19. Role of Reactive Oxygen Species in Neonatal Pulmonary Vascular Disease

    PubMed Central

    Steinhorn, Robin H.

    2014-01-01

    Abstract Significance: Abnormal lung development in the perinatal period can result in severe neonatal complications, including persistent pulmonary hypertension (PH) of the newborn and bronchopulmonary dysplasia. Reactive oxygen species (ROS) play a substantive role in the development of PH associated with these diseases. ROS impair the normal pulmonary artery (PA) relaxation in response to vasodilators, and ROS are also implicated in pulmonary arterial remodeling, both of which can increase the severity of PH. Recent Advances: PA ROS levels are elevated when endogenous ROS-generating enzymes are activated and/or when endogenous ROS scavengers are inactivated. Animal models have provided valuable insights into ROS generators and scavengers that are dysregulated in different forms of neonatal PH, thus identifying potential therapeutic targets. Critical Issues: General antioxidant therapy has proved ineffective in reversing PH, suggesting that it is necessary to target specific signaling pathways for successful therapy. Future Directions: Development of novel selective pharmacologic inhibitors along with nonantioxidant therapies may improve the treatment outcomes of patients with PH, while further investigation of the underlying mechanisms may enable earlier detection of the disease. Antioxid. Redox Signal. 21, 1926–1942. PMID:24350610

  20. Pulmonary CT image registration and warping for tracking tissue deformation during the respiratory cycle through 3D consistent image registration

    PubMed Central

    Li, Baojun; Christensen, Gary E.; Hoffman, Eric A.; McLennan, Geoffrey; Reinhardt, Joseph M.

    2008-01-01

    Tracking lung tissues during the respiratory cycle has been a challenging task for diagnostic CT and CT-guided radiotherapy. We propose an intensity- and landmark-based image registration algorithm to perform image registration and warping of 3D pulmonary CT image data sets, based on consistency constraints and matching corresponding airway branchpoints. In this paper, we demonstrate the effectivenss and accuracy of this algorithm in tracking lung tissues by both animal and human data sets. In the animal study, the result showed a tracking accuracy of 1.9 mm between 50% functional residual capacity (FRC) and 85% total lung capacity (TLC) for 12 metal seeds implanted in the lungs of a breathing sheep under precise volume control using a pulmonary ventilator. Visual inspection of the human subject results revealed the algorithm’s potential not only in matching the global shapes, but also in registering the internal structures (e.g., oblique lobe fissures, pulmonary artery branches, etc.). These results suggest that our algorithm has significant potential for warping and tracking lung tissue deformation with applications in diagnostic CT, CT-guided radiotherapy treatment planning, and therapeutic effect evaluation. PMID:19175115

  1. Biomarkers in Alpha-1 Antitrypsin Deficiency Chronic Obstructive Pulmonary Disease.

    PubMed

    Turino, Gerard M; Ma, Shuren; Cantor, Jerome O; Lin, Yong Y

    2016-08-01

    Biomarkers of pathogenesis in chronic obstructive pulmonary disease (COPD) can significantly accelerate drug development. In COPD related to alpha-1 antitrypsin deficiency, the role of neutrophil elastase and its inhibition by alpha-1 antitrypsin protein focused interest on elastin degradation and the development of pulmonary emphysema. Amino acids desmosine and isodesmosine are unique cross-links in mature elastin fibers and can serve as biomarkers of elastin degradation when measured in body fluids. This review gives a perspective on what has been learned by the earliest measurements of desmosine and isodesmosine followed by later studies using methods of increased sensitivity and specificity and the meaning for developing new therapies. Also included are brief statements on the biomarkers fibrinogen, CC-16, and Aa-Val-360 in COPD. PMID:27564670

  2. Differential regulation of autophagy and mitophagy in pulmonary diseases.

    PubMed

    Aggarwal, Saurabh; Mannam, Praveen; Zhang, Jianhua

    2016-08-01

    Lysosomal-mediated degradation of intracellular lipids, proteins and organelles, known as autophagy, represents a inducible adaptive response to lung injury resulting from exposure to insults, such as hypoxia, microbes, inflammation, ischemia-reperfusion, pharmaceuticals (e.g., bleomycin), or inhaled xenobiotics (i.e., air pollution, cigarette smoke). This process clears damaged or toxic cellular constituents and facilitates cell survival in stressful environments. Autophagic degradation of dysfunctional or damaged mitochondria is termed mitophagy. Enhanced mitophagy is usually an early response to promote survival. However, overwhelming or prolonged mitochondrial damage can induce excessive/pathological levels of mitophagy, thereby promoting cell death and tissue injury. Autophagy/mitophagy is therefore an important modulator in human pulmonary diseases and a potential therapeutic target. This review article will summarize the most recent studies highlighting the role of autophagy/mitophagy and its molecular pathways involved in stress response in pulmonary pathologies. PMID:27402690

  3. [Improving system of prevention and rehabilitation for asbestos-related broncho-pulmonary diseases in workers].

    PubMed

    2011-01-01

    To improve a system of prevention and rehabilitation for broncho-pulmonary diseases among workers engaged into extraction and utilization of chrysotile asbestos, the authors specified major criteria for diagnosis of asbestos-related pulmonary diseases and signs of exposure to asbestos-containing dust, with definition of risk groups for broncho-pulmonary diseases. The authors formulated main concepts of prevention and rehabilitation for asbestos-related pulmonary diseases in workers engaged into asbestos industry. Special attention was paid to harmonization of all medical and technical measures aimed to prevention and liquidation of asbestos-related diseases. PMID:21789804

  4. Chest wall shrapnel-induced beryllium-sensitization and associated pulmonary disease.

    PubMed

    Fireman, E; Shai, A Bar; Lerman, Y; Topilsky, M; Blanc, P D; Maier, L; Li, L; Chandra, S; Abraham, J M; Fomin, I; Aviram, G; Abraham, J L

    2012-10-01

    Chronic beryllium disease (CBD) is an exposure-related granulomatous disease mimicking sarcoidosis. Beryllium exposure-associated disease occurs mainly via inhalation, but skin may also be a source of sensitization. A 65-year-old male with a history of war-related shrapnel wounds was initially diagnosed with pulmonary sarcoidosis. Twenty years later, the possibility of a metal-related etiology for the lung disease was raised. A beryllium lymphocyte proliferation test, elemental analysis of removed shrapnel, and genetic studies were consistent with a diagnosis of CBD. This case demonstrates that retained beryllium-containing foreign bodies can be linked to a pathophysiologic response in the lung consistent with CBD. PMID:23461078

  5. Sildenafil for Chronic Obstructive Pulmonary Disease: A Randomized Crossover Trial

    PubMed Central

    Lederer, David J.; Bartels, Matthew N.; Schluger, Neil W.; Brogan, Frances; Jellen, Patricia; Thomashow, Byron M.; Kawut, Steven M.

    2016-01-01

    Rationale Pulmonary hypertension with exercise is common in chronic obstructive pulmonary disease (COPD) and may contribute to exercise limitation in this disease. We aimed to determine the effects of treatment with sildenafil on exercise capacity in patients with COPD and emphysema. Methods We performed a randomized, double-blind, placebo-controlled 2-period crossover trial of sildenafil thrice daily in ten adults with COPD and emphysema on CT scan without pulmonary hypertension. We randomized study participants to 4 weeks of sildenafil (or placebo) followed by a 1-week washout and then 4 weeks of placebo (or sildenafil). The 2 primary outcomes were the 6-minute walk distance and oxygen consumption at peak exercise. Results Sildenafil had no effect on 6-minute walk distance (placebo-corrected difference = −7.8 m, 95% confidence interval, −23.2 to 7.5 m, p = 0.35) or oxygen consumption at peak exercise (placebo-corrected difference = −0.1 ml/kg/min, 95% confidence interval −2.1 to 1.8 ml/kg/min, p = 0.89). Sildenafil increased the alveolar-arterial oxygen gradient (p = 0.02), worsened symptoms (p = 0.04), and decreased quality-of-life (p = 0.03). Adverse events were more frequent while receiving sildenafil (p = 0.005). Conclusions Routine sildenafil administration did not have a beneficial effect on exercise capacity in patients with COPD and emphysema without pulmonary hypertension. Sildenafil significantly worsened gas exchange at rest and quality of life. (clinicaltrials.gov NCT00104637). PMID:22360383

  6. Can pulmonary vasodilator therapy expand the operative indications for congenital heart disease?

    PubMed

    Inai, Kei

    2015-01-01

    The operability of congenital heart disease with left to right shunt depends on the severity of the pulmonary vascular disease induced by the increased pulmonary blood flow. Although some recommendations exist regarding operative indications according to pathological, hemodynamic, and epidemiological factors, the evidence underlying these recommendations is not conclusive. Recently, oral pulmonary vasodilator therapy has been reported to improve outcomes in patients with idiopathic pulmonary arterial hypertension, and this therapy also appears to be effective in patients with congenital heart disease and pulmonary arterial hypertension, including those with postoperative pulmonary hypertension and Eisenmenger syndrome. It is expected that the availability of novel pulmonary vasodilator therapy will expand the operative indications in patients with congenital heart disease with left to right shunt, but there is currently insufficient evidence to definitively determine this. A multicenter double-blind study should be conducted to further examine this issue. PMID:25787792

  7. Speckle Tracking Echocardiography to Evaluate for Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease.

    PubMed

    Rice, Jessica L; Stream, Amanda R; Fox, Daniel L; Geraci, Mark W; Vandivier, R William; Dorosz, Jennifer L; Bull, Todd M

    2016-10-01

    Pulmonary hypertension (PH) is a common complication of chronic obstructive pulmonary disease (COPD), increasing morbidity and mortality. Current echocardiographic measures have poor predictive value for the diagnosis of PH in COPD. Right ventricular (RV) strain obtained by speckle tracking echocardiography (STE) is a measure of myocardial deformation which correlates with RV function and survival in subjects with pulmonary arterial hypertension. We hypothesized that RV strain measurements would be feasible and correlate with invasive hemodynamic measurements in patients with COPD. Retrospective analysis of RV strain values from subjects with severe COPD with echocardiogram within 48 hours of right heart catheterization was performed. First, 54 subjects were included in the analysis. Right ventricular systolic pressure (RVSP) and RV strain could be estimated in 31% and 57%, respectively. Then, 61% had RV-focused apical views, and of those, RV strain could be obtained for 94%. RV free wall strain correlated with PVR (r = 0.41, p = 0.02). Subjects with pulmonary vascular resistance (PVR) > 3 Wood units (WU) had less negative (worse) RV free wall strain values than those with PVR ≤ 3 WU, with a median strain of -20 (-23, -12) versus -23 (-29, -15), p < 0.05. A receiver operating characteristic curve demonstrated an RV free wall strain of > -23 to be 92% sensitive and 44% specific for identifying PVR > 3 WU (AUC 0.71). RV strain estimates are feasible in the majority of subjects with severe COPD. RV strain correlates with PVR and may improve screening for PH in subjects with COPD. PMID:26829151

  8. Estrogens and development of pulmonary hypertension - Interaction of estradiol metabolism and pulmonary vascular disease

    PubMed Central

    Tofovic, Stevan P.

    2010-01-01

    Severe pulmonary arterial hypertension (PAH) is characterized by clustered proliferation of endothelial cells in the lumina of small size pulmonary arteries resulting in concentric obliteration of the lumina and formation of complex vascular structures known as plexiform lesions. This debilitating disease occurs more frequently in women, yet both animal studies in classical models of PAH and limited clinical data suggest protective effects of estrogens: the estrogen paradox in pulmonary hypertension. Little is known about the role of estrogens in PAH, but one line of evidence strongly suggests that the vascular protective effects of 17β-estradiol (estradiol; E2) are mediated largely by its downstream metabolites. Estradiol is metabolized to 2-hydroxyestradiol (2HE) by CYP1A1/CYP1B1, and 2HE is converted to 2-methoxyestradiol (2ME) by catechol-O-methyl transferase. 2ME is extensively metabolized to 2-methoxyestrone, a metabolite that lacks biologic activity but which may be converted back to 2ME. 2ME has no estrogenic activity and its effects are mediated by estrogen receptors-independent mechanism(s). Notably, in systemic and pulmonary vascular endothelial cells, smooth muscle cells, and fibroblasts 2ME exerts stronger anti-mitotic effects than E2 itself. E2 and 2ME, despite having similar effects on other cardiovascular cells, have opposing effects on endothelial cells; that is, in endothelial cells, E2 is pro-mitogenic, pro-angiogenic and anti- apoptotic, whereas 2ME is antimitogenic, anti-angiogenic and pro-apoptotic. This may have significant ramifications in severe PAH that involves uncontrolled proliferation of monoclonal, apoptosis resistant endothelial cells. Based on its cellular effects, 2ME should be expected to attenuate the progression of disease and provide protection in severe PAH. In contrast, E2, due to its mitogenic, angiogenic, and anti-apoptotic effects (otherwise desirable in normal, quiescent endothelial cells), may even adversely affect

  9. Narratives of breathlessness in chronic obstructive pulmonary disease

    PubMed Central

    Kvangarsnes, Marit; Torheim, Henny; Hole, Torstein; Öhlund, Lennart S

    2013-01-01

    Aims and objectives To explore patient perceptions of chronic obstructive pulmonary disease exacerbation and the patients’ experiences of their relations with health personnel during care and treatment. Background Patients suffering from acute exacerbation of chronic obstructive pulmonary disease often experience life-threatening situations and undergo noninvasive positive-pressure ventilation via bi-level positive airway pressure in a hospital setting. Theory on trust, which often overlaps with the issue of power, can shed light on patient’s experiences during an acute exacerbation. Design Narrative research design was chosen. Methods Ten in-depth qualitative interviews (n = 10) were conducted with patients who had been admitted to two intensive care units in Western Norway during the autumn of 2009 and the spring of 2010. Narrative analysis and theories on trust and power were used to analyse the interviews. Results Because of their breathlessness, the patients perceived that they were completely dependent on others during the acute phase. Some stated that they had experienced an altered perception of reality and had not understood how serious their situation was. Although the patients trusted the health personnel in helping them breathe, they also told stories about care deficiencies and situations in which they felt neglected. Conclusions This study shows that patients with an acute exacerbation of chronic obstructive pulmonary disease often feel wholly dependent on health personnel during the exacerbation and, as a result, experience extreme vulnerability. Relevance to clinical practice The findings give nurses insight into building trust and a good relationship between patient and caregiver during an acute exacerbation of chronic obstructive lung disease. PMID:23889291

  10. Chronic obstructive pulmonary disease management: the evidence base.

    PubMed

    MacIntyre, N R

    2001-11-01

    In long-term management of stable chronic obstructive pulmonary disease (COPD), a number of medications improve pulmonary function test results. The long-term clinical benefits of those drugs would seem intuitive, but there is very little strong evidence that long-term outcomes in COPD are substantially affected by those drugs. Nevertheless, symptom improvement such as dyspnea reduction is certainly strong reason to use those agents. The 2 most compelling bodies of evidence in stable COPD are for oxygen therapy in the chronically hypoxemic patient and pulmonary rehabilitation to improve exercise tolerance and dyspnea. Inhaled corticosteroids also appear to be useful in patients at risk for frequent exacerbations. In acute exacerbations, the rationale for therapy comes in part from the large body of literature regarding acute asthma therapy. Bronchodilator therapy and corticosteroids both seem to reduce the severity and the duration of exacerbations. Moreover, routine antibiotic use seems beneficial, and the role of noninvasive positive-pressure ventilation with patients suffering impending respiratory failure from acute COPD exacerbations is well supported by the literature. PMID:11679148

  11. Unusual Systemic Artery to Pulmonary Artery Malformation Without Evidence of Systemic Disease, Trauma or Surgery

    SciTech Connect

    Geyik, Serdar; Yavuz, Kivilcim; Keller, Frederick S.

    2006-10-15

    Connections between the systemic and pulmonary arterial systems are rare conditions that can be due to either congenital or acquired diseases such as anomalous systemic arterial supply to normal lung, pulmonary sequestration, and systemic supply to pulmonary arteriovenous malformations. Herein, a unique case of systemic artery to pulmonary arterial malformation and its endovascular treatment in a patient with no history of the usual etiologies is reported.

  12. Computed Tomography-based Subclassification of Chronic Obstructive Pulmonary Disease.

    PubMed

    Dirksen, Asger; Wille, Mathilde M W

    2016-04-01

    Computed tomography (CT) is an obvious modality for subclassification of COPD. Traditionally, the pulmonary involvement of chronic obstructive pulmonary disease (COPD) in smokers is understood as a combination of deleterious effects of smoking on small airways (chronic bronchitis and small airways disease) and distal to the airways with destruction and loss of lung parenchyma (emphysema). However, segmentation of airways is still experimental; with contemporary high-resolution CT (HRCT) we can just see the "entrance" of small airways, and until now changes in airway morphology that have been observed in COPD are subtle. Furthermore, recent results indicate that emphysema may also be the essential pathophysiologic mechanism behind the airflow limitation of COPD. The definition of COPD excludes bronchiectasis as a symptomatic subtype of COPD, and CT findings in chronic bronchitis and exacerbations of COPD are rather unspecific. This leaves emphysema as the most obvious candidate for subclassification of COPD. Both chest radiologists and pulmonary physicians are quite familiar with the appearance of various patterns of emphysema on HRCT, such as centrilobular, panlobular, and paraseptal emphysema. However, it has not yet been possible to develop operational definitions of these patterns that can be used by computer software to automatically classify CT scans into distinct patterns. In conclusion, even though various emphysema patterns can be recognized visually, CT has not yet demonstrated a great potential for automated subclassification of COPD, and it is an open question whether it will ever be possible to achieve success equivalent to that obtained by HRCT in the area of interstitial lung diseases. PMID:27115944

  13. Biomarkers in chronic obstructive pulmonary disease: confusing or useful?

    PubMed Central

    Stockley, Robert A

    2014-01-01

    The field of biomarker research has almost reached unmanageable proportions in chronic obstructive pulmonary disease (COPD). The developments of new technology platforms have generated a huge information data base, both cross sectionally and increasingly, longitudinally. The knowledge emerging provides an enormous potential for understanding the disease pathophysiology, for developing markers specific for long-term outcomes, and for developing new therapeutic strategies. However, the excitement must be tempered with an understanding of the limitations of the data collection techniques, and of the variations in disease state, activity, impact, and progression. Nevertheless, the most crucial aspect in interpreting the current literature is the recognition of the relatively superficial characterization of what is a complex group of pathological processes with a common end point of airflow limitation. The current review explores some of these issues together with those areas where real progress appears to have been made, and provides caution on interpretation. PMID:24532968

  14. Phenotype of asthma-chronic obstructive pulmonary disease overlap syndrome.

    PubMed

    Rhee, Chin Kook

    2015-07-01

    Many patients with asthma or chronic obstructive pulmonary disease (COPD) have overlapping characteristics of both diseases. By spirometric definition, patients with both fixed airflow obstruction (AO) and bronchodilator reversibility or fixed AO and bronchial hyperresponsiveness can be considered to have asthma-COPD overlap syndrome (ACOS). However, patients regarded to have ACOS by spirometric criteria alone are heterogeneous and can be classified by phenotype. Eosinophilic inflammation, a history of allergic disease, and smoke exposure are important components in the classification of ACOS. Each phenotype has a different underlying pathophysiology, set of characteristics, and prognosis. Medical treatment for ACOS should be tailored according to phenotype. A narrower definition of ACOS that includes both spirometric and clinical criteria is needed. PMID:26161009

  15. Phenotype of asthma-chronic obstructive pulmonary disease overlap syndrome

    PubMed Central

    2015-01-01

    Many patients with asthma or chronic obstructive pulmonary disease (COPD) have overlapping characteristics of both diseases. By spirometric definition, patients with both fixed airflow obstruction (AO) and bronchodilator reversibility or fixed AO and bronchial hyperresponsiveness can be considered to have asthma-COPD overlap syndrome (ACOS). However, patients regarded to have ACOS by spirometric criteria alone are heterogeneous and can be classified by phenotype. Eosinophilic inflammation, a history of allergic disease, and smoke exposure are important components in the classification of ACOS. Each phenotype has a different underlying pathophysiology, set of characteristics, and prognosis. Medical treatment for ACOS should be tailored according to phenotype. A narrower definition of ACOS that includes both spirometric and clinical criteria is needed. PMID:26161009

  16. Behavioral Medicine Approaches to Chronic Obstructive Pulmonary Disease

    PubMed Central

    Fritzsche, Anja; Trueba, Ana F.; Meuret, Alicia E.; Ritz, Thomas

    2013-01-01

    Background Chronic obstructive pulmonary disease (COPD) is a prevalent respiratory disease and associated with considerable individual and socioeconomic burden. Recent research started examining the role of psychosocial factors for course and management of the disease. Purpose This review provides an overview on recent findings on psychosocial factors and behavioral medicine approaches in COPD. Results Research has identified several important psychosocial factors and effective behavioral medicine interventions in COPD. However, there is considerable need for future research in this field. Conclusions Although beneficial effects of some behavioral medicine interventions have been demonstrated in COPD, future research efforts are necessary to study the effects of distinct components of these interventions, to thoroughly examine promising but yet not sufficiently proven interventions, and to develop new creative interventions. PMID:22351032

  17. Electrocardiographic abnormalities and cardiac arrhythmias in chronic obstructive pulmonary disease.

    PubMed

    Goudis, Christos A; Konstantinidis, Athanasios K; Ntalas, Ioannis V; Korantzopoulos, Panagiotis

    2015-11-15

    Chronic obstructive pulmonary disease (COPD) is independently associated with an increased burden of cardiovascular disease. Besides coronary artery disease (CAD) and congestive heart failure (CHF), specific electrocardiographic (ECG) abnormalities and cardiac arrhythmias seem to have a significant impact on cardiovascular prognosis of COPD patients. Disturbances of heart rhythm include premature atrial contractions (PACs), premature ventricular contractions (PVCs), atrial fibrillation (AF), atrial flutter (AFL), multifocal atrial tachycardia (MAT), and ventricular tachycardia (VT). Of note, the identification of ECG abnormalities and the evaluation of the arrhythmic risk may have significant implications in the management and outcome of patients with COPD. This article provides a concise overview of the available data regarding ECG abnormalities and arrhythmias in these patients, including an elaborated description of the underlying arrhythmogenic mechanisms. The clinical impact and prognostic significance of ECG abnormalities and arrhythmias in COPD as well as the appropriate antiarrhythmic therapy and interventions in this setting are also discussed. PMID:26218181

  18. CFTR gene mutations in isolated chronic obstructive pulmonary disease

    SciTech Connect

    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 normal 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.

  19. [Preparing patients with chronic pulmonary disease for air travel].

    PubMed

    Felkai, Péter; Böszörményi Nagy, György; Gyarmati, Ildikó

    2013-03-01

    Flying is the most important way of travelling in the continually growing international tourism. Number of passengers and those with preexisting diseases, mainly with cardiopulmonary problems, is increasing over years. One of the main tasks of the pre-travel advice is to assess tolerance to hypoxia of the traveler, and specify the necessity, as well as the type and volume of supplementary oxygen therapy. It is indispensable to know the cabin-environment and impact of that on the travelers' health. Travel medicine specialist has to be aware of the examinations which provide information for the appropriate decision on the fit-to-fly condition of the patient. The physician who prepares the patient with chronic obstructive pulmonary disease for repatriation by regular flight and the escorting doctor have to be fully aware of the possibilities, modalities, advantages and contraindications of the on-board oxygen supply and therapy. In this review, the authors give a summary of literature data, outline the tools of in-flight oxygen therapy as well as discuss possibilities for the preflight assessment of patients' condition including blood gas parameters required for safe air travel, as recommended in international medical literature. The preparation process for repatriation of patients with chronic obstructive pulmonary disease is also discussed. PMID:23434882

  20. The objective evaluation of obstructive pulmonary diseases with spirometry

    PubMed Central

    Ozkaya, Sevket; Dirican, Adem; Tuna, Tibel

    2016-01-01

    Airway obstruction is variable in asthma, while it is progressive and persistent in chronic bronchitis and emphysema. However, some of the patients presenting with symptoms of chronic airway diseases have clinical features of both asthma and COPD. The group with “Asthma–COPD Overlap Syndrome” (ACOS) phenotype was characterized by definitely irreversible airway obstruction accompanied by symptoms and signs of reversibility. In this study, we aimed to classify obstructive airway diseases by clinical, radiological, and pulmonary function tests. Patients at Samsun Medical Park Hospital Chest Diseases outpatient clinic were evaluated between January 2013 and April 2016, and a total of 235 patients were included in this study. Mean age of the patients was 55.3±14.5 (15–88) years, and the male/female ratio was 45/190. The baseline pulmonary function test results of the patients were as follows: mean forced vital capacity (FVC) values 2,825±1,108 (710–6,870) mL and 74.3±22.4 (24–155)%, forced expiratory volume in 1 second (FEV1) values 1,789±774 (480–4,810) mL and 58.1±20.0 (20–130)%, FEV1/FVC values 62.5±6.8 (39–70)%. Reversibility criteria following bronchodilator treatment were present in 107 (45.5%) patients. We specified five subgroups for patients according to their clinical, radiological, and pulmonary test findings, namely Group 1 (asthma), Group 2 (ACOS), Group 3 (chronic bronchitis), and Group 4 (emphysema). Additionally, a group of patients who had clinical and spirometric features of both asthma and chronic bronchitis in association with underlying emphysema (emphysema with chronic bronchitis and emphysema with asthma) was defined as the undifferentiated obstruction (UNDO) group. Number and percentage distribution of patients by groups were 58 (24.7%) in the asthma group, 70 (29.8%) in the ACOS group, 61 (26%) in the chronic bronchitis group, 32 (13.6%) in the emphysema group, and 14 (6%) in the UNDO group. In conclusion, in our study

  1. Chronic obstructive pulmonary disease exacerbations: latest evidence and clinical implications

    PubMed Central

    Qureshi, Hammad; Sharafkhaneh, Amir

    2014-01-01

    Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality worldwide and results in an economic and social burden that is both substantial and increasing. The natural history of COPD is punctuated by exacerbations which have major short- and long-term implications on the patient and healthcare system. Evidence-based guidelines stipulate that early detection and prompt treatment of exacerbations are essential to ensure optimal outcomes and to reduce the burden of COPD. Several factors can identify populations at risk of exacerbations. Implementing prevention measures in patients at risk is a major goal in the management of COPD. PMID:25177479

  2. Pulmonary alveolar proteinosis: another autoimmune disease associated with sarcoidosis?

    PubMed

    Boerner, Eda Burcu; Costabel, Ulrich; Wessendorf, Thomas Ernst; Theegarten, Dirk; Hetzel, Martin; Drent, Marjolein; Bonella, Francesco

    2016-01-01

    Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of surfactant phospholipids and lipoproteins within the alveoli.  Here we report on a female patient who was diagnosed with autoimmune PAP and successfully treated with whole lung lavage (WLL). 15 months after PAP diagnosis the patient developed marked fatigue. Additional tests revealed the diagnosis of sarcoidosis. We can only speculate that PAP and sarcoidosis in our patient are linked to each other based on the fact that other autoimmune disorders have also been associated with sarcoidosis. PMID:27055841

  3. The objective evaluation of obstructive pulmonary diseases with spirometry.

    PubMed

    Ozkaya, Sevket; Dirican, Adem; Tuna, Tibel

    2016-01-01

    Airway obstruction is variable in asthma, while it is progressive and persistent in chronic bronchitis and emphysema. However, some of the patients presenting with symptoms of chronic airway diseases have clinical features of both asthma and COPD. The group with "Asthma-COPD Overlap Syndrome" (ACOS) phenotype was characterized by definitely irreversible airway obstruction accompanied by symptoms and signs of reversibility. In this study, we aimed to classify obstructive airway diseases by clinical, radiological, and pulmonary function tests. Patients at Samsun Medical Park Hospital Chest Diseases outpatient clinic were evaluated between January 2013 and April 2016, and a total of 235 patients were included in this study. Mean age of the patients was 55.3±14.5 (15-88) years, and the male/female ratio was 45/190. The baseline pulmonary function test results of the patients were as follows: mean forced vital capacity (FVC) values 2,825±1,108 (710-6,870) mL and 74.3±22.4 (24-155)%, forced expiratory volume in 1 second (FEV1) values 1,789±774 (480-4,810) mL and 58.1±20.0 (20-130)%, FEV1/FVC values 62.5±6.8 (39-70)%. Reversibility criteria following bronchodilator treatment were present in 107 (45.5%) patients. We specified five subgroups for patients according to their clinical, radiological, and pulmonary test findings, namely Group 1 (asthma), Group 2 (ACOS), Group 3 (chronic bronchitis), and Group 4 (emphysema). Additionally, a group of patients who had clinical and spirometric features of both asthma and chronic bronchitis in association with underlying emphysema (emphysema with chronic bronchitis and emphysema with asthma) was defined as the undifferentiated obstruction (UNDO) group. Number and percentage distribution of patients by groups were 58 (24.7%) in the asthma group, 70 (29.8%) in the ACOS group, 61 (26%) in the chronic bronchitis group, 32 (13.6%) in the emphysema group, and 14 (6%) in the UNDO group. In conclusion, in our study, the types of

  4. Does pulmonary rehabilitation reduce peripheral blood pressure in patients with chronic obstructive pulmonary disease?

    PubMed

    Canavan, Jane L; Kaliaraju, Djeya; Nolan, Claire M; Clark, Amy L; Jones, Sarah E; Kon, Samantha S C; Polkey, Michael I; Man, William D-C

    2015-08-01

    Pulmonary rehabilitation (PR) can improve aerobic exercise capacity, health-related quality of life and dyspnoea in patients with chronic obstructive pulmonary disease (COPD). Recent studies have suggested that exercise training may improve blood pressure and arterial stiffness, albeit in small highly selected cohorts. The aim of the study was to establish whether supervised outpatient or unsupervised home PR can reduce peripheral blood pressure. Resting blood pressure was measured in 418 patients with COPD before and after outpatient PR, supervised by a hospital-based team (HOSP). Seventy-four patients with COPD undergoing an unsupervised home-based programme acted as a comparator group (HOME). Despite significant improvements in mean (95% confidence interval) exercise capacity in the HOSP group (56 (50-60) m, p < 0.001) and HOME group (30 (17-42) m, p < 0.001) systolic blood pressure (SBP), diastolic blood pressure (DBP) and mean arterial blood pressure (MAP) did not change in either the HOSP (SBP: p = 0.47; DBP: p = 0.06; MAP: p = 0.38) or HOME group (SBP: p = 0.67; DBP: p = 0.38; MAP: p = 0.76). Planned subgroup analysis of HOSP patients with known hypertension and/or cardiovascular disease showed no impact of PR upon blood pressure. PR is unlikely to reduce blood pressure, and by implication, makes a mechanism of action in which arterial stiffness is reduced, less likely. PMID:26015460

  5. Pulmonary Rehabilitation: The Reference Therapy for Undernourished Patients with Chronic Obstructive Pulmonary Disease

    PubMed Central

    Samaras, Dimitrios; Chambellan, Arnaud; Pichard, Claude

    2014-01-01

    Chronic obstructive pulmonary disease (COPD) combines the deleterious effects of chronic hypoxia, chronic inflammation, insulin-resistance, increased energy expenditure, muscle wasting, and exercise deconditioning. As for other chronic disorders, loss of fat-free mass decreased survival. The preservation of muscle mass and function, through the protection of the mitochondrial oxidative metabolism, is an important challenge in the management of COPD patients. As the prevalence of the disease is increasing and the medical advances make COPD patients live longer, the prevalence of COPD-associated nutritional disorders is expected to increase in future decades. Androgenopenia is observed in 40% of COPD patients. Due to the stimulating effects of androgens on muscle anabolism, androgenopenia favors loss of muscle mass. Studies have shown that androgen substitution could improve muscle mass in COPD patients, but alone, was insufficient to improve lung function. Two multicentric randomized clinical trials have shown that the association of androgen therapy with physical exercise and oral nutritional supplements containing omega-3 polyinsaturated fatty acids, during at least three months, is associated with an improved clinical outcome and survival. These approaches are optimized in the field of pulmonary rehabilitation which is the reference therapy of COPD-associated undernutrition. PMID:24701566

  6. Pulmonary performance of elderly normal subjects and subjects with chronic obstructive pulmonary disease exposed to 0. 3 ppm nitrogen dioxide

    SciTech Connect

    Morrow, P.E.; Utell, M.J.; Bauer, M.A.; Smeglin, A.M.; Frampton, M.W.; Cox, C.; Speers, D.M.; Gibb, F.R. )

    1992-02-01

    Symptoms and changes in pulmonary function of subjects with chronic obstructive pulmonary disease (COPD) and elderly normal subjects, induced by a 4-h exposure to 0.3 ppm NO2, were investigated using a double-blind, crossover design with purified air. The 5-day experimental protocol required approximately 2 wk with at least a 5-day separation between randomized 4-h exposures to either NO2 or air which included several periods of exercise. Over a 2-yr period, COPD subjects, all with a history of smoking, consisting of 13 men and 7 women (mean age of 60.0 yr) and 20 elderly normal subjects of comparable age and sex were evaluated. During intermittent light exercise, COPD subjects demonstrated progressive decrements in FVC and FEV1 compared with baseline with 0.3 ppm NO2, but not with air. Differences in percent changes from baseline data (air-NO2) showed an equivocal reduction in FVC by repeated measures of analysis of variance and cross-over t tests (p less than 0.10). Subgroup analyses suggested that responsiveness to NO2 decreased with severity of COPD; in elderly normal subjects, NO2-induced reduction in FEV1 was greater among smokers than never-smokers. A comparison of COPD and elderly normal subjects also revealed distinctions in NO2-induced responsiveness.

  7. Complications after video-assisted thoracic surgery in patients with pulmonary nontuberculous mycobacterial lung disease who underwent preoperative pulmonary rehabilitation

    PubMed Central

    Morino, Akira; Murase, Kazuma; Yamada, Katsuo

    2015-01-01

    [Purpose] Video-assisted thoracic surgery and preoperative pulmonary rehabilitation are effective in preventing postoperative complications in patients with cardiopulmonary disease. The present study aims to elucidate the presence of postoperative pneumonia and atelectasis in patients with nontuberculous mycobacterial lung disease who underwent lung resection with video-assisted thoracic surgery and preoperative pulmonary rehabilitation. [Subjects and Methods] Nineteen patients with nontuberculous mycobacterial lung disease who had undergone lung resection with video-assisted thoracic surgery and preoperative pulmonary rehabilitation were enrolled in this study. The presence of postoperative pneumonia and atelectasis was evaluated, and preoperative and postoperative pulmonary functions were compared. [Results] Postoperative pneumonia and postoperative atelectasis were not observed. Decreases of pulmonary function were 5.9% (standard deviation, 8.5) in forced vital capacity (percent predicted) and 9.6% (standard deviation, 11.1) in forced expiratory volume in 1 s (percent predicted). [Conclusion] The present study indicates that the combination of lung resection with video-assisted thoracic surgery and preoperative pulmonary rehabilitation in patients with nontuberculous mycobacterial lung disease may be effective in preventing postoperative complications. PMID:26357436

  8. Costs and benefits of Pulmonary Rehabilitation in Chronic Obstructive Pulmonary Disease: a randomized controlled trial

    PubMed Central

    Farias, Catharinne C.; Resqueti, Vanessa; Dias, Fernando A. L.; Borghi-Silva, Audrey; Arena, Ross; Fregonezi, Guilherme A. F.

    2014-01-01

    Objective The current study evaluated the costs and benefits of a simple aerobic walking program for patients with chronic obstructive pulmonary disease (COPD). Method This was a blinded randomized controlled clinical trial that recruited 72 patients diagnosed with COPD, 40 of whom were included in the study and divided into two groups [control group (CG) and pulmonary rehabilitation group (GPR)]. We assessed pulmonary function, distance covered during the 6-minute walk test (6MWT), respiratory and peripheral muscle strength, health-related quality of life (HRQOL), body composition, and level of activities of daily living (ADLs) before and after an 8-week walking program. The financial costs were calculated according to the pricing table of the Brazilian Unified Health System (SUS). Results Only 34 of the 40 patients remained in the final sample; 16 in the CG and 18 in the GPR (FEV1: 50.9±14% predicted and FEV1: 56±0.5% predicted, respectively). The intervention group exhibited improvements in the 6MWT, sensation of dyspnea and fatigue, work performed, BODE index (p<0.01), HRQOL, ADL level (p<0.001), and lower limb strength (p<0.05). The final mean cost per patient for the GPR was R$ 148.75 (~US$ 75.00) and no patient significantly exceeded this value. However, 2 patients in the CG did exceed this value, incurring a cost of R$ 689.15 (~US$ 345.00). Conclusion Aerobic walking demonstrated significant clinical benefits in a cost-efficient manner in patients with COPD. PMID:24838809

  9. Hyaluronan fragments as mediators of inflammation in allergic pulmonary disease.

    PubMed

    Ghosh, Sumit; Hoselton, Scott A; Dorsam, Glenn P; Schuh, Jane M

    2015-05-01

    Asthma is frequently caused and/or exacerbated by sensitization to allergens, which are ubiquitous in many indoor and outdoor environments. Severe asthma is characterized by airway hyperresponsiveness and bronchial constriction in response to an inhaled allergen, leading to a disease course that is often very difficult to treat with standard asthma therapies. As a result of interactions among inflammatory cells, structural cells, and the intercellular matrix of the allergic lung, patients with sensitization to allergens may experience a greater degree of tissue injury followed by airway wall remodeling and progressive, accumulated pulmonary dysfunction as part of the disease sequela. In addition, turnover of extracellular matrix (ECM) components is a hallmark of tissue injury and repair. This review focuses on the role of the glycosaminoglycan hyaluronan (HA), a component of the ECM, in pulmonary injury and repair with an emphasis on allergic asthma. Both the synthesis and degradation of the ECM are critical contributors to tissue repair and remodeling. Fragmented HA accumulates during tissue injury and functions in ways distinct from the larger native polymer. There is gathering evidence that HA degradation products are active participants in stimulating the expression of inflammatory genes in a variety of immune cells at the injury site. In this review, we will consider recent advances in the understanding of the mechanisms that are associated with HA accumulation and inflammatory cell recruitment in the asthmatic lung. PMID:25582403

  10. Hyaluronan fragments as mediators of inflammation in allergic pulmonary disease

    PubMed Central

    Ghosh, Sumit; Hoselton, Scott A.; Dorsam, Glenn P.; Schuh, Jane M.

    2015-01-01

    Asthma is frequently caused and/or exacerbated by sensitization to allergens, which are ubiquitous in many indoor and outdoor environments. Severe asthma is characterized by airway hyperresponsiveness and bronchial constriction in response to an inhaled allergen, leading to a disease course that is often very difficult to treat with standard asthma therapies. As a result of interactions among inflammatory cells, structural cells, and the intercellular matrix of the allergic lung, patients with sensitization to allergens may experience a greater degree of tissue injury followed by airway wall remodeling and progressive, accumulated pulmonary dysfunction as part of the disease sequela. In addition, turnover of extracellular matrix (ECM) components is a hallmark of tissue injury and repair. This review focuses on the role of the glycosaminoglycan hyaluronan (HA), a component of the ECM, in pulmonary injury and repair with an emphasis on allergic asthma. Both the synthesis and degradation of the ECM are critical contributors to tissue repair and remodeling. Fragmented HA accumulates during tissue injury and functions in ways distinct from the larger native polymer. There is gathering evidence that HA degradation products are active participants in stimulating the expression of inflammatory genes in a variety of immune cells at the injury site. In this review, we will consider recent advances in the understanding of the mechanisms that are associated with HA accumulation and inflammatory cell recruitment in the asthmatic lung. PMID:25582403