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  1. Periodontal disease in diabetic patients - clinical and histopathological aspects.

    PubMed

    Corlan Puşcu, Dorina; Ciuluvică, Radu Constantin; Anghel, Andreea; Mălăescu, Gheorghe Dan; Ciursaş, Adina Nicoleta; Popa, Gabriel Valeriu; Agop Forna, Doriana; Busuioc, Cristina Jana; Siloşi, Izabela

    2016-01-01

    Periodontal disease is one of the most frequent diseases affecting people all over the world. The relation between periodontal disease and diabetes mellitus raised the interest both of dentists and doctors treating metabolic diseases, as the two conditions influence one another. In our study, we analyzed a number of 75 patients with diabetes mellitus and periodontal disease that presented to the medical consultory for conditions of the dental maxillary system. The clinical study showed that periodontal disease and diabetes may affect young adults as well, still this pathological association more frequently appears after the age of 50. The disease was identified especially in the women living in urban area. The clinical examination of the dental maxillary system identified the presence of gingival ulcerations, dental calculus, gingival bleeding, radicular leftovers with anfractuous margins, fixed prostheses with an inappropriate cervical adjustment. Of the systemic diseases associated to periodontal disease and diabetes mellitus, there was observed that 66.66% of the patients also suffered from cardiovascular diseases (high blood pressure, ischemic cardiopathy, heart failure), and 37.33% suffered from obesity. The histopathological and immunohistochemical tests highlighted the presence of an inflammatory chronic, intense reaction, mainly formed of lymphocytes, plasmocytes, macrophages and granulocytes, heterogeneously disseminated and alteration of the structure of marginal and superficial periodontium. The inflammatory reaction in the patients with periodontal disease and diabetes was more intense than in the patients with periodontal disease without diabetes.

  2. Attitudes toward clinical trials among patients with sickle cell disease.

    PubMed

    Haywood, Carlton; Lanzkron, Sophie; Diener-West, Marie; Haythornthwaite, Jennifer; Strouse, John J; Bediako, Shawn; Onojobi, Gladys; Beach, Mary Catherine

    2014-06-01

    Background A substantial number of planned clinical trials for sickle cell disease (SCD) have terminated early due to insufficient patient enrollment. Purpose To describe attitudes toward clinical trials among a sample of adults with SCD and identify patient-level factors associated with these attitudes. Methods Our data came from a sample (N = 291) of primarily adults with SCD participating in the Improving Patient Outcomes with Respect and Trust (IMPORT) study, which is a federally funded observational study of SCD patient experiences in seeking healthcare. Attitudes toward clinical trials were assessed using items from the Perceptions of Participation in Clinical Research instrument. Patient factors examined as potential correlates of clinical trial attitudes were demographics, disease severity, engagement in self-care, trust, healthcare experience ratings, and prior history of participation in clinical trials. Multiple regression analyses were used to identify patient-level correlates of clinical trial attitudes. Results Our sample of SCD patients expressed overwhelmingly favorable attitudes about clinical trials, with 77%-92% of our sample expressing agreement with a series of positive statements about clinical trials in general. Demographics, engagement in self-care, healthcare experience ratings, and prior trial participation each explained significant portions of the variability in clinical trial attitudes. Limitations The generalizability of our results to the entire SCD population may be of concern as the study participants were all receiving care at comprehensive sickle cell centers and already participating in clinical research. Conclusion Our results suggest that, in principle, adults with SCD enrolled in an observational study express very positive general attitudes about clinical trial participation and that specific factors attached to particular clinical trial opportunities may play a greater role in a SCD patient's decision to participate than a

  3. Discussing sexuality with patients in a motor neurone disease clinic.

    PubMed

    Marsden, Rachael; Botell, Rachel

    Sexual relationships remain an important aspect of life for people living with motor neurone disease. This article explores the use of the Extended-PLISSIT model when discussing relationships and sexual function with patients and their partners in a motor neurone disease clinic. The model provides a structured approach to assist discussions with patients as well as promoting reflection and exchange of knowledge in the multidisciplinary team. It is a useful model when addressing issues that are sometimes difficult to discuss.

  4. Clinical Analysis of Algerian Patients with Pompe Disease

    PubMed Central

    Medjroubi, M.; Froissart, R.; Taghane, N.; Sifi, K.; Benhabiles, A.; Lemai, S.; Semra, S.; Benmekhebi, H.; Bouderda, Z.; Abadi, N.; Hamri, A.

    2017-01-01

    Pompe's disease is a metabolic myopathy caused by a deficiency of acid alpha-glucosidase (GAA), also called acid maltase, an enzyme that degrades lysosomal glycogen. The clinical presentation of Pompe's disease is variable with respect to the age of onset and rate of disease progression. Patients with onset of symptoms in early infancy (infantile-onset Pompe disease (IOPD)) typically exhibit rapidly progressive hypertrophic cardiomyopathy and marked muscle weakness. Most of them die within the first year of life from cardiac and/or respiratory failure. In the majority of cases of Pompe's disease, onset of symptoms occurs after infancy, ranging widely from the first to sixth decade of life (late-onset Pompe's disease or LOPD). Progression of the disease is relentless and patients eventually progress to loss of ambulation and death due to respiratory failure. The objective of this study was to characterize the clinical presentation of 6 patients (3 with EOPD and the other 3 with LOPD) of 5 families from the East of Algeria. All our patients were diagnosed as having Pompe's disease based on biochemical confirmations of GAA deficiency by dried blood spots (DBS) and GAA gene mutations were analyzed in all patients who consented (n = 4). Our results are similar to other ethnic groups. PMID:28265479

  5. Clinical aspects of Alzheimer's disease in black and white patients.

    PubMed Central

    Hargrave, R.; Stoeklin, M.; Haan, M.; Reed, B.

    1998-01-01

    This article examines the association between ethnicity and psychiatric symptoms in patients with Alzheimer's disease. Data from a cross-sectional study of patients evaluated at nine California Department of Health Alzheimer's Disease Diagnostic and Treatment Centers (ADDTCs) were used. Using the ADDTC patient database, sociodemographic and clinical variables in 207 black patients and 1818 white patients with probable and possible Alzheimer's disease were compared. Logistic and linear regression analysis indicated the following results: 1) black patients had fewer years of education and more often had hypertension, 2) black patients reported shorter duration of illness at the time of initial diagnosis of dementia, 3) black patients had lower Mini-Mental State Examination scores and higher Blessed Roth Dementia Rating Scale scores at the time of initial diagnosis, and 4) black patients more frequently reported insomnia and less frequently reported anxiety. Additional studies are needed to validate these findings and to generate hypotheses about the role of cardiovascular disease and pathophysiology of psychiatric symptoms in ethnic populations with Alzheimer's disease. PMID:9510621

  6. Clinical and genetic data of Huntington disease in Moroccan patients.

    PubMed

    Bouhouche, Ahmed; Regragui, Wafaa; Lamghari, Hind; Khaldi, Khadija; Birouk, Nazha; Lytim, Safaa; Bellamine, Soufiane; Kriouile, Yamna; Bouslam, Naima; Haddou, El Hachmia Ait Ben; Faris, Mustapha Alaoui; Benomar, Ali; Yahyaoui, Mohamed

    2015-12-01

    Huntington's disease (HD) occurs worldwide with prevalence varying from 0.1 to 10/100,000 depending of the ethnic origin. Since no data is available in the Maghreb population, the aim of this study is to describe clinical and genetic characteristics of Huntington patients of Moroccan origin. Clinical and genetics data of 21 consecutive patients recruited from 2009 to 2014 from the outpatient clinic of six medical centers were analyzed. Statistical analysis was performed using descriptive statistics. Twenty one patients from 17 families were diagnosed positive for the IT15 gene CAG expansion. Clinical symptoms were predominantly motor (19/21). Twelve patients had psychiatric and behavioral disorders, and 11 patients had cognitive disorders essentially of memory impairment. Analysis of genetic results showed that 5 patients had reduced penetrant (RP) alleles and 16 had fully penetrant (FP) alleles. The mean CAG repeat length in patients with RP alleles was 38.4 ± 0.54, and 45.37 ± 8.30 in FP alleles. The age of onset and the size of the CAG repeat length showed significant inverse correlation (p <0.001, r = -0.754). Clinical and genetic data of Moroccan patients are similar to those of Caucasian populations previously reported in the literature.

  7. How to predict clinical relapse in inflammatory bowel disease patients

    PubMed Central

    Liverani, Elisa; Scaioli, Eleonora; Digby, Richard John; Bellanova, Matteo; Belluzzi, Andrea

    2016-01-01

    Inflammatory bowel diseases have a natural course characterized by alternating periods of remission and relapse. Disease flares occur in a random way and are currently unpredictable for the most part. Predictors of benign or unfavourable clinical course are required to facilitate treatment decisions and to avoid overtreatment. The present article provides a literature review of the current evidence on the main clinical, genetic, endoscopic, histologic, serologic and fecal markers to predict aggressiveness of inflammatory bowel disease and discuss their prognostic role, both in Crohn’s disease and ulcerative colitis. No single marker seems to be reliable alone as a flare predictor, even in light of promising evidence regarding the role of fecal markers, in particular fecal calprotectin, which has reported good results recently. In order to improve our daily clinical practice, validated prognostic scores should be elaborated, integrating clinical and biological markers of prognosis. Finally, we propose an algorithm considering clinical history and biological markers to intercept patients with high risk of clinical relapse. PMID:26811644

  8. [Clinical approach to hydatid disease in uremic patients].

    PubMed

    Vaccaro, F; Pagano, A; Romano, G; Romano, G

    1996-03-01

    Uremic patients in hemodialytic treatment have the same opportunity to be affected with hydatid disease as healthy people. However, because these patients usually have an immunodeficiency syndrome, it is always necessary to evaluate correctly the most common immune diagnostic procedures; furthermore the clinical approach and successive surgical treatment must evaluate carefully electrolyte and acid-base balances, the cardiovascular system, hemostasis and energy intake. Authors, in this paper, report 8 cases of hydatid disease in as many patients during a period of 27 years. At the beginning they describe diagnostic and therapeutic management; later they emphasize the necessity of a careful immunological diagnostic evaluation (which is often falsely negative), and of radiological, ultrasonographic and scintigraphic diagnosis. They conclude by emphasizing that uremic patients with hemodialytic treatment survive with disease and although a surgical approach of hydatid disease is not really different from others it could be insidious not to consider the peculiar immunological and clinical metabolic state of uremic patients also to perform a correct treatment.

  9. Abdominal wall abscesses in patients with Crohn's disease: clinical outcome.

    PubMed

    Neufeld, David; Keidar, Andrei; Gutman, Mordechai; Zissin, Rivka

    2006-03-01

    Abdominal wall abscess due to Crohn's Disease used to be one of the definitive indications for operative treatment. The advent of interventional radiology, the accessibility to percutaneous drainage, and the availability of new medications raised the possibility of nonoperative treatment of this condition. The clinical presentation, treatment, and follow-up of 13 patients with abdominal wall abscesses secondary to Crohn's Disease were retrospectively reviewed. During a 10-year period (1993-2003), 13 patients with abdominal wall abscess were treated. Five patients had an anterolateral abdominal wall abscess and eight had a posterior abscess (psoas). In 11 patients, 17 drainage procedures were performed: 12 percutaneous and 5 operative. Despite initial adequate drainage and resolution of the abscess, all 13 patients eventually needed resection of the offending bowel segment, which was undertaken in 12 patients. The mean time between abscess presentation and definitive operation was 2 months. Percutaneous drainage is an attractive option in most cases of abdominal abscesses. However, in Crohn's Disease patients with an abdominal wall abscess, we found a high failure rate despite initial adequate drainage. We suggest that surgical resection of the diseased bowel segment should be the definitive therapy.

  10. Clinical characterization of dystonia in adult patients with Huntington's disease.

    PubMed

    van de Zande, N A; Massey, T H; McLauchlan, D; Pryce Roberts, A; Zutt, R; Wardle, M; Payne, G C; Clenaghan, C; Tijssen, M A J; Rosser, A E; Peall, K J

    2017-09-01

    Huntington's disease (HD) is an autosomal dominant, neurodegenerative movement disorder, typically characterized by chorea. Dystonia is also recognized as part of the HD motor phenotype, although little work detailing its prevalence, distribution, severity and impact on functional capacity has been published to date. Patients (>18 years of age) were recruited from the Cardiff (UK) HD clinic, each undergoing a standardized videotaped clinical examination and series of functional assessment questionnaires (Unified Huntington's Disease Rating Scale, Burke-Fahn-Marsden Dystonia Rating Scale and modified version of the Toronto Western Spasmodic Torticollis Rating Scale). The presence and severity of dystonia were scored by four independent neurologists using the Burke-Fahn-Marsden Dystonia Rating Scale and Unified Huntington's Disease Rating Scale. Statistical analysis included Fisher's exact test, Wilcoxon test, anova and calculation of correlation coefficients where appropriate. Forty-eight patients [91% (48/53)] demonstrated evidence of dystonia, with the highest prevalence in the left upper limb (n = 44, 83%), right upper limb most severely affected and eyes least affected. Statistically significant positive correlations (P < 0.05) were observed between dystonia severity and increasing HD disease stage and motor disease duration. Deterioration in functional capacity also correlated with increasing dystonia severity. No significant relationship was observed with age at motor symptom onset or CAG repeat length. We report a high prevalence of dystonia in adult patients with HD, with worsening dystonia severity with increasing HD disease stage and motor disease duration. The recognition and management of dystonic symptoms in routine clinical practice will aid overall symptomatic treatment and functional improvement. © 2017 EAN.

  11. Uncontrolled chronic disease: patient non-compliance or clinical mismanagement?

    PubMed

    Javors, Jonathan R; Bramble, Judith E

    2003-01-01

    A study group of 30 individuals was randomly chosen from 1,379 beneficiaries predicted to be at risk for health care complications at a large, Midwest, industrial company currently experiencing increased health care costs. All 30 individuals had one or more chronic illness, primarily diabetes, cardiovascular disease, or asthma. Through analysis of medical records, a self-reporting health risk assessment survey, and personal contact with both patients and clinicians, each study individual was assessed as to whether his disease(s) was under control, the individual was compliant with his treatment protocol, and whether the supervising clinician was following nationally accepted standards of care. Fewer than 50% of the individuals in the study group had their chronic illness(es) under control. Those individuals whose treatment adhered to national guidelines were significantly more likely to have their disease under control (p < 0.001). For this study, patient compliance was high and unrelated to whether their disease was under control. Behavioral (external) barriers were most often cited as the reason a clinical practitioner did not follow the appropriate national standard of care. Most clinicians were aware of and familiar with the guidelines; a few either did not agree with or misunderstood the guidelines. The results of this study suggest that changing clinical practice behaviors to better ensure compliance to national standards of care may make a substantial difference in chronic disease control.

  12. Clinical characteristics of sleep disorders in patients with Parkinson's disease.

    PubMed

    Mao, Zhi-Juan; Liu, Chan-Chan; Ji, Su-Qiong; Yang, Qing-Mei; Ye, Hong-Xiang; Han, Hai-Yan; Xue, Zheng

    2017-02-01

    In order to investigate the sleep quality and influencing factors in patients with Parkinson's disease (PD), 201 PD patients were enrolled and underwent extensive clinical evaluations. Subjective sleep evaluation was assessed using the Pittsburgh Sleep Quality Index (PSQI), and the Epworth Sleepiness Scale (ESS). It was found that poor sleep quality (77.11%) and excessive daytime sleepiness (32.34%) were commonly seen in PD patients and positively correlated with disease severity. Then 70 out of the 201 PD patients and 70 age- and sex-matched controls underwent a polysomnographic recording. The parameters were compared between PD group and control group and the influencing factors of sleep in PD patients were analyzed. The results showed that sleep efficiency (SE) was significantly decreased (P<0.01), and sleep latency (SL) and the arousal index (AI) were increased (P<0.05) in the PD group as compared with those in the control group. SE and total sleep time (TST) were positively correlated with the Hoehn and Yahr (H&Y) stage. There was significant difference in the extent of hypopnea and hypoxemia between the PD group and the control group (P<0.05). Our results indicate that PD patients have an overall poor sleep quality and a high prevalence of sleep disorder, which may be correlated with the disease severity. Respiratory function and oxygen supply are also affected to a certain degree in PD patients.

  13. Clinical COPD Questionnaire in patients with chronic respiratory disease.

    PubMed

    Canavan, Jane L; Dilaver, Deniz; Clark, Amy L; Jones, Sarah E; Nolan, Claire M; Kon, Samantha S C; Man, William D-C

    2014-10-01

    The Clinical Chronic Obstructive Pulmonary Disease (COPD) Questionnaire (CCQ) is an easy to complete, health-related quality of life questionnaire which has been well-validated in COPD. The responsiveness of the CCQ in chronic respiratory disease patients other than COPD has not been previously described. The study aims were to determine if the CCQ in chronic respiratory disease correlates with other health related quality of life questionnaires, to assess the responsiveness of the CCQ to pulmonary rehabilitation and to determine the minimum important difference. The CCQ, COPD Assessment Test (CAT), the Chronic Respiratory Questionnaire (CRQ) and St George's Respiratory Questionnaire (SGRQ) were measured in 138 chronic respiratory disease patients completing pulmonary rehabilitation. Change in CCQ with pulmonary rehabilitation was correlated with change in the other questionnaires. The minimum important difference of the CCQ was calculated using distribution and anchor-based approaches. The CCQ, CAT, CRQ and SGRQ improved significantly with rehabilitation with effect sizes of -0.43, -0.26, 0.62, -0.37. Change in CCQ correlated significantly with CAT, CRQ and SGRQ (r = 0.53, -0.64, 0.30, all P < 0.0001). The minimum important difference was -0.42 at the population level and -0.4 at the individual level. The CCQ is responsive to pulmonary rehabilitation in chronic respiratory disease patients, with an MID estimated at -0.4 at the individual level. © 2014 Asian Pacific Society of Respirology.

  14. Clinical assessment of patients with peripheral arterial disease.

    PubMed

    Bailey, Marc A; Griffin, Kathryn J; Scott, D Julian A

    2014-12-01

    Peripheral arterial disease (PAD) describes the clinical manifestations of atherosclerosis affecting the circulation in the legs. The severity of PAD is classified according to symptom severity, time course, and anatomical distribution. The signs and symptoms of PAD reflect the degree of circulatory compromise and whether there has been a gradual reduction in the circulation or an abrupt, uncompensated decrease. Accurate clinical assessment underpins decisions on management strategy and should objectively assess the severity of the ischemia and need for revascularization. Clinical history should discriminate symptoms of PAD from other conditions presenting with leg pain, elucidate cardiovascular risk factors and the effect of symptoms on the patient's quality of life. Clinical examination includes signs of general cardiovascular disease and associated conditions before assessing the circulation and viability of the limb. Palpation of peripheral pulses must be augmented by determination of the ankle brachial pressure index using hand held Doppler. A whole patient approach to management is required and must include modification of cardiovascular risk status as well as dealing with the local circulatory manifestation of PAD.

  15. Clinical Assessment of Patients with Peripheral Arterial Disease

    PubMed Central

    Bailey, Marc A.; Griffin, Kathryn J.; Scott, D. Julian A.

    2014-01-01

    Peripheral arterial disease (PAD) describes the clinical manifestations of atherosclerosis affecting the circulation in the legs. The severity of PAD is classified according to symptom severity, time course, and anatomical distribution. The signs and symptoms of PAD reflect the degree of circulatory compromise and whether there has been a gradual reduction in the circulation or an abrupt, uncompensated decrease. Accurate clinical assessment underpins decisions on management strategy and should objectively assess the severity of the ischemia and need for revascularization. Clinical history should discriminate symptoms of PAD from other conditions presenting with leg pain, elucidate cardiovascular risk factors and the effect of symptoms on the patient's quality of life. Clinical examination includes signs of general cardiovascular disease and associated conditions before assessing the circulation and viability of the limb. Palpation of peripheral pulses must be augmented by determination of the ankle brachial pressure index using hand held Doppler. A whole patient approach to management is required and must include modification of cardiovascular risk status as well as dealing with the local circulatory manifestation of PAD. PMID:25435653

  16. [Clinical laboratory diagnosis of trichomoniasis in patients with internal diseases].

    PubMed

    Siuch, N I; Riumin, D V; Lashenkova, N N

    2010-01-01

    Urogenital trichomoniasis is an infectious inflammatory disease of the urogenital system caused by protozoan Trichomonas vaginalis and characterized by rapid dissemination and development of complications. Because laboratory diagnosis by microscopic methods encounters difficulty, we undertook detection of T. vaginalis by microscopic study of native and stained (methylene blue, acridine orange, Gram, Romanovsky-Giemsa) uretheral scrapings. 69 patients having no clinical signs of the disease were examined after sexual contacts with women suffering infection of the urogenital tract. Staining with acridine orange and by Romanovsky-Giemsa method proved the most informative methods for diagnosis of torpid trichomoniasis (85.5 and 75.4% respectively). The study of native samples is of little informative value (5.8%). The data obtained were used to develop an algorithm of microscopic investigation for the examination of patients with urogenital acute, subacute or chronic trichomoniasis and carriers of T. vaginalis.

  17. Positron emission tomography in patients with clinically diagnosed Alzheimer's disease.

    PubMed Central

    McGeer, P L; Kamo, H; Harrop, R; Li, D K; Tuokko, H; McGeer, E G; Adam, M J; Ammann, W; Beattie, B L; Calne, D B

    1986-01-01

    Fourteen patients who had clinically diagnosed Alzheimer's disease with mild to severe dementia (mean age 69.1 years) were evaluated by calculation of local cerebral metabolic rate for glucose (LCMR-gl) based on uptake of 18F-2-fluoro-2-deoxyglucose (FDG) detected with positron emission tomography (PET). PET scanning showed that the patients had significantly lower LCMR-gl values than 11 age-matched neurologically normal volunteers (mean age 66.3 years). The differences were most marked in the temporal cortex, followed by the frontal, parietal and occipital cortex. In each case the LCMR-gl value was below the lowest control value in at least one cortical area and usually in several; the reduction in LCMR-gl and the number of regions involved in the patients increased with the severity of the dementia. Deficits noted in neuropsychologic testing generally correlated with those predicted from loss of regional cortical metabolism. The patients with Alzheimer's disease were also examined with magnetic resonance imaging, computed tomography or both; the degree of atrophy found showed only a poor correlation with the neuropsychologic deficit. Significant atrophy was also noted in some of the controls. A detailed analysis of LCMR-gl values in selected cerebral regions of various sizes refuted the hypothesis that the reduction in cortical glucose metabolism in Alzheimer's disease is due to the filling by metabolically inert cerebrospinal fluid of space created by tissue atrophy. Images Fig. 2 Fig. 3 Fig. 4 Fig. 7 Fig. 8 Fig. 9 PMID:3512063

  18. Functional capacity of Brazilian patients with Parkinson's disease (PD): relationship between clinical characteristics and disease severity.

    PubMed

    Barbieri, Fabio A; Rinaldi, Natalia M; Santos, Paulo Cezar R; Lirani-Silva, Ellen; Vitório, Rodrigo; Teixeira-Arroyo, Cláudia; Stella, Florindo; Gobbi, Lilian Teresa B

    2012-01-01

    The present study had three objectives: (a) to characterize the functional capacity of patients with PD, (b) to assess the relationship between the physical fitness components of functional capacity with clinical characteristics and disease severity, and (c) to compare the physical fitness components of functional capacity with clinical characteristics according to disease severity. The study included 54 patients with idiopathic PD who were distributed into two groups according to PD severity: unilateral group (n=35); and bilateral group (n=19). All patients underwent psychiatric assessment by means of the Hoehn and Yahr (HY) staging of PD, the Unified Parkinson's Disease Rating Scale (UPDRS), the Hospital Anxiety and Depression Scale (HADS-A and HADS-D, respectively), and The Mini-Mental State Examination (MMSE). The physical fitness components of functional capacity were evaluated over a 2-day period, using recommendations by the American Alliance for Health, Physical Education, Recreation and Dance, and the Berg Balance Scale (BBS). Pearson correlation coefficients and multiple regressions were calculated to test the correlation between functional capacity and clinical characteristics, and to predict clinical scores from physical performance, respectively. Clinical variables and physical component data were compared between groups using analysis of variance to determine the effects of disease severity. Patients with advanced disease showed low levels of functional capacity. Interestingly, patients with good functional capacity in one of the physical fitness components also showed good capacities in the other components. Disease severity is a major factor affecting functional capacity and clinical characteristics. Medical providers should take disease severity into consideration when prescribing physical activity for PD patients, since the relationship between functional capacity and clinical characteristics is dependent on disease severity.

  19. Clinical Problems in the Hospitalized Parkinson's Disease Patient: Systematic Review

    PubMed Central

    Gerlach, Oliver HH; Winogrodzka, Ania; Weber, Wim EJ

    2011-01-01

    The problems Parkinson's disease (PD) patients encounter when admitted to a hospital, are known to be numerous and serious. These problems have been inventoried through a systematic review of literature on reasons for emergency and hospital admissions in PD patients, problems encountered during hospitalization, and possible solutions for the encountered problems using the Pubmed database. PD patients are hospitalized in frequencies ranging from 7 to 28% per year. PD/parkinsonism patients are approximately one and a half times more frequently and generally 2 to 14 days longer hospitalized than non-PD patients. Acute events occurring during hospitalization were mainly urinary infection, confusion, and pressure ulcers. Medication errors were also frequent adverse events. During and after surgery PD patients had an increased incidence of infections, confusion, falls, and decubitus, and 31% of patients was dissatisfied in the way their PD was managed. There are only two studies on medication continuation during surgery and one analyzing the effect of an early postoperative neurologic consultation, and numerous case reports, and opinionated views and reviews including other substitutes for dopaminergic medication intraoperatively. In conclusion, most studies were retrospective on small numbers of patients. The major clinical problems are injuries, infections, poor control of PD, and complications of PD treatment. There are many (un-researched) proposals for improvement. A substantial number of PD patients' admissions might be prevented. There should be guidelines concerning the hospitalized PD patients, with accent on early neurological consultation and team work between different specialities, and incorporating nonoral dopaminergic replacement therapy when necessary. © 2011 Movement Disorder Society PMID:21284037

  20. [Morquio A disease: clinical and molecular study of Tunisian patients].

    PubMed

    Khedhiri, Souhir; Chkioua, Latifa; Ferchichi, Salima; Miled, Abdelhedi; Laradi, Sandrine

    2011-01-01

    Type IVA mucopolysaccharidosis or Morquio A disease is a lysosomal storage disease, autosomal recessive, caused by deficiency of the N-acetylgalactosamine-6-sulfate sulfatase or GALNS. The severe phenotype is characterized by a severe skeletal dysplasia without any mental retardation. The aim of this study was to propose a strategy of molecular and prenatal diagnosis of this pathology. A molecular study was carried out on 7 patients MPS IVA issued from 5 unrelated families recruited from different Tunisian regions. All the patients were offspring of consanguineous marriages. The clinical and biologic study confirmed the diagnosis of MPS IVA within the 7 studied patients. Three GALNS mutations were identified by molecular analysis: IVS1+1G>A, G66R and A85T. The unions between Tunisian relatives are important and increase the Morquio A incidence in Tunisia. The identification of GALNS mutations in the Tunisian population permits better understanding of the Morquio A phenotype, a reliable genetic counselling and a molecular prenatal diagnosis to Tunisian at-risk relatives.

  1. A clinical profile of patients with Parkinson's disease and psychosis

    PubMed Central

    Amar, B. R.; Yadav, Ravi; Janardhan Reddy, Y. C.; Pal, Pramod Kumar

    2014-01-01

    Aims: The aim of the study was to study the clinical profile of the patients with Parkinson's disease (PD) and psychosis. Settings and Design: This was a prospective, cross sectional, hospital-based study done at the Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India from September 2009 to January 2011. All patients with PD, diagnosed by United Kingdom PD Society Brain Bank criteria, having with features of psychosis as diagnosed by the neuropsychiatric inventory (NPI) were included. Patients without a caregiver who could validate the patient's symptoms were excluded. Results: A total of 40 patients (5 women, 35 men) with PD with psychosis (mean age: 54.2 ± 11.5 years, mean duration of illness: 6.5 ± 4.5 years, and mean duration of psychosis: 4.3 ± 4.3 years) were included in the study. The Global NPI score was 19.1 ± 11.5. Majority of the patients had pure hallucinations (85%), while the rest had either pure delusions (7.5%) or a combination of delusions and hallucinations (7.5%). In those with hallucinations, visual hallucinations were the commonest (60%) (pure only in 22.5%), followed by auditory (45%), minor hallucinations (45%), and tactile (20%). Only one person reported having olfactory hallucinations (2.5%). Loss of insight was most often observed during the visual hallucinations (52%), followed by tactile (44.4%), auditory (38.9 %), and minor hallucinations (33.3%). Conclusions: In patients with PD and psychosis, pure hallucinations are common and visual hallucinations are the commonest among the hallucinations. A large proportion of patients have minor hallucinations, which need to be recognized early for effective and early management. The limitations of the study were small sample size, use of a single scale to assess psychosis and subjective assessment of insight. PMID:25024570

  2. Default patterns of patients attending clinics for sexually transmitted diseases.

    PubMed Central

    Mahony, J D; Bevan, J; Wall, B

    1978-01-01

    The influence of gender, propaganda, and treatment methods was studied in relation to default behaviour of patients with sexually transmitted diseases. The overall default rate of men and women was similar, but a larger proportion of men defaulted after the initial visit, while the biggest fall-out in women was after the second attendance at the clinic. The institution of a propaganda campaign was followed by a reduction in defaulting. The statistical significance of this is open to question, however: moreover the observed improvement in default rate was not maintained once the propaganda had been relaxed. Men treated for non-gonococcal urethritis by a regimen which included one injection a week for three weeks showed a highly significantly lower default rate compared with those who received tablets alone. PMID:580413

  3. Evolocumab and Clinical Outcomes in Patients with Cardiovascular Disease.

    PubMed

    Sabatine, Marc S; Giugliano, Robert P; Keech, Anthony C; Honarpour, Narimon; Wiviott, Stephen D; Murphy, Sabina A; Kuder, Julia F; Wang, Huei; Liu, Thomas; Wasserman, Scott M; Sever, Peter S; Pedersen, Terje R

    2017-05-04

    evolocumab (2.1% vs. 1.6%). In our trial, inhibition of PCSK9 with evolocumab on a background of statin therapy lowered LDL cholesterol levels to a median of 30 mg per deciliter (0.78 mmol per liter) and reduced the risk of cardiovascular events. These findings show that patients with atherosclerotic cardiovascular disease benefit from lowering of LDL cholesterol levels below current targets. (Funded by Amgen; FOURIER ClinicalTrials.gov number, NCT01764633 .).

  4. [Clinical associations between retinal vascular diseases and cardiovascular diseases in patients with systemic atheromatosis].

    PubMed

    Stefănescu-Dima, Alin; Bătăiosu, Constantin; Sas, Teodor; Puianu, Mihaela

    2013-01-01

    A clinical study was conducted on a sample of 48 patients examined within 3 months. Of these, 27 patients were recruited by ophtalmologic criteria and 21 recruited by cardiologic criteria, 25% of these patients coming for routine check. They were investigated by ophthalmic examination, cardiological examination, imaging and laboratory examination. Testing has shown a strong link between cardiovascular disease and the eye of the patients investigated. The study demonstrated the need for interdisciplinary consultation for patients with vascular complaints in the carotid territory and a close correlation between the vascular and ophthalmologic pathology at this level.

  5. Hemorheological Alteration in Patients Clinically Diagnosed with Chronic Liver Diseases.

    PubMed

    Jang, Bohyun; Han, Ji Won; Sung, Pil Soo; Jang, Jeong Won; Bae, Si Hyun; Choi, Jong Young; Cho, Young I; Yoon, Seung Kew

    2016-12-01

    Since liver function is changed by chronic liver diseases, chronic liver disease can lead to different hemorheological alterations during the course of the progression. This study aims to compare alterations in whole blood viscosity in patients with chronic liver disease, focusing on the gender effect. Chronic liver diseases were classified into three categories by patient's history, serologic markers, and radiologic findings: nonalcoholic fatty liver disease (NAFLD) (n = 63), chronic viral hepatitis B and C (n = 50), and liver cirrhosis (LC) (n = 35). Whole blood viscosity was measured by automated scanning capillary tube viscometer, while liver stiffness was measured by transient elastography using FibroScan®. Both systolic and diastolic whole blood viscosities were significantly lower in patients with LC than NAFLD and chronic viral hepatitis (P < 0.001) in male patients, but not in female patients. In correlation analysis, there were inverse relationships between both systolic and diastolic whole blood viscosity and liver stiffness (systolic: r = -0.25, diastolic: r = -0.22). Whole blood viscosity was significantly lower in male patients with LC than NAFLD or chronic viral hepatitis. Our data suggest that whole blood viscosity test can become a useful tool for classifying chronic liver disease and determining the prognosis for different types of chronic liver diseases.

  6. Hemorheological Alteration in Patients Clinically Diagnosed with Chronic Liver Diseases

    PubMed Central

    2016-01-01

    Since liver function is changed by chronic liver diseases, chronic liver disease can lead to different hemorheological alterations during the course of the progression. This study aims to compare alterations in whole blood viscosity in patients with chronic liver disease, focusing on the gender effect. Chronic liver diseases were classified into three categories by patient’s history, serologic markers, and radiologic findings: nonalcoholic fatty liver disease (NAFLD) (n = 63), chronic viral hepatitis B and C (n = 50), and liver cirrhosis (LC) (n = 35). Whole blood viscosity was measured by automated scanning capillary tube viscometer, while liver stiffness was measured by transient elastography using FibroScan®. Both systolic and diastolic whole blood viscosities were significantly lower in patients with LC than NAFLD and chronic viral hepatitis (P < 0.001) in male patients, but not in female patients. In correlation analysis, there were inverse relationships between both systolic and diastolic whole blood viscosity and liver stiffness (systolic: r = −0.25, diastolic: r = −0.22). Whole blood viscosity was significantly lower in male patients with LC than NAFLD or chronic viral hepatitis. Our data suggest that whole blood viscosity test can become a useful tool for classifying chronic liver disease and determining the prognosis for different types of chronic liver diseases. PMID:27822933

  7. [Clinical stages of patients with Alzheimer disease treated in specialist clinics in Spain. The EACE study].

    PubMed

    Alom Poveda, J; Baquero, M; González-Adalid Guerreiro, M

    2013-10-01

    The diagnostic paradigm of Alzheimer disease (AD) is changing; there is a trend toward diagnosing the disease in its early stages, even before the complete syndrome of dementia is apparent. The clinical stage at which AD is usually diagnosed in our area is unknown. Therefore, the purpose of this study is to describe the clinical stages of AD patients at time of diagnosis. Multicentre, observational and cross-sectional study. Patients with probable AD according to NINCDS-ARDRA criteria, attended in specialist clinics in Spain, were included in the study. We recorded the symptom onset to evaluation and symptom onset to diagnosis intervals and clinical status of AD (based on MMSE, NPI questionnaire, and CDR scale). Participants in this study included 437 specialists representing all of Spain's autonomous communities and a total of 1,707 patients, of whom 1,694 were included in the analysis. Mean MMSE score was 17.6±4.8 (95% CI:17.4-17.9). Moderate cognitive impairment (MMSE between 10 and 20) was detected in 64% of the patients, and severe cognitive impairment (MMSE<10) in 6%. The mean interval between symptom onset and the initial primary care visit was 10.9±17.2 months (95% CI:9.9-11.8), and the interval between symptom onset and diagnosis with AD was 28.4±21.3 months. Results from the EACE show that most AD patients in our area have reached a moderate clinical stage by the time they are evaluated in a specialist clinic. Copyright © 2012 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  8. [Lyme disease in patients with multiple sclerosis: clinical, diagnostic and therapeutic features].

    PubMed

    Baranova, N S; Spirin, N N; Fadeeva, O A; Shipova, E G; Stepanov, I O

    2012-01-01

    Lyme disease is one of the most frequent infections in the Russian Federation. In patients with multiple sclerosis, the Borrelia infection may act as a trigger in some cases and also worsens the clinical course of multiple sclerosis. One hundred patients with definite multiple sclerosis were examined including 19 patients with the combination of multiple sclerosis and Lyme disease. The difficulties of diagnosis, clinical features and treatment of Lyme disease in patients with multiple sclerosis are discussed.

  9. Clinical Presentation and Patient Evaluation in Nonalcoholic Fatty Liver Disease.

    PubMed

    Patel, Vaishali; Sanyal, Arun J; Sterling, Richard

    2016-05-01

    Nonalcoholic fatty liver disease (NAFLD) is a diagnosis of exclusion. Most patients are asymptomatic and diagnosed incidentally. Most patients remain undiagnosed. A high index of suspicion and serologic work-up to rule out alternative causes of liver disease is required. In NALFD, fibrosis correlates with outcomes, including mortality. To diagnose, assess severity, and monitor fibrosis, 2 noninvasive methods can be used. However, noninvasive tests are more helpful at extremes of fibrosis: excluding it or diagnosing advanced fibrosis. Liver biopsy is usually reserved for cases whereby noninvasive tests fail to accurately determine the degree of fibrosis or the diagnosis is unclear.

  10. Phenotypic, immunologic, and clinical characteristics of patients with nontuberculous mycobacterial lung disease in Korea

    PubMed Central

    2013-01-01

    Background This study aimed to elucidate the phenotypic, immunologic, and clinical characteristics of Korean patients with nontuberculous mycobacterial (NTM) lung disease and compare them with non-NTM bronchiectasis (BE) patients. Methods We prospectively recruited patients between 20 and 80 years of age who had nodular BE type NTM lung disease. Phenotypic, immunologic, and clinical characteristics were evaluated through physical examination, laboratory tests, pulmonary function tests, and radiographic examinations. Questionnaires were also answered. The results of the evaluations were compared with the results of non-NTM BE patients. Results A total of 84 patients with NTM lung disease and 47 non-NTM BE patients participated in the study. Mycobacterium avium complex lung disease and M. abscessus lung disease were most common. Patients with NTM lung disease had lower body mass index than non-NTM BE patients. Scoliosis was observed more frequently in patients with NTM lung disease than in non-NTM BE patients. Conclusions Significant similarities were seen between Korean patients with NTM lung disease and patients from other countries. Differences in phenotypic and clinical characteristics between NTM lung disease and non-NTM BE patients suggest differences in the immunopathogenesis of NTM lung disease and non-NTM BE. Trial registration information ClinicalTrials.gov Registration number; NCT01616745 PMID:24274658

  11. Clinical and radiological correlations in patients with gestational trophoblastic disease*

    PubMed Central

    Lima, Lana de Lourdes Aguiar; Parente, Raphael Câmara Medeiros; Maestá, Izildinha; Amim Junior, Joffre; de Rezende Filho, Jorge Fonte; Montenegro, Carlos Antonio Barbosa; Braga, Antônio

    2016-01-01

    Gestational trophoblastic disease is an abnormality of pregnancy that encompasses a group of diseases that differ from each other in their propensity for regression, invasion, metastasis, and recurrence. In the past, it was common for patients with molar pregnancy to present with marked symptoms: copious bleeding; theca lutein cysts; uterus larger than appropriate for gestational age; early preeclampsia; hyperemesis gravidarum; and hyperthyroidism. Currently, with early diagnosis made by ultrasound, most patients are diagnosed while the disease is still in the asymptomatic phase. In cases of progression to trophoblastic neoplasia, staging-typically with Doppler flow studies of the pelvis and chest X-ray, although occasionally with computed tomography or magnetic resonance imaging-is critical to the choice of an appropriate antineoplastic therapy regimen. Because it is an unusual and serious disease that affects women of reproductive age, as well as because its appropriate treatment results in high cure rates, it is crucial that radiologists be familiar with gestational trophoblastic disease, in order to facilitate its early diagnosis and to ensure appropriate follow-up imaging. PMID:27777478

  12. Clinical and radiological correlations in patients with gestational trophoblastic disease.

    PubMed

    Lima, Lana de Lourdes Aguiar; Parente, Raphael Câmara Medeiros; Maestá, Izildinha; Amim Junior, Joffre; de Rezende Filho, Jorge Fonte; Montenegro, Carlos Antonio Barbosa; Braga, Antônio

    2016-01-01

    Gestational trophoblastic disease is an abnormality of pregnancy that encompasses a group of diseases that differ from each other in their propensity for regression, invasion, metastasis, and recurrence. In the past, it was common for patients with molar pregnancy to present with marked symptoms: copious bleeding; theca lutein cysts; uterus larger than appropriate for gestational age; early preeclampsia; hyperemesis gravidarum; and hyperthyroidism. Currently, with early diagnosis made by ultrasound, most patients are diagnosed while the disease is still in the asymptomatic phase. In cases of progression to trophoblastic neoplasia, staging-typically with Doppler flow studies of the pelvis and chest X-ray, although occasionally with computed tomography or magnetic resonance imaging-is critical to the choice of an appropriate antineoplastic therapy regimen. Because it is an unusual and serious disease that affects women of reproductive age, as well as because its appropriate treatment results in high cure rates, it is crucial that radiologists be familiar with gestational trophoblastic disease, in order to facilitate its early diagnosis and to ensure appropriate follow-up imaging.

  13. Weight loss therapy for clinical management of patients with some atherosclerotic diseases: a randomized clinical trial.

    PubMed

    Oshakbayev, Kuat; Dukenbayeva, Bibazhar; Otarbayev, Nurzhan; Togizbayeva, Gulnar; Tabynbayev, Nariman; Gazaliyeva, Meruyert; Idrisov, Alisher; Oshakbayev, Pernekul

    2015-11-25

    The prevalence and burden of atherosclerotic (AS) diseases are increasing during the last twenty years. Some studies show a close relationship between overweight and AS, but influence on AS diseases of different weight loss methods are still studying. The purpose of the research was to study the effectiveness of a weight loss program in AS patients in randomized controlled trial, and to develop a conception of evolution of AS. A randomized controlled prospective clinical trial including 97 people, from them 71 patients with various AS manifestations. Patients were divided in 2 subgroups for non-drug weight loss program, and conventional drug therapy. The weight loss program included calorie restriction with 100-150 kcal/day, fat-free vegetables, salt diet, and optimum physical activity. Statistical analysis was performed using SPSS for Windows version 17.0. The weight loss subgroup lost ranging between 7-20% from an initial weight (P = 0.016). Weight loss was achieved due to fatty mass reduction only (P = 0.005). Hemoglobin levels (P < 0.001), bone mineral density (P < 0.001), percentages of water (P = 0.006) and muscle masses (P = 0.0038) were increased in weight loss subgroup. Ejection fraction (P < 0.0001), systolic output (P < 0.0001) were increased in patients with coronary artery disease. The weight loss program led to a decrease in symptomatic drugs doses up to total abolition. A conception of AS was developed. The weight loss program treated the AS diseases; improved laboratory and instrumental parameters, decreased symptomatic drugs doses. AS development is a logical way of ontogenetic ageing of body fat. ClinicalTrials.gov NCT01700075. State registration is # 0109RK000079, code is O.0475 at the National Center for Scientific and Technical Information of the Republic of Kazakhstan.

  14. Patients with cardiac disease: Changes observed through last decade in out-patient clinics

    PubMed Central

    Cordero, Alberto; Bertomeu-Martínez, Vicente; Mazón, Pilar; Fácila, Lorenzo; Cosín, Juan; Bertomeu-González, Vicente; Rodriguez, Moisés; Andrés, Eva; Galve, Enrique; Lekuona, Iñaki; González-Juanatey, Jose R

    2013-01-01

    AIM: To describe current profile of patients with cardiovascular disease (CVD) and assessing changes through last decade. METHODS: Comparison of patients with established CVD from two similar cross-sectional registries performed in 1999 (n = 6194) and 2009 (n = 4639). The types of CVD were coronary heart disease (CHD), heart failure (HF) and atrial fibrillation (AF). Patients were collected from outpatient clinics. Investigators were 80% cardiologist and 20% primary care practitioners. Clinical antecedents, major diagnosis, blood test results and medical treatments were collected from all patients. RESULTS: An increase in all risk factors, except for smoking, was observed; a 54.4% relative increase in BP control was noted. CHD was the most prevalent CVD but HF and AF increased significantly, 41.5% and 33.7%, respectively. A significant reduction in serum lipid levels was observed. The use of statins increased by 141.1% as did all cardiovascular treatments. Moreover, the use of angiotensin-renin system inhibitors in patients with HF, beta-blockers in CHD patients or oral anticoagulants in AF patients increased by 83.0%, 80.3% and 156.0%, respectively (P < 0.01). CONCLUSION: The prevalence of all cardiovascular risk factors has increased in patients with CVD through last decade. HF and AF have experienced the largest increases. PMID:24009818

  15. Patients with cardiac disease: Changes observed through last decade in out-patient clinics.

    PubMed

    Cordero, Alberto; Bertomeu-Martínez, Vicente; Mazón, Pilar; Fácila, Lorenzo; Cosín, Juan; Bertomeu-González, Vicente; Rodriguez, Moisés; Andrés, Eva; Galve, Enrique; Lekuona, Iñaki; González-Juanatey, Jose R

    2013-08-26

    To describe current profile of patients with cardiovascular disease (CVD) and assessing changes through last decade. Comparison of patients with established CVD from two similar cross-sectional registries performed in 1999 (n = 6194) and 2009 (n = 4639). The types of CVD were coronary heart disease (CHD), heart failure (HF) and atrial fibrillation (AF). Patients were collected from outpatient clinics. Investigators were 80% cardiologist and 20% primary care practitioners. Clinical antecedents, major diagnosis, blood test results and medical treatments were collected from all patients. An increase in all risk factors, except for smoking, was observed; a 54.4% relative increase in BP control was noted. CHD was the most prevalent CVD but HF and AF increased significantly, 41.5% and 33.7%, respectively. A significant reduction in serum lipid levels was observed. The use of statins increased by 141.1% as did all cardiovascular treatments. Moreover, the use of angiotensin-renin system inhibitors in patients with HF, beta-blockers in CHD patients or oral anticoagulants in AF patients increased by 83.0%, 80.3% and 156.0%, respectively (P < 0.01). The prevalence of all cardiovascular risk factors has increased in patients with CVD through last decade. HF and AF have experienced the largest increases.

  16. Effect of creatine monohydrate on clinical progression in patients with Parkinson disease: a randomized clinical trial.

    PubMed

    Kieburtz, Karl; Tilley, Barbara C; Elm, Jordan J; Babcock, Debra; Hauser, Robert; Ross, G Webster; Augustine, Alicia H; Augustine, Erika U; Aminoff, Michael J; Bodis-Wollner, Ivan G; Boyd, James; Cambi, Franca; Chou, Kelvin; Christine, Chadwick W; Cines, Michelle; Dahodwala, Nabila; Derwent, Lorelei; Dewey, Richard B; Hawthorne, Katherine; Houghton, David J; Kamp, Cornelia; Leehey, Maureen; Lew, Mark F; Liang, Grace S Lin; Luo, Sheng T; Mari, Zoltan; Morgan, John C; Parashos, Sotirios; Pérez, Adriana; Petrovitch, Helen; Rajan, Suja; Reichwein, Sue; Roth, Jessie Tatsuno; Schneider, Jay S; Shannon, Kathleen M; Simon, David K; Simuni, Tanya; Singer, Carlos; Sudarsky, Lewis; Tanner, Caroline M; Umeh, Chizoba C; Williams, Karen; Wills, Anne-Marie

    2015-02-10

    yielded t1865.8 = -0.75 (2-sided P = .45). There were no detectable differences (P < .01 to partially adjust for multiple comparisons) in adverse and serious adverse events by body system. Among patients with early and treated Parkinson disease, treatment with creatine monohydrate for at least 5 years, compared with placebo did not improve clinical outcomes. These findings do not support the use of creatine monohydrate in patients with Parkinson disease. clinicaltrials.gov Identifier: NCT00449865.

  17. Van Buchem disease: clinical, biochemical, and densitometric features of patients and disease carriers.

    PubMed

    van Lierop, Antoon H; Hamdy, Neveen A T; van Egmond, Martje E; Bakker, Egbert; Dikkers, Freek G; Papapoulos, Socrates E

    2013-04-01

    Van Buchem disease (VBD) is a rare bone sclerosing dysplasia caused by the lack of a regulatory element of the SOST gene, which encodes for sclerostin, an osteocyte-derived negative regulator of bone formation. We studied the demographic, clinical, biochemical, and densitometric features of 15 patients with VBD (12 adults and 3 children) and 28 related carriers of the gene mutation. The most common clinical findings in patients were facial palsy (100%) and various degrees of hearing impairment (93%); raised intracranial pressure had been documented in 20%. The clinical course of the disease appeared to stabilize in adulthood, with the majority of patients reporting no progression of symptoms or development of complications with time. Carriers of the disease had none of the clinical features or complications of the disease. Sclerostin could be detected in the serum in all but 1 VBD patients (mean 8.0 pg/mL; 95% confidence interval [CI], 4.9-11.0 pg/mL), and were lower than those of carriers (mean 28.7 pg/mL; 95% CI, 24.5-32.9 pg/mL; p < 0.001) and healthy controls (mean 40.0 pg/mL; 95% CI, 34.5-41.0 pg/mL; p < 0.). Serum procollagen type 1 amino-terminal propeptide (P1NP) levels were also significantly higher in adult patients (mean 96.0; 95% CI, 54.6-137.4 ng/mL versus mean 47.8; 95% CI, 39.4-56.2 ng/mL, p = 0.003 in carriers and mean 37.8; 95% CI, 34.5-41.0 ng/mL, p = 0.028 in healthy controls) and declined with age. Bone mineral density (BMD) was markedly increased in all patients (mean Z-score 8.7 ± 2.1 and 9.5 ± 1.9 at the femoral neck and spine, respectively); BMD of carriers was significantly lower than that of patients but varied widely (mean Z-scores 0.9 ± 1.0 and 1.3 ± 1.5 at the femoral neck and spine, respectively). Serum sclerostin levels were inversely correlated with serum P1NP levels (r = -0.39, p = 0.018) and BMD values (femoral neck r = -0.69, p < 0.001; lumbar spine r = -0

  18. Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease.

    PubMed

    Miura, Yukiko; Saito, Takefumi; Fujita, Kazutaka; Tsunoda, Yoshiya; Tanaka, Toru; Takoi, Hiroyuki; Yatagai, Yohei; Rin, Shigen; Sekine, Akimasa; Hayashihara, Kenji; Nei, Takahito; Azuma, Arata

    2014-10-20

    Interstitial lung disease is the most common complication and cause of death among patients with scleroderma. Scleroderma-related interstitial lung disease has usually been treated with cyclophosphamide; however, its effect was evaluated to be modest and long-term administration of this drug is associated with adverse effects. Herein, we report our clinical experience of administering pirfenidone, which is an antifibrotic agent, in five patients with scleroderma-related interstitial lung disease. All patients demonstrated an increase in vital capacity.

  19. [Clinical profile of Chagas and non-Chagas' disease patients with cardiac pacemaker].

    PubMed

    Rincon, Leonor Garcia; Rocha, Manoel Otávio da Costa; Pires, Marco Túlio Baccarini; Oliveira, Bruna Guimarães; Barros, Vladimir da Costa Val; Barros, Marcio Vinicius Lins; Ribeiro, Antônio Luiz Pinho

    2006-01-01

    The aim of this study was to compare Chagas and non-Chagas' disease patients using single or dual-chamber pacemaker in relation to the ejection fraction of the left ventricle, the ventricular stimulation threshold and the occurrence of ventricular arrhythmia. From January, 2001 to November, 2002, 45 Chagas' disease patients and 35 non-Chagas' disease patients, all pacemaker users, were evaluated considering clinical history, echocardiographic study, Holter monitoring and analysis of the pacemaker telemetry data. Chagas' disease patients were significantly younger, but both groups were similar when chest X-Ray variables and right ventricular stimulation threshold were analyzed. Chagas' disease patients had a lower left ventricular ejection fraction and more frequent ventricular arrhythmia during Holter monitoring. A positive correlation between the low ejection fraction of the left ventricle and the intensity of ventricular arrhythmia was observed. In conclusion, among pacemaker user patients, Chagas' disease is related to cardiac markers of worse prognosis.

  20. Clinical and fiberoptic endoscopic assessment of swallowing in patients with chronic obstructive pulmonary disease

    PubMed Central

    Macri, Marina Rodrigues Bueno; Marques, Jair Mendes; Santos, Rosane Sampaio; Furkim, Ana Maria; Melek, Irinei; Rispoli, Daniel; de Alencar Nunes, Maria Cristina

    2013-01-01

    Summary Introduction: Chronic obstructive pulmonary disease is characterized by progressive and partially reversible obstruction of pulmonary airflow. Aim: To characterize swallowing in patients with chronic obstructive pulmonary disease and correlate the findings with the degree chronic obstructive pulmonary disease, heart and respiratory rate, oxygen saturation, and smoking. Method: We conducted a prospective cohort study of 19 patients (12 men and 7 women; age range, 50–85 years) with confirmed medical diagnosis of chronic obstructive pulmonary disease. This study was performed in 2 stages (clinical evaluation and functional assessment using nasolaryngofibroscopy) on the same day. During both stages, vital signs were checked by medical personnel. Results: Clinical evaluation of swallowing in all patients showed the clinical signs of cough. The findings of nasolaryngofibroscopy highlighted subsequent intraoral escape in 5 patients (26.5%). No patient had tracheal aspiration. There was no association of subsequent intraoral escape with degree of chronic obstructive pulmonary disease, heart and respiratory rate, oxygen saturation, or smoking. Conclusion: In patients with chronic obstructive pulmonary disease, there was a prevalence of oral dysphagia upon swallowing and nasolaryngofibroscopy highlighted the finding of subsequent intraoral escape. There was no correlation between intraoral escape and the degree of chronic obstructive pulmonary disease, heart and respiratory rate, oxygen saturation, or smoking. PMID:26106452

  1. Immediate fixed partial denture after tooth extraction in patients with systemic diseases: A clinical report.

    PubMed

    Kang, Hyun Sun; Lee, Su Young

    2016-12-01

    For patients with systemic diseases who face difficulties visiting dental clinics, wearing fixed partial denture in the anterior region on the same day of tooth extraction can reduce the total period of treatment and the number of visits, as well as post-treatment psychological effect on the patient.

  2. Immediate fixed partial denture after tooth extraction in patients with systemic diseases: A clinical report

    PubMed Central

    2016-01-01

    For patients with systemic diseases who face difficulties visiting dental clinics, wearing fixed partial denture in the anterior region on the same day of tooth extraction can reduce the total period of treatment and the number of visits, as well as post-treatment psychological effect on the patient. PMID:28018570

  3. Multiparametric magnetic resonance imaging predicts clinical outcomes in patients with chronic liver disease

    PubMed Central

    Pavlides, Michael; Banerjee, Rajarshi; Sellwood, Joanne; Kelly, Catherine J.; Robson, Matthew D.; Booth, Jonathan C.; Collier, Jane; Neubauer, Stefan; Barnes, Eleanor

    2016-01-01

    Background & Aims Multiparametric magnetic resonance (MR) imaging has been demonstrated to quantify hepatic fibrosis, iron, and steatosis. The aim of this study was to determine if MR can be used to predict negative clinical outcomes in liver disease patients. Methods Patients with chronic liver disease (n = 112) were recruited for MR imaging and data on the development of liver related clinical events were collected by medical records review. The median follow-up was 27 months. MR data were analysed blinded for the Liver Inflammation and Fibrosis score (LIF; <1, 1–1.99, 2–2.99, and ⩾3 representing normal, mild, moderate, and severe liver disease, respectively), T2∗ for liver iron content and proportion of liver fat. Baseline liver biopsy was performed in 102 patients. Results Liver disease aetiologies included non-alcoholic fatty liver disease (35%) and chronic viral hepatitis (30%). Histologically, fibrosis was mild in 54 (48%), moderate in 17 (15%), and severe in 31 (28%) patients. Overall mortality was 5%. Ten patients (11%) developed at least one liver related clinical event. The negative predictive value of LIF <2 was 100%. Two patients with LIF 2–2.99 and eight with LIF ⩾3 had a clinical event. Patients with LIF ⩾3 had a higher cumulative risk for developing clinical events, compared to those with LIF <1 (p = 0.02) and LIF 1–1.99 (p = 0.03). Cox regression analysis including all 3 variables (fat, iron, LIF) resulted in an enhanced LIF predictive value. Conclusions Non-invasive standardised multiparametric MR technology may be used to predict clinical outcomes in patients with chronic liver disease. PMID:26471505

  4. Clinical significance of antibodies against neutrophils in patients with inflammatory bowel disease and primary sclerosing cholangitis.

    PubMed Central

    Seibold, F; Weber, P; Klein, R; Berg, P A; Wiedmann, K H

    1992-01-01

    The presence of perinuclear antibodies against neutrophils (pANCA) has been detected recently in sera of patients with inflammatory bowel disease and primary sclerosing cholangitis. In order to evaluate their clinical significance, sera from 126 patients with inflammatory bowel disease (80 Crohn's disease and 46 ulcerative colitis and 22 patients with primary sclerosing cholangitis were examined for pANCA by indirect immunofluorescence on liver sections and cytocentrifuge slides of neutrophils and by immunoblot. Perinuclear antibodies against neutrophils were found in 83% of patients with ulcerative colitis in 88% of patients with primary sclerosing cholangitis and inflammatory bowel disease, in 40% of patients with primary sclerosing cholangitis but without inflammatory bowel disease, and in 25% of patients with Crohn's disease using the immunofluorescence test. Titres of pANCA ranged from 1:10 to 1:1000 in ulcerative colitis and primary sclerosing cholangitis (median 1:100), whereas in Crohn's disease only four patients had titres of more than 1:10. The occurrence of pANCA did not correlate with clinical activity of Crohn's disease and primary sclerosing cholangitis whereas in ulcerative colitis high titres of pANCA were found mainly in active disease. Using an immunoblot system with sonified neutrophils as antigen, 82% of sera from patients with primary sclerosing cholangitis reacted with up to five different determinants, whereas only 12% of sera from patients with Crohn's disease and 11% of sera with ulcerative colitis detected one of the determinants, suggesting different antigens involved in pANCA reaction. Images Figure 1 Figure 3 Figure 4 Figure 5 PMID:1612483

  5. Clinical strategies for complete denture rehabilitation in a patient with Parkinson disease and reduced neuromuscular control.

    PubMed

    Haralur, Satheesh B

    2015-01-01

    The dentist has a large role in geriatric health care for the ever increasing elder population with associated physical and neurological disorders. The Parkinson disease is progressive neurological disorder with resting tremor, bradykinesia, akinesia, and postural instability. The psychological components of disease include depression, anxiety, and cognitive deficiency. Poor oral hygiene, increased susceptibility for dental caries, and periodontal diseases predispose them to early edentulism. The number of Parkinson affected patients visiting dental clinic seeking complete denture is growing. This case report explains the steps involved in the complete denture rehabilitation of Parkinson patient. The effective prosthesis will help in alleviating functional, aesthetic, and psychological disabilities of the patient.

  6. Clinical Strategies for Complete Denture Rehabilitation in a Patient with Parkinson Disease and Reduced Neuromuscular Control

    PubMed Central

    Haralur, Satheesh B.

    2015-01-01

    The dentist has a large role in geriatric health care for the ever increasing elder population with associated physical and neurological disorders. The Parkinson disease is progressive neurological disorder with resting tremor, bradykinesia, akinesia, and postural instability. The psychological components of disease include depression, anxiety, and cognitive deficiency. Poor oral hygiene, increased susceptibility for dental caries, and periodontal diseases predispose them to early edentulism. The number of Parkinson affected patients visiting dental clinic seeking complete denture is growing. This case report explains the steps involved in the complete denture rehabilitation of Parkinson patient. The effective prosthesis will help in alleviating functional, aesthetic, and psychological disabilities of the patient. PMID:25737785

  7. Shrinking lung syndrome in systemic lupus erythematosus patients; clinical characteristics, disease activity and damage.

    PubMed

    Gheita, Tamer A; Azkalany, Ghada S; El-Fishawy, Hussein S; Nour Eldin, Abeer M

    2011-10-01

    To detect the prevalence of shrinking lung syndrome (SLS) among systemic lupus erythematosus (SLE) patients and study their clinical, laboratory and radiological characteristics and differences in disease activity and damage. The study included 200 Egyptian SLE patients and SLS was considered in those with exertional dyspnea, restrictive pulmonary function tests (PFTs) and elevated copula of the diaphragm. Full history taking, thorough clinical examination, laboratory and relevant radiological investigations were performed for all the patients. High-resolution computed tomography scans of the chest were performed for patients with radiological findings consistent with SLS and those with pulmonary manifestations. The mean age of the patients was 29.3 ± 8.4 years, mean disease duration 5.81 ± 4.32 years and female to male ratio was 9 : 1. SLS was present in 27 patients (13.5%) with a female to male ratio of 3.5 : 1.0. The demographic features, clinical and laboratory manifestations, renal biopsy class, disease activity and damage scores, PFTs and radiological findings of the SLE patients are presented. Shrinking lung syndrome is not rare and presents a considerable subset of SLE patients. In SLE patients with dyspnea or chest pain, SLS should be looked for and PFTs are highly suggestive. © 2011 The Authors. International Journal of Rheumatic Diseases © 2011 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd.

  8. Skin diseases in geriatric patients: our experience from a public skin outpatient clinic in Siena.

    PubMed

    Rubegni, P; Poggiali, S; Nami, N; Rubegni, M; Fimiani, M

    2012-12-01

    With the progressive aging of the Italian population, geriatric health care has become a major issue for health authorities. However, little data is available regarding geriatric skin diseases. In order to provide rapid access to specialist help, in 2003 we created a dermatology clinic dedicated only to geriatric patients age 65 and older. To determine the characteristic pattern and the prevalence of various skin disorders among the geriatric patients seen at the clinic, we performed a retrospective and descriptive study of all skin diseases in patients seen in our office from January 2003 to December 2009. We evaluated: age, proportion and gender for all skin disease categories. A total of 2100 geriatric patients were examined. The male to female ratio was 1.4 to 1. The most common disorder was pruritus "sine materia" (18.9%) followed by benign tumors (13.5%); 9.1% of our patients presented with actinic keratoses and 13.2% with malignant tumors. As reported by others, the quality of life in patients with skin cancer was better than patients with rashes as skin cancer patients tended to wait longer before seeking specialist care. To improve the assessment of skin diseases, we often worked closely with The prevalence of skin diseases in our patients emphasized the importance of educating the elderly about sun protection, the early detection of skin cancer, the use of emollients and proper skin care in general.

  9. The potential role of clinical pharmacy services in patients with cardiovascular diseases.

    PubMed

    Talasaz, Azita Hajhossein

    2012-01-01

    Clinical pharmacy is deemed an integral component of a health care system. The presence of clinical pharmacists in medical rounds could assist physicians in optimizing patients' pharmacotherapy. Moreover, clinical pharmacists may reduce adverse effects and medication errors insofar as they contribute significantly to the detection and management of drug-related problems, not least in patients with cardiovascular diseases, who have the highest rank in the frequency of medication errors. Clinical pharmacists can also collaborate with physicians in the management of cardiovascular risk factors as well as anticoagulation therapy based on patients' specific situations.In summary, the practice of clinical pharmacy is considered a crucial part of a health care team to improve the level of patients' care by increasing the quality of therapy with the least expense for a health care system.

  10. Clinical characteristics of patients with lymphoproliferative neoplasms in the setting of systemic autoimmune diseases.

    PubMed

    Suvajdzic, Nada; Djurdjevic, Predrag; Todorovic, Milena; Perunicic, Maja; Stojanović, Roksanda; Novkovic, Aleksandra; Mihaljevic, Biljana

    2012-09-01

    Clinical features of 40 lymphoproliferative neoplasm patients in the setting of systemic autoimmune diseases managed in the Clinic of Hematology during 1994-2006 were analyzed retrospectively. The classification of systemic autoimmune disease patients was as follows: 15 systemic lupus erythematosus--SLE, 11 rheumatoid arthritis--RA, 12 Sjögren's syndrome--SS, 1 scleroderma, and 1 dermatomyositis. Patients comprised 31 women and 9 men of mean age 55 years (range 33-76). Systemic autoimmune diseases preceeded the development of lymphoproliferative neoplasms in 37/40 (92.5%) patients. Mean latency period between the onset of systemic autoimmune diseases and lymphoproliferative neoplasms occurrence was significantly longer in RA (113 months) than in SLE (75 months) and SS patients (65 months)--P < 0.05. The most frequent lymphoproliferative neoplasms were non-Hodgkin's lymphoma--NHL (35/40; 88%), diffuse large B-cell lymphoma (DBCL)--12 (34%), follicular lymphoma (FC)--7 (20%), small lymphocytic (SL), and marginal zone lymphoma (MZL)--5 (14%) each. The primary site of NHL was extranodal in 18/35 (51.5%) cases. Advanced disease on diagnosis (III + IV clinical stages), constitutional symptoms, and bulky disease were diagnosed in 27/35 (77%), 26/35 (74%), and 3/35 (8.5%) patients, respectively. The overall survival (OS) was as follows (months): DBCL-12, FC-63, SLL-60, and MZL-48. There was no association between the lymphoproliferative neoplasm histological subtype and the systemic autoimmune diseases type or antirheumatic treatment P > 0.05. Our findings are in line with earlier reports showing a high proportion of patients with advanced disease, constitutional symptoms, extranodal manifestations, high grade histology, and low OS in the systemic autoimmune diseases setting.

  11. Use of complementary and alternative medicine among patients with chronic diseases at outpatient clinics.

    PubMed

    Hasan, Syed Shahzad; Ahmed, Syed Imran; Bukhari, Nadeem Irfan; Loon, William Cheah Wei

    2009-08-01

    The primary objective of this study was to evaluate the use of complementary and alternative medicine among patients with chronic diseases at outpatient clinics. Another aim was to identify demographic and socio-economic factors that are associated with CAM use. Face-to-face interviews of conveniently selected patients with chronic diseases were conducted in outpatient clinics of a general hospital. A validated data collection form was used to gather the information regarding pattern, perception, reasons, and perceived effect of CAM on the disease state. The other relevant information including demographics, diagnosis, indication, and treatment were collected from the patients' medical records. Out of 321 patients interviewed in this study, 205 patients were using some form of CAM, and thus the utilisation rate was 63.9%. A significant number of patients (35.5%) were using CAM for diabetes mellitus. Thirteen types of CAM were identified in the study with the most common being vitamins supplements (48.2%), herbal medicines (26.4%), ginseng (4.7%) and traditional Chinese medicine (4.0%). The patients with higher education level, higher income, and aged more than 50 years were independently associated with CAM use. Majority of the patients (77.6%) reported that their condition had improved by using CAM. The present study confirms the high frequency of CAM use among patients with chronic diseases in a Malaysian public hospital. The popularity of CAM indicated the patients' preference towards holistic approach to health care.

  12. Clinical course and prognosis in patients with Gaucher disease and parkinsonism

    PubMed Central

    Lopez, Grisel; Kim, Jenny; Wiggs, Edythe; Cintron, Dahima; Groden, Catherine; Tayebi, Nahid; Mistry, Pramod K.; Pastores, Gregory M.; Zimran, Ari; Goker-Alpan, Ozlem

    2016-01-01

    Objective: The goal of this study was to characterize the parkinsonian phenotype in patients with Gaucher disease (GD) who developed parkinsonism in order to evaluate clinical course and prognosis. Methods: This is a retrospective observational study conducted at the Clinical Center of the NIH, Bethesda, MD, over a period of 10 years. The study included 19 patients with GD and parkinsonism. The severity of Gaucher and parkinsonian symptoms was determined from clinical data including physical, neurologic, pathologic, and neurocognitive evaluations, family histories, imaging studies, olfactory testing, and validated questionnaires. Results: We found an earlier age at onset of parkinsonism and evidence of mild cognitive dysfunction in our cohort. Although the clinical course in some patients was similar to that of idiopathic Parkinson disease with a favorable levodopa response, others exhibited features characteristic of dementia with Lewy bodies. When we examined the patients as a group, we did not observe a uniformly aggressive form of parkinsonism after the initial onset of symptoms, contrary to other published reports. Conclusions: Appreciable clinical variation was seen in this cohort with GD and parkinsonism. Although some patients had early onset and prominent cognitive changes, others had a later, slower course, indicating that GBA1 mutations may not be a reliable prognostic indicator in Parkinson disease in clinical settings. PMID:27123476

  13. Motivation of patients with inflammatory bowel disease to participate in a clinical trial.

    PubMed

    Gehrmann, U; Berger, U; Teich, N; Bruns, T; Stallmach, A; Weber, M

    2016-10-01

    Background: Clinical trials are designed to investigate innovative diagnostic and therapeutic strategies for patients. However, factors that influence patients with inflammatory bowel disease (IBD) and willingness to participate in a clinical trial are unknown. Methods: We developed a questionnaire and asked IBD patients about their willingness to hypothetically participate in a clinical trial and their current health-related quality of life by using the IBDQ. Results: Of 201 distributed questionnaires, 166 were returned and included in the analysis. One-hundred-one (61 %) patients declared their willingness to participate in a clinical trial hypothetically offered in their current situation, whereas 65 (39 %) declined. Among all patients, a trustful relationship between patient and doctor was most important for trial participation. The willingness to help others and to support medical progress were other key issues mentioned. In contrast, those patients inclined to refuse trial participation feared impairment of their current health status, potential side effects, medical examinations, and the expenditure of time and effort. Conclusion: In our cohort of IBD patients, approximately two-thirds were willing to participate in a clinical trial. We were able to identify a number of factors that should help physicians to directly address fears and break down barriers in order to increase the number of patients willing to participate in clinical trials. © Georg Thieme Verlag KG Stuttgart · New York.

  14. Gender differences in disease activity and clinical features in newly diagnosed systemic lupus erythematosus patients.

    PubMed

    Muñoz-Grajales, C; González, L A; Alarcón, G S; Acosta-Reyes, J

    2016-10-01

    The objective of this paper is to compare disease activity and clinical features at diagnosis in male and female patients with systemic lupus erythematosus (SLE). This was a cross-sectional study in which every male patient (n = 40) was matched with three female patients of the same age (±5 years) and racial/ethnic group; disease activity as per the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and disease manifestations at the time of diagnosis were compared. Alopecia and anti-Ro antibodies were more frequent in female patients. No statistically significant difference in any other disease characteristics was found. However, male gender was associated with a risk of severe disease activity at the time of diagnosis (as determined by SLEDAI ≥12 score) independent of age, racial/ethnic group, anti-Ro positivity or time to criteria accrual (OR: 3.11 95% CI, 1.09-8.92; p = 0.035). In newly diagnosed SLE patients, male gender is associated with higher disease activity despite the fact that male and female patients seem to experience similar overall disease manifestations. © The Author(s) 2016.

  15. Cat scratch disease: detection of Bartonella henselae DNA in archival biopsies from patients with clinically, serologically, and histologically defined disease.

    PubMed Central

    Scott, M. A.; McCurley, T. L.; Vnencak-Jones, C. L.; Hager, C.; McCoy, J. A.; Anderson, B.; Collins, R. D.; Edwards, K. M.

    1996-01-01

    Serological and epidemiological studies suggest that Bartonella henselae is the etiological agent of cat scratch disease. We designed a study to detect B. henselae in archival biopsies by polymerase chain reaction amplification of the 16S rRNA gene followed by Southern blot hybridization. Forty-two histologically defined cat scratch disease biopsies and eighteen controls were selected for blinded analysis. After testing, charts were reviewed for clinical, immunological, and microbial evidence of infection. Results were correlated with duration of illness and antimicrobial therapy. B. henselae DNA was identified in 27 of 42 (64%) histologically defined patients and 23 of 34 (68%) patients defined both clinically and histologically. There were no false positives (0 of 18). A small subset (n = 14) had cat scratch disease serological tests performed. B. henselae was identified in 8 of 10 serologically positive patients. Polymerase chain reaction detected 50% of our DNA-positive cases (most of these early in the clinical course). Southern blotting of amplicons both doubled sensitivity (detecting patients > 4 weeks into illness) and confirmed B. henselae as the causative species. Our study strongly associates B. henselae with cat scratch disease, suggesting that it may be the most likely etiological agent in the majority of patients with cat scratch disease. PMID:8952548

  16. Demographic and clinical characteristics associated with quality of life in patients with chronic obstructive pulmonary disease.

    PubMed

    Bentsen, Signe Berit; Miaskowski, Christine; Rustøen, Tone

    2014-04-01

    Despite an increasing interest in the relationships among multiple symptoms and quality of life (QOL), little known about the association between anxiety, depression, and pain and both disease-specific and generic QOL in patients with chronic obstructive pulmonary disease (COPD). In a cross-sectional study of 100 COPD patients, disease-specific QOL was measured by St. George's Respiratory Questionnaire and generic QOL by the QOL scale. Anxiety and depression were evaluated using the Hospital Anxiety and Depression Scale, and pain was assessed with a numeric rating scale. Of the 100 patients, 31 % reported clinically meaningful anxiety, 13 % depression, and 45 % reported the presence of pain. Younger patients (p = 0.02) and those with higher anxiety scores (p = 0.02) reported worse disease-specific QOL. Patients with lower physical function (p = 0.04) and those with higher depression scores (p < 0.001) reported worse generic QOL. Age, comorbidity, physical function, anxiety, depression, and pain explained 19.2 and 49.6 % of the variance in disease-specific and generic QOL scores, respectively. Findings from this study suggest that the relationships between patient characteristics and common symptoms and QOL differ when disease-specific and generic measures of QOL are evaluated. Additional research is warranted to confirm these findings in COPD patients. Clinicians need to evaluate these common symptoms when planning and implementing symptoms management interventions to improve COPD patients' QOL.

  17. Complex Type 2 Reactions in Three Patients with Hansen's Disease from a Southern United States Clinic

    PubMed Central

    Leon, Kristoffer E.; Salinas, Jorge L.; McDonald, Robert W.; Sheth, Anandi N.; Fairley, Jessica K.

    2015-01-01

    In non-endemic countries, leprosy, or Hansen's disease (HD), remains rare and is often underrecognized. Consequently, the literature is currently lacking in clinical descriptions of leprosy complications in the United States. Immune-mediated inflammatory states known as reactions are common complications of HD. Type 1 reactions are typical of borderline cases and occur in 30% of patients and present as swelling and inflammation of existing skin lesions, neuritis, and nerve dysfunction. Type 2 reactions are systemic events that occur at the lepromatous end of the disease spectrum, and typical symptoms include fever, arthralgias, neuritis, and classic painful erythematous skin nodules known as erythema nodosum leprosum. We report three patients with lepromatous leprosy seen at a U.S. HD clinic with complicated type 2 reactions. The differences in presentations and clinical courses highlight the complexity of the disease and the need for increased awareness of unique manifestations of lepromatous leprosy in non-endemic areas. PMID:26304919

  18. Complex Type 2 Reactions in Three Patients with Hansen's Disease from a Southern United States Clinic.

    PubMed

    Leon, Kristoffer E; Salinas, Jorge L; McDonald, Robert W; Sheth, Anandi N; Fairley, Jessica K

    2015-11-01

    In non-endemic countries, leprosy, or Hansen's disease (HD), remains rare and is often underrecognized. Consequently, the literature is currently lacking in clinical descriptions of leprosy complications in the United States. Immune-mediated inflammatory states known as reactions are common complications of HD. Type 1 reactions are typical of borderline cases and occur in 30% of patients and present as swelling and inflammation of existing skin lesions, neuritis, and nerve dysfunction. Type 2 reactions are systemic events that occur at the lepromatous end of the disease spectrum, and typical symptoms include fever, arthralgias, neuritis, and classic painful erythematous skin nodules known as erythema nodosum leprosum. We report three patients with lepromatous leprosy seen at a U.S. HD clinic with complicated type 2 reactions. The differences in presentations and clinical courses highlight the complexity of the disease and the need for increased awareness of unique manifestations of lepromatous leprosy in non-endemic areas.

  19. Herpes Genitalis in Patients Attending a Clinic for Sexually Transmitted Diseases

    PubMed Central

    Lawee, David; Gutman, Mory; Hrytzay, Millie; McLachlin, Jeanette

    1983-01-01

    In a prospective study of 210 patients attending a hospital-based sexually transmitted disease clinic, we documented the prevalence of genital herpes infection (GHI) and its association with gonococcal infection (GI). Herpes simplex virus type 2 was cultured from 58% of symptomatic patients and 0.5% of asymptomatic patients. The ratio of GI to GHI was 41:31 by clinical criteria. The laboratory-confirmed ratio was 41:18. These ratios are much higher than those normally used to estimate the caseload of GHI. PMID:21283317

  20. PREVALENCE AND CLINICAL MANIFESTATIONS OF MALE PATIENTS WITH ANOGENITAL WARTS ATTENDING A SEXUALLY TRANSMITTED DISEASE CLINIC PRIOR HPV VACCINE RECOMMENDATION.

    PubMed

    Jiamton, Sukhum; Leeyaphan, Charussri; Maneeprasopchoke, Pitchaya; Omcharoen, Viboon

    2014-11-01

    Human papillomavirus (HPV) infection is the most common sexually transmitted infection worldwide among men who have sex with men (MSM). A quadrivalent HPV vaccine has been recommended for men in the United States since 2011. We conducted a retrospective study to determine the male anogenital wart burden and patient characteristics at a sexually transmitted disease (STD) clinic to provide baseline data regarding HPV infection. We reviewed the charts of male patients who attended a STD clinic between January 2007 and December 2011 and were diagnosed with having anogenital warts by clinical examination. A total of 181 patients were included in the study. The mean age of patients was 31.1 years, of which 22.7% were MSM and 14.9% had human immunodeficiency virus (HIV) infection. The prevalences of anogenital warts were 22.6% among MSM and 15.1% among HIV infected patients. The prevalence of anogenital warts increased between 2007 and 2011. Compared with patients without anogenital warts at the same STD clinic, patients with anogenital warts were significantly younger and more likely to have multiple sexual partners. Among the HIV infected patients, 63% were MSM; they had a significantly higher anogenital wart recurrence rate. Male anogenital warts posed a significant burden at the STD clinic. A preventive program is needed for anogenital warts, especially among MSM.

  1. [Clinical significance of determination of serum B7-H4 in patients with malignant hematologic diseases].

    PubMed

    Wang, Xiao-Mei; Hu, Guo-Yan; Liu, Wei; Zheng, Shu-Hua; Lv, Jing; Wang, Hong-Mei; Xu, Jun-Fa

    2010-09-01

    To study the clinical significance of determination of serum B7-H4 in patients with malignant hematologic diseases. Serum B7-H4 levels were determined in 65 patients with leucemia, 34 patients with lymphoma, 12 patients with multiple myeloma as well as in 50 healthy controls. The serum B7-H4 levels in patients with lymphoma [(38.81+/-10.34) kappag/L] were significantly higher than healthy controls [(31.62+/-9.850) kappag/L] (P<0.01). But there are no significant difference of B7-H4 levels in serum among patients with leucemia, patients with multiple myeloma and healthy controls. These results suggest that the B7-H4 may correlated with lymphoma, but uncorrelated with leucemia and multiple myeloma. Measurement of serum B7-H4 level provide useful information for distinctive diagnosis of different kinds of malignant hematologic diseases.

  2. Long-term clinical outcomes of patients with rheumatoid arthritis and concomitant coronary artery disease.

    PubMed

    Spartera, Marco; Godino, Cosmo; Baldissera, Elena; Campochiaro, Corrado; La Spina, Ketty; Aiello, Patrizia; Salerno, Anna; Cera, Michela; Magni, Valeria; Jabbour, Richard J; Dagna, Lorenzo; Tresoldi, Moreno; Cappelletti, Alberto; Alfieri, Ottavio; Colombo, Antonio; Sabbadini, Maria Grazia; Margonato, Alberto

    2017-01-01

    Rheumatoid arthritis (RA) is associated with high morbidity and mortality predominately due to increased cardiovascular risk. Few reports are available regarding the management of coronary artery disease (CAD) in RA patients and the long-term clinical outcomes after coronary revascularization. All consecutive patients with RA were identified by retrospective review at a rheumatology tertiary center in Milan, Italy between 2001 and 2013. RA patients affected by significant CAD (RA-CAD+) were prospectively followed for major adverse cardiovascular and cerebrovascular events (MACCE) after percutaneous coronary revascularization (RA-PCI), coronary artery bypass grafting (RA-CABG) or medical therapy (RA-MT). Among 936 patients with RA, the presence of clinically significant CAD was found in 5.6% (53 patients, RA-CAD+). Of these, 32 patients (60%) underwent PCI (RA-PCI), 10 patients (19%) underwent CABG (RA-CABG) and 11 patients (21%) treated with MT (RA-MT). After a mean follow-up of 9±7 years, the rate of MACCE was 56% in RA-PCI patients, 50% in RA-CABG and 27% in RA-MT patients (P=0.184). The high MACCE rate was mainly driven by repeat coronary revascularization (47%) in the RA-PCI group and high rate of strokes (30%) in RA-CABG patients. In patients with rheumatoid arthritis and concomitant coronary artery disease (RA-CAD+), we observed at long-term follow-up a high MACCE rate, predominantly in those who underwent coronary revascularization.

  3. Clinical utility of viscoelastic tests of coagulation in patients with liver disease.

    PubMed

    Mallett, Susan V; Chowdary, Pratima; Burroughs, Andrew K

    2013-08-01

    The prothrombin time (PT) and international normalised ratio (INR) are used in scoring systems (Child-Pugh, MELD, UKELD) in chronic liver disease and as a prognostic tool and for dynamic monitoring of hepatic function in acute liver disease. These tests are known to be poor predictors of bleeding risk in liver disease; however, they continue to influence clinical management decisions. Recent work on coagulation in liver disease, in particular thrombin generation studies, has led to a paradigm shift in our understanding and it is now recognised that haemostasis is relatively well preserved. Whole blood global viscoelastic tests (TEG(®) /ROTEM(®) ) produce a composite dynamic picture of the entire coagulation process and have the potential to provide more clinically relevant information in patients with liver disease. We performed a systematic review of all relevant studies that have used viscoelastic tests (VET) of coagulation in patients with liver disease. Although many studies are observational and small in size, it is clear that VET provide additional information that is in keeping with the new concepts of how coagulation is altered in these patients. This review provides the basis for large scale, prospective outcome studies to establish the clinical value of these tests. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  4. Empagliflozin and Clinical Outcomes in Patients with Type 2 Diabetes, Established Cardiovascular Disease and Chronic Kidney Disease.

    PubMed

    Wanner, Christoph; Lachin, John M; Inzucchi, Silvio E; Fitchett, David; Mattheus, Michaela; George, Jyothis T; Woerle, Hans-Juergen; Broedl, Uli C; von Eynatten, Maximilian; Zinman, Bernard

    2017-09-13

    Background -Empagliflozin, a sodium glucose cotransporter 2 inhibitor, reduced cardiovascular morbidity and mortality in patients with type 2 diabetes and established cardiovascular disease in the EMPA-REG OUTCOME(®) trial. Urinary glucose excretion with empagliflozin decreases with declining renal function, resulting in less potency for glucose lowering in patients with kidney disease. We investigated the effects of empagliflozin on clinical outcomes in patients with type 2 diabetes, established cardiovascular disease and chronic kidney disease. Methods -Patients with type 2 diabetes, established cardiovascular disease and estimated glomerular filtration rate (eGFR) ≥30 mL/min/1.73m(2) at screening were randomized to receive empagliflozin 10 mg, empagliflozin 25 mg, or placebo once daily in addition to standard of care. We analyzed cardiovascular death, hospitalization for heart failure, all-cause hospitalization and all-cause mortality in patients with prevalent kidney disease (defined as eGFR <60 mL/min/1.73m(2) and/or urine albumin-creatinine ratio [UACR] >300 mg/g) at baseline. Additional analyses were performed in subgroups by baseline eGFR (<45, 45 to <60, 60 to <90, ≥90 mL/min/1.73m2) and baseline UACR (>300, 30 to ≤300, <30 mg/g). Results -Of 7020 patients treated, 2250 patients had prevalent kidney disease at baseline, of whom 67% had a diagnosis of type 2 diabetes for >10 years, 58% were receiving insulin and 84% were taking angiotensin converting enzyme inhibitors or angiotensin receptor blockers. In patients with prevalent kidney disease at baseline, empagliflozin reduced the risk of cardiovascular death by 29% compared with placebo (hazard ratio [HR] 0.71 [95% CI 0.52, 0.98]), the risk of all-cause mortality by 24% (HR 0.76 [95% CI 0.59, 0.99]), the risk of hospitalization for heart failure by 39% (HR 0.61 [95% CI 0.42, 0.87]) and the risk of all-cause hospitalization by 19% (HR 0.81 [95% CI 0.72, 0.92]). Effects of empagliflozin on these

  5. Application of cloud database in the management of clinical data of patients with skin diseases.

    PubMed

    Mao, Xiao-fei; Liu, Rui; DU, Wei; Fan, Xue; Chen, Dian; Zuo, Ya-gang; Sun, Qiu-ning

    2015-04-01

    To evaluate the needs and applications of using cloud database in the daily practice of dermatology department. The cloud database was established for systemic scleroderma and localized scleroderma. Paper forms were used to record the original data including personal information, pictures, specimens, blood biochemical indicators, skin lesions,and scores of self-rating scales. The results were input into the cloud database. The applications of the cloud database in the dermatology department were summarized and analyzed. The personal and clinical information of 215 systemic scleroderma patients and 522 localized scleroderma patients were included and analyzed using the cloud database. The disease status,quality of life, and prognosis were obtained by statistical calculations. The cloud database can efficiently and rapidly store and manage the data of patients with skin diseases. As a simple, prompt, safe, and convenient tool, it can be used in patients information management, clinical decision-making, and scientific research.

  6. Clinical Relevance of Bronchial Anthracofibrosis in Patients with Chronic Obstructive Pulmonary Disease Exacerbation

    PubMed Central

    Kim, Hyera; Shin, Kyung-Min; Lim, Jae-Kwang; Oh, Serim; Kim, Min Jung; Lee, Yong Dae; Kim, Miyoung; Lee, Jaehee; Kim, Chang-Ho

    2014-01-01

    Background Bronchial anthracofibrosis (BAF), which is associated with exposure to biomass smoke in inefficiently ventilated indoor areas, can take the form of obstructive lung disease. Patients with BAF can mimic or present with an exacerbation of chronic obstructive pulmonary disease (COPD). The purpose of the current study was to investigate the prevalence of BAF in Korean patients with COPD exacerbation as well as to examine the clinical features of these patients in order to determine its clinical relevance. Methods A total of 206 patients with COPD exacerbation were divided into BAF and non-BAF groups, according to computed tomography findings. We compared both clinical and radiologic variables between the two groups. Results Patients with BAF (51 [25%]) were older, with a preponderance of nonsmoking women; moreover, they showed a more frequent association with exposure to wood smoke compared to those without BAF. However, no differences in the severity of illness and clinical course between the two groups were observed. Patients in the BAF group had less severe airflow obstruction, but more common and severe pulmonary hypertension signs than those in the non-BAF group. Conclusion Compared with non-BAF COPD, BAF may be associated with milder airflow limitation and more frequent signs of pulmonary hypertension with a more severe grade in patients presenting with COPD exacerbation. PMID:25309607

  7. Clinical characteristics and healthcare utilization of patients with multicentric Castleman disease.

    PubMed

    Casper, Corey; Teltsch, Dana Y; Robinson, Don; Desrosiers, Marie-Pierre; Rotella, Philip; Dispenzieri, Angela; Qi, Ming; Habermann, Thomas; Reynolds, Matthew W

    2015-01-01

    Multicentric Castleman disease (MCD) is a rare lymphoproliferative disease. Little is known about how patient clinical features and healthcare utilization varies by human immunodeficiency virus (HIV) status and disease subtype. Data of MCD patients identified between 2000 and 2009 were collected from medical records at two United States treatment centres. Clinical, demographic, and biochemical characteristics, drug therapies and medical utilization were descriptively reported by HIV status and cell histology, and statistically compared with the Fisher's Exact and Kruskal-Wallis tests. Patients (n = 59) had a pathologically and clinically confirmed MCD diagnosis: plasmacytic (42%), hyaline vascular (29%) and mixed (15%); 10% had HIV infection. In the first year after diagnosis, MCD patients on average saw a healthcare provider more than six times, were hospitalized at least once and underwent frequent radiological and laboratory testing. Rituximab was the most commonly used drug therapy, followed by corticosteroids and conventional chemotherapy. One- and 2-year survival was excellent in HIV-negative patients (100% and 97%, respectively) but inferior for HIV-positive cases (67% and 67%, respectively). Heterogeneous treatment decisions were observed in this MCD study; HIV status was the only distinguishing clinical criteria associated with pharmacotherapies. Additional research is necessary to guide treatment of this rare lymphoproliferative disorder. © 2014 John Wiley & Sons Ltd.

  8. Trace metal concentrations in hair and nails from Alzheimer's disease patients: Relations with clinical severity.

    PubMed

    Koseoglu, Emel; Koseoglu, Rahmi; Kendirci, Murat; Saraymen, Recep; Saraymen, Burak

    2017-01-01

    Metals, especially transition metals, seem to be important in the pathogenesis of Alzheimer disease. This study aims to determine the relationship of trace metal elements to the pathogenesis and/or course of Alzheimer Disease in terms of clinical severity. The hair and nail trace metal levels of 62 Alzheimer Disease patients at different clinical stages (21 mild, 20 moderate, 21 severe) and 60 healthy control subjects were measured by using inductively coupled plasma-mass spectrometry. The statistical comparisons were performed with regards to the study groups, clinical stages, disease duration and age. The patient and control groups were significantly different from each other in regards to Mn, Fe, Cu, Cd, Hg (p<0.001), Zn (p<0.01) in nail concentrations and, Na, Al, Pb, Co (p<0.001), Fe, Mn (p=0.001), Hg, Cu, Cd, K in hair concentrations (p<0.01). No difference was detected in the levels of Mg and Ca. Nail Na level showed differences among different clinical stages of the disease (p<0.01). In comparing the mild degree Alzheimer patients to the control group; significant differences were detected in nail Mn, Fe, Cu, Co (p<0.001), Hg, Zn (p<0.01) and, hair Pb, Al (p<0.001), Na, K levels (p<0.01). Our results have shown that transition and posttransition metals are especially important metals for the disease process. The relation of nail Na level with clinical stages of AD is an interesting new finding, making someone to think that alkali metals may be important in the progression of the disease. Copyright © 2016. Published by Elsevier GmbH.

  9. POSTSURGICAL RECURRENT CUSHING DISEASE: CLINICAL BENEFIT OF EARLY INTERVENTION IN PATIENTS WITH NORMAL URINARY FREE CORTISOL.

    PubMed

    Carroll, Ty B; Javorsky, Bradley R; Findling, James W

    2016-10-01

    To assess the performance of biochemical markers in the detection of recurrent Cushing disease (CD), as well as the potential benefit of early intervention in recurrent CD patients with elevated late-night salivary cortisol (LNSC) and normal urinary free cortisol (UFC). The design was a single-center, retrospective chart review. Patients treated by the authors from 2008-2013 were included. Recurrence was defined by postsurgical remission of CD with subsequent abnormal LNSC, UFC, or dexamethasone suppression test (DST). We identified 15 patients with postsurgical recurrent CD after initial remission; all but one underwent testing with LNSC, DST, and UFC. Although 12 of 15 patients had normal UFC at time of recurrence, DST was abnormal in 11 of 15, and all 14 patients with LNSC results had ≥1 elevated measurement. Nine patients (7 with normal UFC) showed radiologic evidence of a pituitary tumor at time of recurrence. Among the 14 patients with available follow-up data, 12 have demonstrated significant improvement since receiving treatment. Five patients underwent repeat pituitary surgery and 4 achieved clinical and biochemical remission. Eight patients received mifepristone or cabergoline, and 6 showed clinical and/or biochemical improvement. Three patients (2 with prior mifepristone) underwent bilateral adrenalectomy and 2 demonstrated significant clinical improvements. LNSC is more sensitive than UFC or DST for detection of CD recurrence. Prompt intervention when LNSC is elevated, despite normal UFC, may yield significant clinical benefit for many patients with CD. Early treatment for patients with recurrent CD should be prospectively evaluated, utilizing LNSC elevation as an early biochemical marker. ACTH = adrenocorticotropic hormone CD = Cushing disease CS = Cushing syndrome CV = coefficient of variation DST = dexamethasone suppression test IPSS = inferior petrosal sinus sampling LNSC = late-night salivary cortisol QoL = quality of life TSS = transsphenoidal

  10. Clinical care of two patients with Ebola virus disease in the United States.

    PubMed

    Lyon, G Marshall; Mehta, Aneesh K; Varkey, Jay B; Brantly, Kent; Plyler, Lance; McElroy, Anita K; Kraft, Colleen S; Towner, Jonathan S; Spiropoulou, Christina; Ströher, Ute; Uyeki, Timothy M; Ribner, Bruce S

    2014-12-18

    West Africa is currently experiencing the largest outbreak of Ebola virus disease (EVD) in history. Two patients with EVD were transferred from Liberia to our hospital in the United States for ongoing care. Malaria had also been diagnosed in one patient, who was treated for it early in the course of EVD. The two patients had substantial intravascular volume depletion and marked electrolyte abnormalities. We undertook aggressive supportive measures of hydration (typically, 3 to 5 liters of intravenous fluids per day early in the course of care) and electrolyte correction. As the patients' condition improved clinically, there was a concomitant decline in the amount of virus detected in plasma.

  11. Clinical correlates of Charcot-Marie-Tooth disease in patients with pes cavus deformities.

    PubMed

    Karakis, Ioannis; Gregas, Matt; Darras, Basil T; Kang, Peter B; Jones, H Royden

    2013-04-01

    Given its association with Charcot-Marie-Tooth disease (CMT), pes cavus is a common reason for referral to a neurologist. We investigated clinical features that may predict CMT in children with pes cavus. In this study we retrospectively reviewed pes cavus patients referred to Boston Children's Hospital in the past 20 years. Patients were categorized as idiopathic or CMT, based on EMG/genetic testing, and their clinical features were compared. Of the 70 patients studied, 33 had idiopathic pes cavus, and 37 had genetically confirmed CMT. Symptoms of weakness, unsteady gait, family history of pes cavus and CMT, and signs of sensory deficits, distal atrophy and weakness, absent ankle jerks, and gait abnormalities were associated with CMT. In children with pes cavus, certain clinical features can predict CMT and assist in selection of patients for further, potentially uncomfortable (EMG) and expensive (genetic) confirmatory investigations. Copyright © 2012 Wiley Periodicals, Inc.

  12. Establishing clinical severity for PROMIS(®) measures in adult patients with rheumatic diseases.

    PubMed

    Nagaraja, Vivek; Mara, Constance; Khanna, Puja P; Namas, Rajaie; Young, Amber; Fox, David A; Laing, Timothy; McCune, William J; Dodge, Carol; Rizzo, Debra; Almackenzie, Maha; Khanna, Dinesh

    2017-10-05

    Different patient-reported outcome (PRO) measures are used for rheumatic diseases (RD). The aims of this study are-(1) Identify PROMIS(®) domains most relevant to care of patients with RD, (2) Collect T-Score metrics in patients with RD, and (3) Identify clinically meaningful cut-points for these domains. A convenience sample of RD patients was recruited consecutively during clinic visits, and asked to complete computer-adaptive tests on thirteen Patient-Reported Outcomes Measurement Information System (PROMIS(®)) instruments. Based on discussion with clinical providers, four measures were chosen to be relevant and actionable (from rheumatologists' perspective) in RD patients. Data from RD patients were used to develop clinical vignettes across a range of symptom severity. Vignettes were created based on most likely item responses at different levels on the T-score metric (mean = 50; SD = 10) and anchored at 5-point intervals (0.5 SDs). Patients with RD (N = 9) and clinical providers (N = 10) participated as expert panelists in separate one-day meetings using a modified educational standard setting method. Four domains (physical function, pain interferences, sleep disturbance, depression) that are actionable at the point-of-care were selected. For all domains, patients endorsed cut-points at lower levels of impairment than providers by 0.5 to 1 SD (e.g., severe impairment in physical function was defined as a T-score of 35 by patients and 25 by providers). We used a modified educational method to estimate clinically relevant cut-points to classify severity for PROMIS measures This allows for meaningful interpretation of PROMIS(®) measures in a clinical setting of RD population.

  13. Clinical Characteristics and Treatment Outcomes of Patients with Macrolide-Resistant Mycobacterium massiliense Lung Disease.

    PubMed

    Choi, Hayoung; Kim, Su-Young; Lee, Hyun; Jhun, Byung Woo; Park, Hye Yun; Jeon, Kyeongman; Kim, Dae Hun; Huh, Hee Jae; Ki, Chang-Seok; Lee, Nam Yong; Lee, Seung-Heon; Shin, Sung Jae; Daley, Charles L; Koh, Won-Jung

    2017-02-01

    Macrolide antibiotics are cornerstones in the treatment of Mycobacterium massiliense lung disease. Despite the emergence of resistance, limited data on macrolide-resistant M massiliense lung disease are available. This study evaluated the clinical features and treatment outcomes of patients and the molecular characteristics of macrolide-resistant M massiliense isolates. We performed a retrospective review of medical records and genetic analyses of clinical isolates from 15 patients who had macrolide-resistant M massiliense lung disease between September 2005 and February 2015. Nine patients (60%) had the nodular bronchiectatic form of the disease, and six (40%) had the fibrocavitary form. Before the detection of macrolide resistance, three patients (20%) were treated with macrolide monotherapy, four (27%) with therapy for presumed Mycobacterium avium complex infections, and eight (53%) with combination antibiotic therapy for M massiliense lung disease. The median treatment duration after the detection of resistance was 18.7 months (interquartile range, 11.2 to 39.8 months). Treatment outcomes were poor, with a favorable outcome being achieved for only one patient (7%), who underwent surgery in addition to antibiotic therapy. The 1-, 3-, and 5-year mortality rates were 7, 13, and 33%, respectively. Of the 15 clinical isolates, 14 (93%) had point mutations at position 2058 (n = 9) or 2059 (n = 5) of the 23S rRNA gene, resulting in macrolide resistance. Our study indicates that treatment outcomes are poor and mortality rates are high after the development of macrolide resistance in patients with M massiliense lung disease. Thus, preventing the development of macrolide resistance should be a key consideration during treatment.

  14. Effect of a Brief Video Intervention on Incident Infection among Patients Attending Sexually Transmitted Disease Clinics

    PubMed Central

    Warner, Lee; Klausner, Jeffrey D; Rietmeijer, Cornelis A; Malotte, C. Kevin; O'Donnell, Lydia; Margolis, Andrew D; Greenwood, Gregory L; Richardson, Doug; Vrungos, Shelley; O'Donnell, Carl R; Borkowf, Craig B

    2008-01-01

    Background Sexually transmitted disease (STD) prevention remains a public health priority. Simple, practical interventions to reduce STD incidence that can be easily and inexpensively administered in high-volume clinical settings are needed. We evaluated whether a brief video, which contained STD prevention messages targeted to all patients in the waiting room, reduced acquisition of new infections after that clinic visit. Methods and Findings In a controlled trial among patients attending three publicly funded STD clinics (one in each of three US cities) from December 2003 to August 2005, all patients (n = 38,635) were systematically assigned to either a theory-based 23-min video depicting couples overcoming barriers to safer sexual behaviors, or the standard waiting room environment. Condition assignment alternated every 4 wk and was determined by which condition (intervention or control) was in place in the clinic waiting room during the patient's first visit within the study period. An intent-to-treat analysis was used to compare STD incidence between intervention and control patients. The primary endpoint was time to diagnosis of incident laboratory-confirmed infections (gonorrhea, chlamydia, trichomoniasis, syphilis, and HIV), as identified through review of medical records and county STD surveillance registries. During 14.8 mo (average) of follow-up, 2,042 patients (5.3%) were diagnosed with incident STD (4.9%, intervention condition; 5.7%, control condition). In survival analysis, patients assigned to the intervention condition had significantly fewer STDs compared with the control condition (hazard ratio [HR], 0.91; 95% confidence interval [CI], 0.84 to 0.99). Conclusions Showing a brief video in STD clinic waiting rooms reduced new infections nearly 10% overall in three clinics. This simple, low-intensity intervention may be appropriate for adoption by clinics that serve similar patient populations. Trial registration: http://www.ClinicalTrials.gov (#NCT

  15. [Clinical aspects of the evolution of dental caries and periodontal disease in patients treated with corticosteroids].

    PubMed

    Lăcătuşu, St; Ghiorghe, Angela

    2004-01-01

    Patients treated with adrenal glucocorticoids may run a higher risk of dental caries, both as a result of their medical condition and of the physical and physiological effects of their pharmacotherapy. Our clinical study reports about patients treated with glucocorticoids who were also having an odonto-periodontal condition. They were examined and we found rampant caries and periodontal diseases. The slow evolution of asymptomatic periodontal disease encouraged destruction of teeth in root caries. The rampant caries were correlated with immunodeficiency and treatment of these caries must take into account the general treatment.

  16. Clinical and immunological data of nine patients with chronic mucocutaneous candidiasis disease

    PubMed Central

    Dotta, Laura; Scomodon, Omar; Padoan, Rita; Timpano, Silviana; Plebani, Alessandro; Soresina, Annarosa; Lougaris, Vassilios; Concolino, Daniela; Nicoletti, Angela; Giardino, Giuliana; Licari, Amelia; Marseglia, Gianluigi; Pignata, Claudio; Tamassia, Nicola; Facchetti, Fabio; Vairo, Donatella; Badolato, Raffaele

    2016-01-01

    This paper describes the heterogeneous clinical phenotype of a cohort of nine patients diagnosed with heterozygous mutations in STAT1. We report data of extended immunophenotyping over time and we show lung damage in four patients. The increased phosphorylation of STAT1 in response to IFNγ and IFNα stimulation proves the gain-of-function nature of the defects. The data are supplemental to our original article concurrently published “Clinical heterogeneity of dominant chronic mucocutaneous candidiasis disease: presenting as treatment-resistant candidiasis and chronic lung disease” [1], where additional results and interpretation of our research can be found. PMID:26981552

  17. Detection of borreliae in archived sera from patients with clinically suspect Lyme disease.

    PubMed

    Lee, Sin Hang; Vigliotti, Jessica S; Vigliotti, Veronica S; Jones, William; Shearer, David M

    2014-03-11

    The diagnoses of Lyme disease based on clinical manifestations, serological findings and detection of infectious agents often contradict each other. We tested 52 blind-coded serum samples, including 20 pre-treatment and 12 post-treatment sera from clinically suspect Lyme disease patients, for the presence of residual Lyme disease infectious agents, using nested PCR amplification of a signature segment of the borrelial 16S ribosomal RNA gene for detection and direct DNA sequencing of the PCR amplicon for molecular validation. These archived sera were split from the samples drawn for the 2-tier serology tests performed by a CDC-approved laboratory, and are used as reference materials for evaluating new diagnostic reagents. Of the 12 post-treatment serum samples, we found DNA evidence of a novel borrelia of uncertain significance in one, which was also positive for the 2-tier serology test. The rest of the post-treatment sera and all 20 control sera were PCR-negative. Of the 20 pre-treatment sera from clinically suspect early Lyme disease patients, we found Borrelia miyamotoi in one which was 2-tier serology-negative, and a Borrelia burgdorferi in two-one negative and one positive for 2-tier serology. We conclude that a sensitive and reliable DNA-based test is needed to support the diagnosis of Lyme disease and Lyme disease-like borreliosis.

  18. Elbow clinical, ultrasonographic and radiographic study in patients with inflammatory joint diseases.

    PubMed

    Uson, Jacqueline; Miguélez-Sánchez, Roberto; de Los Riscos, Marina; Martínez-Blasco, María Jesús; Fernández-Espartero, Cruz; Villaverde-García, Virginia; Garrido, Jesús; Naredo, Esperanza

    2016-03-01

    The main objective of this cross-sectional observational study was to investigate the relationship between clinical, ultrasonographic (US) and radiographic elbow features in patients with inflammatory joint diseases (IJD). The secondary objective was to evaluate the association between regional clinical elbow diagnoses and imaging findings. Consecutive patients with IJD attending follow-up visits were assessed for elbow pain and standardized elbow examination. Seven regional clinical diagnoses were defined. Digital elbow radiographs were read for 9 abnormalities. A standardized elbow grayscale (GS) and power Doppler (PD) scan recorded 13 defined abnormalities. Analysis encompassed 361 clinical, 361 US and 340 radiographic elbow assessments from 181 patients. US and clinical assessments showed an overall higher agreement than radiographic and clinical assessments (68.8 vs 59.1%, p = 0.001). When structural US abnormalities were compared with radiographic findings, agreement was slightly higher than when comparing all US abnormalities with radiographic findings (77.3%, k 0.533 and 73.5%, k 0.492). Enthesophytes, the most common abnormalities, were not associated with clinical findings. Subclinical US-synovitis and US-enthesopathy were found, respectively, in 17.3 and 14.1% of the clinically normal elbows. Clinical elbow arthritis prevalence and bias-adjusted kappa (PABAK) agreement was good for radiographic fat pad sign, PD-synovitis and GS-synovitis. Clinical elbow enthesopathy PABAK agreement was moderate for GS-enthesopathy and radiographic calcifications. US showed acceptable agreement with clinical and radiographic assessments for detecting elbow inflammatory and structural abnormalities in patients with IJD. Because US detected more abnormalities than radiography and has the capability to detect more subclinical abnormalities, US may be potentially used as a first-line elbow diagnostic tool in this clinical setting.

  19. Unrevealed Depression Involves Dysfunctional Coping Strategies in Crohn's Disease Patients in Clinical Remission

    PubMed Central

    Viganò, Caterina; Calzolari, Roberta; Marinaccio, Paola Marianna; Bezzio, Cristina; Furfaro, Federica; Ba, Gabriella; Maconi, Giovanni

    2016-01-01

    Background and Aims. This study investigated the proportion of CD patients in clinical remission with clinical depression, and coping strategies in those with severe depressive disorders. Materials and Methods. One hundred consecutive CD patients in clinical remission were screened for anxiety and depression by using Hospital Anxiety and Depression Scale and patients with depressive symptoms were further investigated by means of Cognitive Behavioural Assessment 2.0 and Beck Depression Inventory (BDI). Afterwards the coping strategies were assessed through the Brief-COPE questionnaire. Results. Twenty-one patients had anxious symptoms and 16 had depressive symptoms with or without anxiety. Seven of these patients (43.8%) showed significant depressive symptoms. Compared to patients without psychiatric disorders, these patients showed significant lower score in “positive reframing” (p: 0.017) and in “planning” (p: 0.046) and higher score in “use of instrumental social support” (p < 0.001), in “denial” scale (p: 0.001), and in “use of emotional social support” (p: 0.003). Conclusions. Depressed CD patients in clinical remission may have dysfunctional coping strategies, meaning that they may not be able to implement functional strategies to manage at best stress related with their disease. PMID:26823663

  20. Clinical Utility of Serologic Testing for Celiac Disease in Asymptomatic Patients

    PubMed Central

    2011-01-01

    Executive Summary Objective The objective of this evidence-based analysis was to evaluate the clinical utility of serologic testing for celiac disease in asymptomatic individuals presenting with one of the non-gastrointestinal conditions evaluated in this report. The clinical utility was based on the effects of a gluten-free diet (GFD) on outcomes specific to each of these conditions. The prevalence of celiac disease in asymptomatic individuals and one of these non-gastrointestinal conditions was also evaluated. Clinical Need and Target Population Celiac Disease Celiac disease is an autoimmune disease characterized by a chronic inflammatory state of the proximal small bowel mucosa accompanied by structural and functional changes. Technology Under Evaluation Serologic Tests for Celiac Disease There are a number of serologic tests for celiac disease available. Serologic tests are automated with the exception of the anti-endomysial antibody test, which is more time-consuming and operator-dependent than the other tests. Research Questions What is the prevalence of asymptomatic celiac disease in patients presenting with one of the non-gastrointestinal conditions evaluated? What is the effect of the gluten-free diet on condition-specific outcomes in patients with asymptomatic celiac disease presenting with one of the non-gastrointestinal conditions evaluated? What is the clinical utility of serologic testing for celiac disease in asymptomatic patients presenting with one of the non-gastrointestinal conditions evaluated? The clinical utility was defined as the impact of the GFD on disease specific outcomes. What is the risk of all-cause mortality and lymphoma in individuals with asymptomatic celiac disease? What is the budget impact of serologic testing for celiac disease in asymptomatic subjects presenting with one of the non-gastrointestinal conditions evaluated? Research Methods Study Population The study population consisted of individuals with newly diagnosed celiac

  1. The clinical significance of brain microbleeds in patients with Alzheimer's disease: Preliminary study

    PubMed Central

    Heo, Jae-Hyeok; Im, Dong-Gyu; Lee, Seung-Hyeon; Ahn, Jin-Young

    2016-01-01

    Background: Microbleeds (MBs) are observed frequently in Alzheimer's disease (AD) and suggested to play a crucial role in the pathophysiology, but their clinical significance remains unclear. Materials and Methods: The study recruited 100 patients with AD who were diagnosed at the memory clinic in Seoul Medical Center in 2014. For each patient, baseline characteristics, neuropsychological tests, cerebrovascular risk factors, medial temporal lobe atrophy (MTLA), and severity of small vessel disease (SVD) according to the existence of MBs were evaluated. Results: The prevalence of MBs in patients with AD was 33%. The percentage of male gender, the severity of SVD and MTLA were significantly increased in MB(+) group. The MB(+) group showed more severe MTLA and SVD than MB(−) group. Conclusions: These results suggested that MBs might reflect the burden of amyloid and ischemic vascular pathology. PMID:27994360

  2. Atrophy, hypometabolism and clinical trajectories in patients with amyloid-negative Alzheimer's disease.

    PubMed

    Chételat, Gaël; Ossenkoppele, Rik; Villemagne, Victor L; Perrotin, Audrey; Landeau, Brigitte; Mézenge, Florence; Jagust, William J; Dore, Vincent; Miller, Bruce L; Egret, Stéphanie; Seeley, William W; van der Flier, Wiesje M; La Joie, Renaud; Ames, David; van Berckel, Bart N M; Scheltens, Philip; Barkhof, Frederik; Rowe, Christopher C; Masters, Colin L; de La Sayette, Vincent; Bouwman, Femke; Rabinovici, Gil D

    2016-09-01

    See O'Sullivan and Vann (doi:10.1093/aww166) for a scientific commentary on this article.About 15% of patients clinically diagnosed with Alzheimer's disease do not show high tracer retention on amyloid positon emission tomography imaging. The present study investigates clinical and demographic features, patterns of brain atrophy and hypometabolism and longitudinal clinical trajectories of these patients. Forty amyloid-negative patients carrying a pre-scan diagnosis of Alzheimer's disease dementia from four centres were included (11/29 females/males; mean age = 67 ± 9). Detailed clinical histories, including the clinical diagnoses before and after the amyloid scan and at follow-up, were collected. Patients were classified according to their pre-scan clinical phenotype as amnestic (memory predominant), non-amnestic (predominant language, visuospatial or frontal symptoms), or non-specific (diffuse cognitive deficits). Demographic, clinical, neuropsychological, magnetic resonance imaging and (18)F-fluorodeoxyglucose positon emission tomography data were compared to 27 amyloid-positive typical Alzheimer's disease cases (14/13 females/males; mean age = 71 ± 10) and 29 amyloid-negative controls (15/14 females/males; mean age = 69 ± 12) matched for age, gender and education. There were 21 amnestic, 12 non-amnestic, and seven non-specific amyloid-negative Alzheimer's disease cases. Amyloid-negative subgroups did not differ in age, gender or education. After the amyloid scan, clinicians altered the diagnosis in 68% of amyloid-negative patients including 48% of amnestic versus 94% of non-amnestic and non-specific cases. Amnestic amyloid-negative cases were most often reclassified as frontotemporal dementia, non-amnestic as frontotemporal dementia or corticobasal degeneration, and non-specific as dementia with Lewy bodies or unknown diagnosis. The longer-term clinical follow-up was consistent with the post-scan diagnosis in most cases (90%), including in amnestic amyloid

  3. Clinical utility of serologic testing for celiac disease in asymptomatic patients: an evidence-based analysis.

    PubMed

    2011-01-01

    The objective of this evidence-based analysis was to evaluate the clinical utility of serologic testing for celiac disease in asymptomatic individuals presenting with one of the non-gastrointestinal conditions evaluated in this report. The clinical utility was based on the effects of a gluten-free diet (GFD) on outcomes specific to each of these conditions. The prevalence of celiac disease in asymptomatic individuals and one of these non-gastrointestinal conditions was also evaluated. CELIAC DISEASE: Celiac disease is an autoimmune disease characterized by a chronic inflammatory state of the proximal small bowel mucosa accompanied by structural and functional changes. TECHNOLOGY UNDER EVALUATION: SEROLOGIC TESTS FOR CELIAC DISEASE: There are a number of serologic tests for celiac disease available. Serologic tests are automated with the exception of the anti-endomysial antibody test, which is more time-consuming and operator-dependent than the other tests. What is the prevalence of asymptomatic celiac disease in patients presenting with one of the non-gastrointestinal conditions evaluated?What is the effect of the gluten-free diet on condition-specific outcomes in patients with asymptomatic celiac disease presenting with one of the non-gastrointestinal conditions evaluated?What is the clinical utility of serologic testing for celiac disease in asymptomatic patients presenting with one of the non-gastrointestinal conditions evaluated? The clinical utility was defined as the impact of the GFD on disease specific outcomes.What is the risk of all-cause mortality and lymphoma in individuals with asymptomatic celiac disease?What is the budget impact of serologic testing for celiac disease in asymptomatic subjects presenting with one of the non-gastrointestinal conditions evaluated? The study population consisted of individuals with newly diagnosed celiac disease without any symptoms consistent with the disease presenting with one of the non-gastrointestinal conditions

  4. Willingness to Use Health Insurance at a Sexually Transmitted Disease Clinic: A Survey of Patients at 21 US Clinics.

    PubMed

    Pearson, William S; Cramer, Ryan; Tao, Guoyu; Leichliter, Jami S; Gift, Thomas L; Hoover, Karen W

    2016-08-01

    To survey patients of publicly funded sexually transmitted disease (STD) clinics across the United States about their willingness to use health insurance for their visit. In 2013, we identified STD clinics in 21 US metropolitan statistical areas with the highest rates of chlamydia, gonorrhea, and syphilis according to Centers for Disease Control and Prevention surveillance reports. Patients attending the identified STD clinics completed a total of 4364 surveys (response rate = 86.6%). Nearly half of the insured patients were willing to use their health insurance. Patients covered by government insurance were more likely to be willing to use their health insurance compared with those covered by private insurance (odds ratio [OR] =  3.60; 95% confidence interval [CI] = 2.79, 4.65), and patients covered by their parents' insurance were less likely to be willing to use their insurance compared with those covered by private insurance (OR = 0.72; 95% CI = 0.52, 1.00). Reasons for unwillingness to use insurance were privacy and out-of-pocket cost. Before full implementation of the Affordable Care Act, privacy and cost were barriers to using health insurance for STD services. Barriers to using health insurance for STD services could be reduced through addressing issues of stigma associated with STD care and considering alternative payment sources for STD services.

  5. Intestinal protozoa infections among patients with ulcerative colitis: prevalence and impact on clinical disease course.

    PubMed

    Yamamoto-Furusho, Jesús K; Torijano-Carrera, Emma

    2010-01-01

    Epidemiological and microbiologic studies suggest that enteropathogenic microorganisms play a substantial role in the clinical initiation and relapses of inflammatory bowel disease. To explore the prevalence of intestinal protozoa in patients with ulcerative colitis (UC) and its impact on clinical disease course. A total of 215 patients with definitive diagnosis of UC were studied. Fresh feces samples taken from all UC patients were examined immediately using trichrome-staining methods. A total of 103 female and 112 male UC patients were analyzed. The mean age at diagnosis was 30.5 +/- 10.8 years. The prevalence of overall parasitic infections was 24% and distributed as follows: Blastocystis hominis in 22 patients (10%), Endolimax nana in 19 cases (9%), and Entamoebahistolytica in 11 cases (5%). A significantly increased frequency of protozoa infection was found in those patients with persistent activity and intermittent activity as compared to active than inactive group (p = 1 x 10(-7), OR 13.05, 95% CI 4.28-42.56, and p = 0.003, OR 1.42-14.47, respectively). Interestingly, this association remained significant when we compared the persistent activity group versus intermittent activity group (p = 0.003, OR 2.97, 95% CI 1.35-6.59). Subgroup analysis showed no association between protozoa infection (E. histolytica, B. hominis, and E. nana) and other clinical variables such as gender, extent of disease, extraintestinal complications, medical treatment and grade of disease activity. The prevalence of intestinal protozoa infections in Mexican UC patients was 24% and these microorganisms could be a contributing cause of persistent activity despite medical treatment in our population. 2010 S. Karger AG, Basel.

  6. [Clinical features of patients with Basedow Graves disease seen at a university hospital].

    PubMed

    Lanas, Alejandra; Díaz, Patricia; Eugenin, Daniela; González, Franco; Cid, Pía; Cordero, Francisco; Araya, Verónica; Liberman, Claudio; Barberan, Marcela; Gac, Patricio; Saldías, Neil; Pineda, Pedro

    2017-04-01

    Basedow Graves disease (BGD) is the leading cause of hyperthyroidism. The characteristics of patients seen at a university hospital may differ from those described in the general population. To describe the clinical features of patients with BGD seen at a university hospital. Review of medical records of all patients seen at our hospital between 2009 and 2014 with the diagnosis of thyrotoxicosis, hyperthyroidism or BGD. Clinical features, laboratory results and treatments were recorded. We reviewed clinical records of 272 patients; 15 had to be excluded due to incomplete data. BGD was present in 77.9% (n = 212). The mean age of the latter was 42 years (range 10-81) and 76% were women. Ninety six percent were hyperthyroid at diagnosis and thyroid stimulating hormone was below 0.1 mIU/L in all patients. Median free thyroxin and triiodothyronine levels were 3.26 ng/dl and 3.16 ng/ml, respectively. Thyrotropin-receptor antibodies were positive in 98.5% and 85.7% had positive thyroid peroxidase antibodies. Graves orbitopathy (GO) was clinically present in 55% of patients. Of this group, 47% had an active GO, 26% had a moderate to severe disease and 7.8% had sight-threatening GO. As treatment, 26% received radioiodine, 44% anti-thyroid drugs exclusively, 28% underwent thyroidectomy and 2% did not require therapy. In this group of patients, we observed a greater frequency of severe eye disease and a high rate of surgical management. This finding could be explained by referral to highly qualified surgical and ophthalmological teams.

  7. Association of nailfold capillary changes with disease activity, clinical and laboratory findings in patients with dermatomyositis

    PubMed Central

    Shenavandeh, Saeedeh; Zarei Nezhad, Maryam

    2015-01-01

    Background: The present study aimed to investigate the Nailfold Capillaroscopy (NC) features of the patients with dermatomyositis (DM) and its correlation with their disease activity indices, physical findings, and laboratory results. Methods: The present cross-sectional study was conducted on 27 DM patients above 16 years old who had referred to an(there are 3 clinics not one) outpatient rheumatology clinics from 2012 to 2013. Nailfold capillaroscopy and calculation of disease activity indices were performed separately for all the patients by two rheumatologists who were blinded to each other's results. Statistical analyses were performed using chi-square and Mann-Whitney U tests. Results: The mean age of the patients was 39.2±14.1 years with the mean disease duration of 13.1±15.2 months (range: 1-72 months). Myopathic electromyography (EMG) findings showed a strong association with scleroderma pattern (p=0.015). However, disease activity in each organ system and global disease activity showed no significant association between scleroderma pattern and other NC findings. (Disease activity in each organ system and also global disease activity were both assessed to see if they are associated with scleroderma pattern and other NC findings so if we use between it means we are looking for an association between scleroderma pattern and other NC findings and this is not what we have done and is wrong.) Conclusion: This study revealed no significant relationship between disease activity indices and NC features. Thus, it may be more precise to interpret the results of NC in conjunction with other physical and laboratory findings. PMID:26793626

  8. Challenges in the clinical measurement of ocular surface disease in glaucoma patients

    PubMed Central

    Pflugfelder, Stephen C; Baudouin, Christophe

    2011-01-01

    Ocular surface disease (OSD) is common among glaucoma patients. Clinical assessment of OSD can be challenging. This review focuses on some of the limitations relating to both subjective and objective measures of OSD, including dry eye. A survey of the literature was conducted to identify the caveats associated with different methods of assessing OSD. The effect of preservatives on the ocular surface, with respect to glaucoma patients in particular, was also reviewed. Objective methods for assessing ocular surface health and disease include the Schirmer test, tear break-up time, fluorescein turnover, corneal and conjunctival staining, tear osmolarity, and vital dyes. These measures all have limitations in terms of their ability to grade the severity of OSD. Previous studies using the OSD Index showed a mild-to-moderate correlation to dry eye disease severity. Other scoring systems for dry eye have shown a relationship to patient symptom scores or quality of life. Due to the challenges clinicians face concerning both subjective and objective ocular surface health assessments, discerning clinical improvement in ocular surface disease can be a challenge. Further research is needed in order to optimize existing clinical methods and/or identify alternative techniques for assessing OSD in the glaucoma population. PMID:22125404

  9. Risk and clinical characteristics of lymphoma in Korean patients with inflammatory bowel diseases: a multicenter study.

    PubMed

    Park, Soo-Kyung; Ye, Byong Duk; Lee, Changhyun; Im, Jong Pil; Kim, Young-Ho; Kim, Seon-Ok; Byeon, Jeong-Sik; Myung, Seung-Jae; Yang, Suk-Kyun; Kim, Jin-Ho

    2015-02-01

    Studies of lymphoma risk in Western inflammatory bowel disease (IBD) patients show conflicting results; however, none have examined the lymphoma risk and clinical characteristics of Asian IBD patients. Patients with lymphoma were identified in an IBD database from 3 tertiary referral centers in Seoul, Korea. The standardized incidence ratio (SIR) of lymphoma was estimated using data from the Korea Central Cancer Registry of the National Cancer Center. The risk of lymphoma in relation to specific medications was also explored. Seven cases of lymphoma (0.1%) were identified in 6585 IBD patients. The median age at lymphoma diagnosis was 43 years (range, 33 to 70 y) and the median duration of IBD at lymphoma diagnosis was 96.1 months (range, 15.1 to 171.6 mo). Three patients had underlying ulcerative colitis and 4 had Crohn's disease (CD). Non-Hodgkin lymphoma was diagnosed in 5 patients (71.4%) and Hodgkin disease (HD) in 2 patients (28.6%). The SIR of lymphoma was 2.03 [95% confidence interval (CI), 0.81-4.18] in the entire IBD patients. Both the SIR of lymphoma in CD patients (9.31; 95% CI, 1.13-33.62) and the SIR of HD (13.16; 95% CI, 1.59-47.53) in IBD patients were increased. The SIR of lymphoma in patients who were exposed to thiopurines was 5.93 (95% CI, 1.61-15.18). The risk of lymphoma in CD patients and the risk of HD in IBD patients seem to be increased in Korea. Thiopurine may be related with the risk of lymphoma in Korean IBD patients.

  10. Detecting HIV among persons accompanying patients to an infectious diseases clinic.

    PubMed

    Bischof, Jason J; Bell, Lynda L; Pierce, Jonah K; Cooper, Katherine L; Heine, Amy D; Quinlivan, Evelyn Byrd; Gay, Cynthia L

    2015-01-01

    Infectious diseases (ID) clinics are locations where members of at risk social networks, including sex partners of HIV-infected patients, make contact with a medical care setting when they accompany HIV-positive patients to appointments. We implemented a free point-of-care rapid HIV testing program for anyone accompanying a patient to the University of North Carolina ID clinic. Acceptability of the program among the general clinic population was assessed via an anonymous survey 1 year after program implementation. Basic frequencies of those who underwent and received results of rapid HIV testing, the proportion of positive rapid tests and confirmatory HIV tests performed, and the level of University of North Carolina ID clinic patient satisfaction with the HIV testing program were calculated. Between October 2007 and June 2013, 450 (99.6%) of 452 individuals tested in the program received their results on the same day as testing. Twenty-two individuals (4.9%) tested HIV positive, of which 16 (72.7%) were newly positive, including 3 never previously tested. Excluding previously diagnosed individuals, HIV prevalence was 3.6% (16/446). Among those testing positive by rapid testing, 19 (86.4%) had confirmatory testing and immediately entered into HIV care at the clinic. The high positivity and confirmatory HIV rates in our program confirm that the provision of rapid HIV testing in an ID clinic capitalizes on missed opportunities among an at-risk population and allows immediate linkage to care.

  11. Evolution after switching to biosimilar infliximab in inflammatory bowel disease patients in clinical remission.

    PubMed

    Guerrero Puente, Lourdes; Iglesias Flores, Eva; Benítez, José Manuel; Medina Medina, Rosario; Salgueiro Rodríguez, Isabel; Aguilar Melero, Patricia; Cárdenas Aranzana, Manuel Jesús; González Fernández, Rafael; Manzanares Martin, Bárbara; García-Sánchez, Valle

    2017-08-30

    The biosimilar of infliximab (CT-P13) has been approved for the same indications held by the infliximab reference product (Remicade(®)); however, there are few clinical data on switching in inflammatory bowel disease (IBD). The aim of this study was to assess the efficacy, safety, bioavailability profile and factors associated with relapse after switching to biosimilar infliximab in IBD patients in clinical remission. Observational study with IBD patients treated with Remicade(®) for at least 6 months and in clinical remission for at least 3 months who switched to infliximab biosimilar. The incidence of relapse, adverse effects and possible changes in drug bioavailability (trough level and antidrug antibodies) were evaluated. Thirty six patients were included (63.9% CD) with a mean follow-up of 8.4 months (SD±3.5). The 13.9% had clinical relapse. The longer clinical remission time before switching (HR=0.54, 95% CI=0.29-0.98, P=.04) and detectable infliximab levels at the time of switching (HR=0.03, 95% CI=0.001-0.89, P=.04) were associated with a lower risk of relapse. No differences were found between infliximab levels at the time of switching and at weeks 8 and 16 (P=.94); 8.3% of the patients had some adverse event, requiring the suspension of biosimilar in one patient for severe pneumonia. Switching to biosimilar infliximab in a real-life cohort of IBD patients in clinical remission did not have a significant impact on short-term clinical outcomes. The factors associated with relapse were similar to those expected in patients continuing with Remicade(®). Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

  12. The hidden Niemann-Pick type C patient: clinical niches for a rare inherited metabolic disease.

    PubMed

    Hendriksz, Christian J; Anheim, Mathieu; Bauer, Peter; Bonnot, Olivier; Chakrapani, Anupam; Corvol, Jean-Christophe; de Koning, Tom J; Degtyareva, Anna; Dionisi-Vici, Carlo; Doss, Sarah; Duning, Thomas; Giunti, Paola; Iodice, Rosa; Johnston, Tracy; Kelly, Dierdre; Klünemann, Hans-Hermann; Lorenzl, Stefan; Padovani, Alessandro; Pocovi, Miguel; Synofzik, Matthis; Terblanche, Alta; Then Bergh, Florian; Topçu, Meral; Tranchant, Christine; Walterfang, Mark; Velten, Christian; Kolb, Stefan A

    2017-05-01

    Niemann-Pick disease type C (NP-C) is a rare, inherited neurodegenerative disease of impaired intracellular lipid trafficking. Clinical symptoms are highly heterogeneous, including neurological, visceral, or psychiatric manifestations. The incidence of NP-C is under-estimated due to under-recognition or misdiagnosis across a wide range of medical fields. New screening and diagnostic methods provide an opportunity to improve detection of unrecognized cases in clinical sub-populations associated with a higher risk of NP-C. Patients in these at-risk groups ("clinical niches") have symptoms that are potentially related to NP-C, but go unrecognized due to other, more prevalent clinical features, and lack of awareness regarding underlying metabolic causes. Twelve potential clinical niches identified by clinical experts were evaluated based on a comprehensive, non-systematic review of literature published to date. Relevant publications were identified by targeted literature searches of EMBASE and PubMed using key search terms specific to each niche. Articles published in English or other European languages up to 2016 were included. Several niches were found to be relevant based on available data: movement disorders (early-onset ataxia and dystonia), organic psychosis, early-onset cholestasis/(hepato)splenomegaly, cases with relevant antenatal findings or fetal abnormalities, and patients affected by family history, consanguinity, and endogamy. Potentially relevant niches requiring further supportive data included: early-onset cognitive decline, frontotemporal dementia, parkinsonism, and chronic inflammatory CNS disease. There was relatively weak evidence to suggest amyotrophic lateral sclerosis or progressive supranuclear gaze palsy as potential niches. Several clinical niches have been identified that harbor patients at increased risk of NP-C.

  13. Comorbidities of patients in tiotropium clinical trials: comparison with observational studies of patients with chronic obstructive pulmonary disease

    PubMed Central

    Miravitlles, Marc; Price, David; Rabe, Klaus F; Schmidt, Hendrik; Metzdorf, Norbert; Celli, Bartolome

    2015-01-01

    Background There is an ongoing debate on whether patients with chronic obstructive pulmonary disease (COPD) seen in real-life clinical settings are represented in randomized controlled trials (RCTs) of COPD. It is thought that the stringent inclusion and exclusion criteria of RCTs may prevent the participation of patients with specific characteristics or risk factors. Methods We surveyed a database of patients recruited into 35 placebo-controlled tiotropium RCTs and also conducted a systematic literature review of large-scale observational studies conducted in patients with a documented diagnosis of COPD between 1990 and 2013. Patient demographics and comorbidities with a high prevalence in patients with COPD were compared between the two patient populations at baseline. Using the Medical Dictionary for Regulatory Activities (MedDRA; v 14.0), patient comorbidities in the pooled tiotropium RCTs were classified according to system organ class, pharmacovigilance (PV) endpoints, and Standardised MedDRA Queries to enable comparison with the observational studies. Results We identified 24,555 patients in the pooled tiotropium RCTs and 61,361 patients among the 13 observational studies that met our search criteria. The Global initiative for chronic Obstructive Lung Disease (GOLD) staging of patients in the RCTs differed from that in observational studies: the proportion of patients with GOLD stages I+II disease ranged from 40.0% to 51.5% in the RCTs but 24.5% to 44.1% in the observational studies; for GOLD stage III or IV disease these ranges were 7.2%–45.8% (RCTs) and 13.7–42.1% (observational studies). The comorbidities with the highest prevalence reported in the RCTs and observational studies were: hypertension (39.4%–40.0% vs 40.1%–60.6%), other ischemic heart disease (12.3%–14.2% vs 12.5%–41.0%), diabetes (10.3%–10.9% vs 4.0%–38.9%), depression (8.5%–9.5% vs 17.0%–20.6%), and cardiac arrhythmia (7.8%–11.4% vs 11.3%–15.8%). Conclusion The

  14. Collaborative practice model between cardiologists and clinical pharmacists for management of patients with cardiovascular disease in an outpatient clinic.

    PubMed

    Ripley, Toni L; Adamson, Philip B; Hennebry, Thomas A; Van Tuyl, Joseph S; Harrison, Donald L; Rathbun, R Chris

    2014-03-01

    The increasing prevalence of cardiovascular disease (CVD) has prompted leading cardiovascular organizations to advocate utilization of a team approach to patient care that includes nonphysician providers. In spite of that, the American College of Cardiology reported that nonphysician providers are underutilized in the management of patients with CVD. A survey of cardiologists revealed that the underutilization is a result of lack of understanding of how best to involve nonphysician providers in the health care team. Clinical pharmacists are one category of nonphysician providers that have recognized effectiveness in managing patients with CVD. No example of a comprehensive model of collaboration between cardiologists and clinical pharmacists is described in the literature that could serve to close this gap in understanding. The objective of this report is to describe a model of cardiologist-clinical pharmacist collaboration in the longitudinal management of patients with CVD that has been successfully implemented in 2 diverse settings. The implementation, evolution, scope of practice, required pharmacist training, logistical elements needed for success, and implementation barriers are reviewed. A summary of the patients referred to the clinic are examined as well.

  15. Clinical Features of Patients with Castleman's Disease Complicated by
    Systemic Lupus Erythematosus.

    PubMed

    Zhang, Lu; Cao, Xin-Xin; Wang, Shu-Jie; Zhou, Dao-Bin; Li, Jian

    2016-10-10

    Objective To investigate the clinical features of patients with Castleman's disease (CD) and systemic lupus erythematosus (SLE). Methods According to the diagnostic information between 1994 to 2014 extracted from the database of the Medical Record Department of Peking Union Medical College Hospital (PUMCH),patients with CD and SLE were included. A thorough literature review utilizing the key words of "Castleman's disease","systemic lupus erythematosus","SLE",and "lupus" was performed in PubMed during the same period. Cases with detailed clinical information were included while cases without detailed information were excluded from the analysis of this study. Results Nine patients worldwide were available for analysis [2 cases from PUMCH,accounted for 0.03%(2/6502) of all patients diagnosed as SLE and 1.0% (2/100) of patients diagnosed as CD during the same period] with a male-to-female ratio of2:7. The median age at diagnosis of CD was 39.0 years (range:21- 60 years). All patients were diagnosed as multicentric CD with generalized peripheral lymphadenopathy. Pathologic examination showed a balanced distribution:plasma cell variant:hyaline-vascular variant:mixed variant=3:3:3. Fever was the most common symptom (88.9%,8/9). Blood system was the most commonly involved system (88.9%,8/9) and kidneys were the most commonly involved organ (88.9%,8/9). Autoimmune thrombocytopenia (AITP) was observed in 55.6% (5/9) of patients,which was significantly higher than the general SLE patients (15.0%) (P<0.01). None of the 9 patients had evidence of central nervous system involvement. Conclusions CD complicated by SLE is a rare clinical condition. Compared to the general SLE population,this subgroup of patients may have higher rate of AITP and lower rate of central nervous system involvement.

  16. A clinical model to identify patients with high-risk coronary artery disease.

    PubMed

    Yang, Yelin; Chen, Li; Yam, Yeung; Achenbach, Stephan; Al-Mallah, Mouaz; Berman, Daniel S; Budoff, Matthew J; Cademartiri, Filippo; Callister, Tracy Q; Chang, Hyuk-Jae; Cheng, Victor Y; Chinnaiyan, Kavitha; Cury, Ricardo; Delago, Augustin; Dunning, Allison; Feuchtner, Gudrun; Hadamitzky, Martin; Hausleiter, Jörg; Karlsberg, Ronald P; Kaufmann, Philipp A; Kim, Yong-Jin; Leipsic, Jonathon; LaBounty, Troy; Lin, Fay; Maffei, Erica; Raff, Gilbert L; Shaw, Leslee J; Villines, Todd C; Min, James K; Chow, Benjamin J W

    2015-04-01

    This study sought to develop a clinical model that identifies patients with and without high-risk coronary artery disease (CAD). Although current clinical models help to estimate a patient's pre-test probability of obstructive CAD, they do not accurately identify those patients with and without high-risk coronary anatomy. Retrospective analysis of a prospectively collected multinational coronary computed tomographic angiography (CTA) cohort was conducted. High-risk anatomy was defined as left main diameter stenosis ≥50%, 3-vessel disease with diameter stenosis ≥70%, or 2-vessel disease involving the proximal left anterior descending artery. Using a cohort of 27,125, patients with a history of CAD, cardiac transplantation, and congenital heart disease were excluded. The model was derived from 24,251 consecutive patients in the derivation cohort and an additional 7,333 nonoverlapping patients in the validation cohort. The risk score consisted of 9 variables: age, sex, diabetes, hypertension, current smoking, hyperlipidemia, family history of CAD, history of peripheral vascular disease, and chest pain symptoms. Patients were divided into 3 risk categories: low (≤7 points), intermediate (8 to 17 points) and high (≥18 points). The model was statistically robust with area under the curve of 0.76 (95% confidence interval [CI]: 0.75 to 0.78) in the derivation cohort and 0.71 (95% CI: 0.69 to 0.74) in the validation cohort. Patients who scored ≤7 points had a low negative likelihood ratio (<0.1), whereas patients who scored ≥18 points had a high specificity of 99.3% and a positive likelihood ratio (8.48). In the validation group, the prevalence of high-risk CAD was 1% in patients with ≤7 points and 16.7% in those with ≥18 points. We propose a scoring system, based on clinical variables, that can be used to identify patients at high and low pre-test probability of having high-risk CAD. Identification of these populations may detect those who may benefit

  17. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

    NASA Astrophysics Data System (ADS)

    Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

    2016-09-01

    To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862) while for subacute ILD patients (disease duration 3–6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM.

  18. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis.

    PubMed

    Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

    2016-09-12

    To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862); while for subacute ILD patients (disease duration 3-6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM.

  19. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

    PubMed Central

    Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

    2016-01-01

    To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862); while for subacute ILD patients (disease duration 3–6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM. PMID:27615411

  20. The Enhanced liver fibrosis score is associated with clinical outcomes and disease progression in patients with chronic liver disease.

    PubMed

    Irvine, Katharine M; Wockner, Leesa F; Shanker, Mihir; Fagan, Kevin J; Horsfall, Leigh U; Fletcher, Linda M; Ungerer, Jacobus P J; Pretorius, Carel J; Miller, Gregory C; Clouston, Andrew D; Lampe, Guy; Powell, Elizabeth E

    2016-03-01

    Current tools for risk stratification of chronic liver disease subjects are limited. We aimed to determine whether the serum-based ELF (Enhanced Liver Fibrosis) test predicted liver-related clinical outcomes, or progression to advanced liver disease, and to compare the performance of ELF to liver biopsy and non-invasive algorithms. Three hundred patients with ELF scores assayed at the time of liver biopsy were followed up (median 6.1 years) for liver-related clinical outcomes (n = 16) and clear evidence of progression to advanced fibrosis (n = 18), by review of medical records and clinical data. Fourteen of 73 (19.2%) patients with ELF score indicative of advanced fibrosis (≥9.8, the manufacturer's cut-off) had a liver-related clinical outcome, compared to only two of 227 (<1%) patients with ELF score <9.8. In contrast, the simple scores APRI and FIB-4 would only have predicted subsequent decompensation in six and four patients respectively. A unit increase in ELF score was associated with a 2.53-fold increased risk of a liver-related event (adjusted for age and stage of fibrosis). In patients without advanced fibrosis on biopsy at recruitment, 55% (10/18) with an ELF score ≥9.8 showed clear evidence of progression to advanced fibrosis (after an average 6 years), whereas only 3.5% of those with an ELF score <9.8 (8/207) progressed (average 14 years). In these subjects, a unit increase in ELF score was associated with a 4.34-fold increased risk of progression. The ELF score is a valuable tool for risk stratification of patients with chronic liver disease. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. CFTR genotype and clinical outcomes of adult patients carried as cystic fibrosis disease.

    PubMed

    Bonadia, Luciana Cardoso; de Lima Marson, Fernando Augusto; Ribeiro, Jose Dirceu; Paschoal, Ilma Aparecida; Pereira, Monica Corso; Ribeiro, Antonio Fernando; Bertuzzo, Carmen Silvia

    2014-05-01

    There are nearly 2000 cystic fibrosis transmembrane regulator (CFTR) mutations that cause cystic fibrosis (CF). These mutations are classified into six classes; on the one hand, the first three classes cause severe disease involvement in early childhood, on the other hand, the Class IV, V and VI mutations cause minor severe disease in the same age. Nowadays, with therapeutic advances in CF management and competence of pediatricians, physicians of adults have to deal with two groups of CF patients: (i) adults diagnosed in childhood with severe mutations and (ii) adults who initiated symptoms in adulthood and with Class IV, V and VI mutations. The aim of this study was to analyze adults from a clinical center, treated as CF disease, screening the CFTR genotype and evaluating the clinical characteristics. Thirty patients followed as CF disease at the University Hospital were enrolled. After a complete molecular CFTR negative screening and sweat test levels between 40 and 59mEq/L, five patients were characterized as non-CF disease and were excluded. Molecular screening was performed by CFTR gene sequencing/MLPA or by specific mutation screening. Clinical data was obtained from medical records. The patients were divided into three groups: (1) patients with Class I, II and III mutations in two CFTR alleles; (2) genotype with at least one allele of Class IV, V or VI CFTR mutations and, (3) non-identified CFTR mutation+one patient with one allele with CFTR mutation screened (Class I). There was an association of CFTR class mutation and sodium/chloride concentration in the sweat test (sodium: p=0.040; chloride: p=0.016), onset of digestive symptoms (p=0.012), lung function parameter (SpO2 - p=0.016), Bhalla score (p=0.021), age at diagnosis (p=0.008) and CF-related diabetes (p=0.029). There was an association between Pseudomonas aeruginosa chronic colonization (as clinical marker for the lung disease status) and lung impairment (FEV1% - p=0.027; Bhalla score - p=0.021), CF

  2. Long-term clinical outcomes of patients with rheumatoid arthritis and concomitant coronary artery disease

    PubMed Central

    Spartera, Marco; Godino, Cosmo; Baldissera, Elena; Campochiaro, Corrado; La Spina, Ketty; Aiello, Patrizia; Salerno, Anna; Cera, Michela; Magni, Valeria; Jabbour, Richard J; Dagna, Lorenzo; Tresoldi, Moreno; Cappelletti, Alberto; Alfieri, Ottavio; Colombo, Antonio; Sabbadini, Maria Grazia; Margonato, Alberto

    2017-01-01

    Background: Rheumatoid arthritis (RA) is associated with high morbidity and mortality predominately due to increased cardiovascular risk. Few reports are available regarding the management of coronary artery disease (CAD) in RA patients and the long-term clinical outcomes after coronary revascularization. Methods and results: All consecutive patients with RA were identified by retrospective review at a rheumatology tertiary center in Milan, Italy between 2001 and 2013. RA patients affected by significant CAD (RA-CAD+) were prospectively followed for major adverse cardiovascular and cerebrovascular events (MACCE) after percutaneous coronary revascularization (RA-PCI), coronary artery bypass grafting (RA-CABG) or medical therapy (RA-MT). Among 936 patients with RA, the presence of clinically significant CAD was found in 5.6% (53 patients, RA-CAD+). Of these, 32 patients (60%) underwent PCI (RA-PCI), 10 patients (19%) underwent CABG (RA-CABG) and 11 patients (21%) treated with MT (RA-MT). After a mean follow-up of 9±7 years, the rate of MACCE was 56% in RA-PCI patients, 50% in RA-CABG and 27% in RA-MT patients (P=0.184). The high MACCE rate was mainly driven by repeat coronary revascularization (47%) in the RA-PCI group and high rate of strokes (30%) in RA-CABG patients. Conclusion: In patients with rheumatoid arthritis and concomitant coronary artery disease (RA-CAD+), we observed at long-term follow-up a high MACCE rate, predominantly in those who underwent coronary revascularization PMID:28337386

  3. Clinical features of chronic granulomatous disease: a series of 26 patients from a single center.

    PubMed

    Turul-Ozgür, Tuba; Türkkani-Asal, Gülten; Tezcan, Ilhan; Köker, M Yavuz; Metin, Ayşe; Yel, Leman; Ersoy, Fügen; Sanal, Ozden

    2010-01-01

    Chronic granulomatous disease is a genetically determined immunodeficiency disorder affecting phagocytic cells rendering them unable to kill certain bacteria and fungi. The present study is a single-center retrospective study that aimed to document the clinical course of 26 children, with a median age of 2.5 years, from 21 families diagnosed as chronic granulomatous disease from 1989-2008. A median delay of 39 months was observed between the onset of infections and age at diagnosis. Pneumonia was the most common initial manifestation of the disease followed by lymphadenitis, skin abscess and diarrhea. An AR inheritance was predominant in the study group. All patients received antibacterial and antifungal prophylaxis, resulting in a marked decrease in the incidence of infections. Overall mortality was 19.2%. These results showed that all features in our group (clinical, progression and outcome) were similar to the literature except for the predominance of autosomal recessive form.

  4. The effects of the Kumamoto earthquake on the clinical symptoms of patients with Parkinson's disease.

    PubMed

    Hori, Hiroko; Kuratomi, Aki; Ishizaki, Masatoshi; Sakamoto, Tetsuro; Nishida, Yasuto; Ando, Yukio

    2017-08-31

    Our hospital is the designated treatment base for intractable neurological diseases in the Kumamoto Prefecture. It is located in the center of the prefecture where the major 7.3-magnitude Kumamoto earthquake was recorded in 2016. In order to examine whether this earthquake affected the clinical symptoms of patients with Parkinson's disease in this hospital, we investigated outpatients examined up to 4 weeks after the earthquake. The subjects were 26 consecutive patients without any changes in anti-Parkinson's disease treatment or their attending physician during the examination period. All of the items in Part III of the Unified Parkinson's Disease Rating Scale (UPDRS), which is a clinician-scored scale for monitoring and evaluating motor function, were confirmed with the subjects before and after the earthquakes. After the earthquakes, worsened symptoms were found in 7 patients and 7 patients felt better. On the UPDRS, worsened symptoms were most commonly found among the items examining "muscle rigidity" and "slowness of movement and decreased movement" among the 7 patients with exacerbated symptoms. After the earthquake, clinical symptoms worsened significantly in women (P = 0.0188), patients with mild symptoms (P = 0.0111), and those who suffered a high degree of personal loss, such as those whose homes were damaged, who were forced to take refuge, or who had to sleep in their car (P = 0.0184). The mental and emotional burden due to the earthquake might be particularly high in the group of patients with worsened symptoms, suggestive of a relationship between stress and the exacerbation of parkinsonian symptoms.

  5. A Palpable Painless Axillary Mass as the Clinical Manifestation of Castleman's Disease in a Patient with Hepatitis C Disease

    PubMed Central

    Papazafiropoulou, Athanasia K.; Angelidi, Angeliki M.; Kousoulis, Antonis A.; Christofilidis, Georgios; Sagia, Chariklia; Kaftanidou, Liountmila; Manoloudaki, Kassiani; Tsavari, Aikaterini; Kranidiotis, Georgios; Kamaratos, Alexandros; Melidonis, Andreas

    2016-01-01

    Introduction. Castleman's disease (CD) is a rare lymphoproliferative disorder. CD is divided into two clinical subtypes: the most common unicentric and the less usual multicentric subtype. The majority of unicentric CD affects the mediastinum, while neck, abdomen, and axilla are less common locations. Case Presentation. Herein, we describe a rare case of unicentric CD in the right axilla in a 36-year-old white male with a medical history of hepatitis C virus infection admitted to our hospital due to palpation of a painless mass in the right axilla. Complete excision of the lesion was performed and, one year after the diagnosis, patient was free of the disease. Conclusions. Although infrequent, it is important to include CD in the differential diagnosis when evaluating axillary lymphadenopathy particularly in young patients with a low-grade inflammation process and chronic disease even in the absence of an abnormal blood picture or organomegaly. PMID:27313621

  6. Relationship between clinical and pathologic findings in patients with chronic liver diseases

    PubMed Central

    Lu, Lun-Gen; Zeng, Min-De; Mao, Yi-Min; Li, Ji-Qiang; Qiu, De-Kai; Fang, Jing-Yuan; Cao, Ai-Ping; Wan, Mo-Bin; Li, Cheng-Zhong; Ye, Jun; Cai, Xiong; Chen, Cheng-Wei; Wang, Ji-Yao; Wu, Shan-Ming; Zhu, Jin-Shui; Zhou, Xia-Qiu

    2003-01-01

    AIM: To explore the relationship between clinical findings of patients with chronic liver diseases and the pathologic grading and staging of liver tissues. METHODS: The inflammatory activity and fibrosis of consecutive liver biopsies from 200 patients were determined according to the diagnosis criteria of chronic hepatitis in China established in 1995. A comparative analysis was carried out for 200 patients with chronic liver diseases by comparing their clinical manifestations, serum biochemical markers with the grading and staging of liver tissues. RESULTS: It was revealed that age, index of clinical symptoms and physical signs were obviously relevant to the pathologic grading and staging of liver tissues (P < 0.05). Blood platelet, red blood cells, aspartate aminotransferase (AST), N-terminal procollagen III (PIII NP) were apparently correlated with the degree of inflammation. PGA (prothrombin time, GGT, apoprotein A1) index, PGAA (PGA + △2-macroglobublin) index, albumin and albumin/globulin were relevant to both inflammation and fibrosis. Hyaluronic acid (HA) was an accurate variable for the severity of hepatic inflammation and fibrosis. The combination of serum markers for fibrosis could increase the diagnostic accuracy. It was notable that viral replication markers were not relevant to the degree of inflammation and fibrosis. CONCLUSION: There is a good correlation between clinical findings and the pathologic grading and staging of liver tissues, which may give aid to the noninvasive diagnosis of liver fibrosis. PMID:14669336

  7. Elderly patients with chronic kidney disease: do they really need referral to the nephrology clinic?

    PubMed

    McClure, Mark; Jorna, Thomas; Wilkinson, Laura; Taylor, Joanne

    2017-10-01

    Chronic kidney disease (CKD) is becoming increasingly common, especially in the elderly. In the UK, there has been a marked increase in the awareness and detection of CKD over the last decade. This is largely attributable to the introduction of automated estimated glomerular filtration rate (eGFR) reporting and renal indicators in the primary care Quality and Outcomes Framework (QOF) initiative, both of which were introduced in 2006. These two initiatives have had a significant impact on referral patterns to renal services. Across the UK there has been a sustained increase in patients referred to nephrology clinics. The increased referrals have led to an older patient cohort, for whom specialist nephrology input is of questionable clinical benefit. This study aims to assess the outcomes of such patients referred to nephrology clinics in Dorset. Retrospective data were collected on all new referrals to the nephrology outpatient clinic at Dorset County Hospital between April 2006 and March 2007. We specifically examined all patients >80 years of age who had CKD Stage 4 or 5. Outcomes of interest included the rate of decline in eGFR, renal-specific management implemented by the clinic, need for renal replacement therapy and death. These outcomes were used to compare the difference between those patients kept under regular follow-up in the nephrology clinic and those discharged back to primary care. Patients were followed up until March 2014. In all, 124 patients who were ≥80 years of age had CKD Stage 4 (115 patients) or 5 (9 patients). The mean age was 84.4 (range 80-95) years. In all, 66 patients were kept under regular follow-up in the clinic and 58 patients were discharged back to primary care. Patients kept under follow-up tended to have a lower median eGFR at referral (22 mL/min/1.73 m(2) versus 26 mL/min/1.73 m(2); P = 0.051) and had a significantly more rapid decline in mean eGFR over the next 7 years (1.58 mL/min/1.73 m(2)/yr versus 0.357

  8. Clinical review of treatment options for major depressive disorder in patients with coronary heart disease.

    PubMed

    Alosaimi, Fahad D; Baker, Brian

    2012-11-01

    It is established that the prevalence of major depressive disorder (MDD) in coronary heart disease (CHD) populations is high and is associated with increased mortality. In this systematic review, we examined the evidence for the effective treatment of MDD in CHD patients by reviewing randomized control trials (RCTs) between 1980 and 2011 and then assessing whether these treatments were clinically meaningful. A total of 8 RCTs were retrieved. Sertraline, citalopram, and mirtazapine were safe from a cardiac perspective, but only sertraline and citalopram were clearly more effective than placebo in CHD patients with moderate-to-severe type, recurrent MDD, or MDD episode onset before the CHD event. Augmenting sertraline with omega-3 fatty acids did not result in superior depression outcomes. Cognitive-behavioral therapy was equivocally superior to usual care. Interpersonal psychotherapy was only superior to clinical management in patients with high baseline functional status. Exercise is a potential treatment for those with mild depression.

  9. [Clinical analysis of 7 patients with Gerstmann syndrome secondary to cerebral vascular disease].

    PubMed

    Zhou, Xiang-qin; He, Rui; Liu, Zhi; Wang, Chang-qing

    2002-10-01

    To analyze clinical features of patients with Gerstmann syndrome (GS). We retrospectively analysed the clinical manifestations of 7 patients (6 men and 1 woman) with GS secondary to cerebral vascular diseases and reviewed the literatures. The age ranged from 51 to 70 years with a mean of 70 years. They all had sudden onset and the tetrad of GS-finger agnosia, left-right disorientation, agraphia and acalculia, 3 patients accompanied by incomplete aphasia, 3 by anomic aphasia, 2 by alexia and 1 by constructional apraxia. Cranial computed tomographic scan showed low-density focus of the left parietal lobe in 6 cases and high-density focus of the left parietal lobe in 1 case. GS has the high value in localization and the lesion is mainly localized to angular gyrus of the dominant hemisphere.

  10. Kidney disease health literacy among new patients referred to a nephrology outpatient clinic.

    PubMed

    Burke, M T; Kapojos, J; Sammartino, C; Gray, N A

    2014-11-01

    Knowledge about kidney disease among the general population is poor but has not been assessed in the population selected for referral to nephrology care. This study aimed to determine patients' understanding of chronic kidney disease (CKD) when first presenting to a nephrology clinic. Newly referred patients to a nephrology clinic were surveyed with open-ended questions about their understanding of CKD causes, symptoms and management. Two hundred and ten patients were surveyed. Median age was 66.5 years (interquartile range 52-77), 50.5% female and mean body mass index 29.7 ± 6.8 kg/m(2) . Prevalence of risk factors for CKD included 31% diabetic, 62% hypertension, 19% family history of CKD and 2% Aboriginal or Torres Strait Islander. CKD stage prevalence was 0 (8%), 1 (24%), 2 (11%), 3 (38.5%), 4 (18%) and 5 (0.5%). Eighty-two per cent were referred by their primary care physician and 29% had seen a nephrologist previously. Kidney Health Australia was mentioned by 2.4%. Sixteen per cent were unsure why they had been referred. CKD causes identified by patients were unsure (40%), alcohol (29%), hypertension (16%) and diabetes (14%). Symptoms identified included asymptomatic (16%), kidney pain (17%) and other (42%). Management suggested by patients was uncertain (51%), dialysis (32%) and anti-hypertensive medication (16%). Eighty-two per cent reported unsatisfactory education from their primary care physician. New patients referred to a renal outpatient department had poor knowledge about kidney disease. Education of patients should begin in primary care prior to referral. For most patients, education programmes need to be targeted at a simplistic level. © 2014 The Authors; Internal Medicine Journal © 2014 Royal Australasian College of Physicians.

  11. Relationship between skin diseases and extracutaneous complications of diabetes mellitus: clinical analysis of 750 patients.

    PubMed

    Demirseren, Duriye Deniz; Emre, Selma; Akoglu, Gulsen; Arpacı, Dilek; Arman, Aysegul; Metin, Ahmet; Cakır, Bekir

    2014-02-01

    The relationship between skin diseases and extracutaneous complications in diabetes mellitus (DM) is unclear. We aimed to investigate the relationship between skin disorders and diabetic neuropathy, nephropathy, and retinopathy in patients with DM. A total of 750 patients with DM were prospectively enrolled. Demographic and clinical features, skin disorders, glycosylated hemoglobin (HbA1c) levels, and presence of nephropathy, neuropathy, and retinopathy were noted. Of the patients, 38.0% had neuropathy, 23.3% had nephropathy, and 22.9% had retinopathy. Any skin disorder was present in 79.2% (n = 594) of patients. The most common skin manifestations were cutaneous infections (47.5%), xerosis (26.4%), and inflammatory skin diseases (20.7%). The frequency of cutaneous infections, fungal infections, diabetic foot, rubeosis faciei, and pigmented purpuric dermatitis was higher in patients with nephropathy than in those without nephropathy. Cutaneous infections, diabetic foot, rubeosis faciei, and diabetic dermopathy were more common in patients with neuropathy. Fungal infections, diabetic foot, rubeosis faciei, diabetic dermopathy, and pigmented purpuric dermatitis were more frequent in patients with retinopathy. Patients with HbA1c ≥8 mmol/mL had more skin disorders than those with HbA1c <8 mmol/mL (P < 0.05 for all). Skin disorders may be clues to the presence of associated microvascular complications of DM.

  12. Attitudes and expectations of patients with neuromuscular diseases about their participation in a clinical trial.

    PubMed

    Gargiulo, M; Herson, A; Michon, C C; Hogrel, J Y; Doppler, V; Laloui, K; Herson, S; Payan, C; Eymard, B; Laforêt, P

    2013-01-01

    This study aimed to gain a better understanding of the psychological impact of participating in a clinical trial for patients with Pompe disease (Acid Maltase Deficiency). Attitudes and expectations of adult patients with neuromuscular diseases regarding medical trials are as yet unreported. In order to learn about the psychological consequences of participating in a clinical trial, we conducted a prospective assessment of patients with late-onset Pompe Disease, a rare genetic condition, for which no treatment had been available before. This psychological study was carried out as an ancillary study to the randomized double-blind placebo-controlled trial described elsewhere (van der Ploeg et al., 2010). We assessed patients (n=8) at inclusion, and at 12 and 18 months for six psychological dimensions: depression (Beck Depression Inventory, BDI), hopelessness (Beck Hopelessness Scale, BHS), anxiety (STAI A-B), quality of life (Whoqol-26), social adjustment (S.A.S-self-report) and locus of control (IPC Levenson). We produced a self-administered questionnaire in order to assess the attitudes, motivations and expectations of patients during the trial. At 12 months, mean social adjustment (SAS-SR, P=0.02) had improved, and at 18 months mean depression score had improved as well (BDI, P=0.03). The quality of life of patients (Whoqol-26) remained unchanged. Throughout the study, patients were more likely to have an internal locus of control than an external one (IPC Levenson). The self-administered questionnaire showed that patients' expectations were disproportionate compared to the medical information they had received starting the trial. For all patients, the first motivation for being enrolled in a clinical trial was "to help research", for half of them the motivation was to "improve their health". Whether patients believed to be part of one group or another (placebo or treatment) depended on their subjective perception of improvement during the trial. Given the small

  13. Frequency and Clinical Characteristics of Hydroxychloroquine Retinopathy in Korean Patients with Rheumatologic Diseases

    PubMed Central

    2017-01-01

    This study aimed to evaluate the frequency and clinical characteristics of hydroxychloroquine (HCQ) retinopathy in Korean patients with rheumatologic diseases. We retrospectively reviewed medical records of 310 patients taking HCQ. Ophthalmic examinations included spectral-domain optical coherence tomography (SD-OCT), automated visual field test, and fundus autofluorescence. The severity of retinopathy was categorized as early, moderate, or severe, and the location was categorized as parafoveal, pericentral, or mixed pattern. Among 310 patients, 9 patients (2.9%) were diagnosed as HCQ retinopathy. Among the patients with HCQ use ≥ 5 years (n = 174), the frequency was 5.2%. Only 1 (11.1%) of the 9 patients was symptomatic. The mean daily dose per kilogram of real body weight of the 9 patients was 5.6 mg, and only 3 had used 6.5 mg or more. Four of the 9 patients had severe HCQ retinopathy. Six of the 9 patients showed pericentral or mixed pattern of retinal damage. Consequently, the frequency of HCQ retinopathy in Korean patients was not low, especially when administered at a high cumulative dose and for a long duration. Screening of HCQ retinopathy by the recommended guidelines that include SD-OCT seems useful and should be done to detect retinal damage earlier in patients with chronic exposure to HCQ. PMID:28145658

  14. Predictors of physician referral for patient recruitment to Alzheimer disease clinical trials.

    PubMed

    Galvin, James E; Meuser, Thomas M; Boise, Linda; Connell, Cathleen M

    2009-01-01

    Inadequate recruitment into Alzheimer disease clinical trials is an important threat to the validity and generalizability of the studies. The majority of dementia patients are first evaluated by community-based physicians; however, physician perceptions of clinical research are largely unknown. A survey was distributed to 3123 physicians in 3 states; 370 were returned. Survey items assessed attitudes, perceived benefits of and barriers to referral to clinical research, and physicians use of the internet for medical information. The mean age of the respondents was 50.6+/-10.8 years; 70% were male, 78% white, 61% were primary care providers; 63% used the internet > or =3 times/week. No demographic or medical specialty differences existed between those who were likely (n=193) and unlikely (n=162) to refer patients to clinical trials. Differences were discovered in perceived benefits reported by physicians who were more likely to refer, whereas differences in perceived barriers existed in primary care compared with specialists. Referral to clinical trials is predicted by close proximity to a research center [odds ratio (OR): 4.0; 95% confidence interval (CI), 1.1-15.6] and availability of internet information regarding diagnostic evaluation (OR: 2.3; 95% CI, 1.1-4.7). Primary barriers included concerns about exposure of patients to uncomfortable procedures (OR: 4.7; 95% CI, 1.2-18.7) and lack of time to discuss research participation (OR: 6.8; 95% CI, 1.4-32.3). Proximity to a research center and availability of diagnostic clinical tools are strong predictors of clinical trial referral. Concern over risks to patients and lack of time are strong barriers. These results suggest that dementia outreach education targeted to physicians should emphasize the importance of clinical trials with a focus on discussing research participation in a time-efficient manner and increasing awareness of risk reduction and the safety of research protocols. Providing easy access to up

  15. Randomised clinical trial: the safety and tolerability of Trichuris suis ova in patients with Crohn's disease.

    PubMed

    Sandborn, W J; Elliott, D E; Weinstock, J; Summers, R W; Landry-Wheeler, A; Silver, N; Harnett, M D; Hanauer, S B

    2013-08-01

    Recent evidence suggests that embryonated eggs of the porcine whipworm Trichuris suis ova (TSO) may be an effective treatment for inflammatory bowel disease (IBD). To assess the safety and tolerability of TSO following a single dose in patients with Crohn's disease. This was a sequential dose-escalation (500, 2500 and 7500 viable embryonated TSO), randomised, double-blind, placebo-controlled study to evaluate the safety of a single dose of oral suspension TSO in patients with Crohn's disease. Twelve patients were randomised into each of three cohorts. Patients were assessed 1, 3, 5, 7, 9, 11 and 14 days following dosing (via a telephone call and diary symptom collection through 14 days postdose) for adverse events, changes to concomitant medications and gastrointestinal (GI) signs and symptoms. Patients were again assessed at Months 1, 2 and 6. Eighteen males and 18 females were enrolled, ages 20 to 54 years. All patients were dosed and completed the initial 2-month follow-up period (five patients did not attend their 6-month study visit). GI disorders were reported with the highest frequency; 7 (25.9%) TSO-treated patients and 3 (33.3%) placebo-treated patients. No dose-dependent relationship was observed, with 3 (33.3%) placebo, 4 (44.4%) TSO 500, 0 (0.0%) TSO 2500 and 3 (33.3%) TSO 7500 patients experiencing at least one GI event, and no clinically meaningful changes in GI signs and symptoms. A single dose of Trichuris suis ova up to 7500 ova was well tolerated and did not result in short- or long-term treatment-related side effects. Clinicaltrials.gov NCT01576461. © 2013 John Wiley & Sons Ltd.

  16. Clinical outcome of patients with refractory Kawasaki disease based on treatment modalities

    PubMed Central

    Kim, Hyun Jung; Lee, Hyo Eun; Yu, Jae Won

    2016-01-01

    Purpose Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. Methods We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. Results A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. Conclusion No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients. PMID:27610181

  17. Clinical outcome of patients with refractory Kawasaki disease based on treatment modalities.

    PubMed

    Kim, Hyun Jung; Lee, Hyo Eun; Yu, Jae Won; Kil, Hong Ryang

    2016-08-01

    Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients.

  18. Distinguishing SWEDDs patients with asymmetric resting tremor from Parkinson's disease: a clinical and electrophysiological study.

    PubMed

    Schwingenschuh, Petra; Ruge, Diane; Edwards, Mark J; Terranova, Carmen; Katschnig, Petra; Carrillo, Fatima; Silveira-Moriyama, Laura; Schneider, Susanne A; Kägi, Georg; Palomar, Francisco J; Talelli, Penelope; Dickson, John; Lees, Andrew J; Quinn, Niall; Mir, Pablo; Rothwell, John C; Bhatia, Kailash P

    2010-04-15

    Approximately 10% of patients diagnosed clinically with early Parkinson's disease (PD) have normal dopaminergic functional imaging (Scans Without Evidence of Dopaminergic Deficit [SWEDDs]). An important subgroup of SWEDDs are those with asymmetric rest tremor resembling parkinsonian tremor. Clinical and pathophysiological features which could help to distinguish SWEDDs from PD have not been explored. We therefore studied clinical details including non-motor symptoms in 25 tremulous SWEDDs patients in comparison to 25 tremor-dominant PD patients. Blinded video rating was used to compare examination findings. Electrophysiological tremor parameters and also response to a cortical plasticity protocol using paired associative stimulation (PAS) was studied in 9 patients with SWEDDs, 9 with tremor-dominant PD (with abnormal dopamine transporter single photon emission computed tomography findings), 8 with segmental dystonia, and 8 with essential tremor (ET). Despite clinical overlap, lack of true bradykinesia, presence of dystonia, and head tremor favored a diagnosis of SWEDDs, whereas re-emergent tremor, true fatiguing or decrement, good response to dopaminergic drugs, and presence of non-motor symptoms favored PD. A single tremor parameter could not differentiate between groups, but the combination of re-emergent tremor and highest tremor amplitude at rest was characteristic of PD tremor. SWEDDs and segmental dystonia patients exhibited an abnormal exaggerated response to the PAS protocol, in contrast to a subnormal response in PD and a normal response in ET. We conclude that despite clinical overlap, there are features that can help to distinguish between PD and SWEDDs which may be useful in clinical practice. The underlying pathophysiology of SWEDDs differs from PD but has similarities with primary dystonia.

  19. Modeling using clinical examination indicators predicts interstitial lung disease among patients with rheumatoid arthritis

    PubMed Central

    Wang, Yao; Song, Wuqi; Wu, Jing; Li, Zhangming; Mu, Fengyun; Li, Yang; Huang, He; Zhu, Wenliang

    2017-01-01

    Interstitial lung disease (ILD) is a severe extra-articular manifestation of rheumatoid arthritis (RA) that is well-defined as a chronic systemic autoimmune disease. A proportion of patients with RA-associated ILD (RA-ILD) develop pulmonary fibrosis (PF), resulting in poor prognosis and increased lifetime risk. We investigated whether routine clinical examination indicators (CEIs) could be used to identify RA patients with high PF risk. A total of 533 patients with established RA were recruited in this study for model building and 32 CEIs were measured for each of them. To identify PF risk, a new artificial neural network (ANN) was built, in which inputs were generated by calculating Euclidean distance of CEIs between patients. Receiver operating characteristic curve analysis indicated that the ANN performed well in predicting the PF risk (Youden index = 0.436) by only incorporating four CEIs including age, eosinophil count, platelet count, and white blood cell count. A set of 218 RA patients with healthy lungs or suffering from ILD and a set of 87 RA patients suffering from PF were used for independent validation. Results showed that the model successfully identified ILD and PF with a true positive rate of 84.9% and 82.8%, respectively. The present study suggests that model integration of multiple routine CEIs contributes to identification of potential PF risk among patients with RA. PMID:28243535

  20. Genetic Markers Associated with Clinical Outcomes in Patients with Inflammatory Bowel Disease.

    PubMed

    Yamamoto-Furusho, Jesús K; Fonseca-Camarillo, Gabriela

    2015-11-01

    Genetic factors play a significant role in determining inflammatory bowel disease (IBD) susceptibility. Epidemiologic data support genetic contribution to the pathogenesis of IBD, which include familial aggregation, twin studies, and racial and ethnic differences in disease prevalence. Recently, several new genes have been identified to be involved in the genetic susceptibility to IBD. The characterization of novel genes potentially will lead to the identification of therapeutic agents and clinical assessment of phenotype and prognosis in patients with IBD. The development of genetic markers associated with clinical outcomes in patients with IBD will be very important in the future. The progress of molecular biology tools (microarrays, proteomics, and epigenetics) have progressed the field of the genetic markers discovery. The advances in bioinformatics coupled with cross-disciplinary collaborations have greatly enhanced our ability to retrieve, characterize, and analyze large amounts of data generated by the technological advances. The techniques available for markers development are genomics (single nucleotide polymorphism genotyping, pharmacogenetics, and gene expression analyses) and proteomics. This could be a potential great benefit in predicting the course of disease in individual patients and in guiding appropriate medical therapy.

  1. Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis.

    PubMed

    Xu, Y; Yang, C S; Li, Y J; Liu, X D; Wang, J N; Zhao, Q; Xiao, W G; Yang, P T

    2016-01-01

    Clinically amyopathic dermatomyositis (CADM) is a unique subset of dermatomyositis, showing a high incidence of lung involvements. The aim of this study is to identify risk factors, other than melanoma differentiation-associated protein (MDA)-5, for developing rapidly progressive-interstitial lung disease (RP-ILD) in patients with CADM. Forty CADM patients, in whom 11 patients developed RP-ILD, were enrolled. Clinical features and laboratory findings were compared between the patients with and without RP-ILD. We found that skin ulceration, CRP, serum ferritin, anti-MDA5 Ab, and lymphocytopenia were significantly associated with ILD. Multivariate logistic regression analysis indicated that anti-MDA5 Ab(+), elevated CRP, and decreased counts of lymphocyte were independent risk factors for RP-ILD, which can provide a precise predict for RP-ILD in CADM patients. When anti-MDA5 Ab(+) was removed from the multivariate regression model, using skin ulcerations, elevated serum ferritin and decreased counts of lymphocyte can also precisely predict RP-ILD. Except for MDA-5, more commonly available clinical characteristics, such as skin ulcerations, serum ferritin, and count of lymphocyte may also help to predict prognosis in CADM.

  2. Clinical management of restless legs syndrome in end-stage renal disease patients.

    PubMed

    Sahli, Zeyad T; Jo, Jae; Mousa, Shaker A; Tarazi, Frank I

    2017-02-01

    Restless legs syndrome (RLS) is a common neurological movement disorder, characterized by restless and unpleasant sensations in the deep inside of legs. The symptoms of RLS are less noticeable during daytime, but more prevalent at night. Therefore, the disorder can induce low quality of life, insomnia, and impairment of daytime activity. RLS in end-stage renal disease (ESRD) patients is especially problematic due to premature discontinuation of dialysis and increased mortality. The prevalence of RLS among dialysis patients is much higher compared to the prevalence of the same disorder in patients with normal renal functions. Even though there are recommended treatment guidelines for the general population established by Medical Advisory Board of the RLS foundation, which include the use of dopamine agonists, levodopa, gabapentin, benzodiazepines, and opioids, limited information is available on the effects of these therapies in ESRD patients. Since the existing clinical data were extrapolated from small sample sizes in short-term clinical trials, further clinical studies are still needed to better assess the efficacy, safety, and tolerability of these medications in patients with ESRD.

  3. Clinical pharmacy-led disease and medicine management programme for patients with COPD

    PubMed Central

    Khdour, Maher R; Kidney, Joseph C; Smyth, Bronagh M; McElnay, James C

    2009-01-01

    AIM The aim was to investigate the impact of a disease and medicine management programme, focusing on self-management in patients with chronic obstructive pulmonary disease (COPD). METHODS One hundred and seventy-three patients (mean age 67 years; 54% female) were recruited; 86 patients were randomly assigned to an intervention group and 87 to a usual care (control) group. Intervention patients received education on disease state, medications and breathing techniques. Patients were given booklets and a customized action plan (antibiotic and oral steroid to be initiated promptly by patients for exacerbations). Patients were followed up at 6 and 12 months during a scheduled visit. The St George's Respiratory Questionnaire (SGRQ), COPD Knowledge and Morisky adherence questionnaires were administered to all patients at baseline, 6 and 12 months. Outcome measures included hospital admissions, emergency department (ED) visits, health-related quality of life (HRQoL) and medication adherence. RESULTS Over the 12-month period in the intervention group, ED visits decreased by 50% (P= 0.02) and hospitalization by approximately 60% (P= 0.01). On the SGRQ, differences reached statistical significance on the symptom (−7.5; P= 0.04) and impact (−7.4; P= 0.03) subscales but not on the physical activity subscale. There was a significant difference between the intervention and usual care groups regarding knowledge scores (75.0 vs. 59.3; P= 0.001) and good adherence to medication (77.8% vs. 60.0%, P= 0.019). There was no significant difference regarding smoking between study groups. CONCLUSIONS The clinical pharmacy-led management programme can improve adherence, reduce the need for hospital care in patients with COPD and improve aspects of their HRQoL. PMID:19843062

  4. Cross-sectional analysis of patient phone calls to an inflammatory bowel disease clinic

    PubMed Central

    Corral, Juan E.; Yarur, Andres J.; Diaz, Liege; Simmons, Okeefe L.; Sussman, Daniel A.

    2015-01-01

    Background Patients with inflammatory bowel disease (IBD) require close follow up and frequently utilize healthcare services. We aimed to identify the main reasons that prompted patient calls to gastroenterology providers and further characterize the “frequent callers”. Methods This retrospective cross-sectional study included all phone calls registered in medical records of IBD patients during 2012. Predictive variables included demographics, psychiatric history, IBD phenotype, disease complications and medical therapies. Primary outcome was the reason for call (symptoms, medication refill, procedures and appointment issues). Secondary outcome was the frequency of changes in management prompted by the call. Results 209 patients participated in 526 calls. The mean number of calls per patient was 2.5 (range 0-27); 49 (23.4%) patients met the criterion of “frequent caller”. Frequent callers made or received 75.9% of all calls. Crohn’s disease, anxiety, extra-intestinal manifestations and high sedimentation rate were significantly associated with higher call volume. 85.7% of frequent callers had at least one call that prompted a therapeutic intervention, compared to 18.9% of non-frequent callers (P<0.001). The most common interventions were ordering laboratory or imaging studies (15.4%), dose adjustments (12.1%), changes in medication class (8.4%), and expediting clinic visits (8.4%). Conclusion Most phone calls originated from a minority of patients. Repeated calling by the same patient and new onset of gastrointestinal (GI) and non-GI symptoms were important factors predicting the order of diagnostic modalities or therapeutic changes in care. Triaging calls to IBD healthcare providers for patients more likely to require a change in management may improve healthcare delivery. PMID:26126710

  5. The incidence and clinical characteristics by gender differences in patients with Kikuchi–Fujimoto disease

    PubMed Central

    Jung, In Young; Ann, Hea Won; Kim, Jung Ju; Lee, Se Ju; Kim, Jinnam; Seong, Hye; Oh, Dong Hyun; Kim, Yong Chan; Kim, Eun Jin; Jeong, Su Jin; Ku, Nam Su; Choi, Jun Yong; Song, Young Goo; Kim, June Myung

    2017-01-01

    Abstract Kikuchi–Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes (LNs). Although initially described in young women, KFD also occurs in men. There are no reports on the clinical manifestations and characteristics of male KFD patients. Therefore, this study was conducted to assess the incidence of KFD among males, as well as the most frequent clinical characteristics of these patients. A retrospective, cross-sectional study was performed at a tertiary hospital of patients pathologically confirmed as having KFD from LN biopsy specimens. Clinical and laboratory data, and treatment outcomes of the enrolled patients, were analyzed by gender. A total of 254 patients diagnosed with KFD were enrolled. There were 189 females and 65 males (2.9:1). The mean age was 32.6 ± 11.3 years. Compared to the female patients, the males had more frequent manifestations of fever (48% vs 67%, P = 0.008), headache (9% vs 20%, P = 0.013), bilateral lymphadenopathy (31% vs 46%, P = 0.029), thrombocytopenia (14% vs 29%, P = 0.014), elevated C-reactive protein (CRP) (35% vs 78.4%, P < 0.001), elevated liver enzymes (15% vs 41%, P < 0.001), and elevated lactate dehydrogenase (LDH) (61% vs 80%, P = 0.021). Male patients had fewer autoimmune features (9% vs 2%, P = 0.043) and fewer positive antinuclear antibodies (32% vs 10%, P = 0.006). In this study, 25.6% of the enrolled patients were male, with a 2.9:1 female-to-male sex ratio. Male patients showed a distinctive profile characterized by a higher frequency of fever, headache, bilateral lymphadenopathy, and thrombocytopenia, as well as elevated liver enzymes, CRP, and LDH. PMID:28296758

  6. Clinical Correlates of Apathy in Patients Recently Diagnosed with Parkinson's Disease: The ANIMO Study

    PubMed Central

    Cubo, Esther; Benito-León, Julián; Coronell, Carlos; Armesto, Diana

    2012-01-01

    Objective Little is known about apathy in the early stages of Parkinson's disease (PD). We determined the clinical correlates of apathy in a large representative sample of patients recently diagnosed with PD (ANIMO study). Methods PD patients, diagnosed within 2 years of inclusion, were recruited in 102 outpatient clinics situated in 82 populations throughout Spain. Apathy was quantified using the Lille Apathy Rating Scale (LARS). Clinical comparisons and correlations were performed using nonparametric tests. Regression analyses were used to test the association of clinical variables with apathy. Results We recruited 557 PD patients (60.3% men) with a mean age of 68.8 ± 9.7 years, and UPDRS motor score of 21.1 ± 10.8. Apathy only was diagnosed in 186 (33.4%), and apathy and depression in 215 patients (38.6%). Patients with higher comorbidity (OR = 1.10, 95% CI 1.01−1.20, p = 0.001), motor impairment (OR = 1.07, 95% CI 1.03−1.10, p < 0.0001), and lower education (OR = 2.16, 95% CI 1.21−;3.85, p = 0.009) had higher odds of having apathy, in contrast to patients living in a rural environment (OR = 0.35, 95% CI 0.32–0.85, p = 0.01), and left predominant PD motor laterality (OR = 0.34, 95% CI 0.13–0.88, p = 0.01). LARS scores were significantly correlated with UPDRS motor scores (rs = 0.44, p < 0.001), predominantly with axial score (rs = 0.43, p < 0.001). Conclusions In PD, apathy is a very common and disabling nonmotor symptom separable from depression. Patients living in a rural environment, with lower comorbidity and motor impairment, higher education background, and left predominant PD motor laterality are at lower risk of suffering from apathy. PMID:22236943

  7. Clinical correlates of apathy in patients recently diagnosed with Parkinson's disease: the ANIMO study.

    PubMed

    Cubo, Esther; Benito-León, Julián; Coronell, Carlos; Armesto, Diana

    2012-01-01

    Little is known about apathy in the early stages of Parkinson's disease (PD). We determined the clinical correlates of apathy in a large representative sample of patients recently diagnosed with PD (ANIMO study). PD patients, diagnosed within 2 years of inclusion, were recruited in 102 outpatient clinics situated in 82 populations throughout Spain. Apathy was quantified using the Lille Apathy Rating Scale (LARS). Clinical comparisons and correlations were performed using nonparametric tests. Regression analyses were used to test the association of clinical variables with apathy. We recruited 557 PD patients (60.3% men) with a mean age of 68.8 ± 9.7 years, and UPDRS motor score of 21.1 ± 10.8. Apathy only was diagnosed in 186 (33.4%), and apathy and depression in 215 patients (38.6%). Patients with higher comorbidity (OR = 1.10, 95% CI 1.01-1.20, p = 0.001), motor impairment (OR = 1.07, 95% CI 1.03-1.10, p < 0.0001), and lower education (OR = 2.16, 95% CI 1.21-3.85, p = 0.009) had higher odds of having apathy, in contrast to patients living in a rural environment (OR = 0.35, 95% CI 0.32-0.85, p = 0.01), and left predominant PD motor laterality (OR = 0.34, 95% CI 0.13-0.88, p = 0.01). LARS scores were significantly correlated with UPDRS motor scores (r(s) = 0.44, p < 0.001), predominantly with axial score (r(s) = 0.43, p < 0.001). In PD, apathy is a very common and disabling nonmotor symptom separable from depression. Patients living in a rural environment, with lower comorbidity and motor impairment, higher education background, and left predominant PD motor laterality are at lower risk of suffering from apathy. Copyright © 2012 S. Karger AG, Basel.

  8. Amyloid negativity in patients with clinically diagnosed Alzheimer disease and MCI

    PubMed Central

    Horng, Andy; Fero, Allison; Jagust, William J.

    2016-01-01

    Objective: To examine the clinical and biomarker characteristics of patients with amyloid-negative Alzheimer disease (AD) and mild cognitive impairment (MCI) from the Alzheimer's Disease Neuroimaging Initiative (ADNI), a prospective cohort study. Methods: We first investigated the reliability of florbetapir− PET in patients with AD and patients with MCI using CSF-Aβ1–42 as a comparison amyloid measurement. We then compared florbetapir− vs florbetapir+ patients with respect to several AD-specific biomarkers, baseline and longitudinal cognitive measurements, and demographic and clinician report data. Results: Florbetapir and CSF-Aβ1–42 +/− status agreed for 98% of ADs (89% of MCIs), indicating that most florbetapir− scans were a reliable representation of amyloid status. Florbetapir− AD (n = 27/177; 15%) and MCI (n = 74/217, 34%) were more likely to be APOE4-negative (MCI 83%, AD 96%) than their florbetapir+ counterparts (MCI 30%, AD 24%). Florbetapir− patients also had less AD-specific hypometabolism, lower CSF p-tau and t-tau, and better longitudinal cognitive performance, and were more likely to be taking medication for depression. In MCI only, florbetapir− participants had less hippocampal atrophy and hypometabolism and lower functional activity questionnaire scores compared to florbetapir+ participants. Conclusions: Overall, image analysis problems do not appear to be a primary explanation of amyloid negativity. Florbetapir− ADNI patients have a variety of clinical and biomarker features that differ from their florbetapir+ counterparts, suggesting that one or more non-AD etiologies (which may include vascular disease and depression) account for their AD-like phenotype. PMID:26968515

  9. Amyloid negativity in patients with clinically diagnosed Alzheimer disease and MCI.

    PubMed

    Landau, Susan M; Horng, Andy; Fero, Allison; Jagust, William J

    2016-04-12

    To examine the clinical and biomarker characteristics of patients with amyloid-negative Alzheimer disease (AD) and mild cognitive impairment (MCI) from the Alzheimer's Disease Neuroimaging Initiative (ADNI), a prospective cohort study. We first investigated the reliability of florbetapir- PET in patients with AD and patients with MCI using CSF-Aβ1-42 as a comparison amyloid measurement. We then compared florbetapir- vs florbetapir+ patients with respect to several AD-specific biomarkers, baseline and longitudinal cognitive measurements, and demographic and clinician report data. Florbetapir and CSF-Aβ1-42 +/- status agreed for 98% of ADs (89% of MCIs), indicating that most florbetapir- scans were a reliable representation of amyloid status. Florbetapir- AD (n = 27/177; 15%) and MCI (n = 74/217, 34%) were more likely to be APOE4-negative (MCI 83%, AD 96%) than their florbetapir+ counterparts (MCI 30%, AD 24%). Florbetapir- patients also had less AD-specific hypometabolism, lower CSF p-tau and t-tau, and better longitudinal cognitive performance, and were more likely to be taking medication for depression. In MCI only, florbetapir- participants had less hippocampal atrophy and hypometabolism and lower functional activity questionnaire scores compared to florbetapir+ participants. Overall, image analysis problems do not appear to be a primary explanation of amyloid negativity. Florbetapir- ADNI patients have a variety of clinical and biomarker features that differ from their florbetapir+ counterparts, suggesting that one or more non-AD etiologies (which may include vascular disease and depression) account for their AD-like phenotype. © 2016 American Academy of Neurology.

  10. Clinical utility of the 6-min walk test for patients with moderate Parkinson's disease.

    PubMed

    Kobayashi, Eiji; Himuro, Nobuaki; Takahashi, Mitsuhiko

    2017-03-01

    The aim of this study was to test the clinical utility of the 6-min walk test (6MWT) for patients with moderate Parkinson's disease (PD) through a determination of factors related to this test. This was a descriptive, observational study carried out at a General Hospital, in-patients. Twenty-four patients with moderate PD were studied. We used Hoehn and Yahr stage ratings (HY stage), Unified Parkinson Disease Rating Scales (UPDRS) motor examination score, 6MWT, Berg Balance scale, Timed 'Up & Go' test (TUG), 10-m walk test (10-m walk speed, 10-m walk steps and cadence), and the energy cost of walking (Ec). The average HY stage was 3.1±0.5 and 6MWT was 340.8±110.9 m. TUG (r=-0.68, P<0.01) and Ec (r=-0.65, P<0.01) were correlated significantly with 6MWT. Multiple regression analysis with age, HY stage, TUG, cadence, and Ec as variables indicated a significant degree of variability in the 6MWT results (R=0.77, P<0.001). The TUG (β=-0.47, P<0.01) and Ec (β=-0.4, P<0.01) were correlated independently with the 6MWT results. In contrast, age, HY stage, and cadence were not independently correlated. The 6MWT is a simple tool for assessing walking capacity for patients with PD. In this study, we confirmed the convergent validity and clinical utility of the 6MWT for patients with moderate PD. The 6MWT is useful for clinical assessment to guide the planning of rehabilitation treatment for patients with moderate PD.

  11. Prediction of Dengue Disease Severity among Pediatric Thai Patients Using Early Clinical Laboratory Indicators

    PubMed Central

    Potts, James A.; Gibbons, Robert V.; Rothman, Alan L.; Srikiatkhachorn, Anon; Thomas, Stephen J.; Supradish, Pra-on; Lemon, Stephenie C.; Libraty, Daniel H.; Green, Sharone; Kalayanarooj, Siripen

    2010-01-01

    Background Dengue virus is endemic in tropical and sub-tropical resource-poor countries. Dengue illness can range from a nonspecific febrile illness to a severe disease, Dengue Shock Syndrome (DSS), in which patients develop circulatory failure. Earlier diagnosis of severe dengue illnesses would have a substantial impact on the allocation of health resources in endemic countries. Methods and Findings We compared clinical laboratory findings collected within 72 hours of fever onset from a prospective cohort children presenting to one of two hospitals (one urban and one rural) in Thailand. Classification and regression tree analysis was used to develop diagnostic algorithms using different categories of dengue disease severity to distinguish between patients at elevated risk of developing a severe dengue illness and those at low risk. A diagnostic algorithm using WBC count, percent monocytes, platelet count, and hematocrit achieved 97% sensitivity to identify patients who went on to develop DSS while correctly excluding 48% of non-severe cases. Addition of an indicator of severe plasma leakage to the WHO definition led to 99% sensitivity using WBC count, percent neutrophils, AST, platelet count, and age. Conclusions This study identified two easily applicable diagnostic algorithms using early clinical indicators obtained within the first 72 hours of illness onset. The algorithms have high sensitivity to distinguish patients at elevated risk of developing severe dengue illness from patients at low risk, which included patients with mild dengue and other non-dengue febrile illnesses. Although these algorithms need to be validated in other populations, this study highlights the potential usefulness of specific clinical indicators early in illness. PMID:20689812

  12. An acenocoumarol dosing algorithm using clinical and pharmacogenetic data in Spanish patients with thromboembolic disease.

    PubMed

    Borobia, Alberto M; Lubomirov, Rubin; Ramírez, Elena; Lorenzo, Alicia; Campos, Armando; Muñoz-Romo, Raul; Fernández-Capitán, Carmen; Frías, Jesús; Carcas, Antonio J

    2012-01-01

    Appropriate dosing of coumarins is difficult to establish, due to significant inter-individual variability in the dose required to obtain stable anticoagulation. Several genetic and other clinical factors have been associated with the coumarins dose, and some pharmacogenetic-guided dosing algorithms for warfarin and acenocoumarol have been developed for mixed populations. We recruited 147 patients with thromboembolic disease who were on stable doses and with an international normalized ratio (INR) between 2 and 3. We ascertained the influence of clinical and genetic variables on the stable acenocoumarol dose by multiple linear regression analysis in a derivation cohort (DC; n = 117) and developed an algorithm for dosing that included clinical factors (age, body mass index and concomitant drugs) and genetic variations of VKORC1, CYP2C9, CYP4F2 and APOE. For purposes of comparison, a model including only clinical data was created. The clinical factors explained 22% of the dose variability, which increased to 60.6% when pharmacogenetic information was included (p<0.001); CYP4F2 and APOE variants explained 4.9% of this variability. The mean absolute error of the predicted acenocoumarol dose (mg/week) obtained with the pharmacogenetic algorithm was 3.63 vs. 5.08 mg/week with the clinical algorithm (95% CI: 0.88 to 2.04). In the testing cohort (n = 30), clinical factors explained a mere 7% of the dose variability, compared to 39% explained by the pharmacogenetic algorithm. Considering a more clinically relevant parameter, the pharmacogenetic algorithm correctly predicted the real stable dose in 59.8% of the cases (DC) vs. only 37.6% predicted by the clinical algorithm (95% CI: 10 to 35). Therefore the number of patients needed to genotype to avoid one over- or under-dosing was estimated to be 5.

  13. An Acenocoumarol Dosing Algorithm Using Clinical and Pharmacogenetic Data in Spanish Patients with Thromboembolic Disease

    PubMed Central

    Borobia, Alberto M.; Lubomirov, Rubin; Ramírez, Elena; Lorenzo, Alicia; Campos, Armando; Muñoz-Romo, Raul; Fernández-Capitán, Carmen; Frías, Jesús; Carcas, Antonio J.

    2012-01-01

    Appropriate dosing of coumarins is difficult to establish, due to significant inter-individual variability in the dose required to obtain stable anticoagulation. Several genetic and other clinical factors have been associated with the coumarins dose, and some pharmacogenetic-guided dosing algorithms for warfarin and acenocoumarol have been developed for mixed populations. We recruited 147 patients with thromboembolic disease who were on stable doses and with an international normalized ratio (INR) between 2 and 3. We ascertained the influence of clinical and genetic variables on the stable acenocoumarol dose by multiple linear regression analysis in a derivation cohort (DC; n = 117) and developed an algorithm for dosing that included clinical factors (age, body mass index and concomitant drugs) and genetic variations of VKORC1, CYP2C9, CYP4F2 and APOE. For purposes of comparison, a model including only clinical data was created. The clinical factors explained 22% of the dose variability, which increased to 60.6% when pharmacogenetic information was included (p<0.001); CYP4F2 and APOE variants explained 4.9% of this variability. The mean absolute error of the predicted acenocoumarol dose (mg/week) obtained with the pharmacogenetic algorithm was 3.63 vs. 5.08 mg/week with the clinical algorithm (95% CI: 0.88 to 2.04). In the testing cohort (n = 30), clinical factors explained a mere 7% of the dose variability, compared to 39% explained by the pharmacogenetic algorithm. Considering a more clinically relevant parameter, the pharmacogenetic algorithm correctly predicted the real stable dose in 59.8% of the cases (DC) vs. only 37.6% predicted by the clinical algorithm (95% CI: 10 to 35). Therefore the number of patients needed to genotype to avoid one over- or under-dosing was estimated to be 5. PMID:22911785

  14. Clinical significance of cytomegalovirus infection in patients with inflammatory bowel disease.

    PubMed

    Garrido, Elena; Carrera, Elisa; Manzano, Rebeca; Lopez-Sanroman, Antonio

    2013-01-07

    Cytomegalovirus (CMV) infection is common in humans. The virus then enters a "latency phase" and can reactivate to different stimuli such as immunosuppression. The clinical significance of CMV infection in inflammatory bowel disease is different in Crohn's disease (CD) and ulcerative colitis (UC). CMV does not interfere in the clinical course of CD. However, CMV reactivation is frequent in severe or steroid-resistant UC. It is not known whether the virus exacerbates the disease or simply appears as a bystander of a severe disease. Different methods are used to diagnose CMV colitis. Diagnosis is classically based on histopathological identification of viral-infected cells or CMV antigens in biopsied tissues using haematoxylin-eosin or immunohistochemistry, other tests on blood or tissue samples are currently being investigated. Polymerase chain reaction performed in colonic mucosa has a high sensitivity and a positive result could be associated with a worse prognosis disease; further studies are needed to determine the most appropriate strategy with positive CMV-DNA in colonic mucosa. Specific endoscopic features have not been described in active UC and CMV infection. CMV colitis is usually treated with ganciclovir for several weeks, there are different opinions about whether or not to stop immunosuppressive therapy. Other antiviral drugs may be used. Multicenter controlled studies would needed to determine which subgroup of UC patients would benefit from early antiviral treatment.

  15. The influence of adrenoreactive preparations on clinical and haemodynamic parameters in patients with ischaemic heart disease.

    PubMed

    Piatak, O A; Yanovskii, G V; Lizogub, V G

    1991-01-01

    The influence of anaprilin, oxprenolol, nonachlazine, prazosin and levodopa on the clinical course and haemodynamics was investigated in 138 patients with ischaemic heart disease in whom selective coronary angiography had revealed an up to 50 percent stenosis of one of coronary arteries. The clinical picture of IHD patients with a high tolerance of physical exercise was characterized by a preponderance of spontaneous angina pectoris accompanied by painless myocardial ischaemia. A certain role in the genesis of these disturbances is played by the relative increase in the activity of alpha 1-adrenoreceptors and decrease in beta-adrenoreceptor activity. Prazosin and nonachlazine in monotherapy reduced the number of anginal attacks and episodes of painless myocardial ischaemia.

  16. Community Health Worker Support for Disadvantaged Patients With Multiple Chronic Diseases: A Randomized Clinical Trial.

    PubMed

    Kangovi, Shreya; Mitra, Nandita; Grande, David; Huo, Hairong; Smith, Robyn A; Long, Judith A

    2017-10-01

    To determine whether a community health worker (CHW) intervention improved outcomes in a low-income population with multiple chronic conditions. We conducted a single-blind, randomized clinical trial in Philadelphia, Pennsylvania (2013-2014). Participants (n = 302) were high-poverty neighborhood residents, uninsured or publicly insured, and diagnosed with 2 or more chronic diseases (diabetes, obesity, tobacco dependence, hypertension). All patients set a disease-management goal. Patients randomly assigned to CHWs also received 6 months of support tailored to their goals and preferences. Support from CHWs (vs goal-setting alone) led to improvements in several chronic diseases (changes in glycosylated hemoglobin: -0.4 vs 0.0; body mass index: -0.3 vs -0.1; cigarettes per day: -5.5 vs -1.3; systolic blood pressure: -1.8 vs -11.2; overall P = .08), self-rated mental health (12-item Short Form survey; 2.3 vs -0.2; P = .008), and quality of care (Consumer Assessment of Healthcare Providers and Systems; 62.9% vs 38%; P < .001), while reducing hospitalization at 1 year by 28% (P = .11). There were no differences in patient activation or self-rated physical health. A standardized CHW intervention improved chronic disease control, mental health, quality of care, and hospitalizations and could be a useful population health management tool for health care systems. clinicaltrials.gov identifier: NCT01900470.

  17. Measuring Quality of Life in Pediatric Patients With Inflammatory Bowel Disease: Psychometric and Clinical Characteristics

    PubMed Central

    Perrin, James M.; Kuhlthau, Karen; Chughtai, Aziz; Romm, Diane; Kirschner, Barbara S.; Ferry, George D.; Cohen, Stanley A.; Gold, Benjamin D.; Heyman, Melvin B.; Baldassano, Robert N.; Winter, Harland S.

    2011-01-01

    Objective To extend development of a pediatric inflammatory bowel disease (IBD) health-related quality of life (HRQoL) measure by determining its factor structure and associations of factors with generic HRQoL measures and clinical variables. Patients and Methods Cross-sectional survey of children and adolescents ages 8 years to 18 years and their parents attending any of 6 US IBD centers, recruited from either existing registry of age-eligible subjects or visits to participating centers. The survey included generic (Pediatric Quality of Life Inventory) and IBD-specific (Impact Questionnaire) quality of life measures, disease activity, and other clinical indicators. We carried out factor analysis of Impact responses, comparing resulting factors with results on the generic HRQoL and the clinical measures. Results We included 220 subjects (161 with Crohn disease and 59 with ulcerative colitis). Initial confirmatory factor analysis did not support the 6 proposed Impact domains. Exploratory factor analysis indicated 4 factors with good to excellent reliability for IBD responses: general well-being and symptoms, emotional functioning, social interactions, and body image. Two items did not load well on any factor. The 4 factors correlated well with the Pediatric Quality of Life Inventory and subscales. Children with higher disease activity scores and other indicators of clinical activity reported lower HRQoL. Conclusions This study provides further characteristics of a HRQoL measure specific to pediatric IBD and indicates ways to score the measure based on the resulting factor structure. The measure correlates appropriately with generic HRQoL measures and clinical severity indicators. PMID:18223375

  18. Association Between Ustekinumab Trough Concentrations and Clinical, Biomarker, and Endoscopic Outcomes in Patients With Crohn's Disease.

    PubMed

    Battat, Robert; Kopylov, Uri; Bessissow, Talat; Bitton, Alain; Cohen, Albert; Jain, Anjali; Martel, Myriam; Seidman, Ernest; Afif, Waqqas

    2017-09-01

    Ustekinumab, an inhibitor of the p40 subunit of interleukins 12 and 23, is an effective treatment for patients with Crohn's disease (CD). Trough concentrations of tumor necrosis factor (TNF) antagonists and presence of anti-drug antibodies are associated with important clinical and endoscopic outcomes. We investigated associations between trough concentrations of ustekinumab and clinical, biomarker, and endoscopic outcomes of real-world patients with CD. We recruited 62 patients with CD who were either refractory or intolerant to TNF antagonists, treated with ustekinumab from April 2014 to September 2015. Patients received 90 mg of ustekinumab subcutaneously at weeks 0, 1, and 2 during induction and 90 mg every 4 or 8 weeks during maintenance. Clinical, biomarker, and endoscopic outcomes, trough concentrations of ustekinumab, and anti-drug antibodies were assessed at both week 10 postinduction therapy and at week 26 or later during maintenance therapy in a prospective longitudinal patient cohort or at week 26 or later during maintenance therapy in a cross-sectional patient cohort. Analysis was performed on data combined from both maintenance cohorts, which had similar outcomes at week 26 or later. A primary analysis determined if ustekinumab drug trough concentrations were associated with clinical response (reduction in Harvey Bradshaw Index score of 3 or greater), clinical remission (Harvey Bradshaw Index score <5), steroid-free clinical remission, biomarker (serum level of C-reactive protein [CRP] or level of fecal calprotectin) reduction, biomarker normalization (serum level of CRP below 5 mg/L or level of fecal calprotectin below 200 μg/g), endoscopic response (simple endoscopic score for CD reduced by 50% or more), or endoscopic remission (simple endoscopic score for CD of 2 or less). At week 26 or beyond, 80.7% of patients had a clinical response, 66.1% were in clinical remission, 50.0% were in steroid-free clinical remission, 58.9% had an endoscopic

  19. Clinical Factors and Disease Course Related to Diagnostic Delay in Korean Crohn's Disease Patients: Results from the CONNECT Study.

    PubMed

    Moon, Chang Mo; Jung, Sung-Ae; Kim, Seong-Eun; Song, Hyun Joo; Jung, Yunho; Ye, Byong Duk; Cheon, Jae Hee; Kim, You Sun; Kim, Young-Ho; Kim, Joo Sung; Han, Dong Soo

    2015-01-01

    Diagnostic delay frequently occurs in Crohn's disease (CD) patients because of diagnostic limitations. However, diagnostic delay and its related factors remain poorly defined. Therefore, we aimed to identify the predictors associated with diagnostic delay and to evaluate the impact of diagnostic delay on clinical course in a Korean CD patient cohort. We performed a multicenter retrospective analysis of 1,047 CD patients registered in the Crohn's Disease Clinical Network and Cohort study in Korea. The mean interval of diagnostic delay was 16.0 ± 33.1 months. Multivariate analysis showed that older age at diagnosis (≥40 years) (p = 0.014), concomitant upper gastrointestinal (UGI) disease (p = 0.012) and penetrating disease behavior at diagnosis (p = 0.001) were positively associated with long diagnostic delay (≥18 months). During the longitudinal follow-up, long diagnostic delay was independently predictive of further development of intestinal stenosis (hazard ratio [HR], 1.43; 95% confidence interval [CI], 1.07-1.93; p = 0.017), internal fistulas (HR, 1.62; 95% CI, 1.12-2.33; p = 0.011), and perianal fistulas (HR, 1.38; 95% CI, 1.06-1.80; p = 0.016). However, as for the risk of abscess formation, bowel perforation, and CD-related abdominal surgery, no significant association with diagnostic delay was observed. Older age at diagnosis, UGI involvement, and penetrating behavior are associated with long diagnostic delay in Korean CD patients. Moreover, diagnostic delay is associated with an increased risk of CD-related complications such as intestinal stenosis, internal fistulas, and perianal fistulas.

  20. Effect of Creatine Monohydrate on Clinical Progression in Patients With Parkinson Disease

    PubMed Central

    2015-01-01

    IMPORTANCE There are no treatments available to slow or prevent the progression of Parkinson disease, despite its global prevalence and significant health care burden. The National Institute of Neurological Disorders and Stroke Exploratory Trials in Parkinson Disease program was established to promote discovery of potential therapies. OBJECTIVE To determine whether creatine monohydrate was more effective than placebo in slowing long-term clinical decline in participants with Parkinson disease. DESIGN, SETTING, AND PATIENTS The Long-term Study 1, a multicenter, double-blind, parallel-group, placebo-controlled, 1:1 randomized efficacy trial. Participants were recruited from 45 investigative sites in the United States and Canada and included 1741 men and women with early (within 5 years of diagnosis) and treated (receiving dopaminergic therapy) Parkinson disease. Participants were enrolled from March 2007 to May 2010 and followed up until September 2013. INTERVENTIONS Participants were randomized to placebo or creatine (10 g/d) monohydrate for a minimum of 5 years (maximum follow-up, 8 years). MAIN OUTCOMES AND MEASURES The primary outcome measure was a difference in clinical decline from baseline to 5-year follow-up, compared between the 2 treatment groups using a global statistical test. Clinical status was defined by 5 outcome measures: Modified Rankin Scale, Symbol Digit Modalities Test, PDQ-39 Summary Index, Schwab and England Activities of Daily Living scale, and ambulatory capacity. All outcomes were coded such that higher scores indicated worse outcomes and were analyzed by a global statistical test. Higher summed ranks (range, 5–4775) indicate worse outcomes. RESULTS The trial was terminated early for futility based on results of a planned interim analysis of participants enrolled at least 5 years prior to the date of the analysis (n = 955). The median follow-up time was 4 years. Of the 955 participants, the mean of the summed ranks for placebo was 2360 (95

  1. [The clinical characteristics of 346 patients with IgG4-related disease].

    PubMed

    Zhang, P P; Zhao, J Z; Wang, M; Feng, R E; Liu, X W; Lai, X M; Li, X J; Zeng, J G; Shi, H J; Zhu, H D; Xue, W; Zhang, H; Chen, Y Y; Fei, L Y; Peng, X F; Zeng, F C; Zhang, Y M; Zhang, W

    2017-09-01

    Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66.5%) and 116 females (33.5%). The mean age of disease onset was (53.8±14.2) years old. The mostly common involved organs were lymph nodes (56.4%) and submandibular glands (52.6%). Other affected organs and manifestations included: swelling of the lacrimal glands (46.5%), autoimmune pancreatitis (38.4%), pulmonary involvement (28.0%), sclerosing cholangitis (25.4%), naso-sinusitis (23.4%), parotid gland swelling (21.7%), retroperitoneal fibrosis (19.9%), large arteries involvement (9.5%), kidney involvement (obstructive nephropathy caused by retroperitoneal fibrosis was excluded) (6.9%), skin lesions (6.4%). Rare features consisted of thyroid glands, pituitary glands, gastrointestinal tract, pachymeningitis, pericardium, sclerosing mediastinitis and orchitis. The majority of patients had multi-organ involvement, such as 74.3% patients with 3 and more, 18.2% and 7.5% patients with 2 and single organ involvement respectively. The average IgG4-RD responder index (IgG4-RD RI) was 13.21±5.70. History of allergy was found in 172 (49.7%) patients. As to the laboratory tests, elevated serum IgG4 levels were confirmed in 285 (94.1%) patients, which was positively correlated with IgG4-RD RI. There were 33.5% patients receiving monotherapy of glucocorticoid, 52.6% treated with glucocorticoids combined with immunosuppressive agents, 4.9% patients with immunosuppressant only, and 9.0% patients with mild disease not receiving medication. The majority

  2. Heartburn during sleep: a clinical marker of gastro-oesophageal reflux disease in morbidly obese patients.

    PubMed

    Fornari, F; Madalosso, C A S; Callegari-Jacques, S M; Gurski, R R

    2009-02-01

    Gastro-oesophageal reflux disease (GORD) and morbid obesity are entities with increasing prevalence. New clinical strategies are cornerstones for their management. The aim of this study was to assess the prevalence of heartburn during sleep (HDS) and whether this symptom predicts the presence of objective GORD parameters and increased heartburn perception in morbidly obese patients. Ninety-one consecutive morbidly obese patients underwent clinical evaluation, upper gastrointestinal endoscopy and oesophageal pH monitoring. HDS was characterized when patients replied positively to the question, 'Does heartburn wake you from sleep?'. A General Score for Heartburn (GSH) ranging between 0 and 5 was assessed with the question 'How bad is your heartburn?'. HDS was reported by 33 patients (36%). More patients with HDS had abnormal acid contact time or reflux oesophagitis than patients without HDS (94%vs 57%, P < 0.001). HDS had a positive predictive value of 94% (0.95 CI 82-98), sensitivity of 48% (0.95 CI 37-60%) and specificity of 93% (0.95 CI 77-98%) for detection of GORD. A higher proportion of patients with HDS perceived heartburn preceded by acid reflux in diurnal (39%vs 9%; P < 0.001) periods during pH-metry. HDS patients showed higher GSH (2.4 +/- 0.5 vs 1.7 +/- 0.4; P < 0.0001) compared with patients who denied HDS but reported diurnal heartburn. HDS occurs in a significant minority of patients with morbid obesity and has high positive predictive value for GORD. Symptomatic reflux during the sleep seems to be a marker of increased heartburn perception in this population.

  3. Clinical and genetic characteristics in patients with Huntington's disease from China.

    PubMed

    Yang, Jing; Chen, Ke; Wei, Qianqian; Chen, Yongping; Cao, Bei; Burgunder, Jean-Marc; Shang, Hui-Fang

    2016-10-01

    Huntington's disease (HD) is a neurodegenerative disease caused by the expansion of unstable CAG repeats in the HTT gene. There are scarce data about HD in China. Fifty-eight HD patients were consecutively recruited and assessed using the Unified HD Rating Scale (UHDRS) motor section and UHDRS behaviour assessment (UHDRS-b). Genetic analyses were also conducted. Thirty-three women and Twenty -five men were diagnosed with a mean age of 46.1 ± 11.2 years and a mean number of CAG triplet repeats 44.6 ± 4.4. CAG triplet repeat number was negatively correlated with age at onset, and positively correlated with UHDRS-b total score, and its subdomains including depressed mood, low self-esteem, anxiety and irritability. On the other hand, negative correlations were identified between age at onset and UHDRS-b total score, and its subdomains include low self-esteem, anxiety, suicidal thought, irritability and apathy. Disease durations were correlated with UHDRS motor scores and anxiety domain of UHDRS-b. This is the largest series of Chinese HD patients with demographic, clinical and genetic data confirms the demographic features of Chinese HD patients are comparable to those in other ethnic backgrounds. CAG triplet repeat number may also predict the severity of behaviour problems in HD patients besides its predication for age of onset.

  4. PREDICTORS OF PHYSICIAN REFERRAL FOR PATIENT RECRUITMENT TO ALZHEIMER DISEASE CLINICAL TRIALS

    PubMed Central

    Galvin, James E.; Meuser, Thomas M.; Boise, Linda; Connell, Cathleen M

    2009-01-01

    Background Inadequate recruitment into Alzheimer disease (AD) clinical trials is an important threat to the validity and generalizability of the studies. The majority of dementia patients are first evaluated by community-based physicians; however, physician perceptions of clinical research are largely unknown. Methods A survey was distributed to 3,123 physicians in three states; 370 were returned. Survey items assessed attitudes, perceived benefits of and barriers to referral to clinical research and physicians use of the internet for medical information. Results The mean age of the respondents was 50.6 ± 10.8y; 70% were male, 78% Caucasian, 61% were primary care providers; 63% used the internet ≥3 times/week. No demographic or medical specialty differences existed between those who were likely (n=193) and unlikely (n=162) to refer patients to clinical trials. Differences were discovered in perceived benefits reported by physicians who were more likely to refer, while differences in perceived barriers existed in primary care compared with specialists. Referral to clinical trials is predicted by close proximity to a research center (OR:4.0,95%CI:1.1–15.6) and availability of internet information regarding diagnostic evaluation (OR:2.3,95%CI:1.1–4.7). Primary barriers included concerns about exposure of patients to uncomfortable procedures (OR:4.7,95%CI:1.2–18.7) and lack of time to discuss research participation (OR:6.8,95%CI:1.4–32.3). Conclusions Proximity to a research center and availability of diagnostic clinical tools are strong predictors of clinical trial referral. Concern over risks to patients and lack of time are strong barriers. These results suggest that dementia outreach education targeted to physicians should emphasize the importance of clinical trials with a focus on discussing research participation in a time-efficient manner and increasing awareness of risk reduction and the safety of research protocols. Providing easy access to up

  5. Clinical and Molecular Epidemiology of Haemophilus influenzae Causing Invasive Disease in Adult Patients

    PubMed Central

    Puig, Carmen; Grau, Imma; Tubau, Fe; Calatayud, Laura; Pallares, Roman; Liñares, Josefina

    2014-01-01

    Objectives The epidemiology of invasive Haemophilus influenzae (Hi) has changed since the introduction of the Hi type b (Hib) vaccine. The aim of this study was to analyze the clinical and molecular epidemiology of Hi invasive disease in adults. Methods Clinical data of the 82 patients with Hi invasive infections were analyzed. Antimicrobial susceptibility, serotyping, and genotyping were studied (2008–2013). Results Men accounted for 63.4% of patients (whose mean age was 64.3 years). The most frequent comorbidities were immunosuppressive therapy (34.1%), malignancy (31.7%), diabetes, and COPD (both 22%). The 30-day mortality rate was 20.7%. The majority of the strains (84.3%) were nontypeable (NTHi) and serotype f was the most prevalent serotype in the capsulated strains. The highest antimicrobial resistance was for cotrimoxazole (27.1%) and ampicillin (14.3%). Twenty-three isolates (32.9%) had amino acid changes in the PBP3 involved in resistance. Capsulated strains were clonal and belonged to clonal complexes 6 (serotype b), 124 (serotype f), and 18 (serotype e), whereas NTHi were genetically diverse. Conclusions Invasive Hi disease occurred mainly in elderly and those with underlying conditions, and it was associated with a high mortality rate. NTHi were the most common cause of invasive disease and showed high genetic diversity. PMID:25379704

  6. Chronic granulomatous disease in Latin American patients: clinical spectrum and molecular genetics.

    PubMed

    Agudelo-Flórez, Piedad; Prando-Andrade, Carolina Cardoso; López, Juan Alvaro; Costa-Carvalho, Beatriz Tavares; Quezada, Arnoldo; Espinosa, Francisco Jose; de Souza Paiva, Maria Aparecida; Roxo, Persio; Grumach, Anete; Jacob, Cristina Abe; Carneiro-Sampaio, Magda Maria Salles; Newburger, Peter E; Condino-Neto, Antonio

    2006-02-01

    Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by early onset of recurrent and severe infections. The molecular defects causing CGD are heterogeneous and lead to absence, low expression, or malfunctioning of one of the phagocyte NADPH oxidase components. The aim of this study was to analyze the clinical features and to investigate the molecular genetic defects of Latin American patients with CGD. The study included 14 patients. The diagnosis was based on a history of recurrent severe infections, impaired respiratory burst, and the demonstration of an underlying mutation by single strand conformation polymorphism (SSCP) or RT-PCR analysis, followed by genomic DNA or cDNA sequencing. Seven unrelated patients were found to have the X-linked form of CGD (X-CGD). Heterogeneous mutations affected the CYBB gene: two insertions, one substitution, and four splice site defects; two of them are novel. Seven patients presented with one of the autosomal recessive forms of CGD (A47-CGD); all had the most common mutation, a DeltaGT deletion in exon 2 of the NCF1 gene. Pneumonia was the most frequent clinical feature, followed by pyoderma, sinusitis, otitis, and liver abscess. Patients with X-CGD were more likely to have initial infections before age 2 years and to have inflammatory obstructive granulomas later. None of the patients had severe adverse reactions to BCG immunization. X-CGD patients from Latin America showed a high degree of molecular heterogeneity, including two novel mutations. Their clinical characteristics included early onset of infections and eventual obstructive granulomas. A47-CGD represented 50% of the reported cases, a higher prevalence than reported in other series.

  7. Clinical applications of stereotactic radiation therapy for oligometastatic cancer patients: a disease-oriented approach

    PubMed Central

    Ricardi, Umberto; Badellino, Serena; Filippi, Andrea Riccardo

    2016-01-01

    Oligometastases from solid tumors are currently recognized as a distinct clinical entity, corresponding to an intermediate state between local and widespread disease. It has been suggested that local ablative therapies (including surgery, radiofrequency ablation and radiation therapy) play an important role in this setting, in combination or not with systemic therapies, particularly in delaying disease progression and hopefully in increasing the median survival time. Stereotactic body radiation therapy (SBRT) rapidly emerged in recent years as one of the most effective and less toxic local treatment modalities for lung, liver, adrenal, brain and bone metastases. The aim of this review was to focus on its clinical role for oligometastatic disease in four major cancer subtypes: lung, breast, colorectal and prostate. On the basis of the available evidence, SBRT is able to provide high rates of local tumor control without significant toxicity. Its global impact on survival is uncertain; however, in specific subpopulations of oligometastatic patients there is a trend towards a significant improvement in progression-free and overall survival rates; these important data might be used as a platform for clinical decision-making and establish the basis for the current and future prospective trials investigating its role with or without systemic treatments. PMID:26962198

  8. Clinical determinants and prognostic significance of the electrocardiographic strain pattern in chronic kidney disease patients.

    PubMed

    Cordeiro, Antonio C; Moraes, Aline A I; Cerutti, Virginia; França, Faustino; Quiroga, Borja; Amodeo, Celso; Picotti, Juliano C; Dutra, Lucas V; Rodrigues, Gabriel D; Amparo, Fernanda C; Lindholm, Bengt; Carrero, Juan Jesús

    2014-05-01

    The electrocardiographic (ECG) strain pattern (Strain) is a marker of left ventricular hypertrophy (LVH) severity that provides additional prognostic information beyond echocardiography (ECHO) in the community level. We sought to evaluate its clinical determinants and prognostic usefulness in chronic kidney disease (CKD) patients. We evaluated 284 non-dialysis-dependent patients with CKD stages 3 to 5 (mean age, 61 years [interquartile range, 53-67 years]; 62% men). Patients were followed for 23 months (range, 13-32 months) for cardiovascular (CV) events and/or death. Strain patients (n = 37; 13%) were using more antihypertensive drugs, had higher prevalence of peripheral vascular disease and smoking, and higher levels of C-reactive protein, cardiac troponin, and brain natriuretic peptide (BNP). The independent predictors of Strain were: left ventricular mass index (LVMI), BNP, and smoking. During follow-up, there were 44 cardiovascular events (fatal and non-fatal) and 22 non-CV deaths; and Strain was associated with a worse prognosis independently of LVMI. Adding Strain to a prognostic model of LVMI improved in 15% the risk discrimination for the composite endpoint and in 12% for the CV events. Strain associates with CV risk factors and adds prognostic information over and above that of ECHO-assessed LVMI. Its routine screening may allow early identification of high risk CKD patients.

  9. Clinical challenges of thyroid eye disease in HIV-positive patients on highly active antiretroviral therapy.

    PubMed

    Edmunds, Matthew R; Mellington, Faye; Ford, Rebecca L; Torlinska, Barbara; Manavi, Kaveh; Boelaert, Kristien

    2015-03-01

    Graves' disease (GD) as an immune reconstitution inflammatory syndrome during highly active antiretroviral therapy (HAART) for HIV has previously been reported. However, clinical challenges associated with HIV in the context of thyroid eye disease (TED) are not as well-characterized. To determine the frequency of coexisting HIV and TED, describe TED presentation and course in the context of HIV, and evaluate management difficulties as well as potential solutions. Cross-sectional study of all patients with coexisting GD and HIV at University Hospitals Birmingham (2003-2014). Retrospective case note review to identify TED with particular reference to HAART regimen, CD4+ T-cell count, HIV viral load, and TED activity and severity. Of 783 subjects with GD and 1186 with HIV, 11 were identified with both GD and HIV. Of these, three had clinical features of TED; each was of Afro-Caribbean origin, was in their fourth decade, and initially presented with undetectable CD4 T cells and high HIV viral loads. All went on to develop GD >3 years after commencing HAART, with normal CD4 count and undetectable viral load at the time of GD diagnosis. The full spectrum of TED was represented, with two subjects requiring orbital decompression surgery. TED in the context of HIV is uncommon. Many challenges exist in such patients, particularly HAART drug interactions with antithyroid and immunosuppressant medications. To better understand TED in HIV and to counsel patients with this copathology most effectively, future multicenter surveillance is required.

  10. Home-based pulmonary rehabilitation in patients with chronic obstructive pulmonary disease: a randomized clinical trial

    PubMed Central

    Dias, Fernanda Dultra; Sampaio, Luciana Maria Malosá; da Silva, Graziela Alves; Gomes, Évelim LF Dantas; do Nascimento, Eloisa Sanches Pereira; Alves, Vera Lucia Santos; Stirbulov, Roberto; Costa, Dirceu

    2013-01-01

    Introduction Pulmonary rehabilitation (PR) is a multidisciplinary program of care for patients with chronic obstructive pulmonary disease (COPD) with the goal of improving the functional capacity and quality of life, as well as maintaining the clinical stability of COPD sufferers. However, not all patients are available for such a program despite discomfort with their condition. The aim of this study was to evaluate the effects of a home-based PR (HBPR) program on functional ability, quality of life, and respiratory muscle strength and endurance. Patients and methods Patients with COPD according to the Global Initiative of Chronic Obstructive Lung Disease were randomized (double-blind) into two groups. One group performed a protocol at home with aerobic and muscle strength exercises and was called the intervention group; the other group received only instructions to perform breathing and stretching exercises, characterizing it as the control group (CG). We assessed the following variables at baseline and 2 months: exercise tolerance (incremental shuttle walk test and upper limb test), respiratory muscle (strength and endurance test), and health-related quality of life (Airways Questionnaire 20). Results There were no significant changes after the intervention in either of the two groups in exercise tolerance and quality of life. However, the intervention group had improved respiratory endurance compared with the CG, while the CG presented a decrease in the load sustained by the respiratory muscles after the HBPR. Conclusion A program of HBPR with biweekly supervision (although not enough to provide significant improvements in physical capacity or quality of life) played an important role in maintaining the stability of the clinical features of patients with COPD; the patients had no worsening of symptoms during the intervention period according to the daily log. PMID:24235824

  11. Factors that predict poor clinical course among patients hospitalized with pelvic inflammatory disease.

    PubMed

    Terao, Miyoko; Koga, Kaori; Fujimoto, Akihisa; Wada-Hiraike, Osamu; Osuga, Yutaka; Yano, Tetsu; Kozuma, Shiro

    2014-02-01

    The aim of this study was to identify factors that can predict clinical course among patients hospitalized with pelvic inflammatory disease (PID). Ninety-three patients who needed hospitalization with a diagnosis of PID were retrospectively studied. Patients who were discharged within 7 days by conservative treatment were defined as favorable course cases (n = 44). Patients who needed more than 7 days of hospitalization and/or surgery were defined as poor course cases (n = 49). Twenty variables were evaluated by univariate and logistic regression analysis: age, history of pregnancy/delivery, gynecological open/laparoscopic surgery, PID, oral contraceptives/intrauterine device use and intrauterine operation before onset, body temperature, signs of peritoneal irritation, vomiting/diarrhea, abnormal vaginal discharge, endometriosis/fibroid/adenomyosis/any cystic lesion detected by ultrasonography, white blood cell counts/C-reactive protein (CRP) levels . The cut-off value was calculated by receiver-operator curve (ROC) analysis. Factors associated with poor clinical course were advanced age (P < 0.01), history of gynecological open surgery (P < 0.05), any cystic lesion detected by ultrasonography (P < 0.05) and high CRP levels (P < 0.05). High CRP levels and intrauterine operation before onset were independently associated with poor clinical course. The cut-off value for CRP was 4.4 mg/dL. This study identified variables that can predict poor clinical course of PID. These results can assist gynecologists with identifying patients at risk and optimizing the choice of management. © 2013 The Authors. Journal of Obstetrics and Gynaecology Research © 2013 Japan Society of Obstetrics and Gynecology.

  12. [Analysis of clinical features of 6 patients with infantile type glycogen storage disease type II].

    PubMed

    Ding, Juan; Huang, Yu; Yang, Haipo; Zhang, Qingyou; Hou, Xinlin; Liu, Xueqin; Yang, Yanling; Xiong, Hui

    2015-06-01

    To summarize clinical features and diagnosis of Chinese infantile patients with glycogen storage disease type II (GSD II). Six infant patients with GSD II diagnosed from January 2012 to June 2014 in the Department of Pediatrics, Peking University First Hospital were enrolled into this study. Clinical information of the 6 patients, including clinical manifestation, blood biochemistry, chest X-ray, echocardiogram, electrocardiogram, acid alpha-glucosidase (GAA) activity and GAA gene mutation analysis by direct sequencing of polymerase chain reaction (PCR) product were reviewed. Of the 6 patients, five were female and one was male, five of whom were classic infantile type while the other one was atypical. The age of onset ranged from birth to 3-month-old. All patients had varying degrees of generalized muscle weakness, hypotonia and development retardation or retrogression. Other common findings were feeding difficulties in two patients, tongue weakness in two patients, respiratory distress in four patients, macroglossia in one patient, and hepatomegaly in two patients. Left ventricular hypertrophy and cardiomegaly were obvious in all the six patients. All six patients were found to have a enlarged heart in physical examination, and three patients who underwent a chest X-ray examination had an enlarged heart shadow. Four patients who had an echocardiography were found to have myocardial hypertrophy. The electrocardiogram in three patients showed short PR intervals and high voltage. The creatine kinase (CK) levels were three to seven times elevated. The mildest elevated CK was 441 IU/L, and the highest CK level was 1 238 U/L. Assay of GAA enzyme activity in whole blood showed significantly reduced activity (1.3 nmol/ (spot·d) to 2 nmol/(spot·d)) in the patients tested. Gene sequencing in 4 patients showed 8 pathogenic mutations, including 6 missense mutations, one nonsense mutation and one frameshift mutation. The missense mutations were c.998C > A (p.Thr333Lys), c

  13. Reporting disease activity in clinical trials of patients with rheumatoid arthritis: EULAR/ACR collaborative recommendations.

    PubMed

    Aletaha, D; Landewe, R; Karonitsch, T; Bathon, J; Boers, M; Bombardier, C; Bombardieri, S; Choi, H; Combe, B; Dougados, M; Emery, P; Gomez-Reino, J; Keystone, E; Koch, G; Kvien, T K; Martin-Mola, E; Matucci-Cerinic, M; Michaud, K; O'Dell, J; Paulus, H; Pincus, T; Richards, P; Simon, L; Siegel, J; Smolen, J S; Sokka, T; Strand, V; Tugwell, P; van der Heijde, D; van Riel, P; Vlad, S; van Vollenhoven, R; Ward, M; Weinblatt, M; Wells, G; White, B; Wolfe, F; Zhang, B; Zink, A; Felson, D

    2008-10-15

    To make recommendations on how to report disease activity in clinical trials of rheumatoid arthritis (RA) endorsed by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). The project followed the EULAR standardized operating procedures, which use a three-step approach: 1) expert-based definition of relevant research questions (November 2006); 2) systematic literature search (November 2006 to May 2007); and 3) expert consensus on recommendations based on the literature search results (May 2007). In addition, since this is the first joint EULAR/ACR publication on recommendations, an extra step included a meeting with an ACR panel to approve the recommendations elaborated by the expert group (August 2007). Eleven relevant questions were identified for the literature search. Based on the evidence from the literature, the expert panel recommended that each trial should report the following items: 1) disease activity response and disease activity states; 2) appropriate descriptive statistics of the baseline, the endpoints and change of the single variables included in the core set; 3) baseline disease activity levels (in general); 4) the percentage of patients achieving a low disease activity state and remission; 5) time to onset of the primary outcome; 6) sustainability of the primary outcome; 7) fatigue. These recommendations endorsed by EULAR and ACR will help harmonize the presentations of results from clinical trials. Adherence to these recommendations will provide the readership of clinical trials with more details of important outcomes, while the higher level of homogeneity may facilitate the comparison of outcomes across different trials and pooling of trial results, such as in meta-analyses.

  14. Clinical outcomes of erythropoietin use in heart failure patients with anemia of chronic kidney disease.

    PubMed

    Jackevicius, Cynthia A; Fan, Cindy Shutieng; Warner, Alberta

    2014-05-01

    Anemia and chronic kidney disease are common disorders in heart failure (HF) patients and are associated with increased morbidity and mortality. This study assessed clinical outcomes associated with erythropoietin (EPO) treatment in this cardiorenal anemia syndrome (CRAS) population. This was a retrospective cohort study of Veterans Affairs patients with CRAS from January 2003 to December 2006. The primary outcome was a composite of death, acute coronary syndrome (ACS), HF, and stroke. Multiple Cox regression modeling was used to evaluate the outcome in patients prescribed (n = 213) and not prescribed EPO (n = 1845). Adjusted incidence of mortality was statistically significantly higher in EPO than in non-EPO users (33.8% vs 19.7%; hazard ratio 1.40, 95% confidence interval 1.06-1.85; P = .02). The unadjusted composite of cardiovascular events/death was higher in the EPO group, but not statistically significant when adjusted for confounders (P = .12). Crude ACS events were documented in 18.8% and 10.8% patients (P = .001), and stroke events occurred in 22.5% and 18.3% patients (P = .14) in EPO and non-EPO groups, respectively. We found that in CRAS patients, EPO use was associated with increased risk of mortality and a trend toward increased cardiovascular events. Therefore, clinicians considering EPO use in CRAS patients should assess whether any potential benefits outweigh the risks of use. Copyright © 2014 Elsevier Inc. All rights reserved.

  15. Sarcoidosis in patients with mixed connective tissue disease: clinical, genetic, serological and histological observations.

    PubMed

    Szodoray, Peter; Szollosi, Zoltan; Gyimesi, Edit; Takacs, Istvan; Mekkel, Gabriella; Vegh, Judit; Szilagyi, Anna; Zeher, Margit; Szegedi, Gyula; Bodolay, Edit

    2008-06-01

    The objective of this study was to investigate how the development of sarcoidosis influences the disease course of mixed connective tissue disease (MCTD). The cellular composition of MCTD-associated sarcoidosis granulomas was evaluated and also the disease-accompanying T-cell activation and alterations of the serum cytokine levels were measured before and after the therapy. The HLA-DR specific alleles were also assessed. We present two cases with MCTD coexisting sarcoidosis. Serum concentrations of Th1 and Th2 cytokines were assessed by ELISA. Peripheral blood CD3+ total T-cell numbers, CD4+ and CD8+ T-cell subset were determined by flow cytometry. Furthermore, hematoxylin-eosin staining and immunhistochemistry were performed for histological assessment. HLA-DR specific alleles were determined by using PCR-SSP. Elevated number of activated T-cells and high Th1 cytokine levels were detected, mainly IFN-gamma and TNF-alpha. Histologically, CD4+ and CD8+ T-cells were present in the sarcoidosis infiltrations. The haplotypes were to some extent dissimilar from the HLA-DR genotype from patients with MCTD, or sarcoidosis alone. Sarcoidosis enhances the activation of MCTD, based on the laboratory and clinical findings. Our results show that the inflammation is mainly in the effector phase, while granuloma formation is characteristic of the resolution phase of the disease. The assessment of the cytokine network in sarcoidosis-associated MCTD enables us to select the most effective, individualized therapy protocol for these patients.

  16. Gorham's disease: clinical case.

    PubMed

    Sá, Pedro; Marques, Pedro; Oliveira, Carolina; Rodrigues, André Sá; Amorim, Nelson; Pinto, Rui

    2015-01-01

    Gorham's disease, also known as idiopathic massive osteolysis, is a rare pathological condition characterized by vascular proliferation that results in destruction and reabsorption of the bone matrix, of unknown etiology. It was first described by Jackson in 1838, but it was Gorham and Stout, in 1955, who defined this disease as a specific entity. It has variable clinical presentation and generally has progressive behavior. Controversy continues regarding the treatment and there is no standard treatment. This pathological condition generally presents a favorable prognosis. Here, a case of Gorham's disease with involvement of the left hip is presented, in a male patient without relevant antecedents.

  17. Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14

    NASA Astrophysics Data System (ADS)

    Borjali, Davood

    Title: Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14 Kawasaki disease(KD) is a kind of vasculitis diagnosed by clinical manifestation and it caused acquired heart disease in children because of coronary arteries involvement. Method: patient divided to three group of American Japanese and incomplete and also study in two group according to fever days and then clinical features and laboratory data were checked. Result: A total of 150 patients were enrolled during the study period. number of patients with incomplete Kawasaki disease was 128 american group was 28 and Japanese was 4 patients, the most prevalent symptom was scaling of extremities(61 bladder most seen in group with fever more than five days. Keyword: Kawasaki , epidemiology , criteria

  18. Clinical value of detection of immune index and inflammatory reaction changes in patients with autoimmune disease.

    PubMed

    Zhang, X J; Zhou, H Y; Li, Y

    2016-09-23

    Previous studies have shown a close correlation between the generation of B cell autoantibodies and imbalances in T lymphocyte subpopulations and the occurrence of disease. In this study, we have analyzed the effects of abnormal expression of CD4+CD25+-regulatory T cells, T lymphocyte subpopulations, immunoglobulins, complement factors, inflammatory factors, and adhesion molecules in the peripheral blood on the occurrence and development of autoimmune disease. Eighty patients with autoimmune disease were randomly (equally) divided into active-stage and stable-stage disease groups (according to pre-defined criteria). Fifty healthy people were recruited to the control group. The above-mentioned indices were detected by flow cytometry, immunity transmission turbidity, and enzyme-linked immunosorbent assay. We observed an obvious decrease in the CD4+CD25+- regulatory T cell, CD4+ cell, CD4+/CD8+ cell, NK cell, C3, and C4 expression in all three groups; however, this decrease was statistically significant in the active-stage group (P < 0.05). Alternately, we observed a significant increase in the expression of CD8+ cells, immunoglobulin (Ig) A, IgG, IgM, tumor necrosis factor-α, interleukin (IL)-10, IL-17, interferon-g, intercellular adhesion molecule-1, vascular cell adhesion molecule-1, and E-selectin expression in the active-stage group (P < 0.05). Therefore, inflammatory reactions and immune dysfunction occurs during the active-stage of autoimmune disease, and detection of the immune indices and inflammatory and adhesion factors could help evaluate the immune stage in these patients, providing an experimental basis for the determination of disease progression and clinical treatment.

  19. Clinical and humanistic outcomes in patients with gastroesophageal reflux disease converted from omeprazole to lansoprazole.

    PubMed

    Nelson, W W; Vermeulen, L C; Geurkink, E A; Ehlert, D A; Reichelderfer, M

    2000-09-11

    Omeprazole and lansoprazole are 2 proton pump inhibitors (PPIs) currently available in the United States. Both PPIs are approved for the treatment of gastroesophageal reflux disease (GERD) and are commonly converted in therapeutic interchange programs. To measure clinical and humanistic outcomes in patients with GERD converted from treatment with omeprazole to treatment with lansoprazole through a managed care plan policy. Patients with heartburn or GERD receiving omeprazole covered by a local health plan were surveyed by telephone. Data collected included symptom frequency, severity, over-the-counter heartburn preparation use, diet, lifestyle, and overall satisfaction. Patients were then converted to therapy with lansoprazole and again interviewed after at least 30-day use of the new PPI. Demographic data were obtained from the health plan database for analysis. A total of 105 patients completed both telephone surveys. After the conversion, 37% of the patients experienced more frequent symptoms while awake. Symptom severity score was significantly higher (more severe) after conversion (mean score of 1.34 vs 2.26). Thirty-three percent of study patients consumed more over-the-counter heartburn preparations, and 13% changed their diet more frequently due to heartburn symptoms after conversion. Overall patient satisfaction score decreased significantly (less satisfied) after conversion (mean score of 9.0 vs 7.2). There were no significant differences in alcohol and tobacco consumption before and after conversion, while patients consumed significantly less caffeine after conversion. After the PPI therapeutic interchange from omeprazole to lansoprazole, patients with GERD or heartburn previously stabilized while receiving omeprazole experienced more severe symptoms and expressed decreased patient satisfaction. These results suggest a need to monitor symptoms after similar interchange programs.

  20. Clinical significance of screening for subclinical Cushing's disease in patients with pituitary tumors.

    PubMed

    Tamada, Daisuke; Kitamura, Tetsuhiro; Otsuki, Michio; Oshino, Satoru; Saitoh, Youichi; Shimomura, Iichiro

    2016-01-01

    Cushing's syndrome (CS) is a clinical state caused by chronic excess of glucocorticoid, and results in hypertension, impaired glucose tolerance, and dyslipidemia. Recently, a mild state of pituitary CS without typical Cushingoid appearance (subclinical Cushing's disease; SCD) has been identified. However, the true prevalence of SCD and its effect on metabolic disorders remain obscure. The aim of this prospective study was to determine the prevalence of SCD according to the guideline proposed by the working group of the Japanese Ministry of Health, Welfare and Labor, and to assess the outcome of surgery on metabolic disorders. The prevalence of SCD was investigated in 105 consecutive patients diagnosed with pituitary adenomas by MRI. ACTH-dependent hypercortisolism was diagnosed based on the results of the 0.5 mg dexamethasone suppression test (serum cortisol >3.0 μg/dL) plus one positive finding of the following two tests: midnight serum cortisol level >5.0 μg/dL or ACTH increase >50% after 1-deamino-5-D-arginine vasopressin (DDAVP) challenge. The final diagnosis of SCD was established by positive staining for ACTH in surgically-excised pituitary adenoma. Three patients (4.8%) were diagnosed with SCD among 62 patients with pituitary adenoma. Transsphenoidal adenomectomy partially resulted in improvement of blood pressure and glucose metabolism in SCD patients. Our results emphasize the importance of SCD screening in patients with pituitary tumors, especially in those patients with metabolic disorders.

  1. Clinical and economic aspects of sevelamer therapy in end-stage renal disease patients.

    PubMed

    Ossareh, Shahrzad

    2014-01-01

    Phosphate control is still a great challenge in chronic kidney disease (CKD), and in spite of the great improvements in dialysis techniques, achievement of the goals for mineral metabolism control is still far from ideal. Aluminum hydroxide has been largely abandoned due to the high risk of aluminum toxicity, while the use of calcium-based phosphate binders may cause hypercalcemia, overzealous parathyroid suppression, and extraskeletal calcification. Sevelamer hydrochloride has been introduced as an efficient medication for phosphate control, with a lower risk of hypercalcemia and parathyroid suppression. Various clinical trials have compared the risk of vascular calcification between sevelamer and calcium salts with inconsistent results. In spite of these inconsistencies, the Kidney Disease Outcomes Quality Initiative (KDOQI) suggests non-calcium phosphate binders as the preferred phosphate binder in dialysis patients with severe vascular and/or other soft-tissue calcifications and in those with hypercalcemia or parathyroid hormone (PTH) <150 mg/dL. The Kidney Disease Improving Global Outcome (KDIGO) limits the use of non-calcium phosphate binders to patients with hypercalcemia. Regarding the effect on mortality, the results of clinical trials are again inconsistent. The other important aspect of using sevelamer is the issue of price, which is substantially higher than calcium-based phosphate binders. Reviewing the studies on economic aspects shows that sevelamer increases quality-adjusted life-years (QALY) and possibly life years, with a higher cost compared to calcium-based phosphate binders. In conclusion, sevelamer is a very useful drug for phosphate control, reduction of hypercalcemia, and lessening the risk of adynamic bone disease, with probable reduction in vascular calcification and possible reduction in mortality rate. It has a higher economic burden on health care systems compared to calcium-based phosphate binders. This may affect its extensive use

  2. Multimorbidity of chronic diseases among adult patients presenting to an inner-city clinic in Ghana

    PubMed Central

    2013-01-01

    Background Very little is known about multimorbidity and chronic diseases in low and middle income countries, particularly Sub-Saharan Africa, and more information is needed to guide the process of adapting the health systems in these countries to respond adequately to the increasing burden of chronic diseases. We conducted a hospital-based survey in an urban setting in Ghana to determine the prevalence of multimorbidity and its associated risk factors among adult patients presenting to an inner city clinic. Methods Between May and June 2012, we interviewed adult patients (aged 18 years and above) attending a routine outpatient clinic at an inner-city hospital in Accra using a structured questionnaire. We supplemented the information obtained from the interviews with information obtained from respondents’ health records. We used logistic regression analyses to explore the risk factors for multimorbidity. Results We interviewed 1,527 patients and retrieved matching medical records for 1,399 (91.6%). The median age of participants was 52.1 years (37–64 years). While the prevalence of multimorbidity was 38.8%, around half (48.6%) of the patients with multimorbidity were aged between 18–59 years old. The most common combination of conditions was hypertension and diabetes mellitus (36.6%), hypertension and musculoskeletal conditions (19.9%), and hypertension and other cardiovascular conditions (11.4%). Compared with patients aged 18–39 years, those aged 40–49 years (OR 4.68, 95% CI: 2.98–7.34), 50–59 years (OR 12.48, 95% CI: 8.23–18.92) and 60 years or older (OR 15.80, 95% CI: 10.66–23.42) were increasingly likely to present with multimorbidity. While men were less likely to present with multimorbidity, (OR 0.71, 95% CI: 0.45–0.94, p = 0.015), having a family history of any chronic disease was predictive of multimorbidity (OR 1.43, 95% CI: 1.03–1.68, p = 0.027). Conclusions Multimorbidity is a significant problem in this population

  3. Clinical relevance of Helicobacter pylori virulence factors in Iranian patients with gastrointestinal diseases.

    PubMed

    Bagheri, Nader; Azadegan-Dehkordi, Fatemeh; Rafieian-Kopaei, Mahmoud; Rahimian, Ghorbanali; Asadi-Samani, Majid; Shirzad, Hedaytollah

    2016-11-01

    Helicobacter pylori (H. pylori) usually colonizes the gastric mucosa of more than 50% of the human population, causing an infection that may appear in early childhood and can persist for life. H. pylori is suggested as the main cause of peptic ulcer and chronic gastritis. It is also associated with gastric cancer. Its severity and symptoms depend on environmental factors, host susceptibility and bacterial components, which allow H. pylori to switch between commensalism and pathogenicity. H. pylori is genetically highly variable, and the variability which affects H. pylori virulence factors might be useful in identifying the strains with different degrees of pathogenicity. The geographic distribution of distinct H. pylori genotypes is largely unknown and should be established. The prevalence of more pathogenic genotypes in certain areas may have important epidemiological consequences. It also might be associated with the severity of H. pylori related diseases in such regions. Given that Iran is located in the Middle East and Asian populations have revealed high levels of gastric cancer, it is of clinical interest to clarify the potential of H. pylori virulence markers in predicting the associated clinical outcomes. In this review, clinical relevance of adhesion molecules and significant virulence factors of H. pylori in Iranian patients with gastrointestinal diseases are discussed in comparison to other countries. Copyright © 2016 Elsevier Ltd. All rights reserved.

  4. [Development of clinical practice guidelines for patients with comorbidity and multiple diseases].

    PubMed

    Bernabeu-Wittel, M; Alonso-Coello, P; Rico-Blázquez, M; Rotaeche del Campo, R; Sánchez Gómez, S; Casariego Vales, E

    2014-01-01

    The management of patients with comorbidity and polypathology represents a challenge for all healthcare systems. Clinical practice guidelines (CPGs) have limitations when applied to this population. The aim of this study is to propose the terminology and methodology for optimally approach comorbidity and polypathology in the CPGs. Based on a literature review, we suggest a number of proposals for the approach in different phases of CPG preparation, with special attention to the inclusion of clusters of comorbidity in the initial questions the implementation of indirect evidence, the burden of disease management for patients and their environment, when establishing recommendations, as well as the strategies of dissemination and implementation. These proposals should be developed in greater depth with the implication of more agents in order to have valid and useful tools for this population.

  5. Development of clinical practice guidelines for patients with comorbidity and multiple diseases.

    PubMed

    Bernabeu-Wittel, M; Alonso-Coello, P; Rico-Blázquez, M; Rotaeche Del Campo, R; Sánchez Gómez, S; Casariego Vales, E

    2014-01-01

    The management of patients with comorbidity and polypathology represents a challenge for all healthcare systems. Clinical practice guidelines (CPGs) have limitations when applied to this population. The aim of this study is to propose the terminology and methodology for optimally approach comorbidity and polypathology in the CPGs. Based on a literature review, we suggest a number of proposals for the approach in different phases of CPG preparation, with special attention to the inclusion of clusters of comorbidity in the initial questions the implementation of indirect evidence, the burden of disease management for patients and their environment, when establishing recommendations, as well as the strategies of dissemination and implementation. These proposals should be developed in greater depth with the implication of more agents in order to have valid and useful tools for this population.

  6. [Multivascular disease in clinical practice].

    PubMed

    Despotović, Nebojsa; Zdravković, Mihajlo

    2002-01-01

    Multiple arterial disease is presented by coexistence of ischaemic heart disease, carotid disease and peripheral obliterate arterial disease. Atherosclerosis is the main factor for onset of the disease. Among 150 patients with clinical manifestations of obliterate disease of at least two aforementioned arterial systems, we examined by many noninvasive and invasive procedures the existence and degree of obliterate arterial disease of coronary, carotid and peripheral arteries of the lower extremities. The results revealed the statistically significant correlation among: ischaemic heart disease and carotid disease (r = 0.939; p < 0.01); ischaemic heart disease and peripheral arterial disease (r = 0.834; p < 0.05); ischaemic heart disease, peripheral arterial disease and carotid disease (r = 0.986; p < 0.01). The results pointed out that whenever clinical manifestations of obliterate disease of peripheral arteries are present, there is also need for routine examination of existent coronary artery disease.

  7. Clinical and genetic spectra in a series of Chinese patients with Charcot-Marie-Tooth disease.

    PubMed

    Wang, Rui; He, Jin; Li, Jin-Jing; Ni, Wang; Wu, Zhi-Ying; Chen, Wan-Jin; Wang, Yi

    2015-12-07

    The aim of this study was to determine the clinical features and frequencies of genetic subtypes in a series of patients with Charcot-Marie-Tooth (CMT) disease from Eastern China. Patients were divided into three subtypes, CMT1, CMT2 and hereditary neuropathy with liability to pressure palsy (HNPP), according to their electrophysiological manifestations. Multiplex ligation-dependent probe analysis (MLPA) was performed to detect duplications/deletions in the PMP22 gene. The coding regions and splice sites of the GJB1, MPZ, MFN2 and GDAP-1 genes were determined by direct sequencing. Among the 148 patients in the study, 37.2% of the cases had mutations in genes assessed. The mutation detection rate was higher in patients with family histories than in spontaneous cases. PMP22 duplication (13.5%) was predominant in this group of patients, followed by PMP22 deletion (11.5%), and point mutations in GJB1 (8.8%), MPZ (2.0%) and MFN2 (0.7%). Three novel mutations (c.151T>C and c.310 A>G in GJB1 and c.1516 C>G in MFN2) were detected. A small deletion in PMP22 exon 4 was detected in a patient with severe CMT1. Genetic tests have great value in CMT patients with family histories. The frequency of PMP22 duplications was lower in Asian patients than in others. We suggest that genetic testing strategies in CMT patients should be primarily based on electromyography data. Copyright © 2015 Elsevier B.V. All rights reserved.

  8. Characteristics of Patients Referred to a Pediatric Infectious Diseases Clinic With Unexplained Fever.

    PubMed

    Statler, Victoria A; Marshall, Gary S

    2016-09-01

    Older case series established diagnostic considerations for children meeting a priori definitions of fever of unknown origin (FUO). No recent study has examined the final diagnoses of children referred for unexplained fever. This study was conducted with a retrospective chart review of patients referred to a pediatric infectious diseases clinic from 2008 to 2012 for unexplained fever. Sixty-nine of 221 patients were referred for "prolonged" unexplained fever. Ten of these were not actually having fever, and 11 had diagnoses that were readily apparent at the initial visit. The remaining 48 were classified as having FUO. The median duration of reported fever for these patients was 30 days; 15 had a diagnosis made, 5 of which were serious. None of the serious FUO diagnoses were infections. Of 152 patients with "recurrent" unexplained fever, 92 had an "intermittent" fever pattern, and most of these had sequential, self-limited viral illnesses or no definitive diagnosis made. Twenty of the 60 patients with a "periodic" fever pattern were diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. Overall, 166 patients either were not having fever, had self-limited illnesses, or ultimately had no cause of fever discovered. Only 12 had a serious illness, 2 of which were infections (malaria and typhoid fever). Most children referred with unexplained fever had either self-limited illnesses or no specific diagnosis established. Serious diagnoses were unusual, suggesting that these diagnoses rarely present with unexplained fever alone, or that, when they do, the diagnoses are made by primary care providers or other subspecialists. © The Author 2015. Published by Oxford University Press on behalf of the Pediatric Infectious Diseases Society. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

  9. A clinical study of integrating acupuncture and Western medicine in treating patients with Parkinson's disease.

    PubMed

    Chen, Fang-Pey; Chang, Ching-Mao; Shiu, Jing-Huei; Chiu, Jen-Hwey; Wu, Ta-Peng; Yang, Jen-Lin; Kung, Yen-Ying; Chen, Fun-Jou; Chern, Chang-Ming; Hwang, Shinn-Jang

    2015-01-01

    Complementary therapy with acupuncture for Parkinson's disease (PD) has been studied for quite a long time, but the effectiveness of the treatment still remains unclear. The aim of this study is to evaluate the integrated effects of acupuncture treatment in PD patients who received western medicine. In the short-term acupuncture treatment study, 20 patients received acupuncture therapy twice a week in acupoints DU 20, GB 20, LI 11, LI 10, LI 4, GB 31, ST 32, GB 34 and GB 38 along with western medicine for 18 weeks, and 20 controlled patients received western medicine only. In the long-term acupuncture treatment, 13 patients received acupuncture treatment twice a week for 36 weeks. The outcome parameters include Unified Parkinson's disease rating scale (UPDRS), Beck Anxiety Inventory (BAI), Beck Depression Inventory-Version 2 (BDI-II), and WHO quality of life (WHOQOL). In the short-term clinical trial, a higher percentage of patients in the acupuncture group had score improvement in UPDRS total scores (55% vs. 15%, p = 0.019), sub-score of mind, behavior and mood (85% vs. 25%, p < 0.001), activity of daily living (65% vs. 15%, p = 0.003), mobility (40% vs. 15%, p = 0.155) and complication of treatment (75% vs. 15%, p < 0.001), BDI-II score (85% vs. 35%, p = 0.003), and WHOQOL score (65% vs. 15%, p = 0.003) when compared to control group at the end of the 18 weeks' follow up. After 36 weeks of long-term acupuncture treatment, the mean UPDRS total scores and sub-score of mentation, behavior and mood, sub-score of complications of therapy and BDI-II score decreased significantly when compared to the pretreatment baseline. In conclusion, acupuncture treatment had integrated effects in reducing symptoms and signs of mind, behavior, mood, complications of therapy and depression in PD patients who received Western medicine.

  10. Association Between Gastroesophageal Reflux Disease After Pneumatic Balloon Dilatation and Clinical Course in Patients With Achalasia

    PubMed Central

    Min, Yang Won; Lee, Jin Hee; Min, Byung-Hoon; Lee, Jun Haeng; Kim, Jae J; Rhee, Poong-Lyul

    2014-01-01

    Background/Aims The occurrence of gastroesophageal reflux disease (GERD) is known to be associated with lower post-treatment lower esophageal sphincter pressure in patients with achalasia. This study aimed to elucidate whether GERD after pneumatic balloon dilatation (PD) has a prognostic role and to investigate how the clinical course of GERD is. Methods A total of 79 consecutive patients who were first diagnosed with primary achalasia and underwent PD as an initial treatment were included in this retrospective study. Single PD was performed using a 3.0 cm balloon. The patients were divided into two groups: 1) who developed GERD after PD (GERD group) and 2) who did not develop GERD after PD (non-GERD group). GERD was defined as pathological acid exposure, reflux esophagitis or typical reflux symptoms. Results Twenty one patients (26.6%) developed GERD after PD during follow-up. There were no significant differences between the two groups in demographic or clinical factors including pre- and post-treatment manometric results. All patients in GERD group were well responsive to maintenance proton pump inhibitor therapy including on demand therapy or did not require maintenance. During a median follow-up of 17.8 months (interquartile range, 7.1–42.7 months), achalasia recurred in 15 patients (19.0%). However, the incidence of recurrence did not differ according to the occurrence of GERD after PD. Conclusions GERD often occurs after even a single PD for achalasia. However, GERD after PD is well responsive to PPI therapy. Our data suggest that GERD after PD during follow-up does not appear to have a prognostic role. PMID:24840373

  11. Clinical characteristics and prognostic impact of bacterial infection in hospitalized patients with alcoholic liver disease.

    PubMed

    Park, Jin Kyoung; Lee, Chang Hun; Kim, In Hee; Kim, Seon Min; Jang, Ji Won; Kim, Seong Hun; Kim, Sang Wook; Lee, Seung Ok; Lee, Soo Teik; Kim, Dae-Ghon

    2015-05-01

    Bacterial infection is an important cause of death in patients with liver cirrhosis. The aim of this study was to investigate the clinical characteristics and prognostic impact of bacterial infection in hospitalized patients with alcoholic liver disease (ALD). We retrospectively analyzed data from 409 patients consecutively admitted to a tertiary referral center with ALD diagnosis. Of a total of 544 admissions, 133 (24.4%) cases presented with bacterial infection, of which 116 were community-acquired whereas 17 were hospital-acquired. The common types of infection were pneumonia (38%), biliary tract infection (17%), soft tissue infection (12%), and spontaneous bacterial peritonitis (9%). Diabetes, serum Na <135 mM/L, albumin <2.5 g/dL, C-reactive protein ≥20 mg/L, systemic inflammatory response syndrome (SIRS) positivity were independently associated with bacterial infection in patients with ALD. Overall 30-day and 90-day mortalities in patients with bacterial infection were significantly (P < 0.001) higher than those without infection (22.3% vs. 5.1% and 32.3% vs. 8.2%, respectively). Furthermore, bacterial infection (HR, 2.2; 95% CI, 1.049-4.579, P = 0.037), SIRS positivity (HR, 2.5; 95% CI, 1.240-4.861, P = 0.010), Maddrey's discriminant function score ≥32 (HR, 2.3; 95% CI, 1.036-5.222, P = 0.041), and hemoglobin <12 g/dL (HR, 2.4; 95% CI, 1.081-5.450, P = 0.032) were independent predictors of short-term mortality. In conclusion, bacterial infection and SIRS positivity predicted short-term prognosis in hospitalized patients with ALD. A thorough evaluation at admission or on clinical deterioration is required to detect possible infection with prompt management.

  12. A novel assessment tool for clinical care of patients with autoinflammatory disease: juvenile autoinflammatory disease multidimensional assessment report.

    PubMed

    Konukbay, Dilek; Gattorno, Marco; Yildiz, Dilek; Frenkel, Joost; Acikel, Cengizhan; Sozeri, Betul; Makay, Balahan; Aktay Ayaz, Nuray; Barut, Kenan; Kisaarslan, Aysenur; Bilginer, Yelda; Karaman, Dursun; Peru, Harun; Simsek, Dogan; Aydog, Ozlem; Unsal, Erbil; Gunduz, Zubeyde; Fidanci, Berna Eren; Kone-Paut, Isabelle; Kasapcopur, Ozgur; Ravelli, Angelo; Ozen, Seza; Demirkaya, Erkan

    2016-01-01

    To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care. The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome. It is proposed for use as both a proxy-report and a patient self-report, with the suggested age range of 8-18 years for use as a self-report. 250 children with FMF were included in the study. Total of 179 forms were filled up by parents and patients, and 71 forms were filled up by parents having children less than 8 years. Completing and scoring the JAIMAR can be done in 15 minutes. For the JAIMAR's dimensions, the Cronbach's alpha coefficient for internal consistency was between 0.507-0.998. There was a significant and a positive correlation between the test-retest scale scores (ICC=0.607-0.966). Concerning construct validity, all factors loadings were above 0.30. For the criterion validity, the correlation level between each dimension and the related scale ranged from medium (r=0.329, p<0.0001) to large (r=0.894, p<0.0001). The parents' proxy-reported and children's self-reported data were outstandingly concordant (r=0.770-0.989). The development of the JAIMAR introduces a new and multi-dimensional approach in paediatric rheumatology practice. It is a new tool for children with auto-inflammatory dis-ease and it may help enhance their quality of care.

  13. Clinical and Laboratory Findings in Patients with δ-Storage Pool Disease: A Case Series.

    PubMed

    Selle, Fabien; James, Chloé; Tuffigo, Marie; Pillois, Xavier; Viallard, Jean-François; Alessi, Marie-Christine; Fiore, Mathieu

    2017-02-01

    Platelet δ-storage pool disease (δ-SPD) is a platelet function disorder characterized by a reduction in the number or content of dense granules. Reports on δ-SPD are mostly limited to case presentations. We aimed to retrospectively describe a series of patients with δ-SPD to better characterize the disease. We studied 16 patients with congenital or acquired δ-SPD. Lumiaggregometry, α- and δ-granules content, platelet ultrastructure, αIIbβ3 integrin, and glycoprotein Ib (GPIb) activation were assessed. Most of the patients generally demonstrate mild to moderate bleeding diathesis. Platelet aggregation studies showed moderate abnormalities with variable profiles, while all the individuals had almost complete absence of adenosine triphosphate release. Mepacrine capture, CD63 expression, and study of dense granules by electron microscopy enabled to distinguish different subtypes of δ-SPD with quantitative or qualitative defect. Surprisingly, significantly decreased GPIb expression levels after platelet activation with thrombin receptor activating peptide 50 μM were found, suggesting that GPIb-impaired mobilization may represent an additional feature of the disorder. In conclusion, δ-SPD represents a complex disorder with various clinical and biological aspects, requiring a great deal of expertise to be properly diagnosed.

  14. Clinical Immunophenotype at Disease Onset in Previously Healthy Patients With Cryptococcal Meningitis.

    PubMed

    Xu, Lie; Huang, Qin; Lin, Jin-Ran; Zhu, Cui-Yun; Li, Xin-Hua; Ye, Shan-Ke; Zhu, Ai-Hong; Chen, Dai-Hong; Zhang, Cheng-Feng; Chen, Liang; Ling, Yun

    2016-02-01

    Cryptococcal meningitis (CM) is a global disease with significant morbidity and mortality. Although low peripheral blood cluster of differentiation 4 (CD4) cell counts are found to be related to a high burden of cryptococcus in HIV-infected patients, little is known about possible immune defects in previously healthy patients (PHPs). We performed a retrospective study of 41 CM patients treated from January 2005 to December 2014 who did not have HIV-infection. There were 33 PHPs and 8 not previously healthy patients (non-PHPs). We analyzed clinical test data pertaining to peripheral blood T cells, antibodies, inflammation markers, and cerebral spinal fluid (CSF) completed during the disease onset phase and 5 years following diagnosis. PHPs had significantly higher counts of cluster of differentiation 3 (CD3), cluster of differentiation 4 (CD4), and cluster of differentiation 45 (CD45) cells, and lower percentages of CD8 cells than non-PHPs (P < 0.05). Measurements of inflammatory markers and immunoglobulin in blood were comparable except for lower immunoglobulin A (IgA) levels in non-PHPs (P = 0.0410). Examination of CSF revealed lower white blood cell (WBC) counts in non-PHPs. Five-year mortality in PHPs was higher than in non-PHPs (22.0% vs 12.5%) but this was not statistically significant (P > 0.05). Multivariate analysis revealed that higher immunoglobulin G (IgG) levels in serum during disease onset may be an independent predictor of mortality (P = 0.015). In conclusion, PHPs demonstrate an immunophenotype that is distinct from that of non-PHPs, leading to an improved understanding of the immunology of cryptococcal meningitis.

  15. Clinical optical coherence tomography combined with multiphoton tomography of patients with skin diseases.

    PubMed

    König, Karsten; Speicher, Marco; Bückle, Rainer; Reckfort, Julia; McKenzie, Gordon; Welzel, Julia; Koehler, Martin J; Elsner, Peter; Kaatz, Martin

    2009-07-01

    We report on the first clinical study based on optical coherence tomography (OCT) in combination with multiphoton tomography (MPT) and dermoscopy. 47 patients with a variety of skin diseases and disorders such as skin cancer, psoriasis, hemangioma, connective tissue diseases, pigmented lesions, and autoimmune bullous skin diseases have been investigated with (i) state-of-the-art OCT systems for dermatology including multibeam swept source OCT, (ii) the femtosecond laser multiphoton tomograph, and (iii) dermoscopes. Dermoscopy provides two-dimensional color images of the skin surface. OCT images reflect modifications of the intratissue refractive index whereas MPT is based on nonlinear excitation of endogenous fluorophores and second harmonic generation. A stack of cross-sectional OCT "wide field" images with a typical field of view of 5 x 2 mm(2) gave fast information on the depth and the volume of the lesion. Multiphoton tomography provided 0.36 x 0.36 mm(2) horizontal/diagonal optical sections within seconds of a particular region of interest with superior submicron resolution down to a tissue depth of 200 mum. The combination of OCT and MPT provides a unique powerful optical imaging modality for early detection of skin cancer and other skin diseases as well as for the evaluation of the efficiency of treatments.

  16. Serum Cytokine Levels and Their Relation to Clinical Features in Patients with Autoimmune Liver Diseases

    PubMed Central

    Akberova, Dilyara; Kiassov, Andrei P.

    2017-01-01

    Serum cytokine levels were explored in a combined group of patients with autoimmune liver diseases (AILDs) and separately in patients with autoimmune hepatitis (AIH) and overlap syndrome. Overall, 60 patients with AILD, among them 32 patients with AIH and 28 patients with overlap syndrome, were included in the cross-sectional study. Serum cytokine levels were measured at baseline and compared to those of 21 healthy controls. Patients with AILD had significantly higher levels of IL-6 (0.70 (range 0.17–99.86) in patients with AILD compared to 0.40 (range 0.14–2.65) in controls, p < 0.01), IL-8 (1.66 (0.45–34.58) versus 0.53 (0.35–2.38), resp., p < 0.01), and TNF-α (2.61 (0.23–120.88) versus 1.65 (0.21–7.54), resp., p < 0.01). Adjusted logistic regression analysis revealed a pronounced relation of IL-8 and AILD, 48.36 (3.63–643.60), as well as AIH, 18.54 (1.08–318.54), and overlap syndrome, 23.85 (2.37–240.23), while the associations between the level of other cytokines and AILD were assessed as nonsignificant. In the language of absolute numbers, the increase of IL-8 serum level by 1 pg/mL had increased the chance for a patient to find himself in a group of AILD by 48.36 times. Also, high IL-8 serum levels were strongly related to clinical parameters. PMID:28299346

  17. HLA class II associations with rheumatic heart disease among clinically homogeneous patients in children in Latvia

    PubMed Central

    Stanevicha, Valda; Eglite, Jelena; Sochnevs, Arturs; Gardovska, Dace; Zavadska, Dace; Shantere, Ruta

    2003-01-01

    Genetic control of immune reactions has a major role in the development of rheumatic heart disease (RHD) and differs between patients with rheumatic fever (RF). Some authors think the risk of acquiring RHD is associated with the HLA class II DR and DQ loci, but other views exist, due to the various HLA-typing methods and ways of grouping cases. Our goal was to determine the relations between HLA class II alleles and risk of or protection from RF in patients with relatively homogeneous clinical manifestations. A total of 70 RF patients under the age of 18 years were surveyed in Latvia. HLA genotyping of DRB1*01 to DRB1*18 and DQB1*0201-202, *0301-305, *0401-402, *0501-504, and *0601-608 was performed using polymerase chain reaction sequence-specific primers. Data for a control group of 100 healthy individuals typed for HLA by the same method were available from the databank of the Immunology Institute of Latvia. Of the RF patients, 47 had RHD and 8 had Sydenham's chorea. We concluded that HLA class II DRB1*07-DQB1*0401-2 and DRB1*07-DQB1*0302 could be the risk alleles and HLA class II DRB1*06 and DQB1*0602-8, the protective ones. Patients with mitral valve regurgitation more often had DRB1*07 and DQB1*0401-2, and patients with multivalvular lesions more often had DRB1*07 and DQB1*0302. In Sydenham's chorea patients, the DQB1*0401-2 allele was more frequent. Genotyping control showed a high risk of RF and RHD in patients with DRB1*01-DQB1*0301-DRB1*07-DQB1*0302 and DRB1*15-DQB1*0302-DRB1*07-DQB1*0303. PMID:14680508

  18. Cataract surgery in patients with ocular surface disease: An update in clinical diagnosis and treatment

    PubMed Central

    Afsharkhamseh, Neda; Movahedan, Asadolah; Motahari, Hooman; Djalilian, Ali R.

    2014-01-01

    In this article we review essentials of diagnosis and management of ocular surface disease in patients who undergo cataract surgery. It is clearly shown that dry eye disease worsens following the cataract surgery in patients with prior history of ocular surface disease, Also new cases of dry eye might appear. Current strategies for the timely diagnosis and proper management of dry eye syndrome in the face of cataract surgery patients are mainly emphasized. To achieve the best outcome in cataract surgery, a healthy ocular surface is crucial. While ocular surface preparation is indispensable in patients with established ocular surface disease, it is also helpful in those with minimal signs or symptoms of surface disease. The current approach begins with early diagnosis and drastic management of ocular surface disease before cataract surgery using a stepwise regimen customized to each patient and disease severity. These measures are continued throughout and after the surgery. PMID:25278791

  19. Adalimumab induction and maintenance therapy achieve clinical remission and response in Chinese patients with Crohn's disease

    PubMed Central

    Ran, Zhi Hua; Gao, Xiang; Chen, Minhu; Zhong, Jie; Sheng, Jian-Qiu; Kamm, Michael A; Travis, Simon; Wallace, Kori; Mostafa, Nael M; Shapiro, Marisa; Li, Yao; Thakkar, Roopal B; Robinson, Anne M

    2016-01-01

    Background/Aims This was a Phase 2 study (NCT02015793) to evaluate the pharmacokinetics, safety, and efficacy of adalimumab in Chinese patients with Crohn's disease (CD). Methods Thirty, adult Chinese patients with CD (CD Activity Index [CDAI] 220–450; high-sensitivity [hs]-C-reactive protein [CRP] ≥3 mg/L) received double-blind adalimumab 160/80 mg or 80/40 mg at weeks 0/2, followed by 40 mg at weeks 4 and 6. An open-label extension period occurred from weeks 8–26; patients received 40 mg adalimumab every other week. Serum adalimumab concentration and change from baseline in fecal calprotectin (FC) were measured during the double-blind period. Clinical remission (CDAI <150), response (decrease in CDAI ≥70 points from baseline), and change from baseline in hs-CRP were assessed through week 26. Nonresponder imputation was used for missing categorical data and last observation carried forward for missing hs-CRP/FC values. No formal hypothesis was tested. Adverse events were monitored. Results Mean adalimumab serum concentrations during the induction phase were 13.9–18.1 µg/mL (160/80 mg group) and 7.5−9.5 µg/mL (80/40 mg group). During the double-blind period, higher remission/response rates and greater reductions from baseline in hs-CRP and FC were observed with adalimumab 160/80 mg compared to that with 80/40 mg. Adverse event rates were similar among all treatment groups. Conclusions Adalimumab serum concentrations in Chinese patients with CD were comparable to those observed previously in Western and Japanese patients. Clinically meaningful remission rates and improvement in inflammatory markers were achieved with both dosing regimens; changes occurred rapidly with adalimumab 160/80 mg induction therapy. No new safety signals were reported. PMID:27175116

  20. Etiology of Genital Ulcer Disease in Male Patients Attending a Sexually Transmitted Diseases Clinic: First Assessment in Cuba.

    PubMed

    Noda, Angel A; Blanco, Orestes; Correa, Consuelo; Pérez, Lissette; Kourí, Vivian; Rodríguez, Islay

    2016-08-01

    Sexually transmitted diseases (STDs) and in particular genital ulcer disease (GUD) have a major impact on morbidity and mortality in developing countries. The World Health Organization recommends the use of syndromic guidelines for the treatment of sexually transmitted infections (STIs) in resource-constrained countries. Surveillance of autochthonous etiologies provides epidemiological information contributing to the prevention and treatment of STIs. We investigated the etiology and factors associated with GUD among male patients attending a STD clinic in Havana, Cuba. Swabs from genital ulcers of 113 male patients, collected from May 2012 to June 2015, were analyzed using PCR for herpes simplex virus types 1 and 2, Treponema pallidum, Haemophilus ducreyi, and Chlamydia trachomatis. We also investigated the clinical and epidemiological characteristics associated with the presence of these pathogens in GUD. At least one of the pathogens was detected in 70% of patients. The occurrence of the pathogens was herpes simplex virus type 2 (HSV-2) (51.3%), T. pallidum (29.2%), and C. trachomatis (1.8%). Co-infections occurred as follows: T. pallidum-HSV-2 (10.6%), C. trachomatis-HSV-2 (0.9%) and C. trachomatis-T. pallidum (0.9%). Herpes simplex virus type 1 and H. ducreyi were not detected. Ages 15 to 40 years, HIV-positive serostatus, and no condom use were significant risk factors for the presence of HSV-2 in genital ulcers. Our preliminary results highlight the predominance of HSV-2 and T. pallidum as the leading GUD etiologies in the study population and identified risk factors associated with HSV-2. This information should help to inform guidelines for better management of GUD in Havana, Cuba.

  1. Determining the Minimally Important Difference in the Clinical Disease Activity Index for Improvement and Worsening in Early Rheumatoid Arthritis Patients.

    PubMed

    Curtis, J R; Yang, S; Chen, L; Pope, J E; Keystone, E C; Haraoui, B; Boire, G; Thorne, J C; Tin, D; Hitchon, C A; Bingham, C O; Bykerk, V P

    2015-10-01

    Simplified measures to quantify rheumatoid arthritis (RA) disease activity are increasingly used. The minimum clinically important differences (MCID) for some measures, such as the Clinical Disease Activity Index (CDAI), have not been well-defined in real-world clinic settings, especially for early RA patients with low/moderate disease activity. Data from Canadian Early Arthritis Cohort patients were used to examine absolute change in CDAI in the first year after enrollment, stratified by disease activity. MCID cut points were derived to optimize the sum of sensitivity and specificity versus the gold standard of patient self-reported improvement or worsening. Sensitivity, specificity, positive predictive values, and negative predictive values were calculated against patient self-reported improvement (gold standard) and for change in pain, Health Assessment Questionnaire (HAQ), and Disease Activity Score in 28 joints (DAS28) improvement. Discrimination was examined using the area under receiver operator curves. Similar methods were used to evaluate MCIDs for worsening for patients who achieved low disease activity. A total of 578 patients (mean ± SD age 54.1 ± 15.3 years, 75% women, median [interquartile range] disease duration 5.3 [3.3, 8.0] months) contributed 1,169 visit pairs to the improvement analysis. The MCID cut points for improvement were 12 (patients starting in high disease activity: CDAI >22), 6 (moderate: CDAI 10-22), and 1 (low disease activity: CDAI <10). Performance characteristics were acceptable using these cut points for pain, HAQ, and DAS28. The MCID for CDAI worsening among patients who achieved low disease activity was 2 units. These minimum important absolute differences in CDAI can be used to evaluate improvement and worsening and increase the utility of CDAI in clinical practice. © 2015, American College of Rheumatology.

  2. Clinical pharmacokinetics and pharmacodynamics of mycophenolate in patients with autoimmune disease.

    PubMed

    Abd Rahman, Azrin N; Tett, Susan E; Staatz, Christine E

    2013-05-01

    Mycophenolic acid (MPA), the active drug moiety of mycophenolate, is a potent immunosuppressant agent, which is increasingly being used in the treatment of patients with various autoimmune diseases. An understanding of the pharmacokinetics and pharmacodynamics of mycophenolate in this population should assist the clinician with rational dosage decisions. This review aims to provide an overview of the published literature on the clinical pharmacokinetics of mycophenolate in autoimmune disease and a briefer summary of current pharmacodynamic knowledge, and to identify areas of potential future research in this field. A literature search was conducted using PubMed and EMBASE databases as well as bibliographies of relevant articles and 'on-line early' pages of key journals. Twenty-six pharmacokinetic/pharmacodynamic studies of mycophenolate in people with autoimmune disease were identified and appraised. Twenty-two of these studies used non-compartmental analysis techniques and four used population modelling methods to estimate mycophenolate pharmacokinetic parameters. Seven studies linked mycophenolate exposure to treatment outcomes. Only four studies measured free (unbound) as well as total mycophenolate exposure and only two studies characterised MPA disposition following enteric-coated mycophenolate sodium (EC-MPS) administration. Across all studies MPA displayed erratic and complex pharmacokinetics with substantial between-subject variability. Based on total drug measurement, the dose-normalised MPA area under the plasma concentration-time curve (AUC) from 0 to 12 h post-dose (AUC12) varied at least five- to ten-fold between subjects. Typical values for apparent oral clearance (CL/F) of MPA during nonlinear mixed-effects modelling ranged from 8.3 to 25.3 L/h. Patient renal function, serum albumin levels, sex, ethnicity, food intake, concurrent administration of interacting drugs such as antacids, metal-containing medications and proton pump inhibitors and

  3. Intestinal amebiasis in a group of patients with ulcerative colitis: influence on clinical course of the disease.

    PubMed

    Vukobrat-Bijedic, Zora; Husic-Selimovic, Azra; Bijedic, Nina; Bjelogrlic, Ivana; Djuran, Aleksandra

    2013-01-01

    Ulcerative colitis (UC) is a common disease with a chronic and relapsing presentation requiring regular clinical follow up. Epidemiological and microbiologic studies suggest that enteropathogenic microorganisms play a substantial role in the clinical presentation and extent of inflammatory bowel disease. To evaluate the presence of intestinal infections by Entamoeba hystolitica in patients with ulcerative colitis, their impact on clinical outcome, and to identify associated risk factors. A total of 31 patients hospitalized on Gastroenterohepatology Department with patohystologically proved ulcerative colitis were studied. Fresh feces samples taken from 20 patients were examined immediately using Eosin and Lugol-staining methods and analyzing the presence of vegetative and MIFC (Meriolat and Iod staining). A total of 16 female and 15 male hospitalized UC patients were analysed in a period of two years (2010-2011). The mean age at diagnosis was 43 years. We analyzed relation of amoeba infection with localization of ulcerative colitis. Our results indicate that amoeba infection is related to extent of disease (they were mostly present in pancolitis). Presence of amoeba is not related to age nor gender. Furthermore, presence of amoeba was not associated with more severe clinical course of disease. Similarly, higher value of serum marker of inflammation was not associated with amoeba infection. Amoeba infections in UC patients treated at Gastroenterohepatology Department was not related to the grade of disease activity, and other clinical variables such as gender, age and parameters of inflammation. These microorganisms could be a contributing cause of extended localization of disease.

  4. Arthropathy of Wilson's disease. Study of clinical and radiological features in 32 patients.

    PubMed Central

    Golding, D N; Walshe, J M

    1977-01-01

    The principal clinical features and radiological findings relating to the locomotor system have been studied in 32 consecutive hospital admissions of patients with Wilson's disease. 5 of these patients were recently diagnosed and had as yet received no treatment, while 27 were routine admissions for follow-up and biochemical supervision of their illness. No patient was specifically included or excluded from the series because of the presence or absence of locomotor symptoms. The most common radiological abnormality was a generalized increase of radiolucency, interpreted as skeletal demineralization (21 cases), followed by premature osteoarthrosis (8 cases). Changes in the spine were common and included osteochondritis, reduction of intervertebral joint spaces, osteoarthrosis, and a tendency to squaring of vertebral bodies. Other bony changes included fluffy irregularity of femoral trochanters, osteochondritis dissecans of the knees, osteophytic protrusions at bone ends, and bunches of tongue-like osteophytes at joint margins. The symptoms associated with these radiological abnormalities comprised back pain and stiffness with restricted movement, pain and stiffness of knees, hips, and wrists, and tenderness to pressure over margins of affected joints. Joint hypermobility was also observed in 9 patients. Episodes of acute polyarthritis with serological changes were seen in 5 cases; all these episodes appeared to be related directly to treatment with penicillamine. Images PMID:857745

  5. Different aspects of dysexecutive syndrome in patients with moyamoya disease and its clinical subtypes.

    PubMed

    Fang, Lingling; Huang, Jia; Zhang, Qian; Chan, Raymond C K; Wang, Rong; Wan, Weiqing

    2016-08-01

    OBJECTIVE Dysexecutive syndrome is common in patients with moyamoya disease (MMD), a chronic cerebrovascular disease that is characterized by stenosis of the bilateral internal carotid arteries and progressive collateral revascularization, and MMD can be classified as ischemic or hemorrhagic according to the disease presentation and history. In this study, the authors aimed to determine which aspects of executive function are impaired in patients with MMD, in addition to the specific dysexecutive functions present among its clinical subtypes and the mechanisms underlying dysexecutive function in these patients. METHODS The authors administered 5 typical executive function tests (the Stroop test, the Hayling Sentence Completion Test [HSCT], the verbal fluency [VF] test, the N-back test, and the Sustained Attention to Response Task [SART]) to 49 patients with MMD and 47 IQ-, age-, education-, and social status-matched healthy controls. The dysexecutive questionnaire (DEX) was also used to assess participants' subjective feelings about their executive function. A total of 39 of the patients were evaluated by CT perfusion (CTP) before the assessments were performed, and the correlations among the performances of the patients on the above tests with the parameters of cerebral blood volume, cerebral blood flow (CBF), mean transit time (MTT), and time-to-peak (TTP) in the frontal lobes of these patients were also analyzed. RESULTS Many aspects of executive function in the patients with MMD were significantly poorer than those in the healthy controls, and the patients performed particularly poorer on the VF test, HSCT, N-back test, and SART. The patients with hemorrhagic MMD exhibited worse executive inhibition, executive processing, and semantic inhibition compared with those with ischemic MMD, but the latter group presented a worse working memory and poorer sustained attention. There were no significant differences in the DEX scores between the patients with MMD and

  6. Examining chronic care patient preferences for involvement in health-care decision making: the case of Parkinson's disease patients in a patient-centred clinic.

    PubMed

    Zizzo, Natalie; Bell, Emily; Lafontaine, Anne-Louise; Racine, Eric

    2017-08-01

    Patient-centred care is a recommended model of care for Parkinson's disease (PD). It aims to provide care that is respectful and responsive to patient preferences, values and perspectives. Provision of patient-centred care should entail considering how patients want to be involved in their care. To understand the participation preferences of patients with PD from a patient-centred care clinic in health-care decision-making processes. Mixed-methods study with early-stage Parkinson's disease patients from a patient-centred care clinic. Study involved a modified Autonomy Preference Index survey (N=65) and qualitative, semi-structured in-depth interviews, analysed using thematic qualitative content analysis (N=20, purposefully selected from survey participants). Interviews examined (i) the patient preferences for involvement in health-care decision making; (ii) patient perspectives on the patient-physician relationship; and (iii) patient preferences for communication of information relevant to decision making. Preferences for participation in decision making varied between individuals and also within individuals depending on decision type, relational and contextual factors. Patients had high preferences for communication of information, but with acknowledged limits. The importance of communication in the patient-physician relationship was emphasized. Patient preferences for involvement in decision making are dynamic and support shared decision making. Relational autonomy corresponds to how patients envision their participation in decision making. Clinicians may need to assess patient preferences on an on-going basis. Our results highlight the complexities of decision-making processes. Improved understanding of individual preferences could enhance respect for persons and make for patient-centred care that is truly respectful of individual patients' wants, needs and values. © 2016 The Authors. Health Expectations Published by John Wiley & Sons Ltd.

  7. [Adequacy of clinical interventions in patients with advanced and complex disease. Proposal of a decision making algorithm].

    PubMed

    Ameneiros-Lago, E; Carballada-Rico, C; Garrido-Sanjuán, J A; García Martínez, A

    2015-01-01

    Decision making in the patient with chronic advanced disease is especially complex. Health professionals are obliged to prevent avoidable suffering and not to add any more damage to that of the disease itself. The adequacy of the clinical interventions consists of only offering those diagnostic and therapeutic procedures appropriate to the clinical situation of the patient and to perform only those allowed by the patient or representative. In this article, the use of an algorithm is proposed that should serve to help health professionals in this decision making process. Copyright © 2014 SECA. Published by Elsevier Espana. All rights reserved.

  8. Does this patient with shoulder pain have rotator cuff disease?: The Rational Clinical Examination systematic review.

    PubMed

    Hermans, Job; Luime, Jolanda J; Meuffels, Duncan E; Reijman, Max; Simel, David L; Bierma-Zeinstra, Sita M A

    2013-08-28

    Rotator cuff disease (RCD) is the most common cause of shoulder pain seen by physicians. To perform a meta-analysis to identify the most accurate clinical examination findings for RCD. Structured search in MEDLINE, EMBASE, and CINAHL from their inception through May 2013. For inclusion, a study must have met the following criteria: (1) description of history taking, physical examination, or clinical tests concerning RCD; (2) detailing of sensitivity and specificity; (3) use of a reference standard with diagnostic criteria prespecified; (4) presentation of original data, or original data could be obtained from the authors; and (5) publication in a language mastered by one of the authors (Danish, Dutch, English, French, German, Norwegian, Spanish, Swedish). Likelihood ratios (LRs) of symptoms and signs of RCD or of a tear, compared with an acceptable reference standard; quality scores assigned using the Rational Clinical Examination score and bias evaluated with the Quality Assessment of Diagnostic Accuracy Studies tool. Twenty-eight studies assessed the examination of referred patients by specialists. Only 5 studies reached Rational Clinical Examination quality scores of level 1-2. The studies with quality scores of level 1-2 included 30 to 203 shoulders with the prevalence of RCD ranging from 33% to 81%. Among pain provocation tests, a positive painful arc test result was the only finding with a positive LR greater than 2.0 for RCD (3.7 [95% CI, 1.9-7.0]), and a normal painful arc test result had the lowest negative LR (0.36 [95% CI, 0.23-0.54]). Among strength tests, a positive external rotation lag test (LR, 7.2 [95% CI, 1.7-31]) and internal rotation lag test (LR, 5.6 [95% CI, 2.6-12]) were the most accurate findings for full-thickness tears. A positive drop arm test result (LR, 3.3 [95% CI, 1.0-11]) might help identify patients with RCD. A normal internal rotation lag test result was most accurate for identifying patients without a full-thickness tear (LR, 0

  9. Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease.

    PubMed

    Kanno, Hiroshi; Kuratsu, Jun-ichi; Nishikawa, Ryo; Mishima, Kazuhiko; Natsume, Atushi; Wakabayashi, Toshihiko; Houkin, Kiyohiro; Terasaka, Shunsuke; Shuin, Taro

    2013-01-01

    Central nervous system (CNS) hemangioblastoma (HB) is one of the most common manifestations in von Hippel-Lindau disease (VHL), but large-scale studies on clinical features of CNS HB in VHL are scarce. On the basis of the results of a questionnaire, we collected data of VHL patients with CNS HB. The total number of CNS HBs in 111 VHL patients (male 59, female 52) was 264 with the following distributions: cerebellar, 65.4 %; brainstem, 9.9 %; spinal cord, 23.9 %; and pituitary, 1. 1 %. The follow-up period was 0.6 to 39.2 years, with the mean 12.5 years. Patients bearing brainstem or spinal cord HB also had another HB significantly more frequently than those bearing cerebellar HBs (P < 0.05). The mean onset age of CNS HB was 29.1 years, and that of patients bearing a single HB (mean 34.4 years) was significantly greater than that of multiple HBs (mean 25.7 years). Patients with multiple HBs under 40 years are more dominant than those with a single HB. The distribution rate of brainstem HB is significantly smaller in patients below 30 years than patients above 29 years. Although ECOG PS score increased along with number of operations, the onset age decreased with increasing number of operations. The mean ECOG PS score of patients below 20 years is significantly smaller than patients above 19 years. When the onset age of CNS HB is under 40 years, and CNS HB is located at the brainstem or spinal cord HB, the probability of multiple occurrence can be predicted. Since patients with an onset age under 20 years old preserve a high performance status, early detection of CNS HB would be important. In addition, since a multiple operations aggravate performance status, number of operations should be reduced.

  10. Effects of Mongersen (GED-0301) on Endoscopic and Clinical Outcomes in Patients With Active Crohn's Disease.

    PubMed

    Feagan, Brian G; Sands, Bruce E; Rossiter, Guillermo; Li, Xiaobin; Usiskin, Keith; Zhan, Xiaojiang; Colombel, Jean-Frédéric

    2017-08-25

    GED-0301 is an antisense oligodeoxynucleotide with a sequence complementary to the Smad7 mRNA transcript. Smad7 is a negative regulator of transforming growth factor-β, which is increased in the intestinal mucosa of patients with active Crohn's disease (CD). We randomly assigned 63 CD patients to 4-, 8-, or 12-week treatment groups receiving oral GED-0301 (160 mg/day). The primary objective was to determine GED-0301's effect on endoscopic CD measures; secondary objectives included effects on clinical activity. Endoscopic improvement was observed in 37% of participants with evaluable endoscopy results at week 12. At week 12, 32% (4 weeks), 35% (8 weeks), and 48% (12 weeks) of patients receiving GED-0301 were in remission (CD activity index score <150); corresponding reductions from baseline in mean CD activity index scores were -124, -112, and -133 points. No new safety signals were observed. These findings support a GED-0301 benefit in active CD. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

  11. Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease: A Randomized Clinical Trial.

    PubMed

    Frank, Samuel; Testa, Claudia M; Stamler, David; Kayson, Elise; Davis, Charles; Edmondson, Mary C; Kinel, Shari; Leavitt, Blair; Oakes, David; O'Neill, Christine; Vaughan, Christina; Goldstein, Jody; Herzog, Margaret; Snively, Victoria; Whaley, Jacquelyn; Wong, Cynthia; Suter, Greg; Jankovic, Joseph; Jimenez-Shahed, Joohi; Hunter, Christine; Claassen, Daniel O; Roman, Olivia C; Sung, Victor; Smith, Jenna; Janicki, Sarah; Clouse, Ronda; Saint-Hilaire, Marie; Hohler, Anna; Turpin, Denyse; James, Raymond C; Rodriguez, Ramon; Rizer, Kyle; Anderson, Karen E; Heller, Hope; Carlson, Alexis; Criswell, Susan; Racette, Brad A; Revilla, Fredy J; Nucifora, Frederick; Margolis, Russell L; Ong, MaryJane; Mendis, Tilak; Mendis, Neila; Singer, Carlos; Quesada, Monica; Paulsen, Jane S; Brashers-Krug, Thomas; Miller, Amanda; Kerr, Jane; Dubinsky, Richard M; Gray, Carolyn; Factor, Stewart A; Sperin, Elaine; Molho, Eric; Eglow, Mary; Evans, Sharon; Kumar, Rajeev; Reeves, Christina; Samii, Ali; Chouinard, Sylvain; Beland, Monica; Scott, Burton L; Hickey, Patrick T; Esmail, Sherali; Fung, Wai Lun Alan; Gibbons, Clare; Qi, Lina; Colcher, Amy; Hackmyer, Cory; McGarry, Andrew; Klos, Kevin; Gudesblatt, Mark; Fafard, Lori; Graffitti, Laura; Schneider, Daniel P; Dhall, Rohit; Wojcieszek, Joanne M; LaFaver, Kathrin; Duker, Andrew; Neefus, Erin; Wilson-Perez, Hilary; Shprecher, David; Wall, Paola; Blindauer, Karen A; Wheeler, Lynn; Boyd, James T; Houston, Emily; Farbman, Eric S; Agarwal, Pinky; Eberly, Shirley W; Watts, Arthur; Tariot, Pierre N; Feigin, Andrew; Evans, Scott; Beck, Chris; Orme, Constance; Edicola, Jon; Christopher, Emily

    2016-07-05

    Deutetrabenazine is a novel molecule containing deuterium, which attenuates CYP2D6 metabolism and increases active metabolite half-lives and may therefore lead to stable systemic exposure while preserving key pharmacological activity. To evaluate efficacy and safety of deutetrabenazine treatment to control chorea associated with Huntington disease. Ninety ambulatory adults diagnosed with manifest Huntington disease and a baseline total maximal chorea score of 8 or higher (range, 0-28; lower score indicates less chorea) were enrolled from August 2013 to August 2014 and randomized to receive deutetrabenazine (n = 45) or placebo (n = 45) in a double-blind fashion at 34 Huntington Study Group sites. Deutetrabenazine or placebo was titrated to optimal dose level over 8 weeks and maintained for 4 weeks, followed by a 1-week washout. Primary end point was the total maximal chorea score change from baseline (the average of values from the screening and day-0 visits) to maintenance therapy (the average of values from the week 9 and 12 visits) obtained by in-person visits. This study was designed to detect a 2.7-unit treatment difference in scores. The secondary end points, assessed hierarchically, were the proportion of patients who achieved treatment success on the Patient Global Impression of Change (PGIC) and on the Clinical Global Impression of Change (CGIC), the change in 36-Item Short Form- physical functioning subscale score (SF-36), and the change in the Berg Balance Test. Ninety patients with Huntington disease (mean age, 53.7 years; 40 women [44.4%]) were enrolled. In the deutetrabenazine group, the mean total maximal chorea scores improved from 12.1 (95% CI, 11.2-12.9) to 7.7 (95% CI, 6.5-8.9), whereas in the placebo group, scores improved from 13.2 (95% CI, 12.2-14.3) to 11.3 (95% CI, 10.0-12.5); the mean between-group difference was -2.5 units (95% CI, -3.7 to -1.3) (P < .001). Treatment success, as measured by the PGIC, occurred in 23 patients (51

  12. Clinical benefits of home-based pulmonary rehabilitation in patients with chronic obstructive pulmonary disease.

    PubMed

    de Sousa Pinto, Juliana M; Martín-Nogueras, Ana M; Calvo-Arenillas, José I; Ramos-González, Jacinto

    2014-01-01

    To evaluate the benefits of home-based pulmonary rehabilitation (PR) in patients with severe and very severe chronic obstructive pulmonary disease (COPD). Randomized clinical trial involving 58 patients. Pulmonary function, quality of life evaluated by the Saint George Respiratory Questionnaire, breathlessness evaluated by the London Chest Activity of Daily Living Scale, and exercise tolerance evaluated by 6-minute walk distance were assessed at baseline and at 12 weeks. The program consisted of 2 weekly visits by a physiotherapist in the first 2 weeks, followed by visits twice a month, as well as weekly telephone calls. Training included breathing and stretching exercises and strength exercises (upper and lower limbs), along with endurance training, including walking, stair climbing, cycling, and treadmill walking, depending on available patient resources. The treatment group (TG; n = 23) and control group (CG; n = 18) completed the study. Following the intervention, no statistically significant differences were found in pulmonary function in the TG and CG. The TG exhibited statistically significant differences in the activity domain (P = .008), impact domain (P < .001), and total scores of the Saint George Respiratory Questionnaire (P < .001). In addition, the TG demonstrated statistically significant differences in all domains of the London Chest Activity of Daily Living Scale and no differences were observed in the CG after 12 weeks. There was a statistically significant difference in the 6-Minute Walk Distance in the TG after rehabilitation (P = .008). This study offers evidence that home-based PR promotes benefits in the quality of life, breathlessness in activities of daily living, and exercise capacity in patients with severe and very severe COPD. Home-based PR must be considered as part of the treatment for patients who live far from hospitals even in severe COPD.

  13. Dyslipidemia and cardiovascular disease risk profiles of patients attending an HIV treatment clinic in Harare, Zimbabwe.

    PubMed

    Zhou, Danai Tavonga; Kodogo, Vitaris; Chokuona, Kudzai Fortunate Vongai; Gomo, Exnevia; Oektedalen, Olav; Stray-Pedersen, Babill

    2015-01-01

    The chronic inflammation induced by human immunodeficiency virus (HIV) contributes to increased risk of coronary heart disease (CHD) in HIV-infected individuals. HIV-infected patients generally benefit from being treated with antiretroviral drugs, but some antiretroviral agents have side effects, such as dyslipidemia and hyperglycemia. There is general consensus that antiretroviral drugs induce a long-term risk of CHD, although the levels of that risk are somewhat controversial. The intention of this cross-sectional study was to describe the lipid profile and the long-term risk of CHD among HIV-positive outpatients at an HIV treatment clinic in Harare, Zimbabwe. Two hundred and fifteen patients were investigated (females n=165, mean age 39.8 years; males n=50; mean age 42.0 years). Thirty of the individuals were antiretroviral-naïve and 185 had been on antiretroviral therapy (ART) for a mean 3.9±3.4 years. All participants had average lipid and glucose values within normal ranges, but there was a small difference between the ART and ART-for total cholesterol (TC) and high-density lipoprotein (HDL). Those on a combination of D4T or ZDV/NVP/3TC and PI-based ART were on average oldest and had the highest TC levels. Framingham risk showed 1.4% prevalence of high CHD risk within the next ten years. After univariate analysis age, sex, TC/HDL ratio, HDL, economic earnings and systolic BP were associated with medium to high risk of CHD. After multivariate regression analysis and adjusting for age or sex only age, sex and economic earnings were associated with medium to high risk of CHD. There is small risk of developing CHD, during the next decade in HIV infected patients at an HIV treatment clinic in Harare.

  14. Clinical presentations and outcomes of patients with Ebola virus disease in Freetown, Sierra Leone.

    PubMed

    Ji, Ying-Jie; Duan, Xue-Zhang; Gao, Xu-Dong; Li, Lei; Li, Chen; Ji, Dong; Li, Wen-Gang; Wang, Li-Fu; Meng, Yu-Hua; Yang, Xiao; Ling, Bin-Fang; Song, Xue-Ai; Gu, Mei-Lei; Jiang, Tao; Koroma, She-Ku M; Bangalie, James; Duan, Hui-Juan

    2016-11-03

    Clinical and laboratory data were collected and analysed from patients with Ebola virus disease (EVD) in Jui Government Hospital in Freetown, Sierra Leone, where patients with EVD were received and/or treated from October 1, 2014 to March 21, 2015 during the West Africa EVD outbreak. The study admitted 285 patients with confirmed EVD and followed them up till the endpoint (recovery or death). EVD was confirmed by quantitative RT-PCR assays detecting blood Ebola virus (EBOV). Among the 285 lab-confirmed EVD cases in Jui Government Hospital, 146 recovered and 139 died, with an overall survival rate of 51.23 %. Patients under the age of 6 years had a lower survival rate (37.50 %). Most non-survivors (79.86 %) died within 7 days after admission and the mean hospitalization time for non-survivors was 5.56 ± 6.11 days. More than half survivors (63.69 %) turned blood EBOV negative within 3 weeks after admission and the mean hospitalization time for survivors was 20.38 ± 7.58 days. High blood viral load (≥10(6) copies/ml) was found to be predictive of the non-survival outcome as indicated by the Receiver Operating Characteristic (ROC) curve analysis. The probability of patients' survival was less than 15 % when blood viral load was greater than 10(6) copies/ml. Multivariate analyses showed that blood viral load (P = 0.005), confusion (P = 0.010), abdominal pain (P = 0.003), conjunctivitis (P = 0.035), and vomiting (P = 0.004) were factors independently associated with the outcomes of EVD patients. Most death occurred within 1 week after admission, and patients at the age of 6 or younger had a lower survival rate. Most surviving patients turned blood EBOV negative within 1-4 weeks after admission. Factors such as high blood viral load, confusion, abdominal pain, vomiting and conjunctivitis were associated with poor prognosis for EVD patients.

  15. Characteristics of stable chronic obstructive pulmonary disease patients in the pulmonology clinics of seven Asian cities

    PubMed Central

    Oh, Yeon-Mok; Bhome, Arvind B; Boonsawat, Watchara; Gunasekera, Kirthi Dias; Madegedara, Dushantha; Idolor, Luisito; Roa, Camilo; Kim, Woo Jin; Kuo, Han-Pin; Wang, Chun-Hua; Lan, Le Thi Tuyet; Loh, Li-Cher; Ong, Choo-Khoon; Ng, Alan; Nishimura, Masaharu; Makita, Hironi; Silverman, Edwin K; Lee, Jae Seung; Yang, Ting; Lin, Yingxiang; Wang, Chen; Lee, Sang-Do

    2013-01-01

    Background and objectives Chronic obstructive pulmonary disease (COPD) is responsible for significant morbidity and mortality worldwide. We evaluated the characteristics of stable COPD patients in the pulmonology clinics of seven Asian cities and also evaluated whether the exposure to biomass fuels and dusty jobs were related to respiratory symptoms, airflow limitation, and quality of life in the COPD patients. Methods This cross-sectional observational study recruited 922 COPD patients from seven cities of Asia. The patients underwent spirometry and were administered questionnaires about their exposure to cigarette smoking, biomass fuels, and dusty jobs in addition to respiratory symptoms and health related quality of life. Results Of the patients, there appeared to be variations from city to city in the history of exposure to biomass fuels and dusty jobs and also in respiratory symptoms of cough, phlegm, wheeze, and dyspnea. These symptoms were more frequent in those COPD patients with a history of exposure to biomass fuels than without and those with a history of exposure to dusty jobs than without (P < 0.01 for all comparisons). Airflow limitation was more severe in those COPD patients with a history of exposure to biomass fuels than without (52.2% predicted versus 55.9% of post-bronchodilator forced expiratory volume in 1 second [FEV1], P = 0.009); quality of life was poorer in those with exposure to biomass fuels than without (40.4 versus 36.2 of the St George’s Respiratory Questionnaire [SGRQ] total score, P = 0.001). Airflow limitation was more severe in those COPD patients with a history of exposure to dusty jobs than without (51.2% predicted versus 57.3% of post-bronchodilator FEV1, P < 0.001); quality of life was poorer in those with dusty jobs than without (41.0 versus 34.6 of SGRQ score, P = 0.006). Conclusion In Asian cities, the characteristics of COPD patients vary and the history of exposure to biomass fuels or dusty jobs was related to frequency

  16. [Clinical and cost effectiveness of rehabilitation programs including physical exercises for patients with ischemic heart disease under conditions of resort and outpatient clinics].

    PubMed

    Shikhova, E V; Guliaeva, S F; Tsarev, Iu K; Chervotkina, L A

    2010-01-01

    The objective of the present study was to evaluate clinical and cost effectiveness of rehabilitation programs including long-term physical training of moderate intensity intended for the management of patients with coronary heart disease (CHD) who had undergone acute coronary events; the programs were adapted to the treatment under conditions of spa resorts, dispensaries, and outpatient clinics. It was shown that rehabilitation of patients presenting with CDH with the use of moderately intensive physical exercises during a long period enhances the effectiveness of application of the available funds due to improved clinical course of coronary heart disease, tolerance of physical load, and quality of life.

  17. Factor analysis of laboratory and clinical measurements of dyspnea in patients with chronic obstructive pulmonary disease.

    PubMed

    Nguyen, Huong Q; Altinger, Julie; Carrieri-Kohlman, Virginia; Gormley, Jenny M; Stulbarg, Michael S

    2003-02-01

    The primary purpose of this study was to determine if there are three distinct factors representing ratings of dyspnea during laboratory exercise, clinical ratings of dyspnea, and pulmonary function in patients with moderate to severe chronic obstructive pulmonary disease (COPD) (n = 92) using factor analysis. Subjects (mean age 66 +/- 7 yrs; FEV1% predicted 44.7 +/- 14.0) were randomly assigned to one of three treatment groups to test the effects of three education and exercise training programs. Outcomes were evaluated at baseline and at 2 months after the intervention. Dyspnea ratings with laboratory exercise (SOB) were measured during incremental (ITT) and endurance (ETT) treadmill tests, and a six-minute walk (6MW) using the modified Borg scale. Clinical measures of dyspnea were measured with the Baseline and Transitional Dyspnea Index (BDI/TDI), UCSD Shortness of Breath Questionnaire (SOBQ), Medical Research Council Dyspnea Scale (MRC), Dyspnea subscale of the Chronic Respiratory Questionnaire (CRQ-D), and a global dyspnea question. Pulmonary function parameters included FEV1% predicted, FEV1/FVC, and RV/TLC. The factor analysis yielded three factors that accounted for 58.7% of the total variance in the data: Factor 1, "Dyspnea with Laboratory Exercise" comprised ETT SOB end, ETT SOB isotime, ITTSOB end, ITT SOB isotime, ITT SOB/Time and ETT SOB/Time. Factor 2, "Clinical Dyspnea," comprised 6MW SOB, 6MW SOB/Feet, BDI, SOBQ, MRC, Global SOB, CRQ-D. Measures of airway resistance (FEV1% predicted, FEV1/FVC) and hyperinflation (RV/TLC) loaded on a third factor, "Pulmonary Function." An additional post hoc factor analysis with post-intervention data provided similar results. The Global SOB question and ITT SOB isostage variables were relatively more sensitive to change compared to the other outcome variables. We conclude that pulmonary function, clinical ratings of dyspnea, and laboratory ratings of dyspnea are three separate and independent factors and should be

  18. Genetic and Clinical Analysis of ABCA4-Associated Disease in African American Patients

    PubMed Central

    Zernant, Jana; Collison, Frederick T; Lee, Winston; Fishman, Gerald A; Noupuu, Kalev; Yuan, Bo; Cai, Carolyn; Lupski, James R; Yannuzzi, Lawrence A; Tsang, Stephen H; Allikmets, Rando

    2014-01-01

    Autosomal recessive Stargardt disease (STGD1) is caused by hundreds of mutations in the ABCA4 gene, which are often specific to racial and ethnic groups. Here, we investigated the ABCA4 variation and their phenotypic expression in a cohort of 44 patients of African American descent, a previously under-characterized racial group. Patients were screened for mutations in ABCA4 by next-generation sequencing and array-comparative genomic hybridization (aCGH), followed by analyses for pathogenicity by in silico programs. Thorough ophthalmic examination was performed on all patients. At least two (expected) disease-causing alleles in the ABCA4 gene were identified in 27 (61.4%) patients, one allele in 11 (25%) patients, and no ABCA4 mutations were found in six (13.6%) patients. Altogether, 39 different disease-causing ABCA4 variants, including seven new, were identified on 65 (74%) chromosomes, most of which were unique for this racial group. The most frequent ABCA4 mutation in this cohort was c.6320G>A (p.(R2107H)), representing 19.3% of all disease-associated alleles. No large copy number variants were identified in any patient. Most patients reported later onset of symptoms. In summary, the ABCA4 mutation spectrum in patients of West African descent differs significantly from that in patients of European descent, resulting in a later onset and “milder” disease. PMID:25066811

  19. Awareness and Knowledge of Emergent Ophthalmic Disease Among Patients in an Internal Medicine Clinic.

    PubMed

    Uhr, Joshua H; Mishra, Kapil; Wei, Chen; Wu, Albert Y

    2016-04-01

    Emergent ophthalmic disease can lead to permanent visual impairment or blindness if medical attention is delayed. Awareness and knowledge of emergent ophthalmic disease may be important for early medical presentation and maximization of visual prognosis in some cases. To assess public awareness and knowledge of 4 emergent ophthalmic diseases. This cross-sectional study was conducted from June 1 to July 30, 2015, in the waiting rooms of the outpatient internal medicine resident clinic at Mount Sinai Hospital. A written survey was administered to evaluate awareness and knowledge of retinal detachment, acute angle-closure glaucoma, giant cell arteritis, and central retinal artery occlusion. Awareness of each disease was assessed by whether participants knew what the diseases were (yes or no). Knowledge was evaluated by responses to 3 questions for each disease, including 1 question about basic pathophysiologic features, 1 question about basic symptoms, and 1 question about basic treatment options. All English-speaking patients who were physically and cognitively able to fill out the survey without assistance were considered eligible and offered the opportunity to participate during times of survey distribution; 237 completed the survey. Demographic information, including age, sex, race, income, and educational level, was collected. Data were assessed from August 1 to 7, 2015. Awareness of each ophthalmic disease was determined by the proportion of respondents who answered yes, and knowledge was determined by the proportion of aware respondents who answered the knowledge questions correctly. Two hundred thirty-seven patients (of 227 who gave complete demographic information, 76 men [33.5%], 151 women [66.5%], and mean [SD] age, 51.3 [16.8] years) completed the survey. Awareness of each of the diseases studied was low; 61 of 220 respondents (27.7%; 95% CI, 21.8%-33.6%) were aware of retinal detachment; 32 of 219 respondents (14.6%; 95% CI, 9.9%-19.3%), acute angle

  20. Activating Patients for Sustained Chronic Disease Self-Management: Thinking Beyond Clinical Outcomes.

    PubMed

    Dye, Cheryl J; Williams, Joel E; Evatt, Janet H

    2016-04-01

    This article describes the impact of an 8-week community program implemented by trained volunteers on the hypertension self-management of 185 patients who were batch randomized to intervention or wait-list control groups. Compared with control group participants, a higher proportion of treatment group participants moved from the cognitive to behavioral stages of motivational readiness for being physically active (P < .001), practicing healthy eating habits (P = .001), handling stress well (P = .001), and living an overall healthy lifestyle (P = .003). They also demonstrated a greater average increase in perceived competence for self-management, F(1.134) = 4.957, P = .028, η2 = .036, and a greater increase in mean hypertension-related knowledge, F(1.160) = 16.571, P < .0005, η(2) = .094. Enduring lifestyle changes necessary for chronic disease self-management require that psychosocial determinants of health behavior are instilled, which is typically beyond standard medical practice. We recommend peer-led, community-based programs as a complement to clinical care and support the increasing health system interest in promoting population health beyond clinical walls. © The Author(s) 2016.

  1. Home infusion of intravenous velaglucerase alfa: Experience from pooled clinical studies in 104 patients with type 1 Gaucher disease.

    PubMed

    Elstein, Deborah; Burrow, T Andrew; Charrow, Joel; Giraldo, Pilar; Mehta, Atul; Pastores, Gregory M; Lee, Hak-Myung; Mellgard, Björn; Zimran, Ari

    The introduction of a home therapy option during clinical trials of velaglucerase alfa in patients with type 1 Gaucher disease marked the first time that home infusions have been permitted during a clinical trial for an investigational drug for Gaucher disease. Home infusions were an available option in 4 open-label velaglucerase alfa clinical studies to eligible patients who received their initial infusions at a clinic. Patients who participated in the home therapy option and received at least 10% of their infusions at home (n=100) received a range of 11.6%-100% of their scheduled infusions at home (median 87.5%), excluding infusions received at the clinic during protocol-mandated visits. The length of time over which individual patients received home therapy ranged from 13days to 4.56years (median 0.57years). During the time that home therapy was available, 2904 of 3572 (81.3%) infusions were administered at home. Ten patients experienced 62 infusion-related adverse events (IRAEs) during 38 home infusions, with malaise, pain, hypertension, fatigue, and headache being reported most frequently. No notable differences were found between the type and severity of IRAEs experienced at home and those experienced at the clinic. Home infusions administered by trained and qualified medical personnel were successfully introduced into the velaglucerase alfa clinical development program, and fewer than 10% of patients experienced IRAEs in the home setting. Local labeling and practice guidelines should be consulted for administration of velaglucerase alfa infusions at home.

  2. Clinical pharmacy cardiac risk service for managing patients with coronary artery disease in a health maintenance organization.

    PubMed

    Sandhoff, Brian G; Nies, Leslie K; Olson, Kari L; Nash, James D; Rasmussen, Jon R; Merenich, John A

    2007-01-01

    A clinical pharmacy service for managing the treatment of coronary artery disease in a health maintenance organization is described. Despite the proven benefits of aggressive risk factor modification for patients with coronary artery disease (CAD), there remains a treatment gap between consensus- and evidence-based recommendations and their application in patient care. In 1998, Kaiser Permanente of Colorado developed the Clinical Pharmacy Cardiac Risk Service (CPCRS) to focus on the long-term management of patients with CAD to improve clinical outcomes. The primary goals of the CPCRS are to increase the number of CAD patients on lipid-lowering therapy, manage medications shown to decrease the risk of future CAD-related events, assist in the monitoring and control of other diseases that increase cardiovascular risk, provide patient education and recommendations for nonpharmacologic therapy, and act as a CAD information resource for physicians and other health care providers. Using an electronic medical record and tracking database, the service works in close collaboration with primary care physicians, cardiologists, cardiac rehabilitation nurses, and other health care providers to reduce cardiac risk in the CAD population. Particular attention is given to dyslipidemia, blood pressure, diabetes mellitus, and tobacco cessation. Treatment with evidence-based regimens is initiated and adjusted as necessary. Over 11,000 patients are currently being followed by the CPCRS. A clinical pharmacy service in a large health maintenance organization provides cardiac risk reduction for patients with CAD and helps close treatment gaps that may exist for these patients.

  3. Simple blood tests as predictive markers of disease severity and clinical condition in patients with venous insufficiency.

    PubMed

    Karahan, Oguz; Yavuz, Celal; Kankilic, Nazim; Demirtas, Sinan; Tezcan, Orhan; Caliskan, Ahmet; Mavitas, Binali

    2016-09-01

    Chronic venous insufficiency (CVI) is a progressive inflammatory disease. Because of its inflammatory nature, several circulating markers were investigated for predicting disease progression. We aimed to investigate simple inflammatory blood markers as predictors of clinical class and disease severity in patients with CVI. Eighty patients with CVI were divided into three groups according to clinical class (grade 1, 2 and 3) and score of disease severity (mild, moderate and severe). The basic inflammatory blood markers [neutrophil, lymphocyte, mean platelet volume (MPV), white blood cell (WBC), platelet, albumin, D-dimer, fibrinogen, fibrinogen to albumin ratio, and neutrophil to lymphocyte ratio] were investigated in each group. Serum neutrophil, lymphocyte, MPV, platelet count, D-dimer and neutrophil to lymphocyte ratio levels were similar among the groups (P > 0.05). Although the serum WBC levels were significant in the clinical severity groups (P < 0.05), it was useless to separate each severity class. However, albumin, fibrinogen and the fibrinogen to albumin ratio were significant predictors of clinical class and disease severity. Especially, the fibrinogen to albumin ratio was detected as an independent indicator for a clinical class and disease severity with high sensitivity and specificity (75% sensitivity and 87.5% specificity for clinical class and 90% sensitivity and 88.3% specificity for disease severity). Serum fibrinogen and albumin levels can be useful parameters to determine clinical class and disease severity in patients with CVI. Moreover, the fibrinogen to albumin ratio is a more sensitive and specific predictor of the progression of CVI.

  4. Clinical utility of esomeprazole for treatment of gastroesophageal reflux disease in pediatric and adolescent patients

    PubMed Central

    Cardile, Sabrina; Romano, Claudio

    2012-01-01

    Gastroesophageal reflux is a common condition in the pediatric population, with an increasing incidence in the last few years. It can be defined as an effortless retrograde movement of gastric contents into the esophagus related to complex multifactorial pathogenesis, involving anatomical, hormonal, environmental, and genetic factors. In some cases, it may be associated with esophageal or extraesophageal symptoms (heartburn and regurgitation), and is defined as gastroesophageal reflux disease (GERD). The therapeutic approach to gastroesophageal reflux in infants and children is often conservative, including changes in lifestyle (eg, posture and thickening of meals). If these children remain symptomatic after lifestyle changes (nutrition, feeding, and positional modification), or present with clinical red flags (poor weight gain, recurrent respiratory symptoms, or hematemesis) and complications of GERD (esophagitis, bleeding, stricture, Barrett’s esophagus, or adenocarcinoma) it may be necessary to set up a proper diagnostic protocol. Proton pump inhibitors have been recommended as the most effective acid suppression therapy for adults and pediatric patients. Esomeprazole, the S-isomer of omeprazole, is the only single-isomer proton pump inhibitor available. The paper assesses the safety and tolerability of esomeprazole in pediatric and adolescent patients. PMID:24600284

  5. Quality of care in patients with psoriasis: an initial clinical study of an international disease management programme.

    PubMed

    de Korte, J; Van Onselen, J; Kownacki, S; Sprangers, M A G; Bos, J D

    2005-01-01

    Patients with psoriasis have to cope with their disease for many years or even throughout their entire life. To provide optimal care, a disease management programme was developed. This programme consisted of disease education, disease management training, and psychological support, together with topical treatment. To test a disease management programme in dermatological practice, to assess patients' satisfaction with this programme, and adherence to topical treatment. Additionally, disease severity and quality of life were assessed. An initial clinical investigation was conducted in 10 European treatment centres. A total of 330 patients were included. Patient satisfaction, adherence, disease severity and quality of life were measured with study-specific and standardized self-report questionnaires. Patients reported a high degree of satisfaction with the programme, and a high degree of adherence to topical treatment. Disease severity and quality of life significantly improved. The programme was well received by the participating professionals. The disease management programme was found to be a useful tool in the management of psoriasis, providing patients with relief from the burden of psoriasis in everyday life. A full-scale evaluation is recommended.

  6. Bringing Stability to the Chronic Obstructive Pulmonary Disease Patient: Clinical and Pharmacological Considerations for Frequent Exacerbators.

    PubMed

    Gulati, Swati; Wells, J Michael

    2017-03-03

    Acute exacerbations of chronic obstructive pulmonary disease (AECOPD) are critical events associated with an accelerated loss of lung function, increased morbidity, and excess mortality. AECOPD are heterogeneous in nature and this may directly impact clinical decision making, specifically in patients with frequent exacerbations. A 'frequent exacerbator' is a sub-phenotype of chronic obstructive pulmonary disease (COPD) and is defined as an individual who experiences two or more moderate-to-severe exacerbations per year. This distinct subgroup has higher mortality and accounts for more than half of COPD-related hospitalizations annually. Thus, it is imperative to identify individuals at risk for frequent exacerbations and choose optimal strategies to minimize risk for these events. New paradigms for using combination inhalers and the introduction of novel oral compounds provide expanded treatment options to reduce the risk and frequency of exacerbations. The goals of managing frequent exacerbators or patients at risk for AECOPD are: (1) maximizing bronchodilation; (2) reducing inflammation; and (3) targeting specific molecular pathways implicated in COPD and AECOPD pathogenesis. Novel inhaler therapies including combination long-acting muscarinic agents plus long-acting beta agonists show promising results compared with monotherapy or a long-acting beta agonist inhaled corticosteroid combination in reducing exacerbation risk among individuals at risk for exacerbations and among frequent exacerbators. Likewise, oral medications including macrolides and phosphodiesterase-4 inhibitors reduce the risk for AECOPD in select groups of individuals at high risk for exacerbation. Future direction in COPD management is based on the identification of various subtypes or 'endotypes' and targeting therapies based on their pathophysiology. This review describes the impact of AECOPD and the challenges posed by frequent exacerbators, and explores the rationale for different

  7. The impact of a clinic move on vulnerable patients with chronic disease: a Geographic Information Systems (GIS) analysis.

    PubMed

    Bazemore, Andrew; Diller, Philip; Carrozza, Mark

    2010-01-01

    Changing locations disrupts the populations served by primary health care clinics, and such changes may differentially affect access to care for vulnerable populations. Online geographic information systems mapping tools were used to define how the relocation of a family medicine center impacted access to care for black and Hispanic patients with chronic disease. Maps created from practice management data revealed a distinct shift in black and Hispanic patients with chronic disease being served in the new location. Geographic information systems tools are valuable aids in defining changing service areas of primary health care clinics.

  8. Evaluation of clinical pharmacist's interventions in an infectious diseases ward and impact on patient's direct medication cost.

    PubMed

    Khalili, Hossein; Karimzadeh, Iman; Mirzabeigi, Parastoo; Dashti-Khavidaki, Simin

    2013-04-01

    A clinical pharmacist is a key member of the antimicrobial multidisciplinary team involved in patients' pharmacotherapy monitoring. The aim of this study was to determine the frequency and type of medication errors, the type of clinical pharmacy interventions, acceptance of pharmacist interventions by health-care provider team, nursing staff satisfaction with clinical pharmacy services, and the probable impact of clinical pharmacy interventions on decreasing direct medication costs at an infectious diseases ward in Iran. All clinical pharmacist interventions such as preventing medication errors were recorded in a previously designed pharmacotherapy monitoring forms. Direct medication cost of patients admitted during the study period was compared with that of subjects hospitalized at the same ward during the year before the intervention period to determine the impact of clinical pharmacy interventions on direct medication costs. The 3 most frequent medication error types were incorrect dose (35.5%), omission error (24.3%), and incorrect medication (14.3%). The mean number of clinical pharmacist intervention per patient was 3.2. Forty percent of clinical pharmacists' interventions are moderate to major clinical significant. Thirty nine percent of clinical pharmacist's interventions had moderate to major financial benefits in present study. The direct medication cost per patient was decreased about 3.8% following clinical pharmacist's interventions. Our data demonstrated that incorrect dose was the most frequent medication error in the infectious diseases ward. Major portion of clinical pharmacist interventions were accepted by physicians and nursing staff. Clinical pharmacist interventions non-significantly decreased the direct medication cost of patients. Copyright © 2012 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

  9. Sequential Waves of Gene Expression in Patients with Clinically Defined Dengue Illnesses Reveal Subtle Disease Phases and Predict Disease Severity

    PubMed Central

    Sun, Peifang; García, Josefina; Comach, Guillermo; Vahey, Maryanne T.; Wang, Zhining; Forshey, Brett M.; Morrison, Amy C.; Sierra, Gloria; Bazan, Isabel; Rocha, Claudio; Vilcarromero, Stalin; Blair, Patrick J.; Scott, Thomas W.; Camacho, Daria E.; Ockenhouse, Christian F.; Halsey, Eric S.; Kochel, Tadeusz J.

    2013-01-01

    Background Dengue virus (DENV) infection can range in severity from mild dengue fever (DF) to severe dengue hemorrhagic fever (DHF) or dengue shock syndrome (DSS). Changes in host gene expression, temporally through the progression of DENV infection, especially during the early days, remains poorly characterized. Early diagnostic markers for DHF are also lacking. Methodology/Principal Findings In this study, we investigated host gene expression in a cohort of DENV-infected subjects clinically diagnosed as DF (n = 51) and DHF (n = 13) from Maracay, Venezuela. Blood specimens were collected daily from these subjects from enrollment to early defervescence and at one convalescent time-point. Using convalescent expression levels as baseline, two distinct groups of genes were identified: the “early” group, which included genes associated with innate immunity, type I interferon, cytokine-mediated signaling, chemotaxis, and complement activity peaked at day 0–1 and declined on day 3–4; the second “late” group, comprised of genes associated with cell cycle, emerged from day 4 and peaked at day 5–6. The up-regulation of innate immune response genes coincided with the down-regulation of genes associated with viral replication during day 0–3. Furthermore, DHF patients had lower expression of genes associated with antigen processing and presentation, MHC class II receptor, NK and T cell activities, compared to that of DF patients. These results suggested that the innate and adaptive immunity during the early days of the disease are vital in suppressing DENV replication and in affecting outcome of disease severity. Gene signatures of DHF were identified as early as day 1. Conclusions/Significance Our study reveals a broad and dynamic picture of host responses in DENV infected subjects. Host response to DENV infection can now be understood as two distinct phases with unique transcriptional markers. The DHF signatures identified during day 1–3 may have

  10. Towards rational approaches of health care utilization in complex patients: an exploratory randomized trial comparing a novel combined clinic to multiple specialty clinics in patients with renal disease-cardiovascular disease-diabetes.

    PubMed

    Weber, Catherine; Beaulieu, Monica; Djurdjev, Ognjenka; Er, Lee; Taylor, Paul; Ignaszewski, Andrew; Burnett, Shelley; Levin, Adeera

    2012-10-01

    Optimal utilization of health care resources for patients with chronic conditions is an increasing focus of health care policy researchers and clinicians. Kidney disease, diabetes (DM) and cardiovascular disease (CVD) often coexist within one individual, but current systems are designed to manage individual conditions. We sought to examine if streamlining medical care of complex patients (two or more conditions) is associated with similar, worse or improved outcomes using a randomized controlled study design. Patients attending a kidney care clinic (KCC) and at least one other specialty clinic of interest (DM, CVD) were randomly assigned to either the 'combined clinic (CC)' arm, where resources from all three were integrated into one clinic, or to the 'standard care' arm with continued attendance at multiple specialty clinics (MC), including the KCC. The primary outcome was hospitalization rate and sample size was calculated based on non-inferiority. Of 150 subjects enrolled, 11 subjects exited before study commencement: 139 remained for final analysis. Other than older age in the MC group (P = 0.009), the demographics were comparable. Hospitalization rates were not different (95% CI for the difference: 0.013-0.207; P = 0.03). Similar proportions in each group achieved clinical and laboratory targets. Mortality (13%) and dialysis (32%) rates were the same between groups. Differences in the cost of clinic visits alone were $86,400 per year in favor of the CC. Medical care of complex patients may be delivered in a single combined specialty clinic as compared to multiple disease specific clinics without compromising patient care or important health outcomes, with demonstrable outpatient costs savings.

  11. Estimating the Ratio of Patients with a Certain Disease Between Hospitals for the Allocation of Patients to Clinical Trials Using Health Insurance Claims Data in Japan.

    PubMed

    Takeda, Toshihiro; Mihara, Naoki; Murata, Taizo; Shimai, Yoshie; Okada, Katsuki; Manabe, Shiro; Matsumura, Yasushi

    2016-01-01

    In clinical trials, investigating the ratio of patients with each disease who are treated in a hospital is important for determining the number of patients who are allocated to hospitals. The Japanese health insurance claims data includes standardized disease and medicine data. However, the disease data has some problems in terms of reliability, because the healed diseases are sometimes not deleted or because a disease that a patient does not actually have is registered to claim the cost of the examination. On the other hand, therapeutic medicines are administered to target particular diseases. In this study, we developed a system for estimating the number of patients with each disease using the disease data and the therapeutic medicine data. We converted the ICD-10 code to a 4-grade classification code so that we could predict the diseases in the shallow layer (e.g. gastrointestinal disease) when it was difficult to predict the precise diseases in the deep layer (e.g. gastric ulcers). A table showing the disease code and the corresponding therapeutic medicine code was provided by the Japan Pharmaceutical Information Center (JAPIC). We calculated the disease probability score from the diseases and therapeutic medicines and recorded the predicted disease. For the system evaluation, we used the health insurance claims data from Osaka University Hospital for January 2015. A total of 58,526 diseases were predicted from the health insurance claims data of 18,393 patients. One hundred twenty patients were randomly extracted for use in a chart review that was performed by an expert physician. Two hundred twenty-four of 329 predicted diseases, were correctly predicted; 56 were reasonably predicted, and 49 were incorrectly predicted. The main disease was correctly predicted in 71 patients. In conclusion, we could estimate the number of patients with each disease using the health insurance claims data with a certain degree of accuracy.

  12. Correlates of condom coupon redemption among urban sexually transmitted disease clinic patients.

    PubMed

    Witte, S; el-Bassel, N; Krishnan, S; Schilling, R; Bidassie, B

    1999-01-01

    This study expands upon coupon distribution strategies used to measure male condom acquisition in HIV/AIDS prevention by incorporating both female and male condoms and examining factors related to coupon redemption among urban STD clinic patients.

  13. Clinical and genetic profile of patients with seronegative coeliac disease: the natural history and response to gluten-free diet.

    PubMed

    Dore, Maria Pina; Pes, Giovanni Mario; Dettori, Ivana; Villanacci, Vincenzo; Manca, Alessandra; Realdi, Giuseppe

    2017-01-01

    Patients with clinical, genetic and histological features of coeliac disease (CD), but negative for serological markers, pose a significant clinical problem. The aim of this study was to outline a specific profile, and to evaluate the natural history and response to gluten-free diet (GFD) of patients with seronegative CD. patients with duodenal mucosa damage Marsh I, II and III stages, HLA DQ2/DQ8 haplotype and clinical features suggestive of CD, but negative for CD serology, were defined as seronegative CD patients. Other common causes of duodenal mucosa damage were excluded. HLA-DR and DQ genotype/haplotype between all Marsh stages of patients with seronegative and seropositive CD were compared. Clinical features, laboratory testing and histological findings were evaluated after a GFD and a gluten rechallenge. A long follow-up period was available. 48 patients fulfilled diagnostic criteria over a 4-year period. Clinical phenotype and HLA-DR and DQ frequencies between patients with seronegative and seropositive CD was similar. However, Marsh I stage was more prevalent in seronegative patients (42% vs 22%; p<0.05). After a 1-year GFD trial, clinical symptoms, histological features and laboratory testing improved in 40 patients and worsened in those who underwent a 6-months gluten challenge. Five patients with seronegative CD (25%) experienced the occurrence of autoimmune diseases during a median follow-up of 133 months (range 72-192). Patients with seronegative CD did not display a specific profile. They benefitted from GFD as patients with seropositive CD. Waiting for more sensitive serological markers, the diagnosis of seronegative CD remains a diagnosis of exclusion.

  14. Clinical and genetic profile of patients with seronegative coeliac disease: the natural history and response to gluten-free diet

    PubMed Central

    Dore, Maria Pina; Pes, Giovanni Mario; Dettori, Ivana; Villanacci, Vincenzo; Manca, Alessandra; Realdi, Giuseppe

    2017-01-01

    Objectives Patients with clinical, genetic and histological features of coeliac disease (CD), but negative for serological markers, pose a significant clinical problem. The aim of this study was to outline a specific profile, and to evaluate the natural history and response to gluten-free diet (GFD) of patients with seronegative CD. Methods patients with duodenal mucosa damage Marsh I, II and III stages, HLA DQ2/DQ8 haplotype and clinical features suggestive of CD, but negative for CD serology, were defined as seronegative CD patients. Other common causes of duodenal mucosa damage were excluded. HLA–DR and DQ genotype/haplotype between all Marsh stages of patients with seronegative and seropositive CD were compared. Clinical features, laboratory testing and histological findings were evaluated after a GFD and a gluten rechallenge. A long follow-up period was available. Results 48 patients fulfilled diagnostic criteria over a 4-year period. Clinical phenotype and HLA−DR and DQ frequencies between patients with seronegative and seropositive CD was similar. However, Marsh I stage was more prevalent in seronegative patients (42% vs 22%; p<0.05). After a 1-year GFD trial, clinical symptoms, histological features and laboratory testing improved in 40 patients and worsened in those who underwent a 6-months gluten challenge. Five patients with seronegative CD (25%) experienced the occurrence of autoimmune diseases during a median follow-up of 133 months (range 72–192). Conclusions Patients with seronegative CD did not display a specific profile. They benefitted from GFD as patients with seropositive CD. Waiting for more sensitive serological markers, the diagnosis of seronegative CD remains a diagnosis of exclusion. PMID:28944073

  15. Review of the chronology of clinical manifestations in 60 patients with Behçet's disease.

    PubMed

    Alpsoy, Erkan; Donmez, Levent; Bacanli, Ali; Apaydin, Cemil; Butun, Bulent

    2003-01-01

    The natural course of Behçet's disease is not fully known. The aim of the present study was to determine the occurrence of the symptoms retrospectively in chronologic order in patients with Behçet's disease, diagnosed according to the criteria of the International Study Group for Behçet's Disease. A total of 60 consecutive patients (29 male and 31 female; aged 35.87 +/- 9.84 years) were involved in the study. The symptoms of the disease were retrospectively recorded in the time order of the manifestations per patient. Oral ulcer was the most commonly observed onset manifestation (51 of 60 patients: 85%), followed by genital ulcer (13 of 60 patients: 21.7%) and articular symptoms (10 of 60 patients: 16.7%). The duration between the oral ulcer and the fulfillment of diagnostic criteria was calculated to be 3.77 +/- 4.43 years. The same duration was 2.50 +/- 4.74 and 2.11 +/- 3.44 years for genital ulcer and articular symptoms, respectively. The duration between the time point of fulfillment of diagnostic criteria and the diagnosis (2.83 +/- 2.3 years) was found to be longer in female patients (3.2 +/- 2.5 years). The duration was also longer in patients having only mucocutaneous lesions (3.18 +/- 2.5 years) than in patients having serious organ involvement such as eye disease (1.63 +/- 0.7 years; p < 0.05). Our study indicates that oral ulcer is the onset manifestation in the majority of the patients and the disease is often diagnosed with a delay of several years after the appearance of the onset sign. Copyright 2003 S. Karger AG, Basel

  16. Impact of certolizumab pegol on patient-reported outcomes in rheumatoid arthritis and correlation with clinical measures of disease activity.

    PubMed

    Pope, Janet; Bingham, Clifton O; Fleischmann, Roy M; Dougados, Maxime; Massarotti, Elena M; Wollenhaupt, Jürgen; Duncan, Benjamin; Coteur, Geoffroy; Weinblatt, Michael E

    2015-11-27

    The effect of certolizumab pegol (CZP) on patient-reported outcomes (PROs) was investigated in 1063 patients with rheumatoid arthritis (RA) from the REALISTIC trial (double-blind, placebo-controlled to week 12, open-label to week 28; randomized 4:1 [CZP:placebo]). Correlations between PROs and RA signs and symptoms, and the relative efficacy of these measures, were examined. Adults with RA and an inadequate response to at least one disease-modifying antirheumatic drug were enrolled. PROs assessed included physical function (using the Health Assessment Questionnaire-Disability Index), pain, fatigue, sleep disturbance, Patient Global Assessment of Disease Activity (PtGA), Routine Assessment of Patient Index Data 3 (RAPID3), and Rheumatoid Arthritis Disease Activity Index (RADAI). Early significant and clinically meaningful improvements in all PROs were observed to week 12 with CZP vs. placebo and were maintained to the end of the trial (week 28). At week 12, up to one-third more CZP patients showed improvements compared with placebo that were greater than or equal to the minimal clinically important difference (MCID) in fatigue, sleep problems, pain, PtGA, RADAI, and RAPID3. The changes in PROs were correlated with clinical measures of disease activity, including the Disease Activity Score in 28 joints using C-reactive protein as well as tender and swollen joint counts. Rapid improvements in PROs were seen in patients with RA treated with CZP. The magnitude of improvement exceeded the MCID in multiple domains and demonstrated that CZP improves aspects of health-related quality of life that are meaningful to patients and superior to placebo. PROs provide information complementary to clinical outcomes in assessment of treatment benefits. ClinicalTrials.gov identifier: NCT00717236 . Registered on 15 July 2008.

  17. Clinical evaluation of periodontal disease in patients with rheumatoid arthritis: A cross-sectional study.

    PubMed

    Pons-Fuster, Alvaro; Rodríguez Agudo, Consuelo; Galvez Muñoz, Pepe; Saiz Cuenca, Encarna; Pina Perez, Francisca M; Lopez-Jornet, Pia

    2015-10-01

    The aim of this study was to determine the presence of periodontal disease among patients with rheumatoid arthritis (RA) in comparison with a control group. The study included 44 patients diagnosed with RA according to American Rheumatism Association (ARA) criteria, who were attending the Morales Meseguer Hospital Rheumatology Service (Murcia, Spain), and 41 control subjects. Patients younger than 18 years or patients suffering systemic diseases that could affect the immune system were excluded. Age, sex, smoking habits, alcohol consumption, and body mass index were registered. Each patient underwent a full periodontal examination. Bleeding on probing was significantly greater in the RA group (0.9 ± 0.36) than the control (P < .001). The Plaque Index was significantly higher in the RA group (0.76 ± 0.34) versus the control group (0.55 ± 0.2) (P < .001). RA patients showed a 0.13 increased risk of periodontal disease (95% confidence interval, 0.05-0.37). Patients with RA suffered a higher risk of periodontal disease and for this reason these patients must be instructed to intensify their oral hygiene regimes.

  18. Clinical outcomes in managed-care patients with coronary heart disease treated aggressively in lipid-lowering disease management clinics: the alliance study.

    PubMed

    Koren, Michael J; Hunninghake, Donald B

    2004-11-02

    This study sought to determine if an aggressive, focused low-density lipoprotein cholesterol (LDL-C)-lowering strategy was superior to usual care for coronary heart disease (CHD) patients enrolled in health maintenance organization or Veterans Administration settings. Statin therapy benefits are well established. No prospective, randomized studies have tested strategies to optimize these benefits in a "real-world" setting. A total of 2,442 CHD patients with hyperlipidemia were randomized to either an aggressive treatment arm using atorvastatin or usual care and followed for 51.5 months on average. Atorvastatin-group patients were titrated to LDL-C goals of <80 mg/dl (2.1 mmol/l) or a maximum atorvastatin dose of 80 mg/day. Usual-care patients received any treatment deemed appropriate by their regular physicians. End point assessments were complete in 958 atorvastatin-group and 941 usual-care patients. Partial assessments occurred in 259 patients in the atorvastatin group and 284 patients in the usual care group who did not complete four years of study participation because of adverse events, withdrawn consent, or follow-up loss. The primary efficacy parameter was time to first cardiovascular event. A total of 289 (23.7%) patients in the atorvastatin group compared with 333 (27.7%) patients in the usual care group experienced a primary outcome (hazard ratio, 0.83; 95% confidence interval 0.71 to 0.97, p = 0.02). This reduction in morbidity was largely due to fewer non-fatal myocardial infarctions (4.3% vs. 7.7%, p = 0.0002). Levels of LDL-C were reduced more (34.3% vs. 23.3%, p < 0.0001) and National Cholesterol Education Program goals (LDL-C <100 mg/dl) more likely met at end-of-study visits (72.4% vs. 40.0%) in patients receiving atorvastatin compared with those receiving usual care. An aggressive, focused statin therapy management strategy outperformed usual care in health maintenance organization and Veterans Administration clinic patients with CHD.

  19. Drug-induced hypersensitivity syndrome: clinical and biologic disease patterns in 24 patients.

    PubMed

    Ben m'rad, Mona; Leclerc-Mercier, Stéphanie; Blanche, Philippe; Franck, Nathalie; Rozenberg, Flore; Fulla, Yvonne; Guesmi, Myriam; Rollot, Florence; Dehoux, Monique; Guillevin, Loïc; Moachon, Laurence

    2009-05-01

    Drug-induced hypersensitivity syndrome (DIHS), also called drug rash with eosinophilia and systemic symptoms (DRESS), is a severe reaction usually characterized by fever, rash, and multiorgan failure, occurring 1-8 weeks after drug introduction. It is an immune-mediated reaction involving macrophage and T-lymphocyte activation and cytokine release, although no consensus has been reached as to its etiology. The skin, hematopoietic system, and liver are frequently involved. DIHS can mimic severe sepsis, viral infection, adult-onset Still disease (AOSD), or lymphoproliferation.We describe 24 consecutive patients with DIHS who were hospitalized between September 2004 and March 2008. Criteria for inclusion in this observational study were suspected drug reaction, eosinophilia >or=500/microL and/or atypical lymphocytes, involvement of at least 2 organs (skin being 1 of them), with suggestive chronology and exclusion of other diagnoses. Our cohort of 12 women and 12 men had a median age of 49 years (range, 22-82 yr), and 11 had skin phototype V or VI. Patients with mild or no rash were immunocompromised (7/24)- defined as treatment with prednisone (>or=10 mg/d) and another immunosuppressant drug, or human immunodeficiency virus infection. All patients were febrile (>38 degrees C), 14 had localized or generalized edema, 7 had pharyngitis, 8 had lymphadenopathy, 22 had hepatitis, 4 had nephritis, 2 had noninfectious and nonlithiasic angiocholitis or cholecystitis. Ten patients were hypotensive, 5 of whom had associated laboratory signs and/or imaging findings suggestive of acute myocardial dysfunction. Half of the patients had hemogram abnormalities, including eosinophilia. Nine DIHS patients fulfilled the Fautrel criteria for AOSD diagnosis, including glycosylated ferritin <20% in 4/11, with or without laboratory characteristics of hemophagocytosis. Twenty DIHS episodes occurred during the less sunny months of October to March.We determined 25-hydroxyvitamin D3 (25[OH]D3

  20. Clinical predictors of disease progression in multiple sclerosis patients with relapsing onset in a nation-wide cohort.

    PubMed

    Alroughani, R A; Akhtar, S; Ahmed, S F; Al-Hashel, J Y

    2015-01-01

    Predicting disease progression over time is challenging despite the available literature data. To assess whether baseline clinical variables of MS patients would predict the conversion to progressive phase of the disease. Utilizing the national MS registry, patients who had relapsing onsets and had confirmed EDSS score at baseline and follow-up visits were included. Primary progressive MS and CIS patients were excluded. Clinical variables (gender, age at onset, disease duration, number of relapses, EDSS score) were collected. The end point was conversion to secondary progressive MS. Chi Square and multivariable logistic regression were used to determine the influence of clinical variables on disease progression. Data of 803 MS patients with relapsing onset were analyzed. Eighty five (10.6%) patients reached the end point. The risk of disease progression was significantly higher in men (p=0.015), in patients who developed MS≥40 years of age (p=0.041) and who had ≥3 relapses during their disease course (p<0.001). Spinal cord presentation at onset was predictive of progression (aOR=2.01; p=0.06) while optic neuritis at onset was associated with lower risk of progression (aOR=0.30; p=0.03). EDSS score at first visit did not influence disease progression when tested at 2 different cutoffs (EDSS<4 vs. ≥4 and EDSS<6 vs. ≥6) using multivariable logistic regression analysis (p=0.960 and p=0.866), respectively. Men and patients who presented at age 40 yeas or beyond had increased risk of MS progression. Spinal cord symptoms at onset and 3 or more relapses were predictive of progression.

  1. Breast cancer in patients treated for Hodgkin's disease: clinical and pathological analysis of 76 cases in 63 patients.

    PubMed

    Cutuli, B; Dhermain, F; Borel, C; de Larochefordiere, A; Graic, Y; de Lafontan, B; Dilhyudy, J M; Mignotte, H; Tessier, E; Tortochaux, J; N'Guyen, T; Bey, P; Le Mevel-Le Pourhiet, A; Velten, M; Arriagada, R

    1997-12-01

    In a retrospective multicentric analysis, 63 women treated between 1941 to 1988 for Hodgkin's disease (HD) subsequently developed 76 breast cancers (BC). The median age at diagnosis of HD was 26 years (range 7-67), and 22 women (35%) were 20 years old or less. Exclusive radiotherapy (RT) was used in 36 women (57%) and combined modalities with chemotherapy (CT) in 25 (39%). Breast cancer occurred after a median interval of 16 years (range 2-40) and the median age at diagnosis of the first BC was 42 years (range 25-73). TNM classification (UICC, 1978) showed 10 T0 (non-palpable lesions) (13%), 20 T1 (26%), 22 T2 (29%), 8 T3 (11%), 7 T4 (9%) and 9 Tx (12%), giving altogether a total of 76 tumours, including, respectively, 5 and 8 bilateral synchronous and metachronous lesions. Among the 68 tumours initially discovered, 53 ductal infiltrating, one lobular infiltrating and two medullary carcinomas were found. Moreover, two fibrosarcomas and 10 ductal carcinoma in situ (DCIS) were also found. Among 50 axillary dissections for invasive carcinomas, histological involvement was found in 31 cases (62%). 45 tumours were treated by mastectomy, without (n = 35) or with (n = 10) RT. 27 tumours had lumpectomy, without (n = 7) or with RT (n = 20). 2 others received RT only, and one only CT. 7 patients (11%) developed isolated local recurrence. 20 patients (32%) developed metastases and all died; 38 are in complete remission, whereas 5 died of intercurrent disease. The 5-year disease-specific survival rate by the Kaplan-Meier method was 61%. The 5-year disease-specific survival rate for pN0, pN1-3 and pN > or = 3 groups were 91%, 66% and 0%, respectively (P < 0.0001) and 100%, 88%, 64% and 23% for the T0, T1, T2 and T3T4 groups, respectively. These secondary BCs seem to be of two types: a large number of aggressive tumours with a very unfavourable prognosis (especially in the case of pN > 3 and/or T3T4); and many tumours with a 'slow development' such as DCIS and microinvasive

  2. Clinical hints and precipitating factors in patients suffering from Meniere's disease.

    PubMed

    Rauch, Steven D

    2010-10-01

    Meniere disease is one of the most fascinating and most vexing of all clinical conditions encountered by the otolaryngologist. Operationally speaking, a Meniere ear is a fragile ear. In fact, Meniere disease can and should be redefined as a degenerating inner ear that has impairment of one or more homeostatic systems, resulting in instability of hearing and balance function. This updated definition is a valuable guide to the clinical epidemiology and presentation of Meniere disease and to understanding the effects of conservative treatments. In the absence of a definitive test for Meniere disease, the greatest challenge for the clinician may be differentiating this condition from migraine. Ultimately, Meniere vertigo attacks are controllable in more than 99% of cases, but hearing loss and other auditory symptoms tend to be unresponsive to treatment.

  3. Perceived everyday racism, residential segregation, and HIV testing among patients at a sexually transmitted disease clinic.

    PubMed

    Ford, Chandra L; Daniel, Mark; Earp, Jo Anne L; Kaufman, Jay S; Golin, Carol E; Miller, William C

    2009-04-01

    More than one quarter of HIV-infected people are undiagnosed and therefore unaware of their HIV-positive status. Blacks are disproportionately infected. Although perceived racism influences their attitudes toward HIV prevention, how racism influences their behaviors is unknown. We sought to determine whether perceiving everyday racism and racial segregation influence Black HIV testing behavior. This was a clinic-based, multilevel study in a North Carolina city. Eligibility was limited to Blacks (N = 373) seeking sexually transmitted disease diagnosis or screening. We collected survey data, block group characteristics, and lab-confirmed HIV testing behavior. We estimated associations using logistic regression with generalized estimating equations. More than 90% of the sample perceived racism, which was associated with higher odds of HIV testing (odds ratio = 1.64; 95% confidence interval = 1.07, 2.52), after control for residential segregation, and other covariates. Neither patient satisfaction nor mechanisms for coping with stress explained the association. Perceiving everyday racism is not inherently detrimental. Perceived racism may improve odds of early detection of HIV infection in this high-risk population. How segregation influences HIV testing behavior warrants further research.

  4. [Hope for Huntington's disease patients: first clinical gene silencing study in progress].

    PubMed

    Rollnik, Jens D

    2017-08-01

    Huntington's disease (HD) is an autosomal-dominant inherited neurodegenerative disorder, characterized by motor, psychiatric and cognitive symptoms for which as yet no causal treatment is available. It has a prevalence of 1 : 10 000 in Germany. Its cause is a mutation in the Huntington gene (CAG-repeat). The mutation induces a polyglutamine expansion in the huntingtin protein (HTT). Mutant HTT (mHTT) has cytotoxic properties, aggregates in the cell and leads to complex pathophysiological disturbances ending in cell death. This review explains the principles of gene silencing which suppresses transcription and translation of huntingtin. One way to achieve gene silencing is the use of antisense oligonucleotides (ASO) that bind to pre-mRNA. Since August 2015, a first clinical trial with ASO (study drug: IONIS-HTTRx) in early manifest HD patients is in progress (NCT02519036). Results from this study could lead to a first causal treatment option in HD. Georg Thieme Verlag KG Stuttgart · New York.

  5. Clinical and genetic data on Lafora disease patients of Serbian/Montenegrin origin.

    PubMed

    Kecmanović, M; Jović, N; Keckarević-Marković, M; Keckarević, D; Stevanović, G; Ignjatović, P; Romac, S

    2016-01-01

    Lafora disease (LD) is an autosomal recessive, progressive disorder characterized by myoclonus and seizures, inexorable neurologic deterioration, cognitive decline and poor prognosis. LD is caused by mutations either in the EPM2A or in NHLRC1 genes. Here we report clinical and genetic findings on 14 LD patients from 10 families of Serbian/Montenegrin origin. Molecular diagnostics was performed by sequencing the coding regions of the EPM2A and NHLRC1 genes. In addition, haplotype analysis of the chromosomes carrying the two most frequent mutations (c.1048-1049delGA and deletion of the whole NHLRC1 gene) using eight different markers flanking the NHLRC1 gene was conducted. We identified one new mutation (c.1028T>C) along with the 3 previously reported mutations (c.1048-1049delGA, c.990delG, deletion of the whole NHLRC1 gene), all of which were located on the NHLRC1 gene. The two predominant mutations (c.1048-1049delGA and complete NHLRC1 gene deletion) appear to be founder mutations. In addition to documenting the genetic heterogeneity observed for LD, our study suggests that mutations in the NHLRC1 gene may be a common cause of LD in the Serbian/Montenegrin population, primarily because of a founder effect.

  6. The impact of virtual admission on self-efficacy in patients with chronic obstructive pulmonary disease - a randomised clinical trial.

    PubMed

    Emme, Christina; Mortensen, Erik L; Rydahl-Hansen, Susan; Østergaard, Birte; Svarre Jakobsen, Anna; Schou, Lone; Phanareth, Klaus

    2014-11-01

    To investigate how virtual admission during acute exacerbation influences self-efficacy in patients with chronic obstructive pulmonary disease, compared with conventional hospital admission. Telemedicine solutions have been highlighted as a possible way to increase self-efficacy in patients with chronic diseases, such as chronic obstructive pulmonary disease. However, little is known about how telemedicine-based virtual admission as a replacement of hospital admission during acute exacerbation affects chronic obstructive pulmonary disease patients' self-efficacy. This study was a nonblinded, randomised clinical multicentre trial. The study was a substudy to The Virtual Hospital, investigating the feasibility and safety of telemedicine-based treatment at home for patients with acute exacerbation of chronic obstructive pulmonary disease. Participants were consecutively randomised to virtual admission or conventional hospital admission. Data from 50 patients were analysed. Self-efficacy was assessed at baseline, three days after discharge, and also six weeks and three months after discharge, using the Danish version of 'The chronic obstructive pulmonary disease self-efficacy scale'. Intergroup comparison showed no significant differences between the two groups at baseline, three days after discharge, six weeks after discharge or three months after discharge. Furthermore, intragroup comparison did not reveal significant differences in the chronic obstructive pulmonary disease self-efficacy scale mean sum score within the two groups. The results of the study suggest that there is no difference between self-efficacy in chronic obstructive pulmonary disease patients undergoing virtual admission, compared with conventional hospital admission. However, the anticipated sample size could not be reached, which prompts caution regarding interpretation of the findings. This study provides new insight into how virtual admission affects chronic obstructive pulmonary disease

  7. [Oral lesions associated with human immunodeficiency virus disease in adult patients, a clinical perspective].

    PubMed

    Donoso-Hofer, Francisca

    2016-10-01

    Among the clinical manifestations which may occur in HIV/AIDS patients, oral lesions are relevant because there are easily accessible and usually the diagnosis is made through clinical features. Some oral manifestations are strongly related with HIV/AIDS patients indicating infection and progression to AIDS and also allow monitoring the success or failure of using antiretroviral therapy. The massive introduction of antiretroviral therapy has changed the morbidity and mortality, frequency, type of clinical manifestation and the timing of the classic opportunistic complications. The aim of this review is to provide an updated of the classical clinical features of the most frequent and relevant HIV/AIDS oral manifestations, considering the fundamental clinical features for their diagnosis.

  8. The role of the clinical pharmacist in the care of patients with cardiovascular disease.

    PubMed

    Dunn, Steven P; Birtcher, Kim K; Beavers, Craig J; Baker, William L; Brouse, Sara D; Page, Robert L; Bittner, Vera; Walsh, Mary Norine

    2015-11-10

    Team-based cardiovascular care, including the use of clinical pharmacists, can efficiently deliver high-quality care. This Joint Council Perspectives paper from the Cardiovascular Team and Prevention Councils of the American College of Cardiology provides background information on the clinical pharmacist's role, training, certification, and potential utilization in a variety of practice models. Selected systematic reviews and meta-analyses, highlighting the benefit of clinical pharmacy services, are summarized. Clinical pharmacists have a substantial effect in a wide variety of roles in inpatient and ambulatory settings, largely through optimization of drug use, avoidance of adverse drug events, and transitional care activities focusing on medication reconciliation and patient education. Expansion of clinical pharmacy services is often impeded by policy, legislation, and compensation barriers. Multidisciplinary organizations, including the American College of Cardiology, should support efforts to overcome these barriers, allowing pharmacists to deliver high-quality patient care to the full extent of their education and training.

  9. National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary--clinical characteristics and disease course of 44 patients with juvenile dermatomyositis.

    PubMed

    Constantin, T; Ponyi, A; Orbán, I; Molnár, K; Dérfalvi, B; Dicso, F; Kálovics, T; Müller, J; Garami, M; Sallai, A; Balogh, Z; Szalai, Z; Fekete, G; Dankó, K

    2006-05-01

    Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs. Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM). A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease

  10. Impact of Helminth Infection on the Clinical and Microbiological Presentation of Chagas Diseases in Chronically Infected Patients

    PubMed Central

    Salvador, Fernando; Sulleiro, Elena; Sánchez-Montalvá, Adrián; Martínez-Gallo, Mónica; Carrillo, Eugenia; Molina, Israel

    2016-01-01

    Abstract Background Helminth infections are highly prevalent in tropical and subtropical countries, coexisting in Chagas disease endemic areas. Helminth infections in humans may modulate the host immune system, changing the Th1/Th2 polarization. This immunological disturbance could modify the immune response to other infections. The aim of this study is to evaluate the relationship between clinical, microbiological and epidemiological characteristics of Chagas disease patients, with the presence of helminth infection. Methods A prospective observational study was conducted at Vall d’Hebron University Hospital (Barcelona, Spain). Inclusion criteria were: age over 18 years, diagnosis of Chagas disease, and not having received specific treatment for Chagas disease previously to the inclusion. The study protocol included Chagas disease assessment (cardiac and digestive evaluation, detection of T. cruzi DNA measured by PCR in peripheral blood), and helminth infection diagnosis (detection of IgG anti-Strongyloides stercoralis by ELISA, microscopic examination of stool samples from three different days, and specific faecal culture for S. stercoralis larvae). Results Overall, 65 patients were included, median age was 38 years, 75.4% were women and most of them came from Bolivia. Cardiac and digestive involvement was present in 18.5% and 27.7% of patients respectively. T. cruzi PCR was positive in 28 (43.1%) patients. Helminth infection was diagnosed in 12 (18.5%) patients. No differences were observed in clinical and epidemiological characteristics between patients with and without helminth infection. Nevertheless, the proportion of patients with positive T. cruzi PCR was higher among patients with helminth infection compared with patients without helminth infection (75% vs 35.8%, p = 0.021). Conclusions We observed a high prevalence of S. stercoralis infection among chronic Chagas disease patients attended in our tropical medicine unit. Strongyloidiasis was associated

  11. Impact of Helminth Infection on the Clinical and Microbiological Presentation of Chagas Diseases in Chronically Infected Patients.

    PubMed

    Salvador, Fernando; Sulleiro, Elena; Sánchez-Montalvá, Adrián; Martínez-Gallo, Mónica; Carrillo, Eugenia; Molina, Israel

    2016-04-01

    Helminth infections are highly prevalent in tropical and subtropical countries, coexisting in Chagas disease endemic areas. Helminth infections in humans may modulate the host immune system, changing the Th1/Th2 polarization. This immunological disturbance could modify the immune response to other infections. The aim of this study is to evaluate the relationship between clinical, microbiological and epidemiological characteristics of Chagas disease patients, with the presence of helminth infection. A prospective observational study was conducted at Vall d'Hebron University Hospital (Barcelona, Spain). Inclusion criteria were: age over 18 years, diagnosis of Chagas disease, and not having received specific treatment for Chagas disease previously to the inclusion. The study protocol included Chagas disease assessment (cardiac and digestive evaluation, detection of T. cruzi DNA measured by PCR in peripheral blood), and helminth infection diagnosis (detection of IgG anti-Strongyloides stercoralis by ELISA, microscopic examination of stool samples from three different days, and specific faecal culture for S. stercoralis larvae). Overall, 65 patients were included, median age was 38 years, 75.4% were women and most of them came from Bolivia. Cardiac and digestive involvement was present in 18.5% and 27.7% of patients respectively. T. cruzi PCR was positive in 28 (43.1%) patients. Helminth infection was diagnosed in 12 (18.5%) patients. No differences were observed in clinical and epidemiological characteristics between patients with and without helminth infection. Nevertheless, the proportion of patients with positive T. cruzi PCR was higher among patients with helminth infection compared with patients without helminth infection (75% vs 35.8%, p = 0.021). We observed a high prevalence of S. stercoralis infection among chronic Chagas disease patients attended in our tropical medicine unit. Strongyloidiasis was associated with significantly higher proportion of positive T

  12. Clinical Outcomes of Thirteen Patients with Acute Chagas Disease Acquired through Oral Transmission from Two Urban Outbreaks in Northeastern Brazil

    PubMed Central

    Bastos, Claudilson J. C.; Aras, Roque; Mota, Gildo; Reis, Francisco; Dias, Juarez Pereira; de Jesus, Robson Silva; Freire, Miralba Silva; de Araújo, Eline G.; Prazeres, Juliana; Grassi, Maria Fernanda Rios

    2010-01-01

    Background Outbreaks of orally transmitted Trypanosoma cruzi continue to be reported in Brazil and are associated with a high mortality rate, mainly due to myocarditis. Methods This study is a detailed report on the disease progression of acute Chagas disease in 13 patients who were infected during two micro-outbreaks in two northeastern Brazilian towns. Clinical outcomes as well as EKG and ECHO results are described, both before and after benznidazole treatment. Results Fever and dyspnea were the most frequent symptoms observed. Other clinical findings included myalgia, periorbital edema, headache and systolic murmur. Two patients died of cardiac failure before receiving benznidazole treatment. EKG and ECHO findings frequently showed a disturbance in ventricular repolarization and pericardial effusion. Ventricular dysfunction (ejection fraction <55%) was present in 27.3% of patients. After treatment, EKG readings normalized in 91.7% of patients. Ventricular repolarization abnormalities persisted in 50% of the patients, while sinus bradycardia was observed in 18%. The systolic ejection fraction normalized in two out of three patients with initially depressed ventricular function, while pericardial effusion disappeared. Conclusions Myocarditis is frequently found and potentially severe in patients with acute Chagas disease. Benznidazole treatment may improve clinical symptoms, as well as EKG and ECHO findings. PMID:20559542

  13. A national survey on the pediatric cardiologist's clinical approach for patients with Kawasaki disease.

    PubMed

    Kahwaji, I Y; Connuck, D M; Tafari, N; Dahdah, N S

    2002-01-01

    In 1994, the American Heart Association (AHA) published the most recent guidelines for long-term cardiovascular management of Kawasaki disease. Since then, recent publications have shed new light on different diagnostic, prognostic, and management issues. We sought the opinion of pediatric cardiologists practicing in U.S. fellowship programs on the subject by means of a multiple-choice survey. Two questions addressed therapy in the acute phase, each preceded by a statement from related literature. Ten duplicate questions addressed the long-term cardiovascular management in five sets of paired questions; each question was first given in reminiscence of a clinical situation and then preceded by a statement from particular publications representative of new information that has become available since the publication of the 1994 AHA guidelines. All questions were provided in the same mailing. Replies were received from 97 participants practicing at 29 institutions. For the acute illness, 21% of respondents do not use high-dose aspirin, and 50% support reassessment of current guidelines. Universal intravenous immune globulin (IVIG) administration is followed by 97%, among whom 20% agree that evaluation of selection criteria is needed. For long-term management, 60-75% advocate regular follow-up of risk level I patients, and 80% favor periodic follow-up, with stress imaging (34-40%), for risk level II. For risk level IV more respondents favor stress echocardiography as opposed to nuclear imaging, in consonance with recent literature. For risk levels III and IV, 36-40% perform coronary angiography on a regular basis, whereas 60% do so when coronary symptoms are present or when stress imaging suggests myocardial ischemia. Finally, 19-25% of respondents do not routinely advise healthy lifestyle to patients free of coronary artery lesions. In conclusion, the guidelines for conventional therapy in the acute phase and long-term cardiovascular management need to be revised.

  14. Wilson disease: novel mutations in the ATP7B gene and clinical correlation in Brazilian patients.

    PubMed

    Deguti, Marta M; Genschel, Janine; Cancado, Eduardo L R; Barbosa, Egberto R; Bochow, Bettina; Mucenic, Marcos; Porta, Gilda; Lochs, Herbert; Carrilho, Flair J; Schmidt, Hartmut H-J

    2004-04-01

    Wilson disease (WD) is a rare inherited autosomal recessive disorder caused by a defect in a metal transporting P-type ATPase, resulting in copper overload in various tissues and cells. The aim was to assess both the phenotype in Brazilian WD patients and the corresponding ATP7B genotype. Sixty subjects belonging to 46 pedigrees diagnosed as WD were included in this study. Direct sequencing of all 21 exons within ATP7B and their flanking introns was performed. Demographic, clinical, laboratory and histopathological data at the time of diagnosis were obtained. We identified twenty-five mutations, twelve of them reported for the first time. The c.3402delC mutation had the highest allelic frequency (30.8%), followed by the c.2123T>C (p.L708P) (16.7%). Exons 8 and 15 were the site of 62.5% of the mutations. The common European mutation c.3207C>A (p.H1069Q) was not present at all. Phenotype varied greatly among individuals with the same ATP7B genotype. Our data confirm the heterogeneity of ATP7B genotype in Brazilian WD patients. The mutational spectrum is compatible with the Brazilian history of Mediterranean immigration; however, new mutations, and different frequencies and phenotype associated with the previously known mutations characterize this population. Exons 8 and 15 should be preferentially screened in WD cases from Brazil. Phenotype variation among subjects with the same ATP7B genotype suggests that modifying factors play an additional role in the pathogenesis of WD.

  15. Skin diseases among children attending the out patient clinic of the University of Nigeria teaching hospital, Enug.

    PubMed

    Emodi, L J; Ikefuna, A N; Uchendu, U; Duru, U A

    2010-12-01

    The prevalence of skin diseases varies worldwide and information regarding local prevalence of its various causes may help the development of policies towards better management. To document the types of skin disorders seen among children attending the University of Nigeria Teaching Hospital, Enugu( UNTH). The medical register from the children out patient clinic was used to obtain information on pattern and outcome of skin diseases seen from January 1996 to December 2005. Of the 16,337 children seen in children.s out patient clinic (CHOP), 1506 (1.3%) had a skin disease. Age range was one week to 16 years with a mean ± SD of 3.89 ± 3.8 years. Children aged 0 - 5 years constituted 70.24% of patients with skin diseases. The commonest skin condition was pyoderma (29.81%) seen mainly in those below 5 years, followed by scabies (13.55%). Approximately 1/3 (33.3%) of the patients were referred to the dermatology clinic. Infectious skin diseases constitute a high percentage of skin disorders encountered in paediatrics. Almost half (48.4%) of the skin problems were diagnosed as non-specific dermatitis suggesting the need of better dermatological training of the paediatric residents.

  16. Does improving patient-practitioner communication improve clinical outcomes in patients with cardiovascular diseases? A systematic review of the evidence.

    PubMed

    Schoenthaler, Antoinette; Kalet, Adina; Nicholson, Joseph; Lipkin, Mack

    2014-07-01

    To conduct a systematic literature review appraising the effects of interventions to improve patient-practitioner communication on cardiovascular-related clinical outcomes. Databases were searched up to March 27, 2013 to identify eligible studies that included interventions to improve patient and/or practitioner communication skills and assessment of a cardiovascular-related clinical outcome in adults ≥18 years of age. Fifteen papers were reviewed: the primary focus in seven studies was the patient; seven included a practitioner-focused intervention and one targeted both. Two patient-focused and two practitioner-focused studies demonstrated a beneficial effect of the intervention compared to a control group. Patient-focused studies were designed to improve patients' information-seeking and question-asking skills with their practitioner. Practitioner-focused studies were designed to either improve practitioner's general patient-centered communication or risk communication skills. Few interventions targeting patient-practitioner communication have assessed the impact on cardiovascular-related clinical outcomes, limiting the ability to determine effectiveness. Additional rigorous research supported by theoretical frameworks and validated measurement is needed to understand the potential of patient-practitioner communication to improve cardiovascular-related clinical outcomes. Investments in communication skills trainings in medical education and practice are needed in order to attain the full potential of patient-centered care on cardiovascular-related clinical outcomes. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  17. Clinical correlations of infliximab trough levels and antibodies to infliximab in South Korean patients with Crohn’s disease

    PubMed Central

    Oh, Eun Hye; Ko, Dae-Hyun; Seo, Hyungil; Chang, Kiju; Kim, Gwang-Un; Song, Eun Mi; Seo, Myeongsook; Lee, Ho-Su; Hwang, Sung Wook; Yang, Dong-Hoon; Ye, Byong Duk; Byeon, Jeong-Sik; Myung, Seung-Jae; Yang, Suk-Kyun; Park, Sang Hyoung

    2017-01-01

    AIM To investigate the clinical implications of infliximab trough levels (IFX-TLs) and antibodies to infliximab (ATI) levels in Crohn’s disease (CD) patients in Asian countries. METHODS IFX-TL and ATI level were measured using prospectively collected samples obtained with informed consent from CD patients being treated at Asan Medical Center, South Korea. We analyzed the correlations between IFX-TLs/ATI levels and the clinical activity of CD (quiescent vs active disease) based on the CD activity index, C-reactive protein level, and physician’s judgment of patients’ clinical status at enrollment. The impact of concomitant immunomodulators was also investigated. RESULTS This study enrolled 138 patients with CD (84 with quiescent and 54 with active disease). In patients with quiescent and active diseases, the median IFX-TLs were 1.423 μg/mL and 0.163 μg/mL, respectively (P < 0.001) and the median ATI levels were 8.064 AU/mL and 11.209 AU/mL, respectively (P < 0.001). In the ATI-negative and -positive groups, the median IFX-TLs were 1.415 μg/mL and 0.141 μg/mL, respectively (P < 0.001). In patients with and without concomitant immunomodulator use, there were no differences in IFX-TLs (0.632 μg/mL and 1.150 μg/mL, respectively; P = 0.274) or ATI levels (8.655 AU/mL and 9.017 AU/mL, respectively; P = 0.083). CONCLUSION IFX-TL/ATI levels were well correlated with the clinical activity in South Korean CD patients. Our findings support the usefulness of IFX-TLs/ATI levels in treating CD patients receiving IFX in clinical practice. PMID:28293096

  18. Correlation of Promis Scales and Clinical Measures Among Chronic Obstructive Pulmonary Disease Patients With and Without Exacerbations

    PubMed Central

    Irwin, Debra E.; Atwood, Charles A.; Hays, Ron D.; Spritzer, Karen; Liu, Honghu; Donohue, James F.; Leidy, Nancy Kline; Yount, Susan E.; DeWalt, Darren A.

    2014-01-01

    Purpose The Patient-Reported Outcomes Measurement Information System (PROMIS®) initiative was developed to advance the methodology of PROs applicable to chronic diseases. Chronic obstructive pulmonary disease (COPD) is a progressive chronic disease associated with poor health. This study was designed to examine the correlation of PROMIS health-related quality of life (HRQOL) scales and clinical measures among COPD patients. Methods A cross-sectional analysis was conducted comparing patients who were stable (n = 100) with those currently experiencing a COPD exacerbation (n=85). All PROMIS measures for adults available at the time of the study (2008), disease-targeted and other HRQOL instruments, health literacy, percent predicted FEV1, and a 6-minute walk distance were assessed when patients were considered clinically stable. Results Stable COPD patients reported significantly (p≤0.05) better health-related quality of life on PROMIS domains than patients experiencing an exacerbation. PROMIS domain scores were significantly (p≤0.01) correlated with each of legacy measures. Six-minute walk scores were most highly correlated with the PROMIS physical function domain scores (r=0.53) followed by the fatigue (r=-0.26), social (r=0.24) and to a lesser extent depression (r=-0.23) and anxiety (r=-0.22) domain scores. Percent predicted FEV1 score was significantly associated with PROMIS physical function scores (r=0.27). Conclusion This study provides support for the validity of the PROMIS measures in COPD patients. PMID:25307510

  19. A multicenter phase II prospective clinical trial of glucocorticoid for patients with untreated IgG4-related disease.

    PubMed

    Masaki, Yasufumi; Matsui, Shoko; Saeki, Takako; Tsuboi, Hiroto; Hirata, Shintaro; Izumi, Yasumori; Miyashita, Taiichiro; Fujikawa, Keita; Dobashi, Hiroaki; Susaki, Kentaro; Morimoto, Hisanori; Takagi, Kazutaka; Kawano, Mitsuhiro; Origuchi, Tomoki; Wada, Yoko; Takahashi, Naoki; Horikoshi, Masanobu; Ogishima, Hiroshi; Suzuki, Yasunori; Kawanami, Takafumi; Kawanami Iwao, Haruka; Sakai, Tomoyuki; Fujita, Yoshimasa; Fukushima, Toshihiro; Saito, Masatoshi; Suzuki, Ritsuro; Morikawa, Yuko; Yoshino, Tadashi; Nakamura, Shigeo; Kojima, Masaru; Kurose, Nozomu; Sato, Yasuharu; Tanaka, Yoshiya; Sugai, Susumu; Sumida, Takayuki

    2017-09-01

    Although glucocorticoids are effective for patients with IgG4-related disease, the treatment has not yet been standardized. Therefore, the treatment strategy should be established. Patients who fulfilled the comprehensive diagnostic criteria for definite IgG4-related disease were started on prednisolone (0.6 mg/kg body weight) with the dose reduced every two weeks. The subsequent maintenance dose and need for prednisolone were determined for individual patients. The primary endpoint was the complete remission (CR) rate at one year. Secondary endpoints included overall response rate (ORR), the maintenance dose, the relapse rate, and adverse events. This study enrolled 61 patients. After clinicopathological review, three patients were excluded, and one, 13, and 44 patients were diagnosed with probable, possible, and definite IgG4-related disease, respectively. Of the 44 patients with definite IgG4-RD, 29 (65.9%) achieved CR, and the ORR was 93.2%. No patient was refractory to primary treatment. The most frequent adverse events were glucose intolerance. Six patients relapsed. Glucocorticoid treatment is usually effective for patients with IgG4-RD, and we should examine the possibility of other disorders when a patient is glucocorticoid refractory. Some patients are misdiagnosed, making central clinicopathological review of diagnosis very important in conducting clinical studies.

  20. Effect of oral lactulose on clinical and immunohistochemical parameters in patients with inflammatory bowel disease: a pilot study

    PubMed Central

    Hafer, Anne; Krämer, Sigrid; Duncker, Swantje; Krüger, Martin; Manns, Michael P; Bischoff, Stephan C

    2007-01-01

    Background The prebiotic potential of lactulose is well established and preclinical studies demonstrated a protective effect of lactulose in murine models of colitis. The aim of the present study was to investigate the clinical and histological efficacy of lactulose in patients with inflammatory bowel disease (IBD), for which probiotic therapy yielded promising results. Methods Patients were treated with standard medication alone or combined with 10 g lactulose daily as adjuvant therapy for 4 months. Clinical efficacy of treatment was assessed using clinical activity indices, a quality of life index (IBDQ), endoscopic scores, defecation frequency and monitoring corticosteroid medication. Orsomucoid, alpha1-antitrypsin and other laboratory parameters were determined. In addition, in some participants colonic biopsies were analyzed with haematoxylin-eosin staining or with antibodies against HLA-DR, CD68, IgA and CD3, and evaluated systematically. All measurements were performed both at enrolment and at the end of the trial. Results 14 patients presenting ulcerative colitis (UC) and 17 patients presenting Crohn's disease (CD), most of them in a clinically active state, were enrolled in this pilot study. After 4 month no significant improvement of clinical activity index, endoscopic score or immunohistochemical parameters was observed in CD or UC patients receiving lactulose in comparison to the control group. However, significant improvement of quality of life was observed in UC patients receiving lactulose compared to the control group (p = 0.04). Conclusion The findings of the present pilot study indicate that oral lactulose has no beneficial effects in IBD patients in particular with regard to clinical activity, endoscopic score or immunohistochemical parameters. The importance of the beneficial effect of lactulose in UC patients regarding the quality of life needs further evaluation in larger controlled clinical trials. Trial registration Current Controlled Trials

  1. System analysis of gene mutations and clinical phenotype in Chinese patients with autosomal-dominant polycystic kidney disease

    PubMed Central

    Jin, Meiling; Xie, Yuansheng; Chen, Zhiqiang; Liao, Yujie; Li, Zuoxiang; Hu, Panpan; Qi, Yan; Yin, Zhiwei; Li, Qinggang; Fu, Ping; Chen, Xiangmei

    2016-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder mainly caused by mutation in PKD1/PKD2. However, ethnic differences in mutations, the association between mutation genotype/clinical phenotype, and the clinical applicable value of mutation detection are poorly understood. We made systematically analysis of Chinese ADPKD patients based on a next-generation sequencing platform. Among 148 ADPKD patients enrolled, 108 mutations were detected in 127 patients (85.8%). Compared with mutations in Caucasian published previously, the PKD2 mutation detection rate was lower, and patients carrying the PKD2 mutation invariably carried the PKD1 mutation. The definite pathogenic mutation detection rate was lower, whereas the multiple mutations detection rate was higher in Chinese patients. Then, we correlated PKD1/PKD2 mutation data and clinical data: patients with mutation exhibited a more severe phenotype; patients with >1 mutations exhibited a more severe phenotype; patients with pathogenic mutations exhibited a more severe phenotype. Thus, the PKD1/PKD2 mutation status differed by ethnicity, and the PKD1/PKD2 genotype may affect the clinical phenotype of ADPKD. Furthermore, it makes sense to detect PKD1/PKD2 mutation status for early diagnosis and prognosis, perhaps as early as the embryo/zygote stage, to facilitate early clinical intervention and family planning. PMID:27782177

  2. Giant lymph node hyperplasia (Castleman's disease): a clinical study of eight patients.

    PubMed Central

    Baruch, Y.; Ben-Arie, Y.; Kerner, H.; Lorber, M.; Best, L. A.; Gershoni-Baruch, R.

    1991-01-01

    We report on 8 patients with giant lymph node hyperplasia (GLNH), diagnosed over a 10-year period. The age of the patients at diagnosis, the clinical presentation and the histological subtype varied, indicating that GNLH is a heterogeneous condition. One case was associated with liver cirrhosis, and in another patient bacterial endocarditis was diagnosed post mortem. Our study shows that GLNH is localized and benign in the young, and diffuse and aggressive in the elderly. It is concluded that GLNH should be separated into 3 clinical entities--namely, localized, systemic and reactive GLNH--defined by their clinical presentation and course, and correlated or not correlated with the histological findings. Images Figure 1 Figure 2 Figure 3 PMID:2068030

  3. Adrenal medullary transplantation to the caudate nucleus in Parkinson's disease. Initial clinical results in 18 patients.

    PubMed

    Allen, G S; Burns, R S; Tulipan, N B; Parker, R A

    1989-05-01

    Results from a pilot study of adrenal medullary autotransplantation for Parkinson's disease are presented. Eighteen patients were studied; 12 were followed up for 1 year, and 6 were followed up for 6 months. Four of 12 patients showed distinct improvement in the signs and symptoms of their disease, as assessed using the Columbia Rating Scale, at 1 year; none showed distinct deterioration. The 6 patients who were followed up for only 6 months were an average of 20 years older and generally more severely affected. None distinctly improved. Morbidity was considered to be minor and transient among the first 12 patients, while 4 of the last 6 patients experienced alteration in mental status lasting as long as several months. This problem has led us to conclude that older patients with preexisting cognitive impairment should not be included in future studies until the benefits are more clearly established. However, we believe that the distinct and persistent improvement seen in some of the younger patients warrants the initiation of a well-designed, randomized, and controlled trial of adrenal medullary autotransplantation for the purpose of confirming these results and assessing the effect of the procedure on the natural progression of Parkinson's disease.

  4. Diseases affecting patients returning from abroad: Experience of a travel clinic in Japan from 2004 to 2014.

    PubMed

    Yoshimura, Yukihiro; Miyata, Nobuyuki; Miyajima, Makiko; Sakamoto, Yohei; Amano, Yuichiro; Tachikawa, Natsuo

    2017-01-01

    The number of patients returning from or staying abroad is likely to increase in the future. We performed a retrospective study of patients returning from abroad in our travel clinic in Japan. All patients presenting within 6 months of traveling abroad between 2004 and 2014 were included in the present study. A total of 2374 (mean age, 35 years) patients were seen by doctors specializing in treating infectious diseases. Of these, 918 were females and 87 of them lived abroad. Diagnoses and exposure regions were recorded for all patients. The most frequent region visited before attending our clinic was Southeast Asia (n = 1050, 44%), with a median duration for staying abroad of 8 days. The major purposes for overseas travel were tourism (n = 1302, 55%) and business (n = 684, 29%). Of the 2399 individual diagnoses made, the most frequent were diseases of the gastrointestinal system (n = 1083, 45%), skin and soft tissue (n = 440, 18%), systemic febrile disease without specific systems (419, 18%), and the respiratory system (353, 15%). The relative incidences of specific diseases changed drastically due to significant disease outbreaks, such as pandemic influenza in 2009. Exposure regions remained relatively constant throughout the study period, except for Japan. Vaccine-preventable diseases accounted for 5.3% of all the diseases, and 402 (26%) patients received pre-travel consultation and prophylaxis with vaccines and/or anti-malarial drug. We should make an effort to make more people notice the risk of travel and properly perform prophylaxis. Copyright © 2016 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

  5. [Clinical study of unrelated umbilical cord blood transplantation for 25 patients with hematological diseases].

    PubMed

    Chang, Hui-Rong; Cheng, Zhen; Chen, Guang-Hua; Sun, Ai-Ning; Qiao, Man; Ma, Xiao; Wang, Ying; Chen, Feng; Jin, Huan-Ying; Lu, Yao; Zhang, Jia-Qing; Fu, Cheng-Cheng; Jin, Zheng-Min; Tang, Xiao-Wen; Han, Ye; Qiu, Hui-Ying; Wu, De-Pei

    2014-06-01

    This study was purposed to investigate the engraftment, graft-versus-host disease (GVHD), transplantation related mortality (TRM), relapse and survival in hematologic patients received unrelated umbilical cord blood transplantation (UCBT). A total of 25 patients with hematological disease underwent UCBT, including 8 pediatric and 17 young adult patients. Among them 3 cases received single unit of UCBT and 22 cases received double units of UCBT. For donor/recipients human leukocyte antigen (HLA) matching: HLA 6/6 loci matched in 9 cases, HLA 4-5/6 loci matched in 16 cases. There were 19 patients with hematologic malignancies, including 3 cases in the period of disease progression and 6 cases of non-hematologic malignancies. Conditioning regimens were TBI/Cy ± Flu ± ATG or BuCy ± Flu ± ATG for 21 patients and Cy+Flu+ATG for 4 patients. For prophylaxis of acute graft-versus-host disease (aGVHD) the regimen of cyclosporine (CsA) as dominant drug was used. The results showed that among 16 patients (80.0%) achieved engraftment, 20 patients survived for more than 42 d after transplantation. The cumulative neutrophil recovery rate on day 42 after transplant was 64.0%, with a median time of 17.0 d;the cumulative platelet recovery rate on day 100 after transplant was 60.0 %, with a median time of 35.0 d. The cumulative rate of grade II-IV and III-IV aGVHD after transplantation 100 d was 44.0% and 30.7%, respectively. Until the end of the follow-up, the cumulative rate of TRM was 54.3%. For all the patients, overall survival rate was 42.7%. Out of 17 evaluable patients with hematologic malignancies 7 cases (41.2%) survived to date, and only 1 case relapsed, so event-free survival rate was 35.3%. Out of 5 evaluable patients with non-hematologic malignancies, 4 patients survived and 2 patients were in stable engraftment state, 2 cases with autologous hematopoietic recovery. Among 3 cases of hematologic malignancies at advanced stage, only 1 case survived to date. It is

  6. Prevalence and associated factors of comorbid skin diseases in patients with schizophrenia: a clinical survey and national health database study.

    PubMed

    Wu, Bai-Yao; Wu, Bo-Jian; Lee, Shin-Min; Sun, Hsiao-Ju; Chang, Yun-Ting; Lin, Ming-Wei

    2014-01-01

    To examine the epidemiology of and possible risk factors for skin diseases in patients with schizophrenia. All of 337 patients with schizophrenia were recruited from the therapeutic community of a psychiatric hospital and underwent a detailed skin examination. The National Health Insurance Research Database (NHIRD) was used to compare the prevalence of skin diseases between patients with schizophrenia and those without. In the clinical survey, fungal infection (61.4%) and dermatitis (46.9%) were the most common skin diseases. Clozapine users had a lower risk of fungal infection than those on typical antipsychotics [odds ratio (OR)=0.49, 95% confidence interval (CI)=0.30-0.81]. Obese patients were more likely to have fungal infections than those without (OR=1.93, 95% CI=1.20-3.09), and those with diabetes had an increased risk of bacterial infection than those without (OR=2.0, 95% CI=1.06-3.75). NHIRD revealed that the overall prevalence of skin diseases, including infections, dermatitis, hyperkeratosis, pilosebaceous disease, androgenic alopecia, xerosis and stasis, were higher in patients with schizophrenia than in those without (75.1% vs. 72.6%, P=.01). The prevalence of skin diseases is high in patients with schizophrenia, for whom proper skin care is necessary to improve their life quality. Copyright © 2014 Elsevier Inc. All rights reserved.

  7. Urinary Podocyte Loss Is Increased in Patients with Fabry Disease and Correlates with Clinical Severity of Fabry Nephropathy

    PubMed Central

    Fall, Brent; Scott, C. Ronald; Mauer, Michael; Shankland, Stuart; Pippin, Jeffrey; Jefferson, Jonathan A.; Wallace, Eric; Warnock, David; Najafian, Behzad

    2016-01-01

    Chronic kidney disease is a major complication of Fabry disease. Podocytes accumulate globotriaosylceramide inclusions more than other kidney cell types in Fabry patients. Podocyte injury occurs early in age, and is progressive. Since injured podocytes detach into the urine (podocyturia), we hypothesized that podocyturia would increase in Fabry patients and correlate with clinical severity of Fabry nephropathy. Urine specimens from 39 Fabry patients and 24 healthy subjects were evaluated for podocyturia. Most of the Fabry patients and many healthy subjects had podocyturia. The number of podocytes per gram of urine creatinine (UPodo/g Cr) was 3.6 fold greater in Fabry patients (3,741 ± 2796; p = 0.001) than healthy subjects (1,040 ± 972). Fabry patients with normoalbuminuria and normoproteinuria had over 2-fold greater UPodo/g Cr than healthy subjects (p = 0.048). UPodo/gCr was inversely related to eGFR in male patients (r = -0.69, p = 0.003). UPodo/gCr was directly related to urine protein creatinine ratio (r = 0.33; p = 0.04) in all Fabry patients. These studies confirm increased podocyturia in Fabry disease, even when proteinuria and albuminuria are absent. Podocyturia correlates with clinical severity of Fabry nephropathy, and potentially may be of prognostic value. PMID:27992580

  8. Urinary Podocyte Loss Is Increased in Patients with Fabry Disease and Correlates with Clinical Severity of Fabry Nephropathy.

    PubMed

    Fall, Brent; Scott, C Ronald; Mauer, Michael; Shankland, Stuart; Pippin, Jeffrey; Jefferson, Jonathan A; Wallace, Eric; Warnock, David; Najafian, Behzad

    2016-01-01

    Chronic kidney disease is a major complication of Fabry disease. Podocytes accumulate globotriaosylceramide inclusions more than other kidney cell types in Fabry patients. Podocyte injury occurs early in age, and is progressive. Since injured podocytes detach into the urine (podocyturia), we hypothesized that podocyturia would increase in Fabry patients and correlate with clinical severity of Fabry nephropathy. Urine specimens from 39 Fabry patients and 24 healthy subjects were evaluated for podocyturia. Most of the Fabry patients and many healthy subjects had podocyturia. The number of podocytes per gram of urine creatinine (UPodo/g Cr) was 3.6 fold greater in Fabry patients (3,741 ± 2796; p = 0.001) than healthy subjects (1,040 ± 972). Fabry patients with normoalbuminuria and normoproteinuria had over 2-fold greater UPodo/g Cr than healthy subjects (p = 0.048). UPodo/gCr was inversely related to eGFR in male patients (r = -0.69, p = 0.003). UPodo/gCr was directly related to urine protein creatinine ratio (r = 0.33; p = 0.04) in all Fabry patients. These studies confirm increased podocyturia in Fabry disease, even when proteinuria and albuminuria are absent. Podocyturia correlates with clinical severity of Fabry nephropathy, and potentially may be of prognostic value.

  9. Double-Blind Randomized Clinical Trial: Gluten versus Placebo Rechallenge in Patients with Lymphocytic Enteritis and Suspected Celiac Disease

    PubMed Central

    Carrasco, Anna; Ibarra, Montserrat; Temiño, Rocío; Salas, Antonio; Esteve, Maria

    2016-01-01

    Background The role of gluten as a trigger of symptoms in non-coeliac gluten sensitivity has been questioned. Aim To demonstrate that gluten is the trigger of symptoms in a subgroup of patients fulfilling the diagnostic criteria for non-coeliac gluten sensitivity (NCGS), which presented with lymphocytic enteritis, positive celiac genetics and negative celiac serology. Methods Double-blind randomized clinical trial of gluten vs placebo rechallenge. Inclusion criteria: >18 years of age, HLA-DQ2/8+, negative coeliac serology and gluten-dependent lymphocytic enteritis, and GI symptoms, with clinical and histological remission at inclusion. Eighteen patients were randomised: 11 gluten (20 g/day) and 7 placebo. Clinical symptoms, quality of life (GIQLI), and presence of gamma/delta+ cells and transglutaminase deposits were evaluated. Results 91% of patients had clinical relapse during gluten challenge versus 28.5% after placebo (p = 0.01). Clinical scores and GIQLI worsened after gluten but not after placebo (p<0.01). The presence of coeliac tissue markers at baseline biopsy on a gluten-free diet allowed classifying 9 out of the 18 (50%) patients as having probable ‘coeliac lite’ disease. Conclusion This proof-of-concept study indicates that gluten is the trigger of symptoms in a subgroup of patients fulfilling the diagnostic criteria for NCGS. They were characterized by positive celiac genetics, lymphocytic enteritis, and clinical and histological remission after a gluten-free diet. Trial Registration ClinicalTrials.gov NCT02472704 PMID:27392045

  10. Double-Blind Randomized Clinical Trial: Gluten versus Placebo Rechallenge in Patients with Lymphocytic Enteritis and Suspected Celiac Disease.

    PubMed

    Rosinach, Mercè; Fernández-Bañares, Fernando; Carrasco, Anna; Ibarra, Montserrat; Temiño, Rocío; Salas, Antonio; Esteve, Maria

    2016-01-01

    The role of gluten as a trigger of symptoms in non-coeliac gluten sensitivity has been questioned. To demonstrate that gluten is the trigger of symptoms in a subgroup of patients fulfilling the diagnostic criteria for non-coeliac gluten sensitivity (NCGS), which presented with lymphocytic enteritis, positive celiac genetics and negative celiac serology. Double-blind randomized clinical trial of gluten vs placebo rechallenge. >18 years of age, HLA-DQ2/8+, negative coeliac serology and gluten-dependent lymphocytic enteritis, and GI symptoms, with clinical and histological remission at inclusion. Eighteen patients were randomised: 11 gluten (20 g/day) and 7 placebo. Clinical symptoms, quality of life (GIQLI), and presence of gamma/delta+ cells and transglutaminase deposits were evaluated. 91% of patients had clinical relapse during gluten challenge versus 28.5% after placebo (p = 0.01). Clinical scores and GIQLI worsened after gluten but not after placebo (p<0.01). The presence of coeliac tissue markers at baseline biopsy on a gluten-free diet allowed classifying 9 out of the 18 (50%) patients as having probable 'coeliac lite' disease. This proof-of-concept study indicates that gluten is the trigger of symptoms in a subgroup of patients fulfilling the diagnostic criteria for NCGS. They were characterized by positive celiac genetics, lymphocytic enteritis, and clinical and histological remission after a gluten-free diet. ClinicalTrials.gov NCT02472704.

  11. Clinical Management of Patients with Ebola Virus Disease in Well-resourced Settings.

    PubMed

    Kato, Yasuyuki

    2015-01-01

    In outbreak response against Ebola virus disease (EVD), hospitals isolating the patients have a vital role to control disease transmission in communities. As of May 2015, there have been 7 suspected cases of EVD reported in Japan, but all of them were negative for ebolavirus. When a suspected case traveling from West Africa had no direct contact with EVD patients, the probability of EVD would be generally low. Patients with EVD seem more infectious when they have gastrointestinal symptoms. The peak of disease is usually observed at day 7-10 of illness. Over 25 patients with EVD have been treated in Europe and North America during the current outbreak. Lower mortality rate observed in the well-resourced settings could be attributable to aggressive supportive therapy including mechanical ventilation and renal replacement therapy. The safety and effectiveness of investigational drugs remain unknown. Protecting healthcare workers from infection is so important that guidelines on personal protective equipment and post-exposure prophylaxis are developing. Although the number of designated hospitals has increased across Japan, the current medical care system for patients with highly infectious diseases deserves reconsideration.

  12. The clinical characteristics of gastroesophageal reflux disease in patients with laryngeal symptoms who are referred to gastroenterology.

    PubMed

    Oh, J-H; Choi, M-G; Park, J-M; Lim, C-H; Cho, Y-K; Lee, I-S; Kim, S-W; Chung, I-S

    2013-07-01

    The prevalence of gastroesophageal reflux disease (GERD) has increased recently in Asia-Pacific countries. However, little is known about its prevalence and clinical characteristics in GERD patients with atypical symptoms in Asia. The aim of this study was to investigate the clinical characteristics of GERD in patients who had laryngeal symptoms in Korea. Data were gathered retrospectively from patients who presented with atypical symptoms, such as throat discomfort, globus pharyngeus, hoarseness, and chronic cough. They underwent a 24-hour ambulatory intraesophageal pH monitoring and filled in a validated reflux questionnaire. Overall, 128 patients (36 men and 92 women) with laryngeal symptoms were included. Of these 128, 43 patients (34%) had erosive esophagitis or pathological reflux from 24-hour ambulatory pH monitoring, and 24 (19%) had a positive Bernstein test or positive symptom index from 24-hour pH monitoring. Sixty-one patients (48%) had no evidence of reflux esophagitis on upper endoscopy and pathological acid reflux on 24-hour pH monitoring. Fifty-six patients (44%) had weekly heartburn or regurgitation. Typical symptoms and dyspepsia were significantly more common in patients with GERD who had laryngeal symptoms than non-GERD. Fifty-two percent of patients had laryngeal symptoms that were associated with GERD. The presence of typical reflux symptoms and dyspepsia are risk factors for GERD in patients who present with laryngeal symptoms. © 2012 Copyright the Authors. Journal compilation © 2012, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

  13. The value of power Doppler ultrasound in patients with rheumatoid arthritis in clinical remission: Reclassifying disease activity?

    PubMed

    Vergara, Facundo; Ruta, Santiago; Rosa, Javier; Marín, Josefina; García-Mónaco, Ricardo; Soriano, Enrique R

    2017-03-18

    The aim of the present study was to describe the ultrasound (US) findings in patients with rheumatoid arthritis (RA) in clinical remission, and to evaluate the ability of power Doppler (PD) US to reclassify disease activity in these patients. We included consecutive patients with RA according to 2010 American College of Rheumatology/European League Against Rheumatism criteria, who were in clinical remission by the Disease Activity Score (DAS28<2.6). Twenty joints of both hands were assessed by US. PD signal was evaluated on a semi-quantitative scale from 0 to 3. Three different US-modified DAS28 (US-DAS28) were constructed, replacing the clinical swollen joint count by the PD US joint count using PD score ≥1, ≥2 or ≥3, respectively. Eighty-six patients were included. Twenty-three (26.7%) patients had at least one joint with abnormal US-positive PD signal. Thirteen percent of patients were reclassified to low disease activity by applying the US-DAS28 when joints were considered active with a PD signal ≥1; 12%, when a PD signal ≥2 was considered, and 2% of the patients were reclassified when a PD score of 3 was considered. No patients were reclassified to a level of moderate or high activity applying US-DAS28. Although around a quarter of patients with RA in clinical remission showed PD US features indicating residual activity, only a small percentage were reclassified to a state of low activity and none to a level of moderate or high activity, applying the proposed US-DAS28. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  14. [Late AIDS diagnosis in patients hospitalized in Clinic of Infectious Diseases and Hepatology PAM in years 2003-2007].

    PubMed

    Bander, Dorota; Leszczyszyn-Pynka, Magdalena; Boroń-Kaczmarska, Anna

    2009-01-01

    Assessment of the phenomenon of the late HIV diagnosis, as defined by the disease diagnosis alongside with the AIDS defining illness (late HIV testing). Retrospective epidemiological and clinical data analysis of patients with confirmed HIV infection, treated in the Chair and Department of Infectious Diseases and Hepatology Pomeranian Medical University, Szczecin from 2003 to 2007 in comparison to data from 1989-2002. Data of 275 patients aged 1-71 years (mean 35+/-10) who attended the clinic for the first time in period 2003-2007 were collected. Individuals of male gender were prevailing in the analyzed group (78%), the percentage of women have been changing over the years with 32% in 2003, through 9% in 2005 to 30% in 2007. More than half of the patients presented clinical symptoms of immune deficiency: without AIDS--36% and with AIDS--22%. Lymphocyte T CD4+ count < 200 cells/microl among 118 of 275 new patients (43%) was noted. In the period 2003-2007, 59% of new patients were started on cART treatment. Late AIDS diagnosis was confirmed among 61% patients, with considerably higher frequency among men--84%. Late testers were three years older if compared to the whole of the newly diagnosed patients. The most common AIDS defining illness was the pulmonary and extrapulmonary tuberculosis, diagnosed in 13/51(25%) patients. In most of cases HIV-infected patients are referred to the infectious diseases specialist when the clinical symptoms of immune deficiency occur, often being deeply immunocompromised. It is truly terrifying, that this situation has not truly changed since 1989. This notion creates the field for discussion on the universality and accessibility of HIV screening among Poles, in order to introduce a more common diagnostics.

  15. Clinical features of patients diagnosed with degenerative rotator cuff tendon disease: a 6-month prospective-definitive clinical study from turkey.

    PubMed

    Koca, Tuba Tülay; Arslan, Aydın; Özdemir, Filiz; Acet, Günseli

    2017-08-01

    [Purpose] In rotator cuff tendon disease (RCTD), anamnesis is as important as clinical findings and anatomic/radio diagnostic examination. This study aimed to investigate the clinical features of patients diagnosed with RCTD using clinical and radiological methods between March 2015 and August 2015 at Malatya Research and Training Hospital Physical Medicine and Rehabilitation department. [Subjects and Methods] The study included 178 patients who were diagnosed with RCTD (128 females and 50 males). A questionnaire comprising 33 questions was given to each patient. [Results] Eighty-eight of the patients (49.9%) had an involvement on their right side, eighty-four (47.1%) had an involvement on their left side, and 6 had bilateral involvement. Mean visual analog scale (VAS) score was found to be statistically significant in favor of female patients. A statistically significant correlation was found between educational status and VAS. The body mass index (BMI) of the female patients was found to be higher than that of the male patients. Statistically significant correlation between doing risky work and gender showed that males were at a higher risk. [Conclusion] RCDT is more seen in people who have high BMI and are at their 50s. Pain complaint and BMI were found higher in female patients. DM, thyroid and cardiac diseases were seen more in the patients who are diagnosed with RCTD relative to the healthy population.

  16. Clinical and epidemiological characteristics of young patients with Peyronie's disease: a retrospective study.

    PubMed

    Paulis, Gianni; Cavallini, Giorgio; Barletta, Davide; Turchi, Paolo; Vitarelli, Antonio; Fabiani, Andrea

    2015-01-01

    The average age of men affected by Peyronie's disease (PD) is approximately 50-55 years, but cases have been reported even in adolescence. Several studies have already investigated the presence of PD in young men, and these studies reported a PD prevalence that varies between 1.5% and 10.8%. Having noticed a greater number of young patients in our centers in recent years, we decided to carry out a retrospective study to evaluate the prevalence of PD in patients aged <40 years, as well as to investigate any possible difference in evolution based on the age of PD patients. We selected a sample of patients (n=271) with a similar time of onset of disease. We then stratified all 271 patients into two groups: group A (age <40 years [n=46]) and group B (age ≥40 years [n=225]). All 271 patients were evaluated for the following variables: penile plaque volume, degree of penile curvature, penile pain, and erectile function. Plaque volume was measured in cm(3) by dynamic penile color Doppler sonography after administration of intracavernosal alprostadil 10 mcg. The number of younger patients was 46, accounting for 16.9% of the whole sample. Our study showed more frequent appearance and greater progression of penile curvature in younger patients. The average angle of penile curvature and average score of penile pain intensity in the younger men were significantly higher than in patients aged ≥40 years (P=0.025 and P=0.0001, respectively). In the younger patients, not only was the pain more intense (visual analog scale [VAS] of 5.2 versus 3.8), but it was also more frequently present than in patients aged ≥40 years (78.2% versus 62.2%) (P=0.042). We may conclude that since PD in young patients has a more acute onset and a greater possibility of progression, it should be treated conservatively as soon as it is diagnosed.

  17. Mild clinical behaviour of Crohn disease in elderly patients in a Latin American country: A case-control study

    PubMed Central

    Yamamoto-Furusho, Jesús K; Sarmiento-Aguilar, Andrea

    2015-01-01

    BACKGROUND: Crohn disease is characterized by fluctuating clinical behaviour, which is influenced by various factors. There are no data from Latin America that evaluate the clinical behaviour of Crohn disease in elderly patients. OBJECTIVE: To evaluate the clinical course of elderly onset Crohn disease compared with younger onset in the Mexican population. METHODS: The present analysis was a case-control study that included 132 patients with a histopathological diagnosis of Crohn disease between 1983 and 2013 in an inflammatory bowel disease clinic of a tertiary care centre. Statistical analysis was performed using SPSS version 17 (IBM Corporation, USA) and descriptive statistics, χ2 and Fisher’s exact test for categorical variables and Student’s t test for numerical variables. Univariate and multivariate analysis were performed to identify associated risk factors and OR was calculated. RESULTS: A total of 132 patients (73 men and 59 women) were divided into two groups according to age at diagnosis: 27 cases (>60 years of age) and 105 controls (≤60 years of age). Factors influencing the clinical course of Crohn disease in the elderly were: female sex (OR 2.55 [95% CI 1.06 to 6.10]; P=0.02); colonic location (OR 0.22 [95% CI 0.03 to 0.89]; P=0.02); mild clinical behaviour of disease (OR 10.08 [95% CI 3.74 to 27.17]; P=0.0001); response to medical treatment (OR 2.85 [95% CI 1.08 to 7.48]; P=0.02); frequent use of sulfasalazine (OR 4.46 [95% CI 1.22 to 16.28]; P=0.03); less use of azathioprine (OR 0.38 [95% CI 0.13 to 1.03]; P=0.04); and long-term remission (OR 4.96 [95% CI 1.70 to 14.48]; P=0.002). CONCLUSION: Elderly patients with Crohn disease had a mild clinical course characterized by the lack of escalation to immunosuppressive and anti-tumour necrosis factor therapy, as well as long-term remission. PMID:25996614

  18. Mild clinical behaviour of Crohn disease in elderly patients in a Latin American country: A case-control study.

    PubMed

    Yamamoto-Furusho, Jesús K; Sarmiento-Aguilar, Andrea

    2015-01-01

    Crohn disease is characterized by fluctuating clinical behaviour, which is influenced by various factors. There are no data from Latin America that evaluate the clinical behaviour of Crohn disease in elderly patients. To evaluate the clinical course of elderly onset Crohn disease compared with younger onset in the Mexican population. The present analysis was a case-control study that included 132 patients with a histopathological diagnosis of Crohn disease between 1983 and 2013 in an inflammatory bowel disease clinic of a tertiary care centre. Statistical analysis was performed using SPSS version 17 (IBM Corporation, USA) and descriptive statistics, χ2 and Fisher's exact test for categorical variables and Student's t test for numerical variables. Univariate and multivariate analysis were performed to identify associated risk factors and OR was calculated. A total of 132 patients (73 men and 59 women) were divided into two groups according to age at diagnosis: 27 cases (>60 years of age) and 105 controls (≤60 years of age). Factors influencing the clinical course of Crohn disease in the elderly were: female sex (OR 2.55 [95% CI 1.06 to 6.10]; P=0.02); colonic location (OR 0.22 [95% CI 0.03 to 0.89]; P=0.02); mild clinical behaviour of disease (OR 10.08 [95% CI 3.74 to 27.17]; P=0.0001); response to medical treatment (OR 2.85 [95% CI 1.08 to 7.48]; P=0.02); frequent use of sulfasalazine (OR 4.46 [95% CI 1.22 to 16.28]; P=0.03); less use of azathioprine (OR 0.38 [95% CI 0.13 to 1.03]; P=0.04); and long-term remission (OR 4.96 [95% CI 1.70 to 14.48]; P=0.002). Elderly patients with Crohn disease had a mild clinical course characterized by the lack of escalation to immunosuppressive and anti-tumour necrosis factor therapy, as well as long-term remission.

  19. A multicentre study of 513 Danish patients with systemic lupus erythematosus. I. Disease manifestations and analyses of clinical subsets.

    PubMed

    Jacobsen, S; Petersen, J; Ullman, S; Junker, P; Voss, A; Rasmussen, J M; Tarp, U; Poulsen, L H; van Overeem Hansen, G; Skaarup, B; Hansen, T M; Pødenphant, J; Halberg, P

    1998-01-01

    A Danish multicentre study was undertaken of the manifestations, infections, thrombotic events, survival and predictive factors of survival in 513 Danish patients with systemic lupus erythematosus (SLE) according to the 1982 classification criteria of the American College of Rheumatology. The mean duration of follow-up was 8.2 years from diagnosis and 12.8 years from first symptom. This paper describes the most common clinical and laboratory manifestations and their relationship to sex and age at the time of onset and diagnosis. Cluster analysis revealed three clinically defined clusters at the time of disease onset. Cluster 1 (57% of patients) consisted of relatively elderly patients without nephropathy or malar rash, but with a high prevalence of discoid lesions. Cluster 2 (18%) consisted of patients with nephropathy, a third of whom also developed serositis and lymphopenia. The patients of the third cluster (25%) all had malar rash and half were photosensitive. Follow-up showed that the patients of cluster 2 developed azotaemia, large proteinuria, arterial hypertension and myositis significantly more often than did the rest of the patients, but the mortality was not increased. The risk of developing renal end-stage disease was highest in men with early-onset disease.

  20. Cognitive decline in patients with Alzheimer's disease and its related factors in a memory clinic setting, Shanghai, China.

    PubMed

    Zhao, Qianhua; Zhou, Bin; Ding, Ding; Teramukai, Satoshi; Guo, Qihao; Fukushima, Masanori; Hong, Zhen

    2014-01-01

    Progressive cognitive decline is a characteristic hallmark of AD. It is important to identify prognostic markers to improve patient care and long-term planning. We aimed to identify the characteristics of disease progression in AD patients, focusing on cognitive decline and its related factors. Clinically diagnosed AD patients in a memory clinic were followed. The mini-mental state examination (MMSE) and a battery of other neuropsychological tests were performed to assess the rate of cognitive decline and to analyze the related factors. A total of 165 AD patients were analyzed for cognitive changes. The MMSE scores declined at a rate of 1.52 points per year. Most neuropsychological test scores deteriorated significantly over time. Younger and early-onset AD patients deteriorated more rapidly than older and late-onset patients in global cognition and executive function. Men declined faster in memory but slower in attention than women. Higher education was associated with more rapid deterioration in visuo-spatial ability. Family history, hypertension and cerebral vascular disease were also associated with disease progression. Attention, executive and visuo-spatial functions deteriorate at faster rates than other cognitive functions in AD patients. Age and age at onset were the main factors that associated with deterioration.

  1. Antiplatelet agents and anticoagulants in patients with chronic kidney disease - from pathophysiology to clinical practice.

    PubMed

    Lutz, Jens; Jurk, Kerstin

    2016-12-05

    Progressive impairment of renal function can lead to uremia, which is associated with thus increasing the risk of bleeding as well as thrombosis. Furthermore, many patients with chronic kidney disease (CKD) have an indication for an anticoagulation or antiplatelet therapy due to atrial fibrillation, coronary artery disease, thromboembolic disease, or peripheral artery disease. The treatment usually includes vitamin-K antagonists (VKAs) and/or platelet aggregation inhibitors. The direct oral anticoagulants (DOACs) inhibiting factor Xa or thrombin activity represent an alternative for heparins and VKAs. However, DOACs can further aggravate the bleeding risk in CKD patients. This is related to a combination of an accumulation of the substance due to the reduced renal clearance, an inhibition of thrombin-mediated platelet activation, and uremia associated factors such as impaired coagulation, platelet function, and platelet-vessel wall. Furthermore, platelet aggregation inhibitors can also influence the bleeding risk, particularly if they are administered in combination with anticoagulants in patients with advanced CKD. In this review we discuss the different mechanisms leading to the increased risk of bleeding and thrombosis as well as the different options and problems related to an antiplatelet or anticoagulation therapy in CKD patients.

  2. Patient Perspectives on Deep Brain Stimulation Clinical Research in Early Stage Parkinson's Disease.

    PubMed

    Heusinkveld, Lauren; Hacker, Mallory; Turchan, Maxim; Bollig, Madelyn; Tamargo, Christina; Fisher, William; McLaughlin, Lauren; Martig, Adria; Charles, David

    2017-01-01

    The FDA has approved a multicenter, double-blind, Phase III, pivotal trial testing deep brain stimulation (DBS) in 280 people with very early stage Parkinson's disease (PD; IDE#G050016). In partnership with The Michael J. Fox Foundation for Parkinson's Research, we conducted a survey to investigate motivating factors, barriers, and gender differences among potentially eligible patients for participation in a trial testing DBS in early PD compared to standard medical treatment. The majority of survey respondents (72%) indicated they would consider learning more about participating. Early PD patients are therefore likely to consider enrolling in trials of invasive therapies that may slow symptom progression and help future patients.

  3. The feasibility and short-term clinical outcomes of single-incision laparoscopic surgery for patients with complex Crohn's disease.

    PubMed

    Maeda, Kiyoshi; Nagahara, Hisashi; Shibutani, Masatsune; Fukuoka, Tatsunari; Nakao, Shigetomi; Yamagami, Hirokazu; Kamata, Noriko; Muguruma, Kazuya; Tanaka, Hiroaki; Toyokawa, Takahiro; Hirakawa, Kosei; Ohira, Masaichi

    2017-09-13

    Single-incision laparoscopic surgery (SILS) offers excellent cosmetic results compared with conventional multi-port laparoscopic surgery. Recently, this technique has been applied to Crohn's disease (CD) with primary ileocolic strictures; however, the application of a laparoscopic approach for complex CD, which involves abscess formation, fistula formation, and recurrent CD, is controversial. The aim of this study was to investigate the safety and feasibility of SILS for patients with complex disease and to compare its clinical results in patients with complex disease vs. those with simple stricture disease. Fifty patients who underwent SILS for CD were divided into two groups: those with complex disease (complex group, n = 25), and those with simple strictures (simple group, n = 25). The preoperative data and clinical outcomes were analyzed and compared between the groups. The operative time, blood loss and length of laparotomy incision were not significantly different between the groups. Although the rate of conversion and need for an additional port tended to be higher in the complex group, the rate of postoperative complications and length of hospital stay did not differ significantly between the groups. SILS may be feasible for carefully selected patients with complex CD.

  4. Oral mucosal lesions in skin diseased patients attending a dermatologic clinic: a cross-sectional study in Sudan

    PubMed Central

    2011-01-01

    Background So far there have been no studies focusing on the prevalence of a wide spectrum of oral mucosal lesions (OML) in patients with dermatologic diseases. This is noteworthy as skin lesions are strongly associated with oral lesions and could easily be neglected by dentists. This study aimed to estimate the frequency and socio-behavioural correlates of OML in skin diseased patients attending outpatient's facility of Khartoum Teaching Hospital - Dermatology Clinic, Sudan. Methods A cross-sectional hospital-based study was conducted in Khartoum from October 2008 to January 2009. A total of 588 patients (mean age 37.2 ± 16 years, 50.3% females) completed an oral examination and a personal interview of which 544 patients (mean age 37.1 ± 15.9 years, 50% females) with confirmed skin disease diagnosis were included for further analyses. OML were recorded using the World Health Organization criteria (WHO). Biopsy and smear were used as adjuvant techniques for confirmation. Data were analysed using the Statistical Package for Social Science (Version 15.0.1). Cross tabulation and Chi-square with Fisher's exact test were used. Results A total of 438 OML were registered in 315 (57.9%, males: 54.6% versus females: 45.6%, p < 0.05) skin diseased patients. Thus, a certain number of patients had more than one type of OML. Tongue lesions were the most frequently diagnosed OML (23.3%), followed in descending order by white lesions (19.1%), red and blue lesions (11%) and vesiculobullous diseases (6%). OML in various skin diseases were; vesiculobullous reaction pattern (72.2%), lichenoid reaction pattern (60.5%), infectious lesions (56.5%), psoriasiform reaction pattern (56.7%), and spongiotic reaction pattern (46.8%). Presence of OML in skin diseased patients was most frequent in older age groups (62.4% older versus 52.7% younger, p < 0.05), in males (63.2% males versus 52.6% females, p < 0.05), patients with a systemic disease (65.2% with systemic versus 51.9% without

  5. Clinical assessment tests in evaluating patients with chronic obstructive pulmonary disease

    PubMed Central

    Chuang, Ming-Lung; Lin, I-Feng; Lee, Chai-Yuan

    2016-01-01

    Abstract Exertional dyspnea scales (EDS) and health-related quality-of-life questionnaires (HRQoLQs) are used to assess chronic obstructive pulmonary disease (COPD). The GOLD guidelines categorize patients according to either 1 of these 2 domains, the lung function and the frequency of acute exacerbations in the preceding year, however with inconsistent results. Combining EDS and HRQoLQs may yield better results; however, the best combination is unclear. Whether the EDS quantifies the exercise capacity or the dyspnea perception is also unclear. The study was designed to correlate the EDS with exercise capacity and dyspnea perception and to evaluate the best combination of the EDS and HRQoLQ. Three EDS were compared by exercise capacity and Borg scores at rest and during exercise in 57 patients with COPD. Three HRQoLQs were compared by 4 domains of clinical assessments, and 2 types of exercise. The strength of correlation |r| was categorized by quartiles from <0.3 to ≥0.6. The EDS was better correlated with exercise capacities (|r| = 0.29–0.65, P < 0.05–<0.0001) than with the resting and exertional Borg scores (|r| = 0.08–0.55, P = NS- <0.0001). The EDS were moderately to strongly interrelated, but this correlation was weaker when including Oxygen-cost Diagram (OCD) (with the modified Medical Research Council, mMRC r = −0.56, with the baseline dyspnea index, BDI r = 0.49 vs. mMRC with BDI r = −0.73); however, the OCD had the strongest correlation with walking distance (r = 0.65, vs mMRC r = −0.59, BDI r = 0.5) and peak oxygen uptake (r = 0.39 vs mMRC r = −0.29, BDI r = 0.36). Among the HRQoLQs, the COPD assessment test (CAT) was most strongly correlated with the St. George Respiratory Questionnaire (SGRQ) (r = 0.77) and similar to the SGRQ regarding significant correlations with the other instruments (|r| = 0.29–0.67 vs. 0.36–0.77) but poorly with walking distance (r = −0.02). The

  6. Clinical impact of upper endoscopy in the management of patients with gastroesophageal reflux disease.

    PubMed

    Wo, John M; Mendez, Christian; Harrell, Steve; Joubran, Rauol; Bressoud, Phillip F; McKinney, William Paul

    2004-12-01

    Esophago-gastro-duodenoscopy (EGD) is frequently performed in patients with heartburn. However, the benefit of an EGD is unclear. The objective is to determine the clinical impact of performing an EGD in the management of GERD. Patients with alarm symptoms or persistent heartburn from primary care and gastroenterology clinics were invited to participate. Patients with known reflux complications or an EGD procedure within 3 yr were excluded. Endpoints for EGD-improving management were prospectively defined: change in medical therapy, dilating esophageal stricture, identifying Barrett's esophagus (BE), finding severe esophagitis (Hetzel-Dent grade 3 or 4), or finding cancer. Two hundred and six patients (59 M/147 F; median age, 48 yr) were enrolled, in which, 124 had alarm symptoms and 82 had persistent heartburn. EGD improved the overall management in 40% of patients with alarm symptoms and 29% with persistent heartburn (p= NS). Medical therapy was altered in only 5% of patients, and no cancer was identified. EGD was more likely to improve management in males with alarm symptoms. It was uncommon for an EGD to alter antireflux therapy. The management was improved in approximately one-third of the patients by dilating esophageal stricture, finding BE to initiate surveillance, or finding severe esophagitis. EGD should be performed when alarm symptoms are present, especially in males.

  7. Impact of pharmaceutical care on the quality of life of patients with Chagas disease and heart failure: randomized clinical trial

    PubMed Central

    2012-01-01

    Background Pharmaceutical care is the direct interaction between pharmacist and patient, in order to improve therapeutic compliance, promote adequate pharmacotherapeutic follow-up, and improve quality of life. Pharmaceutical care may be effective in reducing complications and in improving the quality of life of patients with chronic diseases, like Chagas heart disease, while bringing a positive impact on health system costs. The morbidity and mortality indexes for patients with Chagas heart disease are high, especially if this heart disease is complicated by heart failure. In this setting, we hypothesize that pharmaceutical care might be an important tool for the clinical management of these patients by improving their quality of life, as a better compliance to their treatment and the avoidance and prompt correction of drug-related problems will minimize their symptoms, improve their functional class, and decrease the number of hospital admissions. Therefore, the aim of this trial is to evaluate the contribution of pharmaceutical care to clinical treatment of patients with Chagas heart disease complicated by heart failure. Methods/design A prospective, single-center randomized clinical trial will be conducted in patients with Chagas heart disease complicated by heart failure. A total of 88 patients will be randomly assigned into two parallel groups: an intervention group will receive standard care and pharmaceutical care, and a control group will receive only standard care. Both groups will be subjected to a follow-up period of 12 months. The primary outcome of this trial is the evaluation of quality of life, measured by the 36-item short-form and the Minnesota Living with Heart Failure Questionnaire. Secondary outcomes include drug-related problems, exercise tolerance as measured by the standard six-minute-walk test, and compliance. Discussion Patients with Chagas heart disease complicated by heart failure under pharmaceutical care are expected to improve their

  8. Heart rate prediction for coronary artery disease patients (CAD): Results of a clinical pilot study.

    PubMed

    Müller-von Aschwege, Frerk; Workowski, Anke; Willemsen, Detlev; Müller, Sebastian M; Hein, Andreas

    2015-01-01

    This paper describes the results of a pilot study with cardiac patients based on information that can be derived from a smartphone. The idea behind the study is to design a model for estimating the heart rate of a patient before an outdoor walking session for track planning, as well as using the model for guidance during an outdoor session. The model allows estimation of the heart rate several minutes in advance to guide the patient and avoid overstrain before its occurrence. This paper describes the first results of the clinical pilot study with cardiac patients taking β-blockers. 9 patients have been tested on a treadmill and during three outdoor sessions each. The results have been derived and three levels of improvement have been tested by cross validation. The overall result is an average Median Absolute Deviation (MAD) of 4.26 BPM between measured heart rate and smartphone sensor based model estimation.

  9. Improvement of clinical parameters in patients with gastroesophageal reflux disease after radiofrequency energy delivery

    PubMed Central

    Liu, Hai-Feng; Zhang, Jian-Guo; Li, Jun; Chen, Xiao-Guang; Wang, Wei-An

    2011-01-01

    AIM: To evaluate the efficacy of Stretta procedure with gastroesophageal reflux disease (GERD) based on symptom control, medication changes and oesophagitis grade. METHODS: Ninety patients with a history of GERD underwent Stretta procedure from June 2007 to March 2010. All patients with GERD diagnosed by the presence of endoscopically evidenced oesophagitis or abnormal esophageal pH testing. We evaluated GERD-health-related quality of life, satisfaction, medication use and endoscopy at baseline, 6, 12 mo after treatment. Complications of the procedure were analyzed. RESULTS: We found that patients experienced significant changes in symptoms of GERD after Stretta procedure. The onset of GERD symptom relief was less than 2 mo (70.0%) or 2 to 6 mo (16.7%). The mean GERD-HRQL score was 25.6 (baseline), 7.3 (6 mo, P < 0.01), and 8.1 (12 mo, P < 0.01).The mean heartburn score was 3.3 (baseline), and 1.2 (12 mo, P < 0.05). The percentage of patients with satisfactory GERD control improved from 31.1% at baseline to 86.7% after treatment, and patient satisfaction improved from 1.4 at baseline to 4.0 at 12 mo (P < 0.01). Medication usage decreased significantly from 100% of patients on proton pump inhibitors therapy at baseline to 76.7% of patients showing elimination of medications or only as needed use of antacids/H2-RA at 12 mo. An improvement in endoscopic grade of oesophagitis was seen in 33 of the 41 patients. All patients had either no erosions or only mild erosive disease (grade A) at 6 mo. CONCLUSION: The experience with Stretta procedure confirms that it is well tolerated, safe, effective and durable in the treatment of GERD. The Stretta procedure provides the drug-refractory patients with a new minimally invasive method. PMID:22110270

  10. Does psychological status influence clinical outcomes in patients with inflammatory bowel disease (IBD) and other chronic gastroenterological diseases: An observational cohort prospective study

    PubMed Central

    Mikocka-Walus, Antonina A; Turnbull, Deborah A; Moulding, Nicole T; Wilson, Ian G; Holtmann, Gerald J; Andrews, Jane M

    2008-01-01

    Background Whether there is a temporal relationship between psychological problems and clinical outcomes in patients with diseases of the digestive tract has not been widely researched. Thus, our aims were 1) To observe and compare prospectively clinical outcomes in relation to psychological co-morbidity in patients with inflammatory bowel disease (IBD), irritable bowel syndrome (IBS) and chronic hepatitis C (HCV) and, 2) To test the hypothesis that patients with psychological co-morbidities are less likely to have a satisfactory response to standard treatment at 12 months. Methods Overall, 139 patients were enrolled in this observational cohort prospective study. Over the ensuing year, physical and psychological measures were made at baseline and after 12 months (HADS, SCL90, SF-12 and disease activity measures). A logistic regression was conducted to observe any relationship between baseline characteristics and patients' clinical outcomes after 12 months. Results Overall, there was no relationship between psychological status and quality of life at baseline and relapse at 12 months (p > 0.05). However, patients with inactive disease at baseline were at lower risk of relapse after 12 months (OR = 0.046, CI: 0.012–0.178). No significant relationship was found between psychological problems such as depression/anxiety and a total number of relapses in the IBD group. However, interestingly, patients with an active disease at baseline tended to have a greater number of relapses (OR = 3.07, CI: 1.650–5.738) and CD participants were found at lower risk of relapse than UC participants (OR = 0.382, CI: 0.198–0.736). Conclusion In contrast to previous investigations, this study suggests that there is no temporal relationship between psychological problems at baseline and clinical outcomes over time. Longer and larger prospective studies are needed to better understand this result. PMID:18538012

  11. Eradication of Helicobacter pylori infection might improve clinical status of patients with Parkinson's disease, especially on bradykinesia.

    PubMed

    Liu, Huijing; Su, Wen; Li, Shuhua; Du, Wei; Ma, Xinxin; Jin, Ying; Li, Kai; Chen, Haibo

    2017-09-01

    Previous studies have shown that Helicobacter pylori infection might make clinical status worse in patients with Parkinson's disease and Helicobacter pylori eradication might improve clinical status by modifying the pharmacokinetics of L-dopa. Here, we investigate whether Helicobacter pylori eradication could benefit idiopathic parkinsonism and Helicobacter pylori infection will effect which aspect of motor symptom significantly. A cohort study involving idiopathic Parkinson's disease patients, screened for Helicobacter status by (13)C urea breath test. Clinical status was evaluated by using the Unified Parkinson's Disease Rating Scale (UPDRS) and Hoehn-Yahr stage. If patients had motor complications, they were quantified at the "on" time. The Helicobacter pylori positive patients could choose to receive Helicobacter pylori eradication or not by themselves. Group 1 was Helicobacter pylori negative patients. Group 2 was Helicobacter pylori positive patients who didn't receive eradication treatment. Group 3 was Helicobacter pylori positive patients who received successful eradication treatment. Repeat clinical assessments and (13)C urea breath test was performed at 1year later. Numerical data were expressed as mean±standard deviation (SD) RESULTS: Ninety-four consecutive patients with Parkinson's disease were recruited and underwent the initial (13)C urea breath test, but only forty-eight patients successfully completed the total study. In Group 3, the UPDRS-III scores (=Motor Examination Section Scores) were significantly lower 1year later compared to baseline (18.3±8.38 vs. 25.9±8.37, P=0.007). The differences were main in UPDRS-23 (=Finger Taps) (1.7±1.16 vs. 2.4±1.51, P=0.045), UPDRS-25 (Rapid Alternation Movements of Hands) (1.6±1.35 vs. 2.4±1.71, P=0.031) and UPDRS-26 (=Leg Agility) (1.3±1.25 vs.2.1±0.99, P=0.011). There was difference among three groups in the UPDRS-26 (P=0.040) of clinical status change of one year. The eradication of Helicobacter

  12. The antiplatelet effects of nitrates: is it of clinical significance in patients with cardiovascular disease?

    PubMed

    Zhou, Rui-Hai; Frishman, William H

    2010-01-01

    Organic nitrates have been used for over a century in cardiovascular therapy and are still widely used in the treatment of acute coronary syndromes, chronic angina pectoris, and congestive heart failure. Nitrates, together with sodium nitroprusside, generally referred to as nitrovasodilators, exert their biologic effects via the release of nitric oxide. They are also known as nitric oxide donors. The mechanism of action of these drugs is traditionally believed to lie in their arterial vasodilation and venodilation effects, resulting in an improvement of coronary artery blood supply and/or reduction of cardiac workload in the treatment of coronary artery disease and congestive heart failure. Recently it has been recognized that these drugs also have intrinsic antiplatelet and antithrombotic effects, demonstrated both in vitro and in vivo, which would add further rationale for the use of these drugs in atherothrombotic diseases. Research has shown that nitrovasodilators can nonselectively inhibit platelet aggregation induced by multiple stimuli. However, clinical trials have yielded conflicting results regarding clinical outcome, especially with long-term nitrate use. The potentially beneficial effects of nitrates could be negated by the development of tolerance and the generation of deleterious oxidative stress causing endothelial dysfunction during continuous nitrate administration. Much progress has been made in the development of new nitric oxide donors devoid of oxidant-generating properties. Novel combination therapies with nitrovasodilators plus antioxidants or agents with antioxidant properties have shown promise in reducing or reversing tolerance, potentiating antiplatelet effects, and improving clinical outcome. It is expected that clinical introduction of novel nitrovasodilator regimens will provide a new approach to the prevention and treatment of atherothrombotic diseases. Large-scale clinical trials will ultimately provide the evidence-based answers.

  13. Patient's perceptions of chronic kidney disease and their association with psychosocial and clinical outcomes: a narrative review

    PubMed Central

    Clarke, Amy L.; Yates, Thomas; Smith, Alice C.; Chilcot, Joseph

    2016-01-01

    Patients with chronic kidney disease (CKD) form organized beliefs regarding their illness and treatment. These perceptions influence the coping strategies employed by an individual to manage his/her illness and may act as a predictor for his/her willingness to engage in self-management behaviours. While illness perceptions have been identified as predictors of non-adherence, depression and mortality in dialysis patients, there is a paucity of research in CKD patients not requiring renal replacement therapy. This narrative review synthesizes the existing literature regarding the role of illness perceptions and associated clinical and psychosocial outcomes in non-dialysis CKD patients. Studies were identified following database searches of AMED, BNI, CINAHL, EMBASE, Health Business Elite, HMIC, Medline, PsycINFO and Google Scholar in January 2016. Despite the small evidence base, existing studies indicate that negative illness perceptions are associated with disease progression and a number of psychosocial outcomes in non-dialysis CKD patients. Evidence from other clinical populations suggests that illness perceptions are modifiable through psychological intervention, which may be most effective if delivered early before beliefs have the chance to become more established. Therefore, targeting illness perceptions in the earlier stages of CKD may be optimal. Further studies are now required to ascertain the mechanisms through which illness perceptions predict psychosocial and clinical outcomes in CKD patients and to ultimately test the efficacy of illness perception–based interventions. PMID:27274839

  14. Clinical and Genetic Characteristics of Mexican Patients with Juvenile Presentation of Niemann-Pick Type C Disease

    PubMed Central

    Piña-Aguilar, Raul E.; Vera-Loaiza, Aurea; Chacón-Camacho, Oscar F.; Zenteno, Juan Carlos; Nuñez-Orozco, Lilia; Santillán-Hernández, Yuritzi

    2014-01-01

    Niemann-Pick type C disease (NPC) is a rare lysosomal disease with a protean presentation, ranging from a fatal neonatal course with visceromegaly to an adult presentation with only neurological or psychiatric symptomatology. In this report we describe the genetic and clinical characteristics of 3 Mexican patients from different families with juvenile presentation of NPC. Clinical examination, imaging of central nervous and gastrointestinal system, and EEG were performed. Genetic studies include sequencing and deletion/duplication analysis of NPC1 and NPC2 genes. All patients presented with cognitive impairment, ataxia, and supranuclear vertical gaze palsy; one case had gelastic cataplexy. Also they developed epilepsy and cortical atrophy and two patients had thinning of corpus callosum. The 3 patients were compound heterozygotes for NPC1 sequence variants, including 5 missense and 1 nonsense mutations: p.P1007A and p.F1087L in Case 1; p.Q921P and p.G992R in Case 2; and p.R348∗ and p.V1165M in case 3. Mexican patients with juvenile NPC presented with a variable clinical phenotype and compound heterozygosity. This suggests a relative high frequency of mutation carriers as it is reported for European population. Consequently, clinicians should consider NPC as a diagnosis possibility in any adolescent or young adult patient with juvenile dementia and/or ataxia, even in absence of gelastic cataplexy and supranuclear vertical gaze palsy. PMID:25349751

  15. Granulomatous disease in CVID: retrospective analysis of clinical characteristics and treatment efficacy in a cohort of 59 patients.

    PubMed

    Boursiquot, Jean-Nicolas; Gérard, Laurence; Malphettes, Marion; Fieschi, Claire; Galicier, Lionel; Boutboul, David; Borie, Raphael; Viallard, Jean-François; Soulas-Sprauel, Pauline; Berezne, Alice; Jaccard, Arnaud; Hachulla, Eric; Haroche, Julien; Schleinitz, Nicolas; Têtu, Laurent; Oksenhendler, Eric

    2013-01-01

    Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder. To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria. Clinical and laboratory features of CVID patients were collected from the French DEFI cohort, a prospective study on adults with hypogammaglobulinemia. The medical charts of 55 patients (93 %) of the GD cohort were reviewed. Among 436 subjects with CVID, 59 patients (13.5 %) were diagnosed with GD. Of the 55 patients in whom medical charts were available, 32 patients received treatment for the granulomatous disease. Corticosteroids were the most frequently used drug. Complete response to treatment was infrequent. It was achieved with corticosteroids, cyclophosphamide, hydroxychloroquine, rituximab and methotrexate. Azathioprine, cyclosporine, mycophenolate mofetil, sirolimus, infliximab and thalidomide led to partial or absence of response. Complete and partial responses were observed in lymph nodes, lungs, liver, skin, bone marrow and central nervous system. Absent of response for gastrointestinal tract granulomas was noted in all cases of treatment attempt. CVID patients with GD exhibit a particular biological phenotype. Treatment should be considered in any symptomatic patient or if there is evidence of organ dysfunction. Corticosteroids are the drug of choice in most instances but response to treatment is often unsatisfactory.

  16. Clinical classification of adult patients with chronic intestinal failure due to benign disease: An international multicenter cross-sectional survey.

    PubMed

    Pironi, Loris; Konrad, Denise; Brandt, Chrisoffer; Joly, Francisca; Wanten, Geert; Agostini, Federica; Chambrier, Cecile; Aimasso, Umberto; Zeraschi, Sarah; Kelly, Darlene; Szczepanek, Kinga; Jukes, Amelia; Di Caro, Simona; Theilla, Miriam; Kunecki, Marek; Daniels, Joanne; Serlie, Mireille; Poullenot, Florian; Wu, Jian; Cooper, Sheldon C; Rasmussen, Henrik H; Compher, Charlene; Seguy, David; Crivelli, Adriana; Pagano, Maria C; Hughes, Sarah-Jane; Guglielmi, Francesco W; Kozjek, Nada Rotovnik; Schneider, Stéphane M; Gillanders, Lyn; Ellegard, Lars; Thibault, Ronan; Matras, Przemysław; Zmarzly, Anna; Matysiak, Konrad; Van Gossum, Andrè; Forbes, Alastair; Wyer, Nicola; Taus, Marina; Virgili, Nuria M; O'Callaghan, Margie; Chapman, Brooke; Osland, Emma; Cuerda, Cristina; Sahin, Peter; Jones, Lynn; Lee, Andre D W; Bertasi, Valentino; Orlandoni, Paolo; Izbéki, Ferenc; Spaggiari, Corrado; Díez, Marta Bueno; Doitchinova-Simeonova, Maryana; Garde, Carmen; Serralde-Zúñiga, Aurora E; Olveira, Gabriel; Krznaric, Zeljko; Czako, Laszlo; Kekstas, Gintautas; Sanz-Paris, Alejandro; Jáuregui, Estrella Petrina; Murillo, Ana Zugasti; Schafer, Eszter; Arends, Jann; Suárez-Llanos, José P; Shaffer, Jon; Lal, Simon

    2017-04-19

    The aim of the study was to evaluate the applicability of the ESPEN 16-category clinical classification of chronic intestinal failure, based on patients' intravenous supplementation (IVS) requirements for energy and fluids, and to evaluate factors associated with those requirements. ESPEN members were invited to participate through ESPEN Council representatives. Participating centers enrolled adult patients requiring home parenteral nutrition for chronic intestinal failure on March 1st 2015. The following patient data were recorded though a structured database: sex, age, body weight and height, intestinal failure mechanism, underlying disease, IVS volume and energy need. Sixty-five centers from 22 countries enrolled 2919 patients with benign disease. One half of the patients were distributed in 3 categories of the ESPEN clinical classification. 9% of patients required only fluid and electrolyte supplementation. IVS requirement varied considerably according to the pathophysiological mechanism of intestinal failure. Notably, IVS volume requirement represented loss of intestinal function better than IVS energy requirement. A simplified 8 category classification of chronic intestinal failure was devised, based on two types of IVS (either fluid and electrolyte alone or parenteral nutrition admixture containing energy) and four categories of volume. Patients' IVS requirements varied widely, supporting the need for a tool to homogenize patient categorization. This study has devised a novel, simplified eight category IVS classification for chronic intestinal failure that will prove useful in both the clinical and research setting when applied together with the underlying pathophysiological mechanism of the patient's intestinal failure. Copyright © 2017 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.

  17. Clinical outcomes of kidney transplants on patients with end-stage renal disease secondary to lupus nephritis, polycystic kidney disease and diabetic nephropathy

    PubMed Central

    Nieto-Ríos, John Fredy; Builes-Rodriguez, Sheila Alexandra; Restrepo-Correa, Ricardo Cesar; Aristizabal-Alzate, Arbey; Ocampo-Kohn, Catalina; Serna-Campuzano, Angélica; Cardona-Díaz, Natalia; Giraldo-Ramirez, Nelson Darío; Zuluaga-Valencia, Gustavo Adolfo

    2016-01-01

    Background: Patients with lupus nephritis could progress to end-stage renal disease (10-22%); hence, kidney transplants should be considered as the treatment of choice for these patients. Objective: To evaluate the clinical outcomes after kidney transplants in patients with chronic kidney diseases secondary to lupus nephritis, polycystic kidney disease and diabetes nephropathy at Pablo Tobon Uribe Hospital. Methods: A descriptive and retrospective study performed at one kidney transplant center between 2005 and 2013. Results: A total of 136 patients, 27 with lupus nephritis (19.9%), 31 with polycystic kidney disease (22.8%) and 78 with diabetes nephropathy (57.4%), were included in the study. The graft survivals after one, three and five years were 96.3%, 82.5% and 82.5% for lupus nephritis; 90%, 86% and 76.5% for polycystic kidney disease and 91.7%, 80.3% and 67.9% for diabetes nephropathy, respectively, with no significant differences (p= 0.488); the rate of lupus nephritis recurrence was 0.94%/person-year. The etiology of lupus vs diabetes vs polycystic disease was not a risk factor for a decreased time of graft survival (Hazard ratio: 1.43; 95% CI: 0.52-3.93). Conclusion: Kidney transplant patients with end stage renal disease secondary to lupus nephritis has similar graft and patient survival success rates to patients with other kidney diseases. The complication rate and risk of recurrence for lupus nephritis are low. Kidney transplants should be considered as the treatment of choice for patients with end stage renal disease secondary to lupus nephritis. PMID:27226665

  18. Clinical Application and Pharmacodynamic Monitoring of Apixaban in a Patient with End-Stage Renal Disease Requiring Chronic Hemodialysis.

    PubMed

    Kufel, Wesley D; Zayac, Adam S; Lehmann, David F; Miller, Christopher D

    2016-11-01

    Despite prescribing guidance, limited data exist to describe the use of apixaban in patients with end-stage renal disease (ESRD) requiring hemodialysis (HD). Current apixaban dosing recommendations for this patient population are based largely on a single-dose pharmacokinetic study of eight patients. We describe the clinical application and pharmacodynamic monitoring of apixaban in a 62-year-old 156-kg African-American woman with nonvalvular atrial fibrillation and ESRD requiring hemodialysis who developed calciphylaxis while receiving warfarin therapy. Based on a multidisciplinary clinical judgment decision due to concern for drug accumulation after multiple doses in patients with ESRD receiving HD, she was anticoagulated with apixaban 2.5 mg twice/day, as opposed to 5 mg twice/day as recommended by the package insert. Antifactor Xa monitoring was used, and resultant peak and trough apixaban concentrations were above the upper limit of detection for our clinical laboratory (more than 2.00 IU/ml). On day 7 of her hospitalization, the patient developed gastrointestinal bleeding, and apixaban was discontinued; no further clinical signs of bleeding occurred during her subsequent hospitalization course. Use of the Naranjo Adverse Drug Reaction Probability Scale indicated a probable relationship (score of 6) between apixaban exposure and the manifestation of gastrointestinal bleeding. The patient ultimately died 44 days after the acute bleeding event; however, coagulation concerns were not implicated in the patient's death. To our knowledge, this is the first case report that describes apixaban use and associated antifactor Xa monitoring in a patient with ESRD receiving HD, and it provides concern for current apixaban dosing recommendations in this patient population. Further pharmacokinetic and clinical data are likely necessary to better characterize apixaban use in these patients to optimize safety and efficacy. © 2016 Pharmacotherapy Publications, Inc.

  19. Matrix Metalloproteinases 2 and 9 Are Differentially Expressed in Patients with Indeterminate and Cardiac Clinical Forms of Chagas Disease

    PubMed Central

    Fares, Rafaelle Christine Gomes; Gomes, Juliana de Assis Silva; Garzoni, Luciana Ribeiro; Waghabi, Mariana Caldas; Saraiva, Roberto Magalhães; Medeiros, Nayara Ingrid; Oliveira-Prado, Roberta; Sangenis, Luiz Henrique Conde; Chambela, Mayara da Costa; de Araújo, Fernanda Fortes; Teixeira-Carvalho, Andréa; Damásio, Marcos Paulo; Valente, Vanessa Azevedo; Ferreira, Karine Silvestre; Sousa, Giovane Rodrigo; Rocha, Manoel Otávio da Costa

    2013-01-01

    Dilated chronic cardiomyopathy (DCC) from Chagas disease is associated with myocardial remodeling and interstitial fibrosis, resulting in extracellular matrix (ECM) changes. In this study, we characterized for the first time the serum matrix metalloproteinase 2 (MMP-2) and MMP-9 levels, as well as their main cell sources in peripheral blood mononuclear cells from patients presenting with the indeterminate (IND) or cardiac (CARD) clinical form of Chagas disease. Our results showed that serum levels of MMP-9 are associated with the severity of Chagas disease. The analysis of MMP production by T lymphocytes showed that CD8+ T cells are the main mononuclear leukocyte source of both MMP-2 and MMP-9 molecules. Using a new 3-dimensional model of fibrosis, we observed that sera from patients with Chagas disease induced an increase in the extracellular matrix components in cardiac spheroids. Furthermore, MMP-2 and MMP-9 showed different correlations with matrix proteins and inflammatory cytokines in patients with Chagas disease. Our results suggest that MMP-2 and MMP-9 show distinct activities in Chagas disease pathogenesis. While MMP-9 seems to be involved in the inflammation and cardiac remodeling of Chagas disease, MMP-2 does not correlate with inflammatory molecules. PMID:23856618

  20. [Fournier's gangrene in a patient with Hodgkin's disease: a clinical case].

    PubMed

    Castellini, C; De Nitto, F; Bonomo, S; Bottura, D; Mazzola, F; Ballarin, A; Rosa, G

    2001-01-01

    The authors report a case of Fournier's gangrene in a 54-year-old patient subjected 6 days earlier to chemotherapy for mediastinal Hodgkin's disease. The patient had fever and reported the onset of worsening pain and heat sensations in the inguinal, perineal and scrotal areas. Objectively, there was local oedema followed by the onset of crepitation. The patient had a very low white blood cell count (900/cu.mm). The Patient underwent emergency surgery with multiple, communicating incisions in the inguinal, perineal and scrotal areas, with the removal of necrotic tissue and daily washing with physiological solution and 12% H2O2. He also received antibiotic treatment with metronidazole and gentamicin and 5 cycles of high-pressure oxygen therapy, with disappearance of pain and fever and good local tissue repair.

  1. Clinical variations in Parkinson’s disease patients with or without REM sleep behaviour disorder: a meta-analysis

    PubMed Central

    Zhu, Ruo-lin; Xie, Cheng-juan; Hu, Pan-pan; Wang, Kai

    2017-01-01

    This study aimed to evaluate the clinical variations in patients with Parkinson’s disease (PD) with (PDRBD) or without REM sleep behaviour disorder (RBD) (Non-RBD), and PDRBD patients were classified into Confirmed-RBD (definite diagnosis with polysomnography, PSG) and Probable-RBD (without PSG re-confirmation). The clinical difference between the groups of patients was measured as an odds ratio (OR) or standardized mean difference (SMD, Cohen d). A total of 31 articles with data from 5,785 participants were obtained for our analysis. Overall, the occurrence of Confirmed-RBD was more frequent in male patients (OR = 1.25; p = 0.038), elderly patients (SMD = 0.25; p = 0.000), and patients with longer disease duration (SMD = 0.30; p = 0.000), increased Hoehn-Yahr scale (SMD = 0.30; p = 0.000), and higher UPDRS-III score (SMD = 0.38; p = 0.002). On the other hand, the frequency of Probable-RBD was increased with disease duration (SMD = 0.29; p = 0.000), Hoehn-Yahr scale (SMD = 0.30; p = 0.000), and UPDRS-III score (SMD = 0.26; p = 0.001). Our study indicate that PDRBD patients may have different clinical features compared to patients with Non-RBD. PMID:28091622

  2. Clinical characteristics and peripheral T cell subsets in Parkinson's disease patients with constipation.

    PubMed

    Chen, Yuhua; Yu, Meiling; Liu, Xiaolin; Qu, Hongdang; Chen, Qiming; Qian, Weidong; Wei, Daoxiang; Xu, Wenfang; Ma, Bo; Wu, Wei

    2015-01-01

    Constipation is frequently reported in Parkinson's disease (PD). We evaluated the characteristics of patients with PD and constipation and explored the role of T cell subsets in PD-associated constipation. One hundred and two patients with PD treated at the First Affiliated Hospital of Bengbu Medical College were enrolled in this study between January 2012 and October 2013. All patients completed KESS questionnaires and constipation was rated. The proportions of peripheral blood Thl7 and Treg cells were assessed by flow cytometry in 45 patients. Colonoscopies were performed in six patients. Thirty-one patients with PD reported slow-transit constipation (STC), 15 rectal evacuation disorder (RED) and 33 mixed constipation (Mixed). STC most frequently occurred before onset of PD motor symptoms, while Mixed occurred before or after motor symptoms, and RED occurred most frequently after motor symptoms. CD4+ T cell infiltration in the colonic mucosa was observed in patients with PD and constipation. The frequency of Th17 and Treg cells in patients with PD and constipation was significantly higher than in those without constipation (P<0.001). Among patients with PD and constipation, the frequency of Th17 and Treg cells in STC was the highest. However, there was no difference in the ratio of Th17/Tregs between the patients with PD with and without constipation, or patients with PD and different types of constipations (P>0.05). Constipation reported before the onset of PD motor symptoms was most often STC or Mixed, and PD constipation may be associated with immune activation in the colonic mucosa.

  3. Clinical perspective: Linking psychosocial care to the disease continuum in patients with multiple myeloma.

    PubMed

    Zabora, James; Buzaglo, Joanne; Kennedy, Vicki; Richards, Tiffany; Schapmire, Tara; Zebrack, Brad; Ghobrial, Irene M

    2015-08-01

    A model of psychosocial care specific for patients with multiple myeloma and their caregivers has not yet been proposed. We sought to develop a model of care that considers the specific profile of this disease. The authors, representing a multidisciplinary care team, met in December of 2012 to identify a model of psychosocial care for patients with multiple myeloma and their caregivers. This model was determined by consensus during the meeting and via total agreement following the meeting. The meeting was sponsored by Onyx Pharmaceuticals. The need for targeted psychosocial care for the multiple myeloma patient and caregiver throughout the disease process is essential to ensure quality of life and optimal treatment outcomes. We propose herein the first known model of care for the treatment of multiple myeloma that engages both the patient and their caregivers. Innovative partnerships between psychosocial providers and other entities such as pharmaceutical companies can maximize resources for comprehensive program development. This manuscript proposes a model of care that promotes active engagement in therapies for multiple myeloma while engaging the individual patient and their family caregivers. This treatment approach must be evidence based in terms of distress screening tools, comprehensive psychosocial assessments, and, most importantly, in the interventions and measurements of response that clinicians apply to this population.

  4. Immediately loaded fixed maxillary implant treatment for a patient with advanced periodontal disease: a clinical report.

    PubMed

    Binon, Paul

    2010-12-01

    Patients with a failing maxillary dentition typically require an extended treatment sequence that involves extractions, interim prosthesis wear, grafting and subsequent implant placement, and restoration with an implant-supported fixed or bar-retained prosthesis. Established maxillary implant protocols can take up to 15 or more months to complete. The purpose of this report is to describe a treatment sequence using zygomatic implants in a patient with advanced periodontal disease. A fixed prosthesis was fabricated without grafting, resulting in a shortened treatment time. Copyright © 2010 The Editorial Council of the Journal of Prosthetic Dentistry. Published by Mosby, Inc. All rights reserved.

  5. [Cardiovascular disease in diabetic patients: risk factors, clinical history and prevention].

    PubMed

    Avogaro, Angelo

    2016-12-01

    Cardiovascular disease (CVD) is the leading cause of death in patients with diabetes mellitus (DM) in which there is a rapid evolution and widespread early atherosclerosis, whose causes are manifold. In the presence of hyperglycemia there is the activation of multiple signaling responses involving, first endothelial activation and later dysfunction, which are the first detectable step toward the atherosclerotic disease. A healthy lifestyle is the cornerstone for the prevention and control of CVD in patients with DM, in whom we must pursue the control, not only of blood sugar levels, but also of all risk factors for CVD. The choice for an antidiabetic agent is based not only on its effectiveness but also on its safety. The data obtained from the recent cardiovascular outcome studies such as the LEADER and SUSTAIN-6 for liraglutide and semaglutide, respectively, and the EMPA-REG OUTCOME for empagliflozin, testify the need for the diabetologist of new therapeutic approaches to control glucose levels.

  6. Clinical and mutational spectrum of Japanese patients with Charcot-Marie-Tooth disease caused by GDAP1 variants.

    PubMed

    Yoshimura, A; Yuan, J-H; Hashiguchi, A; Hiramatsu, Y; Ando, M; Higuchi, Y; Nakamura, T; Okamoto, Y; Matsumura, K; Hamano, T; Sawaura, N; Shimatani, Y; Kumada, S; Okumura, Y; Miyahara, J; Yamaguchi, Y; Kitamura, S; Haginoya, K; Mitsui, J; Ishiura, H; Tsuji, S; Takashima, H

    2017-09-01

    Mutations in GDAP1 are responsible for heterogeneous clinical and electrophysiological phenotypes of Charcot-Marie-Tooth disease (CMT), with autosomal dominant or recessive inheritance pattern. The aim of this study is to identify the clinical and mutational spectrum of CMT patients with GDAP1 variants in Japan. From April 2007 to October 2014, using three state-of-art technologies, we conducted gene panel sequencing in a cohort of 1,030 patients with inherited peripheral neuropathies (IPNs), and 398 mutation-negative cases were further analyzed with whole-exome sequencing. We identified GDAP1 variants from 10 patients clinically diagnosed with CMT. The most frequent recessive variant in our cohort (5/10), c.740C>T (p.A247V), was verified to be associated with a founder event. We also detected three novel likely pathogenic variants: c.928C>T (p.R310W) and c.546delA (p.E183Kfs*23) in Case 2 and c.376G>A (p.E126K) in Case 8. Nerve conduction study or sural nerve biopsy of all 10 patients indicated axonal type peripheral neuropathy. We identified GDAP1 variants in approximately 1% of our cohort with IPNs, and established a founder mutation in half of these patients. Our study originally described the mutational spectrum and clinical features of GDAP1-related CMT patients in Japan. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  7. Clinical Study of Serum Homocysteine and Non-Alcoholic Fatty Liver Disease in Euglycemic Patients

    PubMed Central

    Hu, Yanjin; Liu, Jia; Dong, Xuejie; Xu, Yuan; Leng, Song; Wang, Guang

    2016-01-01

    Background Non-alcoholic fatty liver disease (NAFLD) is a chronic liver disease. NAFLD includes a spectrum of hepatic pathologies: simple fatty liver, steatohepatitis and cirrhosis. Insulin resistance may contribute to NAFLD. The liver plays an important role in the production and metabolism of homocysteine (HCY), which is known to be an independent risk factor for cardiovascular disease. High HCY level can aggravate NAFLD by increasing the reactive oxygen species and activating oxidative stress. In this study, we investigated the relationship between HCY and NAFLD in euglycemic patients. Material/Methods A total of 1143 euglycemic patients were recruited: 519 patients with non-alcoholic fatty liver disease (NAFLD) and 624 sex and age-matched controls without NAFLD. Results The NAFLD group had significantly higher HCY level (13.78±5.84 vs. 11.96±3.58 mmol/L, p<0.001), as well as higher body mass index (BMI), total cholesterol (TC), low density lipoprotein cholesterol (LDL-C), triglyceride (TG), glutamic-pyruvic transaminase (ALT), glutamic-oxalacetic transaminase (AST), fasting plasma glucose (FPG), fasting insulin (FINS), homeostasis model assessment for insulin resistance (HOMA-IR), homeostasis model assessment for beta cell function (HOMA-B), and lower high density lipoprotein cholesterol (HDL-C). HCY level was positively correlated with HOMA-IR (r=0.239, p<0.001), TG (r=0.356, p<0.001) and negatively correlated with HDL-C (r=−0.161, p<0.001). In the logistic regression analysis, BMI (beta=0.345, p<0.001), HOMA-IR (beta=0.654, p<0.01), TG (beta=0.881, p<0.001), and HCY (beta=0.04, p=0.044) were the predictors of NAFLD. Conclusions Higher HCY level existed in NAFLD patients and was correlated with the severity of insulin resistance. HCY is an independent risk factor for NAFLD. PMID:27803497

  8. Self-management in patients with inflammatory bowel disease: strategies, outcomes, and integration into clinical care.

    PubMed

    Plevinsky, Jill M; Greenley, Rachel N; Fishman, Laurie N

    2016-01-01

    Self-management, including medication adherence, is associated with improved health and outcomes for patients with inflammatory bowel disease. The concept of self-management is complex, but can be divided into those aspects that involve the individual patient, those that involve the provider-patient relationship, and those that encompass the social environment. At the individual level, enhancing problem-solving skills and self-efficacy have both been shown to improve self-management tasks, particularly adherence to treatment. However, it is critical to consider these domains from a lifespan perspective because these processes by which self-management can be improved are distinct for children, adolescents, young adults, and adults. A particular emphasis is placed on strategies to improve self-management of older adolescents and young adults as they transition from pediatric to adult providers. The review concludes with recommendations for providers, including rationale and techniques for assessing and promoting patient self-efficacy, encouraging the development of problem-solving skills, improving the patient-provider relationship, and enhancing social support. Providers are encouraged to utilize elements of problem-solving skills training, engage in collaborative relationships with their patients, and offer their patients recommendations for how to increase the quality of their social support networks as ways of increasing overall self-management.

  9. Autoimmune thyroid disease in a cohort of Malaysian SLE patients: frequency, clinical and immunological associations.

    PubMed

    Ong, S G; Choy, C H

    2016-01-01

    Autoimmune thyroid disease (ATD) has been associated with other systemic autoimmune diseases. To date, there is limited data on thyroid disorders and autoimmune thyroid disease in Malaysia. The frequency of ATD among 189 systemic lupus erythematosus (SLE) patients was 6.3%, with 2.6% in the hyperthyroid group and 3.7% in the hypothyroid group. Hypothyroidism developed at a much younger mean age (24.3 years), suggesting that SLE might be a predisposing factor for the development of Hashimoto's thyroiditis. There was a higher rate of thyroid peroxidase antibody (TPO) positivity compared with anti-thyroglobulin antibody (Tg) in the hyperthyroid subgroup. This study also demonstrated a greater proportion of ATD patients who demonstrated high titres (≥ 1:6400) of TPO compared with high titres of Tg. Although there was an association between ATD and the presence of anti-Ro/SSA and/or anti-La/SSB antibodies, the absence of sicca symptoms and negative Schirmer's tests suggest a lack of association with secondary Sjogren's syndrome. A novel association between ATD and antiphospholipid syndrome (APS) was detected in our cohort. Hence we propose that patients affected by APS be routinely screened for ATD. © The Author(s) 2015.

  10. Clinical symptoms of FSSG in gastroesophageal reflux disease are critical for PPI treatment: Japanese multi-centers with 185 patients.

    PubMed

    Komatsu-Tanaka, Mio; Iwakiri, Ryuichi; Fujimoto, Kazuma; Fujiwara, Yasuhiro; Inamori, Masahiko; Tanaka, Junji; Shimatani, Tomohiko; Akiyama, Junichi; Ando, Takashi; Manabe, Noriaki; Kinjo, Fukunori; Deguchi, Ryuzo; Kusano, Motoyasu

    2012-11-01

    The main aim of this study was to determine whether questionnaire evaluations of clinical symptoms in gastroesophageal reflux disease were useful to assess proton pump inhibitor therapy. A total of 185 Japanese patients (men, 88; women, 97; age: 55.7 ± 16.1 years) with gastroesophageal reflux disease were enrolled. The patients were divided based on the frequency scale for symptoms of gastroesophageal reflux disease: severe symptoms with scores ≥8 and mild symptoms with scores ≤7. Quality of life was evaluated with the Medical Outcomes Study 8-Item Short-Form Health Survey. All patients were treated with a proton pump inhibitor, rabeprazole (10 mg/day), for 8 weeks. Patients were classified into four groups: reflux esophagitis with severe symptoms (n = 92, 49.7%); reflux esophagitis with mild symptoms (n = 17, 9.2%); non-erosive reflux disease with severe symptoms (n = 66, 35.7%); and non-erosive reflux disease with mild symptoms (n = 10, 5.4%). The dysmotility score was high in non-erosive reflux disease with severe symptoms compared with reflux esophagitis with severe symptoms (9.1 ± 0.5 vs 6.8 ± 0.5, P < 0.05). The symptom score and quality of life in the severe symptoms groups for both reflux esophagitis and non-erosive reflux disease were significantly improved by rabeprazole treatment. Only the reflux score was improved by rabeprazole in the reflux esophagitis with mild symptoms group; no therapeutic effect was observed for the non-erosive reflux disease with mild symptoms group. Low scores on the frequency scale for the symptoms of gastroesophageal reflux disease indicate poor responsiveness to proton pump inhibitor treatment, and high scores indicate good responsiveness. © 2012 The Authors. Digestive Endoscopy © 2012 Japan Gastroenterological Endoscopy Society.

  11. Oral and salivary changes in patients with chronic kidney disease: A clinical and biochemical study

    PubMed Central

    Anuradha, Beela Ram; Katta, Sudheer; Kode, Venkata Satyanarayana; Praveena, Channamsetty; Sathe, Naresh; Sandeep, Nalla; Penumarty, Swati

    2015-01-01

    Background: Both chronic kidney disease (CKD) and its treatment can affect a wide range of tissues and systems. It directly or indirectly affects flow, concentrations and composition of saliva. Hemodialysis can effectively minimize most of these complications to some extent. Aims: The main aim of this study was to know the salivary content of sodium, potassium, calcium, urea, bicarbonate and oral manifestations in patients with CKD. Materials and Methods: For this study, 50 patients diagnosed with CKD and 50 systemically and periodontally healthy individuals were subjected to a detailed general and intraoral examination. Whole un-stimulated saliva samples of all the selected subjects were collected and subjected to calcium (Ca), phosphorous (P), sodium (Na), potassium (K), bicarbonate and urea analysis. Statistical Analysis Used: Paired t-test, Mann–Whitney test. Results: Among 50 study subjects, 26 subjects had reduced salivary flow in the range of 0.1–0.4 ml/min. Intraoral examination of the study subjects revealed pallor, increased deposition of calculus, bleeding gums, metallic taste, hypoplasia of teeth and fissured tongue. There was a significant difference between healthy and prehemodialysis patients in the salivary sodium, potassium, calcium, phosphorus, urea levels and the difference was insignificant in relation to bicarbonate levels. Conclusions: Alterations in salivary calcium, phosphorous, urea, sodium, potassium levels were significantly higher in the study groups when compared to control groups and the difference was insignificant in relation to bicarbonate level. The increased levels in dialysis patients correlated with renal disease severity. PMID:26229271

  12. Hemodynamic stress distribution reflects ischemic clinical symptoms of patients with moyamoya disease.

    PubMed

    Takahashi, Satoshi; Tanizaki, Yoshio; Kimura, Hiroaki; Akaji, Kazunori; Nakazawa, Masaki; Yoshida, Kazunari; Mihara, Ban

    2015-11-01

    Currently, the probability of diagnosing asymptomatic moyamoya disease is increasing. In this study, we consider a less invasive method for predicting future ischemic symptoms in patients with moyamoya disease. We reviewed cerebral blood flow (CBF)-related data obtained by xenon CT imaging (XeCT) in six patients with ischemic-type or asymptomatic moyamoya disease. The data were obtained as volume data using a 320-row CT, and applied to the automated region-of-interest-determining software (3DSRT) and converted to standardized images. Eight CBF-related parameters, including CBF value, cerebrovascular reserve capacity (CVRC), and hemodynamic distribution (hdSD), were compared between asymptomatic hemispheres and ischemic symptomatic hemispheres. A significant difference was determined by a two-sample t test. A difference with p<0.05 was considered significant. When statistically significant differences between parameters of asymptomatic hemispheres and ischemic symptomatic hemispheres were identified, cut-off points were calculated with receiver operating characteristic (ROC) curves. Change in the parameters before and after bypass surgery was also assessed. Of the eight CBF-related parameters evaluated, statistically significant differences between the asymptomatic hemispheres and ischemic hemispheres were observed in the CBF value of the MCA region (CBF-MCA), both at rest and after acetazolamide loading, and in the hdSD, also both at rest and after acetazolamide loading. Of the four statistically significant parameters, ROC analysis revealed that the hdSD at rest and CBF-MCA after acetazolamide loading were the most sensitive and specific parameters (threshold 1.2, sensitivity 1, specificity 1 for hdSD at rest, and threshold 26.44mL/100g/min, sensitivity 1, specificity 1 for CBF-MCA after acetazolamide loading). From the CBF data obtained both before and after surgery from the three patients who had undergone direct bypass surgeries, the hdSD was higher than the

  13. Subacute Cutaneous Lupus Erythematosus: Clinical Characteristics, Disease Associations, Treatments, and Outcomes in a Series of 90 Patients at Mayo Clinic, 1996-2011.

    PubMed

    Alniemi, Dema T; Gutierrez, Albert; Drage, Lisa A; Wetter, David A

    2017-03-01

    To characterize the clinical presentation, laboratory studies, disease associations, and treatments of subacute cutaneous lupus erythematosus (SCLE). A retrospective review of 90 patients with SCLE at Mayo Clinic from January 1, 1996, through October 28, 2011, was performed. The mean patient age at diagnosis was 61 years; 64 patients (71%) were women, and 11 cases (12%) were drug induced (1996-2000, no drug-induced cases; 2001-2005, 2 cases; 2006-2011, 9 cases). Seventeen of 59 patients (29%) with available data were smokers at the time of diagnosis. The SCLE lesions were photodistributed in 75 patients (83%), and 52 (58%) had papulosquamous morphologic findings. Anti-Ro/SS-A positivity was present in 84 of 85 patients tested (99%), whereas 32 of the 85 patients (38%) tested positive for anti-La/SS-B. Associated autoimmune connective tissue diseases included Sjögren syndrome (n=13, 14%) and systemic lupus erythematosus (SLE) (n=8, 9%). Eighteen patients (20%) had at least 4 American College of Rheumatology criteria for SLE; 1 had lupus nephritis, and none had neurologic or notable hematologic sequelae. The most common therapy was hydroxychloroquine, with a complete response noted in 34 of 46 patients (74%) with available follow-up data. Twenty-eight percent of patients with SCLE (n=25) had an associated autoimmune connective tissue disease, although the severe sequelae of SLE, such as nephritis, were rare. The frequency of drug-induced SCLE increased during the study. Most patients responded to treatment with hydroxychloroquine. Copyright © 2016 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

  14. Intravascular disorders of microcirculation in patients with chronic obstructive pulmonary disease: the results of clinical and morphological examination

    NASA Astrophysics Data System (ADS)

    Fiodorova, Tatiana A.

    1999-05-01

    We have evaluated the results of clinical and morphological study of microcirculation and its intravascular factors in 120 patients with chronic obstructive pulmonary diseases (COPD). Conjunctival biomicroscopy with quantitative evaluation of microcirculatory changes we performed. This data were compared with the results of laboratory study of erythrocytes and thrombocytes aggregation, some plasma hemostasis indices and morphological examination of microcirculation. The results of conjunctival biomicroscopy showed the close correlation between the clinical severity of the disease, the degree of respiratory failure and the degree of microcirculatory disorders. Progress of the disease with the development of respiratory failure and cor pulmonale was characterized by the expansion of the process of erythrocytes aggregation to the whole parts of the microcirculatory bad and was associated with perivascular hemorrhages. In some patients with severe COPD laboratory data showed chronic disseminated intravascular microcoagulation (DVS-syndrome). Intravascular platelets, erythrocytes and mixed aggregates which completely cork the vessels and compressed endothelium were uncovered by electron microscopy. Platelets membrane injuring with its degranulation was seen. This discovered correlation between microcirculatory abnormalities in lungs and in conjunctiva in patients with COPD demonstrate that this abnormalities of microcirculation are prevalent. This allows to use in clinical accessible and informative method of conjunctival biomicroscopy to estimate the condition of microcirculation in this pathology.

  15. Self-management in patients with inflammatory bowel disease: strategies, outcomes, and integration into clinical care

    PubMed Central

    Plevinsky, Jill M; Greenley, Rachel N; Fishman, Laurie N

    2016-01-01

    Self-management, including medication adherence, is associated with improved health and outcomes for patients with inflammatory bowel disease. The concept of self-management is complex, but can be divided into those aspects that involve the individual patient, those that involve the provider–patient relationship, and those that encompass the social environment. At the individual level, enhancing problem-solving skills and self-efficacy have both been shown to improve self-management tasks, particularly adherence to treatment. However, it is critical to consider these domains from a lifespan perspective because these processes by which self-management can be improved are distinct for children, adolescents, young adults, and adults. A particular emphasis is placed on strategies to improve self-management of older adolescents and young adults as they transition from pediatric to adult providers. The review concludes with recommendations for providers, including rationale and techniques for assessing and promoting patient self-efficacy, encouraging the development of problem-solving skills, improving the patient–provider relationship, and enhancing social support. Providers are encouraged to utilize elements of problem-solving skills training, engage in collaborative relationships with their patients, and offer their patients recommendations for how to increase the quality of their social support networks as ways of increasing overall self-management. PMID:27601930

  16. Clinical and prognostic differences among patients with light chain deposition disease, myeloma cast nephropathy and both.

    PubMed

    Zand, Ladan; Nasr, Samih H; Gertz, Morie A; Dispenzieri, Angela; Lacy, Martha Q; Buadi, Francis K; Kumar, Shaji; Kyle, Robert A; Fervenza, Fernando C; Sethi, Sanjeev; Dingli, David; Rajkumar, S Vincent; Kapoor, Prashant; McCurdy, Arleigh; Leung, Nelson

    2015-01-01

    In some patients with light chain deposition disease (LCDD) there is also evidence of myeloma cast nephropathy (MCN) on renal biopsy. The purpose of this study was to evaluate the renal and survival outcome of patients with concomitant diagnosis of MCN and LCDD to LCDD and MCN alone. Eighty seven patients were identified and divided into LCDD (n=45), MCN (n=29), and LCDD+ MCN (n=13). Patients with LCDD+ MCN had a worse overall survival (OS) compared to patients with LCDD (p=0.03), but similar to patients with MCN (p=0.4). Death-censored renal survival was no different amongst the groups. Presenting with acute renal failure at time of renal biopsy (HR 7.2, p=0.0002) was an independent poor renal prognostic factor while older age (HR 1.06, p=0.0002), presence of osteolytic lesions (HR 4.4, p<0.0001), and requirement for dialysis or creatinine≥5 mg/dL (HR 3.2, p=0.0006) at time of renal biopsy were independent poor prognostic factors for OS.

  17. Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus.

    PubMed

    Feng, X; Zou, Y; Pan, W; Wang, X; Wu, M; Zhang, M; Tao, J; Zhang, Y; Tan, K; Li, J; Chen, Z; Ding, X; Qian, X; Da, Z; Wang, M; Sun, L

    2014-03-01

    The objective of this study is to evaluate the association of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus (SLE) in a large, multicenter Chinese cohort. Medical records of 1898 SLE inpatients from 15 hospitals were reviewed and classified into three groups according to their ages at disease presentation. Categorical data were analyzed by chi-square test and potentially associated factors were tested by multinomial logistic regression. Among the patients studied, 259 (13.6%) were juvenile onset (≤18 years), 1444 (76.1%) were early onset (>18 and ≤45 years) and 195 (10.3%) were late onset (>45 years). Whenever manifestations occurred, most patients (>80%) were diagnosed within two years. Juvenile-onset patients were more likely to be untreated before admission (p < 0.001) and have mucocutaneous manifestations (p < 0.001), but musculoskeletal symptoms (p < 0.05) and leukopenia (p < 0.05) were less frequent, while comorbidities were much higher in patients with late-onset SLE (p < 0.001). Neuropsychiatric, cardiopulmonary, renal and gastrointestinal involvement, disease activity index and damage scores were similar among three groups. Anti-Sm antibodies were less prevalent in late-onset patients (p < 0.05) and antimalarial drugs were more often applied to juvenile-onset patients (p < 0.001). As expected, mortality was elevated in the late-onset SLE group (p < 0.05), in which nearly half died of infections, which was much higher than those in the other two groups (p < 0.001). Logistic regression confirmed that patients with juvenile- and early-onset disease were associated with high incidence of being untreated prior to admission, and with low incidence of comorbidities as well as deaths caused by infection compared to patients with late-onset lupus. Interestingly, our data showed that more patients with late-onset disease had a SLEDAI score change of >7 at discharge. In

  18. Low-density lipoprotein and apolipoprotein B: clinical use in patients with coronary heart disease.

    PubMed

    Cromwell, William C; Barringer, Thomas A

    2009-11-01

    Managing low-density lipoprotein (LDL) is an integral part of clinical practice. What remains controversial is whether we are using the best measure of LDL quantity for this purpose. Historically, the cholesterol content of LDL particles (LDLC) has been used to express LDL quantity. However, because of variability in the cholesterol carried in LDL particles, frequent disagreement occurs between LDLC and particle measures of LDL quantity, including apolipoprotein B-100 (apo B) or nuclear magnetic resonance (NMR) LDL particle number (LDL-P). Studies consistently demonstrate apo B and LDL-P are superior predictors of coronary heart disease (CHD) risk and superior indicators of low CHD risk on lipid-lowering therapy. Recent recommendations advocate that, in addition to LDLC and non-high-density lipoprotein cholesterol, apo B (or NMR LDL-P) be used as a target of therapy. This article reviews the rationale supporting these recommendations and provides a model for integrating LDL particle measures in clinical practice.

  19. Microscopic Haematuria and Clinical Outcomes in Patients With Stage 3-5 Nondiabetic Chronic Kidney Disease.

    PubMed

    You-Hsien Lin, Hugo; Yen, Chun-Yu; Lim, Lee-Moay; Hwang, Daw-Yang; Tsai, Jer-Chia; Hwang, Shang-Jyh; Hung, Chi-Chih; Chen, Hung-Chun

    2015-10-16

    Microscopic haematuria is proposed as a prognostic factor for renal outcomes in patients with glomerulonephritis. However, the role of haematuria in patients with advanced chronic kidney disease (CKD) or heavy proteinuria has not been investigated. We divided 1799 patients with stage 3-5 nondiabetic CKD into 3 groups according to the results from 3 urinalyses: no haematuria (0-2 red blood cells [RBCs]/hpf ≥2 times), mild haematuria (2-5 RBCs/hpf ≥2 times) and moderate haematuria (≥5-10 RBCs/hpf ≥2 times). The estimated glomerular filtration rate was 25.4 mL/min/1.73 m(2), with a urine protein-to-creatinine ratio (UPCR) of 881 mg/g. The hazard ratios (HRs) of mild and moderate haematuria for end-stage renal disease (ESRD) were 1.28 (95% confidence interval [CI]: 1.05-1.56, P = 0.024) and 1.34 (95% CI: 1.03-1.74, P = 0.030), respectively. The HR of moderate haematuria for mortality was 1.56 (95% CI: 1.11-2.20, P = 0.011). According to subgroup analysis, the HR of moderate haematuria for ESRD in patients with a UPCR of <500 mg/g was more prominent than that in patients with a UPCR of ≥500 mg/g. Microscopic haematuria in patients with stage 3-5 nondiabetic CKD is associated with increased risks of ESRD and mortality.

  20. Healthcare decision-making in end stage renal disease-patient preferences and clinical correlates.

    PubMed

    Jayanti, Anuradha; Neuvonen, Markus; Wearden, Alison; Morris, Julie; Foden, Philip; Brenchley, Paul; Mitra, Sandip

    2015-11-14

    Medical decision-making is critical to patient survival and well-being. Patients with end stage renal disease (ESRD) are faced with incrementally complex decision-making throughout their treatment journey. The extent to which patients seek involvement in the decision-making process and factors which influence these in ESRD need to be understood. 535 ESRD patients were enrolled into the cross-sectional study arm and 30 patients who started dialysis were prospectively evaluated. Patients were enrolled into 3 groups- 'predialysis' (group A), 'in-centre' haemodialysis (HD) (group B) and self-care HD (93 % at home-group C) from across five tertiary UK renal centres. The Autonomy Preference Index (API) has been employed to study patient preferences for information-seeking (IS) and decision-making (DM). Demographic, psychosocial and neuropsychometric assessments are considered for analyses. 458 complete responses were available. API items have high internal consistency in the study population (Cronbach's alpha > 0.70). Overall and across individual study groups, the scores for information-seeking and decision-making are significantly different indicating that although patients had a strong preference to be well informed, they were more neutral in their preference to participate in DM (p < 0.05). In the age, education and study group adjusted multiple linear regression analysis, lower age, female gender, marital status; higher API IS scores and white ethnicity background were significant predictors of preference for decision-making. DM scores were subdivided into tertiles to identify variables associated with high (DM > 70: and low DM (≤30) scores. This shows association of higher DM scores with lower age, lower comorbidity index score, higher executive brain function, belonging in the self-caring cohort and being unemployed. In the prospectively studied cohort of predialysis patients, there was no change in decision-making preference scores after

  1. The Impact of Clinical Pharmacist Support on Patients Receiving Multi-drug Therapy for Coronary Heart Disease in China.

    PubMed

    Zhao, S J; Zhao, H W; Du, S; Qin, Y H

    2015-01-01

    The study determined pharmacist support on patients receiving multi-drug therapy for coronary heart disease by evaluating patient self-care ability, quality of life, and drug therapy compliance. In this study, ninety patients were randomly assigned to an experimental group (n=45) and a control group (n=45). The control group received conventional clinical care. The experimental group received clinical care plus pharmacist support that included medication review, patient education, lifestyle management, discharge guidance, and telephone follow-up. Eighty-five patients completed the study. Self-care ability and quality of life were evaluated before hospital discharge. The experimental group understood their condition better than the control group (P<0.05), the differences between the groups in understanding treatment goals, drug regimens, lifestyle modifications, psychogenic disorders, and satisfaction evaluations were more pronounced (P<0.01). At six-month follow-up, the difference between the groups in drug therapy compliance was P<0.01, as was success rate by intention-to-treat (77.8% vs. 48.9%) and per-protocol (81.4% vs. 52.4%). Two adverse drug reactions occurred in the experimental group and three in the control group. Pharmacist support improved self-care ability, quality of life, drug therapy compliance, and treatment success rate in coronary heart disease patients.

  2. [Expression of elafin in peripheral blood in inflammatory bowel disease patients and its clinical significance].

    PubMed

    Zhang, W; Teng, G G; Tian, Y; Wang, H H

    2016-04-12

    To quantify the expression of elafin mRNA in peripheral blood in patients with inflammatory bowel disease (IBD) and to explore its value in assessment of the activity and severity of IBD. From July 1 2015 to August 15 2015, 23 patients with IBD admitted to Peking University First Hospital were selected, including 15 cases with ulcerative colitis (UC) and 8 cases with Crohn's disease (CD). Among those, 5 cases were in remission (UC 3, CD 2), 6 cases were mild active (UC 3, CD 3), 3 cases were moderate active (UC 1, CD 2), and 9 cases were severe active (UC 8, CD 1). A total of 21 healthy individuals were selected as the control group. Peripheral blood samples of IBD patients and healthy controls were collected. The expression of elafin mRNA in peripheral blood leukocytes was detected by fluorescence quantitative real-time PCR. Mann-Whitney test was performed for comparison between the two groups. The correlation between the expression of elafin mRNA in peripheral blood and IBD activity score was analyzed by Pearson correlation analysis after transformation of variables. The median expression of elafin mRNA in peripheral blood leukocytes in IBD group and control group was 0.005 8 (0.000 2, 0.043 5) and 0.015 3 (0.002 1, 0.175 8), respectively, with no significant difference (P>0.05). However, in the active IBD patients it was lower than that in the controls (0.004 6 (0.000 2, 0.034 8) vs 0.015 3 (0.002 1, 0.175 8), P<0.05) and also lower than that in the remission patients(0.004 6 (0.000 2, 0.034 8) vs 0.023 1 (0.012 6, 0.043 5), P<0.05); in the active UC patients it was lower than that in the controls(0.003 7 (0.000 2, 0.027 0) vs 0.015 3 (0.002 1, 0.175 8), P<0.05). The expression of elafin mRNA in peripheral blood was negatively correlated with modified Mayo score in UC patients (r=-0.513, P<0.05) and with the Crohn's Disease Activity Index (CDAI) of Best score in CD patients (r=-0.889, P<0.05). The expression of elafin mRNA in peripheral blood in active IBD

  3. Serum Calprotectin Discriminates Subclinical Disease Activity from Ultrasound-Defined Remission in Patients with Rheumatoid Arthritis in Clinical Remission

    PubMed Central

    Hulejova, Hana; Zavada, Jakub; Komarc, Martin; Hanova, Petra; Klein, Martin; Mann, Herman; Sleglova, Olga; Olejarova, Marta; Forejtova, Sarka; Ruzickova, Olga; Vencovsky, Jiri; Pavelka, Karel; Senolt, Ladislav

    2016-01-01

    calprotectin to distinguish RA patients in both clinical and ultrasound-defined remission from patients in clinical remission but with residual subclinical disease activity. PMID:27832086

  4. Vascular calcification in patients with chronic kidney disease: types, clinical impact and pathogenesis.

    PubMed

    Román-García, Pablo; Rodríguez-García, Minerva; Cabezas-Rodríguez, Iván; López-Ongil, Susana; Díaz-López, Bernardino; Cannata-Andía, Jorge B

    2011-01-01

    Vascular calcification plays a major role in cardiovascular disease, which is one of the main causes of mortality in chronic kidney disease patients. Vascular calcification is determined by prevalent traditional and uraemia-related (non-traditional) risk factors. It occurs mainly in the arteries, which are classified into three types according to their size and structural characteristics. In addition, vascular calcification has been associated with bone loss and fractures in chronic kidney disease patients and the general population, stressing the fact that both disorders can share pathogenetic pathways. The strategies to control vascular calcification involve several measures, chief among them the control of hyperphosphataemia. Furthermore, it has been recently described that strategies that reduce bone resorption and increase bone mineralization may decrease the risk of vascular calcifications; however, this approach still remains controversial. The mechanisms involved in vascular calcification are complex and not yet fully understood. Phosphorus plays a major role, while other factors related to bone formation have been recently identified. Copyright © 2011 S. Karger AG, Basel.

  5. Incidence of dizziness and vertigo in Japanese primary care clinic patients with lifestyle-related diseases: an observational study

    PubMed Central

    Wada, Masaoki; Takeshima, Taro; Nakamura, Yosikazu; Nagasaka, Shoichiro; Kamesaki, Toyomi; Oki, Hiroshi; Kajii, Eiji

    2015-01-01

    Objective Dizziness and vertigo are highly prevalent symptoms among patients presenting at primary care clinics, and peripheral vestibular disorder (PVD) is their most frequent cause. However, the incidence of PVD has not been well documented. This study aimed to investigate the incidence of dizziness, vertigo, and PVD among patients presenting at a primary care clinic. Design This was an observational study. Setting and participants Between November 2011 and March 2013, we observed 393 patients, all at least 20 years old, who had been treated for chronic diseases such as hypertension, dyslipidemia, and diabetes mellitus for at least 6 months at a primary clinic (Oki Clinic) in Japan. Outcome The main outcome of interest was new incidence of dizziness, vertigo, and PVD events. During the 1-year follow-up period, the otorhinolaryngologist diagnosed and reported new PVD events. Results The mean age of the 393 participants at entry was 65.5 years. Of the study participants, 12.7%, 82.4%, and 92.6% had diabetes mellitus, hypertension, and dyslipidemia, respectively. We followed up all the participants (100%). During the 662.5 person-years of follow-up, 121 cases of dizziness or vertigo (dizziness/vertigo) and 76 cases of PVD were observed. The incidence of dizziness/vertigo and PVD was 194.7 (95% confidence interval: 161.6–232.6) per 1,000 person-years and 115.7 (95% confidence interval: 92.2–142.6) per 1,000 person-years, respectively. There were 61 cases of acute peripheral vestibulopathy, 12 of benign paroxysmal positional vertigo, and three of Meniere’s disease among the 76 PVD patients. Conclusion We reported the incidence of dizziness/vertigo among Japanese primary care clinic patients, which was higher than that usually observed in the general population. Furthermore, we described the incidence of PVD and found that it was a major cause of dizziness/vertigo. PMID:25931828

  6. Clinical features and outcome of patients with inflammatory bowel disease who use narcotics: a case-control study.

    PubMed

    Hanson, Karen A; Loftus, Edward V; Harmsen, W Scott; Diehl, Nancy N; Zinsmeister, Alan R; Sandborn, William J

    2009-05-01

    The role of narcotic use in patients with inflammatory bowel disease (IBD) is poorly defined. We sought to determine the clinical features of patients with IBD who use narcotics and factors associated with the discontinuation of narcotics. A centralized index was used to identify 100 cases (patients with IBD receiving narcotics) and 100 matched controls evaluated in an IBD clinic between 1999 and 2002. Associations of clinical factors with case or control status were summarized as overall percentages and assessed using conditional logistic regression. Associations within cases were assessed using Fisher's exact test. Review of 361 charts identified 100 patients with IBD receiving narcotics (cases). One hundred matched controls were then identified. Cases were significantly associated with: female gender (64% cases versus 45% controls, P = 0.01), >or=2 IBD-related surgeries (42% versus 17%, P < 0.001), moderate-to-severe pain (93% versus 20%, P < 0.001), and moderate-to-severe symptoms (83% versus 33%, P < 0.001). Significant associations were detected for depression (42% versus 19%, P < 0.001), anxiety (19% versus 7%, P = 0.02), history of abuse (sexual, emotional, or physical, 17% versus 3%, P = 0.006), and for substance abuse (excluding alcohol) (14% versus 1%, P = 0.01). Of 39 cases that returned for follow-up, 22 (56%) had discontinued narcotics. Discontinuing narcotics was associated with medical treatment adherence (100% versus 53%), none-to-mild pain (73% versus 18%), and none-to-mild clinical activity (80% versus 24%). Narcotic use in IBD patients is associated with female gender, >or=2 previous surgeries, moderate to severe pain, and clinical disease activity, depression, anxiety, sexual, emotional and physical abuse, and substance abuse.

  7. Severe Legionnaires' Disease Complicated by Rhabdomyolysis and Clinically Resistant to Moxifloxacin in a Splenectomised Patient: Too Much of a Coincidence?

    PubMed Central

    Koufakis, Theocharis; Gabranis, Ioannis; Chatzopoulou, Marianneta; Margaritis, Anastasios; Tsiakalou, Maria

    2015-01-01

    We here report a case of Legionnaires' disease in a splenectomised patient, complicated by rhabdomyolysis and acute renal failure and characterized by a poor clinical response to moxifloxacin. Splenectomy is not included among the factors, typically associated with higher risk or mortality in patients with Legionellosis. However, our report is consistent with previous case reports describing severe Legionella infections in asplenic subjects. The possibility that functional or anatomic asplenia may be a factor predisposing to severe clinical course or poor response to therapy in patients with Legionella infection cannot be excluded, deserving further investigation in the future. More studies are required in order to clarify the underlying pathophysiological mechanisms that connect asplenia, immunological response to Legionella, and pathogen's resistance to antibiotics. PMID:26682076

  8. Clinical and molecular aspects of 30 patients with late-onset Pompe disease (LOPD): unusual features and response to treatment.

    PubMed

    Montagnese, Federica; Barca, E; Musumeci, O; Mondello, S; Migliorato, A; Ciranni, A; Rodolico, C; De Filippi, P; Danesino, C; Toscano, A

    2015-01-01

    Pompe disease is a rare metabolic disorder, due to mutations in the gene encoding acid alpha-glucosidase (GAA), of which infantile and late-onset forms may occur. Aim of the work was to analyze clinical and laboratory data of a cohort of late-onset Pompe disease (LOPD) patients, collected during the last 15 years and to point out unusual phenotypic/genotypic features as well as enzyme replacement therapy (ERT) responses. We diagnosed 30 LOPD patients; at follow-up, they underwent motor, respiratory, cardiac and muscle MRI evaluations. Motor performances were tested by Walton Gardner-Medwin, GSGC and 6MWT tests. Respiratory function was assessed as FVC% in upright/supine position. LOPD presentations were represented by presymptomatic hyperCKemia (37%), proximal/axial muscle weakness (53%) and respiratory impairment (10%). Median diagnostic delay was 8.6 years (± 8.8). Atypical features were observed in 4 patients: marked distal muscle weakness and severe hearing loss at onset, as well as leukoencephalopathy and mesial temporal sclerosis during the disease course. By GAA sequence analysis, two causing mutations were detected in 22/30 patients, only one in the remaining 8 subjects. Overall, 29/30 patients harbored the common c.-32-13T>G mutation (2 were homozygous). Two new DNA variations were discovered (c.2395C>G, c.1771C>T). 14 patients received ERT for up to 60 months. Our study confirms LOPD clinical and genetic heterogeneity: atypical features may contribute to expand the clinical phenotype highlighting its multi-systemic nature. A timely diagnosis could allow early ERT start. An accurate follow-up is recommended to evaluate treatment responses.

  9. Prevalence and Clinical Characteristics of Mental Stress–Induced Myocardial Ischemia in Patients With Coronary Heart Disease

    PubMed Central

    Jiang, Wei; Samad, Zainab; Boyle, Stephen; Becker, Richard C.; Williams, Redford; Kuhn, Cynthia; Ortel, Thomas L.; Rogers, Joseph; Kuchibhatla, Maragatha; O’Connor, Christopher; Velazquez, Eric J.

    2014-01-01

    Objectives The goal of this study was to evaluate the prevalence and clinical characteristics of mental stress–induced myocardial ischemia. Background Mental stress–induced myocardial ischemia is prevalent and a risk factor for poor prognosis in patients with coronary heart disease, but past studies mainly studied patients with exercise-induced myocardial ischemia. Methods Eligible patients with clinically stable coronary heart disease, regardless of exercise stress testing status, underwent a battery of 3 mental stress tests followed by a treadmill test. Stress-induced ischemia, assessed by echocardiography and electrocardiography, was defined as: 1) development or worsening of regional wall motion abnormality; 2) left ventricular ejection fraction reduction ≥8%; and/or 3) horizontal or downsloping ST-segment depression ≥1 mm in 2 or more leads lasting for ≥3 consecutive beats during at least 1 mental test or during the exercise test. Results Mental stress–induced ischemia occurred in 43.45%, whereas exercise-induced ischemia occurred in 33.79% (p = 0.002) of the study population (N = 310). Women (odds ratio [OR]: 1.88), patients who were not married (OR: 1.99), and patients who lived alone (OR: 2.24) were more likely to have mental stress–induced ischemia (all p < 0.05). Multivariate analysis showed that compared with married men or men living with someone, unmarried men (OR: 2.57) and married women (OR: 3.18), or living alone (male OR: 2.25 and female OR: 2.72, respectively) had higher risk for mental stress-induced ischemia (all p < 0.05). Conclusions Mental stress-induced ischemia is more common than exercise-induced ischemia in patients with clinically stable coronary heart disease. Women, unmarried men, and individuals living alone are at higher risk for mental stress-induced ischemia. (Responses of Myocardial Ischemia to Escitalopram Treatment [REMIT]; NCT00574847) PMID:23410543

  10. Clinical questionnaire study of oral health care and symptoms in diabetic vs. non-diabetic predialysis chronic kidney disease patients.

    PubMed

    Vesterinen, Maarit; Ruokonen, Hellevi; Furuholm, Jussi; Honkanen, Eero; Meurman, Jukka H

    2012-04-01

    This paper aims to study oral symptoms (burning mouth sensation, xerostomia, dysphagia, and dysgeusia) and background characteristics among chronic kidney disease (CKD) patients. The hypothesis was that patients experience oral discomfort and show interest towards dental care differently depending on the origin of their kidney disease. One hundred thirty-eight CKD patients at predialysis stage (94 men, 44 women, mean age 54 years) at the Helsinki University Central Hospital participated in the study. The patients were divided into a diabetic nephropathy group and a group of patients with other kidney diseases. The patients had a clinical oral examination and filled in a structured questionnaire. The data were analyzed and compared between the groups (SPSS for Windows version 15.0). T test was used for parameters normally distributed while binomial data were analyzed with cross-tabulations and chi-square test. Contrary to our study hypothesis, no statistically significant differences were seen in the questionnaire study between the diabetic vs. non-diabetic CKD patients in any other study parameter except in the use of medication (10 ± 2.3 vs. 8 ± 3.1 drugs daily, p < 0.05), and working status (23.5% vs. 50% working full time, p < 0.01). No difference was seen in the frequency of oral discomfort among the different groups. Xerostomia, however, was frequently observed among the predialysis patients investigated (41.7% in diabetic, 48.2% in non-diabetic patients). No difference was seen in the frequency of oral discomfort among the different groups of predialysis patients investigated. Clinicians should be aware of nephropathy patients who frequently suffer from oral discomfort, particularly xerostomia.

  11. LRRK2 in Parkinson's disease: genetic and clinical studies from patients.

    PubMed

    Kumari, Udhaya; Tan, E K

    2009-11-01

    Mutations in leucine-rich repeat kinase 2 (LRRK2) (PARK8) are associated with both familial and sporadic forms of Parkinson's disease. Most studies have shown that LRRK2 mutations may explain between 5% and 13% of familial and 1-5% of sporadic Parkinson's disease. Importantly, a common recurrent mutation (G2019S) located in the kinase domain has been reported across most ethnic populations, with the highest prevalence among Ashkenazi Jews and North African Arabs. A recent worldwide meta-analysis pooling data from 24 populations reported a higher occurrence of G2019S in southern than in northern European countries and the penetrance is estimated to be approximately 75% at the age of 79 years. The R1441 'hotspot' amino acid codon residue (G/H/C) in the Ras of complex proteins domain is the second most common site of pathogenic LRRK2 substitutions after G2019S, with most carriers developing symptoms by the age of 75 years. Two polymorphic variants found almost exclusively among Asians (G2385R and R1628P) have been shown to increase the Parkinson's disease risk by approximately two-fold. The mutational event associated with R1628P is more recent, occurring approximately 2500 years ago, compared to estimates of 4000 years for G2385R carriers. LRRK2 mutation carriers generally simulate late onset Parkinson's disease and present with the usual typical clinical features. Genetic testing for G2019S in sporadic late-onset Parkinson's disease can be considered in some situations and may be useful in populations with high carrier status. The identification of asymptomatic mutation and risk variant carriers provides a unique opportunity for recruiting these subjects in potential neuroprotective trials and longitudinal studies to identify biomarkers of neurodegeneration.

  12. Alzheimer's disease biomarker discovery in symptomatic and asymptomatic patients: experimental approaches and future clinical applications.

    PubMed

    Ho, Lap; Fivecoat, Hayley; Wang, Jun; Pasinetti, Giulio Maria

    2010-01-01

    Alzheimer's disease (AD) is the most common form of dementia in the elderly. Current treatments for AD are not as effective as needed, nor is there any definitive antemortem diagnostic. Understanding the biological processes that occur during AD onset and/or progression will improve disease diagnosis and treatment. Recent applications of microarray technologies for analysis of messenger (m) RNA expression profiles have elucidated distinct changes in the brain as a function of AD dementia initiation and progression. However, mRNA analysis underestimates post-transcriptional modifications and therefore provides only a partial view of the molecular changes in the AD brain. Combining mRNA studies with protein expression analysis may provide a more global picture of the biological processes associated with AD dementia. Information gathered could lead to the development of select biological indices (biomarkers) for guiding AD diagnosis and therapy. We will provide a brief background on AD, followed by a review on the applications of microarray, proteomics, as well as microRNA expression profile analysis to develop novel diagnostic strategies that may be useful for the diagnosis AD and for monitoring disease progression. The availability of biomarkers that promote early disease diagnosis, particularly among asymptomatic patients, will lead to the application of personalized medicine in AD.

  13. Sturge-Weber syndrome: clinical spectrum, disease course, and outcome of 30 patients.

    PubMed

    Jagtap, Sujit; Srinivas, G; Harsha, K J; Radhakrishnan, Neelima; Radhakrishnan, Ashalatha

    2013-06-01

    Sturge-Weber syndrome is a heterogeneous neurocutaneous syndrome with facial and leptomeningeal angiomas, glaucoma, seizures, stroke-like episodes, and mental retardation. The authors critically evaluated the clinical manifestations, outcome, and natural history in 30 patients with Sturge-Weber syndrome followed up from January 1985 to May 2010. Of the patients, 15 were males, age at diagnosis ranged from 1 month to 43 years. Typical port-wine stain nevus occurred in 26 (86%), it was bilateral in 2 (8%), and it was absent in 4 (4%). Nine patients had glaucoma (30%), 3 required surgery. Four had transient hemiparesis. All patients had seizures; they were well controlled in 22 (73.3%); in 8 they remained drug resistant. Three patients underwent surgery and became seizure-free. Of the 17 who had mental subnormality, 14 (82.4%) had seizure onset before 2 years. An early age at seizure onset and those with drug-resistant seizures had more severe degree of mental subnormality. Uncontrolled seizures, mental subnormality, visual handicap, and cosmetic disfiguration were the major impediments in life.

  14. Clinical implications of eicosapentaenoic acid/arachidonic acid ratio (EPA/AA) in adult patients with congenital heart disease.

    PubMed

    Kanoh, Miki; Inai, Kei; Shinohara, Tokuko; Tomimatsu, Hirofumi; Nakanishi, Toshio

    2017-07-05

    Recent studies showed that a low ratio between the levels of eicosapentaenoic acid and those of arachidonic acid (EPA/AA) is associated with higher incidence of coronary artery disease and poor prognosis of heart failure, arrhythmia, and cardiac sudden death. However, the clinical implications of EPA/AA in adult patients with congenital heart disease remain unclear. We aimed to assess the prognostic value of EPA/AA regarding cardiac events in adult patients with congenital heart disease. We measured the serum levels of eicosapentaenoic acid and arachidonic acid in 130 adult patients (median age, 31 years) stratified into two groups according to their EPA/AA (low, ≤0.22; high, >0.22). We prospectively analyzed the association between EPA/AA and incidence of cardiac events during a mean observation period of 15 months, expressed in terms of hazard ratio (HR) with 95% confidence interval (95% CI). In the subgroup of patients with biventricular circulation (2VC) (n = 76), we analyzed the same clinical endpoints. In our study population, EPA/AA was not associated with the incidence of arrhythmic events (HR, 1.52; 95% CI, 0.82-2.85; p = 0.19), but low EPA/AA was a predictor of heart failure hospitalization (HR, 2.83; 95% CI, 1.35-6.30; p < 0.01). Among patients with 2VC, an EPA/AA of ≤0.25 was associated with a significantly higher risk of arrhythmic events (HR, 2.55; 95% CI, 1.11-6.41; p = 0.03) and heart failure hospitalization (HR, 5.20; 95% CI, 1.78-18.1; p < 0.01). EPA/AA represents a useful predictor of cardiac events in adult patients with congenital heart disease.

  15. Japanese Dent disease has a wider clinical spectrum than Dent disease in Europe/USA: genetic and clinical studies of 86 unrelated patients with low-molecular-weight proteinuria.

    PubMed

    Sekine, Takashi; Komoda, Fusako; Miura, Kenichiro; Takita, Junko; Shimadzu, Mitsunobu; Matsuyama, Takeshi; Ashida, Akira; Igarashi, Takashi

    2014-02-01

    Dent disease is an X-linked disorder characterized by low-molecular-weight (LMW) proteinuria, hypercalciuria, nephrocalcinosis, urolithiasis and renal dysfunction. Dent disease is caused by mutations in at least two genes, i.e. CLCN5 and OCRL1, and its genetic background and phenotypes are common among European countries and the USA. However, only few studies on Dent disease in Japan, which was originally called 'low-molecular-weight proteinuric disease', have been reported thus far. In this study, we analysed genetic background and clinical phenotype and laboratory data of 86 unrelated Japanese Dent disease patients. The results demonstrated that the genetic basis of Japanese Dent disease was nearly identical to those of Dent disease in other countries. Of 86 unrelated Japanese Dent patients, 61 possessed mutations in CLCN5 (Dent-1), of which 27 were novel mutations; 11 showed mutations in OCRL1 (Dent-2), six of which were novel, and the remaining 14 patients showed no mutations in CLCN5 or OCRL1 (Dent-NI). Despite the similarity in genetic background, hypercalciuria was detected in only 51%, rickets in 2% and nephrocalcinosis in 35%. Although the patients were relatively young, six patients (8%) showed apparent renal dysfunction. Japanese Dent disease has a wider clinical spectrum than Dent disease in Europe and the USA.

  16. Are evoked potentials in patients with adult-onset pompe disease indicative of clinically relevant central nervous system involvement?

    PubMed

    Wirsching, Andreas; Müller-Felber, Wolfgang; Schoser, Benedikt

    2014-08-01

    Pompe disease is a multisystem autosomal recessive glycogen storage disease. Autoptic findings in patients with classic infantile and late-onset Pompe disease have proven that accumulation of glycogen can also be found in the peripheral and central nervous system. To assess the functional role of these pathologic findings, multimodal sensory evoked potentials were analyzed. Serial recordings for brainstem auditory, visual, and somatosensory evoked potentials of 11 late-onset Pompe patients were reviewed. Data at the onset of the enzyme replacement therapy with alglucosidase alfa were compared with follow-up recordings at 12 and 24 months. Brainstem auditory evoked potentials showed a delayed peak I in 1/10 patients and an increased I-III and I-V interpeak latency in 1/10 patients, respectively. The III-V interpeak latencies were in the normal range. Visual evoked potentials were completely normal. Median somatosensory evoked potentials showed an extended interpeak latency in 3/9 patients. Wilcoxon tests comparing age-matched subgroups found significant differences in brainstem auditory evoked potentials and visual evoked potentials. We found that the majority of recordings for evoked potentials were within the ranges for standard values, therefore reflecting the lack of clinically relevant central nervous system involvement. Regular surveillance by means of evoked potentials does not seem to be appropriate in late-onset Pompe patients.

  17. Quality of life in patients with chronic venous disease: influence of the socio-demographical and clinical factors.

    PubMed

    Lozano Sánchez, F S; Sánchez Nevarez, I; González-Porras, J R; Marinello Roura, J; Escudero Rodríguez, J R; Díaz Sánchez, S; Carrasco Carrasco, E

    2013-08-01

    Chronic venous disease (CVD) is a well-defined and known disorder which impact on related-health quality of life (QoL). However, individuals factors which determinate quality of life on CVD are not well defined. The purposes of this study were to describe the QoL in patients with CVD and examine socio-demographical and clinical factors which influence QoL METHODS: One thousand five hundred sixty patients with CVD were evaluated. We calculated for each patient two disease-specific severity scores: The "C" grade (clinical) of the CEAP classification and the Venous Clinical Severity Score (VCSS). Additionally, two QoL questionnaires were recorded: Short-Form Health Survey (SF-12) and Chronic Lower Limb Venous Insufficiency Questionnaire (CIVIQ-20). Two multivariate logistic regression analyses (SF-12 and CIVIQ-20 model) were conducted to determine whether demographic and clinical variables were independently associated with QoL. Both QoL questionnaires indicated that the QoL decreased significantly (P<0.05) as the C grade of CEAP increased. Each increase of 0.10 in VCSS score represented 2% worsening in QoL as measured by CIVIQ-20. Three factors were strongly associated with poor QoL on SF-12: increasing age, prior superficial venous thrombosis (SVT) or prior deep venous thrombosis. In the specific disease CIVIQ-20 questionnaire three factors (higher age, prior SVT and higher weight) were strongly associated with decrease QoL. Increasing disease severity by VCSS is associated with reductions in QoL. Subgroup analysis indicates that there are several significant individual determinants of worsening QoL.

  18. Clinical and histological characterization of oral pemphigus lesions in patients with skin diseases: a cross sectional study from Sudan

    PubMed Central

    2013-01-01

    Background Pemphigus is a rare group of life-threatening mucocutaneous autoimmune blistering diseases. Frequently, oral lesions precede the cutaneous ones. This study aimed to describe clinical and histological features of oral pemphigus lesions in patients with skin disease has been canceled aged 18 years and above, attending outpatient’s facility of Khartoum Teaching Hospital - Dermatology Clinic, Sudan. In addition, the study aimed to assess the diagnostic significance of routine histolopathology along with immunohistochemical (IHC) examination of formalin-fixed, paraffin-embedded biopsy specimens in patients with oral pemphigus. Methods A cross-sectional hospital-based study was conducted from October 2008 to January 2009. A total of 588 patients with confirmed skin has been canceled disease diagnosis completed an oral examination and a personal interview. Clinical evaluations supported with histopathology were the methods of diagnosis. IHC was used to confirm the diagnosis. Location, size, and pain of oral lesions were used to measure the oral disease activity. Results Twenty-one patients were diagnosed with pemphigus vulgaris (PV), 19 of them (mean age: 43.0; range: 20–72 yrs) presented with oral manifestations. Pemphigus foliaceus was diagnosed in one patient. In PV, female: male ratio was 1.1:1.0. Buccal mucosa was the most commonly affected site. Exclusive oral lesions were detected in 14.2% (3/21). In patients who experienced both skin and oral lesion during their life time, 50.0% (9/18) had oral mucosa as the initial site of involvement, 33.3% (6/18) had skin as the primary site, and simultaneous involvement of both skin and oral mucosa was reported by 5.5% (1/18). Two patients did not provide information regarding the initial site of involvement. Oral lesion activity score was higher in those who reported to live outside Khartoum state, were outdoor workers, had lower education and belonged to Central and Western tribes compared with their

  19. Clinical characteristics of rheumatoid arthritis patients undergoing cervical spine surgery: an analysis of National Database of Rheumatic Diseases in Japan.

    PubMed

    Sugita, Shurei; Chikuda, Hirotaka; Kadono, Yuho; Ohtsu, Hiroshi; Takeshita, Katsushi; Nishino, Jinju; Tohma, Shigeto; Tanaka, Sakae

    2014-06-13

    The aim of this study was to examine the clinical characteristics of rheumatoid arthritis (RA) patients who underwent cervical spine surgery using a multicenter observational database. We obtained data from a nationwide observational cohort database of patients with rheumatic diseases (National Database of Rheumatic Diseases by iR-net in Japan (NinJa)) for the fiscal years 2003 to 2011. A total of 39 out of 60 patients who underwent cervical spine surgery for a RA-related cause and whose data were available for two consecutive years (to assess the preoperative patient status) were chosen as cases. Patients with a non-RA-related cause of surgery (e.g., trauma) were excluded. First, we compared the patient characteristics between the cases and total patients in the same fiscal year. Next, 106 eligible controls, who were defined as RA patients enrolled in the same fiscal year as the case subjects, who were matched for age, gender and disease duration (within ±1 year), were selected. We compared the demographic data between the two groups. We also calculated the percentage of patients who underwent cervical spine surgery (surgeries/total number of patients) in fiscal years 2003 to 2011. Although the proportion of patients using biologics linearly increased during study period, the percentage of patients undergoing cervical spine surgeries remained unchanged, at approximately 0.15%. These cases had more tender joints (3 vs. 1, p < 0.01) and exhibited a significantly higher Modified Health Assessment Questionnaire (MHAQ) score (1.13 vs. 0.5, p < 0.01), C-reactive protein (CRP) (1.5 vs. 0.36, p < 0.01), and disease activity score (DAS) 28-CRP (3.63 vs. 2.81, p < 0.01) compared to the controls. Our study revealed that RA patients requiring cervical spine surgery have a higher disease activity (as represented by the DAS28-CRP) and are more functionally disabled (as represented by the MHAQ) than control patients.

  20. Clinical characteristics of rheumatoid arthritis patients undergoing cervical spine surgery: an analysis of National Database of Rheumatic Diseases in Japan

    PubMed Central

    2014-01-01

    Background The aim of this study was to examine the clinical characteristics of rheumatoid arthritis (RA) patients who underwent cervical spine surgery using a multicenter observational database. Methods We obtained data from a nationwide observational cohort database of patients with rheumatic diseases (National Database of Rheumatic Diseases by iR-net in Japan (NinJa)) for the fiscal years 2003 to 2011. A total of 39 out of 60 patients who underwent cervical spine surgery for a RA-related cause and whose data were available for two consecutive years (to assess the preoperative patient status) were chosen as cases. Patients with a non-RA-related cause of surgery (e.g., trauma) were excluded. First, we compared the patient characteristics between the cases and total patients in the same fiscal year. Next, 106 eligible controls, who were defined as RA patients enrolled in the same fiscal year as the case subjects, who were matched for age, gender and disease duration (within ±1 year), were selected. We compared the demographic data between the two groups. We also calculated the percentage of patients who underwent cervical spine surgery (surgeries/total number of patients) in fiscal years 2003 to 2011. Results Although the proportion of patients using biologics linearly increased during study period, the percentage of patients undergoing cervical spine surgeries remained unchanged, at approximately 0.15%. These cases had more tender joints (3 vs. 1, p < 0.01) and exhibited a significantly higher Modified Health Assessment Questionnaire (MHAQ) score (1.13 vs. 0.5, p < 0.01), C-reactive protein (CRP) (1.5 vs. 0.36, p < 0.01), and disease activity score (DAS) 28-CRP (3.63 vs. 2.81, p < 0.01) compared to the controls. Conclusions Our study revealed that RA patients requiring cervical spine surgery have a higher disease activity (as represented by the DAS28-CRP) and are more functionally disabled (as represented by the MHAQ) than control patients. PMID

  1. Prevalence and clinical significance of nonorgan specific antibodies in patients with autoimmune thyroiditis as predictor markers for rheumatic diseases

    PubMed Central

    Elnady, Basant M.; Kamal, Naglaa M.; Shaker, Raneyah H.M.; Soliman, Amal F.; Hasan, Waleed A.; Alghamdi, Hamed A.; Algethami, Mohammed M.; Jajah, Mohamed Bilal

    2016-01-01

    Abstract Autoimmune diseases are considered the 3rd leading cause of morbidity and mortality in the industrialized countries. Autoimmune thyroid diseases (ATDs) are associated with high prevalence of nonorgan-specific autoantibodies, such as antinuclear antibodies (ANA), antidouble-stranded deoxyribonucleic acid (anti-dsDNA), antiextractable-nuclear antigens (anti-ENAs), rheumatoid factor (RF), and anticyclic-citrullinated peptides (anti-CCP) whose clinical significance is unknown. We aimed to assess the prevalence of various nonorgan-specific autoantibodies in patients with ATD, and to investigate the possible association between these autoantibodies and occurrence of rheumatic diseases and, if these autoantibodies could be considered as predictor markers for autoimmune rheumatic diseases in the future. This study had 2 phases: phase 1; in which 61 ATD patients free from rheumatic manifestations were assessed for the presence of these nonorgan-specific autoantibodies against healthy 61 control group, followed by 2nd phase longitudinal clinical follow-up in which cases are monitored systematically to establish occurrence and progression of any rheumatic disease in association to these autoantibodies with its influences and prognosis. Regarding ATD patients, ANA, anti-dsDNA, Anti-ENA, and RF were present in a percentage of (50.8%), (18%), (21.3%), and (34.4%), respectively, with statistically significance difference (P < 0.5) rather than controls. Nearly one third of the studied group (32.8%) developed the rheumatic diseases, over 2 years follow-up. It was obvious that those with positive anti-dsDNA had higher risk (2.45 times) to develop rheumatic diseases than those without. There was a statistically significant positive linear relationship between occurrence of disease in months and (age, anti-dsDNA, anti-CCP, RF, and duration of thyroiditis). Anti-dsDNA and RF are the most significant predictors (P < 0.0001). ATD is more associated with rheumatic

  2. Depression as a Clinical Determinant of Dependence and Low Quality of Life in Elderly Patients with Cardiovascular Disease

    PubMed Central

    Rodrigues, Giselle Helena de Paula; Gebara, Otavio Celso Eluf; Gerbi, Catia Cilene da Silva; Pierri, Humberto; Wajngarten, Mauricio

    2015-01-01

    Background The aging process promotes a progressive increase in chronic-degenerative diseases. The effect of these diseases on the functional capacity has been well recognized. Another health parameter concerns “quality of life related to health”. Among the elderly population, cardiovascular diseases stand out due to the epidemiological and clinical impact. Usually, these diseases have been associated with others. This set of problems may compromise both independence and quality of life in elderly patients who seek cardiologic treatment. These health parameters have not been well contemplated by cardiologists. Objective Evaluating, among the elderly population with cardiovascular disease, which are the most relevant clinical determinants regarding dependence and quality of life. Methods This group was randomly and consecutively selected and four questionnaires were applied: HAQ, SF-36, PRIME-MD e Mini Mental State. Results The study included 1,020 elderly patients, 63.3% women. The group had been between 60 and 97 years-old (mean: 75.56 ± 6.62 years-old). 61.4% were independent or mild dependence. The quality of life total score was high (HAQ: 88.66 ± 2.68). 87.8% of patients had a SF-36 total score > 66. In the multivariate analysis, the association between diagnoses and high degrees of dependence was significant only for previous stroke (p = 0.014), obesity (p < 0.001), lack of physical activity (p = 0.016), osteoarthritis (p < 0.001), cognitive impairment (p < 0.001), and major depression (p < 0.001). Analyzing the quality of life, major depression and physical illness for depression was significantly associated with all domains of the SF-36. Conclusion Among an elderly outpatient cardiology population, dependence and quality of life clinical determinants are not cardiovascular comorbidities, especially the depression. PMID:26131699

  3. Depression as a Clinical Determinant of Dependence and Low Quality of Life in Elderly Patients with Cardiovascular Disease.

    PubMed

    Rodrigues, Giselle Helena de Paula; Gebara, Otavio Celso Eluf; Gerbi, Catia Cilene da Silva; Pierri, Humberto; Wajngarten, Mauricio

    2015-06-01

    The aging process promotes a progressive increase in chronic-degenerative diseases. The effect of these diseases on the functional capacity has been well recognized. Another health parameter concerns "quality of life related to health". Among the elderly population, cardiovascular diseases stand out due to the epidemiological and clinical impact. Usually, these diseases have been associated with others. This set of problems may compromise both independence and quality of life in elderly patients who seek cardiologic treatment. These health parameters have not been well contemplated by cardiologists. Evaluating, among the elderly population with cardiovascular disease, which are the most relevant clinical determinants regarding dependence and quality of life. This group was randomly and consecutively selected and four questionnaires were applied: HAQ, SF-36, PRIME-MD e Mini Mental State. The study included 1,020 elderly patients, 63.3% women. The group had been between 60 and 97 years-old (mean: 75.56 ± 6.62 years-old). 61.4% were independent or mild dependence. The quality of life total score was high (HAQ: 88.66 ± 2.68). 87.8% of patients had a SF-36 total score > 66. In the multivariate analysis, the association between diagnoses and high degrees of dependence was significant only for previous stroke (p = 0.014), obesity (p < 0.001), lack of physical activity (p = 0.016), osteoarthritis (p < 0.001), cognitive impairment (p < 0.001), and major depression (p < 0.001). Analyzing the quality of life, major depression and physical illness for depression was significantly associated with all domains of the SF-36. Among an elderly outpatient cardiology population, dependence and quality of life clinical determinants are not cardiovascular comorbidities, especially the depression.

  4. Clinical and neuroimaging differences between posterior cortical atrophy and typical amnestic Alzheimer’s disease patients at an early disease stage

    PubMed Central

    Peng, Guoping; Wang, Jianqin; Feng, Zhan; Liu, Ping; Zhang, Yafei; He, Fangping; Chen, Zhongqin; Zhao, Kui; Luo, Benyan

    2016-01-01

    To identify clinical and neuroimaging characteristics between posterior cortical atrophy (PCA) and typical amnestic Alzheimer’s disease (tAD) patients at an early disease stage, 16 PCA and 13 age-matched tAD patients were enrolled. Compared with tAD patients, PCA patients showed higher mean recognition and recall test scores, and lower mean calculation, spatial attention, shape discrimination, and writing test scores. Mean right hippocampal volume was larger in PCA patients compared with tAD patients, while cortical gray matter (GM) volume of bilateral parietal and occipital lobes was smaller in PCA patients. Further, when compared with tAD patients, significant hypometabolism was observed in bilateral parietal and occipital lobes, particularly the right occipitotemporal junction in PCA patients. Additionally, there were significant positive correlations in recognition and recall scores with hippocampal volumes. In PCA patients, calculation and visuospatial ability scores are positively associated with GM volume of parietal and occipital lobes. And only spatial attention and shape discrimination scores are positively associated with regional glucose metabolism of parietal and occipital lobes. Therefore, PCA patients display better recognition and recall scores, which are associated with larger hippocampal volumes and poorer performance in visual spatial tasks because of marked GM atrophy and hypometabolism of parietal and occipital lobes. PMID:27377199

  5. Disease-specific dynamic biomarkers selected by integrating inflammatory mediators with clinical informatics in ARDS patients with severe pneumonia.

    PubMed

    Chen, Chengshui; Shi, Lin; Li, Yuping; Wang, Xiangdong; Yang, Shuanying

    2016-06-01

    Acute respiratory distress syndrome (ARDS) is a heterogeneous syndrome that occurs as a result of various risk factors, including either direct or indirect lung injury, and systemic inflammation triggered also by severe pneumonia (SP). SP-ARDS-associated morbidity and mortality remains high also due to the lack of disease-specific biomarkers. The present study aimed at identifying disease-specific biomarkers in SP or SP-ARDS by integrating proteomic profiles of inflammatory mediators with clinical informatics. Plasma was sampled from the healthy as controls or patients with SP infected with bacteria or infection-associated SP-ARDS on the day of admission, day 3, and day 7. About 15 or 52 cytokines showed significant difference between SP and SP-ARDS patients with controls or 13 between SP-ARDS with SP alone and controls, including bone morphogenetic protein-15 (BMP-15), chemokine (C-X-C motif) ligand 16 (CXCL16), chemokine (C-X-C motif) receptor 3 (CXCR3), interleukin-6 (IL-6), protein NOV homolog (NOV/CCN3), glypican 3, insulin-like growth factor binding protein 4 (IGFBP-4), IL-5, IL-5 R alpha, IL-22 BP, leptin, MIP-1d, and orexin B with a significant correlation with Digital Evaluation Score System (DESS) scores. ARDS patients with overexpressed IL-6, CXCL16, or IGFBP-4 had significantly longer hospital stay and higher incidence of secondary infection. We also found higher levels of those mediators were associated with poor survival rates in patients with lung cancer and involved in the process of the epithelial mesenchymal transition of alveolar epithelial cells. Our preliminary study suggested that integration of proteomic profiles with clinical informatics as part of clinical bioinformatics is important to validate and optimize disease-specific and disease-staged biomarkers.

  6. Role of noninvasive ventilation in perioperative patients with neuromuscular disease: a clinical case.

    PubMed

    Marinho, Ana; Guimarães, Mario José; Lages, Neusa Cristina Ribeiro; Correia, Carlos

    2016-01-01

    The inclusion body myositis is an inflammatory myopathy that leads to chronic muscle inflammation associated with muscle weakness. It is characterized by a restrictive ventilatory syndrome requiring ventilatory support under non-invasive ventilation. The authors describe a clinical case and the anaesthetic management of a patient with inclusion body myopathy candidate for vertebroplasty, which highlights the importance of locoregional anaesthesia and of noninvasive ventilation and includes assisted cough techniques, maintained throughout the perioperative period. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

  7. A systematic review on the effectiveness of nurse-led cardiac clinics for adult patients with coronary heart disease.

    PubMed

    Schadewaldt, Verena; Schultz, Tim

    2010-01-01

    Nurse-led clinics offer the possibility of patient education, support and continuity of care for patients with Coronary Heart Disease (CHD) to address and reduce risk factors. The objective of this review is to present the best available evidence related to nurse-led cardiac clinics for adults with CHD by updating an existing systematic review. Twenty databases for published literature and 11 databases or websites for unpublished literature were searched for the period September 2002 to March 2008. Additionally, reference lists of retrieved articles were reviewed and relevant journals were hand searched. Assessment for methodological quality, data extraction and synthesis was undertaken using a systematic review management tool (JBI-SUMARI). Seven RCTs formed the basis for this systematic review, however, in many cases meta-analysis was not possible because of a lack of relevant data. The major nurse-led intervention applied in the clinics consisted of health promotion through educating and counselling the patients. There were no harmful effects on patients with CHD identified when exposed to a nurse-led clinic. Few risk factors were significantly reduced by attending nurse-led clinic, but particular in the long-term no success in risk factor reduction was achieved. Difficulties were obvious in modifying behaviour changes concerning smoking cessation and diet adherence. Nurse-led clinics may positively influence perceived quality of life and general health status, especially physical functioning. The results indicated that care was equivalent to non-nurse led clinics, and there was no greater risk of poorer outcomes in the nurse-led clinics. The effectiveness of clinics might be dependent on the intensity of the nursing support. The combination of counselling and regular assessment of risk factors and health status delivered at nurse-led clinics is supported by the available research, and given that outcomes were in general equivalent between nurse-led and other

  8. Imaging Classification of Autosomal Dominant Polycystic Kidney Disease: A Simple Model for Selecting Patients for Clinical Trials

    PubMed Central

    Irazabal, María V.; Rangel, Laureano J.; Bergstralh, Eric J.; Osborn, Sara L.; Harmon, Amber J.; Sundsbak, Jamie L.; Bae, Kyongtae T.; Chapman, Arlene B.; Grantham, Jared J.; Mrug, Michal; Hogan, Marie C.; El-Zoghby, Ziad M.; Harris, Peter C.; Erickson, Bradley J.; King, Bernard F.

    2015-01-01

    The rate of renal disease progression varies widely among patients with autosomal dominant polycystic kidney disease (ADPKD), necessitating optimal patient selection for enrollment into clinical trials. Patients from the Mayo Clinic Translational PKD Center with ADPKD (n=590) with computed tomography/magnetic resonance images and three or more eGFR measurements over ≥6 months were classified radiologically as typical (n=538) or atypical (n=52). Total kidney volume (TKV) was measured using stereology (TKVs) and ellipsoid equation (TKVe). Typical patients were randomly partitioned into development and internal validation sets and subclassified according to height-adjusted TKV (HtTKV) ranges for age (1A–1E, in increasing order). Consortium for Radiologic Imaging Study of PKD (CRISP) participants (n=173) were used for external validation. TKVe correlated strongly with TKVs, without systematic underestimation or overestimation. A longitudinal mixed regression model to predict eGFR decline showed that log2HtTKV and age significantly interacted with time in typical patients, but not in atypical patients. When 1A–1E classifications were used instead of log2HtTKV, eGFR slopes were significantly different among subclasses and, except for 1A, different from those in healthy kidney donors. The equation derived from the development set predicted eGFR in both validation sets. The frequency of ESRD at 10 years increased from subclass 1A (2.4%) to 1E (66.9%) in the Mayo cohort and from 1C (2.2%) to 1E (22.3%) in the younger CRISP cohort. Class and subclass designations were stable. An easily applied classification of ADPKD based on HtTKV and age should optimize patient selection for enrollment into clinical trials and for treatment when one becomes available. PMID:24904092

  9. Clinical and immunological studies on patients with Graves' disease preoperatively treated with corticosteroids and iodides.

    PubMed

    Kasuga, Y; Sugenoya, A; Kobayashi, S; Kaneko, G; Masuda, H; Fujimori, M; Takahashi, S; Iida, F

    1990-05-01

    The purpose of this study was to evaluate the acute effects of corticosteroid and iodide preoperative therapy in patients with Graves' disease in terms of thyroid function and immunological parameters. The above combination was prescribed for 4 patients who had experienced severe side effects from antithyroid drugs (ATD) in order to reduce the possibility of post-thyroidectomy thyroid storm. Corticosteroids were employed daily for four days, and iodides were given daily for two weeks prior to thyroidectomy. The free T3 values decreased rapidly to euthyroid levels following the administration of both drugs, although the free T4 values were still much higher than normal in 3 of the 4 patients at the time of surgery. By comparison, 3 of 8 patients treated with ATD also had thyroid hormone levels above normal. Studies of lymphocyte subsets revealed that the percentage of helper T cells was significantly less in the corticosteroid-iodide treatment group than in the control and ATD groups. It is thus possible that postoperative thyroid storm might be prevented through corticosteroid-iodide therapy by virtue of the reduction of free T3 values to within the normal range by the time of surgery. The acute suppression of helper T cells was another results of this form of therapy observed.

  10. Clinical and Genotypic Spectrum of Chronic Granulomatous Disease in 71 Latin American Patients: First Report from the LASID Registry.

    PubMed

    de Oliveira-Junior, Edgar Borges; Zurro, Nuria Bengala; Prando, Carolina; Cabral-Marques, Otavio; Pereira, Paulo Vitor Soeiro; Schimke, Lena-Friederick; Klaver, Stefanie; Buzolin, Marcia; Blancas-Galicia, Lizbeth; Santos-Argumedo, Leopoldo; Pietropaolo-Cienfuegos, Dino Roberto; Espinosa-Rosales, Francisco; King, Alejandra; Sorensen, Ricardo; Porras, Oscar; Roxo-Junior, Persio; Forte, Wilma Carvalho Neves; Orellana, Julio Cesar; Lozano, Alejandro; Galicchio, Miguel; Regairaz, Lorena; Grumach, Anete Sevciovic; Costa-Carvalho, Beatriz Tavares; Bustamante, Jacinta; Bezrodnik, Liliana; Oleastro, Matias; Danielian, Silvia; Condino-Neto, Antonio

    2015-12-01

    We analyzed data from 71 patients with chronic granulomatous disease (CGD) with a confirmed genetic diagnosis, registered in the online Latin American Society of Primary Immunodeficiencies (LASID) database. Latin American CGD patients presented with recurrent and severe infections caused by several organisms. The mean age at disease onset was 23.9 months, and the mean age at CGD diagnosis was 52.7 months. Recurrent pneumonia was the most frequent clinical condition (76.8%), followed by lymphadenopathy (59.4%), granulomata (49.3%), skin infections (42%), chronic diarrhea (41.9%), otitis (29%), sepsis (23.2%), abscesses (21.7%), recurrent urinary tract infection (20.3%), and osteomyelitis (15.9%). Adverse reactions to bacillus Calmette-Guérin (BCG) vaccination were identified in 30% of the studied Latin American CGD cases. The genetic diagnoses of the 71 patients revealed 53 patients from 47 families with heterogeneous mutations in the CYBB gene (five novel mutations: p.W361G, p.C282X, p.W483R, p.R226X, and p.Q93X), 16 patients with the common deletion c.75_76 del.GT in exon 2 of NCF1 gene, and two patients with mutations in the CYBA gene. The majority of Latin American CGD patients carry a hemizygous mutation in the CYBB gene. They also presented a wide range of clinical manifestations most frequently bacterial and fungal infections of the respiratory tract, skin, and lymph nodes. Thirty percent of the Latin American CGD patients presented adverse reactions to BCG, indicating that this vaccine should be avoided in these patients. © 2015 Wiley Periodicals, Inc.

  11. Pediatric nail diseases: clinical pearls.

    PubMed

    Oberlin, Kate E

    2017-02-01

    This article highlights pearls shared during a unique and enlightening lecture by Antonella Tosti, MD, a professor at the University of Miami Health System, Florida, on the presentation and management of common pediatric nail diseases. These clinical pearls are shared to help deliver utmost care to our pediatric patients presenting with nail pathology and may help shed light on the management of pediatric nail diseases.

  12. How common is clinically inactive disease in a prospective cohort of patients with juvenile idiopathic arthritis? The importance of definition.

    PubMed

    Shoop-Worrall, Stephanie J W; Verstappen, Suzanne M M; Baildam, Eileen; Chieng, Alice; Davidson, Joyce; Foster, Helen; Ioannou, Yiannis; McErlane, Flora; Wedderburn, Lucy R; Thomson, Wendy; Hyrich, Kimme L

    2017-08-01

    Many criteria for clinically inactive disease (CID) and minimal disease activity (MDA) have been proposed for juvenile idiopathic arthritis (JIA). It is not known to what degree each of these criteria overlap within a single patient cohort. This study aimed to compare the frequency of MDA and CID across different criteria in a cohort of children with JIA at 1 year following presentation. The Childhood Arthritis Prospective Study recruits children at initial presentation to paediatric or adolescent rheumatology in seven UK centres. Children recruited between October 2001 and December 2013 were included. The proportions of children with CID and MDA at 1 year were calculated using four investigator-defined and eight published composite criteria. Missing data were accounted for using multiple imputation under different assumptions. In a cohort of 1415 children and adolescents, 67% patients had no active joints at 1 year. Between 48% and 61% achieved MDA and between 25% and 38% achieved CID using published criteria. Overlap between criteria varied. Of 922 patients in MDA by either the original composite criteria, Juvenile Arthritis Disease Activity Score (JADAS) or clinical JADAS cut-offs, 68% were classified as in MDA by all 3 criteria. Similarly, 44% of 633 children with CID defined by either Wallace's preliminary criteria or the JADAS cut-off were in CID according to both criteria. In a large JIA prospective inception cohort, a majority of patients have evidence of persistent disease activity after 1 year. Published criteria to capture MDA and CID do not always identify the same groups of patients. This has significant implications when defining and applying treat-to-target strategies. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  13. Diagnostic accuracy and clinical relevance of brain natriuretic peptide assay in pediatric patients with congenital heart diseases.

    PubMed

    Cantinotti, Massimiliano; Vittorini, Simona; Storti, Simona; Prontera, Concetta; Murzi, Michele; De Lucia, Vittoria; Recla, Sabine; Assanta, Nadia; Giusti, Sandra; Murzi, Bruno; Bottone, Ugo; Clerico, Aldo

    2009-09-01

    The clinical usefulness of B-type natriuretic peptide (BNP) assay in congenital heart diseases is still controversial. We evaluated the diagnostic accuracy and clinical relevance of this assay in different cardiovascular hemodynamic conditions in pediatric patients with congenital heart diseases. BNP was measured in 173 healthy individuals and in 382 pediatric patients with congenital heart diseases. We identified five subgroups of hemodynamic conditions: left ventricular volume overload, right ventricular volume overload, left ventricular pressure overload, right ventricular pressure overload, and biventricular volume overload. BNP was higher (P<0.0001) in patients (median 49.0 ng/l, range 0.45-14363 ng/l) than in the reference population (median 6.1 ng/l, range 1.0-29.7 ng/l). BNP assay showed a good diagnostic accuracy in discriminating between healthy individuals and patients (area under the receiver operating characteristic curve 0.95, SE 0.009). Lower BNP values were found in right ventricular pressure overload than in left ventricular pressure overload, left ventricular volume overload, or biventricular volume overload. In the left ventricular volume overload subgroup, BNP significantly correlated with peak systolic gradients (rho=0.622, P<0.001) and left ventricular dilatation (rho=0.35, P=0.03). In the right ventricular volume overload subgroup, BNP correlated with right ventricular pressure (rho=0.622, P<0.001) and right ventricular dilatation (rho=0.377, P=0.0077). Moreover, in cyanotic defects, BNP showed a significant correlation with O2 saturation (rho=0.204 P=0.0128). BNP assay showed a good diagnostic accuracy in discriminating between healthy individuals and patients. Congenital heart disease with left ventricular pressure overload and biventricular volume overload has higher BNP values than that with right ventricular pressure overload.

  14. [Cognitive impairment in patients with coronary disease: relationship with clinical variables].

    PubMed

    Pérez-Belmonte, Luis M; Lara, J Pablo; Olalla-Mercadé, Eduardo; Gutiérrez de Loma, Julio; Barbancho, Miguel A; San Román-Terán, Carlos M

    2014-08-04

    Coronary disease has been associated with cognitive disorders. We studied the presence of dysexecutive mild cognitive impairment in patients scheduled for coronary surgery. The executive function of 35 patients was evaluated (Trail Making Test). They were classified into 2 groups: normal performance or cognitive impairment, and we assessed the relationship with others variables (Mann-Whitney, chi-square and multiple regression analysis). The dysexecutive cognitive impairment group (n=7; 20%) showed greater degree of angina (odds ratio [OR] 1.4, 95% confidence interval [95% CI] 1.1-2.6; P=.04), 3-vessels coronary artery disease (OR 1.3, 95% CI 1.08-3.6; P=.04) and body mass index (OR 1.56, 95% CI 1.16-3.65; P=.03) and lower diastolic blood pressure (OR 1.56, 95% CI 1.2-2.98; P=.02), hemoglobin (OR 2.03, 95% CI 1.18-4.05; P=.02) and hematocrit (OR 2.45, 95% CI 1.67-3.99; P<.001); these variables proved to be significant in the test performance considered as a dependent variable (R(2)=0.62). We found a significant prevalence of dysexecutive mild cognitive impairment, which was associated with cardiovascular risk factors. We recommend assessment and monitoring of cognitive performance for probable neurological complications after cardiac surgery. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  15. Lack of Clinical Relevance of ANA and ASMA Positivity in Patients with Liver Transplantation without a History of Autoimmune Diseases

    PubMed Central

    Pellegrini, Lucienne; Parrilli, Gianpaolo; Santonicola, Antonella; Cinquanta, Luigi; Caputo, Cesare

    2017-01-01

    The relevance of isolated autoimmunity elevation in orthotopic liver transplantation (OLT) patients is unknown. Our aim was to analyse how serum autoantibodies change in time and to evaluate their clinical relevance in OLT patients. Patients were invited to provide samples to evaluate ANA, AMA, ASMA, and LKM at the time of enrolment (T0), after 6 months (T6), and after 12 months (T12). We included 114 patients in the study (76% males, median age 62.5 years), finding isolated elevation of at least one serum antibody in up to 80% of them. We described fluctuating positive autoantibodies in the one year of observation, with only 45.6% of patients positive for ANA and less than 2% positive for ASMA, at all three times. Isolated elevation of tissue antibodies was not related to gender, age, HCC at transplant, early rejection, cause of transplantation, immunotherapy taken, and age at the time of the study. We did not detect a higher prevalence of positive autoimmunity in patients with signs of liver injury. ANA and ASMA evaluation in patients with liver transplantation and no history of autoimmune disease has no clinical relevance, since it varies in time and is not related to any risk factors or liver injury. Routine autoimmunity evaluation should be avoided. PMID:28337446

  16. Lack of Clinical Relevance of ANA and ASMA Positivity in Patients with Liver Transplantation without a History of Autoimmune Diseases.

    PubMed

    Pellegrini, Lucienne; Parrilli, Gianpaolo; Santonicola, Antonella; Cinquanta, Luigi; Caputo, Cesare; Ciacci, Carolina; Zingone, Fabiana

    2017-01-01

    The relevance of isolated autoimmunity elevation in orthotopic liver transplantation (OLT) patients is unknown. Our aim was to analyse how serum autoantibodies change in time and to evaluate their clinical relevance in OLT patients. Patients were invited to provide samples to evaluate ANA, AMA, ASMA, and LKM at the time of enrolment (T0), after 6 months (T6), and after 12 months (T12). We included 114 patients in the study (76% males, median age 62.5 years), finding isolated elevation of at least one serum antibody in up to 80% of them. We described fluctuating positive autoantibodies in the one year of observation, with only 45.6% of patients positive for ANA and less than 2% positive for ASMA, at all three times. Isolated elevation of tissue antibodies was not related to gender, age, HCC at transplant, early rejection, cause of transplantation, immunotherapy taken, and age at the time of the study. We did not detect a higher prevalence of positive autoimmunity in patients with signs of liver injury. ANA and ASMA evaluation in patients with liver transplantation and no history of autoimmune disease has no clinical relevance, since it varies in time and is not related to any risk factors or liver injury. Routine autoimmunity evaluation should be avoided.

  17. [The clinical analysis of fulminant Wilson's disease in patients with hepatitis B virus infection: a report of 13 cases].

    PubMed

    Deng, H H; Xu, M

    2016-08-01

    To analyze the clinical features and prognosis of fulminant Wilson's disease (FWD) in patients with hepatitis B virus (HBV) infection. Twenty-seven patients were enrolled in Guangzhou Eighth People's Hospital from 2005 to 2015, including 13 FWD patients with HBV infection and 14 FWD patients without HBV infection. Clinical efficacy and survival rate were evaluated. Baseline biochemical data in two groups were comparable(P>0.05), including total bilirubin, prothrombin activity, serum albumin, alpha fetal protein, alanine transaminase, ceruloplasmin and 24 hours urine copper .Treatment in FWD group with HBV infection was ineffective, including 9(9/13) deaths and 4(4/13) patients receiveing liver transplants. However, 7(7/14)cases in the other group did not response to the treatment, including 6(6/14)deaths and 1(1/14)patient receiving liver transplant. The prognosis in the two groups is significantly different(P=0.006), which is much worse in FWD patients with HBV infection.

  18. [Clinic characteristics of patients with trophoblastic gestational disease complicate with hypertension].

    PubMed

    Tovar-Rodríguez, José María; Medel-Lagunes, Irais del Carmen; Acosta-Altamirano, Gustavo; Vargas-Hernández, Víctor Manuel

    2013-10-01

    The molar pregnancy is complicated with hypertension before 20 weeks, divided into complete mole and partial mole, and in diploid and triploid hydatidiform mola depending on the fetal chromosomes. To determine clinical and laboratory characteristics of patients with hydatidiform mole with and without hypertension, and choriocarcinoma, correlate serum chorionic gonadotropin (hCG) and hypertension. We reviewed 55 cases with histopathologically proven mole, separately analyzed clinical, laboratory and hCG. The prevalence of mole with hypertension was 1:7; with the choriocarcinoma is 1:11. The age and sexual initiation of mole with hypertension was higher (p = .004 and .002 respectively), liver transaminase AST (p =. 004) and lactate dehydrogenase are higher in the group with hypertension (p =. 000). Positive correlation was obtained r = .246 p =. 044 statistically significant between mean arterial pressure and hCG. We reviewed 31 cases of 28 national and international articles, in patients with diploid hydatidiform the product is allowed to live normal and high blood pressure is showing mild preeclampsia, while partial moles are triploid, malformed products, incompatible with life and with hypertension severe like severe pre-eclampsia. The molar pregnancy is partially diagnosed with hypertension in our environment, is not properly analyzed and that most of the products of curettage were not performed genetic testing. The hCG probably participate in the hypertensive disorders of pregnancy.

  19. Transition clinic attendance is associated with improved beliefs and attitudes toward medicine in patients with inflammatory bowel disease

    PubMed Central

    Fu, Nancy; Jacobson, Kevan; Round, Andrew; Evans, Kathi; Qian, Hong; Bressler, Brian

    2017-01-01

    AIM To evaluated the differences in knowledge, adherence, attitudes, and beliefs about medicine in adolescents with inflammatory bowel disease (IBD) attending transition clinics. METHODS We prospectively enrolled patients from July 2012 to June 2013. All adolescents who attended a tertiary-centre-based dedicated IBD transition clinic were invited to participate. Adolescent controls were recruited from university-affiliated gastroenterology offices. Participants completed questionnaires about their disease and reported adherence to prescribed therapy. Beliefs in Medicine Questionnaire was used to evaluate patients’ attitudes and beliefs. Beliefs of medication overuse, harm, necessity and concerns were rated on a Likert scale. Based on necessity and concern ratings, attitudes were then characterized as accepting, ambivalent, skeptical and indifferent. RESULTS One hundred and twelve adolescents were included and 59 attended transition clinics. Self-reported adherence rates were poor, with only 67.4% and 56.8% of patients on any IBD medication were adherent in the transition and control groups, respectively. Adolescents in the transition cohort held significantly stronger beliefs that medications were necessary (P = 0.0035). Approximately 20% of adolescents in both cohorts had accepting attitudes toward their prescribed medicine. However, compared to the control group, adolescents in the transition cohort were less skeptical of (6.8% vs 20.8%) and more ambivalent (61% vs 34%) (OR = 0.15; 95%CI: 0.03-0.75; P = 0.02) to treatment. CONCLUSION Attendance at dedicated transition clinics was associated with differences in attitudes in adolescents with IBD. PMID:28839441

  20. Tubular B7-1 expression parallels proteinuria levels, but not clinical outcomes in adult minimal change disease patients

    PubMed Central

    Lee, Sung Woo; Baek, Seon Ha; Paik, Jin Ho; Kim, Sejoong; Na, Ki Young; Chae, Dong-Wan; Chin, Ho Jun

    2017-01-01

    B7-1 is thought to play a pathogenic role in minimal-change disease (MCD). Recently, however, doubts have arisen regarding the role of B7-1 expression in MCD. Therefore, we aimed to identify the presence and clinical significance of B7-1 expression in MCD patients. The study participants included 28 adult MCD patients for whom kidney specimens were available. The intensity of B7-1 expression was assessed by two independent specialists. We analysed the association between the intensity of B7-1 expression and clinicopathological variables. No B7-1 expression in the glomeruli was observed in any of the 28 patients. Unexpectedly, however, 75.0% of the patients exhibited tubular B7-1 expression, with 35.7% demonstrating weak positive expressions and 39.3% demonstrating strong positive expressions. The level of proteinuria significantly increased as the intensity of tubular B7-1 expression increased. We also found trends of increasing blood urea nitrogen and serum creatinine levels with increased intensity of tubular B7-1 expression. However, we could not observe definite differences in long- and short-term clinical outcomes depending on the intensity of tubular B7-1 expression. In conclusion, B7-1 was expressed in renal tubular cells but not in glomeruli in adult MCD patients. The intensity of tubular B7-1 expression paralleled proteinuria levels, but not clinical outcomes. PMID:28150736

  1. [ANMCO/GICR-IACPR/SICI-GISE Consensus document: Clinical management of patients with stable coronary artery disease].

    PubMed

    Riccio, Carmine; Gulizia, Michele Massimo; Colivicchi, Furio; Di Lenarda, Andrea; Musumeci, Giuseppe; Faggiano, Pompilio Massimo; Abrignani, Maurizio Giuseppe; Rossini, Roberta; Fattirolli, Francesco; Valente, Serafina; Mureddu, Gian Francesco; Temporelli, Pier Luigi; Olivari, Zoran; Amico, Antonio Francesco; Casolo, Giancarlo; Fresco, Claudio; Menozzi, Alberto; Nardi, Federico

    2016-01-01

    Stable coronary artery disease is of epidemiological importance. It is becoming increasingly common due to the longer life expectancy, being strictly related to age and to advances in diagnostic techniques and pharmacological and non-pharmacological interventions.Stable coronary artery disease encompasses a variety of clinical and anatomic presentations, making the identification of its clinical and anatomical features challenging. Therapeutic interventions should be defined on an individual basis according to the patient's risk profile. To this aim, management flow-charts have been reviewed based on sustainability and appropriateness derived from recent evidence. Special emphasis has been placed on non-pharmacological interventions, stressing the importance of lifestyle changes, including smoking cessation, regular physical activity and diet. Adherence to therapy as an emerging risk factor is also discussed.

  2. Clinical and Epidemiological Investigation of TCM Syndromes of Patients with Coronary Heart Disease in China.

    PubMed

    Ren, Yi; Zhang, Minzhou; Chen, Keji; You, Shijie; Li, Jianjun; Guo, Liheng; Wang, Lei

    2012-01-01

    To compare the regional differences in TCM syndromes of patients with coronary heart disease (CHD) between North and South China. A total of 624 patients with a diagnosis of CHD, confirmed by coronary angiography, were included in the comparative analysis to determine the occurrence pattern, characteristics of TCM syndrome distribution, and differences in syndrome combinations and major syndrome types (deficiency or excess) between North and South China. The incidence of CHD tended to be higher in North China (54.6%) compared with that in South China (45.4%). The proportions of patients with a qi-deficiency syndrome (83.7%), turbid phlegm syndrome (68.9%), or blood stasis syndrome (91.5%) were generally higher in the South group, while the proportion of patients with a cold congelation syndrome (7.9%) was identified to be obviously higher in the North group (P < 0.01). Moreover, compared with that in the South group, the overall frequency of syndrome combinations tended to be lower in the North group (P < 0.01); and the most common types of TCM syndrome were excess syndrome (193, 56.6%) and primary deficiency and secondary excess syndrome (244, 86.2%) in the North and South groups, respectively (P < 0.01). A regional difference does exist in the TCM syndromes of patients with CHD between North and South China, indicating that the prevention and treatment of CHD in South China should not only focus on promoting blood circulation and removing blood stasis, but also include supplementing qi and eliminating phlegm.

  3. Clinical and Epidemiological Investigation of TCM Syndromes of Patients with Coronary Heart Disease in China

    PubMed Central

    Ren, Yi; Zhang, Minzhou; Chen, Keji; You, Shijie; Li, Jianjun; Guo, Liheng; Wang, Lei

    2012-01-01

    To compare the regional differences in TCM syndromes of patients with coronary heart disease (CHD) between North and South China. A total of 624 patients with a diagnosis of CHD, confirmed by coronary angiography, were included in the comparative analysis to determine the occurrence pattern, characteristics of TCM syndrome distribution, and differences in syndrome combinations and major syndrome types (deficiency or excess) between North and South China. The incidence of CHD tended to be higher in North China (54.6%) compared with that in South China (45.4%). The proportions of patients with a qi-deficiency syndrome (83.7%), turbid phlegm syndrome (68.9%), or blood stasis syndrome (91.5%) were generally higher in the South group, while the proportion of patients with a cold congelation syndrome (7.9%) was identified to be obviously higher in the North group (P < 0.01). Moreover, compared with that in the South group, the overall frequency of syndrome combinations tended to be lower in the North group (P < 0.01); and the most common types of TCM syndrome were excess syndrome (193, 56.6%) and primary deficiency and secondary excess syndrome (244, 86.2%) in the North and South groups, respectively (P < 0.01). A regional difference does exist in the TCM syndromes of patients with CHD between North and South China, indicating that the prevention and treatment of CHD in South China should not only focus on promoting blood circulation and removing blood stasis, but also include supplementing qi and eliminating phlegm PMID:22536290

  4. Correlates for cardiovascular diseases among diabetic/hypertensive patients attending outreach clinics in two Nairobi slums, Kenya.

    PubMed

    Mugure, Gladys; Karama, Mohamed; Kyobutungi, Catherine; Karanja, Simon

    2014-01-01

    Cardiovascular diseases (CVD) are the leading cause of death in the world. Over 80% of CVD related deaths occur in low- and middle-income countries (LMICs). Diabetes and hypertension, whose prevalence in Kenya is on the rise, are major risk factors for CVD. Despite this, studies indicate that awareness on the management of risk factors for CVD among diabetic/hypertensive patients in African populations is generally low. The aim of the study was to determine the risk factors for CVD among diabetic and/or hypertensive patients attending diabetes and hypertension management clinics in Korogocho and Viwandani slums of Nairobi. Data were collected using questionnaires administered to 206 diabetic/hypertensive patients attending the clinics between July 2010 and February 2011. A review of these patients' medical records was done to determine the history of CVD outcomes such as hypertensive heart diseases, stroke and peripheral arterial diseases. Majority (66.5%) of the study participants were females mainly in the 51-65 age category. The study findings revealed that 73 (33.4%) respondents had CVD outcomes. In addition, 41.8% of the respondents were not aware of the causes of diabetes/hypertension. Age category 51-65 years had the highest (43.8%) number of respondents with CVD. Sex of the respondents and awareness of the link between hypertension and CVD were significantly associated with CVD outcomes (p<0.05) among the respondents. Measures to improve awareness levels among patients at high risk of CVD outcomes are needed to complement other measures to reduce CVD risk among such patients.

  5. Real-world clinical experience of biological disease modifying anti-rheumatic drugs in Malaysia rheumatoid arthritis patients.

    PubMed

    Tan, Bee Eng; Lim, Ai Lee; Kan, Sow Lai; Lim, Chong Hong; Tsang, Esther Ee Ling; Ch'ng, Shereen Suyin; Mohd Noor, Nadiah; Mohd Jamid, Nurulraziquin; Teh, Cheng Lay; Joshua Thundyil, Rachel; Loh, Yet Lin; Chong, Hwee Cheng; Ong, Swee Gaik; Mohamed Ismail, Asmahan; Lee, Yvonne Y L; Gun, Suk Chyn

    2017-07-10

    The effect of biologic disease modifying anti-rheumatic drugs (bDMARDs) in treating rheumatoid arthritis (RA) in real-world clinical practice remains unknown in Southeast Asia. We aimed to assess the efficacy and safety of bDMARDs among Malaysian RA patients treated in routine clinical practice. A retrospective medical chart review of RA patients from 11 government hospitals were conducted from January 2003 to January 2014. A standardized questionnaire was used to abstract patient's demographic, clinical and treatment data. Level of disease activity was measured by DAS28 collected at baseline, 3, 6 and 12 months. Three hundred and one patients were available for analysis, mean age 41 (SD, 10.8) years, mean RA duration 12.3 (SD, 6.9) years and 98% had history of two or more conventional-synthetic DMARDs. There were 467 bDMARD courses prescribed with mean bDMARDs duration use of 12.9 months (SD 14.7). Tumour necrosis factor alpha inhibitors were the most common prescribed bDMARDs (77.1%), followed by Tocilizumab (14.6%) and Rituximab (8.4%). We observed significant improvement in mean DAS28 values from baseline to 3, 6 and 12 months (p < 0.001). Overall, 16.9% achieved DAS28 remission at 6 months. A third (35.6%) of patients reported adverse events, three commonest being infections (46.5%), allergy (22.9%) and laboratory abnormalities (12.9%). 3.7% of our patients had tuberculosis. Biologic DMARDs were effective in treating RA in real-world practice in Malaysia, despite a lower remission rate compared to developed countries. Except for higher rates of tuberculosis, the AEs were similar to the published reports.

  6. Impact of a Clinical Pharmacy Service on the Management of Patients in a Sickle Cell Disease Outpatient Center.

    PubMed

    Han, Jin; Bhat, Shubha; Gowhari, Michel; Gordeuk, Victor R; Saraf, Santosh L

    2016-11-01

    Ambulatory care clinical pharmacy services have expanded beyond primary care settings, but literature supporting the benefits of clinical pharmacy involvement with patients who have rare diseases such as sickle cell disease (SCD) is lacking. Hydroxyurea is the only agent approved by the U.S. Food and Drug Administration for the treatment of SCD; full benefit in controlling pain episodes and other complications is achieved through monitored escalation to a maximum tolerated dose. The primary objective of this analysis was to evaluate the impact of a newly implemented clinical pharmacy service on the management of patients with SCD. We performed a retrospective cross-sectional analysis of 385 adults with SCD who received care between January 1, 2014, and December 31, 2014, at a single Sickle Cell Outpatient Center that implemented a clinical pharmacy service in August 2013. Data were collected on hydroxyurea dose escalation, immunization completion rates, and health maintenance metrics (screening for nephropathy with microalbuminuria testing, retinopathy with annual retinal examinations, and pulmonary hypertension with echocardiography). The impact of the clinical pharmacy service on quality measurements was evaluated by using univariate and multivariate analyses. The number of pharmacist encounters, defined as a clinic visit when a clinical pharmacist interacted with a patient as documented in the medical records, was associated with an improved hydroxyurea dose escalation rate (odds ratio [OR] 1.48, 95% confidence interval [CI] 1.07-2.05, p=0.02). Immunization rates for the 23-valent pneumococcal polysaccharide vaccine, the 13-valent pneumococcal conjugate vaccine, and influenza vaccine were 66%, 47%, and 62%, respectively. The number of pharmacist encounters was associated with improved immunization completion rates (OR 1.38, 95% CI 1.17-1.62, p<0.001). Improved screening for microalbuminuria (OR 2.14, 95% CI 1.60-2.86, p<0.001) and sickle cell retinopathy (OR 1

  7. Clinical effectiveness of low-power laser radiation and functioning of hemosalivatory barrier in patients with rheumatic diseases

    NASA Astrophysics Data System (ADS)

    Gladkova, Natalia D.; Karachistov, Alexander B.; Komarova, Lia G.; Alekseeva, Olga P.; Grunina, Elena A.

    1996-11-01

    We have estimated the clinical effectiveness of several regimes and ways of low power laser therapy (LT) on the basis of a double 'blind', placebo-controlling randomizing comparative test in 454 patients with rheumatic diseases (RD). LT for RD has a well-expressed placebo effect. The level of clinical effect of LT for RD is not so high. We couldn't achieve 'a considerable improvement' in any cases, 'an improvement' was secured in only 18 percent. LT should be viewed as a symptomatic means, with a primary anesthetic and feebly expressed anti-inflammatory effect, which can not influence the course of the rheumatoid process. Only in 15 percent of patients with RD, a sufficient functioning of hemo-salivary barrier was observed, the latter providing a reserve for adaption mechanism, which leads under the influence of stressor agents of medium strength not only to anesthetic, but also to moderately expressed anti- inflammatory effect.

  8. Managing dyspnea in patients with advanced chronic obstructive pulmonary disease: A Canadian Thoracic Society clinical practice guideline

    PubMed Central

    Marciniuk, Darcy D; Goodridge, Donna; Hernandez, Paul; Rocker, Graeme; Balter, Meyer; Bailey, Pat; Ford, Gordon; Bourbeau, Jean; O’Donnell, Denis E; Maltais, Francois; Mularski, Richard A; Cave, Andrew J; Mayers, Irvin; Kennedy, Vicki; Oliver, Thomas K; Brown, Candice

    2011-01-01

    Dyspnea is a cardinal symptom of chronic obstructive pulmonary disease (COPD), and its severity and magnitude increases as the disease progresses, leading to significant disability and a negative effect on quality of life. Refractory dyspnea is a common and difficult symptom to treat in patients with advanced COPD. There are many questions concerning optimal management and, specifically, whether various therapies are effective in this setting. The present document was compiled to address these important clinical issues using an evidence-based systematic review process led by a representative interprofessional panel of experts. The evidence supports the benefits of oral opioids, neuromuscular electrical stimulation, chest wall vibration, walking aids and pursed-lip breathing in the management of dyspnea in the individual patient with advanced COPD. Oxygen is recommended for COPD patients with resting hypoxemia, but its use for the targeted management of dyspnea in this setting should be reserved for patients who receive symptomatic benefit. There is insufficient evidence to support the routine use of anxiolytic medications, nebulized opioids, acupuncture, acupressure, distractive auditory stimuli (music), relaxation, hand-held fans, counselling programs or psychotherapy. There is also no evidence to support the use of supplemental oxygen to reduce dyspnea in nonhypoxemic patients with advanced COPD. Recognizing the current unfamiliarity with prescribing and dosing of opioid therapy in this setting, a potential approach for their use is illustrated. The role of opioid and other effective therapies in the comprehensive management of refractory dyspnea in patients with advanced COPD is discussed. PMID:21499589

  9. Managing dyspnea in patients with advanced chronic obstructive pulmonary disease: a Canadian Thoracic Society clinical practice guideline.

    PubMed

    Marciniuk, Darcy D; Goodridge, Donna; Hernandez, Paul; Rocker, Graeme; Balter, Meyer; Bailey, Pat; Ford, Gord; Bourbeau, Jean; O'Donnell, Denis E; Maltais, Francois; Mularski, Richard A; Cave, Andrew J; Mayers, Irvin; Kennedy, Vicki; Oliver, Thomas K; Brown, Candice

    2011-01-01

    Dyspnea is a cardinal symptom of chronic obstructive pulmonary disease (COPD), and its severity and magnitude increases as the disease progresses, leading to significant disability and a negative effect on quality of life. Refractory dyspnea is a common and difficult symptom to treat in patients with advanced COPD. There are many questions concerning optimal management and, specifically, whether various therapies are effective in this setting. The present document was compiled to address these important clinical issues using an evidence-based systematic review process led by a representative interprofessional panel of experts. The evidence supports the benefits of oral opioids, neuromuscular electrical stimulation, chest wall vibration, walking aids and pursed-lip breathing in the management of dyspnea in the individual patient with advanced COPD. Oxygen is recommended for COPD patients with resting hypoxemia, but its use for the targeted management of dyspnea in this setting should be reserved for patients who receive symptomatic benefit. There is insufficient evidence to support the routine use of anxiolytic medications, nebulized opioids, acupuncture, acupressure, distractive auditory stimuli (music), relaxation, handheld fans, counselling programs or psychotherapy. There is also no evidence to support the use of supplemental oxygen to reduce dyspnea in nonhypoxemic patients with advanced COPD. Recognizing the current unfamiliarity with prescribing and dosing of opioid therapy in this setting, a potential approach for their use is illustrated. The role of opioid and other effective therapies in the comprehensive management of refractory dyspnea in patients with advanced COPD is discussed.

  10. Paired-Pulse Inhibition in the Auditory Cortex in Parkinson's Disease and Its Dependence on Clinical Characteristics of the Patients

    PubMed Central

    Lukhanina, Elena; Berezetskaya, Natalia; Karaban, Irina

    2011-01-01

    We aimed to determine the value of the paired-pulse inhibition (PPI) in the auditory cortex in patients with Parkinson's disease (PD) and analyze its dependence on clinical characteristics of the patients. The central (Cz) auditory evoked potentials were recorded in 58 patients with PD and 22 age-matched healthy subjects. PPI of the N1/P2 component was significantly (P < .001) reduced for interstimulus intervals 500, 700, and 900 ms in patients with PD compared to control subjects. The value of PPI correlated negatively with the age of the PD patients (P < .05), age of disease onset (P < .05), body bradykinesia score (P < .01), and positively with the Mini Mental State Examination (MMSE) cognitive score (P < .01). Negative correlation between value of PPI and the age of the healthy subjects (P < .05) was also observed. Thus, results show that cortical inhibitory processes are deficient in PD patients and that the brain's ability to carry out the postexcitatory inhibition is age-dependent. PMID:21052541

  11. Hybrid Surgery Options for Complex Clinical Scenarios in Adult Patients with Congenital Heart Disease: Three Case Reports

    PubMed Central

    Rapetto, Filippo; Kenny, Damien; Turner, Mark; Parry, Andrew; Stoica, Serban; Uzun, Orhan; Caputo, Massimo

    2017-01-01

    The strategy for the management of adult patients with congenital heart disease (CHD) often represents a challenge for cardiac surgeons and cardiologists due to complex anatomy, wide range of clinical presentations, and a high-risk profile. However, hybrid approach may represent an attractive solution. We report three cases of adult patients previously operated for CHD and recently treated with a hybrid approach in our institution. Case 1: a 76-year-old woman with permanent atrial fibrillation, lung disease, chronic kidney disease, microcytic anemia, and type II diabetes mellitus, previously operated for atrial septal defect closure and pulmonary valvotomy, presented with severe pulmonary regurgitation and advanced right ventricular failure. In order to minimize the surgical risk, a hybrid approach was used: an extensive right ventricular outflow tract (RVOT) plication was followed by implantation of an Edwards Sapien XT prosthesis in the RVOT through the right ventricular apex, without cardiopulmonary bypass. Case 2: a 64-year-old man with previous atrial septum excision and pericardial baffle for partial anomalous pulmonary venous drainage with intact interatrial septum, presented with worsening dyspnea, right ventricular failure, and pulmonary hypertension caused by baffle stenosis. His comorbidities included coronary artery disease, atrial flutter, and previous left pneumonectomy. After performing a redo longitudinal median sternotomy, a 20-mm stent was implanted in the baffle with access through the superior vena cava. Case 3: a 50-year-old man, with previous atrioventricular septal defect repair, followed by mitral valve replacement with a mechanical prosthesis, subsequently developed a paravalvular leak (PVL) with severe mitral regurgitation and severe left ventricular dysfunction. He underwent a transapical PVL device closure with two Amplatzer Vascular Plugs. In our opinion, hybrid surgery is a promising therapeutic modality that increases the available

  12. [A descriptive study of the socio-demographic and clinical profile of the patient with chronic obstructive pulmonary disease].

    PubMed

    Abad-Corpa, Eva; Royo-Morales, Tania; Iniesta-Sánchez, Javier; Rodríguez-Mondéjar, Juan José; Carrillo-Alcaraz, Andrés; Pérez-García, M Carmen; Pérez-Gómez, M Carmen; Saez-Soto, Angeles Rosario

    2011-01-01

    To find out the socio-demographic and clinical profile of the patient with Chronic Obstructive Pulmonary Disease (COPD): characteristics, state of health, situation of disease and social resources. A descriptive, longitudinal and prospective study, in the Morales Meseguer and Reina Sofía Hospitals in Murcia (Spain), was performed between June 2007 and April 2008. The inclusion criteria was hospital admission due to COPD and patients with cognitive deterioration, a hospital stay > 30 days or < 2 days, or were institutionalised, were excluded. Socio-demographic, state of health and social variables were collected using the patient's clinical history and an interview during hospital stay and at discharge. The descriptive statistical analysis was carried out using SPSS v.15. A total of 143 patients were studied, of which 90.2% were males who had a mean age of 72.76 ± 8.04 years, 95.1% were in an inactive work situation and had a mean score on the social problem scale of 8.08 ± 2.1. Most were in a fragile state (71.3%), approximately half (44.8%) suffered heart disease as the main co-morbidity factor, and the impact of the disease on quality of life was 55.1 ± 19.01. The level of knowledge on the therapeutic regime was 3.13 ± 0.7. A total of 72% were independent for carrying out Basic Activities of Daily Living (BADL) after hospital discharge. Most of the population that suffers from COPD were elderly males, who were retired without social problems, with a high percentage of co-morbidity, an intermediate alteration in their quality of life, having a moderate level of knowledge about the therapeutic regime and were independent for BADL, but fragile. Copyright © 2010 Elsevier España, S.L. All rights reserved.

  13. Exome Sequencing of Cell-Free DNA from Metastatic Cancer Patients Identifies Clinically Actionable Mutations Distinct from Primary Disease

    PubMed Central

    Butler, Timothy M.; Johnson-Camacho, Katherine; Peto, Myron; Wang, Nicholas J.; Macey, Tara A.; Korkola, James E.; Koppie, Theresa M.; Corless, Christopher L.; Gray, Joe W.; Spellman, Paul T.

    2015-01-01

    The identification of the molecular drivers of cancer by sequencing is the backbone of precision medicine and the basis of personalized therapy; however, biopsies of primary tumors provide only a snapshot of the evolution of the disease and may miss potential therapeutic targets, especially in the metastatic setting. A liquid biopsy, in the form of cell-free DNA (cfDNA) sequencing, has the potential to capture the inter- and intra-tumoral heterogeneity present in metastatic disease, and, through serial blood draws, track the evolution of the tumor genome. In order to determine the clinical utility of cfDNA sequencing we performed whole-exome sequencing on cfDNA and tumor DNA from two patients with metastatic disease; only minor modifications to our sequencing and analysis pipelines were required for sequencing and mutation calling of cfDNA. The first patient had metastatic sarcoma and 47 of 48 mutations present in the primary tumor were also found in the cell-free DNA. The second patient had metastatic breast cancer and sequencing identified an ESR1 mutation in the cfDNA and metastatic site, but not in the primary tumor. This likely explains tumor progression on Anastrozole. Significant heterogeneity between the primary and metastatic tumors, with cfDNA reflecting the metastases, suggested separation from the primary lesion early in tumor evolution. This is best illustrated by an activating PIK3CA mutation (H1047R) which was clonal in the primary tumor, but completely absent from either the metastasis or cfDNA. Here we show that cfDNA sequencing supplies clinically actionable information with minimal risks compared to metastatic biopsies. This study demonstrates the utility of whole-exome sequencing of cell-free DNA from patients with metastatic disease. cfDNA sequencing identified an ESR1 mutation, potentially explaining a patient’s resistance to aromatase inhibition, and gave insight into how metastatic lesions differ from the primary tumor. PMID:26317216

  14. Major infections, secondary cancers and autoimmune diseases occur in different clinical subsets of chronic lymphocytic leukaemia patients.

    PubMed

    Visentin, Andrea; Imbergamo, Silvia; Gurrieri, Carmela; Frezzato, Federica; Trimarco, Valentina; Martini, Veronica; Severin, Filippo; Raggi, Flavia; Scomazzon, Edoardo; Facco, Monica; Piazza, Francesco; Semenzato, Gianpietro; Trentin, Livio

    2017-02-01

    Major infections (MIs), secondary cancers (SCs) and autoimmune diseases (ADs) are the most common and relevant complications in patients with chronic lymphocytic leukaemia. We performed a single-centre retrospective study to investigate the prevalence of the above quoted complications, the association with most important prognostic markers and their impact on survival (n = 795). Almost one out of three patients experienced at least one complication and only 0.9% of the cohort developed all three complications. One hundred and twenty (20%) subjects developed SC, 98 MI (12%) and 80 AD (10%); these complications seem to occur in a mutually exclusive manner. By Kaplan-Meier analysis we estimated that after 20 years from the diagnosis SC, MI and AD occurred in 48%, 42% and 29% of patients, respectively. Furthermore, we showed that some clinical and biological markers are skewed among patients with different complications and that subjects with MI and SC had a worse prognosis than those with AD and all other patients (p < 0.0001). This study reveals the existence of different clinical subsets of chronic lymphocytic leukaemia patients characterised by an increased and different risk for developing specifically MI, SC and AD. Copyright © 2016 Elsevier Ltd. All rights reserved.

  15. A randomized pilot clinical trial of the safety of pioglitazone in treatment of patients with Alzheimer disease.

    PubMed

    Geldmacher, David S; Fritsch, Thomas; McClendon, McKee J; Landreth, Gary

    2011-01-01

    To evaluate the safety of the peroxisome proliferator-activated receptor gamma agonist pioglitazone in nondiabetic patients with Alzheimer disease (AD) and to explore treatment effect sizes on clinical outcomes. Double-blind, placebo-controlled randomized controlled trial of 18-month duration. Two academic medical center outpatient clinics. Nondiabetic patients meeting research criteria for probable AD were enrolled. Twenty-five of 29 subjects completed the study; no withdrawals were attributable to adverse effects. Subjects received pioglitazone (Actos), titrated to 45 mg daily, or matching placebo, and 200 IU of vitamin E daily. Patients maintained treatment with cholinesterase inhibitors and could begin memantine therapy when it became available during the study. The primary outcome was frequency of reported adverse effects (AEs). Secondary outcomes were measures of cognition, activities of daily living, neuropsychiatric symptoms, and global function. Peripheral edema was the principal AE occurring more frequently in subjects taking pioglitazone than placebo (28.6% vs 0%). This is consistent with the known AE profile of pioglitazone. No group differences in laboratory measures were identified. No significant treatment effect was observed on exploratory analysis of clinical efficacy. Pioglitazone was generally well tolerated in this pilot study. There were no serious or unanticipated adverse events or clinical laboratory changes attributable to pioglitazone over a long-term exposure in nondiabetic patients with AD. The tolerability of pioglitazone in this population and peroxisome proliferator-activated receptor gamma effects in laboratory models of AD support further study of this drug class in earlier disease stages. clinicaltrials.gov Identifier: NCT00982202.

  16. [Rivastigmine as treatment for patients with mild to moderately severe Alzheimer disease under normal clinical practice conditions. The ENTERPRISE study].

    PubMed

    Cruz Jentoft, A J; Hernández, B

    2014-01-01

    Alzheimer disease (AD) causes progressive cognitive decline leading to loss of independence for activities of daily living; rivastigmine is one of the drugs used for symptomatic management. To assess the therapeutic use of different pharmaceutical forms of rivastigmine in patients with AD in normal clinical practice. Cross-sectional, observational, multi-centre study conducted on patients with mild to moderate AD treated with rivastigmine in Spanish outpatient clinics specialising in Geriatrics, Psychiatry, and Neurology. Data regarding use of oral (OR) and transdermal (TDR) rivastigmine, compliance (degree of adherence), and caregiver satisfaction with treatment were evaluated. In total, 2252 patients with a mean age of 77.2 years were included; 60.2% were women. AD was moderate to moderately severe in 58.4%. Rivastigmine treatment was started orally in 54.4% of the patients and transdermally in 45.6%; 35.6% of those who started treatment by the OR route switched to TDR. A single dose adjustment was sufficient for 77.5% of patients on TDR treatment vs 11.8% of patients receiving OR treatment. More patients on TDR treatment (80.8% vs. 57.1% on OR treatment) reached the maximum therapeutic dose of rivastigmine and did so in a shorter period of time (51.6 vs 205.8 days). Compliance rates (60.5% vs 47.2%) and caregivers' satisfaction with treatment (89.4% vs 81.9%) were also higher for TDR. In normal clinical practice, using the TDR route of administration improves dose titration and drug compliance, allowing more patients to reach the maximum recommended dose of rivastigmine in a shorter time period. Copyright © 2012 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  17. Relationship Between Severity Classification of Acute Exacerbation of Chronic Obstructive Pulmonary Disease and Clinical Outcomes in Hospitalized Patients

    PubMed Central

    Sanjuán, Pilar; Huerta, Arturo; Nieto-Codesido, Irene; Ferreira-Gonzalez, Lucía; Sibila, Oriol; Restrepo, Marcos I

    2017-01-01

    Background Limited data are available regarding the impact of the potential validation of the Canadian Thoracic Society (CTS) guidelines recommendations in classifying patients with an acute exacerbation of chronic obstructive pulmonary disease (AECOPD) in simple and complex. The aim of the present study was to assess the CTS recommendations regarding risk stratification on clinical outcomes among patients hospitalized with an AECOPD. Methods We developed a retrospective cohort study of patients admitted to one tertiary hospital with a diagnosis of AECOPD. The main clinical outcome was the percentage of treatment failure. Secondary outcomes were 30-day, 90-day, and 1-year readmission and mortality rate, length of stay in hospital, intensive care unit (ICU) admission rate, time to readmission, and time to death. Multivariate analyses were performed using 1-year mortality rate as the dependent measures. Results One hundred forty-three patients composed the final study population, most of them (106 [74.1%)] classified as complex acute exacerbation (C-AE) of COPD. C-AE patients had similar rate of treatment failure compared with simple acute exacerbation (S-AE) of COPD (31.1% vs. 27%; p = 0.63). There were no differences regarding the length of stay in hospital, ICU admission rate, and 30-day, 90-day, and 1-year readmission rate. C-AE patients had faster declined measures on time to death (691.6 ± 430 days vs. 998.1 ± 355 days; p = 0.02). In the multivariate analysis, after adjusting for comorbidity, lung function and previous treatment, C-AE patients had a significant higher mortality at one year (Odds Ratio [OR] = 4.9 (Confidence Interval [CI] 95%: 1.16-21); p = 0.031). Conclusions In hospitalized patients with an AECOPD, CTS classification, according to the presence of risk factors, was not associated with worse short-term clinical outcomes although it is related with long-term mortality.  PMID:28265524

  18. Molecular and clinical delineation of 12 patients with glycogen storage disease type III in Western Turkey.

    PubMed

    Okubo, Minoru; Ucar, Sema Kalkan; Podskarbi, Teodor; Murase, Toshio; Shin, Yoon S; Coker, Mahmut

    2015-01-15

    Glycogen storage disease type III (GSD III; MIM #232400) is an autosomal recessive inherited disorder characterized by fasting hypoglycemia, growth retardation, hepatomegaly, progressive myopathy, and cardiomyopathy. GSD III is caused by deficiency in the glycogen debranching enzyme (gene symbol: AGL). Molecular analyses of AGL have indicated heterogeneity depending on ethnic groups. In Turkey we reported 13 different AGL mutations from GSD III patients in the Eastern region; however, the full spectrum of AGL mutations in Turkish population remains unclear. Here we investigated 12 GSD III patients mostly from Western Turkey. The full coding exons, their relevant exon-intron boundaries, and the 5'- and 3'-flanking regions of the patients' AGL were sequenced. AGL haplotypes were determined. Splicing mutations were characterized by RNA transcript analysis. Twelve different mutations were identified: 7 novel AGL mutations [69-base pair deletion (c.1056_1082+42del69), 21-base par deletion (c.3940_3949+11del21), two small duplications (c.364_365dupCT and c.1497_1500dupAGAG), and 3 splicing mutations (c.1736-11A>G, c.3259+1G>A and c.3588+2T>G)], along with 5 known mutations (c.1019delA, c.958+1G>A, c.4161+5G>A, p.R864X and p.R1218X). Transcripts of splicing mutations (c.1736-11A>G, c.3588+2T>G and c.4161+5G>A) were shown to cause aberrant splicing. AGL haplotype analyses suggested that c.1019delA and c.958+1G>A are founder mutations in Turkish patients, while p.R864X is a recurrent mutation. Our study broadens the spectrum of AGL mutations and demonstrates that mutations in Western Turkey are different from those in the Eastern region. Copyright © 2014 Elsevier B.V. All rights reserved.

  19. Tremor and clinical fluctuation are related to sleep disorders in Chinese patients with Parkinson’s disease

    PubMed Central

    2014-01-01

    Objective To study the relationship between sleep disturbances and symptoms in patients with Parkinson’s disease (PD). Methods The Parkinson’s Disease Sleep Scale-Chinese Version (PDSS-CV) was used to evaluate the sleep disturbances of PD patients in a cross sectional study. The Unified Parkinson’s Disease Rating Scale (UPDRS) parts II-IV, and the Hoehn & Yahr (H&Y) stage were used to determine the level of motor function in PD and the severity of PD. The Spearman correlation and a multiple regression analysis were used to identify the relationship between sleep disturbances and symptoms of PD. The quantities derived from the UPDRS and the H&Y stage and disease duration were compared between groups of patients either with or without sleep disturbances identified by the PDSS. This study was conducted from December 2011 to March 2012 at the First Affiliated Hospital of Sun Yat-sen University, in Guangzhou. Results A total of 136 PD patients were included in this study. The overall total PDSS score in PD patients was 107.58 ± 23.35 points (range: 30–146). There were significant differences in the disease duration, the H&Y stage, and the UPDRS section subscores between groups of patients either with or without sleep disturbances (Kruskal-Wallis Test, p <0.05). There were significant negative correlations between PDSS scores and the UPDRS subscores, the H&Y stage and the disease duration (Spearman correlation, p < 0.05). The multiple regression analysis indicated that sleep disturbances identified by the PDSS were only associated with daily life activity, tremor intensity and clinical fluctuation (R2 = 0.22, F(3,132) = 12.4, p < 0.001). The correlations were also significant when the contribution of the other two factors was excluded using partial correlations. Conclusions The level of daily life activity and the occurrences of tremor and clinical fluctuation are likely to be important factors that lead to PD patients’ sleep disturbances

  20. Non-familial degenerative disease and atrophy of brainstem and cerebellum. Clinical and CT data in 47 patients.

    PubMed

    Staal, A; Meerwaldt, J D; van Dongen, K J; Mulder, P G; Busch, H F

    1990-03-01

    We studied the clinical features of 47 patients with a non-hereditary degenerative disease and with atrophy of brainstem or cerebellum or both in CT scanning. There was no relation between the CT findings and duration or severity of the disease, nor with the kind of the neurological signs which comprised ataxia, a hypokinetic rigid syndrome, oculomotor abnormalities, upper and lower motor neuron signs, orthostatic hypotension and dementia. The 2 main diagnoses were olivopontocerebellar atrophy (OPCA), or a combination of OPCA and striatonigral degeneration (SND). The differential diagnosis with Parkinson's disease and progressive supranuclear palsy was discussed. We concluded, that a CT scan is warranted in all cases of suspected Parkinson's disease, especially in those without tremor, and in cases of motoneuron disease with broad-based gait. In our patients with mainly hypokinesia and rigidity, levodopa treatment had no or brief beneficial effects. If ataxia predominated, OPCA appeared the most sensible diagnosis; if a hypokinetic-rigid syndrome predominated, the diagnoses SND plus OPCA appeared the most suitable. We assessed the degree of atrophy on CT subjectively, because an interobserver study of 60 normal CT scans, did not produce reliable measurements.

  1. The clinical utility of bivalirudin in patients with coronary artery disease.

    PubMed

    Garner, William L; Linden, Julie A; Chrysant, George S

    2013-03-01

    Bivalirudin, a direct thrombin inhibitor, is an anticoagulant commonly used in invasive cardiology procedures. It has evolved from relative obscurity, as an anticoagulation option only utilized in rare instances of allergy or resistance to heparin products, to the now preferred antithrombotic anticoagulant in the cardiac catheterization laboratory. On the way to displacing unfractionated heparin as the preferred anticoagulant for percutaneous coronary intervention, multiple studies comparing bivalirudin with heparin have consistently shown equivalent ischemic efficacy endpoints (i.e. cardiovascular death, myocardial infarction, etc.), with significant reductions in bleeding. Bleeding has been directly linked to worse hospital outcomes in cardiac patient's undergoing invasive coronary artery revascularization procedures. More recent bivalirudin studies now demonstrate reductions in mortality, which has led to a paradigm shift to bivalirudin as the anticoagulant choice both in elective and emergent coronary procedures. We present the major studies that have brought bivalirudin to the forefront of coronary artery disease, specifically coronary interventional procedures.

  2. Physician - nurse practitioner teams in chronic disease management: the impact on costs, clinical effectiveness, and patients' perception of care.

    PubMed

    Litaker, David; Mion, Lorraine; Planavsky, Loretta; Kippes, Christopher; Mehta, Neil; Frolkis, Joseph

    2003-08-01

    Increasing demand to deliver and document therapeutic and preventive care sharpens the need for disease management strategies that accomplish these goals efficiently while preserving quality of care. The purpose of this study was to compare selected outcomes for a new chronic disease management program involving a nurse practitioner - physician team with those of an existing model of care. One hundred fifty-seven patients with hypertension and diabetes mellitus were randomly assigned to their primary care physician and a nurse practitioner or their primary care physician alone. Costs for personnel directly involved in patient management, calculated from hourly rates and encounter time with patients, and pre- and post-study glycosylated hemoglobin (HbA(1c)), high-density lipoprotein cholesterol (HDL-c), satisfaction with care and health-related quality of life (HRQoL) were assessed. Although 1-year costs for personnel were higher in the team-treated group, participants experienced significant improvements in mean HbA(1c) ( - 0.7%, p = 0.02) and HDL-c ( + 2.6 mg dL( - 1), p = 0.02). Additionally, satisfaction with care improved significantly for team-treated subjects in several sub-scales whereas the mean change over time in HRQoL did not differ significantly between groups. This study demonstrates the value of a complementary team approach to chronic disease management in improving patient-derived and clinical outcomes at modest incremental costs.

  3. Vitamin D-mediated calcium absorption in patients with clinically stable Crohn's disease: a pilot study.

    PubMed

    Kumari, Meena; Khazai, Natasha B; Ziegler, Thomas R; Nanes, Mark S; Abrams, Steven A; Tangpricha, Vin

    2010-08-01

    Vitamin D is the critical hormone for intestinal absorption of calcium. Optimal calcium absorption is important for proper mineralization of bone in the prevention of osteoporosis and osteoporotic fractures, among other important functions. Diseases associated with gut inflammation, such as Crohn's disease (CD), may impair calcium absorption. This pilot study evaluated vitamin D- dependent calcium absorption in subjects with CD. Male subjects with CD (n=4) and healthy age-matched controls (n=5) were studied. All subjects had fractional calcium absorption (FCA; by the dual calcium isotope method), serum 25-hydroxyvitamin D, serum calcium and 24 h urinary calcium excretion measurements at baseline. The FCA in response to vitamin D therapy was re-assessed following administration of oral calcitriol 0.25 mcg twice daily for 1 wk, followed by oral calcitriol 0.50 mcg twice daily for 1 wk. Serum calcium and 24 h urinary calcium determinations were re-assessed after each increasing dose of calcitriol as safety measures. There was no significant difference in calcium FCA at baseline or after increasing doses of calcitriol between the CD and controls. FCA in the control and CD group was approximately 35% at baseline, which increased to 60% after calcitriol therapy. No subject developed hypercalcemia or hypercalciuria. Our results suggest that CD patients have a normal response to vitamin D in enhancing the efficacy of calcium absorption. This suggests that stable CD patients can follow calcium and vitamin D guidelines of non-CD adults. Other factors independent of vitamin D status may impair intestinal calcium absorption in CD, including the degree and location of inflammation, presence of surgical resection and/or use of glucocorticoids.

  4. Consumption of medicinal plants by patients with heart diseases at a pharmacist-managed anticoagulation clinic in Brazil.

    PubMed

    Leite, Paula Mendonça; Castilho, Rachel Oliveira; Ribeiro, Antonio Luiz Pinho; Martins, Maria Auxiliadora Parreiras

    2016-04-01

    Medicinal plants (MP) have been used for many years with the purpose of feeding and curing. Several MP may interfere in drug response and are not always considered as potential drug-interactors in clinical practice. To investigate the consumption of MP by outpatients during a one-year follow-up. Patients with cardiopathy diagnosis and indication(s) for long-term use of warfarin were recruited at a pharmacist-managed anticoagulation clinic of a Brazilian public hospital. This research employed a descriptive method. The consumption of MP was examined regarding the type, frequency and forms of use. A total of 280 patients were studied. Most patients were female (54.6 %) with an average age of 56.8 ± 13.1 years. The consumption of MP was reported by 46 (16.4 %) patients, totalizing 59 occurrences. Lemon, lemon balm and plantain were the most common MP. The main pharmacological uses involved the digestive, urinary, and respiratory tracts. Tea was the predominant form of consumption (87 %). Twelve (33.3 %) plants presented potential herb-warfarin interactions according to the literature. We described the consumption of MP among outpatients characterized by their complex disease status, propensity for adverse events, and socioeconomic limitations. These results may guide pharmacist interventions and procedures to prevent clinical complications.

  5. The adrenal medullary transplant operation for Parkinson's disease: clinical observations in five patients.

    PubMed

    Penn, R D; Goetz, C G; Tanner, C M; Klawans, H L; Shannon, K M; Comella, C L; Witt, T R

    1988-06-01

    The recent report by Madrazo and coworkers on the successful treatment of Parkinson's disease using adrenal medullary tissue transplanted to the caudate nucleus has aroused international interest in the procedure. The present article reports our initial experiences with the operation in five patients and discusses the postoperative effects of the procedure, the protocol used to monitor motor performance, and the need for cooperation with the two registries that have been created to follow morbidity, mortality, and efficacy. We intend to alert the neurosurgeon to important side effects, but not to assess the long term efficacy of the procedure. Postoperatively, a number of transient effects were seen in our patients, the most striking being somnolence, delusions, and lack of significant pain in spite of a large abdominal incision. The only complications have been respiratory. After the early postoperative period, gradual improvement of on-off times and Schwab-England disability scores was seen over 20 weeks. Long term cooperative studies are needed to demonstrate the efficacy of this procedure. neurosurgeons doing transplant operations are urged to join the registries so that uniform information can be collected.

  6. Clinical management of chronic obstructive pulmonary disease patients with muscle dysfunction.

    PubMed

    Gea, Joaquim; Casadevall, Carme; Pascual, Sergi; Orozco-Levi, Mauricio; Barreiro, Esther

    2016-11-01

    Muscle dysfunction is frequently observed in chronic obstructive pulmonary disease (COPD) patients, contributing to their exercise limitation and a worsening prognosis. The main factor leading to limb muscle dysfunction is deconditioning, whereas respiratory muscle dysfunction is mostly the result of pulmonary hyperinflation. However, both limb and respiratory muscles are also influenced by other negative factors, including smoking, systemic inflammation, nutritional abnormalities, exacerbations and some drugs. Limb muscle weakness is generally diagnosed through voluntary isometric maneuvers such as handgrip or quadriceps muscle contraction (dynamometry); while respiratory muscle loss of strength is usually recognized through a decrease in maximal static pressures measured at the mouth. Both types of measurements have validated reference values. Respiratory muscle strength can also be evaluated determining esophageal, gastric and transdiaphragmatic maximal pressures although there is a lack of widely accepted reference equations. Non-volitional maneuvers, obtained through electrical or magnetic stimulation, can be employed in patients unable to cooperate. Muscle endurance can also be assessed, generally using repeated submaximal maneuvers until exhaustion, but no validated reference values are available yet. The treatment of muscle dysfunction is multidimensional and includes improvement in lifestyle habits (smoking abstinence, healthy diet and a good level of physical activity, preferably outside), nutritional measures (diet supplements and occasionally, anabolic drugs), and different modalities of general and muscle training.

  7. Clinical management of chronic obstructive pulmonary disease patients with muscle dysfunction

    PubMed Central

    Casadevall, Carme; Pascual, Sergi; Orozco-Levi, Mauricio; Barreiro, Esther

    2016-01-01

    Muscle dysfunction is frequently observed in chronic obstructive pulmonary disease (COPD) patients, contributing to their exercise limitation and a worsening prognosis. The main factor leading to limb muscle dysfunction is deconditioning, whereas respiratory muscle dysfunction is mostly the result of pulmonary hyperinflation. However, both limb and respiratory muscles are also influenced by other negative factors, including smoking, systemic inflammation, nutritional abnormalities, exacerbations and some drugs. Limb muscle weakness is generally diagnosed through voluntary isometric maneuvers such as handgrip or quadriceps muscle contraction (dynamometry); while respiratory muscle loss of strength is usually recognized through a decrease in maximal static pressures measured at the mouth. Both types of measurements have validated reference values. Respiratory muscle strength can also be evaluated determining esophageal, gastric and transdiaphragmatic maximal pressures although there is a lack of widely accepted reference equations. Non-volitional maneuvers, obtained through electrical or magnetic stimulation, can be employed in patients unable to cooperate. Muscle endurance can also be assessed, generally using repeated submaximal maneuvers until exhaustion, but no validated reference values are available yet. The treatment of muscle dysfunction is multidimensional and includes improvement in lifestyle habits (smoking abstinence, healthy diet and a good level of physical activity, preferably outside), nutritional measures (diet supplements and occasionally, anabolic drugs), and different modalities of general and muscle training. PMID:28066619

  8. Effect of oral eliglustat on splenomegaly in patients with Gaucher disease type 1: the ENGAGE randomized clinical trial.

    PubMed

    Mistry, Pramod K; Lukina, Elena; Ben Turkia, Hadhami; Amato, Dominick; Baris, Hagit; Dasouki, Majed; Ghosn, Marwan; Mehta, Atul; Packman, Seymour; Pastores, Gregory; Petakov, Milan; Assouline, Sarit; Balwani, Manisha; Danda, Sumita; Hadjiev, Evgueniy; Ortega, Andres; Shankar, Suma; Solano, Maria Helena; Ross, Leorah; Angell, Jennifer; Peterschmitt, M Judith

    2015-02-17

    Gaucher disease type 1 is characterized by hepatosplenomegaly, anemia, thrombocytopenia, and skeletal disease. A safe, effective oral therapy is needed. To determine whether eliglustat, a novel oral substrate reduction therapy, safely reverses clinical manifestations in untreated adults with Gaucher disease type 1. Phase 3, randomized, double-blind, placebo-controlled trial conducted at 18 sites in 12 countries from November 2009 to July 2012 among eligible patients with splenomegaly plus thrombocytopenia and/or anemia. Of 72 patients screened, 40 were enrolled. Patients were stratified by spleen volume and randomized 1:1 to receive eliglustat (50 or 100 mg twice daily; n = 20) or placebo (n = 20) for 9 months. The primary efficacy end point was percentage change in spleen volume in multiples of normal from baseline to 9 months; secondary efficacy end points were change in hemoglobin level and percentage changes in liver volume and platelet count. All patients had baseline splenomegaly and thrombocytopenia (mostly moderate or severe), most had mild or moderate hepatomegaly, and 20% had mild anemia. Least-square mean spleen volume decreased by 27.77% (95% CI, -32.57% to -22.97%) in the eliglustat group (from 13.89 to 10.17 multiples of normal) vs an increase of 2.26% (95% CI, -2.54% to 7.06%) in the placebo group (from 12.50 to 12.84 multiples of normal) for an absolute treatment difference of -30.03% (95% CI, -36.82% to -23.24%; P < .001). For the secondary end points, the least-square mean absolute differences between groups all favored eliglustat, with a 1.22-g/dL increase in hemoglobin level (95% CI, 0.57-1.88 g/dL; P < .001), 6.64% decrease in liver volume (95% CI, -11.37% to -1.91%; P = .007), and 41.06% increase in platelet count (95% CI, 23.95%-58.17%; P < .001). No serious adverse events occurred. One patient in the eliglustat group withdrew (non-treatment related); 39 of the 40 patients transitioned to an open-label extension study

  9. Thyroid stimulating hormone suppression post-therapy in patients with Graves' disease: a systematic review of pathophysiology and clinical data.

    PubMed

    Yu, Huan; Farahani, Pendar

    2015-04-08

    Post-treatment hypothyroidism is common in Graves' disease, and clinical guidelines recommend monitoring for it; however, thyroid stimulating hormone (TSH) can remain suppressed in these patients following treatment. The objectives of this study were to explore the proposed pathophysiology behind the phenomenon of post-therapy TSH suppression and to systematically review existing clinical data on post-therapy TSH suppression in patients with Graves' disease. A systematic literature search was performed using EMBASE and PubMed databases, with several combinations of MeSH terms. Bibliography mining was also done on relevant articles to be as inclusive as possible. A total of 18 articles described possible mechanisms for post-therapy TSH suppression. Several of the studies demonstrate evidence of thyrotroph atrophy and hypothesize that this contributes to the ongoing suppression. TSH receptors have been identified in folliculo-stellate cells of the pituitary as well as astroglial cells of the hypothalamus, mediating paracrine feedback. A few studies have demonstrated inverse correlation between autoantibody titres and TSH levels, suggestive of their role in mediating ongoing TSH suppression in patients with Graves' disease. In addition, five studies were identified that provided clinical data on the duration of TSH suppression. Combined data show that 45.5% of patients recover TSH by 3 months after treatment, increasing to 69.3% by 6 months, and plateauing to 73.8% by 12 months (p>0.0001). Sub-analysis also shows that for patients who are TBII negative, 80.7% recover their TSH by 6 months compared with only 58.7% in those who are TBII positive (p= 0.003). Clinical data suggests that TSH recovery is most likely to occur within the first 6 months after treatment, with recovery plateauing at approximately 70% of patients, suggesting that reliance on this assay for monitoring can be very misleading. Furthermore, TBII positivity is associated with lower likelihood of TSH

  10. Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience

    PubMed Central

    Seo, Seyoung; Yoo, Changhoon; Yoon, Dok Hyun; Kim, Shin; Park, Jung Sun; Park, Chan-Sik; Huh, Jooryung

    2014-01-01

    Background Multicentric Castleman's disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. Methods Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis. Results Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival. Conclusion Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors. PMID:25548759

  11. Efficacy of adding sodium alginate to omeprazole in patients with nonerosive reflux disease: a randomized clinical trial.

    PubMed

    Manabe, N; Haruma, K; Ito, M; Takahashi, N; Takasugi, H; Wada, Y; Nakata, H; Katoh, T; Miyamoto, M; Tanaka, S

    2012-07-01

    Nonerosive reflux disease (NERD) is the most common form of gastroesophageal reflux disease. Patients with NERD have a lower response rate to proton pump inhibitors (PPIs) than patients with erosive esophagitis when gauged from relief of heartburn. Sodium alginate decreases the acidity of refluxate and protects the esophageal mucosa. However, whether the addition of sodium alginate to PPI therapy can improve NERD symptoms remains unknown. Accordingly, the aim of this study was to evaluate the efficacy of adding sodium alginate to basal PPI therapy for NERD. Patients who had experienced heartburn on at least 2 days per week during the 1-month period before entering the study and had no endoscopic mucosal breaks (grade M or N according to Hoshihara's modification of the Los Angeles classification) were randomized to one of two treatments for 4 weeks: omeprazole (20 mg once daily) plus sodium alginate (30 mL four times a day) (group A) or omeprazole (20 mg once daily) alone (group B). Eighty-seven patients were enrolled, and 76 patients were randomly assigned to group A (n = 36) or group B (n = 40). Complete resolution of heartburn for at least 7 consecutive days by the end of treatment was significantly more common in group A (56.7%) than in group B (25.7%). One patient from group A had mild drug-related diarrhea that was not clinically serious. In conclusion, omeprazole combined with sodium alginate was better than omeprazole alone in Japanese patients with NERD. © 2011 Copyright the Authors. Journal compilation © 2011, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

  12. Clinical outcomes and resource utilisation in Medicare patients with chronic liver disease: a historical cohort study.

    PubMed

    Younossi, Zobair M; Zheng, Li; Stepanova, Maria; Venkatesan, Chapy; Mishra, Alita

    2014-05-16

    The aim of this study is to assess recent trends in health resource utilisation and patient outcomes of Medicare beneficiaries with chronic liver disease (CLD). Liver-related mortality is the 10th leading cause of death in the USA, and hepatitis C virus (HCV) and obesity-related non-alcoholic fatty liver disease are the major causes of CLD. As the US population ages and becomes more obese, the impact of CLD is expected to become more prominent for the Medicare population. This is a retrospective cohort study of Medicare beneficiaries with a diagnosis of CLD based on inpatient (N=21 576; 14 977 unique patients) and outpatient (N=515 990; 244 196 patients) claims from 2005 to 2010. The study outcomes included hospital length of stay (LOS) and inpatient mortality as well as inpatient and outpatient inflation-adjusted payments. Between 2005 and 2010, there was an annual decrease in LOS of 3.17% for CLD-related hospitalisations. Risk-adjusted in-hospital mortality decreased (OR 0.90, 95% CI 0.87 to 0.94), while short-term postdischarge mortality remained stable (1.00, 0.98 to 1.03). Inpatient per-claim payment increased from $11 769 in 2005 to $12 347 in 2010 (p=0.0006). Similarly, the average yearly payments for outpatient care increased from $366 to $404 (p<0.0001). This change in payment was observed together with a consistent decrease in the proportion of beneficiary-paid amount (25.4-20%, p<0.0001) as opposed to Medicare-paid amount (73.1-80%, p<0.0001). The major predictors of higher outpatient payments were younger age, Asian race or Hispanic ethnicity, living in California, and having more diagnoses and outpatient procedures per claim. The predictors of inpatient spending also included younger age, location and the number of inpatient procedures. Length of inpatient stay and inpatient mortality among Medicare beneficiaries with CLD decreased, while inpatient and outpatient spending increased. Published by the BMJ Publishing Group Limited. For

  13. Multimodality imaging and clinical features in Castleman disease: single institute experience in 30 patients

    PubMed Central

    Tirumani, S H; Rosenthal, M H; Shinagare, A B; Carrasco, R D; Munshi, N C; Ramaiya, N H; Howard, S A

    2015-01-01

    Objective: To analyse imaging features of subtypes of Castleman disease (CD), emphasizing differentiating features from lymphoma. Methods: Institutional review board-approved, Health Insurance Portability and Accountability Act compliant, retrospective study examined 30 patients with CD. 30 patients (females, 20; mean age, 46 years; range, 22–87 years) with histopathologically confirmed CD and pre-treatment imaging formed the analytic cohort. Imaging at presentation in all patients [CT, 30; positron emission tomography (PET)/CT, 5; MR, 4; ultrasound, 3] and subsequent imaging in three cases that developed lymphoma was reviewed by two radiologists in consensus. Results: Subtypes: hyaline-vascular (n = 18); multicentric not otherwise specified (NOS) (n = 6); human herpesvirus 8 associated (n = 2); mixed unicentric (n = 2); pure plasma-cell variant (n = 1); and unicentric NOS (n = 1). Distribution: unicentric (n = 17); and multicentric (n = 13). Nodal sites—unicentric: 13 thoracic, 3 abdominal and 1 cervical; multicentric: 9 abdominal, 8 thoracic, 6 cervical, 5 inguinal, 4 axillary and 4 supraclavicular. On CT, differentiating features from lymphoma were calcification (n = 8; 26.7%) and heterogeneous enhancement (n = 5; 19.2%). No association between CD subtype, degree or enhancement pattern, or calcification was noted. On PET/CT (n = 5), nodes were typically fluorine-18 fludeoxyglucose avid (n = 4). On ultrasound (n = 3), nodes were hypoechoic, homogeneous with posterior acoustic enhancement. On MR (n = 4), nodes were hypointense (n = 2) to isointense (n = 2) on T1 weighted images and isointense (n = 1) to hyperintense (n = 3) on T2 weighted images. All (n = 4) demonstrated homogeneous enhancement. Three cases developed non-Hodgkin's lymphoma, two of the three had larger spleens, and these cases had effusions/ascites. Conclusion: CD can be unicentric or multicentric and involve nodes

  14. The clinical usefulness of a web-based messaging system between patients with Crohn disease and their physicians.

    PubMed

    Jeong, Da Eun; Kim, Kyeong Ok; Jang, Byung Ik; Kim, Eun Young; Jung, Jin Tae; Jeon, Seong Woo; Lee, Hyun Seok; Kim, Eun Soo; Park, Kyung Sik; Cho, Kwang Bum

    2016-06-01

    To avoid missing events associated with clinical activity, the authors previously developed a novel, web-based, self-reporting Crohn disease (CD) symptom diary. However, although this diary provided a means of self-checking based on responses to set questions based on Harvey-Bradshaw index scores, it was limited in terms of describing other specific symptoms. Thus, the authors added a space to the questionnaire, which allows patients to send clinicians questions or a description of unpredictable events. The aim of the present study was to assess the clinical usefulness of this messaging system by analyzing patients' messages.The messaging system between patients and their doctors was included in a webpage created for recording patients' symptom diaries (www.cdsd.or.kr). Using this system, patients can send messages easily at any time and doctors can read and respond to these messages immediately using a smart phone or computer. In the present study, the authors retrospectively reviewed 686 messages sent by 152 patients from July 2012 to July 2014 and patient medical records.Mean patient age was 29.0 ± 11.6 years and the male-to-female ratio was 99:53. Most messages regarded symptoms (381 messages, 55.5%), which was followed by self-reports about general condition (195 messages, 28.4%) and questions about treatment (71 messages, 10.3%). With respect to symptoms, abdominal pain was most common (145 cases, 21.1%) followed by hematochezia (36 cases, 5.2%). Problems about medication were the most frequently associated with treatment (65, 91.5%). Patients above 40 years showed a greater tendency to focus on symptoms and treatment (P = 0.025). The doctor answer rate was 56.3% (n = 386), and based on these responses, an early visit was needed in 28 cases (7.3%).Using this web-based messaging system, patients were able to obtain proper advice from their physicians without visiting clinics or searching the Internet, and in addition, 7.3% of messages prompted an early

  15. American Society of Clinical Oncology provisional clinical opinion: chronic hepatitis B virus infection screening in patients receiving cytotoxic chemotherapy for treatment of malignant diseases.

    PubMed

    Artz, Andrew S; Somerfield, Mark R; Feld, Jordan J; Giusti, Andrew F; Kramer, Barnett S; Sabichi, Anita L; Zon, Robin T; Wong, Sandra L

    2010-07-01

    PURPOSE An American Society of Clinical Oncology (ASCO) provisional clinical opinion (PCO) offers timely clinical direction to ASCO's membership following publication or presentation of potentially practice-changing information. This PCO addresses recommendations for chronic hepatitis B virus (HBV) infection screening in patients receiving cytotoxic or immunosuppressive chemotherapy for treatment of malignant diseases. The Centers for Disease Control and Prevention (CDC) issued Recommendations for Identification and Public Health Management of Persons with Chronic Hepatitis B Virus Infection, recommending screening for hepatitis B infection (hepatitis B surface antigen [HBsAg], antihepatitis B core antigen [anti-HBc], and antibodies to HBsAg [anti-HBs]) for "persons receiving cytotoxic or immunosuppressive therapy (eg, chemotherapy for malignant diseases...)." The evidence is insufficient to determine the net benefits and harms of routine screening for chronic HBV infection in individuals with cancer who are about to receive cytotoxic or immunosuppressive therapy or who are already receiving therapy. Individuals with cancer who undergo certain cytotoxic or immunosuppressive therapies and have HBV infection or prior exposure to HBV may be at elevated risk of liver failure from HBV reactivation. As such, HBV screening requires clinical judgment. Physicians may consider screening patients belonging to groups at heightened risk for chronic HBV infection or if highly immunosuppressive therapy is planned. Highly immunosuppressive treatments include, but are not limited to, hematopoietic cell transplantation and regimens including rituximab. Screening based on a high risk of prior HBV exposure or risk of reactivation due to planned therapeutic regimens should include testing for HBsAg as a serologic marker for HBV infection. In some populations, testing for anti-HBc should also be considered. There is no evidence to support serologic testing for anti-HBs in this context

  16. Factors predicting clinical nurses' willingness to care for Ebola virus disease-infected patients: A cross-sectional, descriptive survey.

    PubMed

    Kim, Ji Soo; Choi, Jeong Sil

    2016-09-01

    The purpose of this study was to identify factors predicting clinical nurses' willingness to care for Ebola virus disease (EVD)-infected patients. Data were collected from 179 nurses employed at 10 hospitals in Korea using self-reporting questionnaires. Only 26.8% of the participants were willing to care for EVD-infected patients. Factors predicting their willingness to provide care were their belief in public service, risk perception, and age. Nurses' willingness to provide care was high when their belief in public service was high, low when their risk perception was high, and low as their age increased. In order to strengthen nurses' willingness to care for EVD-infected patients, education that targets the enhancement of belief in public service should be included in nurse training. Efforts should be directed toward lowering EVD risk perception and developing systematic responses through government-led organized support.

  17. Clinical utility of PKD2 mutation testing in a polycystic kidney disease cohort attending a specialist nephrology out-patient clinic

    PubMed Central

    2012-01-01

    Background ADPKD affects approximately 1:1000 of the worldwide population. It is caused by mutations in two genes, PKD1 and PKD2. Although allelic variation has some influence on disease severity, genic effects are strong, with PKD2 mutations predicting later onset of ESRF by up to 20 years. We therefore screened a cohort of ADPKD patients attending a nephrology out-patient clinic for PKD2 mutations, to identify factors that can be used to offer targeted gene testing and to provide patients with improved prognostic information. Methods 142 consecutive individuals presenting to a hospital nephrology out-patient service with a diagnosis of ADPKD and CKD stage 4 or less were screened for mutations in PKD2, following clinical evaluation and provision of a detailed family history (FH). Results PKD2 mutations were identified in one fifth of cases. 12% of non-PKD2 patients progressed to ESRF during this study whilst none with a PKD2 mutation did (median 38.5 months of follow-up, range 16–88 months, p < 0.03). A significant difference was found in age at ESRF of affected family members (non-PKD2 vs. PKD2, 54 yrs vs. 65 yrs; p < 0.0001). No PKD2 mutations were identified in patients with a FH of ESRF occurring before age 50 yrs, whereas a PKD2 mutation was predicted by a positive FH without ESRF. Conclusions PKD2 testing has a clinically significant detection rate in the pre-ESRF population. It did not accurately distinguish those individuals with milder renal disease defined by stage of CKD but did identify a group less likely to progress to ESRF. When used with detailed FH, it offers useful prognostic information for individuals and their families. It can therefore be offered to all but those whose relatives have developed ESRF before age 50. PMID:22863349

  18. A cross-sectional study of clinical management, and provision of health services and their utilisation, by patients with Parkinson's disease in urban and regional Victoria.

    PubMed

    Lubomski, Michal; Rushworth, R Louise; Lee, Will; Bertram, Kelly; Williams, David R

    2013-01-01

    Our objective was to evaluate and compare clinical management, utilisation of health services and quality of life (QoL) in patients with Parkinson's disease (PD) attending clinics in urban and regional Victoria. A cross-sectional survey was conducted on 210 patients with PD attending specialist neurological clinics in a regional area (Ballarat) (n=97), and an urban area (Melbourne) (n=113), Victoria. Demographic characteristics of patients with PD, QoL, patterns of disease and management and utilisation of medical and allied health services were analysed. Compared to patients with PD from urban clinics, patients in the regional clinic were significantly older and were diagnosed at a later age with a shorter duration of treatment (all p<0.05). Despite no significant difference in disease severity (measured by Unified Parkinson's Disease Rating Scale scores) between the groups, patients in the urban clinic reported a lower QoL (p=0.003). Patients in the regional clinic were more satisfied with their treatment, despite seeing their medical specialist less frequently (p<0.001) and having a higher rate of early misdiagnosis (p=0.015). Patients from regional clinics reported a poorer understanding of their illness than patients in the urban clinic (p=0.049). Half of all respondents were interested in using telemedicine services. Two-thirds (71%) of all patients used allied health services, with patients in the urban clinic utilising more and desiring greater access to these services (p<0.05). In conclusion, we found significant differences in the presentation, management and use of health services between patients accessing regional and urban PD clinics in Victoria. Telemedicine may be an effective, and even desirable, method for facilitating improved diagnosis and referral for appropriate therapies.

  19. Toward comprehensive management tailored to prognostic factors of patients with clinical stages I and II in Hodgkin's disease. The EORTC Lymphoma Group controlled clinical trials: 1964-1987.

    PubMed

    Tubiana, M; Henry-Amar, M; Carde, P; Burgers, J M; Hayat, M; Van der Schueren, E; Noordijk, E M; Tanguy, A; Meerwaldt, J H; Thomas, J

    1989-01-01

    From 1964 to 1987, the EORTC Lymphoma Group conducted four consecutive controlled clinical trials on clinical stages I and II Hodgkin's disease in which 1,579 patients were entered. From the onset the main aim of these trials was to identify the subsets of patients who could be treated safely by regional radiotherapy (RT). Therefore, several prognostic indicators were prospectively registered and progressively used in the trial protocols for the delineation of the favorable and unfavorable subgroups as soon as they were recognized of high predictive value. In the H2 trial (1972 to 1976), the histologic subtype was the only variable taken into account for the therapeutic strategy and the staging laparotomy findings were found to be of prognostic value only in patients with favorable prognostic indicators. In the H5 trial (1977 to 1982), patients were subdivided into two subgroups according to six prognostic indicators. Patients with favorable features were submitted to a staging laparotomy (lap); lap negative patients were randomized between mantle field RT and mantle field plus paraaortic RT. Disease free survival (DFS) and total survival (S) were similar in the two arms. Among patients with unfavorable features, DFS and S were significantly higher in the arm treated by combination of mechlorethamine, vincristine, procarbazine, prednisone (MOPP) chemotherapy (CT) and RT than in the arm treated by total nodal irradiation. Nevertheless, in patients below the age of 40, the overall survival rates were equivalent in the two arms. In the H6 trial, the delineation of the favorable subgroup was based on (a) absence of systemic symptoms and elevated ESR, (b) no more than one or two lymph node areas involved. The aim of the study was to assess the impact on survival of a therapeutic strategy including staging laparotomy. At a 4-year follow-up, no difference in survival was evidenced. In patients with unfavorable prognostic indicators, 3 MOPP-RT-3 MOPP were compared with 3

  20. [Investigation of the clinical course and treatment of prion disease patients in the akinetic mutism state in Japan].

    PubMed

    Iwasaki, Yasushi; Mori, Keiko; Ito, Masumi

    2012-01-01

    Twelve cases (one Gerstmann-Sträussler-Scheinker syndrome (P102L; definite), one genetic Creutzfeldt-Jakob disease (CJD) (V180I; definite) and ten sporadic CJD (7 MM1-type definite, 3 probable)), who reached the akinetic mutism state, were investigated with regard to their clinical course and treatment. They were hospitalized for a total of 3,968 days in the akinetic mutism state. In the nine definite cases, the median period from the akinetic mutism state to death was 22 months (average: 27.0 ± 23.3 months, range: 3-80 months) and median total disease duration was 27 months (average: 34.2 ± 30.1 months, range: 5-102 months). In the seven definite sporadic CJD cases, the median period from akinetic mutism to death was 21 months (average: 17.0 ± 9.6 months, range 3-28 months), and median total disease duration was 24 months (average: 20.6 ± 10.0 months, range: 5-31 months). Nasal-tube feeding was performed in all cases. Symptomatic treatments such as parenteral nutrition and antibiotic drugs were administered for complications such as respitory and urinary tract infections and digestive symptoms. Patients received rehabilitation and hot spring therapy regularly until death. Gastrostomy and/or tracheotomy was not performed in any case, the patients were not intubated nor was mechanical ventilation (including non-invasive positive pressure ventilation) applied. Vasoactive drugs were not administered. Clonazepam was administered for myoclonus in four patients but not in another three when myoclonus appeared. It is unclear whether the treatment influenced the duration of myoclonus. Our observations indicate that the extended survival period among Japanese prion disease patients is likely due to the management procedures implemented for prion disease in Japan, which are usually continued after the patients reach the akinetic mutism state. We speculate that nasal-tube feeding is the crucial factor that results in the prolonged disease duration of prion disease

  1. Platelet surface expression of SDF-1 is associated with clinical outcomes in the patients with cardiovascular disease.

    PubMed

    Rath, Dominik; Chatterjee, Madhumita; Bongartz, Angela; Müller, Karin; Droppa, Michal; Stimpfle, Fabian; Borst, Oliver; Zuern, Christine; Vogel, Sebastian; Gawaz, Meinrad; Geisler, Tobias

    2017-01-01

    Platelet surface expression levels of stromal cell derived factor 1 (SDF-1) are elevated in acute coronary syndrome and associated with LVEF% improvement after myocardial infarction (MI). Platelet SDF-1 might facilitate thrombus formation and endomyocardial expression of SDF-1 is enhanced in inflammatory cardiomyopathy and positively correlates with myocardial fibrosis. The influence of platelet SDF-1 on outcome in the patients with symptomatic coronary artery disease (CAD) is to the best of our knowledge unknown. Blood samples of 608 consecutive CAD patients were collected during the percutaneous coronary intervention and analyzed for surface expression of SDF-1 by flow cytometry. The primary combined endpoint was defined as the composite of either MI, or ischemic stroke, or all-cause death. Secondary endpoints were defined as the aforementioned single events. The patients with baseline platelet SDF-1 levels above the third quartile showed a significantly worse cumulative event-free survival when compared to the patients with lower baseline SDF-1 levels (first to third quartile) (log rank 0.009 for primary combined endpoint and log rank 0.016 for secondary endpoint all-cause death). Multivariate Cox regression analysis showed that SDF-1 levels above the third quartile were independently associated with the primary combined endpoint and the secondary endpoint all-cause death. We provide first clinical evidence that high platelet expression levels of SDF-1 influence clinical outcomes in CAD patients in a negative way.

  2. Transcatheter Arterial Embolization in Patients with Kidney Diseases: an Overview of the Technical Aspects and Clinical Indications

    PubMed Central

    Rao, Pramod; Kwak, Byung-Kook; Ota, Shinichi; De Lin, Ming; Liapi, Eleni; Geschwind, Jean-François

    2010-01-01

    Therapeutic embolization is defined as the voluntary occlusion of one or several vessels, and this is achieved by inserting material into the lumen to obtain transient or permanent thrombosis in the downstream vascular bed. There are a number of indications for this approach in urological practice, in particular for the patients with parenchymatous or vascular kidney disease. In this review, we present the different embolization techniques and the principally employed occluding agents, and then we present the principal clinical indications and we discuss other pathologies that may benefit from this non-invasive therapy. The complications, side effects and main precautions associated with this approach are also described. PMID:20461179

  3. Clinical applications of radionuclide imaging in the evaluation and management of patients with congenital heart disease.

    PubMed

    Partington, Sara L; Valente, Anne Marie; Landzberg, Michael; Grant, Frederick; Di Carli, Marcelo F; Dorbala, Sharmila

    2016-02-01

    Non-invasive testing of children with congenital heart disease (CHD) began in the 1950s with the introduction of radionuclide studies to assess shunt fractions, pulmonary blood flow, and ventricular contractile function. Echocardiography and cardiac magnetic resonance imaging have since replaced radionuclide imaging in many of these roles. Concurrently, percutaneous and surgical repairs of complex CHD evolved, creating new roles for radionuclide imaging. In this paper on applications of radionuclide imaging in CHD, we review the multiple mechanisms for myocardial ischemia in CHD. We critically compare optimal radionuclide imaging techniques to other imaging modalities for assessing ischemia in CHD. We present the current role of nuclear imaging for assessing viability and pulmonary blood flow. We highlight the value added by advances in dedicated cardiac SPECT scanners, novel reconstruction software, and cardiac PET in performing low-dose radionuclide imaging in CHD. Finally, we discuss the emerging clinical indications for radionuclide imaging in CHD including coronary flow reserve assessment and evaluation of cardiovascular prosthesis and device infections.

  4. Prediction of individual clinical scores in patients with Parkinson's disease using resting-state functional magnetic resonance imaging.

    PubMed

    Hou, YanBing; Luo, ChunYan; Yang, Jing; Ou, RuWei; Song, Wei; Wei, QianQian; Cao, Bei; Zhao, Bi; Wu, Ying; Shang, Hui-Fang; Gong, QiYong

    2016-07-15

    Neuroimaging holds the promise that it may one day aid the clinical assessment. However, the vast majority of studies using resting-state functional magnetic resonance imaging (fMRI) have reported average differences between Parkinson's disease (PD) patients and healthy controls, which do not permit inferences at the level of individuals. This study was to develop a model for the prediction of PD illness severity ratings from individual fMRI brain scan. The resting-state fMRI scans were obtained from 84 patients with PD and the Unified Parkinson's Disease Rating Scale-III (UPDRS-III) scores were obtained before scanning. The RVR method was used to predict clinical scores (UPDRS-III) from fMRI scans. The application of RVR to whole-brain resting-state fMRI data allowed prediction of UPDRS-III scores with statistically significant accuracy (correlation=0.35, P-value=0.001; mean sum of squares=222.17, P-value=0.002). This prediction was informed strongly by negative weight areas including prefrontal lobe and medial occipital lobe, and positive weight areas including medial parietal lobe. It was suggested that fMRI scans contained sufficient information about neurobiological change in patients with PD to permit accurate prediction about illness severity, on an individual subject basis. Our results provided preliminary evidence, as proof-of-concept, to support that fMRI might be possible to be a clinically useful quantitative assessment aid in PD at individual level. This may enable clinicians to target those uncooperative patients and machines to replace human for a more efficient use of health care resources. Copyright © 2016 Elsevier B.V. All rights reserved.

  5. Effects of doxycycline on clinical, microbiological and immunological parameters in well-controlled diabetes type-2 patients with periodontal disease: a randomized, controlled clinical trial.

    PubMed

    Tsalikis, Lazaros; Sakellari, Dimitra; Dagalis, Panagiotis; Boura, Panagiota; Konstantinidis, Antonios

    2014-10-01

    To evaluate the clinical, microbiological and immunological effects of systemic doxycycline as an adjunct to scaling and root planing (SRP) in chronic periodontitis patients with well-controlled type 2 diabetes. Sixty-six patients compliant to oral hygiene (Hygiene Index <20%) allocated to either a test (systemic doxycycline for 21 days) or a control (placebo) group participated in the present randomized controlled trial (RCT). Clinical assessments were recorded at baseline, 3 and 6 months after therapy and included clinical attachment level (CAL), set as the primary outcome of the study, probing pocket depth (PPD), recession (RE) and bleeding on probing (BOP). At the same time points, counts of 15 subgingival species were evalua