Active clinical trials for "Fibrosis"

The purpose of the present study is to evaluate the characteristics, management and clinical course of patients with IPF as treated under real-world in Italian Pulmonary Centres, in terms of symptoms, lung function and exercise tolerance during 12 months of observation.

Investigators will examine temporal and regional dynamic changes in the microbiome of Cystic Fibrosis patients to explore microbiome features that are associated with an inflammatory phenotype. Investigators hypothesize that temporal and spatial differences in lung microbiome are associated with host inflammatory responses. While chronic and polymicrobial airway colonization are commonly recognized in cystic fibrosis (CF), it is unclear what factors of the microbial environment lead to infection with pathogenic microorganism. This is a multi center, longitudinal cohort of adult Cystic Fibrosis subjects recruit4ed from NYU and Columbia to understand how changes in the airway microbiome may affect the host inflammatory responses in Cystic Fibrosis (CF). There will be three approaches to understanding inflammatory responses; 1) a longitudinal assessment of temporal changes in the microbiome over a 6-month period of clinical stability; 2) comparison of the regional differences in airway microbiome between lung segments with more versus less disease; 3) evaluation of functional aspects of the lung microbiome.

Liver biopsy is the reference method for the measurement of liver fibrosis. But it has many limitations, such as sampling error and individual variation in interpreting the results. Currently, serum liver fibrosis markers have been employed as non-invasive diagnosis of liver fibrosis and evaluation of the severity of liver fibrosis. They include laminin (LN), hyaluronic acid (HA), collagen type IV (CIV), and N-terminal propeptide of collagen III (PIIINP). However, few study was conducted to explore the role of these liver fibrosis markers in evaluating the prognosis of liver cirrhosis. Our hypothesis is that LN, HA, CIV, and PIIINP in combination or alone can predict the prognosis of liver cirrhosis.

Pulmonary transplantation is the reference treatment for chronic terminal respiratory failure in patients with cystic fibrosis. These are mainly bi-pulmonary transplants (cardiopulmonary transplants are exceptional). The annual number of pulmonary transplants in France for cystic fibrosis is about 90. In 2013, the transplant involves a total of more than 600 patients with cystic fibrosis. The average age at the time of the transplant is 28.5 years (2013 data, French cystic fibrosis register), compared to 58 years for patients transplanted to all pathologies. Cystic fibrosis accounts for 25% of adult bi-pulmonary grafts. Pediatric transplants are currently very rare. The median survival after pulmonary transplantation in cystic fibrosis is currently 8.5 years (and 10 years when considering patients surviving 3 months, ie excluding early mortality). Cystic fibrosis is the pathology associated with better survival after pulmonary transplantation given the young age of patients (28.5 years on average). The non-respiratory comorbidities associated with transplantation, all underlying pathologies combined, and referenced in the Registry of the International Society for Heart and Lung Transplantation (ISHLT) are: hypertension, diabetes, renal insufficiency, Dyslipidemia, cancers. Their frequency increases with the survival time of transplanted patients. Cystic fibrosis is associated with non-respiratory comorbidities, the frequency of which increases with age - diabetes, osteoporosis, renal insufficiency, hepatopathy, neoplastic pathologies - and may become worse after transplantation. The main objective is to estimate the incidence of non-respiratory co-morbidities after lung transplantation in the cohort of patients with cystic fibrosis grafted in the Rhône-Alpes region.

Descriptive prospective non-interventional multicenter study based on newly collected data of Idiopathic Pulmonary Fibrosis patients followed-up for one year in secondary care settings (Pulmonology Services)

A single-center randomized controlled study comparing endoscopic or interventional therapy guided by the hepatic venous pressure gradient (HVPG) , to standard endosopic variceal ligation plus nonselective beta-blocker therapy (NSBB) in patients with esophageal varices due to liver cirrhosis with a history of esophageal variceal hemorrhage.Primary study outcome of the study is variceal rebleeding episodes occurring within the first years after interventions. Second study outcomes of the study are hepatic encephalopathy occurrence, mortality occurrence, liver transplantation or other cirrhosis-related complications.

Nonalcoholic fatty liver disease (NAFLD) is being recognized as one of the most prevalent causes of chronic liver disease worldwide. The current strategy proposed by the EASL/EASO/EASD European guidelines for the screening of nonalcoholic fatty liver disease (NAFLD) in high-risk population such as type 2 diabetes and patients with obesity leads to an over-referral in hepatology clinics. The proposed study will investigate the optimal strategy for the screening of NAFLD-related advanced fibrosis in patients at high risk of fibrotic NAFLD, such as patients with T2DM or obesity by maximizing the positive predictive value (PPV) using non-invasive blood and elastography-based biomarkers in endocrinology/diabetology clinics in order to reduce the over-referral to hepatology clinics.

The aim of this project is to evaluate the psychological reshuffle induced by ORKAMBI. The particular focus of this study is the consequence of its introduction on anxiety, depression, quality of life and adherence to all cystic fibrosis (CF) treatment. To answer this question investigators will monitor the psychological function of CF adolescents and young adults treated with ORKAMBI and compare them to CF adolescents and young adults not treated with ORKAMBI.

Educational programs are essential for cystic fibrosis patients who need to take several different drugs every day. Compliance is often klow in these patients, and clinicals and paramedics try to increase it, through educational programs. As for inhaled therapies, the CF center of Montpellier uses a video conference tool to educate patients. The program may involve either the physiotherapist or the doctor or both. Through the present study, the investigators would like to evaluate the current practice at the CF center of Montpellier and verify if patients feel that the physiotherapist could handle the program on his own, without the support of a physician.

Cystic Fibrosis related diabetes (CFRD), a major factor of morbid-mortality in CF, is characterized by a preclinical phase of glucose intolerance particularly long reaching up to 10 years. At the physiopathology level, insulin secretion is determinant in the glucose tolerance abnormalities in CF. Indeed insulin secretion is dependent of the CFTR activity at the beta cell surface and inhibition of CFTR leads to a decrease in insulin secretion. Recently, the combination of the lumacaftor, a CFTR corrector, with Ivacaftor, a CFTR potentiator, was studied in patient with CF homozygous for the Phe508 del CFTR mutation patients and showed an improvement of the respiratory state in comparison with the placebo group. These data suggests that lumacaftor in combination with ivacaftor in targeting CFTR action may have an early impact on the insulin-secretion and consequently on the glucose tolerance.