View clinical trials related to Fibrosis.
Filter by:A 5-point decline of forced vital capacity expressed as % predicted, over 6 months, is the current definition of disease progression (fast decline in lung function) in idiopathic pulmonary fibrosis. There is a need for techniques allowing to characterize disease progression earlier, so that treatment may be adapted as early as possible in the lack of a response. Hypothesis. Our hypothesis is that 3-month changes of lung function parameters measured by a novel technique, impulse oscillometry, predicts 6-month changes in forced vital capacity in idiopathic pulmonary fibrosis.
The purpose of the study is to investigate whether the correction of CFTR function by Lumacaftor/Ivacaftor in a patient-derived primary nasal cell model is a surrogate biomarker for respiratory improvement in Orkambi® treated patients.
Following the results of the research team's cross-sectional study conducted in 2014 describing insufficient gynecological care, the research team implemented an on-site gynecological consultation in the adult Cystic Fibrosis (CF) centre in 2015. The study aimed to compare the results of two surveys conducted successively in 2014 and in 2017 on a cohort of women with CF attending the Lyon CF adult referral centre. All the women attending the adult CF center in 2017 were asked to complete the same self-report written questionnaire that was completed in 2014 about their contraceptive choices, gynecological follow-up and cervical screening. Questions concerning gynecological comorbidities were added to the 2017 questionnaire. This current study aimed to evaluate the impact of the implementation of an on-site gynecological consultation on gynecological health of women with CF, as measured through 3 indicators: the proportion of women with regular gynecological follow-up, cervical screening coverage, and contraceptive coverage. The identification of specific gynecological comorbidities was the other objective of this study.
Pulmonary rehabilitation should be initiated and lifelong at the time of diagnosis for patients with IPF. However, the symptoms of the disease and its progression limit clinical options in terms of participation and sustainability in rehabilitation programs. For this purpose, patients with IPF need physiotherapy and rehabilitation options that will not increase the symptoms associated with exercise and contribute to the program in the long term. Neuromuscular electrical stimulation (NMES) is a rehabilitation option that can be applied to specific muscle groups without the ventilator and cardiac load especially in patients who can not actively exercise or have decreased muscle strength. In adult patients with an advanced disease characterized by reduced muscle strength, the use of NMES in addition to aerobic exercise programs is recommended as part of rehabilitation programs. In the literature, no studies investigating the efficacy of NMES have been found in individuals with IPF or interstitial lung disease. NMES application in addition to aerobic exercise seems to be a reasonable option when considering the symptoms of patients with IPF and the progression of the disease. The aim of this project is to investigate the efficacy of NMES in addition to aerobic exercise in IPF patients based on evidence by objective methods.
Cystic fibrosis (CF) is characterized by a decrease in mucociliary clearance, recurrent infections and airway inflammation. This inflammatory process in airway mucosa is persistent, uncontrolled, but, somewhat paradoxically, ineffective for pathogen clearance. Neutrophils are chronically recruited in the airway mucosa by proinflammatory mediators such as Interleukin (IL)-17. However, mechanisms involved in this dysregulated and persistent immune response are not well understood. In this context, a heterogeneous subpopulation of T lymphocytes called "unconventional T cells" (UTC) should deserve greater attention. UTC play a key role in orchestrating the ensuing innate and adaptive immune responses and they are endowed with numerous regulatory and effector properties. UTC mainly establish residency at mucosal sites, including the lung. To date, however, data related to implication and behavior of UTC during cystic fibrosis are extremely limited. The hypothesis is that, given UTC properties, their functions and behavior are altered in CF, and thus, these cells could be implicated in persistent inflammation and poor response to infections. The objective is to study UTC properties and functions in cystic fibrosis using blood and sputum samples of patients with CF, in correlation with comprehensive clinical and microbiological data. The study will enroll adult patients with CF followed-up at University Hospital of Tours, France. For each patient included, blood and sputum samples will be analyzed during 18 months 1/ from routine tests obtained at steady state and 2/ from tests performed during acute exacerbations. UTC will be explored in blood and sputum using flowcytometry approach, to evaluate their relative abundance, activation/inhibition profile and functions (cytokine production and cytotoxic ability). Correlation will be made with clinical status, with longitudinal comparison across the study period for each patient, and comparison with the other patients and healthy volunteers. This study will add significant knowledge in CF immunopathology by comprehensively assess UTC presence, functions and activation in CF. Indeed, UTC could be explored for disease progression marker, and, in a long-term perspective, explored for therapeutic interventions aiming at modulating their function (by activating or inhibiting UTC), to reshape lung immune response during CF.
Chronic HCV infection has relatively slow rate of progression. Liver fibrosis is the main sequlae and usually progressed to cirrhosis after long period (10 to 20 years) [6]. Once cirrhosis is established the disease progression remains unpredictable: cirrhosis can remain indolent for many years in some patients whilst progressing in others to HCC, hepatic decompensation and death. Overall once cirrhosis has developed there is a 1-5% annual risk of HCC and a 3-6% annual risk of hepatic decompensation. Following an episode of decompensation the risk of death in the following year is between 15% and 20% Treatment of chronic HCV has been dramatically changed in the last few years with introduction of direct acting antivirals (DAAs). The new therapies with excellent safety profiles, shorter duration of therapy and marked higher efficacy can be even used in patients with advanced fibrosis and cirrhosis
Cystic fibrosis (CF) is an autosomal recessive genetic disease characterized by recurrent airway infections, affecting many systems including lung, pancreas and sweat glands. Cough is an important defense mechanism for clearing the secretions that increase in respiratory diseases. There have been studies investigating the effect of expiratory muscle training on disease groups such as chronic obstructive pulmonary disease, multiple sclerosis, parkinson's disease, and the elderly and healthy individuals. Studies that evaluating effects of expiratory muscle training in cystic fibrosis are limited in the literature. The aim of this study was to evaluate the effect of cough strength in children and adolescents with CF and the effect of expiratory muscle training program on cough strength, exercise capacity, respiratory muscle strength and quality of life.
Part I- Observational study In the first part , investigator would investigate and compare the energy requirements and substrate utilization in healthy subjects and in patients with liver cirrhosis of various etiologies across the disease severity state viz compensated, stable decompensated, critically ill cirrhotics. The investigator would also enroll patients with chronic kidney disease, critically ill patients of acute liver failure or acute on chronic liver failure as disease controls. Part II- Randomized Controlled Study All the eligible, critically ill cirrhotic patients on mechanical ventilator support would be randomized to a control group (receiving the nutritional therapy as per the standard enteral nutritional practice in a critical care setting) or the intervention group (receiving enteral nutrition based on proposed measured requirements by Indirect calorimetry (IC) given till the patient is in ICU(Intensive care unit). The IC would be done thrice a week in both groups.
This randomized, double-blind, controlled, clinical food study aims to explore KB174, a novel mixture of oligosaccharides, and maltodextrin, an easily digestible polysaccharide, on gut microbiome structure and function in subjects with well-compensated cirrhosis.
1. To determine personal exposure to air pollution in children with cystic fibrosis; 2. To determine airway macrophage uptake of inhaled particulate matter in cystic fibrosis children; 3. To establish whether prostaglandin E2 affects particulate matter removal in airway.