View clinical trials related to Fibrosis.
Filter by:This study will evaluate the efficacy, safety, and pharmacodynamics of elexacaftor (ELX, VX-445) in triple combination (TC) with tezacaftor (TEZ) and ivacaftor (IVA) in subjects with cystic fibrosis (CF) who are homozygous for F508del.
Pirfenidone (PFD), an oral antifibrotic drug with anti-inflammatory and anti-oxidant properties, has been granted marketing authorization by the European Medicine Agency and FDA, for the treatment of idiopathic pulmonary fibrosis (IPF). However, few studies have focused on its clinical utilization in patients with advanced hepatic fibrosis. Therefore, Investigators aim to evaluate a prolonged-release PFD formulation (PR-PFD) plus standard of care management on disease progression in patients with advanced liver fibrosis (ALF). Methods: Patients with diverse chronic liver disease etiology (alcohol-related, hepatitis B or C, autoimmune or fatty liver disease) will be screened with two non invasive liver fibrosis methods (Fibroscan®) and Fibro Test®) and those with ALF (F3 or F4) will be treated for at least 12 months with PR-PFD. Antifibrotic effects Will be assessed at 6 and 12 months; variations greater than 30% in estimated fibrosis scores or 1 point on the METAVIR scale will be considered clinically significant. PFD plasma levels, serum endothelin-1, IL6, TNFα and TGFβ1, Quality of life and fatigue scales will be evaluated. Parametric and non parametric statistics will be utilized and p values lower tan 5% will be considered clinically significant.
Regular bronchial clearance is essential in patients with cystic fibrosis for their bronchial health. SIMEOX® (Physio-Assist, Aix en Provence, France) is an innovative medical device for the drainage of the bronchial tree. By changing the rheological properties of mucus, SIMEOX® helps to mobilize secretions and assists their transport to the upper airways. This technology is based on fundamental research on bronchial mucus rheology. At the present time, SIMEOX® device is mainly used over a short period at the time or after an exacerbation in healthcare structures (hospitals, physiotherapy practices, postcare, and rehabilitation units, etc…). The clinical effects observed in the short term encourages long-term autonomous use by the patients themselves. The overall objective of this study is to evaluate the efficiency and acceptability of SIMEOX® used at home by the patient himself for bronchial clearance in patients with cystic fibrosis.
"The usefulness of respiratory physiotherapy and its execution modalities remains highly debated even though reviews of the literature show that respiratory physiotherapy is able to improve the drainage of bronchial secretions and pulmonary function tests during cystic fibrosis in periods of stability. Different physiotherapy techniques have been developed but the choice of one or the other facing a patient can not currently be recommended. The VirtualChest project, supported by a grant from the National Agency for Research (ANR), aims to develop and validate a physical model of respiratory physiotherapy (6 stages including model establishment pulmonary: bronchial tree and pulmonary mechanics and parietal [steps 1 and 3], a model of mucus [step 2] and modeling the effect of physiotherapy [step 5]). This project is integrated with stages 3 and 5 of this broad project and aims to get on a limited number of children with cystic fibrosis a proof of concept (prediction of drainage efficiency) and especially to feed the proposed physical models in order to subsequent optimization of the model (step 6). The choice cystic fibrosis of the child was justified by the effect demonstrated respiratory physiotherapy, particularly on respiratory functional criteria, and the fact that the parietal mechanics varies physiologically at this age."
Chest physiotherapy plays a crucial role in treatment of lung disease in cystic fibrosis (CF). New airway clearance techniques (ACTs) adapted to individual needs are still being sought to achieve the best effect of airway clearance. The primary aim of this study is to assess the efficacy of a new ACT (Simeox) on pulmonary function in children with CF. 40 CF patients with stable respiratory function will be randomized 1:1 to Simeox or conventional chest physiotherapy (CCPT) therapy (control group) and treated at home during 1 month. After a short washout period, patients will be treated at home onto the alternative treatment for 1 month (crossover design). Lung function, quality of life, pulmonary exacerbation and safety will be evaluated at 1 month for each therapy period.
The purpose of this study is to assess the safety, tolerability, kinetics and test-retest repeatability of the novel LPA1 positron emission tomography (PET) ligand 18F-BMS-986327 in healthy participants and participants with idiopathic pulmonary fibrosis (IPF).
The aim of this study is to assess whether the 1 minute Sit to Stand (STS) test can be used as a measure of submaximal endurance during a pulmonary exacerbation of Cystic Fibrosis (CF) for patients in the acute care setting. We hypothesize that if the STS test is a valid measure of submaximal cardiovascular endurance, it will moderately correlate with 6 minute walk distance (6MWD).
This study evaluated the long-term safety, efficacy, and pharmacodynamics of elexacaftor (ELX, VX-445) in triple combination (TC) with tezacaftor (TEZ) and ivacaftor (IVA) in subjects with cystic fibrosis (CF) who are heterozygous for the F508del mutation and a gating or residual function mutation (F/G and F/RF genotypes).
This study will evaluate the efficacy, safety and pharmacodynamics of elexacaftor (ELX, VX-445) in triple combination (TC) with tezacaftor (TEZ) and ivacaftor (IVA) in subjects with cystic fibrosis (CF) who are heterozygous for F508del and a gating or residual function mutation (F/G and F/RF genotypes).
To evaluate the safety and pharmacokinetics of OP-724 and to determine the recommended dose of OP-724 against Primary Biliary Cholangitis patients.