View clinical trials related to Cystic Fibrosis.
Filter by:This project is an attempt to understand how Orkambi treatment affects exercise capacity and the function of the arteries in CF patients who are homozygous F508del. Our goal is to perform the exercise and vascular measurements every 3 months after a patient starts taking Orkambi.
Respiratory und intestinal microbiome will bei analyzed during a period of 6 months. In a retrospective analysis it will be looked for correlations between microbiome and cf therapy (e.g. inhaled and systemic antibiotics, cftr modifiers, proton pump Inhibitors, enzymes, nutritional habits), clinical status and self reported outcome.
Recent improvements in cystic fibrosis management have enabled an important subset of patients to maintain normal spirometry. Nevertheless, even in these patients with normal spirometry, chest computed tomography (CT) might identify structural lung abnormalities such as bronchiectasis, mucus plugging, bronchiolitis and air trapping. Lung clearance index (LCI) has been shown to correlate well with structural pulmonary alterations seen on CT even in CF patients with well-preserved spirometry. In cystic fibrosis, a high LCI is associated with a worse feeling of illness assessed by the Cystic Fibrosis Questionnaire-Revised (CFQ-R). School-aged CF patients with normal spirometry also have normal aerobic function as assessed by peak oxygen uptake (V̇O2) measured during symptom-limited incremental cardiopulmonary exercise test. However, the ability of LCI to predict ventilatory abnormalities appearing at exercise in CF patients has not been investigated. The investigators therefore aimed to compare physiological parameters at exercise between CF patients with elevated LCI (i.e., LCI above the upper limit of normal [ULN]) and patients with normal LCI, all with preserved spirometry.
Monitoring patients with chronic, inflammatory airways disease particularly in the early stages is hampered by the relative insensitivity of current outcome measures to detect subtle changes. Multiple breath washout is a potential sensitive test that is a useful readout of disease at these early stages but it lacks standardisation and knowledge of variability with reference to standard lung function measures. This is a Cross sectional and longitudinal observation study. The hypothesis is that multiple breath washout-derived indices will provide a robust signal of gas mixing inhomogeneity, correlating with conventional measures of airway disease severity. Multiple breath washout performed on different devices will generate indices which correlate but differ in value.
A survey of adults and parents of children with cystic fibrosis on food insecurity
Pilot study to investigate the effect of choline chloride in cystic fibrosis patients with liver steatosis by comparing their status before and after the intervention
An increase in the prevalence of infections due to non-tuberculous mycobacteria (NTM) is observed in many countries and recent data suggest the circulation of dominant clones with a possibility of human-to-human contamination. The hypothesis is made that these infections are also increasing in France and that dominant NTM clones are circulating. The last French study carried out in 2004 already showed prevalences of up to 10% in certain French regions. It is essential to know the prevalence 8 years later, taking advantage of the new recommendations for the management of patients and samples, which will homogenize practices on French territory. No data are currently available in France on the prevalence of positive serological responses in cystic fibrosis patients. Serological analyzes of the sera collected during this study will enable us to evaluate the performance of serology in mycobacterial culture and to identify patients with no positive respiratory specimen in culture but with positive serology indicating potential contact with a mycobacterium. The establishment of a serological follow-up of these patients will allow to correlate this result with a clinical evolution and / or the detection of NTM in subsequent samples. Serology is an innovative aspect of the CIMENT study.
Although Cystic Fibrosis is a complex genetic disease affecting many organs, lung disease is the primary cause of mortality. The objective of this study is to determine the safety and tolerability of SNSP113 in healthy subjects and subjects with stable cystic fibrosis.
Early detection of pulmonary exacerbations (PEx) in Cystic Fibrosis (CF) patients is important to quickly trigger treatment and reduce respiratory damage. The investigators hypothesize that using home-based connected devices (CDs) in educated patients applying Cumulative sum charts (CUSUM) to monitor physiological parameters (PP) and patients' perception reported (PRP), will allow early detection of PEx. Objective: to study clinical validity of using CDs and evaluate adherence and satisfaction in CF patients and teams Design: 3 phase multicenter study in 36 CF patients aged >=12 years. Phase 1, patients are equipped during 3 months with CDs. PP and PRP to estimate CUSUM parameters are collected. In phase 2, patient's personalized educational plan to manage alerts is built. In phase 3, PP and PRP are collected during 12 months. Clinical validity, change in patients clinical data, quality of Life/Anxiety-Depression/Satisfaction, patients and teams' acceptance and adherence are assessed.
prospective multicentric study protocol in french CF center, exploring adolescent avec parents needs during transition from pediatric CF center to adult CF center