Active clinical trials for "Cystic Fibrosis"

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.

Impacts of the Covid-19 epidemic and associated lockdown measures on the management, health and behaviors of cystic fibrosis patients during the 2020 epidemic

This study will evaluate the performance of wearable technology in cystic fibrosis (CF) participants taking commercial Elexacaftor (ELX)/Tezacaftor (TEZ)/Ivacaftor (IVA) utilizing a fully decentralized trial design.

There is an urgent need for a noninvasive method to diagnose bronchial infections and exacerbations in patients with Cystic Fibrosis (CF). The current method to diagnosed infections involves either collecting a sputum sample or obtaining a bronchoalveolar lavage (BAL). However, some patients cannot produce sputum. At the same time the decision of when a patient has an exacerbation continues to be very subjective. In this exploratory study, we propose a new, noninvasive method to diagnose bronchial infections and to evaluate possible markers of inflammation that can assist in a noninvasive way in the determination of exacerbations.

There are very few studies comparing the accuracy, sensitivity and specificity of HemoCue glucose 201 RT system with the laboratory gold standard. The incidence of cystic fibrosis related diabetes (CFRD) has risen significantly as patients' survival improves. Around 30% of all cystic fibrosis (CF) adult patients have CFRD. Early diagnosis of CFRD is important to slow the deterioration of lung function and nutritional status, both of which increase mortality. The oral glucose tolerance test (OGTT) is the accepted method for detecting CFRD and the Cystic Fibrosis Trust guidelines recommend that patients with CF over the age of twelve years should be screened annually. The World Health Organisation has pointed out that due to the absence of a more specific biological marker to define diabetes, plasma glucose estimation remains the basis of diagnostic criteria. WHO recommends that venous plasma glucose should be the standard method for measuring and reporting glucose concentration in blood. However, it has also recognised that there is a widespread use of capillary sampling. Although fasting values for venous and capillary plasma glucose are the same, in the nonfasting state capillary samples will give higher results than venous samples, and glucose values require conversion which can be problematic. HemoCue Glucose 201 RT (HGS 201 RT) analyzer with plasma conversion provides laboratory quality test results at the point of care for the diagnosis, screening and monitoring of diabetes mellitus. The analyzer is easy to use and the system can be operated by nonlaboratory personnel, also the results can be presented immediately, leading to significant time and resource saving. The patient also benefits from having the result immediately instead of waiting for several hours. With immediate result, the doctor and health care professionals can provide education, support and reassurance, and implement care plans, if appropriate, at the earliest opportunity. The aim of the study is to compare the clinical accuracy of HemoCue Glucose 201 RT with the laboratory standard method in the analysis of blood glucose concentration during oral glucose tolerance test.

The goal of this research study is to collect blood and urine samples from people who have either the R117H type of CF or the non-G551D gating type of CF to be kept for future research.We will also use some of the collected blood to measure the number of neutrophils.

The goal of this research study is to better understand current treatment practices for pulmonary exacerbations (lung infections) and whether the Cystic Fibrosis National Patient Registry (CFFNPR)can be used for this type of study.

Background By means of measurements of series of non-invasive inflammatory markers in exhaled breath (condensate), a reflection of inflammatory processes and oxidative stress, can be obtained. Thereby, these techniques could be important in monitoring asthma and CF lung disease in children. Fractional exhaled nitric oxide (FeNO) and inflammatory markers in exhaled breath condensate (EBC) reflect ongoing inflammation and oxidative stress in the airways. These markers have a promising capacity for monitoring diagnoses of CF and asthma lung disease. Aim To study the course of inflammatory markers in time in children with asthma and CF, in stable periods and during pulmonary exacerbations. In addition, we study the ability of inflammatory markers to predict safe tapering of medical treatment in both populations. To study the course of inflammatory markers in EBC during an exacerbation. To study which IM are already elevated before a clinical exacerbation is evident and can predict exacerbations in time. To study which inflammatory markers can predict safe discontinuation of antibiotics in children with CF, or tapering of inhaled corticosteroids in children with asthma. To study the relationship between inflammatory markers in EBC, the severity and control of CF and asthma, the symptoms and lung function within patients will be analysed. Methods Children with CF (n=30) and children with asthma (n=40) were recruited included from our outpatient clinic. During this longitudinal study patients visit the outpatient clinic were followed-up for 12 months; every two months during one year. patients visited our outpatient clinic. In addition to these standard visits, During exacerbations patients four extra visits were planned during an exacerbation. were asked to visit the University Hospital Maastricht four times. These additional visits were planned with a maximum of two times during the study. By means of a home monitor, children were asked to assess measurements of Besides measurements in the University Hospital, children measured forced expiratory volume in one second (FEV1) at home using a home monitor, to record medication use, and, to record presence and severity of pulmonary symptoms. Outcome parameters were: 1) FeNO assessment in exhaled air, 2) inflammatory markers in EBC, 3) lung function parameters, 4) specific questionnaires to assess asthma and CF control and severity, 5) data originating from the home monitor.

In patients with Cystic Fibrosis (CF) the clinical course of lung disease is crucial for individual prognosis and life expectancy. Imaging modalities are important in the assessment of follow up of structural lung changes and monitoring of pulmonary complications in CF. Although high resolution computed tomography (HRCT) is the accepted gold standard for evaluation of morphological lung changes in CF, chest-X-ray is widely used as standard imaging procedure for assessment and follow up in these young patients due to less radiation exposure. Magnetic resonance imaging (MRI) has not been used for lung imaging in CF so far. Studies from the 80's and early 90's were not able to show any impact for the use of MRI in CF. Due to recent technical developments MRI of the lung became possible. Our study group was able to show that MRI is a competitive imaging modality for evaluating changes of the CF-lung in comparison to the gold standard (HRCT). So far only patients from the age of 6-7 years were examined. According to recent studies CF is a disease which starts in utero. Therefore it can lead to extensive pulmonary changes even in infants and young children. In this age group lung function testing is difficult and not broadly available. An early optimized therapy is crucial for the long term course and outcome of the pulmonary disease. The aim of this study is to evaluate morphological and functional MRI for early diagnosis of lung changes in children (0-6 years) with CF.