View clinical trials related to Cystic Fibrosis.
Filter by:Cystic fibrosis is the most common hereditary autosomal recessive disease in the Caucasian population. The diseases is caused by a mutation of the gene coding for the CFTR protein (Cystic fibrosis transmembrane conductance regulator), an ion channel present at the apical pole of the epithelial cells. The channel dysfunction induces a deficit in hydration and a hyperviscosity of different exocrine secretions. Clinically, Cystic fibrosis is a multi-systemic disease. Pulmonary and pancreatic involvement are classically in the foreground. Degradation of respiratory function, associated with acute and chronic infections, represents the major cause of morbidity and mortality. Pseudomonas aeruginosa is a ubiquitous gram-negative bacillus found primarily in stagnant water. Pseudomonas aeruginosa is capable of colonizing the digestive, pulmonary and urinary mucosa and the skin. This bacterium is incriminated in many opportunistic infections including respiratory infections in patients with cystic fibrosis. Pseudomonas aeruginosa infection is the most common parenchymal lung infection in the Cystic fibrosis community. Pseudomonas aeruginosa chronic carriage represents a factor of poor prognosis associated with an increase in morbidity and mortality. Complications related to chronic carriage of Pseudomonas aeruginosa justify the implementation of strategies of eviction, screening and eradication of acute Pseudomonas aeruginosa infection. In addition to Pseudomonas aeruginosa contamination of patients via the environment, hand and airborne infections between patients with Cystic fibrosis have been reported. Measures to eliminate cross-transmissions have therefore been implemented in a majority of hospitals. The aim of the study is firstly to identify the number of Pseudomonas aeruginosa cross-transmissions between patients with Cystic fibrosis followed-up in Cystic fibrosis center of HUDERF. Investigator will use the Pulsed-Field Gel Electrophoresis to assess the possibility of cross-infection. Depending on the results, Investigator will implement new strategies to avoid future cross-contamination in our different places of care (consultation, hospitalization, physiotherapy…).
This project will test a technology-enabled patient support system (PSS) as a self-management tool for children with CF and their family caregivers.
A major factor in the respiratory health of cystic fibrosis (CF) patients is acquisition of chronic Pseudomonas (P.) aeruginosa infections. The infection rate with P. aeruginosa increases with age and by age 18 years, 80% of patients with CF in the U.S. are infected. Liposomal amikacin for inhalation (LAI; Arikayce™) is a sterile aqueous liposomal suspension consisting of amikacin sulfate encapsulated in liposomes. This formulation of amikacin maximizes the achievable dose and delivery to the lungs of infected patients when delivered via a nebulizer. Because liposome particles are small enough to penetrate and diffuse through sputum into the bacterial biofilm, they deposit drug close to the bacterial colonies (Meers, et al., 2008) (Clancy, et al., 2013), thus improving the bioavailability of amikacin at the infection site. The clinically achievable doses of amikacin in the LAI formulation can effectively increase the half-life of the drug in the lungs, and decrease the potential for systemic toxicity. LAI offers several advantages over current therapies in treating patients with CF with chronic infection caused by P. aeruginosa.
This is a single center, open label, Phase IIa, multiple-ascending dose study in which subjects with mild to moderate Cystic Fibrosis and non CF bronchiectasis (n≤12) will be enrolled. The safety and tolerability of S-1226 composed of PFOB with ascending doses of carbon dioxide (4%, 8%, and 12% CO2) administered twice daily in subjects with Cystic Fibrosis and non CF bronchiectasis will be evaluated. This will be followed by 5 day consecutive treatment using the highest tolerated dose of S1226. Participants can choose additional use of a further four weeks (28 days) of S-1226 therapy at home, using same or a lower tolerated dose.
The aim of this study is to evaluate the reaction time and postural control and to investigate the relationship between reaction time, exercise capacity, muscle oxygenation and balance in children with cystic fibrosis (CF) and non-CF bronchiectasis. 40 patients including 20 CF patients and 20 non-CF bronchiectasis and 20 healthy individuals will be included in this study. Demographic and physical characteristics' will be recorded. Lung function testing will be performed. Balance will be assessed using functional reach test, exercise capacity was measured using the incremental shuttle walking test and reaction time will be assessed using ''Fitlight TrainerTM''. Heart rate, respiratory rate, oxygen saturation, muscle oxygenation, dyspnea and fatigue perception will be measured before and after exercise test and reaction time measurement.
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.
The investigators will evaluate the analgesic efficacy of pulsed radiofrequency on the thoracic paravertebral nerves for patients with chronic mastalgia of fibrocystic disease of the breast
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.
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.