View clinical trials related to Cystic Fibrosis.
Filter by:The purpose of this study is to evaluate safety and tolerability of VX-121 in healthy subjects and in subjects with cystic fibrosis (CF).
Although some studies have brought some evidences about the efficacy of positive expiratory pressure (PEP)-mask therapy as an airway clearance technique, yet it is not clearly understood what is the contribution of this technique in modifying peripheral ventilation inhomogeneity, a typical feature of patients with Cystic Fibrosis (CF). The aim of this study is to investigate how PEP-MASK affects ventilation inhomogeneity in children and adolescents, with moderate to normal CF lung disease by the change in acinar airways (Sacin), lung clearance index (LCI) and conductive airways (Scond) indexes derived from nitrogen multiple-breath washout test (N2MBW).
In order to further enable physiopathology studies, the aim of this project is to validate an in vitro model of cystic fibrosis created using induced pluripotent stemcell (iPS) differentiated bronchial epithelium from cystic fibrosis (CF) patients homozygous for the p.Phe508del mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
The main objective of the project "MucoFong" (19021906 national french program n which Vaincre La Mucoviscidose participated: N82006/ 351) was to determine the fungi present the respiratory tract of CF patients responsible for either colonization or authentic infectious diseases. The Mucofong data allowed the team to provide for the 1st time national French guidelines for the management of CF sputum mycological analysis (MucoMicrobes work group coordinated by Prof. Plésiat published in 2015 in the REMIC book). Nevertheless, the team has a comprehensive database that it still has to analyze beyond these initial results. The main goal today is to clarify the role of fungi in the lung function degradation of these patients by studying the overall risk and estimated impact of fungal colonization in our cohort.
"This is a randomised, double-blind, placebo-controlled multi-centre study to investigate safety and tolerability and to provide pharmacokinetic and pharmacodynamics information of orally inhaled multiple doses (80 mg, 160 mg or 320 mg) of the nebulised neutrophil elastase inhibitor POL6014 in patients with Cystic Fibrosis. The controlled inhalation will occur via the eFlow® nebuliser system (manufacturer: PARI Pharma GmbH, Germany)".
The primary objectives of this study are to assess the safety and efficacy of MS1819-SD vs porcine pancreatic enzyme replacement therapy (PERT) in patients with exocrine pancreatic insufficiency (EPI) due to cystic fibrosis (CF).
Cystic fibrosis is a genetic disease that affects some organs of the human body. Among them, the lungs tend to be the most affected due to the accumulation of mucus in the airways, which in addition to avoiding the passage of air, favors pulmonary infections. With the evolution of the condition, secondary complications arise, such as postural changes, decreased respiratory muscle strength, decreased functional capacity and, consequently, quality of life. Therefore, respiratory muscle training may be an intervention that improves the respiratory condition of these individuals, allowing an improvement in the quality of life and may delay the evolution of respiratory symptoms. Thus, this study aims to investigate a home protocol of respiratory muscle training on respiratory muscle strength, lung function, quality of life, posture and functional capacity in adolescents and adults with cystic fibrosis. The researchers believe that the training can cause an improvement in the studied variables, and can be inserted in the usual treatment of these patients.
The main objective of this project is to evaluate the agreement between nasopharyngeal and sputum specimens in terms of detection of viral infection in Cystic Fibrosis (CF) participants.This is a 12-month national, multicenter prospective study (7 centers). Inclusions concern CF participants (children or adults) with signs of acute respiratory infection seen in consultation or hospitalized in their CF Research Center. A molecular viral multiplex search is performed on both nasopharyngeal and expectoration collected samples for each included participant. Determination of viral detection agreement between the two CF respiratory samples is then performed.
Four methods are routinely used to monitor CO2 in patients. End tidal CO2 (EtCO2) is monitored through the endotracheal tube during general anesthesia. CO2 is also monitored in other healthcare settings transcutaneously (TCO2), via finger stick capillary CO2 (CapCO2), and arterial blood gas (ABG). The purpose of this study is to perform all four measurements simultaneously during general anesthesia to identify which measure provides the most accurate data with the least amount of patient risk.
Patients with chronic lung diseases travelling by plane often suffer with symptoms related to lower oxygen levels they are exposed to while flying. Therefore, patients with respiratory conditions are routinely assessed to establish if they need supplemental oxygen in flight. A hypoxic altitude simulation test (HAST) is often part of this assessment and consists in having patients breathe a oxygen/nitrogen blend with a lower oxygen concentration compared to normal room air, simulating in-flight conditions. Oxygen levels are measured before and after the test through a blood sample (from the earlobe or an artery in the wrist) and with a finger probe. In-flight oxygen is required if the oxygen level in the blood is lower than 6.6 kPa. HASTs are time consuming, costly, and require a dedicated hospital appointment. Using historical data, the Investigators developed scores based on capillary blood gas (blood sample from the earlobe), diagnosis and sex to predict the outcome of the HASTs. The Investigators validated the proposed scores in a separate historic cohort of patients and showed it had good concordance with the HASTs results. In this study, the Investigators want to confirm prospectively if the score, based on blood results (venous and/or earlobe), can predict the outcome of the HASTs and therefore reduce the number of tests performed, travel time for patients, and costs for the NHS. All patients, aged 18 or older, who are having a HAST for clinical purposes at the cardio-respiratory lab at Leeds Teaching Hospital NHS Trust will be invited to take part in the study. The Investigators will record diagnosis, results of HAST and previous spirometry from the medical notes, perform a spirometry if not done in the previous 12 months and collect a blood sample (one tube, 4 mls). With these data, the Investigators will calculate the score and assess its agreement with the outcome of the HAST. Each participant's involvement in the study will last for approximately 90-120 minutes, which is the normal duration of a HAST. The Investigators aim to include up to 280 subjects in the study.