Cystic Fibrosis Clinical Trials

This Study will not provide or recommend any treatment or any changes to your standard medical care. If you participate, there will not be any additional doctor visits outside of your standard medical visits to the Cystic Fibrosis Care Centers. Participation in this Study will not require any additional time ...

This study is designed to evaluate genetic mechanisms of lung disease by surveying polymorphic genes involved in respiratory function and examining gene expression in the lung cells of individuals with pulmonary disease (e.g., alpha 1-antitrypsin deficiency, asthma, chronic obstructive pulmonary disease, cystic fibrosis, sarcoidosis, history of infection, and genetic mutations ...

As well as progressive lung disease, patients with Cystic Fibrosis (CF) suffer pancreatic destruction, leading to slow but progressive insulin deficiency. Deficiency of insulin, a powerful anabolic hormone, causes accelerated decline of weight and lung function (important predictors of early mortality in CF). We analysed Oral Glucose Tolerance Tests sampled ...

The purpose of this study is to find the safest and most effective way to administer IV antibiotics to treat acute pulmonary exacerbations (APEs) in patients with cystic fibrosis (CF) that are caused by pathogens, like Pseudomonas aeruginosa. This study will test the safety and effectiveness of two commonly prescribed ...

Study design. This academic investigator - initiated trial will resolve the key issue whether, and if yes, to what extent and variability, the treatment with lumacaftor in combination with ivacaftor (Orkambi) will reverse the p.Phe508del CFTR - mediated basic defect in p.Phe508del homozygous subjects with cystic fibrosis under real life ...

The aim of this study is to investigate the efficacy of antibiotic therapy with any antibiotic (IV) and IV (Nebcin) tobramycin for 5 days followed by Solution for nebuliser inhalation (Tobi) for 9 days and antibiotic cures using 14 days of tobramycin IV. In the case of positive results, the ...

The co-primary objectives of the trial are to investigate the effect of the use of inhaled colistimethate sodium, administered twice daily via the I-neb for 24 months, compared to placebo in subjects with non-cystic fibrosis bronchiectasis (NCFB) chronically infected with P. aeruginosa on: the frequency of pulmonary exacerbations; the number ...

Current guidelines for referring cystic fibrosis (CF) patients for lung transplantation, based on clinical and functional resting parameters, are insufficient to predict 3-year mortality. Previous studies have shown that sex, impaired pulmonary function, undernutrition and colonization of the respiratory tract by Pseudomonas aeruginosa are associated with a poor prognosis. Current ...

Rationale: Airway disease, featuring early and intense inflammation and leading to progressive lung damage, is the main cause of morbidity and mortality in cystic fibrosis (CF). Mechanisms of CF airway inflammation remain unclear, hampering development of better treatments. Recent introduction of heel-prick screening for CF provides a unique longitudinal cohort ...

Cystic fibrosis (CF) is the most common lethal autosomal recessive disorder in the Caucasian population affecting ~1:3000 children, with a carrier frequency of 1:25. It is a multisystem disorder where pulmonary and sinonasal involvements occur in 90-100% of patients, and up to 86% of children have nasal polyps . Patients ...