Cystic fibrosis (CF) is a lethal inherited disease caused by mutations in the CF
transmembrane conductance regulator (CFTR) gene, which result in impairment of CFTR …

The effects of cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators
on lung function, pulmonary exacerbations, and quality of life have been well documented …

CFTR modulators have dramatically changed the clinical course of CF in those fortunate
enough to receive them. Inevitably, randomised controlled trials during the development of …

There is a strong positive association between nutrition status and lung function in cystic
fibrosis (CF). Improvements in clinical care have increased longevity for individuals with CF …

Abstract The Academy of Nutrition and Dietetics Evidence Analysis Center conducted a
systematic review of the literature to develop an evidence-based practice guideline for …

Exercise is considered as an important component of the package of care delivered to
people with cystic fibrosis (pwCF). However, despite the well-known short-term physiological …

Background While the positive effect of Trikafta on cystic fibrosis (CF) pulmonary disease is
well established, there is limited data about its effect on bone mineral density (BMD), body …

Abstract Objective Elexacaftor/Tezacaftor/Ivacaftor is a cystic fibrosis transmembrane
conductance regulator (CFTR) modulator with the potential to improve exercise capacity …

Objectives In patients with cystic fibrosis (CF) who carry the G551D mutation, treatment with
ivacaftor improves lung function and weight; however, short-and long-term impacts on body …

Background G551D is a class III mutation of the cystic fibrosis transmembrane regulator
(CFTR) that results in impaired chloride channel function in cystic fibrosis (CF). Ivacaftor, a …