Highly effective CFTR modulator drug therapy is increasingly available to those with cystic
fibrosis. Multiple observational research studies are now being conducted to better …

Growth failure is frequently encountered in children with chronic inflammatory conditions like
juvenile idiopathic arthritis, inflammatory bowel disease, and cystic fibrosis. Delayed puberty …

Background Malnutrition is both a frequent feature and a comorbidity of cystic fibrosis (CF),
with nutritional status strongly associated with pulmonary function and survival. Nutritional …

Cystic fibrosis bone disease (CFBD) is a common long-term complication of cystic fibrosis
(CF) that can lead to increased fractures and significant morbidity and mortality in this patient …

CF is caused by defects in the CF transmembrane conductance regulator (CFTR) gene, an
epithelial chloride channel that is widely expressed. The most common complications of CF …

Background Osteoporosis is a bone mineralisation disorder occurring in about one third of
adults with cystic fibrosis. Bisphosphonates can increase bone mineral density and …

Cystic fibrosis (CF) is widely known as a disease of the lung, even though it is in truth a
systemic disease, whose symptoms typically manifest in gastrointestinal dysfunction first. CF …

Cystic fibrosis transmembrane conductance regulator deficient mouse models develop
phenotypes of relevance to clinical cystic fibrosis (CF) including airway …

Mutations within the gene encoding for the chloride ion channel cystic fibrosis
transmembrane conductance regulator (CFTR) results in cystic fibrosis (CF), the most …

Anoctamin-6 (Ano6, TMEM16F) belongs to a family of putative Ca 2+-activated Cl− channels
and operates as membrane phospholipid scramblase. Deletion of Ano6 leads to reduced …