Over the past two decades, bile acids (BAs) have become established as important
signaling molecules that enable fine-tuned inter-tissue communication from the liver, their …

Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on
chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas …

Cystic fibrosis (CF)‐associated liver disease (CFLD) is a hepatobiliary complication of CF.
Current diagnostic modalities rely on nonspecific assessments, whereas liver biopsy is the …

Simple Summary Inflammation is like the proverbial double-edged sword. Controlled short-
term inflammation could be a protective response while uncontrolled inflammation sustains …

Meconium ileus (MI) is often the first manifestation of cystic fibrosis (CF) and occurs in
approximately 20% of patients diagnosed with CF. This article reviews the pathophysiology …

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

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

Objectives: Hepatobiliary complications are a leading cause of morbidity and mortality in
cystic fibrosis (CF) patients. Knowledge of the underlying pathological aspects and optimal …

Cystic fibrosis (CF) is a life-limiting autosomal recessive multisystem disease. While its
burden of morbidity and mortality is classically associated with pulmonary disease, CF also …

Background A previous phase 3 study showed that lumacaftor–ivacaftor was generally safe
and well tolerated over 24 weeks of treatment in children aged 2–5 years with cystic fibrosis …