Cystic fibrosis (CF) is the most common autosomal recessive disease in the Caucasian
population. Cystic fibrosis-related liver disease (CFLD) is defined as the pathogenesis …

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

Peribiliary glands (PBG) are a source of stem/progenitor cells organized in a cellular
network encircling large bile ducts. Severe cholangiopathy with loss of luminal biliary …

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 …

Objective Cystic fibrosis associated liver disease (CFLD) is the third largest cause of
mortality in CF. Our aim was to define the burden of CFLD in the UK using national registry …

Cystic fibrosis (CF) liver disease (CFLD), a leading cause of death in CF, is mostly described
in pediatric populations. Adult‐onset CFLD lacks sufficient characterization and diagnostic …

Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal
hypertension, multilobular cirrhosis, and liver failure. Current guidelines on the detection …

While liver involvement is common in cystic fibrosis, the major liver disorder with impact on
the clinical outcome of individuals with CF is the development of multilobular cirrhosis with …

Individuals with cystic fibrosis (CF) develop complications of the gastrointestinal tract
influenced by genetic variants outside of CFTR. Cystic fibrosis-related diabetes (CFRD) is a …

Although cystic fibrosis (CF) is recognized as a monogenic disease, due to variants within
the CFTR (Cystic Fibrosis Transmembrane Regulator) gene, an extreme clinical …