Cystic fibrosis (CF) is a heritable, multiorgan disease that impacts all tissues that normally
express cystic fibrosis transmembrane conductance regulator (CFTR) protein. While the …

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 monogenic disease caused by mutation of Cftr. CF-associated liver
disease (CFLD) is a common nonpulmonary cause of mortality in CF and accounts for …

Few human genetic diseases can rely on the availability of as many and as diverse animal
models as cystic fibrosis (CF), a multiorgan syndrome caused by functional absence of cystic …

The most typical expression of cystic fibrosis (CF)–related liver disease is a cholangiopathy
that can progress to cirrhosis. We aimed to determine the potential impact of environmental …

Cystic fibrosis (CF) is characterised by a build-up of thick, intransient mucus linings of the
digestive and respiratory mucosa, which disrupts digestive system functioning and …

With the improved treatment of the pulmonary complications of cystic fibrosis (CF),
gastrointestinal problems have become more important in the morbidity in CF. A hallmark of …

Purpose To identify independent imaging features and establish a diagnostic algorithm for
diagnosis of cystic fibrosis (CF)-associated liver disease (CFLD) in CF patients compared to …

Background & Aims The bile acid (BA)-activated farnesoid X receptor (FXR) controls hepatic
BA synthesis and cell proliferation via the intestinal hormone fibroblast growth factor 19 …

The gastrointestinal phenotype of cystic fibrosis (CF) features intestinal bile acid (BA)
malabsorption, impaired intestinal farnesoid X receptor (FXR) activation, and consequently …