Liver disease is a relatively frequent and early complication of cystic fibrosis. The
pathogenesis is apparently multifactorial, with contributions from environmental and genetic …

The biliary tree is a complex network of conduits that begins with the canals of Hering and
progressively merges into a system of interlobular, septal, and major ducts which then …

Bone marrow‐derived mesenchymal stem cell (BM‐MSC) transplantation has been
suggested as an effective therapy for liver cirrhosis. The efficacy and safety of autologous …

Cystic fibrosis–associated liver disease is a chronic cholangiopathy that negatively affects
the quality of life of cystic fibrosis patients. In addition to reducing biliary chloride and …

BACKGROUND & AIMS: Loss of function of the cystic fibrosis transmembrane conductance
regulator (CFTR) in the biliary epithelium reduces bile flow and alkalinization in patients with …

Primary canalicular bile undergoes a process of fluidization and alkalinization along the
biliary tract that is influenced by several factors including hormones, innervation …

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 …

ABSTRACT Liver involvement in Cystic Fibrosis (CF) is much less frequent than both
pulmonary and pancreatic diseases that are present in 80‐90% of CF patients; liver disease …

In the liver, the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) regulates
bile secretion and other functions at the apical membrane of biliary epithelial cells (ie …

BACKGROUND & AIMS: Severe polycystic liver disease can complicate adult dominant
polycystic kidney disease, a genetic disease caused by defects in polycystin-1 (Pkd1) or …