Transjugular intrahepatic portosystemic shunt in non-cirrhotic portal hypertension related to cystic fibrosis in a lung transplant patient

Liver involvement has been commonly described in patients with cystic fibrosis (CF), especially during childhood and adolescence. According to the classical pathogenetic pathway, an impairment of bile composition may lead to intrahepatic biliary plugs, hepatocyte damage, cholestasis and ultimately biliary cirrhosis. Recent findings, showing high prevalence of microthrombi and endothelialitis at histology, without features of cirrhosis, suggested alternative mechanisms that contribute to liver involvement and development of portal hypertension [1,2].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Case Report Source Type: research