HEPATOBILIARY DISEASE ASSOCIATED WITH CYSTIC FIBROSIS IN INFANCY AND CHILDREN

Abstract

This article provides overviews of the hepatobiliary diseases associated with cystic fibrosis (CF). Liver disease in infancy and childhood is often asymptomatic, but serious complications arising from advanced disease, including cirrhosis with portal hypertension, occure more frequent, in the adolescent and adult population. The author presents: – specific hepatic disorders: incidency, specific hepatic disorders: fatty cirrhosis; neonatal cholestasis; evaluation of liver disease; management of liver disease (cholestasis, portal hypertension, hepatic failure); – biliary tract disease: nonvisualization of the gallbladder and microgallbladder; cholelitiasis; distal common duct stricture.

Keywords

• cystic fibrosis
• specific hepatic disorders
• infancy and childhood