Liver disease and bile duct abnormalities in adults with cystic fibrosis.

A quarter of adults with cystic fibrosis, 57 of 233, had abnormal liver function. Patients with hepatic dysfunction were further investigated by ultrasound, hepatobiliary scintigraphy, and endoscopic retrograde cholangiography (ERC). 17 of the 23 patients studied had abnormalities on imaging. All 15 patients who underwent ERC had abnormal intrahepatic ducts, but only 2 had stricture of the common bile duct. These findings suggest that, in patients with cystic fibrosis, intrahepatic impairment of biliary drainage may be important in the pathogenesis of liver disease.