Hepatobiliary pathology in patients with cystic fibrosis

Overt liver disease in cystic fibrosis is a rare condition. Only 1-5% of all patients show a severe disturbance of the liver cell function or portal hypertension. In contrast, liver architecture is much more often disturbed at post mortem examination. The experience is that liver pathology increases with age which will result in rising numbers of patients in the future parallel to the increasing life expectancy of the patients. Bile plugs are commonly found in the portal tract and probably represent the essential abnormality of the liver in CF. Recently new methods have been developed for the investigation of the bile synthesis which will be helpful in the understanding of the CF defect in the liver.