Hepatobiliary fibropolycystic diseases. Two cases of Caroli's disease

Two case reports of Caroli's disease type I are given. The main clinical features were recurrent severe cholangitis, biliary colics, and persistent cholestasis. Both cases demonstrate the predominance in males and the prevalence of symptoms in adults up to the sixth decade. No features of congenital hepatic fibrosis or biliary cirrhosis and no renal pathomorphological changes could be detected. Investigations of their family members did not reveal genetic aspects.