title: Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli's syndrome.
abstract: Congenital cystic dilation of the intrahepatic bile ducts (Caroli's syndrome) is a rare cause of chronic cholestasis and hepatolithiasis in young adults. Long-term prognosis is poor even with surgical drainage. We treated twelve patients who had Caroli's syndrome and intrahepatic stones with ursodeoxycholic acid (UDCA), 10-20 mg/kg daily. The duodenal bile of these patients contained cholesterol crystals, which suggests that the stones were cholesterol rich. UDCA led to sustained clinical remission, return to normal liver function, and dissolution of intrahepatic stones on ultrasound in all patients (nine partial, three complete) after 48 (range 12-114) months' follow-up. Litholytic therapy is indicated for intrahepatic stones in Caroli's syndrome.
authors.affiliation: Gastroenterology Service, Hospital Clínic i Provincial, Barcelona, Spain.
authors.forename: E
authors.initials: E
authors.lastname: Ros
authors.forename: S
authors.initials: S
authors.lastname: Navarro
authors.forename: C
authors.initials: C
authors.lastname: Bru
authors.forename: R
authors.initials: R
authors.lastname: Gilabert
authors.forename: L
authors.initials: L
authors.lastname: Bianchi
authors.forename: M
authors.initials: M
authors.lastname: Bruguera
mesh_terms: D000328:Adult; D001649:Bile Duct Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology; D001653:Bile Ducts, Intrahepatic; D016767:Caroli Disease / Q000150:complications*; D002769:Cholelithiasis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008111:Liver Function Tests; D008297:Male; D014463:Ultrasonography; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
chemical_list: D014580:Ursodeoxycholic Acid