title: [A case of congenital hepatic fibrosis presented with symptom of acute cholangitis].
abstract: Congenital hepatic fibrosis (CHF) is an autosomal recessive disease, presenting principally in children or young adults with portal hypertension, and infrequently associated with cholangitis. It is associated with renal malformation and Caroli's disease. The diagnosis of CHF is usually confirmed by its typical histological features. Cholangitis is a severe and frequently fatal complication. We report a 22-year-old man with congenital hepatic fibrosis who showed the cholangitis without radiological features of cystic dilatation or stone of intrahepatic ducts.
authors.affiliation: Department of Internal Medicine, Wonkwang University College of Medicine, Iksan, Korea.
authors.forename: Chang Soo
authors.initials: CS
authors.lastname: Choi
authors.forename: Hyo Jeong
authors.initials: HJ
authors.lastname: Oh
authors.forename: Byung Soo
authors.initials: BS
authors.lastname: Kim
authors.forename: Eun Young
authors.initials: EY
authors.lastname: Jo
authors.forename: Tae Hyeon
authors.initials: TH
authors.lastname: Kim
authors.forename: Suck Chei
authors.initials: SC
authors.lastname: Choi
authors.forename: Haak Cheoul
authors.initials: HC
authors.lastname: Kim
authors.forename: Ki Jung
authors.initials: KJ
authors.lastname: Yun
mesh_terms: D000208:Acute Disease; D000328:Adult; D002761:Cholangitis / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications* / Q000151:congenital* / Q000175:diagnosis; D008297:Male