[A case of congenital hepatic fibrosis presented with symptom of acute cholangitis].
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.
Choi
Chang Soo
CS
Department of Internal Medicine, Wonkwang University College of Medicine, Iksan, Korea.
Oh
Hyo Jeong
HJ
Kim
Byung Soo
BS
Jo
Eun Young
EY
Kim
Tae Hyeon
TH
Choi
Suck Chei
SC
Kim
Haak Cheoul
HC
Yun
Ki Jung
KJ
D000208:Acute Disease; D000328:Adult; D002761:Cholangitis / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications* / Q000151:congenital* / Q000175:diagnosis; D008297:Male