A Case of Congenital Hepatic Fibrosis Presented with Symptom of Acute Cholangitis.
- Author:
Chang Soo CHOI
1
;
Hyo Jeong OH
;
Byung Soo KIM
;
Eun Young JO
;
Tae Hyeon KIM
;
Suck Chei CHOI
;
Haak Cheoul KIM
;
Ki Jung YUN
Author Information
1. Departments of Internal Medicine, Wonkwang University College of Medicine, Iksan, Korea. kth@wmc.wonkwang.ac.kr
- Publication Type:Case Report ; English Abstract
- Keywords:
Congenital hepatic fibrosis;
Cholangitis
- MeSH:
Acute Disease;
Adult;
Cholangitis/*complications/diagnosis;
Humans;
Liver Cirrhosis/*complications/*congenital/diagnosis;
Male
- From:The Korean Journal of Gastroenterology
2005;46(3):237-241
- CountryRepublic of Korea
- Language:Korean
-
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.
