A Case of Congenital Hepatic Fibrosis Associated with Polycystic Kidney Disease.
- Author:
Hwang CHOI
1
;
Tae Ho HAM
;
Yong Kyoon KIM
;
Hyeon Soo KIM
;
Sung Bae MOON
;
Bo Kyoung KIM
;
Myung Gyu CHOI
;
Jae Kwang KIM
;
Kyu Won CHUNG
;
Hee Sik SUN
Author Information
1. Department of Internal Medicine, College of Medicine The Catholic University of Korea, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Congenital hepatic fibrosis;
Polycystic kidney;
Pancytopenia
- MeSH:
Biopsy;
Christianity;
Diagnosis;
Erythema;
Esophageal and Gastric Varices;
Female;
Fibrosis*;
Hemangioma;
Hepatomegaly;
Humans;
Kidney Diseases, Cystic;
Korea;
Liver;
Liver Diseases;
Liver Function Tests;
Magnetic Resonance Imaging;
Pancytopenia;
Polycystic Kidney Diseases*;
Psoas Muscles;
Spiders;
Splenomegaly;
Tomography, X-Ray Computed;
Young Adult
- From:Korean Journal of Gastrointestinal Endoscopy
1999;19(5):848-852
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital hepatic fibrosis is a developmental abnormality that may appear either sporadically or in a familial form. It is an inherited disease defined pathologically by bands of fibrous tissue within the liver, and is occasionally associated with cystic kidney disease. A 21-year-old woman was admitted to our hospital for evaluation of pancytopenia. She showed esophageal varices, hepatomegaly and splenomegaly, but had normal results on her liver function test. Peripheral stigmata of chronic liver disease such as palmar erythema or spider angioma was not found. Hepatosplenomegaly, polycystic kidney and psoas muscle cyst were detected through an abdominal CT and MRI. The patient is diagnosis was confirmed as congenital hepatic fibrosis using laparoscopic liver biopsy. The first case of congenital hepatic fibrosis associated with polycystic kidney disease in Korea is herein reported.
