Korean J Gastrointest Endosc.
1999 Oct;19(5):848-852.
A Case of Congenital Hepatic Fibrosis Associated with Polycystic Kidney Disease
- Affiliations
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- 1Department of Internal Medicine, College of Medicine The Catholic University of Korea, Seoul, Korea.
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.
MeSH Terms
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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
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