Korean J Gastrointest Endosc > Volume 19(5); 1999 > Article
Korean Journal of Gastrointestinal Endoscopy 1999;19(5): 848-852.
다낭성 신질환을 동반한 선천성 간 섬유화증 1예 ( A Case of Congenital Hepatic Fibrosis Associated with Polycystic Kidney Disease )
최황(Hwang Choi),함태호(Tae Ho Ham),김용균(Yong Kyoon Kim),김현수(Hyeon Soo Kim),문성배(Sung Bae Moon),김보경(Bo Kyoung Kim),최명규(Myung Gyu Choi),김재광(Jae Kwang Kim),정규원(Kyu Won Chung),선희식(Hee Sik Sun)
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. (Korean J Gastrointest Endosc 19: 848∼852, 1999)
Key Words: Congenital hepatic fibrosis, Polycystic kidney, Pancytopenia
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