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선천성 간섬유증 1례

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Author(s)
곽진영강진무박근수김명성김흥식
Keimyung Author(s)
Park, Geun SooKim, Myung SungKim, Heung SikKang, Chin Moo
Department
Dept. of Pediatrics (소아청소년학)
Journal Title
Keimyung Medical Journal
Issued Date
1999
Volume
18
Issue
3
Keyword
Congenital hepatic fibrosisNephronophthisis
Abstract
Congenital hepatic fibrosis is an unusual congenital disorder of the liver of children, and characterized by hard hepatomegaly, portal hypertension with relative preservation of the liver function and underlying architecture, and frequent renal involvement. Histologically the liver is characterized by portal fibrosis and an increase in portal ductal structure but the hepatocytes are not affected.
We have experienced a case of congenital hepatic fibrosis in 5 year old girl. The patient's chief complaints were fever, epigastric pain and abdominal distension. There are marked hepatomegaly and routine CBC. transaminase and bilirubin were within normal limits. Abdominal ultrasonography and CT scan reveal diffusely cystic dilatation of the intrahepatic bile ducts and numerous small calculi in the both medullary portion of kidney(nephronophthisis). Liver biopsy was performed. The microscopic findings are characterized that hepatic lobules surrounded by dense fibrous connective tissue and bile duct hyperplasia, but hepatic parenchyme is normal.
Congenital hepatic fibrosis was confirmed by liver biopsy and also she had nephronophthisis. We present this case with the brief review of literature.
Alternative Title
A case of
Congenital Hepatic fibrosis
Keimyung Author(s)(Kor)
박근수
김명성
김흥식
강진무
Publisher
Keimyung University School of Medicine
Citation
곽진영 et al. (1999). 선천성 간섬유증 1례. Keimyung Medical Journal, 18(3), 470–477.
Type
Article
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/15166
Appears in Collections:
2. Keimyung Medical Journal (계명의대 학술지) > 1999
1. School of Medicine (의과대학) > Dept. of Pediatrics (소아청소년학)
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