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Alagille 증후군 -1예 보고-

Other Titles
 Alagille Syndrome - A Case Report - 
Authors
 Hyosup Shim  ;  Chanil Park  ;  Young Nyun Park  ;  Soon Il Kim 
Citation
 KOREAN JOURNAL OF PATHOLOGY , Vol.38(1) : 56-59, 2004 
Journal Title
 KOREAN JOURNAL OF PATHOLOGY 
ISSN
 1738-1843 
Issue Date
2004
Keywords
Alagille Syndrome ; Intrahepatic Bile Duct ; Heart Disease
Abstract
Alagille syndrome is a rare autosomal dominant disorder showing complicated systemic manifestations, although the hepatic manifestations are predominant in many patients. We report a case of Alagille syndrome in a male baby who presented with a heart murmur at 2 days old and his echocardiography showed aortic stenosis. He presented with acholic stool and jaundice at 3 months old and a liver biopsy revealed paucity of the interlobular bile duct in the portal tract. This progressed to cirrhosis, for which a liver transplantation was performed at 10 months old. The explanted liver showed biliary-type cirrhosis with severe cholestasis. There was an absence of the interlobular bile ducts on microscopic examination. Bile duct paucity, associated with cholestasis, a peculiar face (prominent forehead, deep-set eyes, pointed mandible and bulbous nasal tip), and cardiac anomaly were observed, which were consistent with Alagille syndrome. He died of heart failure.
Files in This Item:
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Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Surgery (외과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Soon Il(김순일) ORCID logo https://orcid.org/0000-0002-0783-7538
Park, Young Nyun(박영년) ORCID logo https://orcid.org/0000-0003-0357-7967
Park, Chan Il(박찬일)
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/112439
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