Cited 0 times in
Alagille 증후군 -1예 보고-
DC Field | Value | Language |
---|---|---|
dc.contributor.author | 김순일 | - |
dc.contributor.author | 박영년 | - |
dc.contributor.author | 박찬일 | - |
dc.date.accessioned | 2015-07-14T17:13:02Z | - |
dc.date.available | 2015-07-14T17:13:02Z | - |
dc.date.issued | 2004 | - |
dc.identifier.issn | 1738-1843 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/112439 | - |
dc.description.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. | - |
dc.description.statementOfResponsibility | open | - |
dc.format.extent | 56~59 | - |
dc.relation.isPartOf | KOREAN JOURNAL OF PATHOLOGY | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.title | Alagille 증후군 -1예 보고- | - |
dc.title.alternative | Alagille Syndrome - A Case Report - | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Pathology (병리학) | - |
dc.contributor.googleauthor | Hyosup Shim | - |
dc.contributor.googleauthor | Chanil Park | - |
dc.contributor.googleauthor | Young Nyun Park | - |
dc.contributor.googleauthor | Soon Il Kim | - |
dc.admin.author | false | - |
dc.admin.mapping | false | - |
dc.relation.journalcode | J02092 | - |
dc.identifier.eissn | 2092-8920 | - |
dc.subject.keyword | Alagille Syndrome | - |
dc.subject.keyword | Intrahepatic Bile Duct | - |
dc.subject.keyword | Heart Disease | - |
dc.contributor.alternativeName | Kim, Soon Il | - |
dc.contributor.alternativeName | Park, Young Nyun | - |
dc.contributor.alternativeName | Park, Chan Il | - |
dc.rights.accessRights | free | - |
dc.citation.volume | 38 | - |
dc.citation.number | 1 | - |
dc.citation.startPage | 56 | - |
dc.citation.endPage | 59 | - |
dc.identifier.bibliographicCitation | KOREAN JOURNAL OF PATHOLOGY , Vol.38(1) : 56-59, 2004 | - |
dc.identifier.rimsid | 56173 | - |
dc.type.rims | ART | - |
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.