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Hepatocellular carcinoma occurring in alagille syndrome

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dc.contributor.authorKim, Bomi-
dc.contributor.authorPark, Sung-Hye-
dc.contributor.authorYang, Hye Ran-
dc.contributor.authorSeo, Jeong Kee-
dc.contributor.authorKim, Woo Sun-
dc.contributor.authorChi, Je G-
dc.date.accessioned2010-01-07-
dc.date.available2010-01-07-
dc.date.issued2005-04-06-
dc.identifier.citationPathol Res Pract. 2005;201(1):55-60.en
dc.identifier.issn0344-0338 (Print)-
dc.identifier.urihttp://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=15807312-
dc.identifier.urihttp://hdl.handle.net/10371/27448-
dc.description.abstractHepatocellular carcinoma only rarely occurs in Alagille syndrome. Here, we report on three cases of hepatocellular carcinoma associated with Alagille syndrome. All three patients were boys and presented with jaundice. In addition, they had the characteristic facial appearance of Alagille syndrome with cardiac, vertebral, and eye anomalies, and all had passed acholic stools from the neonatal period. Liver biopsies were diagnosed as bile duct paucity, compatible with Alagille syndrome in two cases, but the third case showed marked bile duct proliferation at the initial liver biopsy when 7 months old, which made diagnosis difficult. Eventually, all three cases progressed to biliary cirrhosis and hepatocellular carcinoma, which occurred at 17 months, 4 years, and 7 years, respectively. Because of the unusual liver histology and early onset of hepatocellular carcinoma, careful clinicopathologic correlations and close monitoring are required for the diagnosis of Alagille syndrome and for the early detection of hepatocellular carcinoma.en
dc.language.isoenen
dc.publisherElsevieren
dc.subjectAlagille Syndrome/*complications/radiographyen
dc.subjectCarcinoma, Hepatocellular/*etiology/pathology/radiographyen
dc.subjectFatal Outcomeen
dc.subjectHumansen
dc.subjectInfanten
dc.subjectLiver Neoplasms/*etiology/pathology/radiographyen
dc.subjectMaleen
dc.subjectRadiography, Abdominalen
dc.subjectRadiography, Thoracicen
dc.subjectTomography, X-Ray Computeden
dc.titleHepatocellular carcinoma occurring in alagille syndromeen
dc.typeArticleen
dc.contributor.AlternativeAuthor김보미-
dc.contributor.AlternativeAuthor박성혜-
dc.contributor.AlternativeAuthor양혜란-
dc.contributor.AlternativeAuthor서정기-
dc.contributor.AlternativeAuthor김우선-
Appears in Collections:
College of Medicine/School of Medicine (의과대학/대학원)Pediatrics (소아과학전공)Journal Papers (저널논문_소아과학전공)
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