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Resolution of Xanthomas in Alagille Syndrome After Liver Transplantation
Author(s) -
Buckley Deirdre A.,
Higgins Elisabeth M.,
Vivier Anthony W. P.
Publication year - 1998
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1998.tb01314.x
Subject(s) - alagille syndrome , medicine , liver transplantation , cholestasis , jaundice , dysplasia , transplantation , genetic disorder , hepatobiliary disease , pathology , disease
Alagille syndrome (arteriohepatic dysplasia) is a genetic disorder with autosomal dominant transmission which has been localized to chromosome 20p. Cutaneous manifestations include jaundice, pruritus, and widespread xanthomata. We report a child with severe Alagille syndrome in whom orthotopic liver transplantation caused rapid resolution of disfiguring xanthomas.