Resolution of xanthomas in alagille syndrome after liver transplantation

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...

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Veröffentlicht in:	Pediatric dermatology 1998-05, Vol.15 (3), p.199-202
Hauptverfasser:	BUCKLEY, D. A, HIGGINS, E. M, DU VIVIER, A. W. P
Format:	Artikel
Sprache:	eng
Schlagworte:	Alagille Syndrome - complications Alagille Syndrome - surgery Biological and medical sciences Child Dermatology Follow-Up Studies Hand Dermatoses - etiology Hand Dermatoses - physiopathology Humans Liver Transplantation - adverse effects Male Medical sciences Tumors of the skin and soft tissue. Premalignant lesions Xanthomatosis - etiology Xanthomatosis - physiopathology
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Beschreibung
Zusammenfassung:	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.
ISSN:	0736-8046 1525-1470
DOI:	10.1046/j.1525-1470.1998.1998015199.x