• Vol. 30 No. 1, 44–47
  • 15 January 2001

Liver Transplantation in a Child With Severe Hypercholesterolaemia in Alagille Syndrome

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ABSTRACT

Introduction: Liver transplantation is a curative treatment modality in children with end stage liver disease in Alagille syndrome.

Clinical Picture: We report a 3-year-old child with this condition who had severe hypercholesterolaemia, pruritus and extensive xanthomatosis.

Treatment: Liver transplantation was performed in this patient.

Outcome: He recovered well with normalisation of his lipid profile. This procedure also resulted in resolution of the disfiguring xanthomatosis.


Alagille syndrome (AS) or arteriohepatic dysplasia is a genetic disorder transmitted in an autosomal dominant inheritance. The chromosomal abnormality has been identified to the short arm of chromosome 20.

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