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Transplant International

, Volume 13, Issue 3, pp 207–210 | Cite as

Liver transplantation for alpha-1-antitrypsin deficiency in children

  • Andreas A. Prachalias
  • Mohamed Kalife
  • Ruggiero Francavilla
  • Paolo Muiesan
  • Anil Dhawan
  • Alastair Baker
  • D. Hadzic
  • Giorgina Mieli-Vergani
  • Mohamed Rela
  • Nigel D. Heaton
ORIGINAL ARTICLE
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Abstract

Alpha-1-antitrypsin (a1-AT) deficiency is an inborn error of metabolism, which can cause liver disease. The condition is one of the most common genetic disorders in the Caucasian population. Here we review our experience with 21 children suffering from end-stage liver disease due to a1-AT deficiency. All children are PIZZ homozygotes. Nineteen of them initially presented with neonatal jaundice and two with hepatosplenomegaly in childhood. Twenty-five liver transplantions were performed. All children are currently alive at a median follow-up of 40 months. Liver replacement provides the only definite treatment for children with end-stage liver disease associated with a1-AT deficiency. Excellent results can be achieved by reducing waiting time for transplantation and by early referral to a liver transplant centre.

Key words Alpha-1-antitrypsin deficiency Liver transplantation Childhood 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • Andreas A. Prachalias
    • 1
  • Mohamed Kalife
    • 1
  • Ruggiero Francavilla
    • 1
  • Paolo Muiesan
    • 1
  • Anil Dhawan
    • 1
  • Alastair Baker
    • 1
  • D. Hadzic
    • 1
  • Giorgina Mieli-Vergani
    • 1
  • Mohamed Rela
    • 1
  • Nigel D. Heaton
    • 1
  1. 1.Liver Transplant Surgical Service, Paediatric Liver Service, King's College Hospital, Denmark Hill, London SE5 9RS, UKDK

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