Favorable long-term outcome after liver-kidney transplant for recurrent hemolytic uremic syndrome associated with a factor H mutation

J. M. Saland, S. H. Emre, B. L. Shneider, C. Benchimol, S. Ames, J. S. Bromberg, G. Remuzzi, L. Strain, T. H J Goodship

Research output: Contribution to journalArticle

Abstract

A male child initially presented with atypical hemolytic uremic syndrome (HUS) at the age of 4 months and progressed within weeks to end stage renal disease (ESRD). At the age of 2 years he received a live-related kidney transplant from his mother, which, despite initial good function, was lost to recurrent disease after 2 weeks. Complement factor H analysis showed low serum levels and the presence of two mutations on different alleles (c.2918G > A, Cys973Tyr and c.3590T > C, Val197Ala). His survival on dialysis was at risk because of access failure and recurrent bacteremic episodes. Therefore, at the age of 5 years he received a combined liver-kidney transplant with pre-operative plasma exchange. Initial function of both grafts was excellent and this has been maintained for over 2 years. This report suggests that despite setbacks in previous experience, combined liver-kidney transplantation offers the prospect of a favorable long-term outcome for patients with HUS associated with complement factor H mutations. & 2006 The Authors.

Original languageEnglish
Pages (from-to)1948-1952
Number of pages5
JournalAmerican Journal of Transplantation
Volume6
Issue number8
DOIs
Publication statusPublished - Aug 2006

Keywords

  • Combined liver-kidney transplant
  • Factor H
  • HUS
  • Kidney transplant
  • Liver transplant
  • Recurrent

ASJC Scopus subject areas

  • Immunology

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