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

Fingerprint

Complement Factor H
Hemolytic-Uremic Syndrome
Transplants
Kidney
Mutation
Liver
Plasma Exchange
Liver Transplantation
Kidney Transplantation
Statistical Factor Analysis
Chronic Kidney Failure
Dialysis
Alleles
Mothers
Survival
Serum

Keywords

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

ASJC Scopus subject areas

  • Immunology

Cite this

Favorable long-term outcome after liver-kidney transplant for recurrent hemolytic uremic syndrome associated with a factor H mutation. / Saland, J. M.; Emre, S. H.; Shneider, B. L.; Benchimol, C.; Ames, S.; Bromberg, J. S.; Remuzzi, G.; Strain, L.; Goodship, T. H J.

In: American Journal of Transplantation, Vol. 6, No. 8, 08.2006, p. 1948-1952.

Research output: Contribution to journalArticle

Saland, J. M. ; Emre, S. H. ; Shneider, B. L. ; Benchimol, C. ; Ames, S. ; Bromberg, J. S. ; Remuzzi, G. ; Strain, L. ; Goodship, T. H J. / Favorable long-term outcome after liver-kidney transplant for recurrent hemolytic uremic syndrome associated with a factor H mutation. In: American Journal of Transplantation. 2006 ; Vol. 6, No. 8. pp. 1948-1952.
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