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NDT Advance Access originally published online on July 25, 2009
Nephrology Dialysis Transplantation 2009 24(11):3548-3551; doi:10.1093/ndt/gfp377
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© The Author 2009. Published by Oxford University Press [on behalf of ERA-EDTA]. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


Haemolytic uraemic syndrome caused by factor H mutation: is single kidney transplantation under intensive plasmatherapy an option?

Patricia Hirt-Minkowski1, Stefan Schaub1, Michael Mayr1, Jürg A. Schifferli2, Michael Dickenmann1, Véronique Frémeaux-Bacchi3 and Jürg Steiger1

1 Division of Transplantation Immunology and Nephrology 2 Division of Internal Medicine, University Hospital Basel, Basel, Switzerland 3 Assistance Publique-Hôpitaux de Paris, Department of Biological Immunology, Hôpital Européen Georges Pompidou, Paris, France

Correspondence and offprint requests to: Patricia Hirt-Minkowski; E-mail: phirt{at}uhbs.ch



  Abstract

Complement factor H (CFH) mutation is one of the causes of atypical haemolytic uraemic syndrome (aHUS). Patients with CFH mutation-associated aHUS progress often to end-stage renal disease despite plasma exchange therapy. When such patients are transplanted, aHUS recurs almost invariably and causes graft failure making the rationale of single kidney allograft transplantation questionable. Since CFH is synthesized mostly by the liver, combined liver–kidney transplantation has been recommended. However, fatal outcomes have been reported using this strategy. We report a case of successful single kidney allograft transplantation in a patient with a CFH gene mutation (R1210C), who had end-stage renal failure after three flares of aHUS treated with plasma exchange. He received peri- and postoperative infusions of fresh frozen plasma, which to date has prevented recurrence of the disease. He has preserved renal function 1-year post-transplant.

Keywords: complement factor H; haemolytic uraemic syndrome; kidney transplantation

Received for publication: 10. 5.09
Accepted in revised form: 6. 7.09


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