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

Hirt-Minkowski, Patricia and Schaub, Stefan and Mayr, Michael and Schifferli, Jürg A. and Dickenmann, Michael and Frémeaux-Bacchi, Véronique and Steiger, Jürg. (2009) Haemolytic uraemic syndrome caused by factor H mutation : is single kidney transplantation under intensive plasmatherapy an option? Nephrology, dialysis, transplantation, Vol. 24. pp. 3548-3551.

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Official URL: http://edoc.unibas.ch/dok/A6005645

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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.
Faculties and Departments:03 Faculty of Medicine > Bereich Medizinische Fächer (Klinik) > Nephrologie > Transplantationsimmunologie und Nephrologie (Steiger)
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Medizinische Fächer (Klinik) > Nephrologie > Transplantationsimmunologie und Nephrologie (Steiger)
03 Faculty of Medicine > Departement Biomedizin > Former Units at DBM > Immunonephrology (Schifferli)
03 Faculty of Medicine > Departement Biomedizin > Former Units at DBM > Transplantation Immunology and Nephrology (Palmer/Steiger)
UniBasel Contributors:Schifferli, Jürg A. and Steiger, Jürg U. and Dickenmann, Michael Jan and Schaub, Stefan
Item Type:Article, refereed
Article Subtype:Research Article
Bibsysno:Link to catalogue
Publisher:Oxford University Press
ISSN:0931-0509
Note:Publication type according to Uni Basel Research Database: Journal article
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Last Modified:01 Feb 2013 08:46
Deposited On:01 Feb 2013 08:41

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