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Changing strategies for organ transplantation in atypical haemolytic uraemic syndrome: a tertiary case series.

Authors :
Forbes TA
Bradbury MG
Goodship TH
McKiernan PJ
Milford DV
Source :
Pediatric transplantation [Pediatr Transplant] 2013 May; Vol. 17 (3), pp. E93-9. Date of Electronic Publication: 2013 Mar 06.
Publication Year :
2013

Abstract

We present three cases of organ transplantation for atypical haemolytic uraemic syndrome secondary to complement factor H mutation: one isolated renal transplant; one previously reported isolated liver transplant; and one combined liver and kidney transplant. All three patients were treated prior to the licensing of eculizumab for this condition, and all have had favourable outcomes with maintenance of graft function for years following transplantation. We discuss the evolution of transplantation therapy for aHUS over the last two decades. Transplantation decision-making in aHUS has evolved over this time with expanding knowledge of pathophysiology and genetics, alongside refined plasma exchange and anticoagulation protocols and improved centre experience. Our cases demonstrate how individual patient factors within this heterogeneous condition also underlie transplantation decisions and outcomes. Whilst our cases demonstrate that transplantation in aHUS can be a successful long-term treatment providing good quality of life, worldwide experience has proven that most curative treatment for aHUS strategies represents significant risks. Whether new pharmacotherapies such as eculizumab will alter this risk is yet to be determined.<br /> (© 2013 John Wiley & Sons A/S.)

Details

Language :
English
ISSN :
1399-3046
Volume :
17
Issue :
3
Database :
MEDLINE
Journal :
Pediatric transplantation
Publication Type :
Academic Journal
Accession number :
23461281
Full Text :
https://doi.org/10.1111/petr.12066