Toggle Main Menu Toggle Search

Open Access padlockePrints

Plasma therapy in atypical haemolytic uremic syndrome: Lessons from a family with a factor H mutation

Lookup NU author(s): Dr Lisa Turnbull, Professor Tim Goodship


Full text for this publication is not currently held within this repository. Alternative links are provided below where available.


Whilst randomised control trials are undoubtedly the best way to demonstrate whether plasma exchange or infusion alone is the best first-line treatment for patients with atypical haemolytic uremic syndrome (aHUS), individual case reports can provide valuable information. To that effect, we have had the unique opportunity to follow over a 10-year period three sisters with aHUS associated with a factor H mutation (CFH). Two of the sisters are monozygotic twins. A similar natural evolution and response to treatment would be expected for the three patients, as they all presented with the same at-risk polymorphisms for CFH and CD46 and no identifiable mutation in either CD46 or CFI. Our report of different modalities of treatment of the initial episode and of three transplantations and relapses in the transplant in two of them, strongly suggest that intensive plasma exchange, both acutely and prophylactically, can maintain the long-term function of both native kidneys and allografts. In our experience, the success of plasma therapy is dependent on the use of plasma exchange as opposed to plasma infusion alone, the prolongation of daily plasma exchange after normalisation of haematological parameters followed by prophylactic plasma exchange, the use of prophylactic plasma exchange prior to transplantation and the use of prophylactic plasma exchange at least once a week posttransplant with immediate intensification of treatment if there are any signs of recurrence. © The Author(s) 2008.

Publication metadata

Author(s): Davin JC, Strain L, Goodship THJ

Publication type: Article

Publication status: Published

Journal: Pediatric Nephrology

Year: 2008

Volume: 23

Issue: 9

Pages: 1517-1521

ISSN (print): 0931-041X

ISSN (electronic): 1432-198X

Publisher: Springer


DOI: 10.1007/s00467-008-0833-y


Altmetrics provided by Altmetric