%0 Journal Article
%@ 1523-1755
%A Brocklebank, V
%A Johnson, S
%A Sheerin, TP
%A Marks, SD
%A Gilbert, RD
%A Tyerman, K
%A Kinoshita, M
%A Awan, A
%A Kaur, A
%A Webb, N
%A Hegde, S
%A Finlay, E
%A Fitzpatrick, M
%A Walsh, PR
%A Wong, EKS
%A Booth, C
%A Kerecuk, L
%A Salama, AD
%A Almond, M
%A Inward, C
%A Goodship, TH
%A Sheerin, NS
%A Marchbank, KJ
%A Kavanagh, D
%D 2017
%F discovery:1572803
%I ELSEVIER SCIENCE INC
%J Kidney International
%K acute kidney injury, complement, hemolytic uremic syndrome
%N 5
%P 1261-1271
%T Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland
%U https://discovery.ucl.ac.uk/id/eprint/1572803/
%V 92
%X Factor H autoantibodies can impair complement  regulation, resulting in atypical hemolytic uremic  syndrome, predominantly in childhood. There are no trials  investigating treatment, and clinical practice is only  informed by retrospective cohort analysis. Here we  examined 175 children presenting with atypical hemolytic  uremic syndrome in the United Kingdom and Ireland for  factor H autoantibodies that included 17 children with  titers above the international standard. Of the 17, seven  had a concomitant rare genetic variant in a gene encoding  a complement pathway component or regulator. Two  children received supportive treatment; both developed  established renal failure. Plasma exchange was associated  with a poor rate of renal recovery in seven of 11 treated. Six  patients treated with eculizumab recovered renal function.  Contrary to global practice, immunosuppressive therapy to  prevent relapse in plasma exchange–treated patients was  not adopted due to concerns over treatment-associated  complications. Without immunosuppression, the relapse  rate was high (five of seven). However, reintroduction of  treatment resulted in recovery of renal function. All  patients treated with eculizumab achieved sustained  remission. Five patients received renal transplants without  specific factor H autoantibody–targeted treatment with  recurrence in one who also had a functionally significant CFI mutation. Thus, our current practice is to initiate  eculizumab therapy for treatment of factor H  autoantibody–mediated atypical hemolytic uremic  syndrome rather than plasma exchange with or without  immunosuppression. Based on this retrospective analysis  we see no suggestion of inferior treatment, albeit the  strength of our conclusions is limited by the small sample size
%Z Copyright © 2017, International Society of Nephrology. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).