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Case Reports in Nephrology
Volume 2018, Article ID 2781789, 4 pages
Case Report

Clinical Relapses of Atypical HUS on Eculizumab: Clinical Gap for Monitoring and Individualised Therapy

1Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada
2Department of Paediatric Nephrology & Transplantation, The Children’s University Hospital, Temple Street, Dublin 1, Ireland
3Department of Paediatrics, University of Toronto, Toronto, ON, Canada
4Department of Neurology, The Children’s University Hospital, Temple Street, Dublin 1, Ireland
5Institute of Genetic Medicine, Newcastle University, International Centre for Life, Central Parkway, Newcastle upon Tyne, UK
6Department of Paediatric Nephrology, Our Lady’s Children’s Hospital, Crumlin, Dublin 12., Ireland

Correspondence should be addressed to Chia Wei Teoh; ac.sdikkcis@hoet.iewaihc

Received 25 November 2017; Accepted 4 January 2018; Published 6 February 2018

Academic Editor: Salih Kavukcu

Copyright © 2018 Chia Wei Teoh et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Atypical hemolytic uremic syndrome (aHUS) is caused by dysregulation of the complement system. A humanised anti-C5 monoclonal antibody (eculizumab) is available for the treatment of aHUS. We present the first description of atypical HUS in a child with a coexistent diagnosis of a POL-III leukodystrophy. On standard eculizumab dosing regime, there was evidence of ongoing C5 cleavage and clinical relapses when immunologically challenged. Eculizumab is an effective therapy for aHUS, but the recommended doses may not be adequate for all patients, highlighting the need for ongoing efforts to develop a strategy for monitoring of treatment efficacy and potential individualisation of therapy.