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Eculizumab in the treatment of Shiga toxin haemolytic uraemic syndrome

Lookup NU author(s): Dr Patrick Walsh, Dr Sally Johnson



This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


Haemolytic uraemic syndrome (HUS) remains a leading cause of paediatric acute kidney injury (AKI). Haemolytic uraemic syndrome is characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia and AKI. In ~?90% of cases, HUS is a consequence of infection with Shiga toxin-producing E. coli (STEC), most commonly serotype O157:H7. Acute mortality from STEC-HUS is now less than 5%; however, there is significant long-term renal morbidity in one third of survivors. Currently, no specific treatment exists for STEC-HUS. There is growing interest in the role of complement in the pathogenesis of STEC-HUS due to the discovery of inherited and acquired dysregulation of the alternative complement system in the closely related disorder, atypical HUS (aHUS). The treatment of aHUS has been revolutionised by the introduction of the anti-C5 monoclonal antibody, eculizumab. However, the role of complement and anti-complement therapy in STEC-HUS remains unclear. Herein, we review the current evidence of the role of complement in STEC-HUS focusing on the use of eculizumab in this disease.

Publication metadata

Author(s): Walsh PR, Johnson S

Publication type: Review

Publication status: Published

Journal: Pediatric Nephrology

Year: 2019

Volume: 34

Issue: 9

Pages: 1485-1492

Print publication date: 01/09/2019

Online publication date: 30/07/2018

Acceptance date: 11/07/2018

ISSN (print): 0931-041X

ISSN (electronic): 1432-198X


DOI: 10.1007/s00467-018-4025-0