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01-05-2013 | Editorial

Atypical HUS: time to take stock of current guidelines and outcome measures?

Author: Lesley Rees

Published in: Pediatric Nephrology | Issue 5/2013

Abstract

European guidelines for the assessment and management of atypical HUS were written in 2009. Since then our understanding of this group of diseases has advanced. Evidence is emerging that eculizumab, a monoclonal antibody inhibiting C5 activation, is effective, and potentially superior to current treatment with plasmapheresis. The evidence base for the benefits of plasmapheresis consists of case series and small reports. Before we embark on a change of management policy it is vital that we set up a system for genetic diagnosis, standardised protocols and a means to collect predetermined outcome measures, so that we do not make the same mistakes with assessment of the effectiveness of eculizumab as we did for plasmapheresis.

Taylor CM, Machin S, Wigmore SJ, Goodship THJ, on behalf of a working party from the Renal Association, the British Committee for Standards in Haematology and the British Transplant Society (2009) Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom. Br J Haematol 148:37–47PubMedCrossRef

Bu F, Borsa N, Gianluigi A, Smith RJH (2013) Familial atypical hemolytic uremic syndrome: a review of its genetic and clinical aspects. Clin Devel Immunol. doi:10.1155/2012/370426

Ariceta G, Besbas N, Johnson S, Karpman D, Landau D, Licht C, Loirat C, Pecoraro C, Taylor MC, Van de Kar N, VandeWalle J, Zimmerhackl LB, The European Paediatric Study Group for HUS (2009) Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 24:687–696PubMedCrossRef

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Clark WF (2012) Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange. Semin Dial 25:214–219PubMedCrossRef

Zuber J, Fakhouri F, Roumenina LT, Loirat C, Fremeaux-Bacchi V, on behalf of the French study group fo aHUS/C3G (2013) Use of eculizumab for atypical HUS and C3 glomerulopathies. Nat Rev Nephrol 8:643–657CrossRef

Gulleroglu K, Fidan K, Hançer VS3, Umut Bayrakci U, Baskin E, Soylemezoglu O (2013) Neurologic involvement in atypical hemolytic uremic syndrome and successful treatment with eculizumab. Pediatr Nephrol. doi:10.1007/s00467-013-2416-9

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Title: Atypical HUS: time to take stock of current guidelines and outcome measures?
Author: Lesley Rees
Publication date: 01-05-2013
Publisher: Springer-Verlag
Published in: Pediatric Nephrology / Issue 5/2013
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-013-2423-x

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Atypical HUS: time to take stock of current guidelines and outcome measures?

Abstract

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Abstract

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