Journal article
An international consensus approach to the management of atypical hemolytic uremic syndrome in children
Pediatric nephrology (Berlin, West), Vol.31(1), pp.15-39
01/2016
DOI: 10.1007/s00467-015-3076-8
PMID: 25859752
Abstract
Atypical hemolytic uremic syndrome (aHUS) emerged during the last decade as a disease largely of complement dysregulation. This advance facilitated the development of novel, rational treatment options targeting terminal complement activation, e.g., using an anti-C5 antibody (eculizumab). We review treatment and patient management issues related to this therapeutic approach. We present consensus clinical practice recommendations generated by HUS International, an international expert group of clinicians and basic scientists with a focused interest in HUS. We aim to address the following questions of high relevance to daily clinical practice: Which complement investigations should be done and when? What is the importance of anti-factor H antibody detection? Who should be treated with eculizumab? Is plasma exchange therapy still needed? When should eculizumab therapy be initiated? How and when should complement blockade be monitored? Can the approved treatment schedule be modified? What approach should be taken to kidney and/or combined liver-kidney transplantation? How should we limit the risk of meningococcal infection under complement blockade therapy? A pressing question today regards the treatment duration. We discuss the need for prospective studies to establish evidence-based criteria for the continuation or cessation of anticomplement therapy in patients with and without identified complement mutations.
Details
- Title: Subtitle
- An international consensus approach to the management of atypical hemolytic uremic syndrome in children
- Creators
- Chantal Loirat - Service de Néphrologie pédiatrique [Hôpital Robert Debré, Paris]HUS InternationalGema Ariceta - Pediatric Nephrology [Barcelona, Spain]Nesrin Besbas - Department of Pediatric Nephrology [Ankara, Turkey]Martin Bitzan - Division of Nephrology [Montreal, Canada]Anna Bjerre - Department of Pediatrics [Oslo, Norway]Rosanna Coppo - Science of Turin Health Agency [Turin, Italy]Francesco Emma - Bambino Gesù Children’s Hospital [Rome, Italy]Sally Johnson - Department of Paediatric Nephrology [Newcastle Upon Tyne, UK]Diana Karpman - Department of Pediatrics [Lund, Sweden]Daniel Landau - Soroka University Medical Center [Beer Sheva, Israel]Craig Langman - Ann & Robert H. Lurie Children's Hospital of ChicagoAnne-Laure Lapeyraque - Service de Néphrologie [Montréal, Canada]Christoph Licht - The Hospital for sick children [Toronto]Carla Nester - the Stead Family Department of Pediatrics [Iowa City, Iowa, USA]Carmine Pecoraro - Santobono Children’s Hospital [Naples, Italy]Magdalena Riedl - Department of Pediatrics [Innsbruck, Austria]Nicole C.A.J van de Kar - Department of Pediatric Nephrology [Nijmegen, The Netherlands]Johan Van de Walle - Ghent University Hospital [Ghent, Belgium]Marina Vivarelli - Bambino Gesù Children’s Hospital [Rome, Italy]Véronique Frémeaux-Bacchi - Immunologie et Cancérologie IntégrativesHUS Int
- Resource Type
- Journal article
- Publication Details
- Pediatric nephrology (Berlin, West), Vol.31(1), pp.15-39
- DOI
- 10.1007/s00467-015-3076-8
- PMID
- 25859752
- NLM abbreviation
- Pediatr Nephrol
- ISSN
- 0931-041X
- eISSN
- 1432-198X
- Publisher
- Springer Verlag
- Language
- English
- Date published
- 01/2016
- Academic Unit
- Nephrology, Dialysis and Transplantation; Stead Family Department of Pediatrics; Internal Medicine
- Record Identifier
- 9984093359802771
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