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Pediatr Nephrol. 2016 Jan;31(1):15-39. doi: 10.1007/s00467-015-3076-8. Epub 2015 Apr 11.

An international consensus approach to the management of atypical hemolytic uremic syndrome in children.

Author information

1
Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, Department of Pediatric Nephrology, Université Paris Diderot Sorbonne Paris Cité, Paris, France. chantal.loirat@rdb.aphp.fr.
2
Centre Hospitalier Universitaire de Nantes, Department of Nephrology and Immunology, ITUN and INSERM UMR S-1064, Nantes, France.
3
Pediatric Nephrology, Vall d'Hebron Hospital, Autonoma University of Barcelona, Barcelona, Spain.
4
Department of Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
5
Division of Nephrology, Montreal Children's Hospital and Mc Gill University, Montreal, Canada.
6
Department of Pediatrics, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
7
City of the Health and the Science of Turin Health Agency, Regina Margherita Children's Hospital, Turin, Italy.
8
Bambino Gesù Children's Hospital, Rome, Italy.
9
Department of Paediatric Nephrology, Great North Children's Hospital, Newcastle Upon Tyne, UK.
10
Department of Pediatrics, Clinical Sciences, Lund University, Lund, Sweden.
11
Soroka University Medical Center, Beer Sheva, Israel.
12
The Ann and Robert H. Lurie Children's Hospital of Chicago, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
13
Service de Néphrologie, Département de Pédiatrie, CHU Sainte-Justine, Université de Montréal, Montréal, Canada.
14
The Hospital for Sick Children, Toronto, Canada.
15
Departments of Internal Medicine and the Stead Family Department of Pediatrics, University of Iowa, Iowa City, Iowa, USA.
16
Santobono Children's Hospital, Naples, Italy.
17
Department of Pediatrics, Innsbruck Medical University, Innsbruck, Austria.
18
Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
19
University Hospital Ghent, Ghent, Belgium.
20
Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Laboratory of Immunology, INSERM UMRS 1138, Paris, France.

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.

KEYWORDS:

Anti-factor H antibody; Atypical hemolytic uremic syndrome; Children; Combined liver–kidney transplantation; Complement; Eculizumab; Hemolytic uremic syndrome; Kidney transplantation; Plasma exchange; Plasma infusion; Thrombotic microangiopathy

PMID:
25859752
DOI:
10.1007/s00467-015-3076-8
[Indexed for MEDLINE]

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