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

Chantal Loirat 1, * Fadi Fakhouri 2, 3, 4 Gema Ariceta 5 Nesrin Besbas 6 Martin Bitzan 7 Anna Bjerre 8 Rosanna Coppo 9 Francesco Emma 10 Sally Johnson 11 Diana Karpman 12 Daniel Landau 13 Craig Langman 14 Anne-Laure Lapeyraque 15 Christoph Licht 16 Carla Nester 17 Carmine Pecoraro 18 Magdalena Riedl 19 Nicole C.A.J. van de Kar 20 Johan van de Walle 21 Marina Vivarelli 10 Véronique Frémeaux-Bacchi 22, 23
* Corresponding author
22 CRC - Inserm U1138 - Immunologie et Cancérologie Intégratives
CRC (UMR_S_1138 / U1138) - Centre de Recherche des Cordeliers
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.
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Chantal Loirat, Fadi Fakhouri, Gema Ariceta, Nesrin Besbas, Martin Bitzan, et al.. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatric Nephrology, Springer Verlag, 2016, 31 (1), pp.15-39. ⟨10.1007/s00467-015-3076-8⟩. ⟨inserm-02149335⟩



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