Pediatric Nephrology

, Volume 31, Issue 1, pp 15–39 | Cite as

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

  • Chantal Loirat
  • Fadi Fakhouri
  • Gema Ariceta
  • Nesrin Besbas
  • Martin Bitzan
  • Anna Bjerre
  • Rosanna Coppo
  • Francesco Emma
  • Sally Johnson
  • Diana Karpman
  • Daniel Landau
  • Craig B Langman
  • Anne-Laure Lapeyraque
  • Christoph Licht
  • Carla Nester
  • Carmine Pecoraro
  • Magdalena Riedl
  • Nicole C. A. J. van de Kar
  • Johan Van de Walle
  • Marina Vivarelli
  • Véronique Frémeaux-Bacchi
  • for HUS International
Review

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 

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Copyright information

© IPNA 2015

Authors and Affiliations

  • Chantal Loirat
    • 1
  • Fadi Fakhouri
    • 2
  • Gema Ariceta
    • 3
  • Nesrin Besbas
    • 4
  • Martin Bitzan
    • 5
  • Anna Bjerre
    • 6
  • Rosanna Coppo
    • 7
  • Francesco Emma
    • 8
  • Sally Johnson
    • 9
  • Diana Karpman
    • 10
  • Daniel Landau
    • 11
  • Craig B Langman
    • 12
  • Anne-Laure Lapeyraque
    • 13
  • Christoph Licht
    • 14
  • Carla Nester
    • 15
  • Carmine Pecoraro
    • 16
  • Magdalena Riedl
    • 17
  • Nicole C. A. J. van de Kar
    • 18
  • Johan Van de Walle
    • 19
  • Marina Vivarelli
    • 8
  • Véronique Frémeaux-Bacchi
    • 20
  • for HUS International
  1. 1.Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, Department of Pediatric NephrologyUniversité Paris Diderot Sorbonne Paris CitéParisFrance
  2. 2.Centre Hospitalier Universitaire de Nantes, Department of Nephrology and ImmunologyITUN and INSERM UMR S-1064NantesFrance
  3. 3.Pediatric Nephrology, Vall d’Hebron HospitalAutonoma University of BarcelonaBarcelonaSpain
  4. 4.Department of Pediatric NephrologyHacettepe University Faculty of MedicineAnkaraTurkey
  5. 5.Division of NephrologyMontreal Children’s Hospital and Mc Gill UniversityMontrealCanada
  6. 6.Department of PediatricsOslo University HospitalOsloNorway
  7. 7.City of the Health and the Science of Turin Health AgencyRegina Margherita Children’s HospitalTurinItaly
  8. 8.Bambino Gesù Children’s HospitalRomeItaly
  9. 9.Department of Paediatric NephrologyGreat North Children’s HospitalNewcastle Upon TyneUK
  10. 10.Department of Pediatrics, Clinical SciencesLund UniversityLundSweden
  11. 11.Soroka University Medical CenterBeer ShevaIsrael
  12. 12.The Ann and Robert H. Lurie Children’s Hospital of Chicago, Feinberg School of MedicineNorthwestern UniversityChicagoUSA
  13. 13.Service de Néphrologie, Département de Pédiatrie, CHU Sainte-JustineUniversité de MontréalMontréalCanada
  14. 14.The Hospital for Sick ChildrenTorontoCanada
  15. 15.Departments of Internal Medicine and the Stead Family Department of PediatricsUniversity of IowaIowa CityUSA
  16. 16.Santobono Children’s HospitalNaplesItaly
  17. 17.Department of PediatricsInnsbruck Medical UniversityInnsbruckAustria
  18. 18.Department of Pediatric Nephrology, Amalia Children’s HospitalRadboud University Medical CenterNijmegenThe Netherlands
  19. 19.University Hospital GhentGhentBelgium
  20. 20.Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Laboratory of ImmunologyINSERM UMRS 1138ParisFrance

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