Titre
Atypical hemolytic uremic syndrome: from the rediscovery of complement to targeted therapy
Type
article
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Auteur(s)
Fakhouri, F.
Auteure/Auteur
Fremeaux-Bacchi, V.
Auteure/Auteur
Loirat, C.
Auteure/Auteur
Liens vers les personnes
Liens vers les unités
ISSN
1879-0828
Statut éditorial
Publié
Date de publication
2013-09
Volume
24
Numéro
6
Première page
492
Dernière page/numéro d’article
5
Langue
anglais
Notes
Fakhouri, Fadi
Fremeaux-Bacchi, Veronique
Loirat, Chantal
eng
Review
Netherlands
Eur J Intern Med. 2013 Sep;24(6):492-5. doi: 10.1016/j.ejim.2013.05.008. Epub 2013 Jun 5.
Fremeaux-Bacchi, Veronique
Loirat, Chantal
eng
Review
Netherlands
Eur J Intern Med. 2013 Sep;24(6):492-5. doi: 10.1016/j.ejim.2013.05.008. Epub 2013 Jun 5.
Résumé
Atypical hemolytic uremic syndrome (aHUS) is a devastating form of renal thrombotic microangiopathy. In the last five years, we have finally witnessed a dramatic improvement in the management of aHUS patients, and three breakthroughs in our understanding of aHUS have led to such an improvement. The first breakthrough was the emergence of a new clinical picture of aHUS (frequency of adult cases, and overall poor renal prognosis despite plasma therapy). The second breakthrough was the identification of complement alternative pathway dysregulation as a major risk factor for aHUS. The third breakthrough was the availability in clinical practice of the first complement inhibitor, the anti-C5 monoclonal antibody, eculizumab. Available data from case series and prospective studies indicate that eculizumab use has dramatically improved the renal prognosis of aHUS. These breakthroughs have prompted the French working group on aHUS to propose a new algorithm for the management of aHUS in children and in adults. This algorithm will evolve as we gain new insights in the pathogenesis and evolution of aHUS in the eculizumab era.
PID Serval
serval:BIB_F58C4C20E4EF
PMID
Date de création
2022-03-01T09:18:09.383Z
Date de création dans IRIS
2025-05-21T05:44:58Z