Abstract
Atypical hemolytic uremic syndrome (aHUS) is associated with a poorer prognosis, and an increased risk for end-stage renal disease (50%) or death (25%). There are about 50% of patients with aHUS asso-ciated with dysfunctional complement regulation.Eculizumab, a monoclonal anti-C5 antibody, prevents C5 cleavage and the formation of C5a and C5b-9, thus inhibiting complement activation via 3 pathways and its terminal product formation.Therefore, it is applied to treat complement-mediated aHUS in children and its application improved the prognosis of aHUS.Eculizumab has been recommended as first-line treatment in children with complement-mediated aHUS by an international consensus.In this paper, the application of Eculizumab in children with atypical hemolytic uremic syndrome are reviewed briefly. Key words: Atypical hemolytic uremic syndrome; Dysfunctional complement regulation; Eculizumab; Treatment; Child
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