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 associated 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.