Application of Eculizumab in children with atypical hemolytic uremic syndrome
10.3760/cma.j.issn.2095-428X.2018.17.017
- VernacularTitle:依库珠单抗在儿童非典型溶血尿毒综合征中的应用
- Author:
Tingting XIE
1
;
Zihua YU
Author Information
1. 福建医科大学福总临床医学院儿科
- Keywords:
Atypical hemolytic uremic syndrome;
Dysfunctional complement regulation;
Eculizumab;
Treatment;
Child
- From:
Chinese Journal of Applied Clinical Pediatrics
2018;33(17):1351-1353
- CountryChina
- Language:Chinese
-
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.
