依库珠单抗在儿童非典型溶血尿毒综合征中的应用  

Application of Eculizumab in children with atypical hemolytic uremic syndrome

作　　者：谢婷婷 余自华[1] Xie Tingting;Yu Zihua(Department of Pediatrics,Fuzhou General Hospital,Fujian Medical University of Fu General Clinical Medical College,Fuzhou 350025,China)

机构地区：[1]福州总医院儿科、福建医科大学福总临床医学院儿科,350025

出　　处：《中华实用儿科临床杂志》2018年第17期1351-1353,共3页Chinese Journal of Applied Clinical Pediatrics

摘　　要：非典型溶血尿毒综合征（aHUS）预后差，50％的患者进展至终末期肾脏疾病或25％的患者死亡。大约50％的aHUS为补体旁路途径失调aHUS。抗补体C5单克隆抗体依库珠单抗可通过阻止C5裂解及C5a、C5b-9（膜攻击复合物）形成，抑制3种途径的补体活化过程及其终末产物生成而被应用于治疗儿童补体旁路途径失调aHUS，其应用改善了aHUS的预后。国际共识推荐依库珠单抗作为治疗儿童补体旁路途径失调aHUS的一线治疗方案。现就依库珠单抗在儿童补体旁路途径失调aHUS治疗中的应用做简要综述。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 Csa 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.

关 键 词：非典型溶血尿毒综合征 补体旁路途径失调 依库珠单抗 治疗 儿童 

分 类 号：R726.9[医药卫生—儿科]