儿童激素耐药型肾病综合征治疗进展  

Treatment progress of pediatric steroid-resistant nephrotic syndrome

作　　者：阳海平[1] 池欢 李秋[1] Yang Haiping;Chi Huan;Li Qiu(Department of Nephrology,Children′s Hospital of Chongqing Medical University,National Clinical Research Center for Child Health and Disorders,Ministry of Education Key Laboratory of Child Development and Disorders,Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China)

机构地区：[1]重庆医科大学附属儿童医院肾内科,国家儿童健康与疾病临床医学研究中心,儿童发育疾病研究教育部重点实验室,儿科学重庆市重点实验室,重庆400014

出　　处：《中华实用儿科临床杂志》2021年第17期1290-1295,共6页Chinese Journal of Applied Clinical Pediatrics

基　　金：国家自然科学基金(81970618);重庆市教委科学技术研究项目(KJZD-M201900401)。

摘　　要：原发性肾病综合征(PNS)是儿童最常见的肾小球疾病之一,足量泼尼松口服4周后如仍未缓解则为激素耐药型肾病综合征(SRNS)。根据是否存在单基因致病,分为特发性SRNS及遗传性SRNS。目前激素联合钙调神经磷酸酶抑制剂(CNI)是治疗特发性SRNS首选推荐治疗方案,50%~70%的患儿可达完全或部分缓解;少部分特发性SRNS及大部分遗传性SRNS患儿,可能对包括CNI在内2种作用机制的不同免疫抑制剂耐药,会在5~10年逐步进展致终末期肾病。现就儿童SRNS治疗进展进行阐述。Primary nephrotic syndrome(PNS)is one of the most common glomerular diseases in children.Steroid-resistant nephrotic syndrome(SRNS)is defined as no remission of nephrotic syndrome after daily Prednisone treatment at an adequate oral dose for 4 weeks,which is classified as idiopathic SRNS and inherited SRNS according to the presence or absence of monogenic disorder.At present,steroid combined with calcineurin inhibitor(CNI)is preferred to idiopathic SRNS,and 50%-70%of children can achieve complete or partial remission.A small proportion of children with idiopathic SRNS and the majority of inherited SRNS may be resistant to different immunosuppressive agents with two different mechanisms,including CNI.They,unfortunately,gradually progress to end-stage renal disease within 5-10 years.In this paper,the therapeutic advances and future prospects in pediatric SRNS are reviewed.

关 键 词：激素耐药型肾病综合征 免疫抑制剂 治疗 进展 儿童 

分 类 号：R726.9[医药卫生—儿科]