儿童激素抵抗、依赖及复发肾病综合征的治疗

Treatment of steroid-resistant, steroid-dependent and relapse nephrotic syndrome in children

原发性肾病综合征（PNS）是儿童最常见的肾小球疾病。糖皮质激素治疗PNS始于20世纪50年代，并一直是国内外治疗PNS公认的首选药物。80％～90％的PNS患儿初始激素治疗可获得完全缓解，其中10％～20％不复发，10％～20％为非频复发，40％～50％为频复发（FRNS）或激素依赖（SDNS）。约有1／3的FRNS或SDNS患儿因长期或反复使用激素而出现严重的副作用，如生长抑制、高血压、糖尿病、骨质疏松、青光眼、白内障，甚至失明等。10％～20％的PNS儿童表现为耐药（SRNS），超过半数的SRNS儿童在10年内会进展为终末期肾病（ESRD）。因此，FRNS、SDNS和SRNS儿童成为了临床关注的主要对象。笔者系统回顾了近年来国内外关于儿童肾病综合征临床治疗研究、Mate分析和国内外关于肾病综合征治疗指南的文献，对FRNS、SDNS和SRNS糖皮质激素、免疫抑制剂及其他药物的治疗效果进行综合分析，以供临床儿科医师参考。

Primary nephrotie syndrome （PNS） is the most common glomerular disease in children. Treatment of glucocorticoid （GC） for PNS began in 1950. GC is always the first line drug for PNS since then. 80% to 90% of the children with PNS respond to steroid treatment, among which, 10% to 20% have no relapse, 10% to 20% show infrequent relapse, 40% to 50% present with frequent relapse nephrotic syndrome （FRNS） or steroid dependence nephrotic syndrome （SDNS）. 1/3 children with frequent relapse or steroid dependent SDNS show serious side effect because of prolonged and repeated use of GC, such as growth inhibition, hypertension, diabetes, osteoporosis, glaucoma, cataract, and even blindness. 10% to 20% of the children with PNS have steroid resistance nephrotic syndrome （SRNS）, and more than half of the patients progress to end-stage renal disease nephrotic syndrome （ESRD） within 10 years. Therefore,we should pay more attention to children with FRNS, SDNS and SRNS. We reviewed most literatures on clinical trial, meta-analysis and guideline in treatment of NS. We summarized treatment of FRNS, SDNS and SRNS in children for pediatrics or children nephrologists.