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目的探讨激素耐药型肾病综合征(SRNS)患儿大剂量环磷酰胺(CTX)冲击治疗的疗效及影响疗效的相关因素。方法总结本院2004年12月-2009年12月收治的资料完整并进行CTX静脉冲击治疗的38例SRNS患儿的病例资料及随访情况,并就其缓解情况及与临床分型、病理类型、临床指标之间的关系及药物不良反应进行回顾性分析。结果 1.本组完全缓解18例(47%),部分缓解11例(29%),总有效率76%;部分缓解患儿均发生于疗程6个月内,延长疗程无累积缓解率增加;未缓解患儿1例在起病5 a内进展至终末期肾病。2.SRNS患儿单纯型肾病组缓解率高于肾炎型肾病组。3.SRNS病理改变以非微小病变肾病为主,即以系膜增生性肾小球肾炎(MsPGN)、局灶节段性肾小球硬化(FSGS)为主;其中MsPGN缓解率较高,而FSGS缓解率相对较低。4.不良反应发生率为53%,最常见不良反应为一过性胃肠道反应,占36%,其次为轻度脱发(10%)、白细胞降低(7%),无严重感染、出血性膀胱炎发生。结论 CTX冲击联合激素治疗SRNS疗效肯定;临床类型、病理类型等因素可能与疗效及预后有关,单纯型肾病、MsPGN有效率较高,FSGS患儿CTX冲击疗效较差,发展为终末期肾病的风险较高。
Objective To investigate the curative effect of high-dose cyclophosphamide (CTX) in children with steroid-resistant nephrotic syndrome (SRNS) and its related factors. Methods The data and follow-up of 38 children with SRNS who were treated by CTX venous impact in our hospital from December 2004 to December 2009 were analyzed retrospectively. And their clinical characteristics, clinical types, The relationship between clinical indicators and adverse drug reactions were retrospectively analyzed. Results 1. The complete remission in 18 cases (47%), partial remission in 11 cases (29%), the total effective rate was 76%; partial remission occurred in children within 6 months of treatment, extended treatment without cumulative response rate increased; One patient without remission progressed to end stage renal disease within 5 years of onset. 2.SRNS children with simple nephropathy remission rate was higher than nephritis nephropathy group. 3.SRNS pathological changes of non-minimal change of nephropathy, that mesangial proliferative glomerulonephritis (MsPGN), focal segmental glomerulosclerosis (FSGS) mainly; which MsPGN higher remission rate, and FSGS response rate is relatively low. 4. The incidence of adverse reactions was 53%, the most common adverse reactions were transient gastrointestinal reactions, accounting for 36%, followed by mild hair loss (10%), leukopenia (7%), no serious infection, hemorrhagic Cystitis occurs. Conclusions The CTX impact combined with hormone treatment of SRNS is positive. The clinical type and pathological type may be related to the curative effect and prognosis. Simple nephropathy and MsPGN are more effective. The CTX impact in children with FSGS is poor and the risk of developing end-stage renal disease Higher.