激素耐药肾病综合症环磷酰胺冲击治疗疗效观察-第三军医大学学报.DOC

激素耐药肾病综合征环磷酰胺冲击治疗疗效分析 李少军，李 秋，阳海平，陶红，范忠祥，李国安 (400014重庆，重庆医科大学附属儿童医院肾脏免疫科) 摘要 目的 了解儿童激素耐药性肾病（SRNS）患儿大剂量环磷酰胺（CTX）冲击治疗疗效及影响疗效之相关因素。方法 总结我院近年资料完整共38例进行CTX静脉冲击治疗SRNS患儿病例资料，就其缓解情况及与临床分型、病理类型、临床指标之间关系及药物副作用进行回顾性分析。结果 （1）本组完全缓解18例（47%），部分缓解11例（29%）,总有效率76%；完全缓解患儿均发生于疗程6月内，延长疗程无累积缓解率增加。（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MsPGN、FSGS为主，MsPGN缓解率较高。（4）最常见副作用表现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%）。结论 CTX冲击联合激素治疗SRNS患儿疗效肯定；临床类型、病理类型等因素与疗效有关，单纯型、系膜增生型SRNS有效率较高。 关键词 激素耐药肾病综合征 环磷酰胺 儿童 Effect and influencing factors of cyclophosphamide pulse therapy in children with steroid-resistant nephrotic syndrome LI Shaojun， LI Qiu ，YANG Haiping, TAO Hong , FAN Zhongxiang, LI Guoan (Department of Kindey immunity ，the Children’s Hospital Affiliated to Chongqing Medical University， Chongqing400014，China) ［ABSTRACT］ Objective ：To investigate the effect and relevant influencing factors of intravenous cyclophosphamide(CTX) pulse therapy in children with steroid-resistant nephrotic syndrome(SRNS). Methods：CTX pulse therapy was given to 38 children with SRNS between December 2004 and December 2009 and the relevant date on clinical,pathological and laboratory test were collected and analyzed. Results：(1) Eighteen cases(47%) got complete remission and 13 cases(29%) got partial remission, so the total effica of this therapy was 76%. All the cases obtained complete remission got remission in 6 month of beginning therpy and there is no effect of increasing remission to extend the course. (2) In the children with SRNS, the simple nephrotic syndrome(SNS) had the higher remission rate than nephritis nephrotic syndrome(NNS). (3) The initial renal histology showed mesangial proliferative glomerulonephritis(MsPGN) and focal segmental glomerulosclerosis(FSGS), and MsPGN was higher remission rate. (3) Twenty cases(53%) was found to have side effect. Nausea and voimting, losing hairs and the decrease of white blood cells occurred at 36%, 10% and 7%, respectively. These side effects were transient and could