环磷酰胺治疗儿童激素耐药性肾病综合征的临床观察

目的了解大剂量环磷酰胺冲击治疗激素耐药型肾病综合征(SRNS)儿童的临床特点、病理、治疗及随访情况。方法对该院2006年6月—2011年6月进行环磷酰胺(CTX)冲击治疗的20例SRNS患儿病例资料进行回顾性分析。结果①本组完全缓解13例(65%),部分缓解2例(10%),总有效率75%;②CTX冲击治疗SRNS患儿有效组与无效组间性别、24h尿蛋白定量、血清白蛋白、血清胆固醇、肌酐水平均无差异(P>0.05),而无效组发病年龄较有效组小(P<0.05);③SRNS患儿单纯型缓解率高于肾炎型(P<0.05);④SRNS病理改变以MCN、MsPGN、FSGS为主,MCN及MsPGN缓解率较高。⑤毒副作用:本组11例(55%)未见明显CTX毒副作用,8例(40%)副作用是呕吐反应,其次为白细胞减少1例(5%)。远期副作用因随访时间短未评估。结论 CTX冲击治疗SRNS患儿总有效率为75%;可能多种因素共同影响治疗效果。

Clinic observation of cyclophosphamide pulse therapy in children with steroid- resistant nephrotic syndrome

Objective To investigate the clinical characteristics, pathology, treatment and follow-up of intravenous cyclophosphamide （CTX）pulse therapy in children with steroid-resistant nephrotic syndrome （SRNS）. Methods The clinical data of 20 children with SRNS with CTX pulse therapy were retrospectively analyzed between June 2006 and June 2011. Results ①13 cases （65%）got complete remission and 2 cases （29%）got partial remission, so the total effective rate of this therapy was 75%; ②）The study showed that there was no difference between the sex, the levels of ALB, Ch, creatinine and excretions of 24h urinary protein in the effective group and ineffective group （P 〉 0.05）, but compared with effective group, the age of onset in ineffective group was younger （P 〈 0.05） ; ③In the children with SRNS, the simple nephrotic syndrome had the higher remission rate than nephritis nephrotic syndrome （P 〈 0.05） ; ④）The primary renal pathology were minimal change nephritis （MCN）, mesangial proliferative glomcrulonephritis （MsPGN） and focal segmental glomerulosclerosis （FSGS）, and MCN and MsPGN had higher remission rate.⑤11 cases （55%）was found to have no side effect. Nausea and the decrease of white blood cells occurred at 40% and 5%, respectively. Because of a short follow-up period, long-term side effects were not evaluated. Conclusion The total efficacy of CTX pulse therapy in children with SRNS was 75%. There may be many factors to affect the treatment effect of CTX pulse therapy.