泼尼松联合大剂量环磷酰胺治疗壮族儿童难治性肾病综合征的疗效及远期随访  

Short and Long-Term Effects of Prednisone Combined with High-Dose Cyclophosphamide Approach of Different Treatments on Refractory Nephrotic Syndrome in Zhuang Children

作　　者：黄必政[1,2] 林娜[1] 刘运广[1] 莫锦丽[1] 黄月艳[1] 陈霞静[1] 李强[1] 

机构地区：[1]右江民族医学院附属医院儿科,广西百色533000 [2]巴马县人民医院儿科,广西巴马547500

出　　处：《实用儿科临床杂志》2009年第17期1317-1319,1324,共4页Journal of Applied Clinical Pediatrics

基　　金：国家自然科学基金项目资助(30460137);广西自然科学基金项目资助(桂科自0229061);广西科学研究与技术开发计划项目资助(桂科基0141025);广西青年科学基金项目资助(桂科青0542071);广西卫生厅科研基金项目资助(9955)

摘　　要：目的探讨大剂量环磷酰胺(CTX)冲击治疗壮族儿童难治性肾病综合征(RNS)的疗效及远期对性腺的损害。方法采用前瞻性随机方法,通过查随机数字表法将患儿分为CTX连续冲击治疗组(A组)和CTX非连续冲击治疗组(B组)。A组予CTX8～12 mg/(kg.次)加入9 g/L盐水100 mL静脉滴注1～2 h,1次/d,连用8～10次,累积量<120 mg/kg,同时予泼尼松2 mg/(kg.d)口服,尿蛋白转阴4周后,泼尼松改为2 mg/(kg.d),隔日早餐后顿服,连用4周后,每2～4周减量2.5～5.0 mg,总疗程4～6个月。B组予CTX8～12 mg/(kg.次)加入9 g/L盐水100 mL静脉滴注1～2 h,1次/d,连用2 d为1个周期,每4周应用1个周期,连用6～8个周期为1个疗程,累积量<200 mg/kg,泼尼松用法同A组。随访3～17 a。结果1.A组临床治愈率(CCR)为73.60%,完全缓解(CR)率为18.42%,部分缓解(PR)率为5.26%;B组CCR为53.66%,CR率为21.95%,PR率为19.51%,2组临床疗效比较差异有统计学意义(P<0.05)。2.A组冲击治疗1 a后血浆Alb提高和24 h尿蛋白降低较B组快,差异均有统计学意义(P<0.01),2～3 a血浆Alb仍然较B组高,差异有统计学意义(P<0.01),24 h尿蛋白比较差异无统计学意义(P>0.05)。3.二组医疗费用比较差异有统计学意义(P<0.01)。4.二组疗效与其临床分类比较差异均无统计学意义(Pa>0.05),A组不同病理类型间疗效比较差异无统计学意义(P>0.05),B组不同病理类型间疗效比较差异有统计学意义(P<0.01),2组近期不良反应比较差异均无统计学意义(Pa>0.05),远期随访结果比较差异无统计学意义(P>0.05)。结论2种CTX冲击联合泼尼松治疗方法均对壮族儿童RNS有较好疗效,CTX连续冲击治疗较CTX非连续冲击治疗可更快提高血浆Alb水平,使尿蛋白转阴更快,提高CCR,医疗费用低,CTX冲击治疗累积总量<80～200 mg/kg,近期安全,远期未发现性腺损害。Objective To explore the effects of high - dose cyclophosphamide （CTX） stosstherapy on refractory nephrotic syndrome （RNS） and long - term damage to gonad among Zhuang children. Methods The cases were divided into 2 groups, namely continuous CTX - stosstherapy group（ group A） receiving CTX （8 -12 mg/kg, iv, once a day for 8 -10 days with 〈 120 mg/kg- accumulated dose） and prednisone （2 mg/kg orally once a day till urine protein analysis with negative results following 2 mg/kg orally once per two days on weeks 4, subsequently 2 mg/kg with 2.5 - 5.0 mg decrement orally every 2 - 4 weeks, 4 - 6 months - accumulated course of treatment） therapy and non - continuous CTX - stosstherapy group （ group B ） receiving CTX （ 8 - 12 mg/kg, iv, once a day for 2 days, every 4 weeks with 〈 200 mg/kg - accumulated dose） and aforementioned prednisone therapy by means of randomized method. Follow up lasted for 3 - 17 years. Results 1. Therapeutic effects of group A with 73.60% clinic - cure rate （CCR） , 18.42% complete remission（CR） ,and 5.26% partial remission（PR） were better than those in group B（ CCR,CR and PR were 53.66% ,21.95% and 5.26% ,respectively） ,which had significant difference （P 〈 0.05 ）. 2. The cases from group A showed more plasma Alb in 1 -3 years after treatment, compared with those from group B （ P 〈 0.01 ）. The number of 24 h urine protein in 1 year after treatment in group A was less than that in group B （ P 〈 0.05 ） ; however, there was no significant difference of the number of 24 h urine protein in 2 years and 3 years after treatment between 2 groups（ P 〉 0.05 ）. 3. Additionally, there was significant difference for medical expenses （ P 〈 0.01 ）. 4. Moreover, therapeutic effects of group A were not associated with clinical or pathological types of RNS （ P 〉 0.05 ） ; while therapeutic effects of group B were related to pathological types of RNS （ P 〈 0.01 ）, but not to clinical types of RNS （ Pa 〉 0.05 ）. There wa

关 键 词：肾病综合征 环磷酰胺 壮族 儿童 

分 类 号：R726.9[医药卫生—儿科]