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目的:采用小剂量环孢霉素A(CyclospOrin A,CsA)治疗小儿难治性肾病综合征15例,观察其疗效及其副作用。方法:口服环孢霉素A按3～5mg/kg·d,分别于用药一周末和二周末以及用药后每月一次测定血环孢霉素A的浓度(用单克隆抗体法测定),适当调整剂量,维持血药浓度在100～200μg/ml之间,3个月后减为2.5mg/kg·d,维持用药9个月后逐渐减量至停药,总疗程12个月。结果:完全缓解8例,部分缓解4例,无效3例,总有效率达80%(12/15),平均起效时间15天(10～30天),其中对微小病变及系膜增殖性肾小球肾炎疗效显著,对局灶节段性肾小球硬化、膜性增殖性肾小球肾炎、膜性肾病亦有一定疗效,随访1～2年,平均18个月,2例复发(2/15)占13.3%。副作用有血压偏高、高尿酸血症、轻度恶心呕吐厌食、轻度毛发增多、手抖动、齿龈肿胀,未出现肝肾毒性表现。结论:小剂量CsA治疗小儿难治性肾病综合征具有近期疗效好,副作用小,患者耐受良好,并发感染率低等优点,对于使用其它免疫抑制剂疗效差或病人不能耐受、副作用大的病例临床值得应用。对于该药的远期疗效,副作用均有待于进一步探讨。
Objective: To treat 15 children with intractable nephrotic syndrome with low dose cyclosporin A (CsA), and to observe its curative effect and its side effects. Methods: The concentration of cyclosporine A (measured by monoclonal antibody method) was determined by oral cyclosporin A at 3 ~ 5mg / kg · d once a month and two weeks after treatment and once a month after administration. The appropriate Adjust the dose to maintain plasma concentration between 100 ~ 200μg / ml, after 3 months reduced to 2.5mg / kg · d, maintenance medication gradually reduced to withdrawal after 9 months, the total course of 12 months. Results: The complete remission in 8 cases, partial remission in 4 cases, 3 cases, the total effective rate was 80% (12/15), the average onset time of 15 days (10 to 30 days), of which minimal changes and mesangial proliferative Glomerulonephritis significant effect on focal segmental glomerulosclerosis, membranous proliferative glomerulonephritis, membranous nephropathy also have a certain effect, followed up for 1 to 2 years, an average of 18 months, 2 cases of recurrence ( 2/15) accounted for 13.3%. Side effects of high blood pressure, hyperuricemia, mild nausea and vomiting, anorexia, mild hair loss, hand jitter, swollen gums, liver and kidney toxicity did not appear. Conclusion: Small dose CsA treatment of children with refractory nephrotic syndrome with short-term curative effect, small side effects, patients with good, complicated with low infection rate, for the use of other immunosuppressive agents poor or patients can not tolerate side effects Clinical cases worth applying. For the long-term efficacy of the drug, side effects have yet to be further explored.