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目的观察环孢素A(CSA)治疗皮质激素耐药的儿童局灶节段性肾小球硬化(FSGS)的疗效。方法1997年6月至2004年10月中山大学附属第一医院儿科收治皮质激素耐药的FSGS患儿14例,应用CSA进行治疗,开始剂量为5mg/(kg.d),以后根据CSA血质量浓度调整剂量,维持CSA血质量浓度在150~300μg/L。有效者用药6个月至1年后逐渐减量。结果完全缓解10例(71.4%),部分缓解2例(14.3%),无效2例(14.3%),CSA治疗皮质激素耐药的FSGS总有效率达85.7%。CSA治疗期间泼尼松使用剂量减少。完全缓解10例中6例停药,其中5例复发,4例再用CSA治疗,尿蛋白再次转阴,1例放弃治疗。1例原有肾功能不全者治疗后血肌酐进行性升高。结论CSA治疗皮质激素耐药的FSGS有一定的疗效,但停药后复发率高,治疗中需注意CSA副反应。
Objective To investigate the efficacy of cyclosporin A (CSA) in the treatment of focal segmental glomerulosclerosis (FSGS) in children with corticosteroid resistance. Methods From June 1997 to October 2004, 14 children with steroid-resistant FSGS in the pediatric department of First Affiliated Hospital of Sun Yat-sen University were treated with CSA and the initial dose was 5 mg / (kg.d) .According to CSA blood quality Concentration adjustment dose, maintain CSA blood mass concentration of 150 ~ 300μg / L. The effective dose of 6 months to 1 year after gradual reduction. Results The complete response was achieved in 10 cases (71.4%), partial response in 2 cases (14.3%) and ineffectiveness in 2 cases (14.3%). The overall response rate of FSGS to steroid resistant FSGS was 85.7%. Prednisone use dose reduction during CSA treatment. In 6 of 10 patients, complete remission was stopped, of which 5 patients relapsed, 4 patients re-treated with CSA, urine protein turned negative again and 1 patient gave up treatment. One case of renal dysfunction after the original serum creatinine increased. Conclusion CSA has a certain curative effect on steroid-resistant FSGS, but it has a high recurrence rate after treatment. CSA side effects should be paid attention to during treatment.