环孢素A联合激素治疗儿童难治性肾病综合征疗效观察

目的 观察环孢素A（CsA）联合激素治疗儿童难治性肾病综合征的临床疗效。方法 接受CsA联合激素治疗的患儿共23例。按临床类型分为激素耐药组12例、激素依赖组5例、频繁复发组6例。CsA 4-6 mg/（kg·d）连续口服3-6个月,然后每2-4周减少用量的25%,当用量减至1.0-2.5 mg/（kg·d）时维持治疗6-24个月。用药期间定期监测患儿血尿相关生化指标,并观察药物不良反应。结果 完全缓解13例（56.5%）,部分缓解7例（30.4%）,无效3例（13.1%）。激素耐药组、激素依赖组及频繁复发组之间疗效无明显差异,但激素耐药组达到完全缓解所需时间较激素依赖组及频繁复发组长。不同病理类型对CsA的治疗反应无明显差异,血胆固醇高低对CsA的疗效无明显影响。20例缓解患儿中13例（65%）复发,经抗感染治疗和CsA加量后11例（84.6%）再次缓解。CsA的主要不良反应为肝肾功能损害、高血压、多毛、齿龈增生等,经减量或对症治疗后均改善。结论 CsA联合激素治疗儿童难治性肾病安全、有效。

Observation on the clinical effects of cyclosporine A combined with hormones in children with refractory nephrotic syndrome

Objective To observe the clinical effects of cyclosporine A （CsA） in the treatment of refractory nephrotic syndrome （RNS） in children. Methods There were 23 child patients treated with CsA combined with hormones at a CsA dosage of 4 -6 mg for a duration of 3 to 6 months. Then, the dosage was reduced to 25% every 2 to 4 weeks. When the dosage was reduced to 1.0 - 2. 5 mg/（ kg · d）, the minimum dosage was maintained for 6 - 12 months. During medication, biochemical data associated with hematuria were detected periodically and adverse drug reactions were observed closely. Results Thirteen cases （56.5%） were completely relieved, 7 cases （30. 4% ） were partially relieved and 3 cases （13.1%） were ineffective. There were no significant differences in therapeutic effects between the hormone-resistant, hormone-dependent and recurrent groups. However, the time required for the hormone-resistant group to achieve complete relief was longer than that of the hormone-dependent group. Different renal pathological types did not seem to have obvious differences in the reactions of CsA. Serum cholesterin level also did not have obvious differences in the therapeutic effects of CsA. Of the 20 cases that were relieved after treatment, 13 cases （56.5%） relapsed, however, 11 cases （84.6%） were relieved again following antiinfection treatment and the dosage of CsA was increased. The main adverse reactions of CsA were hepatic and renal function damage, hypertension, hairiness, gum proliferation, however, these adverse reactions were all improved following reduction of the dosage and expectant treatment. Conclusion CsA treatment combined with hormones was safe and effective for the treatment of refractory renal disease in children.