环孢素A联合激素治疗儿童原发性肾病综合征的单中心临床疗效观察

Observation on the clinical effects of cyclosporine A combined with glucocorticoids in children with primary nephrotic syndrome from a single center

目的观察环孢素A(Cs A)联合激素治疗儿童肾病综合征的临床疗效及安全性。方法 40例肾病综合征患儿,其中激素耐药型肾病综合征(SRNS)13例,激素依赖型肾病综合征(SDNS)18例,频繁复发型肾病综合征(FRNS)9例,联合使用Cs A(3~5 mg·kg^(-1)·d^(-1))和泼尼松治疗,维持其血药谷浓度100~200μg/L,总疗程2年,起效后9~12个月减量,观察患儿缓解率、24 h尿蛋白定量、血浆胆固醇、CD4^+、CD8^+、CD4^+/CD8^+和不良反应发生情况。结果 24例(60%)完全缓解,7例(17.5%)部分缓解,随着治疗时间的延长,短期完全缓解率逐渐提高。随访3个月时9例(22.5%)无效,给予调整其他免疫抑制剂治疗。随访6、9、12个月,31例患儿临床生化指标明显改善,血浆白蛋白升高,24 h尿蛋白定量、血浆胆固醇、CD4^+、CD4^+/CD8^+降低,与Cs A治疗前存在明显差异,有统计学意义(P<0.01);SRNS与SDNS对Cs A的疗效存在差异,有统计学意义(P<0.05),SRNS与FRNS、SDNS与FRNS对Cs A的疗效存在差异,但无统计学意义(P>0.05),Cs A治疗不同病理类型肾病综合征的疗效无明显差异。Cs A的主要不良反应依次是多毛、牙龈增生、胃肠道反应、轻度高血压、肝肾功能损伤,同时观察到1例大脑后部可逆性脑病综合征,均不影响Cs A继续使用。结论小样本临床研究中,应用Cs A联合激素治疗儿童肾病综合征相对安全有效,对SDNS疗效较佳,应用时需注意Cs A的不良反应。

Objective To observe the clinical effects and safe of cyclosporine A（Cs A） in the treatment of primary nephrotic syndrome in children. Methods There were 40 child patients with nephrotic syndrome treated with Cs A（3-5 mg·kg-1·d-1） combined with glucocorticoids, in which including steroid-resistant nephritic syndrome（SRNS） 13 cases, steroid-dependent nephrotic syndrome（SDNS） 18 cases, frequent-relapses nephrotic syndrome（FRNS） 9 cases. The concentration of Cs A maintained 100-200 μg/L. Total course of treatment was two years, the dose was tapered gradually in 9-12 months after onset, the remission rate of child patients, 24 h urine protein, plasma cholesterol, CD4＋, CD8＋, the ratio of CD4＋/CD8＋, and the occurrence of adverse drug reactions were observed closely. Results 24 cases（60%） were completely remission, 7 cases（17.5%） were partially remission, whose short-term complete remission rate gradually increased along with the duration of treatment. 9 cases（22.5%） were ineffective who were given to adjust other immunosuppressants to further treat after the 3 months follow-up. After the 6, 9, 12 months follow-up, the 31 cases＇ clinical biochemical parameters were significantly improved, serum albumin increased, 24 h urine protein, serum cholesterol, CD4＋ and the ratio of CD4＋/CD8＋ decreased, there was significant difference after the treatment of Cs A and before（P0.01）. The response to Cs A had significant difference in SRNS and SDNS（P0.05）, however, it was to the contray in SRNS and FRNS, SDNS and FRNS（P0.05）. There was no significant difference in the efficacy of different pathological types. The main adverse effects of Cs A included hirsutism, gingival hyperplasia, gastrointestinal reactions, mild hypertension, liver and renal function impairment, meanwhile, one case of reversible encephalopathy syndrome was in back of the brain, and all were acceptable. Conclusion In small samples of clinical study, the application of Cs A combined with hormone to treat children with NS were relatively safe and effective, a better efficacy for SDNS, adverse reactions should be paid attention to.