环孢霉素A治疗儿童难治性肾病综合征的疗效及随访分析  

The therapeutic efficacy of cyclosporin A in the children with refractory nephrotic syndome

作　　者：徐璇[1] 杨曼琼[1] 王复娟[2] 吴小川[2] 

机构地区：[1]湖南省人民医院儿科,长沙410005 [2]中南大学湘雅二医院儿童医学中心儿童肾脏专科

出　　处：《中国医师杂志》2013年第3期312-316,共5页Journal of Chinese Physician

摘　　要：目的观察环孢霉素A（Cyclosporine A；CsA）治疗儿童难治性肾病综合征的临床疗效。方法应用CsA联合泼尼松治疗26例难治性肾病综合征患儿，CsA剂量为3—6mg／（kg·d），疗程3～27（12．69±6．44）个月。治疗前后检测24h尿蛋白定量、血清胆固醇、血清尿素氮、血清肌酐、血清胱抑素（CyC）、血清N-乙酰B-氨基葡萄糖苷酶等相关生化指标，并观察药物不良反应。结果26例患儿中激素耐药12例，激素依赖6例，频繁复发8例。治疗后完全缓解16例，占61．54％，部分缓解8例，占30．77％，未缓解2例，占7．69％，总有效率92．31％。观察指标结果：24h尿蛋白定量：治疗前3．01g/24h，治疗后0．63g／24h，其差异有统计学意义（P〈0．01）；血清胆固醇：治疗前（7．72±3．86）mmol／L，治疗后（7．15±3．23）mmol／L；尿素氮：治疗前（3．93±1．44）mmol/L，治疗后（4．04±1．27）mmol／L；肌酐：治疗前（33．384-13．16）μmol／L，治疗后（35．64±3．53）μmol／L；血清N-乙酰B-氨基葡萄糖苷酶：治疗前（18．96±4．86）u／L，治疗后（20．45±5．85）u／L；以上指标差异均无统计学意义（P〉0．05）。激素抵抗组、激素依赖组及频繁复发组的疗效差异无统计学意义（P〉0．05）。完全缓解患儿随访至6、9、12、18个月的复发率依次为37．5％、31．25％、18．75％、12．5％。8例患儿停药3～6个月，均无复发。CsA的不良反应依次为多毛、震颤、胃肠道反应等，而治疗期间肝、肾毒性并不明显。‘结论CsA联合泼尼松能有效治疗小儿难治性肾病综合征，并可明显减少糖皮质激素的使用剂量，至少1年以上维持治疗疗程可明显减少病情复发。[ Abstract] Objective To evaluate the efficacy of cyclosporine A plus prednisone in the treatment of refractory nephrotic syndrome in children. Methods The 26 refractory nephrotic syndrome children were treated with CsA plus prednisone,3 - 6 mg/（ kg · d）, Po. The duration of treatment was 3 to 27 months （ 12. 69 ± 6.44 ） mos. The 24 h quantitative urinary proteins, serum cholesterol, urea nitrogen, plasma creatinine, N-acetyl beta amino glyeosidase enzymes eystatin C were detected before and after treatment, and the adverse drug reactions were accessed. Results Among 26 cases, 12 cases of steroid-resistant NS, 6 cases of steroid-dependent NS, and 8 cases of frequent relapse NS were included. 16 patients （ 6]. 54% ） were complete remission, 8 patients （30. 77% ） partial remission, 2 cases （7.69%） were non-remission, The total remission rate was 92. 31%. The 24 hours urine protein was 3.0l g and O. 63 g before treatment and after treatment, respectively, with a statistically significant difference （ P 〈 0.01 ） ; serum cholesterol was （7.72 ±3.86） mmol/L and （7. 15 ± 3.23 ） retool/L; nitrogen urea was （3.93 ± 1.44） mmol/L and （4. 04 ± 1.27） mmol/L, ereatinine （33.38 ± 13.16） μmol/L and （35.64 ±3.53）μmoL/L serum N-acetyl beta amino glycosidase enzymes was （ 18.96 ±4. 86）u/L and （20.45 ±5.85）u/L before treatment and after treatment, respectively, without a statistically significant difference （ P 〉 0.05 ）. The response to CsA was no significant difference in SRNS, SDNS and FRNS. Children complete remission, follow-up to 6 months, 9 months, 12 months and 18 months of the recurrence rate was 37.5%, 31.25%, 18.75% and 12. 5%. Eight cases ended treatment 3 - 6 months, all cases were not recurrence. The main adverse effects of CsA included hirsutism, tremble, gastrointestinal reaction and so on, and liver kidney toxicity was not obvious during the therapy course. Conclusions The treatment of CsA in combination with prednisone to children refractory

关 键 词：环孢菌素类 治疗应用 肾病综合征 药物疗法 儿童 随访研究 

分 类 号：R726.9[医药卫生—儿科]