长程环孢素A个体化治疗难治性肾病综合征的疗效及其影响因素  

Clinical Efficacy of Long-term Cyclosporine A and Its Influencing Factors in Individualized Treatment of Refractory Nephrotic Syndrome

作　　者：周吉[1] 徐进[1] 许静[1] 李文静[1] 朱明媚[1] 景霞[1] 

机构地区：[1]南京医科大学附属南京儿童医院药房,南京210000

出　　处：《南昌大学学报（医学版）》2013年第9期21-25,共5页Journal of Nanchang University：Medical Sciences

摘　　要：目的 观察激素耐药性肾病综合征患儿应用环孢素A（CsA）长程治疗的疗效并分析影响疗效的相关因素.方法 30例难治性肾病综合征[病理类型为系膜增生性肾小球肾炎（MsPGN）18例,局灶节段肾小球硬化（FSGS）6例,膜性肾病（MN）2例,微小病变（MCD）肾病1例,肾炎性肾病综合征1例,余2例因条件限制未做病理检查]采用CsA同时联合激素治疗,疗程12个月.27例使用肝药酶抑制剂地尔硫卓.观察临床疗效、不良反应及影响疗效的因素.结果 临床疗效：完全缓解22例（73.3%）,部分缓解6例（20.0%）,无效2例（6.7%）,总有效率为93.3%;平均随访时间15个月,复发率为16.7%.不良反应：毛发增生（80.00%）,胃肠道反应（40.00%）,牙龈出血（23.33%）,关节肿大（6.67%）,生长抑制（3.33%）,肝、肾功能损害（3.33%）.在使用相同剂量CsA（5.0～7.0 mg·kg-1·d-1）的情况下,男性患者的CsA血药浓度水平要低于女性患者（P＜0.05）.CsA血药浓度≤100 μg·L-1时,患者的不良反应发生率（13.4%）较低,CsA血药浓度＞100 μg·L-1时,患者的不良反应发生率（55.6%）相对较高.27例患儿的CsA血药浓度偏低,合并使用肝药酶抑制剂后CsA血药浓度有所提升（P＜0.05）.结论 长程环孢素联合激素治疗儿童难治性肾病综合征有一定疗效,但其疗效及不良反应受多重因素影响.Objective To observe the efficacy of long-term cyclosporine A （CsA） in the treatment of steroid-resistant nephrotic syndrome in children, and to analyze the factors affecting efficacy. Methods Combined treatment with CsA and hormones was performed for 12 months for refractory nephrotic syndrome in 30 children, including 18 cases of mesangial proliferative glomerulonephritis,6 cases of focal segmental glomerulosclerosis, 2 cases of membranous nephropathy, 1 case of minimal change nephropathy and 1 case of nephrosis-nephritis syndrome （two cases did not receive pathologic examination due to the limited conditions）. Moreover, enzyme inhibitor dilt- iazem was used in 27 children. Clinical efficacy, adverse reactions and factors affecting efficacy were observed. Results Among the 30 children,complete remission was achieved in 22 （73.3%）, partial remission in 6 （20.0%） ,and ineffective outcome in 2 （6.7%）. The total effective rate was 93.3% and the recurrence rate was 16. 7% after the follow-up of 15 months. In addition, hair growth was found in 80.00% of the 30 cases,gastrointestinal reactions in 40.00% ,gingival bleeding in 23. 330/00 ,joint swelling in 6. 670//oo ,growth inhibition in 3. 330//oo ,and hepatic and renal dys function in 3.33%. Compared with female children,treatment with CsA （5-7 mg·kg-1·d-1） significantly decreased the blood drug concentrations in male children （P〈0. 05）. The incidence of adverse reactions was 13.4% and 55.6% in children with blood CsA concentrationS100 μg·L-1 and children with blood CsA concentration〉100 μg·L-1 ,respectively. After treatment with diltiazem,blood CsA concentrations significantly increased in the 27 children with low blood CsA levels（P〈0.05）. Conclusion The long-term cyclosporine combined hormones has a certain effect on refractory nephrotic syndrome in children. However,the curative effects and adverse reactions are influenced by muhiple factors.

关 键 词：难治性肾病综合征 环孢素A 激素 临床疗效 不良反应 儿童 

分 类 号：R692.6[医药卫生—泌尿科学]