Xia Zheng-kun, Liu Guang-ling, Gao Yuan-fu, Fu Jie, Fu Yuan-feng, Zhang Lian-feng, Fan Zhong-min
Department of Peadiatrics, Nanjing General Hospital of PLA, Nanjing 210002, China.
Zhonghua Er Ke Za Zhi. 2003 Nov;41(11):813-6.
To evaluate the efficacy of cyclosporin A (CyA) therapy in 83 children with nephrotic syndrome of different pathological types.
Eighty-three children enrolled in this study were all hospitalized children with idiopathic nephrotic syndrome, aged 3 to 14 yrs (average 8.3 yrs) and included 52 males and 31 females. There were 35 cases with steroid-dependent, 17 with steroid resistant and 26 with frequent relapses. CyA was given to each patient with dosage of 5 mg/(kg.d) during the corticosteroid was diminished. The renwal biopsy was performed in all patients before the administration of CyA. The duration of CyA therapy lasted for about 3 to 6 months. The plasma concentration of CyA was monitored.
Eighty-three children with nephrotic syndrome of different pathological types were treated with CyA, including 42 cases of minimal change nephrotic syndrome (MCNS), 31 cases of mesangioproliferative glomerulonephritis (MsPGN), 5 cases of membranoproliferative glomerulonephritis (MPGN) and 4 cases of focal segmental glomerular sclerosis (FSGS). All the 83 patients tolerated well to the CyA treatment. Forty-five cases got complete remission, 23 partial remission, 15 cases no change after one month treatment with CyA in the hospital. The overall response rate was 82%. Patients with different renal pathological types showed different responses. Among them, MCNS and MsPGN exhibited the best response rates of 86% and 84%, respectively; MPGN cases showed a lower response rate and FSGS cases showed the lowest rate. The response time was 7 to 45 days. The blood concentration of CyA was monitored for 1 week and 2 weeks after the drug was given. The effective drug concentration was maintained at 100 to 200 microg/L, and the course lasted for 3 to 6 months. During the follow-up of 83 cases, in 17 of 68 cases the disease relapsed when therapy was tapered or discontinued. The relapse rate was 25%. The results indicated that CyA would be effective to the relapsed cases. The serum creatinine increased temporarily after administration of CyA in 5 cases, N-acetyl-beta-D-glucosaminidase (NAG) in 8 cases and eventually reached the normal range after the adjustment of dosage. The side effects included anorexia, nausea, vomiting and so on.
CyA is one of the effective substitutes for the treatment of nephrotic syndrome, especially for the cases with MCNS and MsPGN. And CyA could control refractory nephrotic syndrome effectively and rapidly. The clinical effect was related to the blood concentration of CyA and pathological types.
评估环孢素A(CyA)治疗83例不同病理类型儿童肾病综合征的疗效。
本研究纳入的83例儿童均为住院的特发性肾病综合征患儿,年龄3至14岁(平均8.3岁),其中男52例,女31例。激素依赖型35例,激素抵抗型17例,频繁复发型26例。在糖皮质激素减量期间,给予每位患者CyA,剂量为5mg/(kg·d)。所有患者在给予CyA前均进行了肾活检。CyA治疗持续约3至6个月。监测CyA的血药浓度。
83例不同病理类型的儿童肾病综合征患者接受了CyA治疗,其中微小病变肾病综合征(MCNS)42例,系膜增生性肾小球肾炎(MsPGN)31例,膜增生性肾小球肾炎(MPGN)5例,局灶节段性肾小球硬化(FSGS)4例。83例患者对CyA治疗耐受性良好。住院1个月CyA治疗后,45例完全缓解,23例部分缓解,15例无变化。总有效率为82%。不同肾脏病理类型的患者反应不同。其中,MCNS和MsPGN的有效率最高,分别为86%和84%;MPGN患者有效率较低,FSGS患者有效率最低。起效时间为7至45天。给药后1周和2周监测CyA血药浓度。有效血药浓度维持在100至200μg/L,疗程持续3至6个月。83例患者随访期间,68例中有17例在治疗减量或停药时疾病复发。复发率为25%。结果表明,CyA对复发病例有效。5例患者给予CyA后血清肌酐暂时升高,8例N-乙酰-β-D-氨基葡萄糖苷酶(NAG)升高,调整剂量后最终恢复正常范围。副作用包括厌食、恶心、呕吐等。
CyA是治疗肾病综合征的有效替代药物之一,尤其适用于MCNS和MsPGN患者。CyA能有效、快速地控制难治性肾病综合征。临床疗效与CyA血药浓度及病理类型有关。