Zhonghua Er Ke Za Zhi 2003 Nov;41(11):813-6
Department of Peadiatrics, Nanjing General Hospital of PLA, Nanjing 210002, China.
Objective: To evaluate the efficacy of cyclosporin A (CyA) therapy in 83 children with nephrotic syndrome of different pathological types.
Methods: 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.
Results: 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.
Conclusion: 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.
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