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对激素依赖型肾病综合征患儿进行为期两年的环孢素治疗。

Two-year cyclosporin treatment in children with steroid-dependent nephrotic syndrome.

作者信息

Inoue Y, Iijima K, Nakamura H, Yoshikawa N

机构信息

Department of Pediatrics, Kobe University School of Medicine, Japan.

出版信息

Pediatr Nephrol. 1999 Jan;13(1):33-8. doi: 10.1007/s004670050558.

DOI:10.1007/s004670050558
PMID:10100286
Abstract

We describe a prospective study of 2-year moderate-dose cyclosporin (CS) treatment in 13 children with steroid-dependent minimal change nephrotic syndrome (MCNS). CS treatment was commenced at 100-150 mg/m2 per day after remission was attained with prednisolone therapy, was adjusted to a target trough level of 100 ng/ml, and was administered for 2 years. The number of relapses during CS treatment significantly decreased compared with before CS treatment, all patients were able to discontinue prednisolone therapy, and steroid toxicity was reduced; 54% of patients remained in remission during CS treatment. Renal biopsies performed before CS treatment all showed MCNS without tubulointerstitial lesions. Creatinine clearance and urinary beta 2-microglobulin levels during CS treatment were normal in all patients, but renal biopsies performed after CS treatment revealed chronic CS nephrotoxicity in 7 patients. Clinical data, including CS dose and CS trough blood levels, were not significantly different between patients with and without nephrotoxicity. In conclusion, 2-year moderate-dose CS treatment in children with steroid-dependent MCNS is effective in preventing relapse and decreasing steroid toxicity. This treatment can, however, result in a high incidence of chronic nephrotoxicity. Renal function is not a reliable indicator of chronic CS nephrotoxicity. Renal biopsy is therefore necessary to monitor chronic CS nephrotoxicity.

摘要

我们描述了一项针对13名依赖类固醇的微小病变肾病综合征(MCNS)儿童进行的为期2年的中等剂量环孢素(CS)治疗的前瞻性研究。在使用泼尼松龙治疗达到缓解后,以每天100 - 150 mg/m²开始CS治疗,调整至目标谷浓度为100 ng/ml,并持续给药2年。与CS治疗前相比,CS治疗期间的复发次数显著减少,所有患者均能够停用泼尼松龙治疗,且类固醇毒性降低;54%的患者在CS治疗期间保持缓解状态。CS治疗前进行的肾活检均显示为无肾小管间质病变的MCNS。所有患者在CS治疗期间的肌酐清除率和尿β2 -微球蛋白水平均正常,但CS治疗后进行的肾活检显示7例患者存在慢性CS肾毒性。有肾毒性和无肾毒性的患者之间的临床数据,包括CS剂量和CS谷血浓度,无显著差异。总之,对依赖类固醇的MCNS儿童进行为期2年的中等剂量CS治疗可有效预防复发并降低类固醇毒性。然而,这种治疗可能导致慢性肾毒性的高发生率。肾功能并非慢性CS肾毒性的可靠指标。因此,有必要进行肾活检以监测慢性CS肾毒性。

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