High-dose mizoribine therapy for childhood-onset frequently relapsing steroid-dependent nephrotic syndrome with cyclosporin nephrotoxicity
High-dose mizoribine therapy for childhood-onset frequently relapsing steroid-dependent nephrotic syndrome with cyclosporin nephrotoxicity
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DOI：
10.1007/s00467-005-2025-3
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发表时间：
2005-12-01
影响因子：
3
通讯作者：
Yamashiro, Y
中科院分区：
文献类型：
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作者：
Ohtomo, Y;Fujinaga, S;Yamashiro, Y
环孢菌素A（CsA）是治疗频繁复发的类固醇依赖性肾病综合征（FR-SDNS）的有效方法，但其使用可并发肾毒性和停药后复发率高。我们报告了9例儿童期发病的FR-SDNS青少年患者，他们长期接受CsA治疗，导致中度至重度CsA肾病（CsAN）。他们接受了高剂量（平均：10.1 mg/kg/天）咪唑立宾（MZR）治疗，试图停止环孢菌素A和/或类固醇治疗，并降低复发频率。7/9例患者在1年随访时停用CsA，但在其余2例患者中，MZR未显示任何有益效果。总的来说，这种高剂量MZR治疗在我们的患者中导致显著的类固醇节省和复发率降低。我们的经验表明，高剂量MZR治疗的FR-SDNS患者谁也CsA依赖似乎是有效的减少CsA暴露，以及在减少复发的频率。
Cyclosporin A (CsA) is an effective treatment for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal. We report 9 adolescent patients with childhood-onset FR-SDNS who had been treated with long-term CsA that resulted in moderate-to-severe CsA nephropathy (CsAN). They were treated with high-dose (mean: 10.1 mg/kg per day) mizoribine (MZR) in an attempt to allow weaning of CsA and/or steroid therapy, and reduce the frequency of relapses. Seven out of 9 patients were weaned off CsA by 1-year follow-up, although in the remaining 2 patients, MZR did not show any beneficial effects. Overall, this high-dose MZR therapy results in significant steroid sparing and reduction in relapse rates in our patients. Our experience shows that high-dose MZR therapy in patients with FR-SDNS who are also CsA-dependent appears to be effective in reducing CsA exposure as well as in decreasing the frequency of relapses.