Pediatr Nephrol. 2010 Jul;25(7):1197-9. doi: 10.1007/s00467-010-1469-2. Epub 2010 Mar 2.
It has been reported (this issue Pediatric Nephrology) that cyclosporine A (CyA) therapy in combination with corticosteroids, angiotensin-converting enzyme inhibitor, and an angiotensin receptor blocker decreased proteinuria in three patients with nephrotic syndrome (NS) due to WT1 mutations. Treatment with calcineurin inhibitors were found to induce a partial remission of proteinuria in several other children with genetic forms of NS, such as mutation in the podocine and in the phospholipase C epsilon gene. CyA therapy has also been reported to be beneficial to patients with Alport syndrome. Recent data have shown that the antiproteinuric effect of CyA in these cases may be due to a non-immunologic mechanism. CyA exerts an antiproteinuria effect by preventing the degradation of the actin organizing protein synaptodpodin and by a downregulation of TRPC6. This mechanism leads to the stabilization of the actin cytoskeleton in the kidney podocytes. This beneficial effect of CyA is interesting, but long-term results regarding function and nephrotoxicity are still missing.
据报道（该问题发表于《儿科肾脏病学》），环孢素 A（CyA）联合皮质类固醇、血管紧张素转换酶抑制剂和血管紧张素受体阻滞剂治疗，可使三例 WT1 突变导致的肾病综合征（NS）患者的蛋白尿减少。研究发现，在其他一些遗传形式的 NS 患儿中，如足细胞和磷脂酶 C epsilon 基因突变的患儿，钙调神经磷酸酶抑制剂治疗可诱导部分缓解蛋白尿。CyA 治疗对 Alport 综合征患者也有益。最近的数据表明，CyA 在这些情况下的抗蛋白尿作用可能是由于非免疫机制。CyA 通过防止肌动蛋白组织蛋白 synaptodpodin 的降解和下调 TRPC6 发挥抗蛋白尿作用。这种机制导致肾脏足细胞中肌动蛋白细胞骨架的稳定。CyA 的这种有益作用很有趣，但关于其功能和肾毒性的长期结果仍不清楚。
