Treatment of idiopathic nephrotic syndrome with cyclosporin A in children.
Treatment of idiopathic nephrotic syndrome with cyclosporin A in children.
复制标题
环孢素A治疗儿童特发性肾病综合征。
作者：
P. Niaudet;M. Broyer;Renée Habib
71例特发性肾病综合征患儿给予环孢素A （CyA, Sandimmun）治疗，其中45例为类固醇依赖且有类固醇毒性迹象，23例为类固醇耐药。环孢素A对80%的类固醇依赖患者有效，允许停止皮质类固醇治疗。然而，当CyA逐渐减少或停药时，这些患者中的大多数复发。在这些患者中，CyA可能需要很长一段时间。相反，CyA在类固醇耐药患者中效果较差；只有7%的患者单独接受CyA治疗获得缓解。环孢素A联合强的松可能是一种有效的替代方案，因为14例患者中有8例通过这种联合治疗进入缓解期。对43例患者进行了连续肾活检，以评估该治疗的潜在肾毒性。18例患者出现明显的肾小管间质病变，可归因于CyA肾毒性。发生慢性肾毒性的风险似乎在类固醇抵抗患者中更高，并且与治疗时间无关。环孢素A可在肾功能正常的患者中发生肾毒性。
Cyclosporin A (CyA, Sandimmun) was given to 71 children with idiopathic nephrotic syndrome, 45 of whom were steroid-dependent with signs of steroid toxicity, and 23 who were steroid-resistant. Cyclosporin A was effective in 80% of the steroid-dependent patients, allowing cessation of corticosteroid treatment. However, most of these patients relapsed when CyA was tapered or withdrawn. In these patients, CyA may be required for long periods of time. Conversely, CyA was less effective in steroid-resistant patients; only 7% achieved remission with CyA alone. Cyclosporin A in association with prednisone may be an effective alternative as 8 out of 14 patients entered remission with this treatment combination. Serial renal biopsies were performed in 43 patients to evaluate the potential nephrotoxicity of the treatment. Eighteen patients developed significant tubulointerstitial lesions which were attributable to CyA nephrotoxicity. The risk of developing chronic nephrotoxicity appears to be higher in steroid-resistant patients, and was not related to the duration of treatment. Cyclosporin A nephrotoxicity can develop in patients with normal renal function.