儿童类固醇和环孢素抵抗性特发性肾病综合征的治疗
Treatment of steroid and cyclosporine-resistant idiopathic nephrotic syndrome in children.
作者信息
Nikibakhsh A A, Mahmoodzadeh H, Karamyyar M, Hejazi S, Noroozi M, Macooie A A
机构信息
Urology-Nephrology and Transplantation Research Centre, Department of Paediatric Nephrology, Urmia University School of Medicine, Iran.
出版信息
Int J Nephrol. 2011;2011:930965. doi: 10.4061/2011/930965. Epub 2011 Sep 22.
Steroid-resistant nephrotic syndrome (SRNS) in children carries a significant risk of progression to end-stage renal failure (ESRF). We report a two-step protocol adapted in children with SRNS. Thirty-seven SRNS were treated with cyclosporine A (CyA) in association with prednisolone (alternate day) for 6 months (first-step treatment). Twelve patients (32.4%) went into complete remission, and 2 (5.4%) got partial remission. The other 23 cases who were steroid and CyA resistant entered a second-step treatment with withdrawing steroids, with CyA (5 mg/kg/day) in association with mycophenolate mofetil (MMF) 30 mg/kg/day for 6 months. Complete remission was observed in 11 cases (47.82%) and partial remission in 2 cases (8.7%). After two steps of treatment, 27/37 children went into total remission. In steroid and CyA-resistant INS, the association of MMF with CyA was able to induce remission in about half cases without relevant side effects.
儿童类固醇抵抗型肾病综合征（SRNS）进展为终末期肾衰竭（ESRF）的风险很大。我们报告了一种适用于SRNS患儿的两步治疗方案。37例SRNS患儿接受环孢素A（CyA）联合泼尼松龙（隔日给药）治疗6个月（第一步治疗）。12例患者（32.4%）完全缓解，2例（5.4%）部分缓解。另外23例对类固醇和CyA耐药的患者进入第二步治疗，停用类固醇，使用CyA（5mg/kg/天）联合霉酚酸酯（MMF）30mg/kg/天治疗6个月。11例（47.82%）完全缓解，2例（8.7%）部分缓解。经过两步治疗，37例患儿中有27例完全缓解。在对类固醇和CyA耐药的特发性肾病综合征（INS）中，MMF与CyA联合使用能够使约半数病例诱导缓解，且无相关副作用。
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