Podracká L, Böör A, Sasinka M
Klinika detí a dorastu LF UPJS, Kosice, Slovenská republika.
Cas Lek Cesk. 2008;147(1):38-43.
The majority of cases of nephrotic syndrome in children is corticosensitive, however in some individuals aggressive cytotoxic therapy is necessary. Cyclosporin A and cyclophosphamide are widely used; however their relative effectiveness in maintaining remission of childhood nephrotic syndrome remains controversial.
Effectiveness of long-term cytotoxic therapy (mean follow-up 7.4 years) in 63 children (mean age 6.19 +/- 4.30 years) with nephrotic syndrome was retrospectively analyzed; 14 patients suffered from corticosensitive, 33 from corticodependent and 16 from corticoresistant nephrotic syndrome. Corticotherapy alone was used in 15 patients (23.8%), cyclophosphamide was added in 27 (42.9%) and cyclosporin A in 38 cases (60.3%), while 17 patients (27%) were on both immunosuppressive agents. 84% children relapsed within 24 months after cessation of cyclosporin A. In 8 corticoresistant nephrotic syndrome patients (61.5%) from 13 children treated with cyclosporin A no remission occurred, in 5 children (38.5%) was remission obtained within 10 weeks, however in 4 of them relapsed disease during cyclosporin A therapy. 19 (70.4%) of 27 patients on cyclophosphamide therapy were in remission, in 8 of them (42.1%) even 2 years after cyclophosphamide therapy.
Cyclophosphamide therapy of childhood nephrotic syndrome is more effective in maintaining long-term remission than cyclosporin A treatment.
儿童肾病综合征的大多数病例对皮质激素敏感，然而在一些个体中，积极的细胞毒性治疗是必要的。环孢素A和环磷酰胺被广泛使用；然而它们在维持儿童肾病综合征缓解方面的相对有效性仍存在争议。
回顾性分析了63例（平均年龄6.19±4.30岁）肾病综合征患儿长期细胞毒性治疗（平均随访7.4年）的有效性；14例为皮质激素敏感型，33例为皮质激素依赖型，16例为皮质激素抵抗型肾病综合征。15例患者（23.8%）仅使用皮质激素治疗，27例（42.9%）加用环磷酰胺，38例（60.3%）加用环孢素A，而17例患者（27%）同时使用两种免疫抑制剂。84%的儿童在停用环孢素A后24个月内复发。在接受环孢素A治疗的13例儿童中的8例皮质激素抵抗型肾病综合征患者（61.5%）未缓解，5例儿童（38.5%）在10周内缓解，但其中4例在环孢素A治疗期间疾病复发。27例接受环磷酰胺治疗的患者中有19例（70.4%）缓解，其中8例（42.1%）甚至在环磷酰胺治疗后2年仍处于缓解状态。
儿童肾病综合征的环磷酰胺治疗在维持长期缓解方面比环孢素A治疗更有效。
