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目的 :儿童难治性肾病是原发性肾病综合征中激素耐药、激素依赖、频繁复发病例的总称 ,但目前尚缺乏有效的治疗措施 ,6 巯基嘌呤是治疗难治性肾病综合征的有效药物之一。国外有学者曾报道与泼尼松联合应用能减少肾病复发 ,延长缓解期。但国内未见将 6 巯基嘌呤用于儿童难治性肾病的治疗 ,作者重点探讨 6 巯基嘌呤在儿童难治性肾病综合征中的治疗作用与不良反应。 方法 :对该科住院的 2 8例原发性肾病综合征患儿 ,根据不同肾病类型 ,在院外已使用激素者 ,如为激素耐药型与频繁复发型入院后逐渐减用激素 ,先减至原剂量隔日疗法 ,减量的同时加用 6 巯基嘌呤 (2mg/kg·d-1)、疗程 1年 ,激素每 2~ 4周减 5~ 10mg ,减至隔天 0 .5mg/kg维持 1年 ;如为激素依赖型入院后继用激素 (2mg/kg·d-1) ,同时加用 6 巯基嘌呤 (2mg/kg·d-1)、疗程 1年 ,待肾病缓解后 ,激素逐渐减量 ,具体方法同激素耐药型和频繁复发型。 结果 :经 6 巯基嘌呤治疗 1个月后 ,患儿尿蛋白明显减少 ,起效时间为9~ 2 8天 ,平均 17天 ,总有效率为 85 .7%。在不同的肾病类型中 ,激素依赖型疗效最好 ,依次为激素耐药型、频繁复发型 ,其有效率分别为 10 0 %、84 .6 %、81.8%。 2 8例均经肾活检明确病理类型 ,其病理类型与疗效的关系 ,以M
Objective: Children with refractory nephropathy is a general term for steroid resistance, hormone dependence and frequent recurrence in primary nephrotic syndrome. However, there are still no effective treatment measures. 6-Mercaptopurine is effective in the treatment of refractory nephrotic syndrome One of the medicines. Some foreign scholars have reported that combined use of prednisone can reduce the recurrence of renal disease, prolong the remission. However, no 6-mercaptopurine has been used in the treatment of childhood refractory nephropathy in China, and the author focuses on the therapeutic effect and adverse reactions of 6-mercaptopurine in refractory nephrotic syndrome in children. Methods: The hospitalized 28 cases of primary nephrotic syndrome in children, according to different types of nephropathy, who have been using the hormone in the hospital, such as hormone-resistant and frequent recurrence after admission to gradually reduce the use of hormones, first reduced To the original dose every other day therapy, while reducing the addition of 6-mercaptopurine (2mg / kg · d-1), a course of treatment, hormone every 2 to 4 weeks by 5 ~ 10mg, reduced to 0.5mg / kg the next day to maintain (2mg / kg · d-1) and 6-mercaptopurine (2mg / kg · d-1) at the same time for 1 year. When the nephropathy is relieved, the hormones gradually decrease Amount of specific methods with hormone-resistant and frequent recurrence. Results: After 1 month treatment with 6-mercaptopurine, urinary protein was significantly decreased in children with onset time of 9 to 28 days, an average of 17 days, the total effective rate was 85.7%. In different types of nephropathy, hormone-dependent curative effect is the best, followed by hormone-resistant type, frequent recurrence, the effective rates were 100%, 84.6%, 81.8%. 28 cases were confirmed by renal biopsy pathological types, the relationship between the pathological type and curative effect to M