环孢素对类固醇依赖型和频繁复发肾病综合征患儿的疗效和不良反应

Ghada Borham, Mahmoud Abd El-aal, mustafa abo-sdera, Rania Abdelatif
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摘要

背景:环孢素是一种钙神经蛋白抑制剂,对依赖类固醇和经常复发的肾病综合征患者有明显的类固醇节省作用。尽管环孢素可长期使用,但它与令人不快的副作用风险增加有关,包括肾毒性、高血压以及多毛症和牙龈肥大等外观症状。目的:评估环孢素对苏哈格大学医院确诊的类固醇依赖型和频繁复发肾病综合征患儿的疗效和副作用。方法:这是一项前瞻性和回顾性相结合的研究:这是一项前瞻性和回顾性相结合的研究,在苏哈格大学医院小儿肾脏病诊所进行,研究对象是被诊断为类固醇依赖型和频繁复发肾病综合征的儿童,他们接受了环孢素作为类固醇稀释剂。研究结果本研究共纳入 50 名肾病患者,平均年龄为 11.4 ± 3.1 (4-15)岁。男女比例为 2.8:1。中位病程为 7 年,四分位数范围为(3.88-9.63)年,中位环孢素使用时间为 4 年,四分位数范围为(1.5-6)年。环孢素治疗后,平均复发次数/患者/年明显减少了82.6%,平均依赖类固醇剂量减少了69.19%。环孢素副作用包括:94%的患者出现多毛,38%的患者出现牙龈增生,32%的患者出现胃肠道紊乱,28%的患者出现肾毒性,26%的患者出现新发高血压。在活检的儿童中,有 14 人(35.9%)出现了明显的组织学肾毒性。结论对于类固醇依赖型和频繁复发肾病综合征患者来说,环孢素是一种既安全又有效的类固醇替代药物。然而,环孢素的安全性也并非毫无例外,不同程度的副作用已被记录在案。
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Cyclosporine effectiveness and adverse outcomes in steroid dependent and frequent relapsing nephrotic syndrome in children
Background: Cyclosporine is a calcineurin inhibitor that exhibits a notable steroid-sparing effect in patients with steroid-dependent and frequent relapsing nephrotic syndrome. Despite long-term cyclosporine use, it has been linked to an increased risk of unpleasant side effects, including nephrotoxicity, hypertension, and cosmetic symptoms like hirsutism and gum hypertrophy. Aim: To assess the effectiveness and side effects of cyclosporine in children diagnosed with steroid-dependent and frequent relapsing nephrotic syndrome at Sohag University Hospital. Methods: This was a combined prospective and retrospective study which was conducted at Pediatric nephrology clinic, Sohag University Hospital on children diagnosed as steroid-dependent and frequent relapsing nephrotic syndrome who received cyclosporine as a steroid sparing agent. Results: This study included total 50 nephrotic patients, there mean age was 11.4 ± 3.1 (4-15) years. Male: female ratio was 2.8:1. The median disease duration was 7 years with interquartile range (3.88-9.63) years, and the median cyclosporine use duration was 4 years with interquartile range (1.5-6) years. The average relapse number/patient/year significantly decreased by 82.6% following cyclosporine treatment, and the mean dependent steroid dose decreased by 69.19%.The recorded cyclosporine side effects were: hirsutism in 94%, gum hyperplasia in 38%, gastrointestinal disturbances in 32%, nephrotoxicity in 28% and new onset hypertension in 26% of cases. Histological nephrotoxicity was evident in 14 individuals (35.9%) of biopsied children. Conclusion: Cyclosporine is deemed both safe and efficacious as a steroid-sparing medication for individuals with steroid-dependent and frequent relapsing nephrotic syndrome. Nonetheless, the safety of cyclosporine is not without exceptions, and various levels of side effects have been documented.
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