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脉冲长春新碱疗法治疗激素抵抗型肾病综合征。

Pulsed Vincristine Therapy in Steroid-Resistant Nephrotic Syndrome.

机构信息

Department of Paediatrics, Faculty of Medicine, University of Peradeniya, Kandy, Sri Lanka.

Outpatient Department, Teaching Hospital Peradeniya, Peradeniya, Sri Lanka.

出版信息

Biomed Res Int. 2017;2017:1757940. doi: 10.1155/2017/1757940. Epub 2017 May 29.

DOI:10.1155/2017/1757940
PMID:28630858
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5467295/
Abstract

Steroid-resistant nephrotic syndrome (SRNS) poses a therapeutic challenge for the paediatric nephrologist. As relentless progression to renal failure occurs with continued proteinuria, such patients will be treated with different cytotoxic medications with variable success rates and side-effects. We present here our findings on administering the anticancer drug vincristine for SRNS patients at a single centre in Sri Lanka. . Between 2002 and 2007, fifty-four children presenting with steroid and cyclophosphamide resistance were treated with vincristine at 1.5 mg/m in weekly intravenous pulses for 8 weeks along with a tapering steroid regimen of 6 months. All patients were closely followed up for 5 years. . Of the 54 patients 39 were males and 15 were females (age range 3.5-11.6 years, median 6.1 years). At the end of the treatment course, 21 patients achieved complete remission while 7 had partial remission and no response was seen in 26 patients. Sustained remission at 6, 12, 24, and 60 months were 15 (27.78%), 11 (20.37%), 9 (16.67%), and 7 (12.96%), respectively. Most side-effects observed were reversible and no serious side-effects were noted during vincristine therapy. . Although its therapeutic mechanisms in nephrotic syndrome are still not elucidated, vincristine appears to be a potent alternative that could be considered for treating SRNS.

摘要

类固醇抵抗型肾病综合征 (SRNS) 对儿科肾病学家来说是一个治疗挑战。由于持续的蛋白尿会导致肾功能不断恶化,因此此类患者将接受不同的细胞毒性药物治疗,但成功率和副作用各不相同。我们在此报告在斯里兰卡的一个中心使用抗癌药物长春新碱治疗 SRNS 患者的结果。 在 2002 年至 2007 年间,54 名对类固醇和环磷酰胺耐药的儿童接受了长春新碱治疗,剂量为 1.5mg/m2,每周静脉推注 8 周,同时给予 6 个月的类固醇逐渐减量方案。所有患者均密切随访 5 年。 在 54 名患者中,39 名男性,15 名女性(年龄 3.5-11.6 岁,中位数 6.1 岁)。在治疗结束时,21 名患者完全缓解,7 名部分缓解,26 名无反应。6、12、24 和 60 个月时的持续缓解率分别为 15(27.78%)、11(20.37%)、9(16.67%)和 7(12.96%)。观察到的大多数副作用是可逆的,在长春新碱治疗期间未出现严重副作用。 尽管其在肾病综合征中的治疗机制尚不清楚,但长春新碱似乎是一种有效的替代药物,可考虑用于治疗 SRNS。

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Cell Biosci. 2017 Jan 3;7:1. doi: 10.1186/s13578-016-0129-z. eCollection 2017.
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Cochrane Database Syst Rev. 2016 Oct 11;10(10):CD003594. doi: 10.1002/14651858.CD003594.pub5.
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Steroid-resistant idiopathic nephrotic syndrome in children: long-term follow-up and risk factors for end-stage renal disease.
儿童特发性类固醇抵抗性肾病综合征的干预措施。
Cochrane Database Syst Rev. 2019 Nov 21;2019(11):CD003594. doi: 10.1002/14651858.CD003594.pub6.
儿童类固醇抵抗型特发性肾病综合征:长期随访及终末期肾病的危险因素
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Inhibition of podocyte FAK protects against proteinuria and foot process effacement.抑制足细胞 FAK 可预防蛋白尿和足突融合。
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