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Glob J Health Sci. 2015 Aug 6;8(4):136-41. doi: 10.5539/gjhs.v8n4p136.
2
Cyclosporine in patients with steroid-resistant nephrotic syndrome: an open-label, nonrandomized, retrospective study.环孢素治疗激素抵抗型肾病综合征患者:一项开放标签、非随机、回顾性研究。
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Rapid Response to Cyclosporin A and Favorable Renal Outcome in Nongenetic Versus Genetic Steroid-Resistant Nephrotic Syndrome.非遗传性与遗传性激素抵抗型肾病综合征对环孢素A的快速反应及良好肾脏转归
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Efficacy and Safety of Rituximab in Children With Steroid- and Cyclosporine-resistant and Steroid- and Cyclosporine-dependent Nephrotic Syndrome.利妥昔单抗治疗激素及环孢素抵抗型和激素及环孢素依赖型儿童肾病综合征的疗效与安全性
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Sequential maintenance therapy with cyclosporin A and mycophenolate mofetil for sustained remission of childhood steroid-resistant nephrotic syndrome.环孢素 A 和霉酚酸酯序贯维持治疗儿童激素耐药性肾病综合征的持续缓解。
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本文引用的文献

1
Chapter 4: Steroid-resistant nephrotic syndrome in children.第4章:儿童类固醇抵抗型肾病综合征
Kidney Int Suppl (2011). 2012 Jun;2(2):172-176. doi: 10.1038/kisup.2012.17.
2
Clinical trial of focal segmental glomerulosclerosis in children and young adults.儿童及青年局灶节段性肾小球硬化症的临床试验。
Kidney Int. 2011 Oct;80(8):868-78. doi: 10.1038/ki.2011.195. Epub 2011 Jul 6.
3
Cyclosporine A and steroid therapy in childhood steroid-resistant nephrotic syndrome.环孢素A与类固醇疗法治疗儿童类固醇抵抗型肾病综合征
Int J Nephrol Renovasc Dis. 2010;3:117-21. doi: 10.2147/IJNRD.S10168. Epub 2010 Aug 24.
4
Treatment of steroid resistant nephrotic syndrome in children.儿童类固醇抵抗型肾病综合征的治疗
Saudi J Kidney Dis Transpl. 2010 May;21(3):484-7.
5
Experience with tacrolimus in children with steroid-resistant nephrotic syndrome.他克莫司治疗儿童激素抵抗型肾病综合征的经验
Pediatr Nephrol. 2009 Aug;24(8):1517-23. doi: 10.1007/s00467-009-1220-z. Epub 2009 Jun 5.
6
Management of steroid resistant nephrotic syndrome.类固醇抵抗型肾病综合征的管理
Indian Pediatr. 2009 Jan;46(1):35-47.
7
Stable kidney function in the second decade after kidney transplantation while on cyclosporine-based immunosuppression.肾移植术后第二个十年,在使用基于环孢素的免疫抑制治疗时肾功能稳定。
Transplantation. 2007 Mar 27;83(6):722-6. doi: 10.1097/01.tp.0000256179.14038.e2.
8
Benefits of cyclosporine absorption profiling in nephrotic syndrome: preprandial once-daily administration of cyclosporine microemulsion improves slow absorption and can standardize the absorption profile.环孢素吸收谱分析在肾病综合征中的益处:环孢素微乳剂每日一次餐前给药可改善吸收缓慢的情况,并能使吸收谱标准化。
Nephrology (Carlton). 2007 Apr;12(2):197-204. doi: 10.1111/j.1440-1797.2007.00773.x.
9
Independent risk factors for chronic cyclosporine induced nephropathy in children with nephrotic syndrome.肾病综合征患儿慢性环孢素诱导性肾病的独立危险因素。
Arch Dis Child. 2006 Aug;91(8):666-70. doi: 10.1136/adc.2005.080960. Epub 2006 May 2.
10
Cyclosporine in steroid dependent and resistant childhood nephrotic syndrome.环孢素治疗儿童激素依赖型和激素抵抗型肾病综合征
Indian Pediatr. 2006 Jan;43(1):14-9.

环孢素疗法在激素抵抗型肾病综合征(SRNS)中的应用:综述

Use of Cyclosporine Therapy in Steroid Resistant Nephrotic Syndrome (SRNS): A Review.

作者信息

Shah Syed Raza, Altaf Areeba, Arshad Mohammad Hussham, Mari Anum, Noorani Sahir, Saeed Eraj, Mevawalla Areesh Amir, Haq Zaiyn Ul, Faquih Muhammad Ehsan

机构信息

Dow Medical College, Dow University of Health Sciences (DUHS).

出版信息

Glob J Health Sci. 2015 Aug 6;8(4):136-41. doi: 10.5539/gjhs.v8n4p136.

DOI:10.5539/gjhs.v8n4p136
PMID:26573045
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4873588/
Abstract

A chronic, progressive disorder Steroid Resistant Nephrotic Syndrome (SRNS) accounts for 10-20% of all children with Nephrotic Syndrome. It is a heterogeneous disorder comprised of persistent edema, proteinuria, hypoalbuminemia and hyperlipidemia. Treatment for steroid-resistant nephrotic syndrome (SRNS) is challenging and children who suffer from SRNS require aggressive treatment to achieve remission. Calcineurin inhibitors have been used more in an empirical manner than on the basis of clear rationale. It was in 1984 when cyclosporine was first considered for the treatment of steroid resistant nephrotic syndrome. Cyclosporin is a calcineurin inhibitor that suppresses immune response by downregulating the transcription of various cytokine genes. Till now many studies have been conducted to determine dosages, duration of therapy, side effects and advantages of cyclosporine. Treatment of SRNS remains a difficult challenge in pediatric nephrology.  Treatment should be individualized according to the underlying histopathology, and clinical and environmental conditions of the children. There is an urgent need to distinguish as soon as possible those patients who may benefit from prolonged immunosuppressive treatment from those who will not benefit from such treatment and who will just suffer from its major side effects. The emerging evidence that the majority of genetic forms of SRNS should receive symptomatic treatment only, should also be clinically tested and studies baring its significance should be evaluated in the future.

摘要

类固醇抵抗型肾病综合征(SRNS)是一种慢性进行性疾病,占所有肾病综合征患儿的10%-20%。它是一种异质性疾病,由持续性水肿、蛋白尿、低蛋白血症和高脂血症组成。类固醇抵抗型肾病综合征(SRNS)的治疗具有挑战性,患有SRNS的儿童需要积极治疗以实现缓解。钙调神经磷酸酶抑制剂的使用更多是基于经验而非明确的理论依据。1984年,环孢素首次被考虑用于治疗类固醇抵抗型肾病综合征。环孢素是一种钙调神经磷酸酶抑制剂,通过下调各种细胞因子基因的转录来抑制免疫反应。到目前为止,已经进行了许多研究来确定环孢素的剂量、治疗持续时间、副作用和优势。SRNS的治疗在儿科肾脏病学中仍然是一项艰巨的挑战。治疗应根据潜在的组织病理学以及儿童的临床和环境状况进行个体化。迫切需要尽快区分哪些患者可能从长期免疫抑制治疗中获益,哪些患者不会从这种治疗中获益,反而会遭受其主要副作用。大多数遗传形式的SRNS仅应接受对症治疗这一新兴证据,也应进行临床检验,并且未来应评估阐明其意义的研究。