Shah Syed Raza, Altaf Areeba, Arshad Mohammad Hussham, Mari Anum, Noorani Sahir, Saeed Eraj, Mevawalla Areesh Amir, Haq Zaiyn Ul, Faquih Muhammad Ehsan
Dow Medical College, Dow University of Health Sciences (DUHS).
Glob J Health Sci. 2015 Aug 6;8(4):136-41. doi: 10.5539/gjhs.v8n4p136.
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.
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。