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Treatment of nephrotic syndrome: going beyond immunosuppressive therapy.
Pediatric Nephrology ( IF 2.6 ) Pub Date : 2019-03-25 , DOI: 10.1007/s00467-019-04225-7
Jinghong Zhao 1 , Zhihong Liu 2
Affiliation  

It is indisputable that immunosuppressive therapy and pathological diagnosis of renal biopsy have greatly improved the prognosis of childhood nephrotic syndrome. Unfortunately, there is no "one-size-fits-all" approach for precise patient stratification and treatment when facing the huge challenges posed by steroid-resistant nephrotic syndrome (SRNS). But genomic medicine has brought a glimmer of light, and the cognition of SRNS has entered a new stage. Based on this, identification of single genetic variants of SRNS has recognized the key role of podocyte injury in its pathogenesis. Targeted treatment of podocyte injury is paramount, and immunosuppressant with podocyte-targeted therapy seems to be more suitable as the first choice for SRNS, that is, we need to pay attention to their additional non-immunosuppressive effects. In the same way, other effect factors of nephrotic syndrome and the related causes of immunosuppressive therapy resistance require us to select reasonable and targeted non-immunosuppressive therapies, instead of only blindly using steroids and immunosuppressants, which may be ineffective and bring significant side effects. This article provides a summary of the clinical value of identification of genetic variants in podocytes and non-immunosuppressive therapy for nephrotic syndrome in children.

中文翻译:

肾病综合征的治疗:超越免疫抑制治疗。

毫无疑问,免疫抑制疗法和肾脏活检的病理学诊断已大大改善了儿童肾病综合征的预后。不幸的是,当面临类固醇耐药性肾病综合征(SRNS)带来的巨大挑战时,没有“千篇一律”的方法可以精确地对患者进行分层和治疗。但是基因组医学带来了一线曙光,SRNS的认知进入了一个新阶段。基于此,对SRNS单个遗传变异的鉴定已认识到足细胞损伤在其发病机理中的关键作用。足细胞损伤的靶向治疗至关重要,而足细胞靶向治疗的免疫抑制剂似乎更适合作为SRNS的首选,也就是说,我们需要注意它们的其他非免疫抑制作用。同样,肾病综合征的其他影响因素和免疫抑制治疗耐药性的相关原因要求我们选择合理且针对性的非免疫抑制疗法,而不是仅盲目地使用类固醇和免疫抑制剂,这可能无效且带来明显的副作用。本文总结了儿童肾病综合征足细胞遗传变异的鉴定和非免疫抑制治疗的临床价值。
更新日期:2020-03-04
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