Focal segmental glomerulosclerosis (FSGS) is a chronic glomerular disease that responds poorly to treatment, with a large proportion of patients progressing to end-stage renal disease in spite of initial aggressive treatment. It is worth emphasizing that the FSGS group has still a high recurrence rate after kidney transplantation. Therefore, understanding the factors involved in the pathogenesis of FSGS will help nephrologists better understand the pathogenesis as well as find out specific targeted therapies. Circulating immune factors have long been implicated in the pathogenesis of FSGS, and recent studies have suggested that soluble urokinase plasminogen activator receptor (suPAR) is one of the good candidates for this hypothesis. The aim of this review study was to analyze the value of suPAR in glomerular disease, especially in clinical studies. In this review study, the PubMed database was searched using relevant keywords (suPAR, circulating permeability factors Children, FSGS, and children). Descriptive and cross-sectional studies were reviewed in the current study with the main focuses on the role of suPAR in FSGS, nephrotic syndrome, and the relation to progression of renal failure, especially the research in children. Overall, 32 studies from different countries were selected. These clinical studies on suPAR have shown the following: (i) the role of suPAR in the diagnosis of FSGS has not yet been confirmed, and (ii) there is strong evidence demonstrating a significant relationship between suPAR and the severity of kidney disease as well as a high value of suPAR in predicting the steroid responsiveness of nephrotic syndrome. Researching on circulating permeability factors in FSGS is a current trend, which opens new avenues in targeted diagnosis and treatment. suPAR is a promising candidate, and urinary suPAR has also shown advantages over serum suPAR; therefore, more research on this issue is needed in the future.

中文翻译：

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可溶性尿激酶纤溶酶原激活物受体 (suPAR) 与儿童肾小球疾病：叙述性综述

局灶节段性肾小球硬化症 (FSGS) 是一种慢性肾小球疾病，对治疗反应不佳，尽管初始积极治疗，仍有很大一部分患者进展为终末期肾病。值得强调的是，FSGS组肾移植术后复发率仍然较高。因此，了解FSGS发病机制所涉及的因素将有助于肾科医生更好地了解发病机制，并找到针对性的靶向治疗。循环免疫因子长期以来一直与 FSGS 的发病机制有关，最近的研究表明，可溶性尿激酶纤溶酶原激活物受体 (suPAR) 是这一假设的良好候选者之一。本综述研究的目的是分析 suPAR 在肾小球疾病中的价值，尤其是在临床研究中。在本综述研究中，使用相关关键字（suPAR、循环渗透因子儿童、FSGS 和儿童）搜索了 PubMed 数据库。本研究回顾了描述性和横断面研究，主要关注 suPAR 在 FSGS、肾病综合征中的作用，以及与肾功能衰竭进展的关系，尤其是儿童研究。总体而言，选择了来自不同国家的 32 项研究。这些关于 suPAR 的临床研究表明：(i) suPAR 在诊断 FSGS 中的作用尚未得到证实，并且 (ii) 有强有力的证据表明 suPAR 与肾脏疾病的严重程度之间也存在显着关系作为 suPAR 在预测肾病综合征类固醇反应性方面的高价值。研究FSGS循环通透性因子是当前的趋势，为靶向诊断和治疗开辟了新的途径。suPAR 是一个很有前景的候选者，尿 suPAR 也显示出优于血清 suPAR 的优势；因此，未来需要对这个问题进行更多的研究。