What is circulating factor disease and how is it currently explained?

What is circulating factor disease and how is it currently explained?
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DOI:
10.1007/s00467-023-05928-8
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发表时间:
2023-11
期刊:
Pediatric nephrology (Berlin, Germany)
影响因子:
--
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其他
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肾病综合征(NS)由低白蛋白血症、高水平蛋白尿和水肿的临床三联征组成,描述了一组具有不同潜在驱动因素的异质性疾病过程。循环因子疾病(CFD)作为NS的驱动因素的存在已经被移植后表现出疾病复发的患者子集以及实验室工作所概括。已经提出并研究了几种循环因子,大致分为蛋白酶组分如可溶性尿激酶型纤溶酶原激活剂(suPAR)、血红素结合蛋白(Hx)和钙/钙调蛋白-丝氨酸蛋白酶激酶(CASK)和其他循环蛋白酶,以及免疫组分如TNF-α、CD 40和心肌营养素样细胞因子-1(CLC-1)。虽然目前还没有明确的方法来评估CFD移植前的风险,但通过研究来自大型国家队列和生物库的“多组学”生物信息学数据,正在出现有希望的工作。
Nephrotic syndrome (NS) consists of the clinical triad of hypoalbuminaemia, high levels of proteinuria and oedema, and describes a heterogeneous group of disease processes with different underlying drivers. The existence of circulating factor disease (CFD) as a driver of NS has been epitomised by a subset of patients who exhibit disease recurrence after transplantation, alongside laboratory work. Several circulating factors have been proposed and studied, broadly grouped into protease components such as soluble urokinase-type plasminogen activator (suPAR), hemopexin (Hx) and calcium/calmodulin-serine protease kinase (CASK), and other circulating proteases, and immune components such as TNF-α, CD40 and cardiotrophin-like cytokine-1 (CLC-1). While currently there is no definitive way of assessing risk of CFD pre-transplantation, promising work is emerging through the study of ‘multi-omic’ bioinformatic data from large national cohorts and biobanks.
DOI: 10.1007/s00467-021-05134-4
发表时间: 2021-11
期刊: Pediatric nephrology (Berlin, Germany)
影响因子: --
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