INSERM U1197, Hôpital Paul Brousse, 14 Avenue Paul Vaillant Couturier, 94800, Villejuif, France.
Université Paris-Saclay, Campus Universitaire d'Orsay, 91405, Orsay, France.
Cell Tissue Res. 2020 Feb;379(2):231-243. doi: 10.1007/s00441-019-03147-y. Epub 2019 Dec 17.
Nephrotic syndrome is traditionally defined using the triad of edema, hypoalbuminemia, and proteinuria, but this syndrome is very heterogeneous and difficult to clarify. Its idiopathic form (INS) is probably the most harmful and essentially comprises two entities: minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). We will consider some hypotheses regarding the mechanisms underlying INS: (i) the presence of several glomerular permeability factors in the sera of patients that alter the morphology and function of podocytes leading to proteinuria, (ii) the putative role of immune cells. Thanks to recent data, our understanding of these disorders is evolving towards a more multifactorial origin. In this context, circulating factors may be associated according to sequential kinetic mechanisms or micro-environmental changes that need to be determined. In addition, the resulting proteinuria may trigger more proteinuria enhancing the glomerular destabilization.
肾病综合征传统上是根据水肿、低白蛋白血症和蛋白尿的三联征来定义的，但这种综合征非常异质，难以明确。其特发性形式（INS）可能是最有害的，主要包括两种实体：微小病变性肾病（MCD）和局灶节段性肾小球硬化症（FSGS）。我们将考虑一些关于 INS 潜在机制的假设：（i）患者血清中存在几种肾小球通透性因子，改变足细胞的形态和功能，导致蛋白尿，（ii）免疫细胞的潜在作用。得益于最近的数据，我们对这些疾病的理解正在朝着更具多因素起源的方向发展。在这种情况下，循环因子可能根据顺序动力学机制或需要确定的微环境变化相关联。此外，由此产生的蛋白尿可能会引发更多的蛋白尿，从而加剧肾小球的不稳定。
