Tamura Hiroshi
Department of Pediatrics, Kumamoto University, Kumamoto 8608556, Japan.
World J Nephrol. 2021 Sep 25;10(5):88-100. doi: 10.5527/wjn.v10.i5.88.
Nephrotic syndrome (NS) is relatively common in children, with most of its histological types being minimal changed disease. Its etiology has long been attributed to lymphocyte (especially T-cell) dysfunction, while T-cell-mediated vascular hyperpermeability increases protein permeability in glomerular capillaries, leading to proteinuria and hypoproteinemia. Based on this etiology, steroids and immunosuppressive drugs that are effective against this disease have also been considered to correct T-cell dysfunction. However, in recent years, this has been questioned. The primary cause of NS has been considered damage to glomerular epithelial cells and podocyte-related proteins. Therefore, we first describe the changes in expression of molecules involved in NS etiology, and then describe the mechanism by which abnormal expression of these molecules induces proteinuria. Finally, we consider the mechanism by which infection causes the recurrence of NS.
肾病综合征(NS)在儿童中较为常见,其组织学类型大多为微小病变型肾病。长期以来,其病因一直被归因于淋巴细胞(尤其是T细胞)功能障碍,而T细胞介导的血管通透性增加会导致肾小球毛细血管中的蛋白质通透性增加,从而导致蛋白尿和低蛋白血症。基于这一病因,对该疾病有效的类固醇和免疫抑制药物也被认为可以纠正T细胞功能障碍。然而,近年来,这一观点受到了质疑。NS的主要病因被认为是肾小球上皮细胞和足细胞相关蛋白的损伤。因此,我们首先描述NS病因中涉及的分子表达变化,然后描述这些分子异常表达诱导蛋白尿的机制。最后,我们探讨感染导致NS复发的机制。