Ahmed Heba Mostafa, Morgan Dalia S, Doudar Noha A, Naguib Mariam S
Department of Pediatrics, Faculty of Medicine, Beni-Suef University, Beni Suef, Egypt.
Department of Clinical and Chemical Pathology, Faculty of Medicine, Beni-Suef University, Beni Suef, Egypt.
Saudi J Kidney Dis Transpl. 2019 Jul-Aug;30(4):769-774. doi: 10.4103/1319-2442.265451.
Nephrotic syndrome (NS) is one of the most common kidney diseases seen in children. It is a disorder characterized by severe proteinuria, hypoproteinemia, hyperlipidemia, and generalized edema resulting from alterations of permeability at the glomerular capillary wall. Endothelin-1 (ET1) has a central role in the pathogenesis of proteinuria and glomerulosclerosis and has a role in assessment of the clinical course of NS in children. This study aims to investigate the relationship between ET1 serum level and the response to steroid therapy in children with primary NS. Serum ET1 levels were evaluated in 55 children with NS. They were classified into two groups: 30 patients with steroid-sensitive NS (SSNS) and 25 patients with steroid-resistant NS (SRNS). The SSNS group was further divided into infrequent-relapsing NS (IFRNS) and steroid-dependent NS (SDNS), while the SRNS group was subdivided into two groups according to renal pathology. ET1 levels were significantly higher in the SRNS group (52.5 ± 45.8 pg/dL) compared to the SSNS group (18.3 ± 17 pg/dL) (P <0.001). Furthermore, ET1 levels were significantly higher in SDNS (54.3 ± 18.6) compared to IFRNS (11.9 ± 7.8, P = 0.001). There was no statistically significant difference in ET1 levels between minimal change disease group and focal segmental glomerulosclerosis group, (P = 0.28). Serum ET1 can be considered as a predictor for response to steroid therapy.
肾病综合征（NS）是儿童中最常见的肾脏疾病之一。它是一种以严重蛋白尿、低蛋白血症、高脂血症和肾小球毛细血管壁通透性改变导致的全身性水肿为特征的病症。内皮素-1（ET1）在蛋白尿和肾小球硬化的发病机制中起核心作用，并且在评估儿童NS的临床病程中发挥作用。本研究旨在探讨原发性NS患儿ET1血清水平与类固醇治疗反应之间的关系。对55例NS患儿的血清ET1水平进行了评估。他们被分为两组：30例类固醇敏感型NS（SSNS）患者和25例类固醇抵抗型NS（SRNS）患者。SSNS组进一步分为罕复发型NS（IFRNS）和类固醇依赖型NS（SDNS），而SRNS组根据肾脏病理分为两组。与SSNS组（18.3±17 pg/dL）相比，SRNS组（52.5±45.8 pg/dL）的ET1水平显著更高（P<0.001）。此外，与IFRNS（11.9±7.8，P = 0.001）相比，SDNS组的ET1水平显著更高（54.3±18.6）。微小病变病组和局灶节段性肾小球硬化组之间的ET1水平无统计学显著差异（P = 0.28）。血清ET1可被视为类固醇治疗反应的预测指标。
