Al Dash Hanaa, Ahmed Heba Mostafa, Shihatah Shireen Ragab, Abdelghaffar Noha Khalifa, Mostafa Mona Gamal, Hussein Sherin Khamis
Department of Pediatrics, Faculty of Medicine, Fayoum University, Fayoum, Egypt.
Department of Pediatrics, Faculty of Medicine, Beni-Suef University, Beni-Suef, Egypt.
Pediatr Nephrol. 2025 Sep 18. doi: 10.1007/s00467-025-06966-0.
Idiopathic nephrotic syndrome (INS) is a significant kidney disorder in pediatrics. Early diagnosis of minimal change disease (MCD) is difficult in children with nephrotic syndrome (NS). Angiopoietin-like protein 4 (ANGPTL4), found on the surface of podocytes, has been linked to nephrotic syndrome (NS) and plays a role in triggering proteinuria. Macrophage migration inhibitory factor (MIF) functions as a crucial modulator of the innate immune system and partly counteracts glucocorticoid-induced immune system inhibition. This study aimed to assess the role of ANGPTL4 and MIF as biomarkers in steroid responsiveness of INS.
This cross-sectional comparative study involved 70 children with NS and 40 healthy children as a control group.
Urinary MIF/creatinine levels were significantly elevated in steroid-resistant nephrotic syndrome (SRNS) relative to in steroid-sensitive nephrotic syndrome (SSNS) and controls (p < 0.001). However, ANGPTL4 levels were significantly elevated in the SSNS group relative to the SRNS and control groups (p < 0.001). Regarding plasma MIF and urinary MIF/creatinine levels, there were no significant differences between MCD and FSGS, whereas ANGPTL4 levels were significantly elevated in MCD relative to FSGS (p < 0.001).
Elevated levels of serum and urinary MIF levels were consistent with SRNS. Furthermore, ANGPTL4 was found to be highly upregulated in SSNS, unlike SRNS, which serves as a potential marker to distinguish between these two diseases.
特发性肾病综合征（INS）是儿科一种重要的肾脏疾病。肾病综合征（NS）患儿中微小病变病（MCD）的早期诊断较为困难。足细胞表面发现的血管生成素样蛋白4（ANGPTL4）与肾病综合征（NS）有关，并在引发蛋白尿中起作用。巨噬细胞移动抑制因子（MIF）作为先天性免疫系统的关键调节因子，部分抵消糖皮质激素诱导的免疫系统抑制作用。本研究旨在评估ANGPTL4和MIF作为生物标志物在INS类固醇反应性中的作用。
本横断面比较研究纳入了70例NS患儿和40例健康儿童作为对照组。
相对于激素敏感型肾病综合征（SSNS）和对照组，激素抵抗型肾病综合征（SRNS）患儿尿MIF/肌酐水平显著升高（p < 0.001）。然而，相对于SRNS组和对照组，SSNS组的ANGPTL水平显著升高（p < 0.001）。关于血浆MIF和尿MIF/肌酐水平，MCD和局灶节段性肾小球硬化症（FSGS）之间无显著差异，而相对于FSGS，MCD中的ANGPTL4水平显著升高（p < 0.001）。
血清和尿MIF水平升高与SRNS一致。此外，与SRNS不同，ANGPTL4在SSNS中高度上调，可作为区分这两种疾病的潜在标志物。
