Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India.
J Trop Pediatr. 2024 Aug 10;70(5). doi: 10.1093/tropej/fmae025.
Idiopathic nephrotic syndrome (NS) is a common glomerular disease in children throughout the world; however, the exact pathogenesis of the disease remains unknown. Several studies have shown that tumour necrosis factor-alpha (TNF-α), a proinflammatory cytokine, plays a significant role in the pathogenesis of NS. The literature lacks sufficient data to establish the relationship between TNF-α and NS. This prospective study was conducted on children aged 1-14 years diagnosed with idiopathic NS. All enrolled individuals were followed up from disease onset or relapse of NS until remission or at least 42 days with steroid therapy if remission was not achieved. Serum TNF-α levels were measured at presentation and remission or after 42 days of steroid therapy if remission was not achieved. The role of TNF-α levels in response to steroid therapy in NS was also assessed. One hundred and twelve children (68% boys) with idiopathic NS were enrolled. The median age (interquartile range) at enrolment was 58.5 (37-84.7) months, while the median age at symptom onset was 47.5 (24-60.7) months. The median TNF-α level at presentation was 7.5 (3.5-12.1) pg/ml, and that at remission was 5.25 (1.62-8.8) pg/ml. The median TNF-α levels among first-episode NS at presentation were 3.98 pg/ml and 1.88 pg/ml (P = .04) at remission, whereas in steroid-resistant NS, it was 6.59 pg/ml at presentation and 9.02 pg/ml at 42 days (P = .45). There was a significant negative correlation between the duration of steroid therapy and TNF-α levels, with a correlation factor of -0.021 and R2 of 0.154 (P≤.001). Serum TNF-α levels decrease with steroid therapy in children with steroid-sensitive NS, which correlates clinically with the achievement of remission.
特发性肾病综合征 (NS) 是全世界儿童中常见的肾小球疾病;然而,疾病的确切发病机制仍不清楚。几项研究表明,肿瘤坏死因子-α (TNF-α),一种促炎细胞因子,在 NS 的发病机制中起重要作用。文献中缺乏足够的数据来确定 TNF-α与 NS 之间的关系。这项前瞻性研究在 1-14 岁被诊断为特发性 NS 的儿童中进行。所有入组的个体均从 NS 发病或复发开始随访,直至缓解或至少在未达到缓解时进行 42 天的类固醇治疗。在发病时和未达到缓解时进行 42 天的类固醇治疗时测量血清 TNF-α 水平。还评估了 TNF-α 水平在 NS 对类固醇治疗反应中的作用。入组了 112 名(68%为男性)特发性 NS 儿童。入组时的中位年龄(四分位距)为 58.5(37-84.7)个月,而症状发作时的中位年龄为 47.5(24-60.7)个月。发病时的中位 TNF-α 水平为 7.5(3.5-12.1)pg/ml,缓解时为 5.25(1.62-8.8)pg/ml。首次发作 NS 患儿发病时的中位 TNF-α 水平分别为 3.98pg/ml 和 1.88pg/ml(P=.04),缓解时分别为 1.88pg/ml 和 1.88pg/ml(P=.04);而在类固醇耐药性 NS 中,分别为 6.59pg/ml 和 9.02pg/ml(P=.45)。类固醇治疗的持续时间与 TNF-α 水平呈显著负相关,相关系数为-0.021,R2 为 0.154(P≤.001)。在对类固醇敏感的 NS 儿童中,随着类固醇治疗,血清 TNF-α 水平降低,这与达到缓解在临床上相关。