Abstract：
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.
摘要：
特发性肾病综合征(NS)是全世界儿童常见的肾小球疾病,该疾病的确切发病机制仍然未知。一些研究表明，肿瘤坏死因子-α（TNF-α），一种促炎细胞因子，在NS的发病机制中起着重要作用。文献缺乏足够的数据来确定TNF-α与NS之间的关系。这项前瞻性研究是对诊断为特发性NS的1-14岁儿童进行的。所有入选的个体从NS的疾病发作或复发进行随访，直到缓解，或者如果未达到缓解，则使用类固醇治疗至少42天。在出现和缓解时或在类固醇治疗42天后，如果未达到缓解，则测量血清TNF-α水平。还评估了TNF-α水平在NS中对类固醇治疗的反应中的作用。招募了112名特发性NS儿童（68％的男孩）。入学时的中位年龄（四分位数范围）为58.5（37-84.7）个月，而症状发作的中位年龄为47.5(24-60.7)个月。报告时的中位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患者的TNF-α水平中位数在缓解时分别为3.98pg/ml和1.88pg/ml（P=0.04），而在类固醇抗性NS中，在给药时为6.59pg/ml,在42天时为9.02pg/ml(P=.45).激素治疗持续时间与TNF-α水平呈显著负相关,相关系数为-0.021，R2为0.154（P≤0.001）。血清TNF-α水平降低与类固醇治疗儿童的类固醇敏感NS,这在临床上与缓解的实现相关。
