Abstract: Primary nephrotic syndrome is the most common glomerular disease in childhood.The 2025 KDIGO Guidelines integrate the latest evidence to update the diagnosis and treatment strategies for childhood steroid-sensitive nephrotic syndrome（SSNS）and steroid-resistant nephrotic syndrome（SRNS）.In diagnosis，some definitions are revised，and a“confirmation period”is proposed to clarify the diagnosis of SRNS；prognosis is related to the response to glucocorticoids and the frequency of relapses. In treatment，an 8-12-week regimen of initial glucocorticoids is recommended，with individualized use of glucocorticoids during infections. For frequently relapsing/steroid-dependent nephrotic syndrome（FR/SDNS），combination therapy with steroid-sparing agents［such as calcineurin inhibitors （CNIs），rituximab，etc.］is suggested. For SRNS，genetic testing should be performed as soon as possible，with CNIs as the initial second-line treatment. The outlook indicates that future research should focus on the exploration of shortening the initial course of glucocorticoids，optimizing the selection and dosage of steroid-sparing agents，achieving stratified diagnosis and treatment by integrating genetics and biomarkers，while paying attention to the safety of B-cell depletion therapies and quality-of-life endpoints to promote precision medicine practice.

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摘要： 原发性肾病综合征是儿童期最常见肾小球疾病。《KDIGO 2025版儿童肾病综合征临床实践指南》整合最新证据，更新儿童激素敏感型肾病综合征（SSNS）与激素耐药型肾病综合征（SRNS）诊治策略。诊断方面修订部分定义，提出“确认期”以明确 SRNS 诊断，预后与激素应答及复发频率相关。治疗方面，初始糖皮质激素推荐8~12周方案，感染期激素使用需个体化，频复发或激素依赖型肾病综合征（FR/SDNS）建议联用激素助减剂如钙调神经磷酸酶抑制剂（CNI）、利妥昔单抗等，SRNS 需尽早行基因检测，CNI 作为初始二线治疗。展望未来需探索缩短激素初始疗程、优化助减剂选择及剂量，结合基因与生物标志物实现分层诊疗，同时关注 B 细胞耗竭疗法安全性及生活质量终点，以推动精准医学实践。

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