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儿童激素依赖频复发型肾病综合征的研究进展及展望
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Abstract:
激素依赖及频复发肾病(steroid-dependent/frequent relapsing nephrotic syndrome, SDNS/FRNS)是儿童原发性肾病综合征(primary nephrotic syndrome, PNS)的重要激素应答类型。SDNS/FRNS患者主要表现为激素依赖及频繁复发，其治疗过程棘手，部分患者甚至进展为终末期肾病，对患者家庭及社会构成负担，其机制仍不清楚，既往研究大多聚焦复杂免疫分子网络协同参与SDNS/FRNS发生、发展。近年来，随着多组学技术开展，对SDNS/FRNS疾病有了新的认识。本文从SDNS/FRNS的临床表现、发病机制(T细胞免疫紊乱、B细胞免疫功能紊乱、调节性T细胞异常及足细胞异常)、最新治疗方案等几个重要环节进行综述，为儿童临床PNS肾脏疾病管理提供新的策略。
Steroid-dependent and frequent relapsing nephrotic syndrome is an important type of response to steroid in primary nephrotic syndrome (PNS) in children. SDNS/FRNS patients develop frequently relapsing or steroid-dependent forms, and the treatment is difficult, some patients even progress to ESRD, which increases the burden of patient’s family and social burden. The pathogenesis of PNS is as yet unknown, most previous studies have focused on complex immune modulation to participate in the occurrence and development of SDNS/FRNS. In recent years, with the development of multi-omics profiling, there has been a new understanding of SDNS/FRNS. In this review, we shed light on clinical manifestations, its pathogenesis (T-cell dysregulation, B-cell dysfunction, circulating regulatory Tcells (Tregs), abnormalities functional alterations of podocytes), and recently treatment protocols, advances in our understanding of the pathogenesis of PNS may help drive new approaches.
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