儿童原发性肾病综合征发病机制研究现况及展望

Current situation and prospect of research on pathogenesis of primary nephrotic syndrome in children

摘要:

原发性肾病综合征（primary nephrotic syndrome，PNS）是儿童最常见的肾小球疾病之一，也是我国儿童慢性肾功能不全的重要病因。目前PNS的发病机制尚未完全清楚。近年来，国内外对PNS的基础及临床研究取得许多进展。本文从该病发病的诱发因素、特定遗传背景、免疫功能紊乱、产生血浆循环因子、肾小球滤过屏障受损等几个重要环节进行综述。目前PNS依然还有很多未解的临床及基础问题，但随着高通量检测、生物信息分析等新技术手段的应用，将有助于发现更多的新分子靶标及标志物，切实推动该病的临床精准诊断与个性化治疗，以最终改善该病的预后。

Abstract:

Primary nephrotic syndrome (PNS) is one of the most common glomerular diseases in children and an important cause of chronic renal insufficiency in children in China. At present, the pathogenesis of PNS is not completely clear. In recent years, many advances have been made in the basic and clinical studies of PNS. In this paper, the authors reviewed the important aspects of the disease, such as the predisposing factors, the specific genetic background, the immune dysfunction, the production of plasma circulating factors, and the damage of glomerular filtration barrier. Nowdays, there are still many unsolved clinical and basic problems in PNS, but with the application of high-throughput sequence, bioinformatics analysis and other new technical means, which will contribute to finding more new molecular targets and markers, effectively promoting the accurate clinical diagnosis and personalized treatment of the disease, and ultimately improving the prognosis of the disease.