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.