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.