关键词: 肾病综合征, 激素抵抗, 局灶节段肾小球硬化症, 足细胞

Abstract: Currently,immunosuppressive therapy  based on pathological diagnosis from renal biopsy,has been accepting as routine algorithm for nephrotic syndrome.Unfortunately,there is no “ onesizefitsall” approach for precise patient stratification and treatment when facing the huge challenges posed by steroidresistant nephrotic syndrome(SRNS),children with SRNS undergoing renal biopsy do not respond to immunosuppressive therapy,and 50% of them will progress to endstage renal disease (ESRD) within 10 years.Extensive researches have proposed the podocyte as a crucial site of cellular injury in SRNS,and more than 70 genes are identified essential for podocyte development,structures and functions,which have been proved to play a key role in the pathogenesis of SRNS/FSGS (focal segmental glomeruloscelerosis).In future,The molecular ontology of SRNS in the era of NGS will ultimately help not only to uncover the pathogenic pathways,redefine diagnostic classification and prognostic assessment,but also to provide targets to guide the personalized medical management.

Key words: nephrotic syndrome, steriod-resistant, focal segmental glomerulosclerosis, podocyte