耐药性肾病综合征相关因素的研究进展

临床经标准激素治疗无效应者称为激素耐药型肾病综合征,激素耐药发生机制的阐明对治疗有着重要的指导意义,近年来越来越多的研究表明基因突变、脂质代谢紊乱、免疫失调、炎症介质等因素在耐药型肾病综合征的发生机制中起着重要的作用。近年来越来越多的研究表明与基因突变有关,其中可发生NPHS2基因突变,在NPHS2基因8个外显子和部分内含子,从而导致激素耐药。GRα表达降低和GRβ表达亢进,可能是亦导致耐药。激素耐药肾病的病理类型呈多样化,表现为数种病理类型,以FSGS、MSPGN、IGM肾病为主。

Research Progress on Related Factors of Steroid - resistant Nephrotic Syndrome

Steroid-resistant nephrotic syndrome(SRNS) is defined by primary resistance to standard steroid therapy.To clarify the mechanism of SRNS is very important for guiding the treatment.In recent years,an increasing number of studies have shown that gene mutations,lipid metabolism,immune disorders,inflammatory mediators and other factors may play an important role in the occurrence of drug resistance mechanisms of nephrotic syndrome.Among them,the mutations in the NPHS2 gene represented a frequent cause of SRNS,which were suspected with homozygous or compound heterozygous mutations in NPHS2.It can be found on every exon of all eight exons of the NPHS2 gene.The decreased level of GRα and the increased level of GRβ may also lead to SRNS.The pathological types of SRNS are various,and the most common types are FSGS,MSPGN,and IGM nephropathy.