Recent Advances in Genetic Aspects and Treatments for Steroid-Sensitive Nephrotic Syndrome in Children

Abstract

This article reviews recent advances in genetic aspects and treatments for steroid-sensitive nephrotic syndrome (SSNS) in children over the previous 5 years. Six genes, which have roles in Rho-like small GTPase activity, have been identified as causes of partially steroid-treatable nephrotic syndrome, and the strongest susceptible gene of SSNS has been located in the HLA DR/DQ locus. For treatment, prolongation of prednisolone therapy beyond 2 to 3 months in the initial episode of SSNS does not reduce the risk of relapse. Rituximab has been established as a treatment for complicated frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome. Rituximab could also be the first-line treatment for early-stage-uncomplicated FRNS/SDNS, although further studies are required to confirm its efficacy and safety. The understanding of pathogenesis and development of new treatments of steroid-sensitive nephrotic syndrome in children made a significant advance over the previous 5 years.