ABSTRACT
Primary nephrotic syndrome (PNS) is one of the most common glomerular diseases in children.Steroid-resistant nephrotic syndrome (SRNS) is defined as no remission of nephrotic syndrome after daily Prednisone treatment at an adequate oral dose for 4 weeks, which is classified as idiopathic SRNS and inherited SRNS according to the presence or absence of monogenic disorder.At present, steroid combined with calcineurin inhibitor (CNI) is preferred to idiopathic SRNS, and 50%-70% of children can achieve complete or partial remission.A small proportion of children with idiopathic SRNS and the majority of inherited SRNS may be resistant to different immunosuppressive agents with two different mechanisms, including CNI.They, unfortunately, gradually progress to end-stage renal disease within 5-10 years.In this paper, the therapeutic advances and future prospects in pediatric SRNS are reviewed.