Childhood Idiopathic Nephrotic Syndrome as a Podocytopathy

Abstract

Idiopathic nephrotic syndrome is the commonest manifestation of glomerular disease in children. The syndrome is characterized by massive proteinuria, hypo-albuminemia, generalized edema, and hyperlipidemia. Although genetic or con-genital forms are now well recognized, nephrotic syndrome is largely acquired. The latter form can be idiopathic or primary (the causes are unknown) and secondary (the causes are known renal or non-renal diseases). Idiopathic nephrotic syndrome consists of the following glomerulonephritides: minimal change nephropathy (MCN), focal segmental glomerulosclerosis (FSGS), membranoproliferative glomerulonephritis (MPGN), mesangial proliferative glomerulonephritis (MesPGN), and membranous nephritis (MN). The etiopathogenesis of nephrotic syndrome has evolved through several hypotheses ranging from immune dysregulation theory and increased glomerular permeability theory to the current concept of podocytopathy. Podocyte injury is now thought to be the basic pathology in the syndrome. The book chapter aims to highlight the mechanisms underlying the pathogenesis of nephrotic syndrome as a podocytopathy.