Nephrotic syndrome in children : functional, morphological and therapeutical aspects

Although cure rates of the idiopathic nephrotic syndrome in children have improved because of advances in immunosuppressive therapy, the pathogenesis and course of the disease remain controversial. We have followed children with nephrotic syndrome to study renal function and morphological changes in kidney biopsies and in an experimental study. Immunosuppressive treatment with cyclosporine in severe nephrotic syndrome has been shown to be effective, but nephrotoxicity is a major concern. Some studies have suggested that glomerular hypertrophy precedes the development into FSGS, which comprises focal and segmental scarring of the glomerulus, a frequent cause of renal failure. In this syndrome the podocyte and its foot processes show foot process effacement, usually reversible.However, if the disease becomes worse, it leads to detachment of the foot processes from the glomerular basement membrane and initiates renal scarring of the glomerulus. DMP and FSGS are regarded as more severe types of the nephrotic syndrome, clinically and histopathologically, than MCNS. In 58 children with MCNS (40), DMP (4) and FSGS (14) we investigated retrospectively the glomerular volume. GFR and ERPF were used to determine renal function. The patients were BSA or age matched. The glomerular volumes in DMP and FSGS were significantly larger than in those with MCNS(P<0.005, P