More questions than answers

Abstract

The child whose idiopathic nephrotic syndrome (INS) defies treatment presents the greatest of challenges to the skills of the pediatric nephrologist. The majority of such patients, on renal biopsy, are found to have the lesion of focal segmental glomerulosclerosis (FSGS), where segments of one or more glomeruli are sclerotic. The glomeruli in the juxtamedullary region are the most likely to be affected by this lesion. With time, more and more glomeruli are affected, glomeruli become totally sclerotic, and renal failure may develop and progress to end-stage renal disease. Some of the patients will have recurrence of the nephrotic syndrome after transplantation and eventually show this typical sclerotic lesion on biopsy of the transplanted kidney, though early in the evolution of the recurrence, the biopsy is more typical of minimal change nephrotic syndrome (MCNS).