Congenital Nephrotic Syndrome with Focal Segmental Glomerular Sclerosis, Renal Insufficiency and Tubular Dysfunction

A patient with congenital nephrotic syndrome with focal segmental glomerular sclerosis is reported. There was moderate renal insufficiency, proximal renal tubular acidosis, hematuria and glycosuria. The glomerular basement membrane showed irregular thickening, splitting of the lamina densa and reticulation of the lamina rara interna. In addition to the features of the nephrotic syndrome and renal insufficiency, tubular dysfunction is one of the early manifestations of this disease.