Management of Finnish congenital nephrotic syndrome by unilateral nephrectomy

Abstract

Finnish congenital nephrotic syndrome is fatal unless managed aggressively; treatment in hospital with albumin infusions and subsequent bilateral nephrectomy allows survival. However, it consistently results in protein malnutrition which may lead to serious infections and severe physical and developmental retardation, as well as the subsequent burden of dialysing an anephric child. We treated a boy by unilateral nephrectomy at 3 months. The reduced protein loss allowed home management with albumin infusions and resulted in a substantial rise in his plasma IgG levels. The glomerular filtration rate declined fairly rapidly after unilateral nephrectomy, so he needed peritoneal dialysis by 16 months, which was straightforward as he still maintained a good urine output. Management by unilateral nephrectomy has allowed this boy to grow and develop normally. The time spent in hospital was minimal and the very high cost of providing albumin preparations was reduced considerably.