Congenital nephrotic syndrome: report of an infant with diffuse mesangial sclerosis.

Abstract

A case of congenital nephrotic syndrome (CNS) caused by diffuse mesangial sclerosis (DMS) is presented. A female baby weighting 2,680 gm was delivered at 35 weeks' gestation. She had early onset of generalized edema, heavy proteinuria, oligouria, uncorrectable hypoalbuminemia, and rapid progression to renal failure. Even after being treated with strong antibiotics (teicoplanin and ceftazidine), the infant died of septic shock with Enterobacter cloacea, only sensitive to imipenem, at the age of 7 days. The necropsy showed diffuse mesangial sclerosis. This case demonstrates that prematurity, low birth body weight and early onset of symptoms are not pathognomonic of the congenital nephrotic syndrome of the Finnish type (CNF). It can also occur in DMS. Besides, empirical antibiotics should be started promptly and should cover the major hospital strains of bacteria if the patient is not well.