The congenital nephrotic syndrome (CNS) is a rare form of neo-natal nephropathy that generally occurs within the first three months of life, and is characterized by proteinuria, placentamegaly, and retarded intrauterine growth. Proteinuria in infants can result from a variety of other causes such as congenital infections (syphilis, CMV, etc.), mercury intoxication, and minimal change lesion, but CNS is the only nephrotic syndrome of neonates that is unresponsive to immunosuppressive drugs.This syndrome was first defined and extensively studied in Finland where this condition was estimated to occur in 1 per 10,000 births, and was found to be an expression of an autosomal recessive inheritence. It has also occured in children without a history of Finnish ancestry.The prognosis for these children is poor, most often resulting in death by the fourth year of life unless a renal transplant is received. A study of 17 transplants in children with CNS reported a 71% graft survival rate over a two year period with no recurrence of nephrotic syndrome in any transplants.
Microcephaly and congenital nephrotic syndrome owing to diffuse mesangial sclerosis: an autosomal recessive syndrome.
