Congenital and infantle nephrotic syndrome

Abstract

Summary Congenital nephrotic syndrome can be present at birth or appears during the first 3 months of life, however, infantile nephrotic syndrome presents during the first year. Finnish-type congenital nephrotic syndrome is an autosomal recessive disease. Nephrotic syndrome present at birth, is severe and does not respond to therapy. Infectious and nutritional complications are frequent. Renal function deteriorates necessitating entry into a dialysis/transplantation program. Diffuse mesangial sclerosis is the second cause of congenital and infantile nephrotic syndrome. It may be isolated or part of Denys–Drash syndrome in association with male pseudohermaphroditism and Wilms' tumor. Nephrotic syndrome is resistant to therapy. Renal failure develops in early childhood. Other causes of congenital and infantile nephrotic syndrome include Pierson syndrome, Galloway syndrome, nephrotic syndrome with podocin mutations, congenital syphilis or toxoplasmosis and maternal alloimunisation to the podocyte protein neutral endopeptidase.