RESUMO
Nephrotic syndrome occurs infrequentiy prior to the second year of life. When nephrotic syndrome does develop during the first year, the course differs from that of older children with nephrotic sydrome, being characterized by an extremely poor prognosis and an almost complete refractoriness to therapy. Despite its low incidence congenital nephrotic syndrome is important, not only because of the severity and the disorder itself but also because the occurrence of nephrotic syndrome in this age group rasies question regarding the etiology of the disease. We experienced one case of congenital nephrotic syndrome which was confirmed by autopsy. The patient was born as a premature infant with body weight 1,400gm and 37weeks of gestational age, to a toxemic mother, gravida 3 and parity 3 in Obsteric Department of Seoul Red Cross Hospital. The patient had an uncomplicated nursery staying even though routine weekly urinalysis did show up various degree of proteinuria and microscopic hematuria without any obvious edema till the age of 50 days with body weight 2,400gm on the discharge. He was lost to be followed at Out Patient Department untill the age of 4months when he was brought to admission because of respiratory distress in generalized edematous state. He died at 7 months of age following progressive down-hill cours, despite treatment with prednisolone and cyclophosphamide for 2 months. At autopsy, almost all of the glomeruli (99%) were sclerotic with occassional creascent formation and tubules showed mircocystic dilataions. It is considered that this case was the first one which was presented on literature in Korea.