Congenital Nephrotic Syndrome of Finnish type in siblings

Congenital nephrotic syndrome is an uncommon disorder.The best known is the congenital nephrotic syndrome Finnish type characterised by irregular cystic dilatations of the tubules. This disease, very frequent in Finland,is familial often inherited with an autosomal recessive mode of inheritance. The nephrotic syndrome starts by three months of age and is severe, resistant to corticosteroids and immunosuppressant. Histologically micro cystic dilatations of the tubules are seen while glomeruli are only slightly modified. Infectious and nutritional complications are more common, due to the massive protein loss. If the child survives renal function deteriorates justifying the need for dialysis and renal transplantation. Prenatal diagnosis is possible by determination of maternal serum and amniotic fluid alpha fetoprotein.