Congenital Nephrotic Syndrome

Several types of renal disease with the nephrotic syndrome are found in the newborn and in infants. Of these, the congenital nephrotic syndrome of Finnish type comprises a distinct entity. It is characterized by large placenta, early manifestation, severe impairment of somatic development, resistance to treatment and inevitably fatal outcome. The most common histological finding is dilated cortical tubules, but this landmark is not present in all cases. This disease is transmitted by an autosomal recessive gene. Another entity with early onset and death in renal insufficiency in early childhood has recently been described under the designation diffuse mesangial sclerosis. The main features of the severe clinical course and very distinctive renal histology are also given here. Other types of nephrotic syndromes in infancy, also often familial in occurrence, need further studies for classification.