["Finnish-type" congenital nephrotic syndrome: two observed cases]

Finnish type Congenital Nephrotic Syndrome (CNS) which manifests itself in the first months of life is a serious disorder transmitted by recessive autosomal transmission. It is rare throughout the world but particularly frequent in Finland where it was described for the first time in 1959. Two observations of congenital nephritic syndrome made in the pediatric ward of the University Hospital Center of Monastir in Tunisia are reported in this article. The authors recall the clinical particularities of this disorder and also its course and treatment. The criteria for diagnosis essentially include the family history a large placenta massive proteinuria with intra-uterine onset the microcystic appearance of the kidneys during the histological examination after other types of congenital nephritic syndrome have been ruled out. Non-specific complications such as neonatal respiratory distress may cause CNS to be missed if attention is not given to the weight of the placenta or tests for proteinuria. The treatment is essentially directed to relieving the symptoms while the prevention and treatment of infections and thrombo-embolic complications is recommended. However survival is possible but at the expense of altering the renal function. In Finland the radical treatment proposed is based on peritoneal dialysis and early kidney transplant but relapses have been observed in children receiving transplants. Because of its neonatal onset its poor prognosis and the lack of any effective treatment this anomaly ideally requires a diagnosis of congenital nephrotic syndrome in at-risk pregnancies.