Congenital nephrotic syndrome

The nephrotic syndrome manifested in the child from the moment of its birth is called congenital. When diagnosing it is necessary to exclude nephrotic syndrome, characterized by intrauterine infection, such as syphilis, cytomegaly, toxoplasmosis, etc. The cause of congenital nephrotic syndrome is also a thrombosis of renal veins. Among all the most forms of congenital nephrotic syndrome, autosomal recessive pathology characterized by microcystosis of the kidneys and more common in persons of Finnish nationality( the so-called congenital nephrotic syndrome of the Finnish type) is of particular importance. The birth of children with a similar pathology in families of a different nationality is extremely rare. The course of pregnancy is severe, the birth is usually premature, the mass of the placenta is 1 / 2-1 / 4 of the mass of the newborn. Symptoms of nephrotic syndrome are usually detected at birth.

Prevention of the birth of children with a Finnish type of congenital nephrotic syndrome became possible on the basis of the determination in the amniotic fluid and serum of pregnant women of high α-fetoprotein content. In this case, abortion was shown. Currently antenatal diagnosis of the congenital Finnish type of nephrotic syndrome is carried out by all pregnant women in the northwestern regions of Finland, where the drift of the mutant gene is established. In other areas of the country antenatal diagnosis is carried out only on direct indications, therefore in the southern and eastern regions of the country and currently are born patients with congenital nephrotic syndrome of the Finnish type. All of them come for treatment in a specialized clinic.

Methods of treating congenital nephrotic syndrome have been developed. The essence of this technique is reduced to the use of a high-calorie and high-protein diet, active treatment of bacterial infections, which contributes to the development of the child during the first year of life. In the future, the essence of therapy is reduced to surgical methods of treatment, in particular renal transplantation.