Congenital nephrotic syndrome

August 12, 2017 17:51 | Genetic Diseases

Nephrotic syndrome, which manifests itself in the child from the moment of his birth is called congenital.In the diagnosis should always be ruled out nephrotic syndrome characterized by intrauterine infection, such as syphilis, salivary gland disease, toxoplasmosis and others. The reason for the formation of congenital nephrotic syndrome and renal vein thrombosis is.Among most forms of congenital nephrotic syndrome it is of particular importance autosomal recessive pathology is characterized by kidney mikrokistozom and occurs more often in people of Finnish nationality (the so-called congenital nephrotic syndrome of the Finnish type ).The birth of children with similar pathology in families of other nationalities is extremely rare.The course of pregnancy difficult, premature birth normally, placenta weight is 1 / 2-1 / 4 of the newborn weight.The symptoms of nephrotic syndrome is usually identified at birth.

Warning birth of children with Finnish type congenital nephrotic syndrome was made possible on

the basis of determination in amniotic fluid and in the serum of pregnant high content of alpha-fetoprotein.In this case, it has been shown to abortion.At present, antenatal diagnosis of congenital nephrotic syndrome of the Finnish type is carried out for all pregnant women in the north-western regions of Finland, where the set drift mutant gene.In other parts of the country antenatal diagnosis is carried out only on direct evidence, so in southern and eastern regions of the country and are now born patients with congenital nephrotic syndrome of the Finnish type.All of them come for treatment in a specialized clinic.

Methods of treatment of congenital nephrotic syndrome.The essence of this method reduces to the use of high-calorie and high-protein diets, active treatment of bacterial infections, which contributes to the development of the baby during the first year of life.In the future, the essence of treatment is reduced to the operational methods of treatment, such as renal transplantation.