Immungenetic characteristics of Alport syndrome and nephrotic syndrome comorbidity in children

In order to study the immunogenetic features of the comorbid course of Alport's syndrome and nephrotic syndrome in children, 65 children aged 3-18 years were observed in our clinic. From this: 35-Nephrotic syndrome (group I); 16-Alport syndrome+Nephrotic syndrome (group II); 14- Alport's syndrome (III-group). As a result of the study, it was found that in Alport's syndrome in children, due to the addition of secondary nephrotic syndrome (hypoproteinemia, hypoalbuminemia, hypercholesterolemia, hypercoagulation, hypoimmunoglobulinemia), disease progression and cases of protein-dependent secondary immune deficiency (kidney and lung ABL, IL-2 increase, C3, C4 components decrease, significant increase in frequency of HLA-DR2 antigen). In Alport's syndrome, protein-dependent secondary immunodeficiency is pathogenetically important in the progression of kidney tissue damage and focal segmental glomerulosclerosis, and ultimately accelerates the development of chronic kidney failure. In the COL4A3 autosomal recessive type of the disease, clinical, laboratory, and immunological changes, decrease in glomerular filtration rate, development of chronic kidney failure are higher than in other mutation groups and are the main risk factor for transition to chronic kidney disease. A significant increase in the frequency of the HLA-DR2 antigen in Alport syndrome is considered a high risk factor for the progression of focal-segmental glomerulosclerosis during the course of the disease.