In recent years, primary immunodeficiencies have turned from the class of rare diseases to the category of more common disorders which may be encountered by doctors of any clinical discipline. The first case of primary immunodeficiency disorder (PID) in Chuvashia was detected in 1993. Since that time, the Department of Internal Diseases with the Course of Clinical Immunology at the I. Ulyanov Chuvash State University registered all the cases of PID diagnosed in the region, introducing them into the Republican Registry of PID. The study was aimed for searching epidemiological indexes, clinical and laboratory manifestations of PID in Chuvash region. The study was based on the patient data obtained by retrospective analysis of 85 case histories of PID patients, treated at different departments of the Republican Clinical Hospital, and the City Chuvash Pediatric Clinical Hospital of Public Health Ministry in 2000-2019, as well as on 49 outpatient records of the patients included into the Regional PID Registry. Various forms of PIDs were diagnosed according to the criteria developed by the European Society for Immunodeficiency and the Pan-American Group on Immunodeficiency (1999). The results of this study showed that the incidence of PID in the Chuivash Region is 3.4:100,000. The incidence of common variable immune deficiency (CVID), the most common form of PID in the region, was 1.58 per 100,000 population. The average age at the time of CVID diagnosis in Chuvash patients was 30.4±16.1 years, and the age of CVID debut was 11.3±15.0 years. The delay in proper diagnosis from the moment of clinical manifestation of CVID was, on average, 17.9 years in the region. At the time of CVID diagnosis, the patients showed marked decrease in the levels of 3 or 2 immunoglobulin classes (IgG and IgA), and T-helper cell contents (CD3+CD4+) in peripheral blood. Prevalence of selective IgA deficiency with сlinical symptoms was 0.83 per 100,000 population of the region, and the incidence of the asymptomatic form of this PID was 1 : 167. In patients with selective IgA deficiency, there were also disorders in the T cell system manifesting as decreased relative number of cytotoxic T-cells as well as elevated IgG and IgM levels. The age of diagnosis of X-linked agammaglobulinemia in the region was 3.5±3.0 years. In addition to disturbances of humoral adaptive immunity in children with this disease, a decrease in absolute T cell numbers was detected. In conclusion, the article describes disturbances of postvaccinal immunity in a pregnant patient with CVID, with asymptomatic clinical course, thus leading to false interpretation of the serological markers of TORCH infections and wrong strategy of pregnancy management.

The timely diagnosis and treatment of post-infectious glomerulonephritis (PIGN) is currently limited by the erased and low-symptom nature of the disease, which leads to the search for informative biological markers of the disease, which can be used as immunological indicators of blood and urine. The study was carried out in order to establish the characteristic changes in the immunological parameters of blood and urine in patients with PIGN. The study included 60 patients with PIGN from among the patients, hospitalized in the nephrology department of the Republican Clinical Hospital of Health Care Ministry of the Chuvash Republic in 2015-2018. In addition to the generally accepted research methods, the patients underwent: 1) the determination of indicators of innate and acquired immune response in the blood (CD3+ -, CD3+ CD4+-, CD3+CD8+-, CD4+CD25+-, CD95+-, CD20+-, CD14+CD282+-, CD14+CD284+- cells; levels of IgG, IgA, IgM, circulating immune complexes, C3, C4) and urine (levels of IgG, IgA, IgM, C3, C4); 2) the determination of the levels of cytokines - IL-1β, Ra-IL-1β, IL-2, IL-4, IL-8, IL-10, IL-17A in blood serum and urine. The data obtained were compared with those of the group of healthy individuals. The changes in blood immunological parameters, identified in the group of patients with PIGN, indicate the activation of innate immunity (the increase in the number of CD14+TLR2+- cells) and the humoral component of adaptive immunity (the increase in the number of B-lymphocytes, hyperimmunoglobulinemia - the increase in IgM and IgA levels) against the background of the decrease in the number of T (CD3+) - lymphocytes and regulatory (CD4+CD25high) - cells, hypocomplementemia (decreased levels of C3, C4). The increase in the content of C3, IgG and IgA was found in the urine. The cytokine profile of blood in patients with PIGN was characterized by the increase in the levels of pro- and anti-inflammatory cytokines IL-1β, Ra-IL-1β, IL-2, IL-8, IL-10, IL-17A, with the exception of IL-4, which remained on the levels of healthy individuals. The cytokine profile of urine in patients was characterized by the increase in the levels of pro-inflammatory cytokines IL-1β, IL-2, IL-8, IL-17A and anti-inflammatory cytokine - IL-10, with no changes in the content of Ra-IL-1β and IL-4. The revealed features of the immunological profile of blood and urine in patients with PIGN reflect the immunopathogenetic mechanisms of this disease.

Liver cirrhosis continues to be an acute problem of modern medicine due to the high rates of its prevalence and mortality. The high mortality rate is caused by the development of the number of life–threatening complications in decompensated forms of liver cirrhosis – hepatorenal syndrome, infections and varicose bleeding. Hepatorenal syndrome and infections are the result of immunological shifts occurring during decompensation of liver cirrhosis. Currently available literature data do not allow us to create a complete picture of the functional state of various links of adaptive immunity with decompensated liver cirrhosis. The aim of the research was to study the characteristic features of adaptive immunity in patients with decompensated liver cirrhosis. Material and methods. The prospective cohort study included 136 patients with decompensated liver cirrhosis, who received inpatient treatment in the hepatological department of the multidisciplinary hospital. The cohort of examined patients was divided into two groups, one of which included patients with liver cirrhosis of viral origin (n = 78), the other – patients with alcoholic liver cirrhosis (n = 58). In addition to the generally accepted standard methods, the patient examination program included immunological tests: identification of T- and B-lymphocytes, immunoregulatory and activated subpopulations of T-lymphocytes by the method of immunophenotyping peripheral blood mononuclear cells using monoclonal antibodies. The serum levels of immunoglobulins IgM, IgG, IgA, circulating immune complexes were determined by immunoturbidimetric method. Results. The study of indicators of the humoral link of adaptive immunity revealed an increase in the number of B cells, an increase in IgM, IgG, IgA and circulating immune complexes in patients with decompensated liver cirrhosis. The cellular link of adaptive immunity was characterized by an increase in the relative content of T helper cells, activated T cells against the background of a decrease in the number of immature T cells and T regulatory cells. Conclusions. The distinctive features of adaptive immunity in patients with decompensated liver cirrhosis are simultaneous activation of both humoral and cellular components, which, apparently, supports the systemic inflammatory process and the associated progressive liver fibrosis.

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