Congenital immunodeficiency is manifested by violations in the work of the immune system, which are associated with various genetic defects. The insufficiency of the cellular and humoral unit of immunity leads to the development of chronic infections and inflammatory damage to organs and tissues.
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Insufficiency of humoral immunity
The insufficiency of humoral immunity is 50-60% of all primary immunodeficiency and is manifested by violation of antibody products (serum proteins, which are formed in the human body in response to hitting alien agents - viruses, bacteria). This group includes an isolated deficiency of immunoglobulin A, an isolated deficiency of immunoglobulins of other classes, the deficit of immunoglobulins of several classes. Insufficiency of humoral immunity is possible at normal concentration of antibodies. This is due to a decrease in the level of antibodies to a specific group of antigens, such as decrease in the level of antibodies to carbohydrate antigens of bacteria.
Isolated immunoglobulin deficiency
Isolated immunoglobulin deficiency and meets most often. It occurs as a result of a defect of finite differentiation of cells secreting immunoglobulin a. In most patients, immunoglobulin deficiency acts asymptomatic. Only a small number of patients have a predisposition to the emergence of pulmonary and intestinal infections.
Insufficiency of the cellular immunity
The insufficiency of cellular immunity is 5-10% of all primary immunodeficiency and is manifested by a violation of proliferation (growth) and differentiation of T-lymphocytes or stem cell insufficiency. Primary violation of cellular immunity in most cases are accompanied by a secondary impaired synthesis of antibodies.
Insufficient formation of T-cells of immunity
T-cell insufficiency determines the increased sensitivity of the body to viruses and mushrooms, the destruction of which is based on the reaction of cellular immunity. Dijorgi syndrome and non-low-weather syndrome are caused by the underdevelopment of thymus during intrauterine development. In such patients, it is not possible to detect the reactions of cellular immunity, and although antibodies are formed in their body, a humoral response is weakened.
Insufficiency in cells of immunity
Insufficiency in lymphocytes leads to congenital Aghammaglobulinemia of Bruton, found only in men. Boys are subject to repeated infections that are caused by glotted bacteria - staphylococci and streptococci. The disease is due to a recessive genome localized on the X-chromosome, and is detected only in boys with a set of genital chromosomes xy. With this form of immunodeficiency, there are no B cells, in lymphoid tissue there are no reproduction centers, as a result, immunoglobulins cannot be detected in serum. At the same time, patients have a normally developed thymus. T-cells in quantitative and functionality do not differ from healthy children.
Reducing the content of gammaglobulin
Hypogammaglobulinemia (Reducing the number of gammaglobulins) is also characterized by purulent infections. Immunoglobulin deficiency is found in highly premature children when the level of immunoglobulins received from the mother.
Combined Insufficiency of Humoral and Cell Immunity
Combined insufficiency of humoral and cellular immunity is 20-25% of all primary immunodeficiency. This group includes diseases due to the primary impaired proliferation (growth) and differentiation of in lymphocytes and T-lymphocytes. Reducing the number of T-lymphocytes and the level of immunoglobulins in the blood is most pronounced with severe combined immunodeficiency. This is one of the most adverse forms of congenital immunodeficiency. It is characterized by the emergence of a variety of infectious (viral, fungal, bacterial) diseases immediately after birth, which leads to early death (usually in the first year of life).
Combined insufficiency of humoral and cellular immunity often accompany other congenital diseases, for example, thrombocytopenia in the syndrome of Viscott-Aldrich or congenital defects of heart and hypocalcemia (decrease in blood calcium content) in the syndrome of DJORI.
