Primary immunodeficiency is a disease that can occur due to the absence or improper functioning of one or more elements of the immune system due to a genetic malfunction. During the formation of the body, disorders in the components of the immune system may occur, which is not always amenable to further compensation.
Since there can be many violations in the failure of the immune system, there are also many types of primary immunodeficiency. The presence of this deviation provokes a person’s susceptibility to certain types of infection, which are characterized by a variety of symptoms.
A few decades ago, primary immunodeficiency was considered a rare phenomenon, and only a few of its varieties were known. At the moment, this disease has passed into the category of the most common.
Manifestations of severe immune disorders are diagnosed almost immediately after the birth of a child, if the disorders are poorly expressed, then they may not manifest themselves until 30-40 years. In addition, there are a number of immunodeficiency conditions that do not have symptoms.
CAUSES OF PRIMARY IMMUNODEFICIENCY
The main cause of primary immunodeficiency is a genetic malfunction in the immune system, which prevents the child from developing a protective mechanism before a certain infection, that is, the body has no protection against it.
TYPES OF IMMUNODEFICIENCY
There are five groups of immunodeficiency, depending on the impaired link of the immune system:
Humoral immunodeficiency associated with a violation of the formation of antibodies.
Cellular immunodeficiency associated with impaired differentiation and division of lymphocytes.
Combined, humoral and cellular immunodeficiency.
Immunodeficiency caused by phagocyte insufficiency.
Complementary immunodeficiency.
SYMPTOMS OF PRIMARY IMMUNODEFICIENCY
The most significant problem with this disease is the body’s susceptibility to infections. People suffering from primary immunity are prone to frequent infections that are not susceptible to treatment, take a long time, with severe symptoms.
Even those children who have normal immunity often suffer from colds. As for children with a deviation in the work of the immune system, they are susceptible to diseases several times more often, in addition, they may have several infections at the same time. Since the child’s body is significantly weakened by diseases, there is a poor weight gain, lagging growth and general development.
Antibacterial therapy for primary immunodeficiency does not bring the expected effect and infections can occur repeatedly, passing into the chronic stage.
The main symptoms of primary immunodeficiency are:
Chronic infections with complications.
Chronic skin infections.
Chronic respiratory tract infections.
Eye infections.
DIAGNOSIS OF PRIMARY IMMUNODEFICIENCY
The diagnosis of primary immunodeficiency is made after a study of humoral and cellular immunity. If the corresponding clinical picture is confirmed, then a blood test is prescribed to the patient. Children are required to study the level of chlorine in sweat and pancreatic functions, allergic skin tests and immunological blood analysis.
TREATMENT OF PRIMARY IMMUNODEFICIENCY
An integrated approach is used to effectively treat this disease:
Timely treatment of infectious diseases.
Surgical treatment, with the replacement of a defective link in the immune system (immunoglobulins, bone marrow transplantation).
Enzymatic therapy.
Vitamin therapy.
Cytokines.
In case of primary immunodeficiency, vaccination with live vaccines is strictly prohibited.
