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1. Classification of primary immunodeficiency states in children
Immunity - a way to ensure and maintain antigenic homeostasis.
Immunodeficiencies Decrease in the functional activity of the main components of the immune system, leading to a violation of the antigenic homeostasis of the body and, above all, to a decrease in the body's ability to defend itself against microbes, manifested in increased infectious morbidity.
Classification of immunodeficiency states:
1) primary immunodeficiencies;
2) secondary immunodeficiencies.
Primary immunodeficiencies are genetically determined monogenic diseases transmitted by an autosomal recessive or X-linked trait. There is also an autosomal dominant mode of inheritance.
Primary immunodeficiencies are congenital disorders of the immune system with defects in one or more of its components (cellular or humoral immunity, phagocytosis, complement system).
Classification of primary immunodeficiency states:
1) pathology of the humoral link of immunity, i.e., insufficiency in the production of antibodies;
2) pathology of the cellular link of immunity mediated by T-lymphocytes;
3) combined forms (SCID) of humoral and lymphocytic insufficiency.
clinical picture immunodeficiency states has common features.
1. Recurrent and chronic infections of the respiratory tract, paranasal sinuses, skin, mucous membranes, gastrointestinal tract, caused by opportunistic infections, protozoa, fungi, tending to generalize, septicemia and torpid to the usual reaction.
2. Hematological deficiencies: leukocytopenia, thrombocytopenia, anemia.
3. Autoimmune disorders: arthritis, scleroderma, chronic active hepatitis, thyroiditis.
4. Sometimes IDS are combined with allergic reactions in the form of eczema, Quincke's edema.
5. Tumors and lymphoproliferative diseases are more common in IDS.
6. Often IDS are combined with malformations.
7. Patients with IDS have digestive disorders, diarrheal syndrome, malabsorption syndrome.
8. Patients with IDS have unusual reactions to vaccination.
9. Reticular dysgenesis (defect of maturation of lymphoid and myeloid cells).
Severe combined immune deficiency manifests itself in the first months of life: symptoms of a severe multiorgan infectious process with malnutrition. From the respiratory system - bronchopulmonary damage. From the gastrointestinal tract - diarrhea.
Author: Pavlova N.V.
>> Forward: Diagnosis of primary immunodeficiencies. Principles of therapy
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