Immunodeficiency we call the condition, which is characterized by a decrease in the function of the immune system and the body's resistance to various infections. From the point of view of etiology (reasons for the development of the disease), primary and secondary immunodeficiencies are distinguished. It should be emphasized that according to clinical signs and laboratory data, secondary and primary immunodeficiencies are very similar, up to the existence of a relationship between the nature of immune disorders and the type of pathogen. The fundamental difference remains the cause that underlies immune disorders: in the primary it is a congenital defect, in the secondary it is acquired.

Acquired (secondary) immunodeficiencies are much more common than primary ones. Usually, secondary immunodeficiencies develop against the background of exposure to the body of adverse environmental factors or various infections. As in the case of primary immunodeficiencies, in secondary immunodeficiencies, either individual components of the immune system, or the entire system as a whole, can be disturbed. Most secondary immunodeficiencies (with the exception of immunodeficiency caused by infection with the HIV virus) are reversible and respond well to treatment. Below we consider in more detail the significance of various adverse factors in the development of secondary immunodeficiencies, as well as the principles of their diagnosis and treatment.

Causes of the disease

І. infectious

1. Viral infections:

a) acute - measles, rubella, influenza, viral parotitis (mumps), chickenpox, hepatitis, herpes, etc.;

b) persistent - chronic hepatitis B, subacute sclerosing panencephalitis, AIDS, etc.;

c) congenital - cytomegaly, rubella (TORCH-complex).

2. Bacterial infections: staphylococcal, pneumococcal, meningococcal, tuberculosis, etc.

3. Protozoal invasion and helminthiases (malaria, toxoplasmosis, leishmaniasis, trichinosis, ascariasis, etc.).

ІІ. Alimentary (violationnutrition):

1. protein-energy deficiency;

2. deficiency of trace elements (Zn, Cu, Fe), vitamins - retinol (A), ascorbic acid (C), alpha-tocopherol (E), folic acid;

3. exhaustion, cachexia, loss of protein through the intestines, kidneys;

4. congenital metabolic disorders;

5. overnutrition, obesity;

6. syndrome of violation of intestinal absorption.

III. Metabolic:

1. chronic renal failure, uremia, nephrotic syndrome;

2. chronic liver disease

3. diabetes

4. hypercatabolism of immunoglobulins.

IV. Conditions that lead to the loss of immunocompetent cells and immunoglobulins (bleeding, lymphorrhea, burns, nephritis).

V. Malignant neoplasms especially lymphoproliferative ones.

VI. Autoimmune diseases.

VII. Exogenous and endogenous intoxications(poisoning, thyrotoxicosis, decompensated diabetes mellitus).

VIII. Immunodeficiency after various influences:

1. physical (ionizing radiation, microwave, etc.);

2. chemical (immunosuppressants, cytostatics, corticosteroids, drugs, herbicides, pesticides, etc.);

3. adverse environmental factors;

4. immunosuppressive methods of treatment: drugs (immunosuppressants, glucocorticosteroids, cytostatics, antibiotics, non-steroidal anti-inflammatory drugs).

5. Occupational hazards, including X-ray exposure, radioactive exposure, biologically active and chemically aggressive substances.

6. Various types of stress (emotional stress, mental trauma, physical, sports overload, etc.).

IX. Anyheavydiseases, surgical interventions, anesthesia, burns.

X. Violation of neurohormonal regulation.

XI. Agefactors: early childhood, old age, pregnancy.

Both primary and secondary immunodeficiencies can be caused by impaired function of one of the main immune systems: humoral (B-system), cellular (T-system), phagocyte system, complement system, or several (combined defects).

Mechanisms of occurrence and development of the disease (pathogenesis)

Acquired (secondary) immunodeficiency is a disorder of the immune system that develops in the post-neonatal period or in adults and is not the result of genetic defects.

Thus, under the term " secondary (acquired) immunodeficiency" it is necessary to understand immunity disorders that occur as a result of somatic and other diseases, as well as other factors and have clinical manifestations ( International Classification of Diseases, X revision).

Acquired (secondary) immunodeficiency- this is a clinical and immunological syndrome: a) developed against the background of a previously normally functioning immune system; b) characterized by a persistent significant decrease in the quantitative and functional indicators of specific and / or non-specific factors of immunoresistance; c) is a risk zone for the development of chronic infectious diseases, autoimmune pathology, allergic diseases and tumor neoplasms.

From this definition of the concept of acquired (secondary) immunodeficiency, its following features follow.

1. Firstly, violations in the immune system are indeed secondary and appear against the background of previously normal health, both clinically and in immunolaboratory terms. This can be found out in a conversation with the patient.

2. Violations in the immune system must be persistent and pronounced. This is an important condition, since it is known that the indicators of the immune system are labile, mobile, which allows its various links to complement and "insure" each other. Therefore, transient, temporary changes in the parameters of immunity may be due to the peculiarities of the situational response.

3. Violations in the immune system should be not only quantitative. The function of certain cells should also be evaluated. There are cases when a decrease in the number, for example, NK cells, was compensated by their increased functional activity. If a decrease in the number of certain cells of the immune system is accompanied by a simultaneous violation of their function, this is certainly the most important laboratory sign of immunodeficiency.

4. Violation in the immune system can affect the indicators of both specific (adaptive) immunity and nonspecific resistance, that is, innate (natural) immunity.

5. Violations in the immune system are characterized by an overwhelming lesion of one of the chains of immunity (cellular, humoral, complementary or phagocytic), other changes in immunological parameters are secondary, as a rule, compensatory in nature. Combined immune disorders are possible.

6. It is important to understand the following. As a rule, a patient who already has clinical signs of secondary immunodeficiency, for example, a chronic infectious inflammatory pathology that is resistant to traditional therapy, gets to see a doctor. In this case, the active intervention of a clinical immunologist is necessary. However, it is important to alert the doctor to the fact that in some so-called practically healthy individuals, immunolaboratory signs of secondary immunodeficiency may be detected, which are accompanied by only indirect clinical signs, such as increased fatigue, which has not yet become chronic. In this case, it is better to talk about transient changes in the immunogram, not supported by the clinic and, in many cases, not requiring the appointment of immunotropic drugs. To clarify the situation, such patients need repeated observation. In this case, it should be remembered that this person is at risk of developing one or another pathology associated with secondary immunodeficiency: infectious, autoimmune, allergic, oncological, etc. At the same time, belonging to the "risk zone" is, fortunately, a reversible situation, and such a person can be helped through immunorehabilitation measures.

Clinical picture of the disease (symptoms and syndromes)

There are three forms of secondary immunodeficiencies:

1. acquired;

2. induced;

3. spontaneous

Acquired a form of secondary immunodeficiency is the acquired immunodeficiency syndrome (AIDS), which develops as a result of damage to the immune system by the human immunodeficiency virus (HIV).

induced form secondary immunodeficiency occurs as a result of specific causes that caused its appearance: X-ray radiation, cytostatic therapy, the use of corticosteroids, trauma and surgical interventions, as well as immunity disorders that develop secondary to the underlying disease (diabetes, liver disease, kidney disease, malignant neoplasms).

spontaneous form secondary immunodeficiency is characterized by the absence of an obvious cause that caused a violation of immune reactivity. Clinically, it manifests itself in the form of chronic, often recurrent infectious and inflammatory processes of the bronchopulmonary apparatus, paranasal sinuses, urogenital and gastrointestinal tract, eyes, skin, soft tissues caused by opportunistic (opportunistic) microorganisms. Therefore, chronic, often recurrent, sluggish, difficult-to-treat inflammatory processes of any localization in adults with traditional means are considered as clinical manifestations of a secondary immunodeficiency state. In quantitative terms, the spontaneous form is the dominant form of secondary immunodeficiency.

Types to them immunodeficiency O V (V depending on the etiological factor):

- refined (infectious, toxic, metabolic, physical, psychogenic, post-traumatic, indicating a specific diagnosis - the disease that caused it);

- unspecified (cryptogenic or essential, or idiopathic, or spontaneous - set in the absence of any etiological factor)

Types specified by him moondef And cit O V

Infectious immunodeficiency is formed as a result of the action of an infectious pathogen, incl. conditionally pathogenic (viral, bacterial, protozoal, fungal, helminthic).

Toxic immunodeficiency develops under conditions of prolonged exposure to exo- and endotoxins, xenobiotics, etc. (exogenous, medical, professional, endogenous, burn, etc.).

Metabolic immunodeficiency develops under conditions of prolonged metabolic disorders, incl. violations of acid-base balance (food, metabolic, protein deficiency, malabsorption, etc.).

Physical immunodeficiency develops as a result of prolonged action of ionizing and ultraviolet radiation on the human body, the action of high frequencies and fields, and the like.

Psychogenic immunodeficiency develops under conditions of prolonged psycho-emotional overload, stress, diseases of the central nervous system, and the like.

Post-traumatic immunodeficiency (including surgical) develops under conditions of severe extensive injuries, burns, large-scale and prolonged surgical interventions, blood loss, lymphorrhea, etc.

Types of Immune System Defects :

Lymphocytic immunodeficiency is characterized by persistent quantitative and / or functional changes in the T-cell link of the immune system.

Humoral immunodeficiency is characterized by persistent quantitative and / or functional changes in the B-cell link of the immune system, including the production of immunoglobulins.

Phagocytic immunodeficiency is characterized by persistent quantitative and / or functional changes in phagocytic cells (monocytes / macrophages, granulocytes) of the immune system.

Complementary immunodeficiency is characterized by persistent changes in the level and activity of complement components.

Combined immunodeficiency is characterized by persistent quantitative and / or functional changes in the parameters of several (two or more) parts of the immune system. It is advisable to single out the leading defect of the immune system (for example, a combined defect with lymphocytic overload).

Classification of secondary immunodeficiency in clinical form :

The autoimmune form is characterized by relevant clinical and laboratory data (hypergamaglobulinemia, elevated CEC levels, etc.).

The allergic form (including IgE-dependent, reaginic) is characterized by relevant clinical (hypersensitivity of the skin and mucous membranes, primarily the respiratory system and gastrointestinal tract) and laboratory data (eosinophilia, elevated IGE levels, etc.).

The immunoproliferative form is characterized by the formation of tumors in different organs and systems with a heaping of the tumor mass of the lymphoid-monocyte-cell composition, an increase in the size of the spleen, tonsils, adenoids, thymus, Peyer's patches, and the like.

The paraneoplastic form is characterized by impaired functioning of the immune system in cancer patients as a result of the action of the tumor on the body and damage to the immune system after the use of antiblastoma drugs (cytostatic therapy, radiation, etc.).

Neurogenic form ( chronic fatigue syndrome, neuroimmune endocrine syndrome, immunodeficiency in mental illness, etc.).

mixed form - characterized by the presence of two or more forms in the patient; it is advisable to single out the leading form (for example, a mixed form with a predominance of autoimmune).

Flow options for them immunodeficiencies :

• Acute - clinical and laboratory signs of immunodeficiency develop and persist for 1 month.
• Subacute - clinical and laboratory signs of immunodeficiency develop and persist for 3 months.
• Chronic - clinical and laboratory signs of immunodeficiency develop and persist for 6 months.
• Recurrent - clinical and laboratory signs immunodeficiency re-form earlier than 6 months after successful treatment.

Degrees of immune deficiency (depending on the absolute number of lymphocytes, the norm of the absolute number of lymphocytes is 1.4-3.2 g / l):

1 degree of immune deficiency - minimal (IN-1) - the absolute number of lymphocytes is 1.4-1.2 g/l; laboratory parameters are reduced by 15-30% of the average normal value. Clinically, immunodeficiency may not manifest itself (compensated form).

2 degree of immune deficiency - medium (IN-2) - the absolute number of lymphocytes is 1.1-0.9 g/l; laboratory parameters are reduced by 35-55% of the average normal value. Clinically, immunodeficiency can be manifested by one or a combination of several clinical syndromes, subacute or chronic variant of development.

3 degree of immune deficiency - high (IN-3) - the absolute number of lymphocytes is less than 0.9 g/l; laboratory values ​​are reduced by more than 55% of the average normal value. Clinically, immunodeficiency is manifested by severe clinical symptoms.

Classification of secondary and m m unodef And cit O V by functional insufficiency :

FN I - the patient keeps working capacity, needs outpatient treatment without issuing a certificate of incapacity for work;

FN II - the patient temporarily loses his ability to work or his working capacity is limited, needs outpatient treatment with the issuance of a certificate of incapacity for work;

FN III - the patient temporarily loses his ability to work or has a permanent disability, needs inpatient treatment and / or an examination of his working capacity.

The main condition for the occurrence of an infectious process is the susceptibility of the macroorganism, i.e. insufficiency of its immunity (immunodeficiency), when even an opportunistic microorganism can cause an infection. Immunodeficiency - relative or absolute is the main cause of infections, since with an increase, stimulation of immunity after vaccination, resistance to many highly virulent pathogens occurs. Thus, by vaccinating the population, smallpox, which claimed millions of people, was eliminated, immunity to measles, poliomyelitis, influenza, hepatitis B, tick-borne encephalitis, yellow fever and other infections is induced. This proves that even highly virulent pathogens cannot overcome the previously mobilized immune barriers of the body. Consequently, the virulence of infectious agents is not absolute, and an organism with a sufficiently high degree of specific and nonspecific immunity activity - i.e. immune - able to resist it.

Predominantly T-cellimmunodeficits

1. T-lymphocytopenic syndrome.

The paracortical zones of the lymph nodes become empty, the lymphoid tissue atrophies. The number of T-lymphocytes is reduced by 15% or more. The diagnosis is established upon re-confirmation against the background of remission of the underlying disease.

Options: autoimmune (with the presence of anti-T-cell antibodies), stress, toxic (drug), viral, dysmetabolic, with sarcoidosis, lymphogranulomatosis, T-leukemia, etc.

Clinical picture: recurrent viral infections with a long course in combination with bacterial infections.

2. Syndrome of T-cell immunoregulatory imbalance.

Immune status: Th-CD4/Ts-CD8 ratio less than 1.4 (the lower, the more pronounced VID). The diagnosis is established by identifying and confirming these disorders during the period of remission of the underlying disease.

Clinical picture:

3. Syndrome of T-cell immunoregulatory imbalance with increased cytotoxic reactivity.

Immune status: Relative lymphocytosis. The immunoregulatory index Th-CD4/Ts-CD8 is less than 1.4 (the lower, the more pronounced VID). The levels of NK-cells (CD16), IgM, IgG are sharply increased, the HCT test is increased. There are signs of the development of a cytotoxic reaction against the background of an intracellular infection, for example, herpes virus, cytomegalovirus infection.

Clinical picture: polymorphic recurrent infections of various localization.

4. Syndrome of deficiency of lymphokines and their receptors.

Set with repeated confirmation.

Predominantly B-cell deficiencies

1. Panhypogammaglobulinemia.

Hypoplasia of lymphoid follicles, atrophic lymph nodes.

Immune status: a decrease in the concentration of gamma globulins in the blood serum, a decrease in the level of natural antibodies, a decrease in the blood and other biological fluids (saliva, secrets) of IgA, M, G with a normal or moderately reduced level and functional activity of T-lymphocytes.

Clinical picture: recurrent bacterial infections of the respiratory tract, lungs, sepsis predominate.

2. Dysimmunoglobulinemia.

Immune status: Change in the ratio between immunoglobulins with a mandatory decrease in the concentration of one of them against the background of normal and elevated levels of others.

3. Antibody deficiency syndrome.

Immune status: Absence of antibodies against identified infectious agents (eg, staphylococcus, streptococcus).

Clinical picture: Recurrent infections.

4. secretory deficiency IgA .

Immune status: There is no (reduced) level of secretory IgA in saliva, tracheobronchial, intestinal and other secrets.

Clinical picture: chronic bronchitis, inflammation of the oral mucosa (periodontal disease), chronic tonsillitis, otitis media, etc.

5. Secondary immunodeficiency in B-cell tumors (Waldenström's plasmacytoma, lymphomas, B-cell leukemias).

6. Secondary ny immunodeficiency with symptoms of dysimmunoglobulinemia and an autoimmune component.

Immune status: Neutrophilic leukocytosis, an increase in the level of plasmacytes, B-lymphocytes, an increase in Th2 (CD4+), CD8+, IgM levels, CEC, complement, ESR, CRP and an increase (less often a decrease) in the activity of phagocytes are characteristic.

Macrophage and granulocyte deficiencies

1. Syndrome of hyperactivation of macrophages-monocytes.

Immune status: monocytosis, increased IL-1 in biological fluids.

Clinical picture: febrile syndrome, arthritis and inflammation of various localization.

2. Pangranulocytopenia, granulocyte deficiency.

Immune status: Agranulocytosis and neutrophilopenia.

Options - autoimmune, allergic, toxic, infectious.

Clinical picture: purulent-septic diseases, ulceration of the mucous membranes.

3. hypereosinophilia syndrome.

Immune status: the number of eosinophils in the blood, secrets, tissues.

4. Deficiency of neutrophil receptors and adhesion molecules.

Immune status: absence or decrease in the number of neutrophils with the corresponding receptors and molecules. Decrease in their adhesion to the cell surface.

Clinical picture:

5. Deficiency of chemotactic activity of neutrophils.

Immune status: decrease in spontaneous and induced motility of neutrophils.

Clinical picture: purulent-septic diseases.

6. Deficiency of metabolic activity of neutrophils.

Immune status: decrease in stimulated HBT test, myeloperoxidase activity, and other enzymes.

Clinical picture: purulent-septic diseases.

7. Deficiency of the absorption function of neutrophils.

Immune status: decrease in phagocytic number and phagocytic index.

Clinical picture: purulent-septic diseases.

8. Deficiency of the digestive activity of neutrophils.

Immune status: Absent or reduced digestion of microorganisms.

Clinical picture: recurrent inflammatory processes, more often - of the skin.

9. Panleukopenic syndrome.

Options: toxic, autoimmune, infectious, radiation. Decrease in the number of all leukocytes, devastation of the bone marrow, inhibition of colony formation.

Clinical picture: severe infections, sepsis.

10. General lymphocytopenic syndrome.

Options: autoimmune with antilymphocyte antibodies; lymphocytolytic, as a result of the destruction of lymphocytes by exogenous factors; viral lymphocytopenia.

Immune status: lymphocytopenia (the number of lymphocytes is 15% or more below normal, the syndrome of "lymphocyte deficiency").

Clinical picture: persistently recurrent localized or generalized bacterial and viral infections, sometimes splenomegaly.

11. Syndrome of polyclonal activation of lymphocytes.

Immune status: antibodies of various specificities to autoantigens are present in the blood, their titer to other antigens increases, hyperplasia of the follicles of the lymph nodes, an increase in the level of IgG immunoglobulins, an increase in CD4 T-helpers and a decrease in the level of CD8 T-suppressors, B-lymphocytes, at close to normal the level of total T-lymphocytes.

Clinical picture: infectious, autoimmune and allergic processes.

12. Lymphadenopathy (localized or generalized).

Options: with a normal level of lymphocytes in the blood; with T-lymphopenia.

Clinical picture: hyperplasia of the lymph nodes, prolonged subfebrile condition, autonomic dysfunction (dystonia, cardialgia, etc.).

13. Hypertrophy Syndrome , hyperplasia of the tonsils and adenoids. Chronic tonsillitis, adenoids; quantitative and functional dysfunction of lymphocytes and cytokines.

14. Posttonsillectomy syndrome.

Clinical picture: recurrent diseases of the upper respiratory tract after removal of the tonsils due to chronic tonsillitis. Recurrent infections of the nasopharynx and in the region of the arches of the tonsils.

Immune status: possibly a moderate decrease in T-lymphocytes, an imbalance in their subpopulations, dysimmunoglobulinemia.

15. postsplenectomy syndrome. Inhibition of the production of antibodies to thymus-independent antigens (streptococci, staphylococci, etc.), T-cell lymphopenia is possible, increased sensitivity to infections.

16. Thymic-lymphatic syndrome characterized by a combination of thymomegaly, adrenal insufficiency and functional activity of lymphocytes.

Clinical picture: adynamia, pale marbled skin, shortness of breath at rest, microlymphadenopathy, hypersympathicotonia.

17. Immune complex pathology syndrome.

Immune status: high levels of immune complexes in the blood, their deposition in tissues, decreased activity of phagocytes, inhibition of the activity of Fc-receptors of lymphocytes;

Clinical picture: vasculitis in immune, allergic, infectious diseases; hepatosplenomegaly, telangiectasia.

Metabolic acquired immunodeficiencies

1. Micronutrient deficiencies.

Zinc deficiency - atrophy of lymphoid tissue, inhibition of the function of T-helpers and neutrophils, enteropathic acrodermatitis.

Copper deficiency - neutropenia, dysfunction of phagocytes and T-lymphocytes.

2. Immunodeficiency in hypovitaminosis.

Vitamin C deficiency - impaired function of phagocytes, inhibition of antibody synthesis.

3. Immunodeficiencies in protein deficiency (alimentary, etc.) and dyslipoproteinemias, disorders of carbohydrate metabolism.

Defects in the complement system

1. Syndrome of hypocomplementemia.

Immune status: Decreased hemolytic activity of complement, increased number of immune complexes in the blood.

Clinical picture: autoimmune, allergic and infectious diseases.

Platelet deficiency.

thrombocytopenic syndrome With antiplatelet antibodies (immune thrombocytopenia).

Options: autoimmune, allergic, toxic, infectious.

Immune status: impaired adhesion and function of platelets.

Clinical picture: thrombocytopenic purpura.

Diagnosis of the disease

First step diagnosis is collection of anamnesis and clarification of complaints patient, which, depending on the type of immunopathology, can vary significantly.

A history of ID usually reveals recurrent infections, the nature and location of which may indicate the type of immunodeficiency. The allergic process has its own characteristics, and only on the basis of anamnesis can sometimes a correct diagnosis be established.

A history of autoimmune diseases has characteristic features, which makes it possible to distinguish them from other types of pathology. Lymphoproliferative and oncological processes also have their own characteristics. The next step is to conduct immunological studies to assess the immune status of a patient with suspected immunodeficiency.

"Immune status" is the state of the immune system of a healthy or sick person at a certain point in time under specific environmental conditions. Assessment of the immune status- this is the process of obtaining a complex of non-specific and specific quantitative and functional indicators that reflect the state of the immune system.

Immunological or immune status (IS) characterized by a set of informative indicators reflecting the state of various parts of the immune system at the time of the study in a particular process or disease. Reflecting the form and variant of the disease, IS indicators serve as the basis for creating immunological "image" of the disease, those. its immunological characteristics, to identify a defective link in it.

Laboratory assessment of the state of the immune system in many respects concretizes the idea of ​​the type and degree of its violations and, in combination with the identification of the pathogen using immunological methods, if any, allows you to choose an immunotherapeutic agent.

Immunodiagnostics- this is the use of a combination of immunological methods to detect a disease or determine the causative agent of a disease in the test material. All methods of immunodiagnostics are divided into 2 groups:

General non-specific methods, characterizing the state of various parts of the immune system: lymphocytes, granulocytes, macrophages, complement. Usually they are used to detect a defect in the immune system, i.e. with immunodeficiencies.

specific methods, allowing to detect antibodies, immune T-lymphocytes, pathogen antigens in the human body or in the external environment. These methods are used to diagnose infections, allergies, autoimmune diseases.

All these methods are used to assess the immune status of a person, i.e. to characterize the state of the immune system.

The diagnostic standard for secondary immunodeficiency states is represented by the following studies:

1. Mandatory laboratory examination:

According to the standards for diagnosis and treatment of the underlying disease;

Study of the immune status (determination of the total number of leukocytes, lymphocytes, subpopulations of T-lymphocytes, B-lymphocytes, the level of immunoglobulins A, M, G, phagocytosis);

Monitoring of identified disorders after a course of therapy.

2. Additional research methods:

Determined by the underlying disease and concomitant pathology;

Special immunological studies depending on clinical manifestations and defects detected during the initial assessment of the immune status by the main most common methods (study of the functional activity of different classes and subclasses of lymphocytes, hemolytic activity of the complement system, nonspecific acute phase indicators, interferon status, immune control of opportunistic infections, etc.) .

3. Instrumental diagnostics:

4. Expert advice:

• According to the standards for diagnosis and treatment of the underlying disease and concomitant pathology.

The main signs of secondary immunodeficiencies:

lack of connection with heredity and genetic conditioning;

occurrence against the background of normal reactivity in connection with the disease, exposure to adverse physical and biological factors, methods or means of treatment;

maintaining a deficit in the treatment of the underlying disease and the elimination of factors that induce it;

absence or prolonged delayed normalization of the immune status.

Treatment of the disease

Conservative treatment

Stages of treatment and immunorehabilitation of patients with acquired immunodeficiency states and secondary immunodeficiencies

1. Elimination of the etiological factor.

2. Antimicrobial therapy.

3. Replacement immunotherapy.

4. Prevention of infection.

5. Immunocorrective therapy.

6. Anti-relapse immunocorrection and immunorehabilitation.

Therapy of patients with secondary immunodeficiencies includes: 1) elimination of symptoms of clinical syndromes, including infectious ones; 2) correction of the immunodeficiency itself; 3) prevention of relapses and complications of immunodeficiency.

I. Stage of immunocorrective therapy (acute period)

1 . T-cell VID virus-induced

Antiviral drugs (acyclovir)

Interferons (α, γ, leukinferon)

T-mimetics (taktivin at a dose of 0.01% solution - 1 ml s / c, thymoptin at a dose of 100 mcg, thymogen 0.01% - 1 ml / m, thymolin 10 mg)

Immunofan 1 ml 0.005% solution IM 1 time per day No. 10

Galavit 200 mg once a day IM No. 10

T-cytokines (IL-2 - roncoleukin, etc.)

• Polyoxidonium at a dose of 6 to 12 mg:

2. B-cell VIDs associated with bacterial infections

Antibacterial (antifungal) drugs

Immunoglobulins (antibodies) in severe cases intravenously:

sandoglobulin 1.0; 3.0; 6.0; 12 g in a vial;

octagam 50, 100, 200 ml in a vial;

intraglobin 2.5 g; 5.0 g;

• normal human immunoglobulin for intravenous administration of biavens 1.0; 2.5.

• pentaglobin 5% - 10.0 ml; 20.0 ml, 50.0 ml.

Replacement therapy is carried out in saturation mode (IgG level is not less than 400 μg/ml), maintenance therapy is carried out under the supervision of an immunologist.

B-mimetics (myelopid 0.003 g, polyoxidonium at a dose of 6 mg to 12 mg)

Broad spectrum immunocorrectors, complex of cytokines

3. Phagocytic

Antibacterial (antifungal drugs)

Broad spectrum immunostimulants:

polyoxidonium at a dose of 6 to 12 mg;

licopid at a dose of 1 mg - 10 mg.

Preparations of granulocyte-macrophage colony-stimulating factors:

molgramostim (leukomax) 150 mcg; 300 mcg; 400 mcg;

filgrastim (neupogen) 300 mcg, 480 mcg.

granocyte (lenograstim) 105, 265 and 365 mcg.

Replacement therapy:

leukomass.

cytokines

4. In case of violation of the synthesis of α- and γ-interferons or the need for its stimulation (with T-cell deficiency, chronic viral infection), the appointment is indicated interferon preparations and its inductors:

natural interferons (human leukocyte interferon, egiferon, leukinferon);

recombinant interferons (reaferon, roferon, viferon, intron);

interferon inducers (amiksin, cycloferon, neovir, poludan);

Vitamins (C and others), trace elements

Complementary Therapy

Extracorporeal methods of immunocorrection:

Extracorporeal immunopharmacotherapy (EIFT);

Plasmapheresis;

Immunosorption

II. Stage of anti-relapse immunorehabilitation (in remission)

• Adaptogens (ginseng, eleutherococcus, etc.)

Immunostimulants of plant origin (immunofan, etc.)

Spa treatment

Physiotherapy (EHF, ultrasound, etc.)

Broad-spectrum immunostimulating vaccines (likopid, ribomunil, VP-4, staphylococcal toxoid)

The type of immunocorrection (general and local) and its specific method (physical, chemical, biological) is determined by the nature of the deficiency and its belonging to one or another variant of the immune system disorder.

The duration of outpatient or inpatient treatment depends on the nature and severity of the disease and ranges from 3 weeks to 2 months.

To reduce the contact of the patient with microorganisms, various methods of isolation are used. Simple and technically sophisticated methods of preventive isolation are known. The first include: the allocation of a separate ward with a sanitary unit (boxing) for the patient; the use by staff of replaceable gowns, masks, gloves, thorough hand washing; patients are forbidden raw fruits, vegetables, dairy products - possible sources of gram-negative bacteria. More sophisticated technologies are aimed at cleaning the air surrounding the patient.

Elimination of the etiological factor is possible when the cause of secondary ID is known - immunosuppressive effects, professional agents, etc., which must be eliminated.

Therapy of infectious complications. Since secondary ID is manifested by infectious complications, antimicrobial therapy occupies a key place in their treatment. The choice of drugs depends on the type of microflora and the characteristics of the VID. However, complex therapy is often necessary due to the presence of associations of microorganisms.

Antimicrobial agents not only reduce the dose of infections, but, by destroying them, create "autovaccines" that stimulate the immune system. Antiviral drugs that prevent viral replication release their nucleic acids to induce interferons, and capsid proteins to activate antibody genesis.

Antibacterial drugs, destroying bacteria, release structures recognized by cells and humoral factors of innate immunity: lipopolysaccharides, peptidoglycans and others that activate immunity, the formation of adaptive immunity. Particularly effective are drugs that not only do not depress, but themselves stimulate the development of immunity.

Antibacterial therapy. In VID, bacterial infections often recur. Treatment includes the main course and maintenance therapy. The principles of rational antibiotic therapy are used. The duration of antibiotic therapy exceeds the period of treatment of ordinary patients by 2-3 times. High doses of broad-spectrum antibiotics, their combinations, long courses of each drug (up to 10-14 days with its effectiveness) are used. The relief of exacerbations of bacterial infections is achieved, as a rule, by sequentially conducting 2-3 or more courses of antibiotic therapy, with a total duration of at least 4-5 weeks. The duration of treatment with one drug is from 10 to 21 days.

For active non-specific and semi-specific therapy, conditionally pathogenic microorganisms are used in the form of heterovaccines, consisting of microbes that colonize the respiratory tract (ribomunil, IRS-19), or immunostimulants (licopid, polyoxidonium) are used.

Nucleic acid preparations, in particular, Na nucleinate, obtained from yeast, have an effect similar to vaccines. It reduces the deficiency of T- and B-cells, IgM, increases resistance to infection by many bacteria, has proven itself in chronic mumps, chronic bronchitis, peptic ulcer, as well as in the treatment of complications of radio- and chemotherapy.

Among the means of immunomodulatory therapy, drugs from the organs of immunity (taktivin, thymalin, myelopid, etc.) are shown. The choice of means is determined by the variant of immunodeficiency, the violation of its certain links.

In VID with insufficiency of the humoral link of immunity during a bacterial infection, the production of immunoglobulins suffers. In case of a staphylococcal infection, human antistaphylococcal immunoglobulin at a dose of 5 IU / kg per day intramuscularly daily for 5-10 days with generalized and 3 ml per day intramuscularly with a localized infection is indicated for such patients, and for other bacterial infections of human immunoglobulin normal at a dose of 3 - 4.5 ml / m daily or every other day 3 - 5 times.

Antifungal therapy. Antifungal drugs in patients with secondary ID are used for therapeutic and prophylactic purposes. Patients with different forms of ID are not equally susceptible to fungal infection. In patients with humoral and many combined defects, fungal infection is rare, so antifungal drugs (nystatin, levorin, fluconazole, diflucan, clotrimazole) are used in prophylactic dosages with repeated courses of antibiotics.

Antifungal therapy acquires leading importance in the treatment of generalized forms of fungal infection. Such patients may have lesions of the skin and mucous membranes with fungi. Candida And Aspergillus, but severe infections are also possible, especially with AIDS and highly pathogenic cancer Histoplasma capsulatum, Coccidiodes immity; rare pheogyphomycosis (Curerelaria spp., Alternaria spp. etc.), zygomycosis ( Rhizopus spp., Mucor spp.), hyalophomycosis (Fusarium spp. etc.) due to endogenous colonization. In these patients, even non-pathogenic yeasts can cause lethal infections.

For a long time, the main antifungal drug for widespread candidiasis was amphotericin B. The advantage of this drug is its high efficiency and the possibility of intravenous and endolumbar administration, which is necessary for visceral candidiasis or candidal meningitis. However, the drug is toxic, which limits its use to the most severe mycoses (with Aspergillus infection, 1 mg/kg/day for 6 months). Less toxic is its liposomal form - ambisome (3-5 mg/kg). Non-life-threatening superficial candida infections, in particular recurrent mucocutaneous candidiasis, are effectively eliminated by drugs of the imidazole group. Ketoconazole (nizoral, orungal) at a dose of 200-400 mg per day leads to the cleansing of mucous membranes from thrush within 24-72 hours. Elimination of skin lesions requires 2-9 weeks. It is advisable to combine enteral treatment with topical application of antifungal ointments and liquids. Despite the good effect observed throughout the course of treatment and in the next period after its completion, drug withdrawal leads to gradual recurrence of fungal infections. Therefore, treatment regimens for patients with chronic candidiasis are individual. The low toxicity of Diflucan and good tolerance by patients allows for long-term treatment even in young children.

For the prevention of pneumocystis pneumonia, fluconazole or intranazol (5-10 mg / kg / day) is used. For the prevention of pneumocystis pneumonia in HIV infection, as well as for treatment, pentamidine (aerosols and IV) is used, and if it is intolerant, dapsone is used. However, if against the background of the use of broad-spectrum antibiotics, febrile condition persists for 5-7 days, and fungi cannot be isolated, amphotericin at a dose of 0.5-0.6 mg/kg/day is recommended.

Antiviral therapy indicated for T-cell and interferon VID. Prevention of some viral infections is achieved through vaccination while maintaining the patient's antibody synthesis. Their deficiency is accompanied by viral encephalitis and meningitis, E CHO - viral infections.

In the treatment of viral respiratory infections in patients with VID, all conventional means are used, as well as additional therapeutic or prophylactic measures that prevent the development of complications, taking into account a specific immunity defect (antibiotics, emergency plasma transfusion, or administration of gamma globulin).

An effective treatment for acute herpetic infections (genital, proctitis, pneumonia) is the drug acyclovir (zovirax) (400 mg orally after 8 hours, anti-relapse - 200-400 mg after 12 hours), the action of which is based on blocking specific enzymes of the virus, with herpes and For cytomegalovirus (CMV) infections, foscarnet (60 mg/kg IV every 8 hours), ganciclovir 5 mg/kg IV every 12 hours, then famciclovir (250 mg orally every 8 hours) are also used. In severe herpes-zoster infection - acyclovir 10 mg / kg IV every 8 hours for 7-14 days; in mild cases, 800 mg orally every 4 hours, or famciclovir 500 mg orally every 8 hours; or valaciclovir 1 g orally every 8 hours.

According to the indications, interferon preparations are prescribed in various dosages, depending on the type of immune defect. Interferon has antiviral, immunomodulatory, antiproliferative and radioprotective effects. The concept of "interferon status" is formulated, the assessment of which is based on the definition of: serum IF, ability to produce α-IF, ability to produce γ-IN.

In this case, the relationship of the components is more significant, rather than individual values ​​(under physiological conditions, the level of serum IF does not exceed 4 IU, and it is represented by a mixture of interferons of various types). γ-interferon is able to cause both stimulating and suppressive effects on the inflammatory process. Its participation in the mechanism of maintaining normal fetal immunosuppression, as well as in preventing the implantation of a fertilized egg with the introduction of intrauterine contraceptives, is assumed. α-interferon is successfully used in the treatment of sepsis, actively stimulating T-cells and neutrophils. Human recombinant α 2 -interferon in combination with antioxidants (viferon) is recommended for the treatment of viral and bacterial infections in newborns and young children (ID risk group), reduces the administration of blood products and reduces the duration of antibiotic therapy in severe forms of infection in the neonatal period. Interferon inducers - cycloferon, amixin, neovir are prescribed in a daily dose of 5-8 mg / kg in repeated courses for 5-10 days with long-term infections - hepatitis, herpes, chlamydial, campylobacteriosis. Poludan is used as eye drops and subconjunctival injections.

Isoprinosine has antiviral activity.

Lincomycin suppresses many viruses (herpes type 1, encephalomyelitis, etc.).

In VID, accompanied by insufficiency of the humoral link of immunity and in severe recurrent course of herpes type 1 (herpes labialis), human immunoglobulin against herpes simplex virus type 1 is administered intramuscularly, 4.5 ml (3 amps.) 1 time in 3 days up to 5 injections . Human immunoglobulin against herpes simplex virus type 2 is used to treat acute or exacerbation of chronic herpes virus infection type 2 (herpes genitalis) - IM 1.5 ml (1 dose) once every three days. The course of treatment - 7 injections, as well as locally - treatment of blistering herpetic eruptions.

Gerpimmune 6 (immunoglobulin against human herpes virus type 6 liquid) for i / m administration is used to treat patients with herpes infection with damage to the nervous system caused by the herpes virus type 6. Enter / m 3 ml (2 amp. 1.5 ml) once for three days up to 9 injections. Produced in ampoules of 1.5 ml.

Human immunoglobulin against Epstein-Barr virus used to treat diseases caused by the Epstein-Barr virus, including encephalitis, encephalomyelitis, meningoencephalitis, arachnoencephalitis, arachnoiditis, encephalopolyradiculitis, infectious mononucleosis. For adults, immunoglobulin is administered intramuscularly at 4.5 ml (3 ampoules of 1.5 ml) 1 time in three days. The course of treatment is up to 5 injections.

Treatment and prevention with intravenous immunoglobulins. With antibody deficiencies and secondary hypogammaglobulinemia, the use of blood plasma preparations and intravenous immunoglobulins is the leading method of treatment and prevention of infections.

In case of deficiency of immunoglobulins (Ig) (agammaglobulinemia), Ig is administered intravenously in saturation mode at 400-800 mg/kg of body weight. Octagam 400-800 mg/kg at a time, course: one injection (200 mg/kg) with an interval of 3-4 weeks. Pentaglobin for newborns at 5 mg/kg daily for 3 days (1.7 ml/kg/hour), for adults - 0.4 ml/kg/hour and up to 15 ml/kg/hour for 3 days.

Native fresh frozen plasma is used at 10-40 ml/kg. Course 1000-2400 ml 2 times a week.

To prevent infection, Ig levels in VID should be maintained at least 4–6 g/l (200–800 mg/kg/month octagam). For the same purpose, native plasma is administered once a month at 15-20 ml/kg.

Preventive immunization. According to the WHO memorandum (1995), live vaccines should not be administered in severe cases of immunosuppression:

with secondary hypogammaglobulinemia

with acquired ID due to lymphomas, lymphogranulomatosis, leukemia and other oncological diseases of the immune system

in the treatment of immunosuppressants and radiation therapy

The effectiveness of vaccination in children with VID is low: with insufficient immunoglobulins due to a quantitative deficiency of antibodies, but immunization with toxoids is safe.

Local therapy in the lesion depends on its type: with manifestations of allergies - corticosteroid ointments (elokom, advantan, prednisolone, etc.), with concomitant infection - triderm, etc.

The use of immunomodulators in acquired (secondary) immunodeficiencies

The main indication for the appointment of immunomodulators is the presence of VID, the diagnosis of which is established by clinical and laboratory data. Initially, 3 groups of people are distinguished: 1) persons (patients) with clinical signs of immune disorders in combination with changes in its parameters detected by immunological methods; 2) persons (patients) who have only clinical signs of impaired immunity (without laboratory data); 3) persons with deviations of SI indicators, but clinically healthy. Immunomodulators are recommended to be prescribed only to patients. It is not recommended to correct changes (probably compensatory) in the immune status in clinically healthy individuals (Manko V.M. et al., 2002).

The combination of local and general immunocorrective therapy allows to achieve the greatest clinical effect. Combined immunocorrection may include a combination of 3-4 means and methods of various effects, mainly affecting different parts of the immune system.

So, for example, Novikov D.K. and co-authors (2005) proposed to combine levamisole, dimexide and heparin in immunodeficiencies with the clinic of pyoinflammatory diseases, since dimexide leveled the negative effect of levamisole on neutrophils, and intradermal heparin increased lymphopoiesis. The introduction of a 30% solution of dimexide was carried out by electrophoresis according to the general method until the 7th day of the postoperative period, and then dimexide was injected locally into the area of ​​the sanitized focus until the patient was discharged from the hospital. Levamisole was prescribed 25-50 mg every other day for 15 days. Instead of levamisole, taktivin (thymalin), polyoxidonium and other immunomodulators can be used. In the presence of a bacterial infection, they should be combined with antibacterial drugs, which also enhance immune responses. Such effects were found in metronidazole, which stimulates the synthesis of antibodies, phagocytosis, interferons.

In severe and moderate chronic recurrent furunculosis with impaired cellular and phagocytic immunity, patients received stable remission with the use of polyoxidonium, lycopid, myelopide.

With ID observed against the background of COPD, a positive effect was observed when prescribing: levamisole, T-activin, sodium nucleinate, diucifon, and others. In some cases, in COPD, the inhalation route of administration of immunomodulators (a combination of dimexide and levamisole solutions) is preferable.

In patients with chronic bronchitis and with various changes in the immune status, remission was obtained during treatment with polyoxidonium or licopid. Polyoxidonium is best prescribed in the acute phase in combination with antibiotic therapy for changes in the lymphoid system and phagocytosis. Positive results have been obtained in stopping exacerbations of infection in patients with bronchial asthma on the background of these immunomodulators.

Prevention of acquired immunodeficiency states. Prevention of secondary immunodeficiencies can be both preventive and anti-relapse. The first is the timely and complete treatment of diseases that may be the cause of these defects; early diagnosis of imbalance in SI, which is the basis of its development; timely correction of this imbalance.

Anti-relapse prevention is based on clinical examination of patients and immunorehabilitation of those who have secondary immunodeficiency. Such patients should be regularly examined and if negative shifts in SI are detected in the dynamics, immunocorrection is necessary. For example, it has been shown that children who have had purulent-septic diseases need immunorehabilitation, because despite clinical recovery, their cellular and humoral immunity indicators are not fully restored: IgG is still reduced; IgM and IgA are at subnormal levels, and some children are above normal, which reflects the body's readiness for reinfection. If the reduced indices of immunological reactivity persist during the remission period, a complex of active rehabilitation measures is carried out.

Nonspecific immunorehabilitation and immunoprophylaxis. An important stage in the treatment of patients is anti-relapse immunoprophylaxis, carried out during the period of remission. The use of "soft" immunostimulants in cases of sufficiently preserved immunity reactivity prevents relapses of the disease, i.e. provides immunorehabilitation. For this purpose, during the remission period, adaptogens, immunostimulants of plant origin (echinacea, ginseng, etc.), as well as vitamins and microelements, are administered orally. Physioimmunotherapy courses (EHF, magnetotherapy, etc.) are used.

Immunoprophylaxis of professional VIDs. Two schemes were tested: the 1st scheme included sodium nucleinate, undevit and eleutherococcus extract; 2nd - riboxin, undevit, eleutherococcus extract, which were prescribed for 20-45 days.

Immunorehabilitation in acquired immunodeficiency states. When compiling a complex of immunocorrective therapy, the first appointment in the complex, as a rule, should be decisive and correspond to the main defect in immunity, then the means of unidirectional action are selected, but mediating a similar effect of therapy through a different mechanism. After the implementation of the main treatment regimen for VID, background therapy is carried out, the complex of which, in turn, is determined by the characteristics of the clinical mask of the immunodeficiency state. The main goal of background therapy is further immunorehabilitation.

Immunorehabilitation of patients with recurrent viral infections of the respiratory tract.

In the acute period, treatment is prescribed that corresponds to the etiology and pathogenesis of a viral infection with the use of antiviral and antibacterial drugs against the background of detoxification and vitamin therapy. In remission (preferably immediately after immunocorrective therapy), it is advisable to prescribe:

1. Extract or infusion of eleutherococcus or ginseng in a therapeutic dose for 1-2 months (immunological adaptogen).

2. Oxygen cocktail with lingonberry and wild rose fruit infusion, 2-3 courses per year, 10 sessions each.

3. Dibazol (inside) in a therapeutic dose for 10-12 days.

4. Antioxidant complexes (vitamins C, A, E, trace elements - zinc, selenium, copper).

In case of contact with acute respiratory viral infections, prophylactically and for the purpose of immunomodulation, interferon is prescribed in the nose and heparin subcutaneously at a therapeutic dose (100 U / kg) for 5-6 days with the control of primary hemostasis after a week.

If the clinical mask of VID is also characterized by subfebrile condition, then it is advisable to introduce cinnarizine, nicotinic and glutamic acids into the background therapy complex after Dibazol. After that, a complex of vitamin herbs and plants with a high content of bioelements in the form of infusions is prescribed for 4-6 months. A good clinical effect can be obtained by prescribing glycine, especially for children with increased neuromuscular excitability, ½ - 1 t 2 r. per day for 10-12 days.

Immunorehabilitation of patients with immunodeficiency with a clinic of recurrent pneumonia, bronchitis, resistant to traditional therapy.

After the completion of the main course of immunocorrective therapy, which should be started in the acute period, we recommend the following dispensary immunorehabilitation:

1. Lysozyme or estifan orally 1 time per day for one week of each month, repeat 3-4 courses (therapeutic dosage, it is better to dilute with milk, do not prescribe if you are intolerant to eggs).

2. Extract of Eleutherococcus (or magnolia vine) for 30 days.

3. Oxygen cocktails with vitaminized syrups every 10 days for 2 months.

4. Glycyram for 10 days after every third month for 10 months or ultrasonic impact on the area of ​​the adrenal glands (3 sessions).

5. Phytotherapy from herbal infusions: mint, St. John's wort, nettle (3-4 times a day for a month, alternately for 10 days of each herb), repeat such courses 2-3 times a year.

6. Background rehabilitation ends with a short scheme (2 times) of immunization with staphylococcal toxoid or a course of bronchomunal for 10-30 days.

In the presence of abscesses that complicate pneumonia, it is advisable to include in the treatment regimen, in parallel with lysozyme, applications with a 25-30% solution of dimexide (can be administered by electrophoresis) on the projection of the affected area up to 8-10 sessions.

Scheme of immunorehabilitation for recurrent bacterial bronchitis.

1. Phytoncidal antibacterial drugs in turn: garlic tincture, chlorphyllipt, calendula tincture 10 days inside at an age dose (1 drop per year of life, but not more than 20 drops);

2. Lysozyme for 10 days orally or by injection (for young children at the rate of 10 mg/kg of body weight);

3. Glycyram for 10-14 days at an age dose to stop the processes of infectious allergization;

4. UHF on the solar plexus area, 5 sessions per course 2 times a year;

5. Probiotics (bifidum-bacterin, lactobacterin, etc.) with a tendency to develop bacterial dysbacteriosis and after antibiotic treatment under the control of the composition of the intestinal bifidoflora. You can use linex, hilak-forte.

Courses of volatile antibacterial drugs can be repeated 2-3 times a year.

Thus, by combining various preparations (it is better to test them in vitro with the patient's leukocytes in order to restore receptor expression and functional activity), it is possible to draw up immunocorrective therapy regimens for each specific case. A prerequisite for immunorehabilitation is immunological control of its effect. When performing it, one should remember the timing of the onset of this effect for each drug used and not rush to cancel the drug, replacing it with another, even if they belong to the same immunological group. Restoring the immunological competence of the body is a long process that requires a thoughtful and necessarily scientifically based approach to therapy, taking into account the characteristics of clinical manifestations at the present time and causally significant pathology.

In cases where there is no real opportunity to conduct an immunological examination of the patient to identify an immune defect and select corrective therapy (for example, in a rural region), therapeutic rehabilitation complexes based on clinical data on the patient can be recommended for practical use.

Such treatment regimens may include:

a) drugs from the group of biogenic stimulants that improve the presentation of various antigens (bacterial, viral, fungal and mixed) through phagocytosis systems

b) means that stimulate anabolic processes

c) drugs that activate redox reactions in tissues (including those in immunocompetent organs)

d) trace elements and their compounds

e) drugs that improve metabolism in the nervous system with indirect mediation in the immune system (amino acid drugs to stimulate protein metabolism and energy processes in brain tissues, increase their respiratory activity, nootropic drugs to form associative links between brain cells; drugs that compensate hypoxia in the central nervous system and improving lipid metabolism, for example, calcium pangamate.

Clinical indications in such cases may be:

1. Recurrence of inflammatory diseases with the risk of developing chronic forms and the formation of a chronic focus of infection.

2. Tendency to generalization of purulent-septic disease (according to clinical data).

3. The presence of unusual or pseudo-allergic side reactions to traditional drugs.

4. Long-term trace astheno-vegetative dysfunction of the nervous system, changes in peripheral blood such as neutropenia, leukopenia, thrombocytopenia, lymphocytopenia, etc.

For prevention development of a chronic disease The following preventive treatment with elements of immunorehabilitation is recommended:

1. Against the background of the subsidence of the acute phase of inflammation, injections of peat (peat distillation) subcutaneously or gumizol are prescribed, 0.3-0.5 ml for preschool children, 1 ml for adults with a course of 15-20 injections every other day, can be alternated with injections of aloe extract liquid (in the absence of intolerance to it).

2. Intravenous administration of sodium thiosulfate (it is also possible orally, but the clinical effect is somewhat weaker) for the purpose of mild desensitization, detoxification and anti-inflammatory action. Clinical effects are mediated by the leveling of excess mediators of increased sensitivity of immediate and delayed types by sulfur compounds, which together determine the morphological equivalent of a chronic process. When administered orally, a 10% solution of sodium thiosulfate is used, depending on age, 1 teaspoon, dessert or tablespoon 3 times a day; with the intravenous method, a 30% solution of 1-1.5 ml is used up to 5 years; 2-3 ml for children over 5 years old, adults 5 ml 1 time per day, the course of treatment is usually 10-14 days.

With a tendency of the infectious process to generalization (the main clinical signs are: general biological unreactivity, asthenia, prolonged disturbance in the microcirculation system, leukocytosis with neutrophilia in the absence of a localized purulent focus or, conversely, leukopenia with a manifest purulent-inflammatory process, inconsistency of the temperature reaction with clinical manifestations, especially against the background of perinatal brain damage) it is recommended:

1. In case of leukopenic reaction of the blood, start the prevention of VID with plasmol subcutaneously for preschool children, 0.2-0.3 ml, for adults, 1 ml of the solution 1 time per day for 10-14 days. With leukocytosis, you should start with dibazol in injections for up to 2 weeks at the age-specific therapeutic dosage.

2. In parallel, a course of vitamin P (in the form of "ascorutin") is prescribed orally, but better, in the form of "urutin" - in injections of 0.2-0.3 ml for young children, 0.3-0.4 ml - preschoolers, adults 1 ml subcutaneously 1 time per day for 20-30 days. Vitamin P stimulates the functional activity of immunocompetent cells, which is probably due to its well-known activating effect on redox processes in tissues.

3. To replenish bactericidal factors, the next appointment may be lysozyme (a preliminary biological test for tolerance is required). Lysozyme is prescribed by injection or by mouth for 7-10 days once a day, as well as echinacea or estifan.

In the presence of unusual reactions to drugs that stimulate intolerance, it is recommended to use a complex of agents that stabilize cell membranes, which contributes to the adaptation of the receptor apparatus of immune system cells:

1. Vitamin E parenterally in the form of intramuscular injections for up to 7-10 days.

2. Zinc oxide is administered enterally to stimulate chemotaxis of polymorphonuclear leukocytes and monocytes, as well as to stabilize the membranes of these cells. Zinc oxide is prescribed in a dose of daily requirement from 4-6 mg in infants to 10-20 mg in other children and adults in 2 divided doses.

3. In parallel - ultrasonic impact on the projection area of ​​the adrenal glands daily course No. 5.

Common in the complex rehabilitation of all types of VID, regardless of clinical manifestations, is the appointment of repeated courses of fortified oxygen cocktails, herbs containing vitamin complexes and bioelements, such as nettle leaves, lingonberries, rose hips, black currants, wild strawberries, blueberries, etc. Almost all children and most patients are shown immunological adaptogens: eleutherococcus, aralia, ginseng, zamaniha, lemongrass, golden root, calamus, levea, which can be prescribed in the form of infusions and extracts. In addition, it must be remembered that all children with acquired ID (these are often children from the group of frequently ill patients) need, along with chemotherapeutic immunorehabilitation, individual exercise therapy and hardening measures, rational nutrition in accordance with the clinical manifestations of ID, rational use of natural factors of their area and resort therapy.

Immunocorrection for adenoiditis and rhinitis

Comprehensive treatment regimens for hyperplasia, tonsils and adenoids:

prosol 10 days

calendula 10 days + estifan 3 weeks

chlorophyllipt 2 weeks inside

IRS-19 - spray, irrigate on tonsils 2 times a day, course 2 weeks

prosol 10 days.

The tonsils shrink and shrink.

A comprehensive treatment regimen for chronic tonsillitis (exacerbations). Criteria - adhesions with arches and signs of chronic intoxication.

amoxicillin or amoxiclav - 7 days in age doses;

leukinferon 5 injections or viferon 5-7 suppositories of 500,000 IU every other day;

prosasol 10 days + plasmol №5;

licopid 10 days, 1 mg for children and 10 mg for adults;

estifan + lugol;

UVR of tonsils No. 5;

dimexide 30% - applications on the submandibular and cervical lymph nodes;

ribomunil 6 weeks, 1 tablet 2 times 4 days a week.

A comprehensive treatment regimen for chronic rhinitis associated with acute respiratory infections

ribomunil 1 tablet 2 times 4 days a week for 6 weeks or IRS-19 - 10 days (nasal spray);

applications with 30% dimexide on the back of the nose and projections of the maxillary sinuses No. 10 every other day;

aloe - nasal electrophoresis;

vasoconstrictor - sanorin is better, the mucosa is less dried.

If inflammatory and infectious diseases bother you and are difficult, perhaps we are talking about immunodeficiency. In this pathological condition, a malfunction occurs in the immune system, against the background of which serious diseases develop that are difficult to treat. The severity and nature of their course depends on the type of immunodeficiency. In some cases, there is a risk of developing serious conditions that threaten health and even life.

What are the types of immunodeficiency

Depending on the factors that led to the disease, all conditions can be divided into primary and secondary immunodeficiency.

Primary immunodeficiency

In this case, we are talking about a congenital disordertransmitted from parents to a child or resulting from a genetic mutation due to the action of toxins on the fetus during fetal development. Although in some cases the cause of immunity disorders remains unclear.

There are various forms of congenital immunodeficiency, in some cases the condition is determined immediately after birth. However, in most cases (about 85%), the disease is diagnosed at a young age, usually before the age of twenty. This form of immunodeficiency accompanies a person until the end of life and affects one or more parts of the immune system:

• With humoral immunodeficiency, antibodies are either produced in insufficient quantities or not synthesized at all, bacteria and their toxins are not neutralized.
• When cellular immunity is impaired, insufficient activity or levels of T-lymphocytes are detected, which leads to impaired antibody production.
• Defects in phagocytosis lead to the fact that the cells of the immune system are not able to destroy pathogenic bacteria, which, in turn, multiply, and an infection develops.
• Complement deficiency - a group of proteins in the blood involved in the destruction of bacteria and their toxins - with complement deficiency, proteins are not able to destroy foreign cells.

Secondary immunodeficiency

Secondary deficiency- a condition that develops against the background of many factors can be detected in both children and adults. There are three forms of the disease: induced, acquired and spontaneous. In the first case, the disease is associated with a specific cause, for example, exposure to radiation, trauma, poisoning with medicines or chemicals, etc., and can also develop as a result of the underlying disease: cancer, kidney disease, liver disease, diabetes, etc. The brightest an example of an acquired form is HIV as a result of infection with a virus. In a disease of spontaneous origin, the cause of impaired immunity has not been identified.

How to suspect immunodeficiency?

Often, especially among parents, the question arises: how to understand - frequent diseases are the result of a weakened immune system or is it an immunodeficiency? What should you pay attention to? There are several warning signs, in the presence of which it is better to visit an immunologist.

• Frequent repetition the same disease of a bacterial nature, for example, purulent otitis media, endless diarrhea, skin infections;
• Infection proceeds in severe, despite the ongoing treatment, improvement does not occur for a long time;
• During the examination for an infectious disease, it was found pathogens rare for this pathology;
• infections have hereditary character, for example, parents also often suffered from the same disease;

Immune deficiencies are characterized by severe infections with constant exacerbations, bronchitis, pneumonia, otitis, sinusitis, lymphadenitis - frequent companions of a person with impaired immunity. Often a person suffers from skin diseases: pyoderma, furunculosis, phlegmon, fungal infections are possible, the appearance of herpes of various localization. Colds are often accompanied by stomatitis.

In addition to clinical manifestations, you can confirm the diagnosis by passing. Screening tests of the first level are carried out in many clinics, in-depth immunological examination can only be done in an institution that has a clinical immunology laboratory. If primary immunodeficiency is suspected, tests can determine the type of mutation that caused the disease and the dysfunctional link in the immune system.

Immunodeficiency in children

Immunodeficiency is a serious diagnosis, meaning that the baby lacks natural protection. Touching a child with hands that have not been washed right this minute, a parental kiss and other completely harmless actions from the point of view of a healthy person are a source of danger for the baby. And the result is the development of serious diseases, in the absence of treatment, often leading to death.

The problem is that with the congenital form, there are no unique primary signs. Common, as many parents believe, infection, gastrointestinal problems - often do not cause alertness. Meanwhile, the disease becomes chronic, complications appear, the usual course of antibiotics is ineffective.

But even by the nature of the infection, it can be assumed which component of the immune system does not work correctly. Insufficiently rapid healing of the umbilical wound, purulent skin lesions may indicate a defect in the phagocytic system. After six months, as a rule, infections appear associated with the disappearance of innate immunity transmitted from the mother. Under the influence of pathogenic pathogens (pneumococci, streptococci, etc.), infections of the respiratory system develop. In processes caused by viruses or fungi, deviations in the link of T-lymphocytes can be assumed. Anxiety should be caused by chronic pneumonia, long-term diarrhea that is difficult to treat, or candidiasis.

In the future, a characteristic feature may be the ease with which infections appear and progress. For example, bronchitis easily turns into severe pneumonia with respiratory failure. Typical signs are digestive disorders, papillomas, fungal infections, etc.

Treatment of immunodeficiencies

Treatment of primary immunodeficiency is a rather difficult task. To do this, it is necessary to accurately determine the impaired link of immunity, and based on the results obtained, therapy is prescribed. With a lack of immunoglobulins, the patient needs replacement therapy throughout his life, he is prescribed serum with antibodies or plasma. With the development of complications of an infectious nature, antibiotic therapy, treatment with antifungal drugs, etc. is necessary. Immunological reconstruction in the primary form of immunodeficiency is possible with bone marrow transplantation.

In the secondary form of immunodeficiency, treatment also begins with finding out the cause of development and its elimination. However, unlike primary immunodeficiency,. First of all, it is necessary to sanitize the focus with the help of antiviral or antibacterial drugs. Therapy is carried out in three directions: immunotropic treatment, replacement therapy (plasma, immunoglobulins, leukocyte mass, etc.), active immunization using vaccines. Vaccine therapy can be prescribed to prevent both infectious and somatic diseases.

Prevention of immunodeficiency

For the prevention of hereditary immunodeficiency, today there is an opportunity to undergo genetic counseling for people who are just planning to have a baby. If the family already has patients with immune disorders, you can be diagnosed for the carriage of the defective gene. In addition, pregnant women may undergo prenatal genetic testing to determine the risk of having an affected child.

Based on the fact that the cause of primary immunodeficiencies may be disorders resulting from the action of various toxins on the fetus during fetal development, pregnant women should avoid contact with harmful substances.

As for the prevention of acquired immunodeficiencies, in this case it can be recommended. Timely treatment of various diseases, maintaining a healthy lifestyle, as well as the rejection of casual relationships in order to avoid HIV infection - these simple recommendations will help to avoid serious consequences.

How to live with immunodeficiency

Regardless of the form of immunodeficiency, all patients without exception should avoid contact with the infection: any one can be fatal for them. Remember: it is impossible not to get infected. Of course, for many, treatment will be lifelong, most likely expensive. In addition, the family expects constant hospitalizations, antibiotics, sick leave for adult patients or parents of sick children.

And most importantly: the life expectancy of patients with a congenital form depends on timely and regular medication! For patients with acquired forms, it is also important to undergo regular examinations to control and prevent sudden progression.

And although there are over 250 types of disorders that lead to immunodeficiency, there are people for whom a malfunction of the immune system and AIDS mean the same thing. But primary immunodeficiency has nothing to do with AIDS, they cannot be infected. But, unfortunately, patients often have to deal with misunderstanding.

By the way, in Russia, for children suffering from dangerous immunity disorders, the Sunflower Charitable Foundation has been created. There is also an organization "Society of Patients with Primary Immunodeficiency" uniting patients and their families. The purpose of the organization is to protect and support patients, including legal, informational and psychological.

Do you know that 90% of patients with immunodeficiency in our country die without receiving help? Late diagnosis, or even its absence, improper treatment, shortage of medicines is our reality. Some have to undergo regular therapy and comply with numerous restrictions. But modern medicine can provide many patients with a fairly long and fulfilling life. But for this it is necessary, first of all, not to dismiss even seemingly trifling complaints, and in case of any violations, consult a doctor. Indeed, in order to identify the cause that does not allow the immune system to function normally, a simple clinical examination is sufficient.

Oksana Matias, general practitioner

Illustrations: Julia Prososova

Immunodeficiency is a condition that is characterized by a decrease in the function of the immune system and the body's resistance to various infections.

From the point of view of etiology (reasons for the development of the disease), we distinguish between primary and secondary immunodeficiencies.

1. Primary immunodeficiencies- This is a group of diseases that is characterized by a decrease in the function of the immune systemoccurring against the background of various genetic disorders. Primary immunodeficiencies are quite rare, about 1-2 cases per 500,000 people. In primary immunodeficiencies, individual components of immunity may be impaired: the cellular link, the humoral response, the phagocyte and compliment system. So, for example, immunodeficiencies with a violation of the cellular link of immunity include such diseases as agamaglobulinemia, DiGiorgio syndrome, Wiskott-Aldrich syndrome, Bruton's disease. Violation of the function of micro and macrophages are observed during chronic granulomatosis, Chediak-Higashi syndrome. Immunodeficiencies associated with a violation of the compliment system are based on a deficiency in the synthesis of one of the factors of this system. Primary immunodeficiencies are present throughout life. Patients with primary immunodeficiency, as a rule, die from various infectious complications.
2. Secondary immunodeficiencies are much more common than primary ones. Usually, secondary immunodeficiencies develop against the background of exposure to the body of adverse environmental factors or various infections. As in the case of primary immunodeficiencies, in secondary immunodeficiencies, either individual components of the immune system, or the entire system as a whole, can be disturbed. Most secondary immunodeficiencies (with the exception of immunodeficiency caused by infection with the HIV virus) are reversible and respond well to treatment. Below we consider in more detail the significance of various adverse factors in the development of secondary immunodeficiencies, as well as the principles of their diagnosis and treatment.

Reasons for the development of secondary immunodeficiency
Factors that can cause secondary immunodeficiency are very diverse. Secondary immunodeficiency can be caused by both environmental factors and internal factors of the body.

In general, all adverse environmental factors that can disrupt the body's metabolism can cause the development of secondary immunodeficiency. The most common environmental factors that cause immunodeficiency include environmental pollution, ionizing and microwave radiation, poisoning, long-term use of certain drugs, chronic stress and overwork. A common feature of the factors described above is a complex negative effect on all body systems, including the immune system. In addition, factors such as ionizing radiation have a selective inhibitory effect on immunity associated with inhibition of the hematopoietic system. People living or working in a polluted environment are more likely to suffer from various infectious diseases and more likely to suffer from cancer. It is obvious that such an increase in the incidence in this category of people is associated with a decrease in the activity of the immune system.

Internal factors that can provoke secondary immunodeficiency include:

Diagnosis of immunodeficiency
Primary immunodeficiency usually appears immediately after the birth of a child or some time after it. To accurately determine the type of pathology, a series of complex immunological and genetic analyzes are carried out - this helps to determine the place of impaired immune defense (cellular or humoral link), as well as determine the type of mutation that caused the disease.

Secondary immunodeficiencies can develop at any time in life. Immunodeficiency can be suspected in the case of frequently recurrent infections, the transition of an infectious disease to a chronic form, the ineffectiveness of conventional treatment, a small but prolonged increase in body temperature. Various tests and tests help to establish an accurate diagnosis of immunodeficiency: complete blood count, determination of blood protein fractions, specific immunological tests.

Treatment of immunodeficiency
Treatment of primary immunodeficiencies is a difficult task. To prescribe a complex treatment, it is imperative to establish an accurate diagnosis with the definition of a disturbed link in the immune defense. With a lack of immunoglobulins, lifelong replacement therapy is carried out with sera containing antibodies or ordinary donor plasma. Immunostimulating therapy with drugs such as Bronchomunal, Ribomunil, Taktivin is also used.

If infectious complications occur, treatment with antibiotics, antiviral or antifungal drugs is prescribed.

In secondary immunodeficiencies, disorders of the immune system are less pronounced than in primary ones. As a rule, secondary immunodeficiencies are temporary. In this regard, the treatment of secondary immunodeficiencies is much simpler and more effective than the treatment of primary disorders of the immune system.

Usually, treatment of secondary immunodeficiency begins with determining and eliminating the cause of its occurrence (see above). For example, the treatment of immunodeficiency against the background of chronic infections begins with the sanitation of foci of chronic inflammation.

Immunodeficiency against the background of vitamin and mineral deficiency is being treated with the help of complexes of vitamins and minerals and various food supplements (BAA) containing these elements. The regenerative capacity of the immune system is great, therefore, the elimination of the cause of immunodeficiency, as a rule, leads to the restoration of the immune system.

To speed up recovery and specific stimulation of immunity, a course of treatment with immunostimulating drugs is carried out. At the moment, a large number of different immunostimulating drugs are known, with different mechanisms of action. Preparations Ribomunil, Christine and Biostim contain antigens of various bacteria and, when introduced into the body, stimulate the production of antibodies and differentiation of active clones of lymphocytes. Timalin, Taktivin - contain biologically active substances extracted from the thymus of animals. Cordyceps - is the most effective immunomodulator that normalizes the immune system as a whole as a system. These drugs have a selective stimulatory effect on a subpopulation of T-lymphocytes. Sodium nucleinate stimulates the synthesis of nucleic acids (DNA and RNA), cell division and differentiation. Various types of interferons increase the overall resistance of the body and are successfully used in the treatment of various viral diseases.

Immunomodulatory substances of plant origin deserve special attention: Immunal, Echinacea rosea extract, and especially Cordyceps.

Bibliography:

• Khaitov R.M., Secondary immunodeficiencies: clinic, diagnosis, treatment, 1999
• Kirzon S.S. Clinical immunology and allergology, M. : Medicine, 1990
• Modern problems of allergology, immunology and immunopharmacology, M., 2002

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