Treatment of immunodeficiency Lakhta. Human immunodeficiency (primary, secondary), causes and treatment

It’s worth thinking about our protective shield already when the first signs of its weakening appear: frequent colds, weakness, dizziness, etc. IDS can be provoked by many factors, so it is necessary to know the nature of its occurrence in order to choose an adequate method for eliminating the disease. An immunologist is called upon to clearly identify the prerequisites that led to the disease.

There are two main types of pathology.

  1. Primary immunodeficiency is a congenital disease caused either by genetic defects or various exposures during fetal development. Depending on the level of exposure and localization of the process, they are: cellular, antibody, combined, expressed by insufficiency of the complement system and defects in phagocytosis.
  2. Secondary immunodeficiency. This pathology is much more common. The disease is caused by a wide variety of environmental factors, which negatively affect almost all elements of the immune system. This group includes acquired immunodeficiency syndrome, known as human immunodeficiency virus (HIV).

The list of causes of secondary immunodeficiency is quite wide:

  • lack of important substances for proper development organism caused by malnutrition;
  • the impact on the body of chronic infections, which, constantly affecting the immune system, reduce its reactivity over time. Also, such diseases have a bad effect on the state of the hematopoietic system, which is responsible for the creation of extremely important lymphocytes;
  • helminthiasis;
  • blood loss or kidney failure;
  • various types of poisoning, prolonged diarrhea, due to which there is a sharp loss of all necessary nutrients;
  • diabetes mellitus or thyroid disorders;
  • oncological diseases.

Procedure process

Preparation

If you or your child are often sick and therapy does not help, it is worth checking your immune system. Before your first visit to the doctor, you can do a little preparation so that the consultation goes quickly and efficiently. For example:

  1. Write down any symptoms you notice.
  2. Gather all previous test results you have ever had.
  3. Do some family medical history.
  4. Make a list of accepted lately drugs and vitamins.
  5. Prepare in advance all the questions you plan to ask the doctor.
Such actions will help the healthcare worker quickly diagnose the disease and prescribe the necessary therapy.

The process of eliminating ailments

Treatment of primary immunodeficiency is carried out using the following methods:

  • etiotropic therapy (in in this case correct the patient’s genomic deficiency);
  • treatment with immunostimulants;
  • transplantation bone marrow, immunoglobulins, thymus cells.

Treatment of immunodeficiency in children, as well as in adults, is carried out under the supervision of a specialist. Secondary IDS is much easier to cure than primary one, because the prerequisites for its occurrence are transient factors. Therefore, it is possible to have an effective suppressive effect on them using correct therapy. After diagnosis and establishment of the true cause, a course of treatment is prescribed.

More often than adults, children are susceptible to secondary IDS, since they have not yet fully developed a mechanism to combat harmful factors environment.

Due to the lack of vitamins and minerals, appropriate vitamin complexes are prescribed. If the presence of a chronic infection is established, then first of all its foci are sanitized.

Immunostimulants help strengthen the immune system after illnesses and operations.

Symptoms

Signs differ depending on the type of disease and may be individual in each person. Among them:

  • frequent infectious disorders that recur from time to time;
  • infection and other blood diseases;
  • developmental delay;
  • problems with the digestive tract;
  • fungal infections;
  • stomatitis;
  • hair loss;
  • allergic reactions;
  • weight loss.

Contraindications

Taking almost every immunostimulant has its own contraindications. Particular care should be taken when treating children with autoimmune problems. Such children should take medications only as prescribed by the attending physician. However, just like pregnant women.

Complications

Characteristic complications for both types of disease are serious infectious pathologies, such as pneumonia, sepsis and others, which depend on the cause of IDS. Early diagnosis can prevent long-term problems.

Prices and clinics

Such a difficult problem should be trusted only to professionals with many years of experience. The portal site will help you select a decent clinic and doctor, including taking into account your financial capabilities.

And heating not working in cold weather - for many this was enough to get sick in the spring. The occurrence of ARVI, colds and almost any disease is inextricably linked with the functioning of the human immune system. Some people drink Kagocel to avoid getting sick, others eat a lot of vegetables and fruits, and still others take vitamins or dietary supplements. Doctor medical sciences and head of the department of immunology of the Russian Children's clinical hospital Irina Kondratenko told The Village whether it is possible to increase immunity, whether yoghurts and vitamin capsules help with this, how stress affects health and what immune memory is.

- How does a person develop immunity?

The immune system, in essence, is engaged in recognizing foreign elements in the body. Such recognition exists even in unicellular organisms, and how more complex organism, the more difficult the protection - both from external factors, and from failures inside. For example, if a tumor cell appears or a cell into which a virus has entered, and viral proteins appear on its surface, such a cell is destroyed. This system is called acquired immunity.

The human immune system is formed before birth, and after birth it actively learns to recognize foreign agents, including pathogens. The first thing we can do to help a child’s immune system is to simply keep him in normal conditions, that is, if the child is healthy, if his immune system works normally, then he should have full contact with external environment, it does not need to be artificially limited.

- If you limit a child from contact with the environment in the hope that he will not get sick, how will this affect the immune system?

Badly. He will not live endlessly under a hood; sooner or later he will have to face the influence of the surrounding world: he will want to take a walk on the street, he will want to eat sand in the sandbox, and so on.

Most children go to kindergarten and school, where they are exposed to significant amounts of microorganisms carried by the people around them. How better baby prepared, that is, the better his immune system is familiar with external aggressors, the less he will get sick.

There is the concept of “immune memory” - this is the body’s ability to remember viruses in order to successfully repel their attacks the next time it encounters them. However, for some viruses the immune memory is short. For example, we get chickenpox once in our life, but we can get the flu a hundred times, because the virus changes quickly and the body does not remember it for a long time.

- It turns out that the older a person is, the better his immunity?

Unfortunately no. On the one hand, with age a person faces a large number of diseases, but on the other hand, the body ages, becomes decrepit, and the immune system along with it. In old age, a person’s immune system weakens; he cannot protect himself from diseases as before.

- So, with age, it becomes more and more difficult to improve immunity?

Look, what kind of regeneration does the child have? Everything heals on him like on a dog. For a teenager, everything is no longer so simple, for a 40-year-old it’s even worse, and for an 80-year-old it’s generally bad. This applies to all systems of the body: cardiovascular, nervous, and immune. A person who takes care of himself, makes his brain work and goes for walks, has a strong body and rarely gets sick. And an elderly, sedentary person who sits a lot in a confined space and is sick with something has a very weak immune system. Just blow on it and that's it. And try to kill the one who skis at 80 years old.

- Is it possible to effectively increase immunity and get sick less?

Increasing immunity is not boiling a kettle, and the opinion that immunity must be increased is not very true. Every intervention in such a complex mechanism as immunity must be justified.

Professor Andrei Petrovich Prodeus (who works at the ninth children's hospital) once conducted a study in six Moscow kindergartens. I don’t remember the exact numbers, but approximately 300 people took part. Before the start of the study, the Soviet system was restored in all kindergartens, in which a nurse worked at the entrance, who did not allow sick children to attend the kindergarten and sent them home with their parents. As a result of the experiment, the incidence of disease in the gardens was halved. Without the use of drugs and immune-improving biological food additives.

Often parents turn to an immunologist with a complaint that their child is constantly sick, for example, twice a month. But in fact, you shouldn’t get sick twice a month, because after fighting the infection, your immunity should be restored. If someone gets sick twice a month, then that's not two various diseases, and there is only one untreated.

The best thing I can advise is not to take sick children to childcare centers, and for adults to try not to suffer from colds on their feet. It’s also worth getting a dog or just imagining that you have one. In other words, go for walks in the morning and evening, and you will be healthy.

To boost immunity, many people drink immunomodulators, of which there are many types, but, unfortunately, the mechanism of the “magic” action of most of them has not been studied and their effectiveness has not been proven.

- Wait. What are immunomodulators?

An immunomodulator is a kind of “magic” smart remedy that regulates the immune system. However, the only modulators that can be used, in my opinion, are drugs containing parts of disease-causing organisms. These organisms are capable of causing an immune response, but they cannot cause disease. Essentially these are small vaccinations. If you follow the instructions and recommendations of an immunologist, treatment with such drugs often has a good effect.

If someone gets sick twice a month then these are not two different diseases, but one untreated

- What kind of small vaccinations?

You know, it is now forbidden to name commercial drugs everywhere. But I have already said that these are drugs created on immunogenic substances from commonly occurring microorganisms that cause infections.

- Are these small vaccinations prescribed in clinics?

They do not need to be prescribed; you do not need a prescription to purchase them. But a competent doctor, of course, can advise them.

- Do Actimel, Immunele and other similar drinks increase immunity?

These drinks are enriched with various beneficial microorganisms, without which we cannot exist. Once in the intestines, where we have many immune cells, they not only improve digestion, but also by complex mechanisms have a very mild positive effect on the immune system.

If before the race If a runner gets an immunogram, he will have the same blood parameters, but if he does at the finish line then the results will be similar to the results of a person with looking heavy immunodeficiency

- How many illnesses per year is considered normal for a resident of a metropolis? That is, to what extent should we not sound the alarm?

According to American standards, a child may suffer from uncomplicated respiratory viral infections 10–12 times a year. By our standards, it is good if a child gets sick no more than six times, and an adult even less.

But this depends on many risk factors: on where and how a person works (in a team or in a separate office), what type of transport and how often he uses it, and other things. For example, if you wear a fur coat on the subway in winter, and then run out into the cold with a wet back, then naturally you will catch a cold. In addition, in the metro there is a closed ventilation system, air circulation is limited, people inhale what they exhale, and then huge amount people. Someone sneezed, coughed - and everyone breathes it all. The same goes for working in a large team: it’s one thing when you’re sitting alone in an office or working at home, and another thing when you’re sitting in a team: someone came in with a cold - and everyone along the chain got sick.

- Why do we get sick more often in winter than in summer, although fewer viruses survive at low temperatures?

Yes, because we wear fur coats on the streets, and it’s hot in transport. Accordingly, our body tolerates temperature changes, and most people are not ready for this. In addition, few people are tempered.

Indeed, the influenza virus does not survive in extreme cold, but there are many other pathogens. In winter, we are faced with many troubles at once: wet weather, changes in atmospheric pressure or severe stress for a variety of reasons - it’s bad for the whole body, and the immune system is the hardest.

- But the immune system does not weaken?

The immune system does not weaken, but is subjected to greater stress. In winter it is cold, damp, people get sick more often. Moreover, if a person fell ill with one disease and has not yet recovered, and someone sneezed on him, then he can get sick again. This happens less often in summer because environment better conditions.

- Does stress experienced by a person affect the immune system?

Immunity is also affected by such simple things as nutrition, rest, and morale. Stress, of course, too. The most obvious example is the stress that athletes experience. For example, if a runner is given an immunogram before a race, he will have the same blood parameters, but if done at the finish line, the results will be similar to the results of a person with a severe form of immunodeficiency.

Emotions excite the cortex and other brain structures; the hypothalamic-pituitary system forces the adrenal cortex to produce more hormones that negatively affect lymphocytes (protective cells). Therefore, if you are tired or overexerted, your immune system has a hard time. But there is no need to swallow medications, including immunomodulators. If possible, you should just rest, calm down, eat well, get vitamins, trace elements and minerals. If you do not have congenital immunity disorders, if you are a healthy person, then this will be enough for the body to start working well again.

Sometimes it even happens that people do not get sick due to some kind of adverse impact, because they are tense and concentrated on some kind of activity: the child got sick - the mother was mobilized, and then the child recovered - the mother relaxed and got an infection. Because the immune system, which is under many influences, reacted incorrectly, internal regulation violated.

- Does taking vitamins affect immunity?

There should be enough vitamins, but primarily due to good nutrition. Naturally, there are times when immune cells there are not enough resources. For example, in the spring, after a long period without berries, fruits and sun, or in regions where there is a lot of meat and little grain, people have a lack of B vitamins. Or a person simply eats monotonous food out of habit - then there are not enough vitamins and there is a need to take additional artificial ones.

Vitamins do not have a direct effect on the immune system: I drank it and there were more lymphocytes. Vitamins have an indirect effect. That is, they help improve the functioning of other systems and organs - and the immune system also becomes easier.

Genetically determined disease does not necessarily manifest itself from birth, it can manifest itself in adulthood: at 15 years, and at 35, and at 70

- How to recognize immunodeficiency?

Looking for illnesses in yourself is a thankless task. Many people feel that their symptoms always correspond to the disease being described.

There are so-called warning signs that may indicate immunodeficiency. Among them, it is worth highlighting more than six otitis media per year, two sinusitis per year, skin problems, taking antibiotics does not help for more than two months, thrush, vaccination complications, developmental delays, micronodules, features of the facial structure, fevers, arthritis, and so on. If you have two signs from the list, then you need to make an appointment with an immunologist.

- What causes immunodeficiencies?

There are a lot of primary immunodeficiencies: these are congenital, genetically determined diseases. Currently, more than 350 forms have been described. Primary immunodeficiencies have different genetic origins and varying degrees of severity. Some are harmless, and some are absolutely incompatible with life; if left untreated, patients can live no more than 12–18 months. Therefore, undiagnosed immunodeficiency in time can lead to death. The overall incidence of primary immunodeficiencies is approximately 1:10,000, although among different forms fluctuates within very wide limits.

Despite the fact that primary immunodeficiencies are of a genetic nature, the disease does not necessarily manifest itself from birth; it can also appear in adulthood: at 15 years, and at 35, and at 70. This does not apply to all forms of primary immunodeficiencies, but only to several , for the majority late start- casuistry. Why this happens is not yet fully understood; genetic defects are also affected various factors which are called epigenetic. It is possible that there are other mechanisms that we have not yet recognized.

Secondary immunodeficiencies are not genetically determined; they are caused by exposure to certain factors: tumors, severe infections, tropical diseases, severe injuries and extensive burns. For example, a child falls ill with blood cancer (leukemia) - they begin to treat him with chemotherapy in order to kill him tumor cells, at the same time they kill non-tumor ones - secondary immunodeficiency develops. Unlike primary ones, secondary immunodeficiencies are transient, that is, after the end of exposure to unfavorable factors, the immune system gradually recovers itself.

- How is immunodeficiency treated?

There are forms that do not even need to be treated. And there are those who conservative treatment won't help. Then it is necessary to change the diseased immune system to a healthy one, that is, to carry out a transplantation of hematopoietic stem cells, from which a healthy immune system is formed. In many forms, if you prescribe the necessary therapy (administer immunoglobulin, use antibiotics and other anti-infective drugs as indicated), you can live like people without the disease.

Immune system diseases are among the most common forms of pathology in modern man. Of the large list of state and commercial medical institutions in St. Petersburg, only a very small part has all the necessary capabilities and highly qualified doctors for the most accurate diagnosis and effective treatment of diseases of the immune system. "Union Clinic" is one of these medical institutions, has extensive experience in the field of clinical immunology, well-deserved authority among professionals and patients.

Immune system Among all the life support systems of the body, it is one of the most significant, performing extremely important functions to protect the body from various foreign substances.

Such substances include:

  • infectious factors dangerous to the body (bacteria, viruses, fungi, protozoa);
  • modified cells that have arisen in the body (for example, tumor cells),
  • components of transplanted organs and tissues,
  • allergens.

All of the listed substances contain agents foreign to the body (antigens). It is antigens that, when they enter the body, cause activation of various parts of the immune system. The work of the immune system is carried out by special cells (granulocytes, macrophages, T- and B-lymphocytes) and organs of the immune system (bone marrow, thymus, spleen, lymph nodes, lymphoid tissue mucous membranes). As a result of the coordinated work of various components of the immune system, antigens are neutralized and safely eliminated from the body.

The most common type of immune system disorder is immunodeficiency.

Immunodeficiency - is a quantitative or functional deficiency of the main components of the immune system.

As a result decreased immune system activity in people with immunodeficiency, as a rule, at the most early stages development of this pathological process symptoms arise that are difficult to explain for the patient and for an inexperienced doctor, the so-called preclinical stage of the disease. At the same time, against the background of apparent general well-being in health, phenomena of rapid fatigue, poor tolerance of mental or physical stress may occur, and the so-called “syndrome” may develop. chronic fatigue" In young people who seem to be in good health, sexual desire often decreases, and in men, potency sometimes decreases. At the earliest stages of immunodeficiency in a number of patients, doctors note an unexplained increase in body weight, and in some cases, weight loss, disorder various types metabolism.

In later periods of the progressive development of an immunodeficiency state, chronic, often recurrent, sluggish viral, bacterial, and fungal infections that are not amenable to conventional therapy are characteristic. Frequency of occurrence, e.g. colds in adults suffering from immunodeficiency, may exceed 4 times a year. These same individuals, more often than people with a normally functioning immune system, develop cancer and develop allergic and autoimmune (i.e., immunoaggressive) diseases. It should be noted that clinical manifestations There are an extremely large number of immunodeficiency states; the pathology can affect any organs and physiological systems of a person, therefore, each patient, as a rule, develops his own unique symptom complex of the disease, the essence of which can only be unraveled by an experienced doctor.

Immunodeficiencies are divided into primary (hereditary) and secondary (acquired).

Primary immunodeficiencies arise long before the birth of a child and are associated with genetic defects in the development and maturation of one or more components of the immune system. They are congenital disorders of the immune system, often accompanied by congenital malformations of other body systems.

Secondary (acquired) immunodeficiencies develop into more late period childhood or adulthood and are not the result of genetic disorders. In quantitative terms, they occupy a dominant position among immunodeficiencies. Secondary immunodeficiencies can be caused by the failure of various components of immunity: humoral and cellular immunity, synthesis of complement components, insufficient activity of phagocytic cells, etc. Often the occurrence of secondary immunodeficiencies is associated with specific reason: x-ray irradiation, taking some medicines. Sometimes immunity disorders develop secondary to the underlying disease; subsequently, they aggravate its course, contribute to the formation of severe complications and adverse outcomes.

Diagnosis of immunodeficiency conditions must be comprehensive. It includes both clinical and laboratory methods (clinical blood test, assessment of immune status, cytokine profile, etc.), which can be performed at Union Clinic.

Clinical evidence of immunodeficiency may include:

  • frequent bacterial, viral, mycotic infections;
  • various lesions skin and visible mucous membranes (rashes, warts, genital warts, acne, etc.);
  • weight loss;
  • intestinal dysfunction (diarrhea, rumbling, bloating, intestinal dysbiosis, etc.);
  • presence of chronic inflammatory diseases (chronic bronchitis, tonsillitis, cholecystitis, prostatitis, cystitis, urethritis, nephritis, sinusitis)

One of the most important tasks of a doctor examining patients with immunodeficiency is to identify possible reasons who contributed to the development of this pathological condition.

The most common causes of immunodeficiency states are:

  • chronic recurrent infectious diseases;
  • anthropogenic factors (ecological deterioration, changes in soil composition, working with organic dyes and lead salts, electromagnetic radiation);
  • poor nutrition (for example, deficiency of proteins, vegetables and fruits containing antioxidants, vitamins, microelements);
  • chronic stress (physical or emotional);
  • unreasonably long-term use of drugs that affect the immune system ( hormonal drugs, immunomodulators, cytostatics). Self-medication;
  • operations performed on the organs of the immune system: removal of the palatine tonsils, thymus gland- thymus, spleen, appendix (appendix), etc.;
  • occupational hazards (contact with salts of heavy metals, electromagnetic radiation, radionuclides);
  • long-term and severe intestinal dysbiosis;
  • chronic intoxication (alcoholism, smoking, drug addiction);
  • diseases in close relatives (allergic and autoimmune diseases, cancer, congenital immunodeficiencies, short life expectancy, etc.).

The more of these factors are present, the greater the chance that the patient has an immunodeficiency condition that requires careful examination and correction.

Help in establishing the presence and clarifying the type of immunodeficiency is provided by special laboratory research methods that can be carried out at the Union Clinic.

The first idea about the state of the immune system can be obtained by assessing the results of a clinical blood test. A sign of immunodeficiency may be a decrease in the level of white blood cells - leukocytes, especially if it is caused by a decrease in the level of lymphocytes, which are active participants immune reactions.

However, a clinical blood test provides only indirect information. If a pathology of the immune system is suspected, a more in-depth laboratory study is required - an immunogram. This study, which makes it possible to more accurately specify and assess the state of immunity, is being successfully carried out at the Union Clinic.

Immune status (immunogram) is a blood test that examines components of the immune system. It takes into account the number of cells (T and B lymphocytes, macrophages, neutrophils), their percentage and functional activity, as well as the “substances” that these cells produce - immunoglobulins (Ig) classes A, M, G, E, components of the complement system. Sometimes “pathological antibodies” are determined in an immunogram - antinuclear factor, rheumatoid factor, antibodies to phospholipids and others.

A special immunological study - cytokine status allows for clinical and laboratory analysis of those regulatory systems that provide management and control over all aspects of the functioning of the immune system. Without performing this section of immunological diagnostics, examination of a patient with suspected and, especially, identified immunodeficiency cannot be considered complete and modern.

Interpretation of immunogram results, including cytokine status, is quite complex and should be carried out exclusively by a medical specialist. Assessment of immunological indicators allows the doctor to clarify in which part of the immune system the breakdown has occurred, ensures the accuracy of laboratory and clinical characteristics of immunodeficiency - the type and severity of the process and, of course, is the rationale for choosing a particular drug that affects the immune system. The type and severity of immunodeficiency determines the type of therapy.

A unique test for the presence of an immune-dependent form of food intolerance, as one of the common options immune disorders, is a blood test for the presence of antibodies to food allergens. It is at the Union Clinic that the above-mentioned diagnostic test has been held since 2002.

Immunomodulators called medicines, which in therapeutic doses restore the functions of the immune system (effective immune defense).

Once again, it must be emphasized that before starting immunomodulatory therapy, an assessment of the patient’s immune status is necessary. The same clinical picture in different patients may be due to defects in various parts of the immune system. The immunogram serves as the basis for starting immunocorrection and is used to assess its effectiveness.

The prescription of a particular drug that affects the immune system should only be carried out by a medical specialist. Unreasonable and uncontrolled prescription of a number of immunomodulators to frequently and long-term ill patients can provoke the development of a severe autoimmune process or “paralyze” the immune system for a long time.

The main points of application for immunomodulatory drugs are immunocompetent cells (macrophages, natural killer cells (NK), neutrophils, T and B lymphocytes), the processes of interaction of such cells or their products (antibodies, cytokines) with the corresponding targets.

General principles applications immunomodulators

1. Immunomodulators are used in combination with antibiotics, antifungals, antiprotozoals or antiviral agents, thereby delivering a “double blow” to infectious agent, and as monotherapy during immunorehabilitation measures.

  • It is advisable to prescribe immunomodulators early (from the first day of using a chemotherapeutic etiotropic agent).
  • The severity of the effect of immunomodulatory therapy during treatment in the acute period of the disease is greater than in the remission stage.
  • The effect of any immunomodulator is multifaceted: it is enough, for example, to activate macrophages, and their release of cytokines will set the entire immune system in motion.

2. A decrease in any one parameter of immunity identified when assessing the immune status of practically healthy person, is not necessarily a basis for prescribing immunomodulatory therapy. Dynamic monitoring of such a patient is indicated.

  • Immunomodulators do not affect unchanged parameters of the immune system.
  • It is advisable to use immunomodulators against the background of immunological monitoring.

It is important to note again that self-medication of immunodeficiency conditions is very dangerous. This can lead to severe irreversible health consequences. We invite everyone in need of highly qualified diagnostic and treatment assistance in the field of clinical immunology to the Union Clinic.

UNION CLINIC guarantees complete confidentiality of your request.

The human immune system is designed to respond in a timely manner to the invasion of foreign elements. Its correct functionality is to recognize the threat and destroy it. Primary immunodeficiency means that the child has not developed a protective mechanism against intrauterine development, or he didn't receive it by hereditary factor. As a result, harmful microorganisms entering his body will cause him maximum harm. The same can be said about atypical cells, which provide negative influence on the state of health, cause pathologies of varying severity.

It is necessary to distinguish between primary and secondary immunodeficiencies. Primary is determined in the baby shortly after birth. His body is deprived of the ability to protect itself from antigens and is susceptible to infectious invasion. This is expressed in the fact that the baby often gets sick, he is overcome by repeated illnesses, he finds it difficult to bear them, and gets complications. Severe forms of primary immunodeficiency lead to death in infancy.

There are rare cases where primary immune deficiency manifested itself in adults. This is possible, but for this a person must have high compensation for a certain type of disease.

The clinical picture of the disease is re-infection, the transition of diseases into a chronic form. What does primary immunodeficiency lead to:

  1. The patient suffers from bronchopulmonary anomalies.
  2. It affects the mucous membranes and skin.
  3. There are problems with the ENT organs.
  4. PIDS usually leads to lymphadenitis, abscesses, osteomyelitis, meningitis, and sepsis.
  5. Certain forms of primary immunodeficiency provoke allergies, autoimmune diseases, and the growth of malignant tumors.

Studying dysfunctions immune defense deals with immunology - the science of the development and formation of a protective mechanism that counteracts the penetration of antigens into the body and destroys cells damaged by harmful substances and microorganisms.

The earlier PIDS is diagnosed, the greater the child’s chances of surviving and continuing life in satisfactory health. Timely determination is important gene mutation, which makes it possible to decide on family planning.

Immunodeficiency is a persistent abnormality of the defense mechanism, which causes a malfunction in the immune response to the influence of antigens. This failure can be of four types:

  • age-related, that is, arising in childhood or old age;
  • acquired due to poor diet, lifestyle, medication, AIDS virus, etc.;
  • developed as a result of various infections;
  • congenital or primary ID.

PIDs are classified depending on the forms and severity of the disease. Primary immunodeficiencies include:

  • ID characterized by damage to several cellular complexes;
  • Reticular dysgenesis, in which stem cells are absent, dooms the newborn to death.
  • Severe combined ID is a hereditary disease caused by dysfunction of B and T lymphocytes.
  • DiGeorge syndrome - or thymus abnormalities, parathyroid glands- underdevelopment or absence of the thymus gland. As a result of the defect, T-lymphocytes are affected, congenital heart defects occur, and deformities in bone structure, the structure of the facial bones, kidney defects and central nervous system dysfunction.
  • Primary immunodeficiency caused by damage to B lymphocytes.
  • Disorders in myeloid cells causing chronic granulomatous disease (CGD) with an abnormality in oxygen metabolism. Defective production of active oxygen leads to chronic fungal and bacterial infections.
  • Defects in complex blood proteins that impair humoral defenses. The complement system may be missing several components.

Need to know! Cellular immunodeficiency is characterized by a deficiency of immunocompetent cells, which include lymphocytes, plasma cells, and macrophages. Humoral immunodeficiency means dysfunction in the production of antibodies.

Symptoms of primary immunodeficiencies

Primary immunodeficiencies are indicated by signs and symptoms. Studying clinical picture progress of the disease, the clinic’s doctors identify a type of immune deficiency. This is facilitated by examination, tests, and history taking to determine genetic pathology.

  1. Primary deficiencies of cellular immunity give rise to viral and fungal infections. Characteristic features repeated colds, severe acute respiratory viral infections, chickenpox, mumps, and frequent manifestations of herpes are considered. The patient suffers from thrush, inflammation of the lungs and gastrointestinal tract caused by fungi. Cellular immunodeficiency increases the risk of cancer and lymphoma.
  2. Insufficient humoral protection is provoked by bacterial infections. These are pneumonia, ulcers on the skin, erysipelas, staphylococcus, streptococcus.
  3. Insufficient level secretory immunoglobulin And it causes damage to the mucous membranes in the mouth, nose, eyes, intestines, and the bronchi suffer.
  4. Combined IDs are characterized by complications of viral and bacterial infections. Manifestations of this form of primary immunodeficiency are nonspecific - they are expressed in developmental defects, tumor processes, lymphoid tissues, thymus gland, megaloblastic anemia.
  5. Congenital neutropenia and dysfunction of granulocyte phagocytosis gives rise to bacterial inflammatory processes with ulcers, abscesses. The result may be sepsis.
  6. Complement-associating primary immunodeficiencies lead to bacterial infections, autoimmune diseases, as well as recurrent swelling on the body and limbs - hereditary angioedema(NAO).

Causes of primary immunodeficiencies

Dysfunctions of the immune system develop in the embryo inside the mother's womb. This process is influenced by various factors. Prenatal diagnosis shows a combination birth defects development of a fetus with immunodeficiency. The etiology of PIDs is based on three pathologies.

  1. Genetic mutations, meaning that changes have occurred in the genes on which immunocompetent cells perform their functions. That is, the process of cell development and differentiation is disrupted. The anomaly is inherited in an autosomal recessive manner, when both parents are carriers of the mutagen. Only a small number of mutations develop spontaneously or germinatively (in germ cells).
  2. A teratogenic factor is the influence of dangerous toxins on the embryo, leading to congenital primary immunodeficiency. ID is provoked by TORCH infections - cytomegalovirus, herpes, rubella, toxoplasmosis in pregnant women.
  3. Unclear etiology. Immune deficiency, the reason for which is not clear.

Such conditions include asymptomatic ID, which manifest themselves infectious complications in provocative situations. If even one of the elements of the defense mechanism undergoes an anomaly, then protective forces weaken, the patient becomes an object for the invasion of various infections.

Diagnosis of primary immune deficiency

Immunodeficiency conditions are identified by type, since primary ID is most often congenital, its type is determined in the first months or weeks. A visit to the doctor is required when frequent illnesses baby, colds, development of fungal, viral, bacterial infections. Anomalies in the development of a child may also depend on primary immunodeficiency. To solve the problem you need urgent diagnostics and immediate initiation of treatment.

The disease identification method includes the following procedures:

  • general examination, during which attention is paid to damage to the skin, mucous membranes, pustular processes, subcutaneous swelling of adipose tissue;
  • study leukocyte formula By general analysis blood, ID is indicated by the presence of leukopenia, neutropenia, agranulocytosis, and other disorders;
  • blood biochemistry shows dysgammaglobulinemia, the presence of uncharacteristic metabolites, indicating primary humoral ID;
  • specific study on the reactions of the immune system. Indicators of activity of immunocompetent cells are studied;
  • molecular genetic analysis - a method of gene sequencing for the type of mutation. This is a way to determine Bruton, DiGeorge, Duncan, and Wiskott-Aldrich syndromes.

The doctor differentiates immunodeficiency states from acquired secondary IDs arising from the influence of radiation, toxic substances, autoimmune diseases, and oncology. In adults, the diagnosis is difficult to make, since the signs are smoothed out and the symptoms are unclear.

Prenatal diagnosis

Determination of primary ID using chorionic villus biopsy is called prenatal identification of the form of the disease. In addition, the culture of cells of fetal fluid and fetal blood is being studied. This complex analyzes, which are indicated in cases where a mutagen is detected in parents.

But to identify X-linked heavy combined immunodeficiency this method gives an accurate result and also clarifies the diagnosis for primary ID syndromes, chronic granulomatosis, and other SCID conditions.

Treatment of primary immunodeficiencies

The different etiologies and pathogenesis of diseases do not allow us to develop a general method of treating pathology. In severe forms, therapeutic treatment not relevant, it brings only temporary relief, but death inevitable from complications of immunodeficiency. In these cases, only bone marrow or embryonic substance of the thymus transplantation helps.

The deficiency of cellular immunity is compensated by the use of specific colony-stimulating drugs. This is replacement immunotherapy with thymalin, tactivin, levamisole and other agents, the choice of which is made by the immunologist. Enzymepathies are corrected by enzymes and metabolites. A common drug in this series is biotin.

Dysglobulinemia (lack of humoral protection) is treated with immunoglobulin replacement, depending on the missing substances of this type. But the main obstacle to the progression of the disease is the prevention of infections. Moreover, vaccination of children with primary ID does not have an effect; it is dangerous.

Prognosis and prevention

With severe primary ID, the child is doomed; he dies in the first year of life. Other pathologies of the immune system are treated as described above. The main task of parents is timely access to a doctor and care for their children. The child should not be allowed to become infected with viral, bacterial, or fungal pathogens.

If you are planning to have a child and have problems with gene mutation, then consultation with an immunologist is mandatory. During pregnancy, you need to undergo prenatal diagnosis, protect yourself from infections and follow all doctor’s recommendations.

For patients with ID, it is important to maintain personal hygiene, care for the oral cavity, nasal mucosa, and eyes carefully, without damaging their integrity. Required balanced diet, avoiding contact with sick people during epidemics, drug prophylaxis infections.

Complications after immunodeficiency

Primary immunodeficiencies lead to serious complications. The consequences may result in the death of a person. Such conditions are considered sepsis, abscesses, pneumonia, and severe infections. Autoimmune diseases are possible when the immune system fails and destroys its own cells. Risk increases oncological diseases and imbalance of the gastrointestinal tract and cardiovascular system.

Conclusion

Primary immunodeficiency is not always a death sentence. You need to be constantly monitored by an immunologist; this will help you maintain a satisfactory quality of life and live a long time.

Immunodeficiency– this is a decrease in the functional activity of the main components of the immune system, leading to a disruption of the body’s defense against microbes, and manifested in increased infectious morbidity.

IN modern world In a metropolis, an immunodeficiency state can develop in any person. The danger of this condition lies in its untimely recognition and treatment, which leads to severe infections, autoimmune diseases and oncological processes.

Immunodeficiency conditions are divided into congenital and acquired or secondary (SID). Basically we meet with secondary immunodeficiencies, and each of us has experienced this condition at least once in our lives. SID refers to disorders of the immune system that develop in old age and, as is commonly believed, are not the result of any genetic defect.

Forms VIEW

Form

Clinical factors

Acquired

Acquired immunodeficiency syndrome

Induced

Reason: radiation, cytostatics, corticosteroids, surgical interventions, injuries, etc.

Spontaneous

Chronic, recurrent, infectious and inflammatory processes of the bronchopulmonary apparatus, paranasal paranasal sinuses, urogenital and gastrointestinal tracts, eyes, skin and soft tissues, caused by opportunistic, opportunistic microorganisms with atypical biological properties and often with multiple antibiotic resistance


Signs VIEW

Signs of VID by which a doctor or the patient himself may suspect an immunodeficiency state

1. Recurrent virus-bacterial infections, characterized by:

  • chronic course;
  • incomplete recovery;
  • unstable remission;
  • unusual pathogens ( opportunistic flora, an opportunistic infection with reduced virulence, with multiple antibiotic resistance).

2. Age, presence of blood relatives with primary immunodeficiency;

3. Unusual reactions to live, attenuated vaccines;

4. Upon examination, the patient may have developmental insufficiency or developmental delay, chronic diarrhea, low-grade fever, increase or complete absence lymph nodes of the tonsils, thymus, skin abscesses, dermatitis, mucosal candidiasis, congenital malformation, impaired development of the facial skull, short stature (dwarfism), increased fatigue;

5. Iatrogenic interventions: chemotherapy, splenectomy, radiation;

6. Prolonged physical and/or psycho-emotional stress;

7. Allergy;

8. Autoimmune diseases;

9. Tumors.

Objectives of immunological research

  • confirm the presence of immunodeficiency;
  • determine the severity of violations;
  • identify the broken link;
  • evaluate the possibilities of selecting an immunocorrector;
  • evaluate the prognosis of the effectiveness of immunotherapy.

Immunotherapy

After a full immunostudy, the immunologist prescribes therapy.

Immunotherapy (correction of immunity)- treatment aimed at strengthening weakened immune defenses, correcting imbalances in ongoing immune reactions, weakening pathologically active immune processes and suppressing auto-aggressive immune reactions. Not all types of immune defense are effective against a specific infectious agent, but only some.

It is necessary to stimulate those parts of the immune system that are effective in protecting against a specific infection that the patient has.