Hypogammaglobulinemia Idiopathic

Idiopathic hypogammoglobinemia (HI) is a group of hereditary and acquired immunodeficiency conditions characterized by a progressive impairment of the production of antibodies of classes IgG, A, M, G and accompanied by clinical symptoms of varying severity. The disease is characterized by the predominance of pathological changes in T lymphocytes [1]. This disease can be caused by a variety of causes, including mutations in immune response genes, viral infections, medications, and even certain foods.

SYMPTOMS

Symptoms of HI can begin at any age, but most often appear before the age of four. Patients may have the following symptoms:

A child in the first year of life may experience a constant cough, nasal congestion, frequent respiratory infections and fever. In some cases, the child appears sick. Hypogammoglakinemia leads to a lack of hygiene skills. Concentration decreases, interest in the outside world and relatives disappears. Neurological signs and emotional instability of the child develop. The clinical picture of the disease is characterized by a decrease or absence of serum immunoglobulin production. The patient has common, indolent infections that are difficult to treat: purulent lymphadenitis, sinusitis, pyelonephritis, etc. A decrease in the humoral immunity aggravates the course of many chronic diseases. Patients develop fungal and microbial microflora that are resistant to standard antibiotics. A severe manifestation of prematurity is a generalized intestinal infection. Patients with HI present with symptoms of hyperimmune globulin, such as frequent malaise, loss of appetite, sleep disturbance and severe fatigue, expressed at the onset of the disease. The child has frequent respiratory infections, as well as tonsillitis and pneumonia. In more severe cases, bronchial asthma may develop.

Hypogammaluminia Idiopathy

Hypogammoluminia is a rare inherited immune system disorder that can have serious health consequences for the affected person. This disease is characterized by a deficiency of IgG, the main group of immunoglobulins in the human body. Without sufficient immunoglobulin, the immune system's protective functions are weakened, increasing the risk of infection by dangerous bacteria, viruses and fungi.

This article describes hypogamy as a disease and explains its symptoms and causes. In addition, the article also focuses on the diagnosis and treatment of this disease. Normally, in a healthy person, the IgG level should be between 7.5 g/l and 14 g/l. In some patients, immunoglobulin levels may drop even below this norm. The most common form of hypogammoblaimunia is primary hypogammoblaimunia, in which the level of all types of immunoglobulins is reduced. Usually in the initial stages this does not manifest itself in any way, but then it leads to frequent colds and diseases of the upper respiratory tract, such as sinusitis

Hypogammoglobulimia (HTG) is a hereditary immunodeficiency disease associated with a lack of gammaglobulin (γ-globulin) in the blood. This protein is part of the immune system and is responsible for protecting the body from infections. HTG can manifest itself in both children and adults in the form of various symptoms, including frequent bacterial and viral infections, anemia, digestive problems, etc.

When a child is born with hypogammactia, their immune system is not able to effectively fight infections. Because of this, the child gets sick more often and is forced to spend more time in the hospital and take additional medications for infections, sometimes stronger than for ordinary patients. There is also a high risk of surgical infections due to a decrease in the level of immune defense. In such cases, the child is vaccinated against common infectious diseases to protect him from them. Treatment for HTG includes immunoglobulin, as well as antibiotics if necessary.

Also, in addition to treatment, the child should receive additional nutrition with a high protein content (high-protein diet) and vitamins to maintain immunity, and parents should take care of hygiene and infection prevention. This helps reduce the risk of complications and speed up the healing process.

Hypogammaglutinia is a decrease in the blood levels of IgG, IgA, IgG3 and/or IgA2 by more than one step, but not less than 0.8 g/l. The following clinical variants of G. and. are distinguished:

1. Hidden deficiency (hypogammalucans), when the content of all classes of immunoglobulins corresponds to reference values, but when studying the level of opsonins in the serum, there is a significant impairment of phagocytosis, accompanied by hyperinflammatory reactions;

2. Transient form, developing in children and young people and manifesting clinically several weeks or months after acute infection;

3. Local form, localized mainly in regional lymph nodes;

4. Generalized form, characterized by impaired immunoreactivity in all lymphoid organs;

5. Iatrogenic form, caused by massive administration of large doses of plasma immunoglobulin to women for the prevention of bone marrow transplant GVHD in pregnant women in the presence of insufficient immune response syndrome. There are 2 types of iatrogenic forms: with the presence of isolated IgM deficiency and with developed G. in combination with other defects of humoral immunity.

The classification of HS is general and does not include its variants in the hereditary form, the genetic features of which can be reflected when formulating the diagnosis. The clinical course of G. can also be complicated by the presence of general disorders of the blood coagulation system (hemolytic anemia, thrombocytopenia, leukopenia), a decrease in the level of T-lymphocytes in the peripheral blood or a violation of their functions (increased level of Th8+, instability of the IL-2 receptor with a normal number of T- lymphocytoch).

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