Agammaglobulinemia, aka – (Bruton’s Disease, X-linked agammaglobulinemia (XLA), Bruton’s agammaglobulinemia)
Bruton’s disease a hereditary disease in which a severe primary immunodeficiency develops (a defect in the body’s immune defense) with a pronounced decrease in the level of gamma globulins in the blood. The disease usually manifests itself in the first months and years of a child’s life, when repeated bacterial infections begin to develop: otitis media, sinusitis, pneumonia, pyoderma, meningitis, and sepsis. When examined in peripheral blood and bone marrow, serum immunoglobulins and B-cells are practically absent. Treatment for agammaglobulinemia is lifelong replacement therapy.
Disease Classification: Immunodeficiencies with predominant antibody deficiency.
Agammaglobulinemia (hereditary hypogammaglobulinemia, Bruton’s disease) is a congenital defect in humoral immunity caused by mutations in the cell genome, which leads to the insufficient synthesis of B-lymphocytes. As a result, the formation of immunoglobulins of all classes is disrupted, and their content in the blood decreases sharply until it is completely absent, and primary immunodeficiency develops.
The low reactivity of the immune system leads to the development of severe recurrent purulent-inflammatory diseases of the upper respiratory tract, bronchi, and lungs, gastrointestinal tract, and meninges. Bruton’s disease occurs exclusively in boys and occurs in about 1-5 out of a million newborns, regardless of race or ethnic group.
Simplified Symptoms: Water Diarrhea, High Body Temperature, Headache, Cough, Dyspnea, and Chills.
Detailed Symptoms: Symptoms of agammaglobulinemia Reduced reactivity of humoral immunity in agammaglobulinemia leads to the development of repeated purulent-inflammatory diseases already in the first year of a child’s life (usually after cessation of breastfeeding – at 6-8 months). At the same time, protective antibodies from the mother no longer enter the child’s body, and their own immunoglobulins are not produced.
By the age of 3-4 years, inflammatory processes become chronic with a tendency to generalization. Purulent infection with agammaglobulinemia can affect various organs and systems. On the part of the ENT organs, purulent sinusitis, ethmoiditis, and otitis media are not uncommon, and purulent otitis media often develops in the first year of a child’s life, and sinusitis – in 3-5 years.
Of the diseases of the bronchopulmonary system, repeated bronchitis, pneumonia, and lung abscesses are observed. Often there is a lesion of the gastrointestinal tract with persistent diarrhea (diarrhea) caused by chronic infectious enterocolitis (the main pathogens are campylobacter, giardia, and rotavirus). Impetigo, microbial eczema, recurrent furunculosis, abscesses, and phlegmon are found on the skin.
Often there is damage to the eyes ( purulent conjunctivitis ), oral cavity ( ulcerative stomatitis, gingivitis ), and musculoskeletal system ( osteomyelitis, purulent arthritis ). In general, the clinical picture of agammaglobulinemia is characterized by a combination of general symptoms observed during a purulent infection (high body temperature, chills, muscle pain and headache, general weakness, sleep and appetite disturbance, etc.) and signs of damage to a specific organ (cough, shortness of breath, difficulty in nasal breathing, purulent discharge, diarrhea, etc.).
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