Agammaglobulinemia: Total or near-total absence of gamma globulin, a protein fraction of the blood rich in antibodies, due to certain genetic diseases such as Bruton agammaglobulinemia or to acquired diseases such as HIV / AIDS.
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Total or near-total absence of gamma globulin, a protein fraction of the blood rich in antibodies, due to certain genetic diseases such as Bruton agammaglobulinemia or to acquired diseases such as HIV/AIDS.
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Causes Agammaglobulinemia is a rare disorder that mainly affects males. It is the result of a genetic abnormality that blocks the development of normal, mature immune system cells called B lymphocytes. As a result, the body produces very little (if any) immunoglobulins in the bloodstream. Immunoglobulins play a major role in the immune response , which protects against illness and infection. Without protective immunoglobulins, people with agammaglobulinemia repeatedly develop infections. People
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Symptoms Symptoms include frequent episodes of: Bronchitis ; Chronic diarrhea ; Conjunctivitis (eye infection); Otitis media (middle ear infection); Pneumonia ; Sinusitis ; Skin infections; Upper respiratory tract infections; Infections typically appear in the first 4 years of life. Other symptoms include: Bronchiectasis (a disease in which the small air sacs in the lungs become damaged and enlarged); Unexplained asthma ;
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Treatment The goal of treatment is to reduce the number and severity of infections, and to provide genetic counseling to affected families. Receiving immunoglobulins (IVIG) through a vein (intravenously) helps boost the immune system by providing the body with the antibodies that are decreased or missing. Routine treatment with IVIG is central to the treatment of this disorder. Antibiotics are often needed to treat bacterial infections.
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