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Agammaglobulinemia
Alternate Names : Bruton's Agammaglobulinemia, X-Linked Agammaglobulinemia
DefinitionAgammaglobulinemia is an inherited disorder characterized by very low levels of protective immunoglobulins; affected people develop repeated infections.
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Overview, Causes, & Risk Factors
Agammaglobulinemia is rare disorder that only affects male as it is inherited as an X-linked recessive trait (like hemophilia). It is the result of a genetic abnormality that blocks normal B-lymphocyte differentiation (development into normal mature cell). As a consequence, the body produces very little (if any) gamma globulin (IgG) and other immunoglobulins in the bloodstream. Immunoglobulins are molecules that play a significant role in the immune response, which protects against illness and infection.
In the absence of protective immunoglobulins, affected individuals develop repeated infections. People with this disorder are particularly susceptible to bacterial infections caused by Hemophilus influenzae, pneumococcus (Streptococcus pneumoniae), and staphylococci as well as repeated viral infections. The upper respiratory tract, lungs, and skin are common sites of infection.
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Definition Agammaglobulinemia Overview, Causes, & Risk Factors Agammaglobulinemia Symptoms & Signs Agammaglobulinemia Prevention Agammaglobulinemia Diagnosis & Tests Agammaglobulinemia Treatment Agammaglobulinemia Prognosis Agammaglobulinemia Complications Calling Your Health Care Provider
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Topics that might be of interest to you Diseases & Conditions
Autoimmune Disorders Bronchitis Conjunctivitis Eczema (Atopic Dermatitis) Immunodeficiency Disorders Lung Disease Malabsorption Pneumonia Sinusitis
Tests & Exams
Immunoelectrophoresis - Serum Quantitative Immunoglobulins - Nephelometry
Other Topics
Antibody Chronic Diarrhea Immune Response Respiratory Titer
Review Date : 10/30/2003
Reviewed By : Donald Accetta, MD, MPH. President, Allergy & Asthma Care, PC, Taunton, MA. Review provided by VeriMed Healthcare Network.
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