|
|
|
| |
|
|
|
|
|
|
| Definition: | An inherited disorder characterized by very low levels of protective immunoglobulins; affected people develop repeated infections.
|
| Alternative Names: | Bruton's agammaglobulinemia; X-linked agammaglobulinemia
|
| Causes, incidence, and 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.
|
|
|
Review Date: 12/1/2001
Reviewed By: Frederic F. Little, M.D., Department of Allergy and Pulmonary/Critical Care Medicine, Boston University School of Medicine, Boston, MA. Review provided by VeriMed Healthcare Network.
|
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2003 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
|
|
