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What is X-Linked Agammaglobulinemia?

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  • Written By: J.L. Drede
  • Edited By: Jacob Harkins
  • Last Modified Date: 10 September 2016
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X-linked agammaglobulinemia is a rare genetic disorder that affects the immune system, making it difficult for a person to fight infection. Many different names are assigned to the disorder such as x-linked hypogammaglobulinemia, Bruton type agammaglobulinemia, Bruton syndrome and sex-linked agammaglobulinemia. The abbreviated name of the disorder is XLA. It is only found in the x-chromosome, which are only found in men.

X-linked agammaglobulinemia prevents the production of antibodies that are usually found in the gamma globulins of blood plasma. These antibodies are the body's main line of defense against bacteria, viruses and other forms of infection or disease. This is happens because people with XLA cannot create mature b-lymphocytes, which are the cells that create the antibodies found in the blood. The disorder is usually discovered in males within the first two years of their life, as they are constantly getting sick from infections that their bodies should be able to fight off easily. Many times it takes a serious conditions such as meningitis, sepsis or pneumonia for a diagnosis of x-linked agammaglobulinemia to be made.

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In addition to repeated infections there are other symptoms of x-linked agammaglobulinemia. These include a lack of tonsils and adenoids, stunted growth, joint disease, and inflammation of the kidneys and muscles. Since many of these symptoms are also symptoms of other, more common, disorders and diseases, a diagnosis of XLA may not be immediately evident. But the sooner the disease is identified in a child the better. Undiagnosed or untreated cases of x-linked agammaglobulinemia almost always end in death from infection.

Once a diagnoses of x-linked agammaglobulinemia is made, treatment that can manage the disorder can begin. This usually involves using gammaglobulin antibodies via an injection or infusion every two to four weeks. Since the disorder never goes away, this treatment must continue for the rest of the person's life.

In addition to the scheduled injections, men with XLA will often additionally use antibiotics to help fight off any infections that may occur before they become life-threatening. Even with the injections and regular medication, side effects of XLA are common and can be debilitating. They including chronic lung disease and sinusitis; bowel disease and recurring enteroviral infections. Each of these side effects are usually treated with additional antibiotics. If caught early and treated regularly, people with x-linked agammaglobulinemia can usually lead normal lives.

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