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Pediatric Agammaglobulinemia

What is X-linked agammaglobulinemia?

X-linked agammaglobulinemia, also called Bruton's agammaglobulinemia or congenital agammaglobulinemia, was the first immunodeficiency disease ever identified. "X-linked" means that the gene which causes this agammaglobulinemia is located on the X chromosome, and therefore primarily affects males because it is unlikely that females will have two altered copies of the gene. The disease causes the child to be unable to produce antibodies that make up gamma globulins in the plasma portion of blood. Antibodies are the body's primary defense against microorganisms (bacteria, viruses). In X-linked agammaglobulinemia, there is a failure of pre-B-lymphocytes to mature into B-lymphocytes (mature B-lymphocytes produce antibodies). As a result, there are no antibodies produced, and the child's body is unable to fight off bacterial infections and some viral infections.

This relatively rare inherited disease causes affected boys to become very ill since they are prone to develop infections in the middle ear, sinuses, and lungs. The infections can also involve the bloodstream or internal organs. With new advances in treatment, most patients diagnosed and treated early are able to lead relatively normal lives, without the need for isolation from potential exposure to microorganisms. In fact, children are encouraged to lead active lives.

Departments

Departments

Allergy and Immunology

At Children’s National, children affected by allergies and complex immunologic disorders benefit from our specialists’ expertise, because our doctors treat children and only children with these conditions.

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