congenital hypogammaglobulinemia


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Related to congenital hypogammaglobulinemia: Congenital hypothyroidism

congenital hypogammaglobulinemia

Etymology: L, congenitus, born with; Gk, hypo, deficiency, gamma, third letter of Greek alphabet; L, globus, small globe, haima, blood
a genetic disease characterized by a deficiency of gamma globulin and antibody in the serum. The cause may be a genetic defect leading to a failure of development of a normal beta-lymphocyte system and immune responses.

congenital hypogammaglobulinemia

(1) Familial hypogammaglobulinemia, OMIM:240500. 
(2) Congenital agammaglobulinaemia(s), see there.

congenital hypogammaglobulinemia

Total immunoglobulin levels that are below 250 mg/dl. Chronic bacterial infections are common. Administration of intravenous immune globulin (beginning at 200 mg/kg per month) is usually effective.
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