X-linked hypogammaglobulinemia


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hypogammaglobulinemia

 [hi″po-gam″ah-glob″u-lin-e´me-ah]
abnormally low levels of all classes of immunoglobulins, associated with heightened susceptibility to infectious diseases; see also agammaglobulinemia, dysglobulinemia, and immunodeficiency.
common variable hypogammaglobulinemia common variable immunodeficiency.
physiologic hypogammaglobulinemia a normal period of hypogammaglobulinemia seen in all infants at about 5–6 months of age as the level of transplacentally acquired maternal immunoglobulins declines before endogenous immunoglobulin synthesis rises to normal levels.
transient hypogammaglobulinemia of infancy prolongation of the normal physiologic hypogammaglobulinemia of infancy caused by delayed development of endogenous immunoglobulin production and associated with increased susceptibility to infections.
X-linked hypogammaglobulinemia X-linked agammaglobulinemia.

X-linked hy·po·gam·ma·glob·u·lin·e·mi·a

, X-linked infantile hypogammaglobulinemia
a congenital, primary immunodeficiency characterized by decreased numbers (or absence) of circulating B lymphocytes with corresponding decrease in immunoglobulins of the five classes; associated with marked susceptibility to infection by pyogenic bacteria (notably, pneumococci and Haemophilus influenzae) beginning after loss of maternal antibodies; X-linked recessive inheritance caused by mutation in the Bruton tyrosine kinase gene (BTK) on Xq.

X-link·ed hy·po·gam·ma·glob·u·lin·e·mi·a

, X-linked infantile hypogammaglobulinemia (lingkt hī'pō-gam'ă-glob'yū-li-nē'mē-ă, in'făn-tīl)
A congenital, X-linked recessive, primary immunodeficiency characterized by decreased numbers (or absence) of circulating B-lymphocytes with corresponding decrease in immunoglobulins; associated with marked susceptibility to infection by pyogenic bacteria after loss of maternal antibodies.
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