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Hyper-IgM Immunodeficiency Syndrome: A rare inherited immunodeficiency syndrome characterized by normal or elevated serum Immunoglobulin m levels with absence of Immunoglobulin g; Immunoglobulin a; and Immunoglobulin e. It results in a profound susceptibility to Bacterial infections and an increased susceptibility to Opportunistic infections. Several subtypes of hyper-IgM immunodeficiency syndrome exist depending upon the location of genetic mutation.
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