genetic immunodeficiency disorders

genetic immunodeficiency disorders

Inherited disorders that affect the function of the immune system. Also known as primary immunodeficiencies. X-linked immunodeficiency disorders include some forms of the severe combined immunodeficiencies (SCID); Wiskott-Aldrich syndrome; X-linked hypogammaglobulinaemia; X-linked agammaglobulinaemia; chronic granulomatous disease; and the X-linked lymphoproliferative syndrome. Autosomal recessive immunodeficiency disorders include other forms of SCID; Di George syndrome; bare lymphocyte syndrome (MHC deficiency); ataxia telangiectasia; and selective immunoglobulin G deficiency.
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Mycobacterial, fungal and other opportunistic infections force the clinician to rule out a large list of conditions associated with secondary immunodeficiency as infectious agents (HIV, Herpesvirus, HTLV), drugs (steroids, immunosuppressants, biologics, chemotherapy), metabolic diseases (diabetes, renal failure, cirrhosis), malignancies (leukemia, lymphomas and solid tumors) and environmental conditions (radiation, heavy metals) (2) but even adult patients can have late onset primary genetic immunodeficiency disorders as chronic granulomatous disease, X-linked agammaglobulinemia, interleukin-12 receptor deficiency or interferongamma (IFN-?

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