107P Severe Combined Immunodeficiency, Wiskott Aldrich Syndrome

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107P Severe Combined Immunodeficiency, Wiskott Aldrich Syndrome

health admin June 12th. 2024, 8:47pm

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Severe combined immunodeficiency (SCID) represents a constellation of genetically distinct syndromes with the common feature of defects in both humoral and cell-mediated immune responses. Affected infants are susceptible to severe recurrent infections by a wide array of pathogens, including bacteria, viruses, fungi, and protozoans; opportunistic infections by Candida, Pneumocystis, CMV, and Pseudomonas also cause serious (and occasionally lethal) disease.

Wiskott-Aldrich syndrome is an X-linked recessive disease characterized by thrombocytopenia, eczema, and a marked vulnerability to recurrent infection, ending in early death; the only treatment is bone marrow transplantation.

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