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• W3031472049 endingPage "268" @default.
• W3031472049 startingPage "207" @default.
• W3031472049 abstract "Abstract Combined immune deficiencies (CIDs) form a group of inherited immune disorders in which both T cell and B cell functions are compromised. Lymphocyte numbers may also be diminished. These disorders affect primarily T cells, typically disturbing their ability to provide assistance for antibody production and therefore causing decreased immunoglobulins. The disorders may also directly affect B cells and/or natural killer (NK) cells, leading to diminished responses to vaccines and pathogen killing. Some CID are caused by variants in genes that are known to cause severe combined immunodeficiency (SCID) but have a hypomorphic effect. In this case, peripheral T cells are produced in greater numbers than in SCID due to less severe – i.e., hypomorphic – mutations that permit partial or full mature T cell egress from the thymus. Alternatively, defects in T cell function can occur at various stages of T cell receptor (TCR) signaling or cytokine responsiveness of mature T cells. Because B cells and NK cells have evolved similar signaling pathways, defects in these response mechanisms can additionally impair B and/or NK cell functions." @default.
• W3031472049 created "2020-06-05" @default.
• W3031472049 creator A5014247790 @default.
• W3031472049 creator A5061668145 @default.
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• W3031472049 creator A5071545468 @default.
• W3031472049 creator A5078457902 @default.
• W3031472049 creator A5079505238 @default.
• W3031472049 date "2020-01-01" @default.
• W3031472049 modified "2023-09-27" @default.
• W3031472049 title "Combined immune deficiencies (CIDs)" @default.
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