immunodeficiency disease, severe combined
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immunodeficiency disease, severe combined,
Any of a group of inherited autosomal or X-linked recessive disorders in which there is partial or complete dysfunction of the immune system. Defects are present in both B- and T-cell–mediated immunity responses and frequently include defective cytokine function. Within 6 months after birth, babies develop infections from bacterial, viral, fungal, or protozoan organisms. Intravenous immune globulin (IVIG) is given to provide antibodies, but a successful bone marrow transplant is required to prevent death. The efficacy of gene therapy is under investigation.See: cytokine; cell-mediated immunity; humoral immunity