Severe combined immunodeficiency
Genetic disorder leading to severe impairment of the immune system
Severe combined immunodeficiency
Severe combined immunodeficiencies (SCIDs) are a rare group of genetic disorders characterized by the body's inability to create functional T cells and B cells. SCIDs are the most severe form of primary immunodeficiency, making their sufferers extremely vulnerable to infection due to their highly compromised immune system. There are at least seven different known genes in which mutations lead to a form of SCID, each of which has different clinical presentations. SCID involves defective antibody response due to either direct involvement with B lymphocytes or through improper B lymphocyte activation due to non-functional T-helper cells. Consequently, both "arms" (B cells and T cells) of the adaptive immune system are impaired due to a defect in one of several possible genes. Some SCID sufferers, such as David Vetter, have become famous for living in a sterile environment to avoid infection, leading to the name bubble boy disease and bubble baby disease. SCID patients are usually affected...
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