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单词 Severe Combined Immunodeficiency Disease
释义

Severe Combined Immunodeficiency Disease

中文百科

严重复合型免疫缺乏症 Severe combined immunodeficiency

(重定向自Severe Combined Immunodeficiency Disease)

严重复合型免疫缺乏症(Severe combined immunodeficiency,缩写 SCID),泛指一群罕见的先天遗传性疾病,患者因为免疫功能缺乏,导致严重的重复性感染,来源包括细菌、病毒及霉菌。若未治疗,多数在出生后一年内死亡。

英语百科

Severe combined immunodeficiency 严重复合型免疫缺乏症

(重定向自Severe Combined Immunodeficiency Disease)

Severe combined immunodeficiency, SCID, also known as alymphocytosis, Glanzmann–Riniker syndrome, severe mixed immunodeficiency syndrome, and thymic alymphoplasia, is a genetic disorder characterized by the disturbed development of functional T cells and B cells caused by numerous genetic mutations that result in heterogeneous 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. SCID is the most severe form of primary immunodeficiencies, and there are now at least nine different known genes in which mutations lead to a form of SCID. It is also known as the bubble baby disease and bubble boy disease because its victims are extremely vulnerable to infectious diseases and some of them, such as David Vetter, have become famous for living in a sterile environment. SCID is the result of an immune system so highly compromised that it is considered almost absent.

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