原发性胸腺发育不全或胸腺缺如是原发性免疫缺陷病的一种类型,亦是重症联合免疫缺陷病(SCID)的主要病因,伴有细胞免疫和体液免疫双重缺陷的综合症,该症患儿免疫器官往往发育不良或不发育。我院经尸解证实胸腺缺如患者——先天性无胸腺症5例报道如下。 Primary thymus hypothyroidism or lack of thymus is a type of primary immunodeficiency disorder and is also a major cause of severe combined immunodeficiency disease (SCID), a syndrome associated with dual defects of cellular immunity and humoral immunity. This disease Children with immune organs are often poorly developed or not. Our hospital confirmed by autopsy thymus absence of patients - 5 cases of congenital athymic report as follows.