原发性胸腺发育不良是原发性免疫缺陷病的一种类型。我院经尸解证实胸腺发育不良6例,现报告如下。 临床资料 男性2例、女性4例。年龄最小7天,最大18月。发病日期最短为生后6天,最长2月。临床表现为出生后反复感染迁延不愈,本组4例因反复感染未能控制而死于败血症,1例小头畸形死于新生儿肺炎,另1例腹泻反复2月,死于鼠伤寒沙门氏菌感染。本组除1例合并小头畸形外,余均无合并其他先天性畸形,生后亦无手脚搐搦出现。胸片检查:有X线摄片3例, Primary thymic dysplasia is a type of primary immunodeficiency disorder. Our hospital confirmed by autopsy thymus dysplasia in 6 cases, are as follows. Clinical data of 2 males and 4 females. The youngest 7 days, maximum 18 months. The shortest onset date is 6 days after birth, the longest in February. Clinical manifestations of repeated infection after birth delay unhealed, the group of 4 patients died of sepsis due to repeated infection failed to control, a case of microcephaly died of neonatal pneumonia, another case of diarrhea repeatedly in February, died of Salmonella typhimurium infection. In addition to a group of patients with small head deformity, I have no other congenital malformations, after birth, no hands and feet convulsions appear. Chest X-ray examination: X-ray 3 cases,