伴有免疫缺陷的共济失调性毛细血管扩张症又名Lowis-Bar征群,是一种常染色体隐性遗传病。近年来我国亦有报道,但1家8例均为此病患者(附图)较为罕见。现报告如下。先证者(Ⅳ_1),女,12岁。于6岁后经常有咳嗽、发热,几乎每月8～4次。自9岁起出现步态蹒 Associated with immunodeficiency ataxia telangiectasia, also known as Lowis-Bar syndrome, is an autosomal recessive disease. In recent years, there are also reports in our country, but one of the eight cases of patients with this disease (with photos) is relatively rare. The report is as follows. The proband (Ⅳ_1), female, 12 years old. After 6 years of age often cough, fever, almost 8 to 4 times per month. Since 9 years old gait staggering