共济失调毛细血管扩张症(Ataxia-telangi-ectasia简称AT)又称Louis-Bar综合征,是累及神经、血管、皮肤、网状内皮系统、内分泌等的原发性免疫缺陷病,也是一种染色体不稳定综合征,亦有人将之归为DNA修复缺陷病,属常染色体隐性遗传。国外统计本病发病率约 Ataxia-telangi-ectasia (AT), also called Louis-Bar syndrome, is a kind of primary immunodeficiency disease involving nerves, blood vessels, skin, reticuloendothelial system, endocrine and so on Chromosomal instability syndrome, some people will be classified as DNA repair defects, autosomal recessive inheritance. Foreign statistics about the incidence of this disease