共济失调毛细血管扩张症。培养的皮肤成纤维细胞放射敏感性评估作为一种诊断测试。
Ataxia telangiectasia. Evaluation of radiosensitivity in cultured skin fibroblasts as a diagnostic test.
作者信息
Cox R, Hosking G P, Wilson J
出版信息
Arch Dis Child. 1978 May;53(5):386-90. doi: 10.1136/adc.53.5.386.
Abstract
The sensitivity to x-ray inactivation of cultured skin fibroblasts from clinically confirmed and suspected cases of ataxia telangiectasia was compared with that of cultures from normal subjects. The results confirm previous observations of an association between ataxia telangiectasia and enhanced in vitro radiosensitivity, and also suggest that clonal survival of x-irradiated cultures of skin fibroblasts is a valuable technique for confirming the diagnosis of the disease.
摘要
将临床确诊和疑似毛细血管扩张性共济失调病例的培养皮肤成纤维细胞对X射线灭活的敏感性与正常受试者的培养细胞进行了比较。结果证实了先前关于毛细血管扩张性共济失调与体外放射敏感性增强之间关联的观察结果，并且还表明，皮肤成纤维细胞经X射线照射后的克隆存活是确诊该疾病的一项有价值的技术。
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