ATAXIA TELANGIECTASIA - NEOPLASIA UNTOWARD RESPONSE TO X-IRRADIATION AND TUBEROUS SCLEROSIS
ATAXIA TELANGIECTASIA - NEOPLASIA UNTOWARD RESPONSE TO X-IRRADIATION AND TUBEROUS SCLEROSIS
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DOI：
10.1001/archpedi.1967.02090270073006
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发表时间：
1967-01-01
影响因子：
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通讯作者：
LIEBNER, EJ
中科院分区：
文献类型：
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作者：
GOTOFF, SP;AMIRMOKRI, E;LIEBNER, EJ
据报道，一位共济失调毛细血管扩张症的患者引起了对腭部淋巴肉瘤放射治疗的不良反应的注意。在对肿瘤部位进行3000射线照射后，患者出现了严重的皮炎和深部组织坏死，导致8个月后死亡。与这种剂量的辐射相关的死亡是非常罕见的，可能与已经描述的共济失调毛细血管扩张症的生物缺陷有关。脑部可见典型的结节性硬化性病变。这两种遗传性疾病之间的联系以前没有报道过，由于这两种疾病都有发生肿瘤的倾向，因此特别令人感兴趣。关于病毒诱导的胸腺切除小鼠肿瘤的发病机制，共济失调毛细血管扩张症患者的恶性发展进行了讨论。
A patient with ataxia telangiectasia is reported to draw attention to an untoward response to radiation therapy for a palatal lymphosarcoma. After 3,000 rads to the tumor site, the patient developed a severe dermatitis and deep tissue necrosis resulting in death 8 months later. A fatality related to this dose of radiation is extemely unusual and may be related to the biological defects already described in ataxia telangiectasia. The typical lesion of tuberous sclerosis was found in the brain. The association of these 2 genetic disorders has not been previously reported, and is of particular interest because of the propensity for neoplasia in both diseases. The development of malignancy in patients with ataxia telangiectasia is discussed with regard to the pathogenesis of viral induced tumors in thymectomized mice.