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Neurology：多聚谷氨酰胺疾病患者癌症患病率较低！

2017-02-16 xing.T MedSci原创

多聚谷氨酰胺疾病癌症
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polyQ患者癌症发病率有所下降，尽管危险因素的发生率较高。有趣的是，皮肤癌的发病率较高，提示神经退行性疾病和皮肤肿瘤的发生之间存在相关性。

多聚谷氨酰胺（polyQ）病是显性遗传的神经系统疾病，是由CAG三核苷酸重复序列编码和扩展而引起的。在对polyQ患者的回顾性研究中报道伴发癌症的比例十分罕见，近日，神经病学领域权威取杂志Neurology上发表了一篇研究文章，研究人员旨在调查polyQ疾病在法国的流行情况。

研究人员在巴黎、图卢兹、斯特拉斯堡和蒙彼利埃的4所大学医院里对连续就诊的Huntington舞蹈病（HD）和脊髓小脑性共济失调（SCA）的患者调查其癌症、心血管疾病以及相关危险因素情况。根据法国人群年龄和性别调整的发病率，研究人员对不同类型的癌症进行了标准化的发病率（SIR）评估。

研究人员对372例HD患者和134例SCA患者进行了调查。在HD患者中SIR表现出显著降低癌症风险：23例观察病例vs. 111.05例预期病例（SIR为0.21，95%可信区间[CI]为0.13-0.31），在SCA患者中：7例观察病例 vs. 34.75例预期病例（SIR为0.23，95%CI为0.08-0.42）。这一结果令人惊讶因为这些患者癌症的风险行为是增加的，在HD的患者有更多的烟草和酒精消耗相比于SCA患者（P＜0.0056）。CAG重复序列大小与癌症或心血管疾病之间没有任何关联。然而，在HD患者中，皮肤癌比预期的更频繁（5 vs. 0.98，SIR为5.11，95%CI为1.65-11.95）。

由此可见，polyQ患者癌症发病率有所下降，尽管危险因素的发生率较高。有趣的是，皮肤癌的发病率较高，提示神经退行性疾病和皮肤肿瘤的发生之间存在相关性。

原始出处：

Giulia Coarelli,et al. Low cancer prevalence in polyglutamine expansion diseases. Neurology. http://dx.doi.org/10.1212/WNL.0000000000003725

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    2017-09-05 hmwwww

    #疾病患者#

    0

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    2017-04-14 12498fa0m11暂无昵称

    #多聚谷氨酰胺#

    0

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    2017-12-14 yinhl1978

    #Neurol#

    0

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    2017-02-18 xinmeili

    #患病率#

    0

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    2017-02-17 ylzr123

    好文，值得点赞，值得拥有，值得收藏！

    0