| 陈振薇,李卓岭,谭蕊,等.肯尼迪病合并烟雾病综合征 1例报告并相关分析[J].安徽医药,2026,30(8):1583-1586. |
| 肯尼迪病合并烟雾病综合征 1例报告并相关分析 |
| Report of a case of Kennedy's disease combined with moyamoya disease syndrome and related analysis |
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| DOI：10.3969/j.issn.1009-6469.2026.08.019 |
| 中文关键词: 肯尼迪病 烟雾病综合征 雄激素受体 急性缺血性卒中 血管重构 |
| 英文关键词: Kennedy's disease Moyamoya disease syndrome Androgen receptors Acute ischemic stroke Vascular remodeling |
| 基金项目: |
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| 摘要点击次数: 123 |
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| 中文摘要: |
| 目的报告 1例脊髓延髓性肌萎缩(又称肯尼迪病)合并烟雾病综合征，旨在提高临床医师对其罕见基因病的认识，并探讨其相关病理机制对脑血管疾病的影响。方法回顾性分析成都中医药大学附属医院 2023年 8月 25日收治的 1例肯尼迪病合并烟雾病综合征病人的临床资料，并复习相关文献探讨相关性分析。结果男， 61岁，因“右侧头胀痛 1个多月”收入院，入院后对其临床表现、实验室检查、影像学检查等进行综合分析，对其发病机制进行相关探讨。结论肯尼迪病由于先天雄激素受体结构和功能的异常，雄激素作用被阻断，影响了脂质代谢、氧化应激、血管内皮细胞功能、血管舒张功能、血管重构等方面，从而诱发或者加重缺血性脑血管病。 |
| 英文摘要: |
| Objective To report a case of spinal and bulbar muscular atrophy (also known as Kennedy's disease) complicated withmoyamoya syndrome, aiming to enhance clinicians' awareness of this rare genetic disorder and explore the impact of its underlyingpathological mechanisms on cerebrovascular diseases.Methods A retrospective analysis was conducted on the clinical data of one pa-tient with Kennedy's disease complicated with moyamoya syndrome admitted to the Affiliated Hospital of Chengdu University of Tradi-tional Chinese Medicine on August 25, 2023. Relevant literature was reviewed to discuss the potential correlation.Results The pa-tient was a 61-year-old male admitted due to "right-sided cephalgia for over one month." A comprehensive analysis of his clinical mani-festations, laboratory findings, and imaging studies was performed, and the pathogenesis was discussed.Conclusion Kennedy's dis-ease, characterized by congenital structural and functional abnormalities of the androgen receptor leading to blocked androgen action,may induce or exacerbate ischemic cerebrovascular diseases by affecting lipid metabolism, oxidative stress, vascular endothelial cellfunction, vasodilation, and vascular remodeling. |
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