| 成人脊肌萎缩症的自然史及其死因探讨(附40例死亡病例分析及2例尸检) |
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| 引用本文： | 徐桂芝,张福林,秦芝九.成人脊肌萎缩症的自然史及其死因探讨(附40例死亡病例分析及2例尸检)[J].上海医学,1992(9). |
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| 作者姓名： | 徐桂芝 张福林 秦芝九 |
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| 作者单位： | 上海医科大学附属华山医院神经病学研究所,上海医科大学附属华山医院神经病学研究所,上海医科大学附属华山医院神经病学研究所 |
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| 摘 要： | 本文报告40例成人脊肌萎缩症死亡患者的起病症状,病变部位、症状演变、病程、死亡经过和2例尸检材料。提出本病可能与运动神经元内雄激素受体丧失及免疫功能紊乱有关。其主要死亡原因为脊髓型呼吸衰竭和并发症。应特别警惕在疾病早期出现的膈肌麻痹。
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| 关 键 词： | 脊肌萎缩症 脊髓型呼吸衰竭 膈肌麻痹 |
NATURAL HISTORY OF ADULT SPINAL MUSCULAR ATROPHY AND STUDY OF THE CAUSE OF DEATH: A REPORT OF 40 CASES |
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| Xu Gui-zhi,et al..NATURAL HISTORY OF ADULT SPINAL MUSCULAR ATROPHY AND STUDY OF THE CAUSE OF DEATH: A REPORT OF 40 CASES[J].Shanghai Medical Journal,1992(9). |
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| Authors: | Xu Gui-zhi |
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| Institution: | Xu Gui-zhi,et al. Huashan Hospital,Shanghai Medical University,Shanghai 200040 |
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| Abstract: | This article reports 40 cases of adult spinal muscular atrophy, with discussion on their earliest symptoms, location of lesion, the development of symptom, and the cause of death. Autopsy was performed in in two cases. Loss of androgen receptors and disturbance of immunologic function were the possible etiology. The major causes of death were "spinal type" respiratory failure and complications. Special attention should be paid to diaphragmatic paralysis in early phase of the disease. |
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| Keywords: | Spinal muscular atrophy: "spinal type" respiratory failure diaphragmatic paralysis |
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