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晚期杜氏肌营养不良症患者的机械通气:家庭管理

Mechanical ventilation of patients with late stage Duchenne muscular dystrophy: management in the home.

作者信息

Alexander M A, Johnson E W, Petty J, Stauch D

出版信息

Arch Phys Med Rehabil. 1979 Jul;60(7):289-92.

PMID:454124
Abstract

Death in the late stage of Duchenne muscular dystrophy is most frequently a consequence of respiratory failure. Since muscles of ventilation become weakened the bellows mechanism fails insidiously. Patients exhibit symptoms of ventilatory insufficiency, the first to be noticed are those associated with CO2 retention: dyspnea, nightmares, increased heart rate, and increased blood pressure. Ten patients with late stage Duchenne muscular dystrophy have been supplied with mechanical aid for ventilatory assistance. The age of onset of respiratory distress needing mechanical assistance varied from 10 to 20 years. Meaningful survival after allegedly reaching the end stage has been from 2 to 7.5 years with an average of 3.4 years. With a caring family, these patients can have a meaningful life, even though they require continuous mechanical ventilatory aid.

摘要

杜兴氏肌营养不良症晚期的死亡最常见的原因是呼吸衰竭。由于通气肌变弱,呼吸机制会不知不觉地失效。患者会出现通气不足的症状,首先被注意到的是与二氧化碳潴留相关的症状:呼吸困难、噩梦、心率加快和血压升高。十名杜兴氏肌营养不良症晚期患者已获得机械通气辅助设备。需要机械辅助的呼吸窘迫发病年龄在10至20岁之间。在据称进入终末期后,有意义的生存期为2至7.5年,平均为3.4年。有了关爱他们的家庭,这些患者即使需要持续的机械通气辅助,也能过上有意义的生活。

相似文献

1
Mechanical ventilation of patients with late stage Duchenne muscular dystrophy: management in the home.晚期杜氏肌营养不良症患者的机械通气:家庭管理
Arch Phys Med Rehabil. 1979 Jul;60(7):289-92.
2
Management of end stage respiratory failure in Duchenne muscular dystrophy.杜氏肌营养不良症终末期呼吸衰竭的管理
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[Home mechanical ventilation by nasal mask in a patient with Duchenne's disease].[杜氏病患者经鼻面罩进行家庭机械通气]
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Ventilator management in Duchenne muscular dystrophy and postpoliomyelitis syndrome: twelve years' experience.杜氏肌营养不良症和脊髓灰质炎后综合征的呼吸机管理:十二年经验
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Weaning from mechanical ventilation: successful use of modified inspiratory resistive training in muscular dystrophy.机械通气撤机:改良吸气阻力训练在肌肉萎缩症中的成功应用
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Life with home mechanical ventilation for young men with Duchenne muscular dystrophy.杜氏肌营养不良症年轻男性的家庭机械通气生活。
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[The silent yet successful revolution of mechanical ventilation in patients with neuromuscular disorders, in particular Duchenne muscular dystrophy].[神经肌肉疾病患者,尤其是杜氏肌营养不良患者机械通气的悄然成功变革]
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[Home mechanical ventilation in Duchenne de Boulogne muscular dystrophy. Mandatory ventilation and preventive ventilation].[杜兴氏进行性肌营养不良症的家庭机械通气。强制性通气和预防性通气]
Rev Mal Respir. 1987;4(5):195-7.

引用本文的文献

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Longitudinal effect of eteplirsen versus historical control on ambulation in Duchenne muscular dystrophy.依特普瑞森与历史对照相比对杜氏肌营养不良症患者步行能力的纵向影响。
Ann Neurol. 2016 Feb;79(2):257-71. doi: 10.1002/ana.24555. Epub 2016 Jan 8.
3
Widening gap in age at muscular dystrophy-associated death between blacks and whites, 1986-2005.
1986 年至 2005 年间,黑人和白人的肌营养不良相关死亡率的年龄差距扩大。
Neurology. 2010 Sep 14;75(11):982-9. doi: 10.1212/WNL.0b013e3181f25e5b.
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Physical medicine and rehabilitation: respirator-dependent patients with duchenne type muscular dystrophy and amyotrophic lateral sclerosis.物理医学与康复:依赖呼吸机的杜氏型肌营养不良症和肌萎缩侧索硬化症患者。
West J Med. 1986 Jun;144(6):735.
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Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy.鼻通气对高碳酸血症型杜氏肌营养不良症患者生存率的影响。
Thorax. 1998 Nov;53(11):949-52. doi: 10.1136/thx.53.11.949.
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Respiratory care in muscular dystrophy.肌肉萎缩症的呼吸护理
Br Med J (Clin Res Ed). 1987 Oct 24;295(6605):1014-5. doi: 10.1136/bmj.295.6605.1014.
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Respiratory failure and mechanical ventilation: pathophysiology and methods of promoting weaning.呼吸衰竭与机械通气:病理生理学及促进撤机的方法
Lung. 1986;164(6):309-24. doi: 10.1007/BF02713656.