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杜氏肌营养不良症终末期呼吸衰竭的管理

Management of end stage respiratory failure in Duchenne muscular dystrophy.

作者信息

Bach J R, O'Brien J, Krotenberg R, Alba A S

出版信息

Muscle Nerve. 1987 Feb;10(2):177-82. doi: 10.1002/mus.880100212.

DOI:10.1002/mus.880100212
PMID:3547120
Abstract

There were 31 Duchenne patients placed on overnight mouth intermittent positive pressure ventilation for severe respiratory insufficiency at the average age of 19.9 years. Most patients had vital capacities less than 200 cc at their last evaluations. Of these, 23 patients are alive at the average age of 27 years and live in the community, although they are dependent on assisted ventilation 24 hours a day. There were 8 patients who died at the average age of 30 years. Although normocapnic during the day, the presence of symptomatic nocturnal hypoventilation or pCO2 over 55 mmHg documented by continuous overnight capnograph study indicates the need for introducing overnight respiratory assistance. Mouth intermittent positive pressure ventilation alone or in combination with other techniques of ventilatory assistance can prolong life while allowing optimal function, attainment of higher levels of education, and home management of patients with Duchenne muscular dystrophy.

摘要

31例杜氏肌营养不良患者因严重呼吸功能不全接受夜间口腔间歇性正压通气治疗,平均年龄19.9岁。大多数患者在最后一次评估时肺活量小于200毫升。其中,23例患者存活,平均年龄27岁,生活在社区,尽管他们每天24小时依赖辅助通气。有8例患者死亡,平均年龄30岁。尽管白天血二氧化碳正常,但夜间症状性通气不足或连续夜间二氧化碳描记图研究记录的pCO2超过55 mmHg表明需要引入夜间呼吸辅助。单独使用口腔间歇性正压通气或与其他通气辅助技术联合使用,可以延长杜氏肌营养不良患者的生命,同时使其保持最佳功能、接受更高水平的教育并在家中得到照料。

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1
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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[The effect of nasal IPPV on patients with respiratory failure during sleep due to Duchenne muscular dystrophy].[经鼻间歇正压通气对杜氏肌营养不良症所致睡眠呼吸衰竭患者的影响]
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Long-term follow-up of nocturnal ventilatory assistance in patients with respiratory failure due to Duchenne-type muscular dystrophy.杜氏型肌营养不良所致呼吸衰竭患者夜间通气辅助的长期随访
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[18 years experience with mechanical ventilation in patients with Duchenne muscular dystrophy].[杜氏肌营养不良症患者机械通气18年经验]
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