面肩肱型肌营养不良症的通气支持
Ventilatory support in facioscapulohumeral muscular dystrophy.
作者信息
Wohlgemuth M, van der Kooi E L, van Kesteren R G, van der Maarel S M, Padberg G W
机构信息
Department of Neurology, University Medical Center Nijmegen, Reinier Postlaan 4, 6500 HB Nijmegen, The Netherlands.
出版信息
Neurology. 2004 Jul 13;63(1):176-8. doi: 10.1212/01.wnl.0000133126.86377.e8.
Respiratory insufficiency due to respiratory muscle weakness is a common complication of many neuromuscular diseases. The prevalence of respiratory failure in facioscapulohumeral muscular dystrophy (FSHD) is unknown. The authors identified 10 FSHD patients on nocturnal ventilatory support at home, representing approximately 1% of the Dutch FSHD population. Severe muscle disease, wheelchair dependency, and kyphoscoliosis appeared to be risk factors for respiratory failure.
呼吸肌无力导致的呼吸功能不全是许多神经肌肉疾病的常见并发症。面肩肱型肌营养不良症（FSHD）中呼吸衰竭的患病率尚不清楚。作者在荷兰确定了10名在家接受夜间通气支持的FSHD患者，约占荷兰FSHD患者总数的1%。严重的肌肉疾病、依赖轮椅以及脊柱侧弯似乎是呼吸衰竭的危险因素。
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