Axen K, Bishop M, Haas F
J Appl Physiol (1985). 1987 Apr;62(4):1647-54. doi: 10.1152/jappl.1987.62.4.1647.
First-breath ventilatory responses to graded elastic and resistive loads were obtained from 15 people with Duchenne muscular dystrophy (DMD), 5 people with facioscapulohumeral MD (FSH), 3 people with Becker MD, and 3 people with limb-girdle MD. For each load tidal volumes from different individuals ranged from relatively small to comparatively large values, indicating a correspondingly wide range of end-inspiratory efforts; strong tidal volume defenders generally employed longer inspirations and higher mean inspiratory airflows than did weak tidal volume defenders; and individual frequency responses were mediated by changes in inspiratory and/or expiratory timing. Thus the loaded breathing responses of people with MD are qualitatively the same as those of quadriplegic and able-bodied people. Quantitatively, however, the DMD group generated considerably larger tidal volumes than did the FSH group during both elastic and resistive loading. These larger tidal volumes were achieved by both longer inspirations (a neurally mediated phenomenon) and higher mean inspiratory airflows (a mechanically and/or neurally mediated phenomenon). These findings, which could not be attributed to differences in respiratory motor function, suggest that there are differences between the respiratory sensory and/or central functions in the Duchenne and facioscapulohumeral types of MD.
对15名杜氏肌营养不良症（DMD）患者、5名面肩肱型肌营养不良症（FSH）患者、3名贝克型肌营养不良症患者和3名肢带型肌营养不良症患者进行了对分级弹性和阻力负荷的首次呼吸通气反应测试。对于每种负荷，不同个体的潮气量范围从相对较小到相对较大的值，这表明吸气末努力程度相应地有很大范围；潮气量较大的人通常比潮气量较小的人采用更长的吸气时间和更高的平均吸气气流；个体频率反应是由吸气和/或呼气时间的变化介导的。因此，肌营养不良症患者的负荷呼吸反应在质量上与四肢瘫痪者和身体健全者的相同。然而，在数量上，在弹性和阻力负荷期间，DMD组产生的潮气量比FSH组大得多。这些较大的潮气量是通过更长的吸气时间（一种神经介导的现象）和更高的平均吸气气流（一种机械和/或神经介导的现象）实现的。这些不能归因于呼吸运动功能差异的发现表明，杜氏和面肩肱型肌营养不良症在呼吸感觉和/或中枢功能方面存在差异。
