Lyager S, Steffensen B, Juhl B
Aarhus Universitet, Fysiologisk Institut.
Ugeskr Laeger. 1993 Jun 21;155(25):1963-7.
In 1990 we examined 25 patients with Duchenne Muscular Dystrophy (DMD) or Spinal Muscular Atrophy type II (SMA-II). The purpose was to elucidate their general physical capability and find indicators for future need of artificial ventilation. We used interviews and measurements. The interviews were based on classifications described as 1) EAMDA, 2) VIGNOS, 3) BROOKE for use with DMD patients. Our own classification (called EK) is described and used. Moreover, we measured five parameters for the mechanical lung function, the strength of eight great muscle groups, and anthropometrical data. In January 1992, between two and 18 months after our examination, seven of the patients (all DMD) required artificial ventilation. Among the patients who needed artificial ventilation, we found the DMDs to have the lowest physical capability, as measured by the interview (EK and EAMDA) and the smallest values for forced vital capacity (FVC) and peak expiratory flow rate (PEF). All 11 patients with SMA-II could administer their lives without ventilator. Comparing their values with the DMD group, we would expect that several would need artificial ventilation. The reason why the SMA-II patients can manage without artificial ventilation is not clarified. Combining our classification (EK) and the value for FVC, we can predict the need for artificial ventilation among the participating DMD patients.
1990年，我们对25例杜氏肌营养不良症（DMD）或Ⅱ型脊髓性肌萎缩症（SMA-II）患者进行了检查。目的是阐明他们的一般身体能力，并找出未来需要人工通气的指标。我们采用了访谈和测量的方法。访谈基于以下几种分类：1）EAMDA，2）VIGNOS，3）用于DMD患者的布鲁克分类法。我们描述并使用了自己的分类法（称为EK）。此外，我们测量了五项机械肺功能参数、八大肌肉群的力量以及人体测量数据。1992年1月，在我们检查后的两到18个月期间，7例患者（均为DMD患者）需要人工通气。在需要人工通气的患者中，我们发现通过访谈（EK和EAMDA）测量，DMD患者的身体能力最低，其用力肺活量（FVC）和呼气峰值流速（PEF）的值最小。所有11例SMA-II患者无需呼吸机就能自理生活。将他们的值与DMD组进行比较，我们预计其中有几例患者会需要人工通气。SMA-II患者无需人工通气就能自理的原因尚不清楚。结合我们的分类法（EK）和FVC值，我们可以预测参与研究的DMD患者中人工通气的需求。
