Ventilatory response in myotonic dystrophy
Ventilatory response in myotonic dystrophy
复制标题
强直性肌营养不良的通气反应
作者：
J. Carroll;C. Zwillich;J. Weil
强直性肌营养不良患者常因肺泡通气不足而发生呼吸衰竭。对低氧和高碳酸血症的缓解反应减弱可以解释这一现象。因此，缺氧和高碳酸血症的反应进行了测量，在7例强直性肌营养不良谁只有轻微的呼吸肌无力。低血糖反应显著降低，而高碳酸反应受到更不规则的影响。这类患者呼吸衰竭的高发生率可能与低氧性呼吸反应降低有关，这是由于潜在的神经源性缺陷所致。
Patients with myotonic dystrophy often develop respiratory failure caused by alveolar hypoventilation. Abnormalities in the ventilatory response to hypoxia and hypercapnia may explain this phenomenon. Accordingly, hypoxic and hypercapnic responses were measured in seven patients with myotonic dystrophy who had only mild respiratory muscle weakness. Hypoxic response was significantly reduced, while hypercapnic response was affected more irregularly. It is possible that the high incidence of respiratory failure in such patients is related to decreased hypoxic ventilatory response, occurring because of an underlying neurogenic deficit.