Vercken J B, Raphael J C, de Lattre J, Fromageot C, Fanjoux J
Service de Réanimation Médicale, Hôpital Raymond Poincaré, Garches, France.
Biomed Pharmacother. 1988;42(5):343-9.
We report a study of an adult with a maltase acid deficiency myopathy. A restrictive respiratory syndrome due to respiratory muscle weakness is associated with paralysis of other muscular groups. In 1982 the patient presented with an alveolar hypoventilation, and mechanical ventilation was required after acute respiratory failure. The patient has received nocturnal mechanical ventilation by tracheostomy at home for 5 years. His clinical status gradually improved in parallel to amelioration of his respiratory condition. Functional respiratory tests improved: initial hypoxia-hypercapnia disappeared, vital capacity increased. The possible mechanisms underlying the improvement are discussed. Increase in pulmonary compliance is an argument to explain the functional improvement observed. Ventilatory response to carbon dioxide was abnormal whereas the ventilatory response to exercise and maxima minute ventilation test were normal. Results are consistent with a respiratory control impairment. The role of mechanical ventilation is difficult to assess in the improvement we observed.
我们报告了一项关于一名患有麦芽糖酶酸缺乏性肌病的成年人的研究。由于呼吸肌无力导致的限制性呼吸综合征与其他肌肉群的麻痹有关。1982年，该患者出现肺泡通气不足，急性呼吸衰竭后需要机械通气。患者在家中通过气管切开术接受夜间机械通气已达5年。其临床状况随着呼吸状况的改善而逐渐改善。功能性呼吸测试有所改善：最初的低氧血症-高碳酸血症消失，肺活量增加。讨论了改善背后可能的机制。肺顺应性增加是解释所观察到的功能改善的一个依据。对二氧化碳的通气反应异常，而对运动的通气反应和最大分钟通气量测试正常。结果与呼吸控制受损一致。在我们观察到的改善中，机械通气的作用难以评估。
