Yakumo Byoin National Sanatorium, Department of Paediatrics, 123 Miyazono-cho, Yamakoshi-gun Yakumo, Hokkaido 049-3198, Japan.
Neuromuscul Disord. 2011 Jan;21(1):47-51. doi: 10.1016/j.nmd.2010.09.006. Epub 2010 Dec 8.
We describe survival in Duchenne dystrophy by invasive and noninvasive ventilation vs. untreated. Patients were untreated prior to 1984 (Group 1), underwent tracheotomy from 1984 until 1991 (Group 2), and were managed by noninvasive mechanical ventilation and cardioprotective medications subsequently (Group 3). Symptoms, vital capacity, and blood gases were monitored for all and spirometry, cough peak flows, carbon dioxide tension, and oximetry for Group 3. Sleep nasal ventilation was initiated for symptomatic hypoventilation. An oximeter and mechanical cough assistance were prescribed for maximum assisted cough peak flow <300 L/m. Patients used continuous noninvasive ventilation and mechanically assisted coughing as needed to maintain pulse oxyhemoglobin saturation ≥95%. Survival was compared by Kaplan-Meier analysis. The 56 of Group 1 died at 18.6±2.9, the 21 Group 2 at 28.1±8.3 years of age with three still alive, and the 88 using noninvasive ventilation had 50% survival to 39.6 years, p<0.001, respectively. We conclude that noninvasive mechanical ventilation and assisted coughing provided by specifically trained physicians and therapists, and cardioprotective medication can result in more favorable outcomes and better survival by comparison with invasive treatment.
我们通过有创和无创通气与未治疗组来描述杜氏肌营养不良症的生存情况。1984 年之前患者未接受治疗（第 1 组），1984 年至 1991 年期间接受气管切开术（第 2 组），随后通过无创机械通气和心脏保护药物进行治疗（第 3 组）。对所有患者进行症状、肺活量和血气监测，并对第 3 组进行肺活量测定、咳嗽峰流速、二氧化碳分压和血氧饱和度监测。对于有症状性低通气的患者，开始使用睡眠鼻通气。对于最大辅助咳嗽峰流速<300 L/m 的患者，开具血氧计和机械咳嗽辅助设备。患者按需使用持续无创通气和机械辅助咳嗽，以维持脉搏血氧饱和度≥95%。通过 Kaplan-Meier 分析比较生存率。第 1 组的 56 名患者在 18.6±2.9 岁时死亡，第 2 组的 21 名患者在 28.1±8.3 岁时死亡，其中 3 人仍然存活，第 3 组的 88 名患者使用无创通气，50%的患者存活至 39.6 岁，p<0.001。我们得出结论，与有创治疗相比，专门的医生和治疗师提供的无创机械通气和辅助咳嗽以及心脏保护药物可以带来更好的结果和更长的生存时间。
