Duchenne muscular dystrophy: Survival by cardio-respiratory interventions
Duchenne muscular dystrophy: Survival by cardio-respiratory interventions
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DOI：
10.1016/j.nmd.2010.09.006
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发表时间：
2011-01-01
影响因子：
2.8
通讯作者：
Minami, Ryoji
中科院分区：
文献类型：
--
作者：
Ishikawa, Yuka;Miura, Toshihiko;Minami, Ryoji
我们描述了Duchenne营养不良患者的存活率，通过有创和无创通气与未经治疗的比较，患者在1984年之前未经治疗（第1组），从1984年到1991年接受气管切开术（第2组），随后接受无创机械通气和心脏保护药物治疗（第3组）。监测所有患者的症状、肺活量和血气，并监测第3组患者的肺功能测定、咳嗽峰值流量、二氧化碳张力和血氧测定。由于症状性通气不足，开始睡眠鼻通气。对于最大辅助咳嗽峰值流量= 95%，处方了血氧计和机械咳嗽辅助。通过Kaplan-Meier分析比较生存率。第1组56例在18.6 +/- 2.9岁时死亡，第2组21例在28.1 +/- 8.3岁时死亡，其中3例仍存活，使用无创通气的88例患者50%生存至39.6年，p < 0.001，我们的结论是，由受过专门训练的医生和治疗师提供的无创机械通气和辅助咳嗽，与侵入性治疗相比，心脏保护药物可以产生更有利的结果和更好的生存率。(C)2010爱思唯尔有限公司版权所有。
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 = 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. (C) 2010 Elsevier B.V. All rights reserved.