Respiratory failure in Pompe disease: Treatment with noninvasive ventilation

被引：74
作者：
Mellies, U
Stehling, F
Dohna-Schwake, C
Ragette, R
Teschler, H
Voit, T
机构：
[1] Univ Essen Gesamthsch, Dept Pediat & Pediat Neurol, D-45122 Essen, Germany
[2] Ruhrlandklin, Dept Pneumol & Sleep Med, Essen, Germany
来源：
NEUROLOGY | 2005年 / 64卷 / 08期
关键词：
D O I：
10.1212/01.WNL.0000158682.85052.C0
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
In this study, noninvasive ventilation (NIV) was prospectively applied to eight patients (35.8 +/- 11.4 years) with late-onset Pompe disease and respiratory failure apparent from severe restrictive lung disease, nocturnal hypoxemia ( 83 +/- 8%), and daytime hypercapnia (66.7 +/- 17.9 mm Hg). The impact of NIV on respiratory function was followed for 34 +/- 17 months. Despite further decrease of vital capacity and inspiratory muscle strength, NIV normalized oxygen saturation during sleep ( 96 +/- 1%), daytime carbon dioxide tensions (44.1 +/- 3.6 mm Hg), and symptoms.
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收藏
页码：1465 / 1467
页数：3
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