Lung MRI and impairment of diaphragmatic function in Pompe disease
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چکیده
منابع مشابه
Lung MRI and impairment of diaphragmatic function in Pompe disease
BACKGROUND Pompe disease is a progressive metabolic myopathy. Involvement of respiratory muscles leads to progressive pulmonary dysfunction, particularly in supine position. Diaphragmatic weakness is considered to be the most important component. Standard spirometry is to some extent indicative but provides too little insight into diaphragmatic dynamics. We used lung MRI to study diaphragmatic ...
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Radioactive xenon-133 was used to study the regional lung function of five patients with unilateral diaphragmatic paralysis unassociated with intrathoracic disease. All patients showed a reduction in total lung capacity to which the affected side contributed an average of 37%. There was a decrease in the amount of inhaled xenon and a lesser decrease in the amount of injected xenon reaching the ...
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ژورنال
عنوان ژورنال: BMC Pulmonary Medicine
سال: 2015
ISSN: 1471-2466
DOI: 10.1186/s12890-015-0058-3