期刊
JOURNAL OF PEDIATRIC REHABILITATION MEDICINE
卷 2, 期 2, 页码 115-122出版社
IOS PRESS
DOI: 10.3233/PRM-2009-0071
关键词
Duchenne muscular dystrophy; neuromuscular disease; respiratory care; restrictive respiratory disease
类别
Duchenne muscular dystrophy is is the most common form of the childhood muscular dystrophies. It follows a predictable clinical course marked by progressive skeletal muscle weakness, lost of ambulation before teen-age and death in early adulthood secondary to respiratory or cardiac failure. Becker muscular dystrophy is less common and has a milder clinical course but also results in respiratory and cardiac failure. Altough recent advances in respiratory care and new technologies have improved the outlook many patients already received only a traditional non-interventional approach. The aims of this work are: to analyse the pathophysiology and natural history of respiratory function in these diseases, to descript their clinical manifestations, to present the diagnostics tools and to provide recommendations for an adequated respiratory care in this particular population based on the updated literature referenced.
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