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Issue title: Emergency Treatment
Article type: Research Article
Authors: Spehrs-Ciaffi, Virginia | Fitting, Jean William | Cotting, Jacques | Jeannet, Pierre-Yves
Affiliations: Centre Hospitalier Universitaire Vaudois, Neuropediatric Unit, Lausanne, Switzerland | Centre Hospitalier Universitaire Vaudois, Respiratory Unit, Lausanne, Switzerland | Centre Hospitalier Universitaire Vaudois, Pediatric Intensive Care Unit, Lausanne, Switzerland
Note: [] Address for correspondence: Virginia Spehrs-Ciaffi, Rue des Prés-duTorrent 26, 1964 Conthey, Switzerland. Tel.: + 41 27 321 36 35; E-mail: [email protected]
Abstract: 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.
Keywords: Duchenne muscular dystrophy, neuromuscular disease, respiratory care, restrictive respiratory disease
DOI: 10.3233/PRM-2009-0071
Journal: Journal of Pediatric Rehabilitation Medicine, vol. 2, no. 2, pp. 115-122, 2009
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