Comparison of Sitting and Supine Forced Vital Capacity in Collagen VI-Related Dystrophy and Laminin α2-Related Dystrophy

BACKGROUND: Progressive, restrictive, respiratory insufficiency is the major cause of morbidity and mortality in Congenital Muscular Dystrophy (CMD). Nocturnal hypoventilation precedes daytime alveolar hypoventilation, and if untreated, may lead to respiratory failure and cor pulmonale. CMD consensu...

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Publicado en:Pediatr Pulmonol
Main Authors: Meilleur, Katherine G., Linton, Melody M., Fontana, Joseph, Rutkowski, Anne, Elliott, Jeffrey, Barton, Mark, McGraw, Peter, Kokkinis, Angela, Donkervoort, Sandra, Leach, Meganne, Jain, Minal, Dastgir, Jahannaz, Collins, James, Szczesniak, Rhonda, Yang, Kelly, Sawnani, Hemant, Bönnemann, Carsten G.
Formato: Artigo
Idioma:Inglês
Publicado: 2017
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Article
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC6309368/
https://ncbi.nlm.nih.gov/pubmed/28085238
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/ppul.23622
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