Rib Cage Deformities Alter Respiratory Muscle Action and Chest Wall Function in Patients with Severe Osteogenesis Imperfecta

BACKGROUND: Osteogenesis imperfecta (OI) is an inherited connective tissue disorder characterized by bone fragility, multiple fractures and significant chest wall deformities. Cardiopulmonary insufficiency is the leading cause of death in these patients. METHODS: Seven patients with severe OI type I...

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Hlavní autoři: LoMauro, Antonella, Pochintesta, Simona, Romei, Marianna, D'Angelo, Maria Grazia, Pedotti, Antonio, Turconi, Anna Carla, Aliverti, Andrea
Médium: Artigo
Jazyk:Inglês
Vydáno: Public Library of Science 2012
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Research Article
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC3338769/
https://ncbi.nlm.nih.gov/pubmed/22558284
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0035965
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