Journal
PHYSICAL MEDICINE AND REHABILITATION CLINICS OF NORTH AMERICA
Volume 23, Issue 1, Pages 75-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.pmr.2011.11.017
Keywords
X-linked myotubular myopathy; Respiratory impedance plethysmography; XLMTM dog; Respiratory dysfunction
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Respiratory dysfunction due progressive weakness of the respiratory muscles, particularly the diaphragm, is a major cause of death in the neuromuscular disease (NMD) X-linked myotubular myopathy (XLMTM). Methods of respiratory assessment in patients are often difficult, especially in those who are mechanically ventilated. The naturally occuring XLMTM dog model exhibits a phenotype similar to that in patients and can be used to determine quantitative descriptions of dysfunction as clinical endpoints for treatment and the development of new therapies. In experiments using respiratory impedance plethysmography (RIP), XLMTM dogs challenged with the respiratory stimulant doxapram displayed significant changes indicative of diaphragmatic weakness.
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