Cardiac and Pulmonary Investigations in Bethlem Myopathy
Why the study?
Does Bethlem myopathy cause cardiac and respiratory abnormalities in affected patients?
Population
50 patients with Bethlem myopathy from 26 families
Design
Cross-sectional
Authors
Discussion
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Member takes
Implication
Respiratory monitoring may warrant consideration in ambulatory Bethlem myopathy; leaves open cardiac sparing in larger prospective cohorts.
Structured PICO
Does Bethlem myopathy cause cardiac and respiratory abnormalities in affected patients?
P
Population
50 patients with Bethlem myopathy from 26 families
I
Intervention
Cardiac examinations (electrocardiography, echocardiography, Holter monitoring) and pulmonary investigations
O
Outcome
Cardiac and respiratory abnormalitiessurrogate
Bethlem myopathy does not appear to have significant cardiac involvement, but respiratory failure can occur even in ambulatory patients.
Cite This Study
Kooi et al. (2006) studied this question.