At a glance
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Dynamic Respiratory Muscle Function in Late-Onset Pompe Disease
In Brief
An observational study evaluating Thoracic MRI, Spirometry, and 4 other interventions for Pompe Disease. Completed, enrolled 14 participants across 1 site.
Detailed Summary
This study intends to evaluate dynamic respiratory motor performance as a valuable measure of pulmonary function in adults with late-onset Pompe disease. The investigators will adopt a strategy that includes comprehensive evaluations of respiratory volume, flow, and timing parameters during resting and loaded breathing. These evaluations will then be associated to the standard clinical measure of maximal inspiratory pressure, the static inspiratory muscle function, as well as magnetic resonance imaging of thoracic expansion and diaphragmatic descent at rest and with exertion. Outcomes in participants with late-onset Pompe disease will be contrasted to the function of age- and gender-matched control subjects. This approach will enable the investigators to evaluate the relationship between dynamic diaphragmatic function and respiratory motor function.
Study Details
Timeline
Interventions
Imaging of the diaphragm will be completed during resting breathing, and then during a maximal voluntary ventilation maneuver.
Upright forced vital capacity (FVC)
A spring-loaded pressure device will resist inspiration. The patient must generate the threshold inspiratory pressure to receive airflow. The changes in breathing timing, flow and volume will be recorded.
Measure the maximal airflow on inhalation
The breathing pattern will be assessed when the subject is seated and in a relaxed state.
Evaluates the time limit that a participant can maintain ventilation while breathing with a submaximal inspiratory threshold load.