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Dynamic Respiratory Muscle Function in Late-Onset Pompe Disease

NCT02354664 · Status: COMPLETED · Type: OBSERVATIONAL · Enrollment: 14

Last updated 2024-06-24

Study results available
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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.

Conditions

  • Pompe Disease

Interventions

OTHER

Thoracic MRI

Imaging of the diaphragm will be completed during resting breathing, and then during a maximal voluntary ventilation maneuver.

OTHER

Spirometry

Upright forced vital capacity (FVC)

OTHER

Inspiratory Load Compensation

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.

OTHER

Maximal Inspiratory Pressure

Measure the maximal airflow on inhalation

OTHER

Resting Breathing Pattern

The breathing pattern will be assessed when the subject is seated and in a relaxed state.

OTHER

Respiratory Muscle Endurance Test

Evaluates the time limit that a participant can maintain ventilation while breathing with a submaximal inspiratory threshold load.

Sponsors & Collaborators

Principal Investigators

  • Barbara K. Smith, PT, PhD · University of Florida

Eligibility

Min Age
20 Years
Max Age
65 Years
Sex
ALL
Healthy Volunteers
Yes

Timeline & Regulatory

Start
2015-04-30
Primary Completion
2016-10-19
Completion
2016-10-19

Countries

  • United States

Study Locations

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Entities

Companies

Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT02354664 on ClinicalTrials.gov