Exercise Intolerance Study in Long Chain Fatty Acid Oxidation Disorders
Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)
50 participants
Aug 1, 2023
OBSERVATIONAL
Conditions
Summary
In this study, the exercise tolerance in patients with long-chain fatty acid oxidation disorders (LcFAOD) will be compared to that of healthy volunteers who are carefully matched in terms of age, sex, BMI and physical activity score. The study aims to assess exercise capacity using cardiopulmonary exercise (CPX) tests and activity monitoring over a 7-day period. The hypothesis of the study is that LcFAOD patients will exhibit greater oxygen debt during short periods of exercise compared to healthy individuals. These findings will contribute to a better understanding of exercise intolerance in LcFAOD patients and may guide the development of targeted interventions, such as exercise training programs and enable the analysis of effectiveness of (new) treatments.
Eligibility
Plain Language Summary
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Interventions
Exercise test with step-change from rest to a relatively low constant workload.
Exercise test with incremental workload until maximal workload.
Determine upper leg muscle size
Determine muscle strength
SQUASH, mMRC, questionnaire designed to report myopathic symptoms in LcFAOD patients.
Locations(1)
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NCT05924555