Fatty Acid Oxidation and Body Weight Regulation in Long-chain Fatty Acid Oxidation Disorders.
Trial Snapshot
- Phase
- Phase 1
- Status
- Completed
- Enrollment
- 13
- Primary Endpoint
- Energy Expenditure
Study Overview
Brief Summary
The study also determines if eating a diet higher in protein alters body composition, energy balance and metabolic control among patients with a long-chain fatty acid oxidation disorder.
Detailed Description
At baseline, the amount of muscle and fat in the whole body and inside the liver and muscle will be measured. All subjects will complete a moderate treadmill exercise test and a test to determine how they use sugar (oral glucose tolerance test). The amount and type of energy burned by each subject will be measured. Subjects will be randomly assigned to follow either a high carbohydrate diet or a reduced carbohydrate, high protein diet for 4 months. At the end of 4 months, the tests will be repeated.
Study Design
- Study Type
- Interventional
- Allocation
- Randomized
- Intervention Model
- Parallel
- Primary Purpose
- Treatment
- Masking
- None
Eligibility Criteria
- Ages
- 7 Years to 45 Years (Child, Adult)
- Sex
- All
- Accepts Healthy Volunteers
- No
Inclusion Criteria
- •diagnosis of a long-chain fatty acid oxidation disorder including VLCAD, CPT2, LCHAD or TFP deficiency
- •7 years old or greater
- •able to comply with diet guidelines
Exclusion Criteria
- •enrolled in another study that alters diet composition
- •cannot complete treadmill exercise study
Outcomes
Primary Outcomes
Energy Expenditure
Time Frame: change from baseline after 4 months of treatment
does energy expenditure and substrate oxidation differ between subjects randomized to the high carbohydrate versus the high protein diet? We measured resting energy expenditure with indirect calorimetry and estimated substrate oxidation with indirect calorimetry results and urine urea nitrogen excretion. We also measured total energy expenditure with doubly labeled water.
Secondary Outcomes
- Body Composition(change from baseline to 4 months of treatment)
Investigators
Melanie B Gillingham
Assistant Professor
Oregon Health and Science University