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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| High carbohydrate diet | Active Comparator | Diet composition of 10% long-chain fatty acids, 20% medium-chain triglycerides, 12% protein and 68% carbohydrate is the current standard of care in long-chain fatty acid oxidation disorders. |
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| High protein diet | Experimental | Diet composition of 10% long-chain fatty acids, 20% medium chain triglycerides, 25% protein and 45% carbohydrate is the comparison diet. Fat content is the same between treatments; only the carbohydrate to protein ratio varies. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Diet counseling | Behavioral | Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home. |
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| Measure | Description | Time Frame |
|---|---|---|
| Energy Expenditure | 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. | change from baseline after 4 months of treatment |
| Measure | Description | Time Frame |
|---|---|---|
| Body Composition | does body composition change more among subjects randomized to the high protein diet compared to the high carbohydrate diet? We measured body composition at baseline and again at the end of the study by DEXA - dual energy x-ray absorptiometry. | change from baseline to 4 months of treatment |
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Inclusion Criteria:
Exclusion Criteria:
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| ID | Title | Description |
|---|---|---|
| FG000 | High Carbohydrate Diet | Diet composition of 10% long-chain fatty acids, 20% medium-chain triglycerides, 12% protein and 68% carbohydrate is the current standard of care in long-chain fatty acid oxidation disorders. Diet counseling: Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home. |
| Title | Milestones | Reasons Not Completed | |||||
|---|---|---|---|---|---|---|---|
| Overall Study |
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| FG001 |
| High Protein Diet |
Diet composition of 10% long-chain fatty acids, 20% medium chain triglycerides, 25% protein and 45% carbohydrate is the comparison diet. Fat content is the same between treatments; only the carbohydrate to protein ratio varies. Diet counseling: Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home. |
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| NOT COMPLETED |
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| ID | Title | Description |
|---|---|---|
| BG000 | High Carbohydrate Diet | Diet composition of 10% long-chain fatty acids, 20% medium-chain triglycerides, 12% protein and 68% carbohydrate is the current standard of care in long-chain fatty acid oxidation disorders. Diet counseling: Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home. |
| BG001 | High Protein Diet | Diet composition of 10% long-chain fatty acids, 20% medium chain triglycerides, 25% protein and 45% carbohydrate is the comparison diet. Fat content is the same between treatments; only the carbohydrate to protein ratio varies. Diet counseling: Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home. |
| BG002 | Total | Total of all reporting groups |
| Units | Counts |
|---|---|
| Participants |
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| Title | Description | Population Description | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Denominator Units Selected | Denominators | Classes | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Age, Continuous | Mean | Standard Deviation | years |
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| Sex: Female, Male | Count of Participants | Participants |
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| Region of Enrollment | Number | participants |
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| Type | Title | Description | Population Description | Reporting Status | Anticipated Posting Date | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Time Frame | Units Analyzed | Denominator Units Selected | Arm/Group Information | Denominators | Classes | Analyses | |||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Primary | Energy Expenditure | 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. | Posted | Mean | Standard Deviation | kcal | change from baseline after 4 months of treatment |
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| Secondary | Body Composition | does body composition change more among subjects randomized to the high protein diet compared to the high carbohydrate diet? We measured body composition at baseline and again at the end of the study by DEXA - dual energy x-ray absorptiometry. | Posted | Mean | Standard Deviation | change in kg | change from baseline to 4 months of treatment |
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| ID | Title | Description | Deaths (Affected) | Deaths (At Risk) | Serious Events (Affected) | Serious Events (At Risk) | Other Events (Affected) | Other Events (At Risk) |
|---|---|---|---|---|---|---|---|---|
| EG000 | High Carbohydrate Diet | Diet composition of 10% long-chain fatty acids, 20% medium-chain triglycerides, 12% protein and 68% carbohydrate is the current standard of care in long-chain fatty acid oxidation disorders. Diet counseling: Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home. | 0 | 6 | 0 | 6 | 0 | 6 |
| EG001 | High Protein Diet | Diet composition of 10% long-chain fatty acids, 20% medium chain triglycerides, 25% protein and 45% carbohydrate is the comparison diet. Fat content is the same between treatments; only the carbohydrate to protein ratio varies. Diet counseling: Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home. | 0 | 7 | 0 | 7 | 0 | 7 |
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| Title | Organization | Phone | Extension | |
|---|---|---|---|---|
| Dr. Melanie Gillingham | Oregon Health & Science University | 503-494-1682 | gillingm@ohsu.edu |
| ID | Term |
|---|---|
| C536353 | VLCAD deficiency |
| C566945 | Trifunctional Protein Deficiency With Myopathy And Neuropathy |
| C535589 | Carnitine palmitoyl transferase 2 deficiency |
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| ID | Term |
|---|---|
| D018752 | Diet, Fat-Restricted |
| D000076107 | Diet, High-Protein Low-Carbohydrate |
| D000073600 | Diet, High-Protein |
| ID | Term |
|---|---|
| D004035 | Diet Therapy |
| D044623 | Nutrition Therapy |
| D013812 | Therapeutics |
| D004032 | Diet |
| D009747 | Nutritional Physiological Phenomena |
| D000066888 | Diet, Food, and Nutrition |
| D010829 | Physiological Phenomena |
| D050528 | Diet, Carbohydrate-Restricted |
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| Male |
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| Sweden |
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