High Protein Diet in Patients With Long-chain Fatty Acid Oxidation Disorders
Primary Purpose
Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency, Carnitine Palmitoyltransferase 2 Deficiency
Status
Completed
Phase
Phase 1
Locations
Study Type
Interventional
Intervention
Diet counseling
Sponsored by
About this trial
This is an interventional treatment trial for Very Long-chain Acyl-CoA Dehydrogenase Deficiency focused on measuring fatty acid oxidation disorders
Eligibility Criteria
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:
- pregnant
- enrolled in another study that alters diet composition
- cannot complete treadmill exercise study
Sites / Locations
Arms of the Study
Arm 1
Arm 2
Arm Type
Active Comparator
Experimental
Arm Label
High carbohydrate diet
High protein diet
Arm Description
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 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.
Outcomes
Primary Outcome Measures
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.
Secondary Outcome Measures
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.
Full Information
NCT ID
NCT01494051
First Posted
December 14, 2011
Last Updated
April 20, 2020
Sponsor
Oregon Health and Science University
1. Study Identification
Unique Protocol Identification Number
NCT01494051
Brief Title
High Protein Diet in Patients With Long-chain Fatty Acid Oxidation Disorders
Official Title
Fatty Acid Oxidation and Body Weight Regulation in Long-chain Fatty Acid Oxidation Disorders.
Study Type
Interventional
2. Study Status
Record Verification Date
April 2020
Overall Recruitment Status
Completed
Study Start Date
January 2006 (undefined)
Primary Completion Date
September 2009 (Actual)
Study Completion Date
January 2010 (Actual)
3. Sponsor/Collaborators
Responsible Party, by Official Title
Principal Investigator
Name of the Sponsor
Oregon Health and Science University
4. Oversight
Data Monitoring Committee
No
5. Study Description
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.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Very Long-chain Acyl-CoA Dehydrogenase Deficiency, Trifunctional Protein Deficiency, Carnitine Palmitoyltransferase 2 Deficiency, Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency
Keywords
fatty acid oxidation disorders
7. Study Design
Primary Purpose
Treatment
Study Phase
Phase 1, Phase 2
Interventional Study Model
Parallel Assignment
Masking
None (Open Label)
Allocation
Randomized
Enrollment
13 (Actual)
8. Arms, Groups, and Interventions
Arm Title
High carbohydrate diet
Arm Type
Active Comparator
Arm Description
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.
Arm Title
High protein diet
Arm Type
Experimental
Arm Description
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.
Intervention Type
Behavioral
Intervention Name(s)
Diet counseling
Other Intervention Name(s)
low fat diet, high carbohydrate diet, high protein diet
Intervention Description
Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home.
Primary Outcome Measure Information:
Title
Energy Expenditure
Description
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.
Time Frame
change from baseline after 4 months of treatment
Secondary Outcome Measure Information:
Title
Body Composition
Description
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.
Time Frame
change from baseline to 4 months of treatment
10. Eligibility
Sex
All
Minimum Age & Unit of Time
7 Years
Maximum Age & Unit of Time
45 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
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:
pregnant
enrolled in another study that alters diet composition
cannot complete treadmill exercise study
12. IPD Sharing Statement
Learn more about this trial
High Protein Diet in Patients With Long-chain Fatty Acid Oxidation Disorders
We'll reach out to this number within 24 hrs