High Protein Diet in Patients With Long-chain Fatty Acid Oxidation Disorders

• ClinicalTrials.gov Identifier: NCT01494051
• Recruitment Status: Completed
• First Posted: December 16, 2011
• Results First Posted: April 21, 2020
• Last Update Posted: April 29, 2020
• Sponsor: Oregon Health and Science University
• Information provided by (Responsible Party): Melanie B Gillingham, Oregon Health and Science University

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.

Condition or disease	Intervention/treatment	Phase
Very Long-chain Acyl-CoA Dehydrogenase Deficiency; Trifunctional Protein Deficiency; Carnitine Palmitoyltransferase 2 Deficiency; Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency	Behavioral: Diet counseling	Phase 1; Phase 2

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 (Clinical Trial)
• Actual Enrollment: 13 participants
• Allocation: Randomized
• Intervention Model: Parallel Assignment
• Masking: None (Open Label)
• Primary Purpose: Treatment
• Official Title: Fatty Acid Oxidation and Body Weight Regulation in Long-chain Fatty Acid Oxidation Disorders.
• Study Start Date: January 2006
• Actual Primary Completion Date: September 2009
• Actual Study Completion Date: January 2010

Arms and Interventions

Arm	Intervention/treatment
Active Comparator: 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.	Behavioral: Diet counseling; Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home.; Other Names: low fat diet; high carbohydrate diet; high protein diet
Experimental: 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.	Behavioral: Diet counseling; Subjects counseled how to follow either the high carbohydrate diet or the high protein diet for 4 months at home.; Other Names: low fat diet; high carbohydrate diet; high protein diet

Outcome Measures

Primary Outcome Measures :

1. 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 Outcome Measures :

1. Body Composition [ Time Frame: change from baseline to 4 months of treatment ]
   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.

Eligibility Criteria

• Ages Eligible for Study: 7 Years to 45 Years (Child, Adult)
• Sexes Eligible for Study: All
• Accepts Healthy Volunteers: No

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

Contacts and Locations

No Contacts or Locations Provided

More Information

• Responsible Party: Melanie B Gillingham, Assistant Professor, Oregon Health and Science University
• ClinicalTrials.gov Identifier: NCT01494051
• Other Study ID Numbers: DK71869-2
• First Posted: December 16, 2011
• Results First Posted: April 21, 2020
• Last Update Posted: April 29, 2020
• Last Verified: April 2020

Keywords provided by Melanie B Gillingham, Oregon Health and Science University:

fatty acid oxidation disorders

Additional relevant MeSH terms:

Protein Deficiency; Deficiency Diseases; Malnutrition; Nutrition Disorders