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

Phase 1

Completed

• Conditions: Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency; Very Long-chain Acyl-CoA Dehydrogenase Deficiency; Trifunctional Protein Deficiency; Carnitine Palmitoyltransferase 2 Deficiency
• Interventions: Behavioral: Diet counseling

• Registration Number: NCT01494051
• Lead Sponsor: Oregon Health and Science University

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 Timeline

• Study Start: 2006-01
• Primary Completion: 2009-09
• Study Completion: 2010-01
• Study First Submitted: 2011-12-14
• Study First Submitted QC: 2011-12-15
• Study First Posted: 2011-12-16
• Status Verified: 2020-04
• Results First Submitted: 2020-04-07
• Results First Submitted QC: 2020-04-07
• Last Update Submitted: 2020-04-20
• Results First Posted: 2020-04-21
• Last Update Posted: 2020-04-29

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 13

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

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
High protein diet	Diet counseling	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.
High carbohydrate diet	Diet counseling	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.

Primary Outcome Measures

Name	Time	Method
Energy Expenditure	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

Name	Time	Method
Body Composition	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.