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

Oregon Health & Science University (OHSU)

Status and phase: Completed; Phase 2; Phase 1

Conditions: Trifunctional Protein Deficiency; Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency; Very Long-chain Acyl-CoA Dehydrogenase Deficiency; Carnitine Palmitoyltransferase 2 Deficiency

Treatments: Behavioral: Diet counseling

Study type: Interventional

Funder types: Other

Identifiers

NCT01494051

DK71869-2

Details and patient eligibility

About

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.

Full 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.

Enrollment: 13 patients

Sex: All

Ages: 7 to 45 years old

Volunteers: No Healthy Volunteers

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

Trial design

Primary purpose: Treatment

Allocation: Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

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13 participants in 2 patient groups

High carbohydrate diet

Active Comparator group

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.

Treatment:

Behavioral: Diet counseling

High protein diet

Experimental group

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.

Treatment:

Behavioral: Diet counseling