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

Fatty Acid Oxidation and Body Weight Regulation in Long-chain Fatty Acid Oxidation Disorders.

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.

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