Metabolic Effects of Medium-Chain Fatty Acids in Patients With Medium-Chain Acyl-CoA Dehydrogenase Deficiency and Healthy Individuals

Metabolic Effects of Medium-Chain Fatty Acids in Patients With Medium-Chain Acyl-CoA Dehydrogenase Deficiency and Healthy Individuals

The aim of this project is to investigate the physiological effects of intake of long-chain fatty acids (LCFA) and medium-chain fatty acids (MCFA) in patients with Medium-Chain Acyl-CoA Dehydrogenase Deficiency (MCADD) and healthy individuals

Male and female MCADD patients and healthy individuals will be included in this study which is a randomized intervention study in a cross over design consisting of two separate test days.

The study involves two test days of four hours each, during which the participants ingests either medium-chain fatty acids (MCFA) or long-chain fatty acids (LCFA) in the form of triacylglycerol oils. The order of fatty acid oils ingested is determined by randomization.

Furthermore, a 3rd test day will be performed to evaluate ectopic fat deposition in the liver and the heart by using MRI scans, with no prior intake of MCFA or LCFA.

Inclusion Criteria MCADD patients:

• Male or female
• Age between 18-80 years old
• Diagnosed Medium-chain acyl-CoA dehydrogenase deficiency (MCADD

Inclusion Criteria Healthy Participants:

• Male or female
• Age between 18-80 years old

Exclusion Criteria both MCADD patients and healthy participants:

• Diabetes
• Kidney- or liver disease
• Use of beta blockers or diuretics
• Pregnant, lactating or planning to become pregnant within the study period
• Regular intake of substantial amounts of coconut oil, palm kernel oil or any other source abundant in MCFA
• Ongoing cancer treatment
• Metabolic or absorptive disorders, gastric bypass operation, or use of medication affecting metabolism or food absorption
• Inability, physically or mentally, to comply with the procedure required by the study protocol as evaluated by the primary investigator, study manager or clinical responsible