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

University of Copenhagen30 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

BASIC_SCIENCE

Masking

SINGLE

Enrollment

Conditions

Medium-chain Acyl-CoA Dehydrogenase Deficiency

Interventions

Medium-Chain Fatty Acid (MCFA)OTHER

The participants will consume Medium-Chain Fatty Acid (MCFA) followed by Long-Chain Fatty Acid (LCFA)

Long-Chain Fatty Acid (LCFA)OTHER

The participants will consume Long-Chain Fatty Acid (LCFA) followed by Medium-Chain Fatty Acid (MCFA).

Eligibility

Sex: ALLMin age: 18 YearsMax age: 80 YearsHealthy volunteers:

Medical Language ↔ Plain English

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

Locations (1)

University of Copenhagen

Copenhagen, Denmark

RECRUITING

Outcomes

Primary Outcomes

Ketone Bodies

To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on circulating ketone body concentrations.