Medium-chain Acyl-CoA Dehydrogenase Deficiency
Conditions
Keywords
MCADD, Lipid metabolism, Ketone bodies, Medium-Chain Fatty Acid (MCFA), Long-Chain Fatty Acid (LCFA)
Brief summary
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
Detailed description
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.
Interventions
OTHERMedium-Chain Fatty Acid (MCFA)
The participants will consume Medium-Chain Fatty Acid (MCFA) followed by Long-Chain Fatty Acid (LCFA)
OTHERLong-Chain Fatty Acid (LCFA)
The participants will consume Long-Chain Fatty Acid (LCFA) followed by Medium-Chain Fatty Acid (MCFA).
Sponsors
University of Copenhagen
Copenhagen Neuromuscular Center
Center of Inherited Metabolic Diseases, Copenhagen University Hospital Rigshospitalet
Section of Molecular Physiology, Department of Nutrition, Exercise and Sports, Faculty of Science, University of Copenhagen, Denmark
Study design
Allocation
RANDOMIZED
Intervention model
CROSSOVER
Primary purpose
BASIC_SCIENCE
Masking
SINGLE (Subject)
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
Yes
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Ketone Bodies | During both individual test days | To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on circulating ketone body concentrations. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Resting metabolic rate | During both individual test days | To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on resting metabolic rate. |
| Respiratory exchange ratio | During both individual test days | To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on respiratory exchange ratio. |
| Vasodilator Function | During both individual test days | To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on vasodilator function. |
| Fatty Acid Composition | During both individual test days | To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on fatty acid composition. |
| Metabolites | During both individual test days | To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on circulating ketone body concentrations. |
| Hepatokines | During both individual test days | To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on circulating hepatokine concentrations. |
| Beta cell function | During both individual test days | To measure the effect of Medium-Chain Fatty Acid (MCFA) and Long-Chain Fatty Acid (LCFA) on Beta cell function. |
| Ectopic Fat Deposition | On a separate 3rd test day | MRI scans will be performed to evaluate ectopic fat deposition in the liver and the heart with no prior intake of MCFA or LCFA. |
Countries
Denmark
Contacts
CONTACTKasper S Jørgensen, PhD student
CONTACTAndreas M Fritzen, Associate Professor
PRINCIPAL_INVESTIGATORAndreas M Fritzen, Associate Professor
University of Copenhagen
Outcome results
None listed