NCT02635269UnknownNot Applicable
Fat and Sugar Metabolism During Exercise in Patients With Metabolic Myopathy
Conditions
Interventions
Trial Snapshot
- Phase
- Not Applicable
- Sponsor
- Rigshospitalet, Denmark
- Enrollment
- 60
- Locations
- 2
- Primary Endpoint
- Total fat oxidation (rate of disappearance) during prolonged moderate intensity exercise. Measured as micro mol per kg per minute.
Study Overview
Brief Summary
This study aims to characterize the pathophysiological mechanisms of 21 different metabolic myopathies. The study will focus on exercise capacity and the metabolic derangement during exercise.
Detailed Description
Patients will be compared to a group of matched healthy control subjects (matched for age, gender, physical activity level and BMI) for comparison of the primary and secondary outcome measures.
Methods:
Each subject will go through 3 test days.
Test day 1:
- International Physical Activity Questionnaire (IPAQ). The results will be used to match the patients to control subjects.
- DEXA-scan. This is used to estimate the volume of distribution for stable isotope tracers.
- Maximal oxidative capacity test (max-test). On a cycle ergometer, subjects will exercise for approximately 15 minutes with increasing resistance until exhaustion. The peak oxidative and workload capacities will be compared to the healthy controls and will be used to find the workload that corresponds to 55-65 % of VO2max used in the following exercise tests.
- Blood samples. Samples will be drawn before exercise and at exhaustion and will be analyzed for the plasma concentrations of glucose, lactate, ammonia, creatine kinase and myoglobin.
Study Design
- Study Type
- Interventional
- Allocation
- Na
- Intervention Model
- Single Group
- Primary Purpose
- Basic Science
- Masking
- None
Eligibility Criteria
- Ages
- 15 Years to 80 Years (Child, Adult, Older Adult)
- Sex
- All
- Accepts Healthy Volunteers
- Yes
Inclusion Criteria
- •Men and women with genetically and/or biochemically verified LCHAD Deficiency
- •Healthy controls needs to be healthy to be included, evaluated by the investigator.
Exclusion Criteria
- •Competing conditions at risk of compromising the results of the study.
- •Pregnancy or breastfeeding
- •Cardiac or pulmonary disease contraindicating peak exercise testing or strenuous exercise.
- •Inability to understand the purpose of the trial or corporate for the conduction of the experiments.
- •Veins that are too difficult to puncture for blood sampling or insertion of intravenous catheters, evaluated by the investigator
- •Moderate to severe muscle weakness, that prevents the subject completing 10 minutes of cycle-ergometry exercise at 60-70 % of VO2peak, evaluated by the investigator.
- •Participation in other trials that may interfere with the results.
- •Intake of medications that may interfere with the results or may compromise exercise performance, as evaluated by the investigators.
- •Donation of blood within 30 days prior to conduction of the tests on test day 1.
Arms & Interventions
Sugar
Experimental
The subjects exercise on the cycle-ergometer until exhaustion or for a maximum of 1 hour at an intensity that corresponds to 60-65% of VO2max.
Intervention: Sugar (Other)
Outcomes
Primary Outcomes
Total fat oxidation (rate of disappearance) during prolonged moderate intensity exercise. Measured as micro mol per kg per minute.
Time Frame: Measured at at time (minutes) -120, -20, -10, 0, 10, 20, 30, 40, 50, 60. Zero is the indicatet start of 1 hour cycling test. Measured on day 2 of participation
Measured using stable isotope technique (\[U-13C\]-palmitate, \[1,1,2,3,3-2H5\]-glycerol and \[6.6-2H2\]-glucose ) and indirect calorimetry.
Secondary Outcomes
- Maximal oxidative capacity (mL*kg-1*minutes-1)(Day 1 of participation)
- Self-evaluated exertion (Rate of Perceived Exertion (RPE)) during prolonged exercise, with and without blood sugar maintained by an infusion of a 10% glucose solution. Measured using a Borg-scale (6-20).(During 1 hour cycling test 1 and 1 hour cycling test 2 on day 2 and 4 of participation.)
- Oxygen consumption rate (VO2) during prolonged exercise, with and without blood sugar maintained by an infusion of a 10% glucose solution. Measured in mL.(During 1 hour cycling test 1 and 1 hour cycling test 2 on day 2 and 4 of participation.)
- Total glucose oxidation (rate of disappearance) during prolonged moderate intensity exercise. Measured as micro mol per kg per minute.(Measured at at time (minutes) -120, -20, -10, 0, 10, 20, 30, 40, 50, 60. Zero is the indicatet start of 1 hour cycling test. Measured on day 2 of participation)
- Heart rate (HR) during prolonged exercise, with and without blood sugar maintained by an infusion of a 10% glucose solution. Measured in beats per minute.(During 1 hour cycling test 1 and 1 hour cycling test 2 on day 2 and 4 of participation.)
- Maximal workload capacity. Measured in watt(Day 1 of participation)
- Endurance measured as minutes:seconds(Day 2 and 4 of participation)
Investigators
Karen Lindhardt Madsen
MD
Rigshospitalet, Denmark
Study Sites (2)
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