Myotonic Dystrophy, PrOteiN and Diet study
- Conditions
- myotonic dystrophy type 1, neuromuscular disease, metabolism, body composition
- Registration Number
- NL-OMON25314
- Lead Sponsor
- Academisch Ziekenhuis Maastricht
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Other
- Sex
- Not specified
- Target Recruitment
- 0
Inclusion Criteria
Age 18 years or older.
-Legally competent adult.
Exclusion Criteria
-Implantation of pacemaker or ICD device, a implantable insulin device, a neurostimulator, internal hearing aid or artificial heart valve.
-Implantation of orthopaedic prostheses, screws or plates.
Study & Design
- Study Type
- Observational non invasive
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method The main study endpoints are:<br /><br>- total energy expenditure measured by DLW and respiration chamber; <br /><br>- resting metabolic rate (RMR) measured by respiration chamber; <br /><br>- substrate selection at rest measured by respiration chamber.
- Secondary Outcome Measures
Name Time Method Secondary endpoints include:<br /><br>- Physical activity level (PAL) measured by accelerometer;<br /><br>- Whole-body skeletal muscle mass measured by MRI;<br /><br>- Quadriceps muscle cross-sectional area measured by CT; <br /><br>- Maximal grip strength measured by dynamometer;<br /><br>- Body composition expressed as leg and whole-body lean tissue mass measured by DEXA;<br /><br>- Muscle tissue morphology (including fibre size, split fibres, location of nucleii, regenerating and degenerating fibres, connective tissue, inflammatory cells, inclusions and storage material), muscle fibre differentiation, muscle fibre type specific cross-sectional area and vascularization, all determined by microscopically performed histological muscle biopsy assessment;<br /><br>- Muscle fiber biochemical analysis: cytochrome C oxidase enzym activity;<br /><br>- Maximal oxygen uptake capacity (VO2 max) and aerobic and anaerobic ventilatory thresholds determined by cycling exercise test;