Various Forms of Skeletal Muscle Dystrophy
Conditions
Keywords
muscle dystrophy, energy metabolism, calorimetry, metabolic chamber, microdialysis, exercise
Brief summary
The aim of this project to characterize systemic and organ-specific (subcutaneous adipose tissue and skeletal muscle) metabolic responses at rest and during standardized, moderate exercise after an oral glucose load in patients with various geneticaly heritable foms of skeletal muscle dystrophies. Metabolic responses will be monitored by indirect calorimetry (canopy and/or metabolic chamber at rest and during exercise) and microdialysis (adipose tissue and skeletal muscle at rest) before and after an oral glucose load (75 g).
Interventions
oral glucose load (75 g) after an 12h overnight fast at rest accompanied by blood sampling and dialysate sampling (adipose tissue and skeletal muscle), and calorimetry (canopy) over three hours.
oral glucose load (75 g) after an 12h overnight fast followed by a defined (0.5 W / kg), moderate bicycle exercise i a metabolic chamber
Sponsors
Study design
Eligibility
Inclusion criteria
* controls: healthy, no metabolic, endocrine, neurologic or other diseases * patients: genetically confirmed diagnosis of muscle dystrophy
Exclusion criteria
* known hemorrhagic diseases / coagulopathies * intake of oral anti-coagulant drugs (control of PTT, INR and platelet count before study entry) * insulin-dependent Diabetes mellitus * pregnancy and lactation period * chronic use of non-steroidal antiphlogistics * alcohol and drug abuse * Claustrophobia • gleichzeitige Teilnahme an einer anderen Studie; Teilnahme an einer Studie vor weniger als zwei Monaten Unfähigkeit, die mündlichen und schriftlichen Informationen zur Studie zu verstehen und das schriftliche Einverständnis zu geben
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| muscle dialysate lactate | 15 minutes intervals after glucose over three hours | marker for anaerobic glycolysis |
| muscle dialysate pyruvate | 15 minutes intervals after glucose over three hours | marker for aerobic glycolysis |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| muscle tissue perfusion | 15 minutes intervals after glucose over three hours | measured by microdialysis and ethanol dial / ethanol perf ratio (no dimensions) |
| energy expenditure | 15 minutes intervals after glucose over three hours | measured by indirect calorimetry either at rest (canopy device) or during exercise (metabolic chamber) |
Countries
Germany