Interaction of Muscle Dystrophy, Glucose Metabolism and Insulin Sensitivity

Study Overview
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Brief Summary
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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).

Conditions
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Various Forms of Skeletal Muscle Dystrophy

Intervention / Treatment
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* Diagnostic Test: oral glucose load at rest
* Diagnostic Test: oral glucose load and exercise


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 65 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| MD patients<br>patients with different forms of muscle dystrophy, for example facioscapulohumeral dystrophy dysferlinopathy caveolinopathy | Diagnostic Test: oral glucose load at rest<br>* 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.<br>Diagnostic Test: oral glucose load and exercise<br>* 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<br>|
| Controls<br>- healthy age- and sex-matched controls (10 men, 10 women) | Diagnostic Test: oral glucose load at rest<br>* 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.<br>Diagnostic Test: oral glucose load and exercise<br>* 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<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| muscle dialysate lactate | marker for anaerobic glycolysis | 15 minutes intervals after glucose over three hours | 
| muscle dialysate pyruvate | marker for aerobic glycolysis | 15 minutes intervals after glucose over three hours | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| muscle tissue perfusion | measured by microdialysis and ethanol dial / ethanol perf ratio (no dimensions) | 15 minutes intervals after glucose over three hours | 
| energy expenditure | measured by indirect calorimetry either at rest (canopy device) or during exercise (metabolic chamber) | 15 minutes intervals after glucose over three hours | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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muscle dystrophy, energy metabolism, calorimetry, metabolic chamber, microdialysis, exercise