Study of Muscle Wasting and Altered Metabolism in Patients With Myotonic Dystrophy
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|ClinicalTrials.gov Identifier: NCT00004769|
Recruitment Status : Completed
First Posted : February 25, 2000
Last Update Posted : January 28, 2013
OBJECTIVES: I. Examine the interrelationships between muscle wasting (phenotype), the degree of myotonic dystrophy (DM) gene expression (genotype) in patients with DM.
II. Characterize the insulin resistance in these patients. III. Assess the glucose uptake in the leg and forearm tissues of these patients.
IV. Determine the stability of the DM gene lesion in muscles over a 5-10 year period.
|Condition or disease|
|Myotonic Muscular Dystrophy|
PROTOCOL OUTLINE: Patients are placed on a meatless diet 3 days prior to study entry.
During the first 5-day hospital stay, patients receive an oral glucose tolerance test, an intravenous glucose tolerance test, and an intravenous infusion of insulin and glucose (dextrose) to determine the degree of insulin resistance. Patients also receive dual x-ray absorptiometry (DEXA) scan and total body potassium count to measure muscle mass. Patients undergo strength testing and physical fitness screening. A needle biopsy is performed to investigate the genetic alterations associated with this disease.
During the second 3-day hospital stay, patients receive an intravenous infusion of insulin, stable isotopic glucose, and stable isotopic glycerol.
During the third 3-day hospital stay, a catheter is placed in the femoral artery, femoral vein, and in each arm. Patients receive an infusion of stable isotopic glucose, stable isotopic phenylalanine, and insulin. Measurements of the balance of amino acids and glucose across the forearm and leg are completed. Green dye is infused to measure blood flow in the leg.
|Study Type :||Observational|
|Actual Enrollment :||130 participants|
|Observational Model:||Case Control|
|Official Title:||Myotonic Dystrophy:Muscle Wasting and Altered Metabolism|
|Study Start Date :||December 1993|
|Actual Primary Completion Date :||March 2000|
|Actual Study Completion Date :||March 2000|
Subjects with myotonic dystrophy
Disease controls 1
Subjects with FSHD
Disease controls 2
Subjects with CMT
- Quantitative myometry (QMT) [ Time Frame: Visit 1 ]
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00004769
|Study Chair:||Richard T. Moxley, III||University of Rochester|