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The purpose of the study is to compare the effects of L-citrulline and metformin and their combination therapy on muscle function and force in patients with Becker muscular dystrophy (BMD).
This is a single center, not-randomized open study. The study medication consists of L-citrulline and metformin administered orally; 5g citrulline and 500mg metformin tablets will be given 3 times daily.
Half of the patients will be treated with metformin during the first 6 weeks of the study. The other patients will receive initially L-citrulline for 6 weeks, before all patients will be treated with the combination therapy (metformin and L-citrulline) for another 6 week period.
The study lasts 12 weeks and includes the screening visit and 3 further visits at baseline, week 6 and week 12.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Metformin and Metformin & L-Citrulline | Experimental | 1500 mg/d metformin for 6 weeks, followed by 1500 mg/d metformin and 15 g/d L-citrulline for 6 further weeks |
|
| L-Citrulline and Metformin & L-Citrulline | Experimental | 15 g/d L-citrulline for 6 weeks, followed by 1500 mg/d metformin and 15 g/d L-citrulline for 6 further weeks |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Metformin and Metformin & L-Citrulline | Drug |
| ||
| L-Citrulline and Metformin & L-Citrulline |
| Measure | Description | Time Frame |
|---|---|---|
| Mean change of motor function measure (MFM) D1 subscore after 6 and 12 weeks | week 6 and week 12 |
| Measure | Description | Time Frame |
|---|---|---|
| MFM total score and six minute walking distance (6MWD) | week 6 and week 12 | |
| Change of muscle fat content (MFC) (assessed by MRI) | week 6 and week 12 | |
| Change of muscle metabolism (assessed by dual energy x-ray absorptiometry (DEXA) and indirect calorimetry) |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Dirk Fischer, MD | University Children's Hospital Basel | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University Children's Hospital | Basel | Canton of Basel-City | 4031 | Switzerland |
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| ID | Term |
|---|---|
| D020388 | Muscular Dystrophy, Duchenne |
| ID | Term |
|---|---|
| D009136 | Muscular Dystrophies |
| D020966 | Muscular Disorders, Atrophic |
| D009135 | Muscular Diseases |
| D009140 | Musculoskeletal Diseases |
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| ID | Term |
|---|---|
| D008687 | Metformin |
| D002956 | Citrulline |
| ID | Term |
|---|---|
| D001645 | Biguanides |
| D006146 | Guanidines |
| D000578 | Amidines |
| D009930 | Organic Chemicals |
| D000599 |
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| Drug |
|
| week 6 and week 12 |
| Change of laboratory parameters (oxidative and nitrosative stress) | week 6 and week 12 |
| D009468 | Neuromuscular Diseases |
| D009422 | Nervous System Diseases |
| D040181 | Genetic Diseases, X-Linked |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| Amino Acids, Diamino |
| D000596 | Amino Acids |
| D000602 | Amino Acids, Peptides, and Proteins |