Body composition, muscle strength, and physical function of patients with Bethlem myopathy and Ullrich congenital muscular dystrophy
| Autor: | Daniela Cocchi, Francesca Bruno, Maria Teresa Miscione, Riccardo Orsini, Cesare Faldini, Luciano Merlini, Claudio Ripamonti, Giuliana Nervuti, Massimo Pellegrini |
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| Přispěvatelé: | Miscione M.T., Bruno F., Ripamonti C., Nervuti G., Orsini R., Faldini C., Pellegrini M., Cocchi D., Merlini L. |
| Jazyk: | angličtina |
| Rok vydání: | 2013 |
| Předmět: |
Adult
Male medicine.medical_specialty Vital capacity Contracture Article Subject Ullrich congenital muscular dystrophy lcsh:Medicine Overweight BODY MASS lcsh:Technology General Biochemistry Genetics and Molecular Biology Muscular Dystrophies law.invention FEV1/FVC ratio Young Adult Absorptiometry Photon law Internal medicine medicine Humans Muscle Strength lcsh:Science General Environmental Science Adiposity Sclerosis lcsh:T Chemistry lcsh:R Bethlem myopathy General Medicine medicine.disease Cardiology Physical therapy Body Composition lcsh:Q Female medicine.symptom Body mass index Body composition Spirometer Research Article |
| Zdroj: | The Scientific World Journal The Scientific World Journal, Vol 2013 (2013) |
| Popis: | Objective.To determine the contributions of body mass, adiposity, and muscularity to physical function and muscle strength in adult patients with Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD).Materials and Methods.Evaluation involved one UCMD and 7 BM patients. Body composition was determined by body mass index (BMI) and dual-energy-X-ray-absorptiometry (DXA), muscle strength by dynamometry, physical function by the distance walked in 6 minutes (6MWD), forced vital capacity (FVC) by a spirometer.Results.Six participants were of normal weight and 2 overweight based on BMI; all were sarcopenic based on appendicular fat free mass index (AFFMI); and 7 were sarcopenic obese based on AFFMI and % fat mass. Average muscle strength was reduced below 50% of normal. The 6MWD was in BM patients 30% less than normal. FVC was reduced in 4 of the BM patients. Muscle strength had a good correlation with the physical function variables. Correlation between muscle strength and BMI was poor; it was very high with AFFMI. AFFMI was the best single explicator of muscle strength and physical function.Conclusion.Muscle mass determined by DXA explains most of the variability of the measures of muscle strength and physical function in patients with BM and UCMD. |
| Databáze: | OpenAIRE |
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