Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular Dystrophy
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 compo...
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| Format: | Article |
| Language: | English |
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Wiley
2013-01-01
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| Series: | The Scientific World Journal |
| Online Access: | http://dx.doi.org/10.1155/2013/152684 |
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| author | Maria Teresa Miscione Francesca Bruno Claudio Ripamonti Giuliana Nervuti Riccardo Orsini Cesare Faldini Massimo Pellegrini Daniela Cocchi Luciano Merlini |
| author_facet | Maria Teresa Miscione Francesca Bruno Claudio Ripamonti Giuliana Nervuti Riccardo Orsini Cesare Faldini Massimo Pellegrini Daniela Cocchi Luciano Merlini |
| author_sort | Maria Teresa Miscione |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-bef15e34f6e943f7874fb95da1b81d5f |
| institution | Kabale University |
| issn | 1537-744X |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | The Scientific World Journal |
| spelling | doaj-art-bef15e34f6e943f7874fb95da1b81d5f2025-02-03T05:46:47ZengWileyThe Scientific World Journal1537-744X2013-01-01201310.1155/2013/152684152684Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular DystrophyMaria Teresa Miscione0Francesca Bruno1Claudio Ripamonti2Giuliana Nervuti3Riccardo Orsini4Cesare Faldini5Massimo Pellegrini6Daniela Cocchi7Luciano Merlini8Department of Orthopaedics, Istituto Ortopedico Rizzoli, IRCCS, 40136 Bologna, ItalyDepartment of Statistical Sciences, University of Bologna, 40126 Bologna, ItalyMedicina Generale, Istituto Ortopedico Rizzoli, IRCCS, 40136 Bologna, ItalyDirezione Sanitaria, Istituto Ortopedico Rizzoli, IRCCS, 40136 Bologna, ItalyDepartment of Orthopaedics, Istituto Ortopedico Rizzoli, IRCCS, 40136 Bologna, ItalyDepartment of Orthopaedics, Istituto Ortopedico Rizzoli, IRCCS, 40136 Bologna, ItalyDepartment of Public Health Sciences, University of Modena and Reggio Emilia, 41122 Modena, ItalyDepartment of Statistical Sciences, University of Bologna, 40126 Bologna, ItalyLaboratory of Musculoskeletal Cell Biology, Istituto Ortopedico Rizzoli, IRCCS, 40136 Bologna, ItalyObjective. 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.http://dx.doi.org/10.1155/2013/152684 |
| spellingShingle | Maria Teresa Miscione Francesca Bruno Claudio Ripamonti Giuliana Nervuti Riccardo Orsini Cesare Faldini Massimo Pellegrini Daniela Cocchi Luciano Merlini Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular Dystrophy The Scientific World Journal |
| title | Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular Dystrophy |
| title_full | Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular Dystrophy |
| title_fullStr | Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular Dystrophy |
| title_full_unstemmed | Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular Dystrophy |
| title_short | Body Composition, Muscle Strength, and Physical Function of Patients with Bethlem Myopathy and Ullrich Congenital Muscular Dystrophy |
| title_sort | body composition muscle strength and physical function of patients with bethlem myopathy and ullrich congenital muscular dystrophy |
| url | http://dx.doi.org/10.1155/2013/152684 |
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