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Non-osteogenic muscle hypertrophy in children with McArdle disease

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dc.contributor.author Rodríguez Gómez, Irene
dc.contributor.author Santalla Hernández, Alfredo
dc.contributor.author Díez Bermejo, Jorge
dc.contributor.author Munguía-Izquierdo, Diego
dc.contributor.author Alegre Durán, Luis María
dc.contributor.author Nogales-Gadea, Gisela
dc.contributor.author Arenas, Joaquín
dc.contributor.author Martín Casanueva, Miguel Ángel
dc.contributor.author Lucía Mulas, Alejandro
dc.contributor.author Ara, Ignacio
dc.date.accessioned 2018-06-20T07:57:26Z
dc.date.available 2018-06-20T07:57:26Z
dc.date.issued 2018
dc.identifier.citation Rodríguez-Gómez, I., Santalla, A., Díez-Bermejo, J., Munguía-Izquierdo, D., Alegre, L. M., Lucía Mulas, A., ... & Ara, I. (2018). Non-osteogenic muscle hypertrophy in children with McArdle disease. Journal of inherited metabolic disease. 41(6), 1037–1042. https://doi.org/10.1007/s10545-018-0170-7 spa
dc.identifier.issn 0141-8955
dc.identifier.issn 1573-2665
dc.identifier.uri http://hdl.handle.net/11268/7312
dc.description.abstract Introduction McArdle disease is an inborn disorder of muscle glycogen metabolism that produces exercise intolerance, and has been recently associated with low values of lean mass (LM) and bone mineral content (BMC) and density (BMD) in affected adults. Here we aimed to study whether this bone health problem begins in childhood. Methods Forty children and adolescents were evaluated: 10 McArdle disease and 30 control children (mean age of both groups, 13 ± 2y). Body composition was evaluated by dual-energy X-ray absorptiometry and creatine kinase (CK) levels were determined in the patients as an estimate of muscle damage. Results Legs bone mass was significantly lower in patients than in controls (−36% for BMC and −22% for BMD). Moreover, patients had significantly higher LM values in the legs than controls, whereas no difference was found for fat mass. CK levels were positively associated with LM in McArdle patients. A correlation was found between LM and BMD variables in the control group but not in McArdle patients. Conclusion We have identified a ‘non-osteogenic muscle hypertrophy’ in children with McArdle disease. This phenomenon warrants special attention since low osteogenesis at an early age predicts a high risk for osteoporosis later in life. spa
dc.description.sponsorship SIN FINANCIACIÓN spa
dc.language.iso eng spa
dc.subject.other McArdle disease spa
dc.subject.other Niños spa
dc.title Non-osteogenic muscle hypertrophy in children with McArdle disease spa
dc.type article spa
dc.description.impact 4.287 JCR (2018) Q1, 36/174 Genetics & Heredity, 33/145 Endocrinology & Metabolism; Q2, 38/136 Medicine, Research & Experimental, spa
dc.identifier.doi 10.1007/s10545-018-0170-7
dc.rights.accessRights closedAccess spa
dc.subject.uem Niños - Enfermedades spa
dc.subject.uem Músculos - Fisiología spa
dc.subject.unesco Enfermedad spa
dc.subject.unesco Fisiología humana spa
dc.subject.unesco Metabolismo spa
dc.description.filiation UEM spa
dc.relation.publisherversion http://ezproxy.universidadeuropea.es/login?url=http:/ /dx.doi.org/10.1007/s10545-018-0170-7 spa
dc.peerreviewed Si spa


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