{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,5]],"date-time":"2026-07-05T08:31:20Z","timestamp":1783240280499,"version":"3.54.6"},"reference-count":0,"publisher":"Georg Thieme Verlag KG","issue":"06","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropediatrics"],"published-print":{"date-parts":[[2018,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>To evaluate the potential bone defect in neuromuscular diseases, we conducted a longitudinal study including three groups of patients: 14 Duchenne muscular dystrophies (DMD) and 2 limb-girdle muscular dystrophies (LGMD); 3 Becker muscular dystrophies (BeMD) and 7 spinal muscular atrophies (SMA). Yearly osteodensitometries assessed body composition and bone mineral density (BMD) associated with bone markers and leptin. Along the 7-year study, 107 osteodensitometries showed that bone status evolved to osteopenia in most patients except BeMD. When analyzing the crude values, BMD improved with age in BeMD and SMA but not in DMD\/LGMD. The correlation using the Z-scores displayed a decrease in BMD with age in DMD\/LGMD for all regions, in SMA at total body less head, whereas BMD increased in BeMD at lumbar spine. As observed in healthy persons, muscular mass and bone tissue were significantly correlated. Glucocorticoids were deleterious on trabecular and cortical bone. Leptin was high in most patients and correlated to fat mass and bone parameters. This study confirms a secondary bone defect in neuromuscular diseases, further confirming the functional relationship between bone and muscle and arguing for regular bone follow-up in patients to prevent fracture risk. Adipose tissue seems to interfere with bone remodeling in neuromuscular diseases.<\/jats:p>","DOI":"10.1055\/s-0038-1666846","type":"journal-article","created":{"date-parts":[[2018,7,6]],"date-time":"2018-07-06T22:55:43Z","timestamp":1530917743000},"page":"397-400","source":"Crossref","is-referenced-by-count":5,"title":["Secondary Bone Defect in Neuromuscular Diseases in Childhood: A Longitudinal \u201cMuscle-Bone Unit\u201d Analysis"],"prefix":"10.1055","volume":"49","author":[{"given":"C.","family":"Ribstein","sequence":"first","affiliation":[{"name":"Centre de comp\u00e9tence des neuropathies de cause rare et centre de comp\u00e9tence des pathologies neuro-musculaires, Service de G\u00e9n\u00e9tique M\u00e9dicale, H\u00f4pital Estaing, Clermont-Ferrand, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Courteix","sequence":"first","affiliation":[{"name":"Laboratoire des Adaptations M\u00e9taboliques \u00e0 l'Exercice en Conditions Physiologiques et Pathologiques (AME2P), Universit\u00e9 Clermont Auvergne, Clermont-Ferrand, France"},{"name":"CRNH Auvergne, Clermont-Ferrand, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Rabiau","sequence":"first","affiliation":[{"name":"Centre de comp\u00e9tence des neuropathies de cause rare et centre de comp\u00e9tence des pathologies neuro-musculaires, Service de G\u00e9n\u00e9tique M\u00e9dicale, H\u00f4pital Estaing, Clermont-Ferrand, France"},{"name":"INSERM-CIC 1405, Unit\u00e9 CRECHE, CHU Clermont-Ferrand, Clermont-Ferrand, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Bommelaer","sequence":"first","affiliation":[{"name":"Centre de comp\u00e9tence des neuropathies de cause rare et centre de comp\u00e9tence des pathologies neuro-musculaires, Service de G\u00e9n\u00e9tique M\u00e9dicale, H\u00f4pital Estaing, Clermont-Ferrand, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y.","family":"Bourdeau","sequence":"first","affiliation":[{"name":"Centre de comp\u00e9tence des neuropathies de cause rare et centre de comp\u00e9tence des pathologies neuro-musculaires, Service de G\u00e9n\u00e9tique M\u00e9dicale, H\u00f4pital Estaing, Clermont-Ferrand, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Pereira","sequence":"first","affiliation":[{"name":"Unit\u00e9 de Biostatistiques (DRCI), CHU Clermont-Ferrand, Clermont-Ferrand, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Sarret","sequence":"additional","affiliation":[{"name":"Centre de comp\u00e9tence des neuropathies de cause rare et centre de comp\u00e9tence des pathologies neuro-musculaires, Service de G\u00e9n\u00e9tique M\u00e9dicale, H\u00f4pital Estaing, Clermont-Ferrand, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2018,7,6]]},"container-title":["Neuropediatrics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0038-1666846.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,11,12]],"date-time":"2018-11-12T23:33:22Z","timestamp":1542065602000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0038-1666846"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,7,6]]},"references-count":0,"journal-issue":{"issue":"06","published-online":{"date-parts":[[2018,11,12]]},"published-print":{"date-parts":[[2018,12]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0038-1666846","relation":{},"ISSN":["0174-304X","1439-1899"],"issn-type":[{"value":"0174-304X","type":"print"},{"value":"1439-1899","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,7,6]]}}}