{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T14:29:30Z","timestamp":1777645770069,"version":"3.51.4"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,6,10]],"date-time":"2019-06-10T00:00:00Z","timestamp":1560124800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Congenital myotonic dystrophy (CDM) is a rare neuromuscular disorder characterized by severe hypotonia and muscle weakness at birth that tends to improve with age. Understanding lean body and bone mass in this population has important research and clinical implications. The main objective of this study was to determine whether older children with CDM had muscle mass similar to healthy controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Thirty\u2010five patients with CDM (3\u201013 years old) were enrolled. We analyzed lean body mass (LBM) and bone mineral content using the mechanostat framework, which allows calculation of z\u2010scores for sex, age, and height.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All patients had low LBM z\u2010scores (muscle mass); however, higher LBM z\u2010score was positively correlated with age (<jats:italic>r<\/jats:italic> = 0.45, <jats:italic>P<\/jats:italic> = 0.006), showing that it is closer to normal in older patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Unlike other neuromuscular diseases, older children with CDM have a muscle mass closer to age\u2010matched controls, consistent with the clinical profile of increasing strength in childhood. <jats:italic>Muscle Nerve<\/jats:italic> <jats:bold>60<\/jats:bold>: 176\u2013179, 2019<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.26509","type":"journal-article","created":{"date-parts":[[2019,5,10]],"date-time":"2019-05-10T14:54:02Z","timestamp":1557500042000},"page":"176-179","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Body composition in patients with congenital myotonic dystrophy"],"prefix":"10.1002","volume":"60","author":[{"given":"Delia","family":"Ceballos\u2010S\u00e1enz","sequence":"first","affiliation":[{"name":"Paediatric Neurology Department Children's Hospital, London Health Science Centre  800 Commissioners Road East, PO Box 5012, London Ontario Canada N6A 5W9"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eugenio","family":"Zapata\u2010Aldana","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health, Max Rady College of Medicine, Rady Faculty of Health Sciences University of Manitoba  Winnipeg Manitoba Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephanie","family":"Singeris","sequence":"additional","affiliation":[{"name":"Paediatric Neurology Department Children's Hospital, London Health Science Centre  800 Commissioners Road East, PO Box 5012, London Ontario Canada N6A 5W9"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rhiannon","family":"Hicks","sequence":"additional","affiliation":[{"name":"Paediatric Neurology Department Children's Hospital, London Health Science Centre  800 Commissioners Road East, PO Box 5012, London Ontario Canada N6A 5W9"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3917-4257","authenticated-orcid":false,"given":"Nicholas","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Neurology Virginia Commonwealth University  Richmond Virginia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Craig","family":"Campbell","sequence":"additional","affiliation":[{"name":"Paediatric Neurology Department Children's Hospital, London Health Science Centre  800 Commissioners Road East, PO Box 5012, London Ontario Canada N6A 5W9"},{"name":"Department of Paediatrics, Clinical Neurological Sciences and Epidemiology Schulich School of Medicine and Dentistry, Western University  London Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,6,10]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(12)70204-1"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2012.12.070"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25482"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-160165"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(88)90064-0"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/BPO.0b013e3181982bf6"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002845"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pmr.2012.08.005"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.121493"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2004.06.009"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcb.25265"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2004.01.022"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.49.5.1457"},{"key":"e_1_2_7_15_1","unstructured":"SeemanN CampbellC. Mechanostat theory used to understand bone complications in children with Duchenne muscular dystrophy. Presented at the Muscle Study Group Meeting; September 21\u201323 2009; Buffalo New York. Available athttp:\/\/musclestudygroup.org\/wp-content\/uploads\/2016\/04\/Campbell.pdf. Accessed May 16 2019."},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.22099"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2016.11.005"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/165019701750300609"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmus.26509","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.26509","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.26509","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.26509","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,10]],"date-time":"2023-09-10T05:22:54Z","timestamp":1694323374000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.26509"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,6,10]]},"references-count":17,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,8]]}},"alternative-id":["10.1002\/mus.26509"],"URL":"https:\/\/doi.org\/10.1002\/mus.26509","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,6,10]]},"assertion":[{"value":"2018-08-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-05-07","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-06-10","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}