{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T22:35:14Z","timestamp":1773009314930,"version":"3.50.1"},"reference-count":29,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T00:00:00Z","timestamp":1694736000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100008084","name":"Neurof\u00f6rbundet","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008084","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Individuals with spinal muscular atrophy (SMA) are at risk of developing skeletal problems. This study investigated bone mineral density (BMD), bone turnover markers and motor function in children and adolescents with SMA type 2 and type 3 over a two\u2010year period. The effect of nusinersen was studied in a subgroup.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Single\u2010centre study, including 20 patients, 2\u201318\u2009years, of whom ten patients received nusinersen treatment. BMD was measured by dual\u2010energy X\u2010ray absorptiometry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All patients had low BMD levels at baseline; mean Z\u2010score \u22122.3 for total body less head (TBLH) and \u22122.9 for total hip left (THL). Significant correlations were found both at baseline and for the follow\u2010up change for motor function and Z\u2010scores (TBLH and THL). For the whole study group, reduced bone formation and unchanged bone resorption, assessed by bone\u2010specific alkaline phosphatase (BALP) (<jats:italic>p<\/jats:italic>\u2009=\u20090.0006, ES\u2009=\u2009\u20130.83) and C\u2010terminal cross\u2010linking telopeptide of type I collagen (CTX), respectively, were found over the study period. However, BALP decreased less in the nusinersen treatment group, which suggests a positive development on bone mass in these patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Bone health evaluation is important in follow\u2010up programmes for SMA patients. Further investigations are warranted for individuals on survival motor neuron\u2010targeted treatments.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.16974","type":"journal-article","created":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T08:25:23Z","timestamp":1694766323000},"page":"2589-2600","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Low bone mineral density and reduced bone\u2010specific alkaline phosphatase in 5q spinal muscular atrophy type 2 and type 3: A 2\u2010year prospective study of bone health"],"prefix":"10.1111","volume":"112","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9191-6927","authenticated-orcid":false,"given":"Anna\u2010Karin","family":"Kroksmark","sequence":"first","affiliation":[{"name":"Region V\u00e4stra G\u00f6taland Sahlgrenska University Hospital, Queen Silvia Children's Hospital  Gothenburg Sweden"},{"name":"Department of Health and Rehabilitation, Institute of Neuroscience and Physiology, Sahlgrenska Academy University of Gothenburg  Gothenburg Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lars","family":"Alberg","sequence":"additional","affiliation":[{"name":"Region V\u00e4stra G\u00f6taland Sahlgrenska University Hospital, Queen Silvia Children's Hospital  Gothenburg Sweden"},{"name":"Department of Paediatrics, Institute of Clinical Sciences, Sahlgrenska Academy University of Gothenburg  Gothenburg Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar","family":"Tulinius","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Institute of Clinical Sciences, Sahlgrenska Academy University of Gothenburg  Gothenburg Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2123-7838","authenticated-orcid":false,"given":"Per","family":"Magnusson","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry, and 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