{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,11]],"date-time":"2026-04-11T15:33:31Z","timestamp":1775921611662,"version":"3.50.1"},"reference-count":22,"publisher":"Korean Society for Bone and Mineral Research","issue":"4","license":[{"start":{"date-parts":[[2023,11,30]],"date-time":"2023-11-30T00:00:00Z","timestamp":1701302400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["e-jbm.org"],"crossmark-restriction":true},"short-container-title":["J Bone Metab"],"published-print":{"date-parts":[[2023,11,30]]},"abstract":"<jats:p>Background: Spinal muscular atrophy (SMA) is a group of rare, inherited neuromuscular disorders. Bone health is often a neglected issue in children with SMA. This study aimed to evaluate the bone health status of children with SMA in Hong Kong.Methods: This retrospective study included children with SMA who were managed in the Neuromuscular Disorder Clinics of 2 quaternary centers in Hong Kong. Bone health status was assessed by fracture history, bone mineral density (BMD) measured by dual energy X-ray absorptiometry, and serum 25-hydroxy-vitamin D (25[OH]D) level.Results: Thirty-two children were included (males, 12). The median age was 10.8 years. BMD assessments were performed in 17 patients (SMA type 1=2, type 2=8, type 3=7). Low BMD was observed in 16 out of 17 patients. Four had a history of long bone fractures and were started on bisphosphonates. SMA types, age at last visit, sex, ambulation, and 25(OH)D level were not associated with fracture history or BMD Z-scores. Only one fulfilled the 2019 International Society for Clinical Densitometry (ISCD) pediatric definition of osteoporosis, with both low BMD and a history of clinically significant fracture.Conclusions: Children with SMA on disease-modifying treatments commonly had Low BMD and a history of fractures, but osteoporosis was uncommon according to the 2019 ISCD pediatric definition. A special definition of osteoporosis may be needed for this high-risk group.<\/jats:p>","DOI":"10.11005\/jbm.2023.30.4.319","type":"journal-article","created":{"date-parts":[[2023,12,4]],"date-time":"2023-12-04T23:58:15Z","timestamp":1701734295000},"page":"319-327","update-policy":"https:\/\/doi.org\/10.11005\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Bone Health Status of Children with Spinal Muscular Atrophy"],"prefix":"10.11005","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7897-716X","authenticated-orcid":true,"given":"Joanna 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