title: Fracture in Duchenne Muscular Dystrophy: Natural History and Vitamin D Deficiency.
abstract: The present study examined the natural history of fracture and vitamin D levels in Duchenne muscular dystrophy patients, who are vulnerable to osteoporosis and fractures. Retrospective analysis of a cohort of 48 Duchenne muscular dystrophy patients revealed that 43% of patients experienced ≥1 fracture. Fracture probabilities at ages 6, 9, 12, and 15 years were 4%, 9%, 31%, and 60% respectively, accelerating around the time of ambulation loss (mean age 11.8 ± 2.7 years). Chronic corticosteroid therapy was utilized in 69% of patients and was associated with all vertebral fractures. A history of vitamin D deficiency occurred in 84%, and 35% were currently deficient. Despite chronic vitamin D supplementation, 38% remained deficient. These results demonstrate that osteoporosis and fracture remain major concerns in Duchenne muscular dystrophy. Bone health should be optimized well before loss of ambulation, however current levels of vitamin D supplementation may be inadequate given high levels of deficiency.
authors.affiliation: Discipline of Paediatrics, School of Women's and Children's Health, UNSW Medicine, University of New South Wales, Sydney, Australia.
authors.forename: Nadia
authors.initials: N
authors.lastname: Perera
authors.affiliation: Department of Neurology, Sydney Children's Hospital, Randwick, Australia.
authors.forename: Hugo
authors.initials: H
authors.lastname: Sampaio
authors.affiliation: Discipline of Paediatrics, School of Women's and Children's Health, UNSW Medicine, University of New South Wales, Sydney, Australia Department of Endocrinology, Sydney Children's Hospital, Randwick, Australia.
authors.forename: Helen
authors.initials: H
authors.lastname: Woodhead
authors.affiliation: Discipline of Paediatrics, School of Women's and Children's Health, UNSW Medicine, University of New South Wales, Sydney, Australia Department of Neurology, Sydney Children's Hospital, Randwick, Australia m.farrar@unsw.edu.au.
authors.forename: Michelle
authors.initials: M
authors.lastname: Farrar
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001315:Australia; D015992:Body Mass Index; D015519:Bone Density; D002648:Child; D019587:Dietary Supplements; D050723:Fractures, Bone / Q000188:drug therapy / Q000453:epidemiology* / Q000503:physiopathology; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000188:drug therapy / Q000453:epidemiology* / Q000503:physiopathology; D010024:Osteoporosis / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology; D011239:Prednisolone / Q000627:therapeutic use; D011336:Probability; D012189:Retrospective Studies; D014807:Vitamin D / Q000627:therapeutic use; D014808:Vitamin D Deficiency / Q000178:diet therapy / Q000453:epidemiology* / Q000503:physiopathology
chemical_list: D000305:Adrenal Cortex Hormones; D014807:Vitamin D; D011239:Prednisolone
keywords: Duchenne muscular dystrophy; bone health; fracture; osteoporosis; vitamin D