Fractures and bone health monitoring in boys with Duchenne muscular dystrophy managed within the Scottish Muscle Network

Joseph, S. et al. (2019) Fractures and bone health monitoring in boys with Duchenne muscular dystrophy managed within the Scottish Muscle Network. Neuromuscular Disorders, 29(1), pp. 59-66. (doi: 10.1016/j.nmd.2018.09.005) (PMID:30473133)

Abstract

There are limited reports of radiologically confirmed fractures and bone health monitoring in with Duchenne muscular dystrophy. We performed a retrospective study of 91 boys, with a median age of 11.0 years, who are currently managed in Scotland with the aim to assess the frequency of radiologically confirmed fractures and report on bone health monitoring in relation to International Care Consensus Guidance. Of these boys, 59 (65%) were receiving glucocorticoid (GC) therapy and 23 (25%) had received previous treatment. Of those currently on GC, 37 (63%) had an assessment of bone mineral density and none had routine imaging for vertebral fractures during the study period. Of the 91 boys, 44 (48%) had sustained at least one symptomatic radiographically confirmed fracture. The probability of sustaining a first symptomatic fracture was 50% by 12.8 years old (95%CI: 12.1, 13.6). The most common sites for non-vertebral fracture were the femur and tibia. In this review of boys with DMD, almost half had sustained at least one radiologically confirmed symptomatic fracture. There is a need for standardized bone health monitoring in DMD that includes routine imaging of the spine to identify vertebral fractures, given the persistence of insult to the skeleton in these boys.

• Item Type: Articles
• Additional Information: SJ is supported by a clinical research training fellowship funded by the Chief Scientist Office Scotland, Muscular Dystrophy UK and Action Duchenne.
• Keywords: Bone mineral density, glucocorticoid, osteoporosis, skeletal fragility, vitamin D.
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Wong, Dr Jarod and Horrocks, Dr Iain and Wang, Cunyi and Ahmed, Professor Syed Faisal and Joseph, Dr Shuko
• Authors: Joseph, S., Wang, C., Di Marco, M., Horrocks, I., Abu-Arafeh, I., Baxter, A., Cordeiro, N., McLellan, L., McWilliam, K., Naismith, K., Stephen, E., Ahmed, S. F., and Wong, S. C.
• College/School: College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing; College of Science and Engineering > School of Mathematics and Statistics
• Journal Name: Neuromuscular Disorders
• Publisher: Elsevier
• ISSN: 0960-8966
• ISSN (Online): 1873-2364
• Published Online: 25 September 2018
• Copyright Holders: Copyright © 2018 Elsevier B.V.
• First Published: First published in Neuromuscular Disorders 29(1): 59-66
• Publisher Policy: Reproduced in accordance with the publisher copyright policy