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

Shuko Joseph, Cunyi Wang, Marina Di Marco, Iain Horrocks, Ishaq Abu-Arafeh, Alex Baxter, Nuno Cordeiro, Linda McLellan, Kenneth McWilliam, Karen Naismith, Elma Stephen, S. Faisal Ahmed, Sze Choong Wong (Lead / Corresponding author)

Research output: Contribution to journalArticle

8 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)59-66
Number of pages8
JournalNeuromuscular Disorders
Volume29
Issue number1
Early online date25 Sep 2018
DOIs
Publication statusPublished - Jan 2019

Keywords

  • Bone mineral density
  • Glucocorticoid
  • Osteoporosis
  • Skeletal fragility
  • Vitamin D

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    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., & Wong, S. C. (2019). Fractures and bone health monitoring in boys with Duchenne muscular dystrophy managed within the Scottish Muscle Network. Neuromuscular Disorders , 29(1), 59-66. https://doi.org/10.1016/j.nmd.2018.09.005