The time to and determinants of first fractures in boys with Duchenne muscular dystrophy

Abstract

Summary

Boys with vertebral fractures (VF) identified through routine spine radiographs had milder, less symptomatic, and fewer VF compared to those diagnosed with VF following consultation for back pain. Spontaneous (i.e., medication-unassisted) reshaping of fractured vertebral bodies was absent. Long bone fractures were present even before Duchenne muscular dystrophy (DMD) diagnosis in some boys.

Introduction

The objective of the study was to determine the time to and characteristics of first fractures in Duchenne muscular dystrophy.

Methods

This study was a retrospective longitudinal study of 30 boys with DMD <18 years. Boys were classified into four groups according to their first fracture: those with VF identified on routine lateral spine radiographs, those with VF diagnosed following consultation for back pain, those with long bone fractures, and those without fractures.

Results

Compared to boys diagnosed with VF as their initial fracture following consultation for back pain, those with VF surveillance radiographs had shorter durations of glucocorticoid (GC) therapy at the time of VF diagnosis (median 1.6 versus 5.3 years, p < 0.01), higher areal (mean ± standard deviation −1.4 ± 0.7 versus −3.1 ± 0.8, p = 0.01), and volumetric (−0.3 ± 0.5 versus −2.6 ± 0.8, p < 0.01) lumbar spine bone mineral density Z-scores, as well as fewer VF (median 1.4 versus 5.2 per person, p < 0.01) and a lower median spinal deformity index (median 1.5 versus 9.5, p < 0.01). Vertebral body reshaping following VF was not observed. Ten boys sustained a long bone fracture as their first fracture at a mean age of 8.9 ± 4.0 years; four of these boys later sustained a total of 27 incident VF.

Conclusions

Routine lateral spine radiographs led to detection of VF in their earlier stages, vertebral body reshaping following VF was absent, and VF were frequent after the first long bone fracture. These results support the inclusion of a lateral spine radiograph starting at the time of GC initiation as part of routine bone health monitoring in DMD.

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Acknowledgments

This work was supported by the following programs and organizations: (1) LMW: The Canadian Institutes for Health Research New Investigator Program, The Canadian Child Health Clinician Scientist Program, the Children’s Hospital of Eastern Ontario (CHEO) Research Institute, The University of Ottawa Research Chair Program and the CHEO Departments of Pediatrics and Surgery; (2) JM: The CHEO Research Institute. (3) VNK: The CHEO Department of Surgery.

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Correspondence to L. M. Ward.

Ethics declarations

The study was approved by the CHEO Research Ethics Board.

Conflicts of interest

Leanne M. Ward has been a consultant to Novartis and Amgen Pharmaceuticals. Jinhui Ma, Hugh J McMillan, Gülay Karagüzel, Claire Goodin, Julie Wasson, Mary Ann Matzinger, Poppy DesClouds, Danielle Cram, Marika Page, Victor N Konji and Brian Lentle declare that they have no conflict of interest.

Funding

University of Ottawa Research Chair Programme and the CHEO Departments of Pediatrics and Surgery.

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Ma, J., McMillan, H.J., Karagüzel, G. et al. The time to and determinants of first fractures in boys with Duchenne muscular dystrophy. Osteoporos Int 28, 597–608 (2017). https://doi.org/10.1007/s00198-016-3774-5

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Keywords

  • Duchenne muscular dystrophy
  • First fractures
  • Glucocorticoids
  • Osteoporosis