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Keywords:

  • Charcot–Marie–Tooth disease;
  • cohort study;
  • epidemiology, fracture;
  • osteoporosis

ABSTRACT

Introduction: In this study we evaluated fracture risk in patients with Charcot–Marie–Tooth (CMT) disease. Methods: We conducted a retrospective cohort study using the UK Clinical Practice Research Datalink (1987–2012). Each patient with CMT disease was matched with up to 6 patients without a history of CMT disease. The outcome measure was fractures. Results: The risk of non-osteoporotic fracture was statistically significantly increased [adjusted hazard ratio (AHR) 1.47, 95% confidence interval (CI) 1.01–2.14], whereas risk of any and osteoporotic fracture did not reach statistical significance compared with control patients [AHR 1.31 (95% CI 0.98–1.74) and AHR 1.10 (95% CI 0.69–1.74), respectively]. Conclusions: CMT patients have a 1.5-fold increased risk for non-osteoporotic fracture. Studies with larger numbers of CMT patients and with additional data on CMT subtype, bone mineral density, and functional status should be performed to confirm a true association between CMT and an increased risk of fracture. Muscle Nerve 50: 919–924, 2014