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Biphosphonates in the treatment of low bone mass in steroid-treated boys with duchenne's muscular dystrophy
  1. R Kulshrestha1,
  2. E Hoyle2,
  3. R Quinlivan1
  1. 1Paediatrics, RJAH Orthopaedic and District Hospital, Oswestry, UK
  2. 2Paediatrics, Sheffield University, Sheffield, UK

Abstract

Background Osteoporosis and an increased fracture risk are common in Duchenne muscular dystrophy (DMD). Corticosteroid therapy given to prolong mobility increases the severity of osteoporosis and risk of fracture which can have serious functional consequences.

Aims To examine biphosphonate effect on bone mineral density (BMD) in steroid treated boys with DMD and low BMD.

Methods The authors retrospectively reviewed case notes of 22 patients with DMD for details of fractures, BMD z-scores at the lumbar spine, regimen cumulative corticosteroid use and biphosphonate treatment.

Results 19/22 patients were treated with corticosteroids. Mean age was 11.7 years (6.3–21.3 years) and the mean duration of steroid intake was 4.1 years (0.5–6 years). 7/19 patients taking corticosteroids had 10 incidents of bone fractures. Four incidents (40%) were vertebral fractures and the rest were long bone fractures. The mean spinal z-score of patients on steroids was −1.75 while for those without steroid treatment was −1.8 (p value: 0.9). Six patients on steroids were treated with oral risedronate 35 mg every 2 weeks of which five had good compliance. Only one incident of fracture was recorded while the patients were on risedronate (p value: 0.05). The mean spinal z-scores before and after residronate treatment was −2.04 and −1.32, respectively (p value: 0.242).

Conclusions The boys with DMD treated with steroids are at risk of bone fractures, particularly the vertebrae. The incidence of vertebral fractures in this group is lower than that quoted by the previous authors (75%). The BMD z-score was almost same for patients with and without steroid treatment. In steroid-treated boys, biphosphonate had a positive effect on reduction in the incidence of fractures and on BMD z-score though the improvement is not statistically significant.

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