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Risk factors associated with prevalent vertebral fractures in Duchenne muscular dystrophy.
Phung, Kim; McAdam, Laura; Ma, Jinhui; McMillan, Hugh J; Jackowski, Stefan; Scharke, Maya; Matzinger, Mary-Ann; Shenouda, Nazih; Koujok, Khaldoun; Jaremko, Jacob L; Smit, Kevin; Walker, Scott; Hartigan, Colleen; Khan, Nasrin; Konji, Victor N; MacLeay, Lynn; Page, Marika; Sykes, Elizabeth; Robinson, Marie-Eve; Alos, Nathalie; Cummings, Elizabeth A; Ho, Josephine; Sbrocchi, Anne Marie; Stein, Robert; Saleh, David; Craven, B Catharine; Dang, Utkarsh J; Siminoski, Kerry; Rauch, Frank; Ward, Leanne M.
Afiliação
  • Phung K; Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada.
  • McAdam L; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Ma J; Holland Bloorview Kids Rehabilitation Hospital, Toronto, ON, Canada.
  • McMillan HJ; Department of Health Research Methods, Evidence, and Impact, McMaster University, Hamilton, ON, Canada.
  • Jackowski S; Montreal Children's Hospital, McGill University, Montreal, QC, Canada.
  • Scharke M; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Matzinger MA; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Shenouda N; Department of Medical Imaging, University of Ottawa, Ottawa, ON, Canada.
  • Koujok K; Department of Medical Imaging, University of Ottawa, Ottawa, ON, Canada.
  • Jaremko JL; Department of Medical Imaging, University of Ottawa, Ottawa, ON, Canada.
  • Smit K; Department of Radiology and Diagnostic Imaging, University of Alberta, Edmonton, AB, Canada.
  • Walker S; Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada.
  • Hartigan C; Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada.
  • Khan N; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Konji VN; Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada.
  • MacLeay L; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Page M; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Sykes E; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Robinson ME; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Alos N; Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada.
  • Cummings EA; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Ho J; The Ottawa Pediatric Bone Health Research Group, Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
  • Sbrocchi AM; Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada.
  • Stein R; CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada.
  • Saleh D; IWK Health Centre, Dalhousie University, Halifax, NS, Canada.
  • Craven BC; Alberta Children's Hospital, University of Calgary, Calgary, AB, Canada.
  • Dang UJ; Montreal Children's Hospital, McGill University, Montreal, QC, Canada.
  • Siminoski K; London Health Sciences Centre, Western University, London, ON, Canada.
  • Rauch F; Kingston Health Sciences Centre, Queen's University, Kingston, ON, Canada.
  • Ward LM; Department of Medicine, Temerty Faculty of Medicine, University Health Network, University of Toronto, Toronto, ON, Canada.
Osteoporos Int ; 34(1): 147-160, 2023 Jan.
Article em En | MEDLINE | ID: mdl-36342539
ABSTRACT
Patients with Duchenne muscular dystrophy (DMD) have a high fracture burden due to progressive myopathy and steroid-induced osteoporosis. This study in males with DMD showed that markers of systemic glucocorticoid exposure including shorter stature, greater bone age delay, and lower lumbar spine bone mineral density were associated with spine fragility.

INTRODUCTION:

Fragility  fractures are frequent in DMD. The purpose of this study was to identify clinical factors associated with prevalent vertebral fractures (VF) in boys, teens/young adults with Duchenne muscular dystrophy (DMD).

METHODS:

This was a cross-sectional study of males aged 4-25 years with DMD. VF were evaluated using the modified Genant semi-quantitative method on T4-L4 lateral spine radiographs. Areal bone mineral density (aBMD) was measured at the lumbar spine (LS) and used to estimate volumetric BMD (vBMD). Clinical factors were analyzed for their association with the Spinal Deformity Index (SDI, the sum of the Genant grades).

RESULTS:

Sixty participants were enrolled (mean age 11.5 years, range 5.4-19.5). Nineteen participants (32%) had a total of 67 VF; 23/67 VF (34%) were moderate or severe. Participants with VF were shorter (mean height Z-score ± standard deviation - 3.1 ± 1.4 vs. - 1.8 ± 1.4, p = 0.001), had longer glucocorticoid exposure (mean duration 6.0 ± 3.3 vs. 3.9 ± 3.3 years, p = 0.027), greater bone age (BA) delay (mean BA to chronological age difference - 3.2 ± 3.4 vs. - 1.3 ± 1.2 years, p = 0.035), and lower LSaBMD Z-scores (mean - 3.0 ± 1.0 vs. - 2.2 ± 1.2, p = 0.023). There was no difference in LSvBMD Z-scores. Multivariable Poisson regression showed that every 0.1 mg/kg/day increment in average glucocorticoid daily dose was associated with a 1.4-fold SDI increase (95% confidence interval 1.1-1.7, p = 0.013). Greater BA delay (p < 0.001), higher weight Z-score (p = 0.004), decreased height Z-score (p = 0.025), and lower LSvBMD Z-score (p = 0.025) were also associated with SDI increase.

CONCLUSION:

Readily measurable clinical variables were associated with prevalent VF in males with glucocorticoid-treated DMD. These variables may be useful to identify candidates for primary osteoporosis prevention after glucocorticoid initiation.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Osteoporose / Fraturas da Coluna Vertebral / Distrofia Muscular de Duchenne / Fraturas Ósseas Idioma: En Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Canadá

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Osteoporose / Fraturas da Coluna Vertebral / Distrofia Muscular de Duchenne / Fraturas Ósseas Idioma: En Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Canadá