Searchable abstracts of presentations at key conferences on calcified tissues
Bone Abstracts (2019) 7 P46 | DOI: 10.1530/boneabs.7.P46

ICCBH2019 Poster Presentations (1) (226 abstracts)

Vertebral fractures are more prevalent than long bone fractures in boys with glucocorticoid-treated Duchenne Muscular Dystrophy: Results of a prospective observational study

Stefan A Jackowski 1 , Laura C McAdam 2 , Jinhui Ma 3 , Hugh J McMillan 4 , Victor N Konji 1 , Mary Ann Matzinger 5 , Nazih Shenouda 5 , Jacob L Jaremko 6 , Khaldoun Koujok 5 , Nasrin Khan 1 , Karine Khatchadourian 4 , Marie-Eve Robinson 7 , Lynn MacLeay 1 , David S Saleh 8 , Kerry Siminoski 6, , Frank Rauch 7 & Leanne M Ward 4

1Children’s Hospital of Eastern Ontario Research Institute, University of Ottawa, Ottawa, Canada; 2Department of Pediatrics, University of Toronto, Toronto, Canada; 3Department of Health Research Methods, Evidence, and Impact McMaster University, Hamilton, Canada; 4Department of Pediatrics, University of Ottawa, Ottawa, Canada; 5Department of Medical Imaging, University of Ottawa, Ottawa, Canada; 6Department of Radiology and Diagnostic Imaging, University of Alberta, Edmonton, Canada; 7Department of Pediatrics, McGill University, Montreal, Canada; 8Department of Pediatrics, Queen’s University, Kingston, Canada; 9Department of Medicine, University of Alberta, Edmonton, Canada.

Objectives: Osteoporosis is a frequent cause of morbidity in boys with glucocorticoid (GC)-treated Duchenne Muscular Dystrophy (DMD). We sought to determine the frequency and characteristics of the two most debilitating types of fractures, vertebral and long bone (VF and LBF), in pediatric DMD.

Methods: This was a prospective, bi-centre, single-visit observational study in boys 4 to 17 years of age with genetically-confirmed DMD. The bone health assessment included radiologically-confirmed low-trauma LBF, a lateral spine radiograph for VF assessment (T4 to L4) according to the Genant method (triple read by pediatric radiologists), anthropometry, pubertal staging, nutritional assessment, muscle function testing, and serum bone metabolism markers.

Results: Sixty boys with DMD were enrolled (mean age 11.5±3.9 years); 56/60 were GC-treated. Eighteen boys (30%), all GC-treated, had a total of 59 VF (64% of VF were mild, 27% moderate, 9% severe); 13/18 boys had mild VF as the worst grade (72%), 4 moderate and 1 severe. Twenty-three % of VF were at T6 and T7, and the median number of VF per patient was 2.0 (range 1 to 14). Forty-one % of boys with any VF were asymptomatic, including 3/5 boys with moderate/severe VF. There were 12 LBF in 10/60 boys (17%, all GC-treated). The most frequent sites of LBF were the humerus (50%), followed by the radius (17%), tibia (17%) and femur (16%). 3 boys had both VF and LBF. There were no significant differences in anthropometry, pubertal stage, calcium and vitamin D intake, and muscle function testing between boys with and without fractures (all, P>0.08). However, boys with VF, but not with LBF, had lower P1NP (111±62 vs 221±156 ng/ml, P=0.002) and CTX (0.25±0.23 vs 0.47±0.35 ng/ml, P=0.02).

Conclusions: In this cohort, VF were more frequent in boys with GC-treated DMD than LBFs; upper and lower extremity LBF occurred with equal frequency. Most boys with VF were asymptomatic; therefore, VF would have gone undetected in the absence of a spine radiograph (including in 60% of boys with moderate/severe VF). Low bone turnover may be a clinical marker of VF in this setting.

Disclosure: The authors declared no competing interests.

Volume 7

9th International Conference on Children's Bone Health


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