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Neuropediatrics
DOI: 10.1055/a-2417-0441
Original Article

Frequent Unrecognized Vertebral Fractures Associated with Increased Body Fat Mass in Children and Adolescents with Duchenne Muscular Dystrophy

Kanlaya Prasiw
1   Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
,
Chaiyos Khongkhatithum 
1   Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
,
Praman Fuangfa
2   Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
,
Arpakorn Kositwattanarerk
2   Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
,
Pat Mahachoklertwattana
1   Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
,
Preamrudee Poomthavorn 
1   Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
› Author Affiliations Funding Statement The study was supported by a research grant from the Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
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Abstract

Objective Patients with Duchenne muscular dystrophy (DMD) have an increased risk of vertebral fractures (VFs). Ethnic variations may partly contribute to the fracture risk. This study aimed to demonstrate the VFs and body fat mass in Asian patients with DMD.

Methods Demographic data and DMD-related parameters of the enrolled patients were collected. Lateral thoracolumbar spine radiographs were performed for VF assessment. The Genant classification was applied for VF severity grading (mild, moderate, and severe). Body composition analysis using dual-energy X-ray absorptiometry was performed. Serum calcium, phosphate, intact parathyroid hormone, and 25-hydroxyvitamin D concentrations were determined.

Results There were 25 children and adolescents with DMD enrolled. The median (interquartile range [IQR]) age was 12.9 (9.6–19.3) years. Nine patients (36%) had VFs with a total of 31 sites of VFs (mild, N = 10; moderate, N = 3; and severe, N = 18). These VFs had never been recognized prior to this study. Comparing with the non-VF group, the VF group received a significantly greater cumulative prednisolone equivalent dose (1,258 [948–1,664] vs. 291 [17–823] mg/kg, p = 0.003). Body fat mass, represented by fat mass index and body fat percentage Z-scores, was greater in the VF group (2.46 [2.21–2.51] vs. 1.63 [0.36–2.07], p = 0.011 and 4.4 [3.1–5.5] vs. 1.8 [0.6–3.5], p = 0.008, respectively). No differences in serum calciotropic hormones and vitamin D status were demonstrated between patients with and without VFs.

Conclusions VFs were frequent in patients with DMD. Patients with VFs had greater cumulative glucocorticoid dose and body fat mass than those without VFs.

Keywords

vertebral fracture - osteoporosis - Duchenne muscular dystrophy - glucocorticoid - body composition


Publication History

Received: 23 May 2024

Accepted: 13 September 2024

Accepted Manuscript online:
17 September 2024

Article published online:
09 October 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Birnkrant DJ, Bushby K, Bann CM. et al; DMD Care Considerations Working Group. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management. Lancet Neurol 2018; 17 (03) 251-267
    CrossrefPubMedSearch in Google Scholar
  • 2 Mendell JR, Moxley RT, Griggs RC. et al. Randomized, double-blind six-month trial of prednisone in Duchenne's muscular dystrophy. N Engl J Med 1989; 320 (24) 1592-1597
    CrossrefPubMedSearch in Google Scholar
  • 3 Perera N, Sampaio H, Woodhead H, Farrar M. Fracture in Duchenne muscular dystrophy: natural history and vitamin D deficiency. J Child Neurol 2016; 31 (09) 1181-1187
    CrossrefPubMedSearch in Google Scholar
  • 4 Liaw J, Billich N, Carroll K. et al. Fracture risk and impact in boys with Duchenne muscular dystrophy: a retrospective cohort study. Muscle Nerve 2023; 67 (06) 489-496
    CrossrefPubMedSearch in Google Scholar
  • 5 Bernabe-García M, Rodríguez-Cruz M, Atilano S. et al. Body composition and body mass index in Duchenne muscular dystrophy: role of dietary intake. Muscle Nerve 2019; 59 (03) 295-302
    CrossrefPubMedSearch in Google Scholar
  • 6 Hsu YH, Venners SA, Terwedow HA. et al. Relation of body composition, fat mass, and serum lipids to osteoporotic fractures and bone mineral density in Chinese men and women. Am J Clin Nutr 2006; 83 (01) 146-154
    CrossrefPubMedSearch in Google Scholar
  • 7 Bishop N, Arundel P, Clark E. et al; International Society of Clinical Densitometry. Fracture prediction and the definition of osteoporosis in children and adolescents: the ISCD 2013 Pediatric Official Positions. J Clin Densitom 2014; 17 (02) 275-280
    CrossrefPubMedSearch in Google Scholar
  • 8 Ward LM. Glucocorticoid-induced osteoporosis: why kids are different. Front Endocrinol (Lausanne) 2020; 11: 576
    CrossrefPubMedSearch in Google Scholar
  • 9 Singh A, Schaeffer EK, Reilly CW. Vertebral fractures in Duchenne muscular dystrophy patients managed with deflazacort. J Pediatr Orthop 2018; 38 (06) 320-324
    CrossrefPubMedSearch in Google Scholar
  • 10 Ma J, McMillan HJ, Karagüzel G. et al. The time to and determinants of first fractures in boys with Duchenne muscular dystrophy. Osteoporos Int 2017; 28 (02) 597-608
    CrossrefPubMedSearch in Google Scholar
  • 11 Houde S, Filiatrault M, Fournier A. et al. Deflazacort use in Duchenne muscular dystrophy: an 8-year follow-up. Pediatr Neurol 2008; 38 (03) 200-206
    CrossrefPubMedSearch in Google Scholar
  • 12 Suthar R, Reddy BVC, Malviya M. et al. Bone density and bone health alteration in boys with Duchenne Muscular Dystrophy: a prospective observational study. J Pediatr Endocrinol Metab 2021; 34 (05) 573-581
    CrossrefPubMedSearch in Google Scholar
  • 13 Tung JY, Lam TP, Chan SH. Bone microarchitectural alterations in boys with Duchenne muscular dystrophy on long-term glucocorticoid treatment. J Bone Miner Metab 2021; 39 (04) 606-611
    CrossrefPubMedSearch in Google Scholar
  • 14 Kepley AL, Nishiyama KK, Zhou B. et al. Differences in bone quality and strength between Asian and Caucasian young men. Osteoporos Int 2017; 28 (02) 549-558
    CrossrefPubMedSearch in Google Scholar
  • 15 Liu XS, Walker MD, McMahon DJ. et al. Better skeletal microstructure confers greater mechanical advantages in Chinese-American women versus white women. J Bone Miner Res 2011; 26 (08) 1783-1792
    CrossrefPubMedSearch in Google Scholar
  • 16 Lauderdale DS, Jacobsen SJ, Furner SE, Levy PS, Brody JA, Goldberg J. Hip fracture incidence among elderly Asian-American populations. Am J Epidemiol 1997; 146 (06) 502-509
    CrossrefPubMedSearch in Google Scholar
  • 17 Department of Health, Ministry of Public Health. National Growth References for Thai Children Aged 5–19 Years. Bangkok: Ministry of Public Health; 2020
    Search in Google Scholar
  • 18 Kim J, Jung IY, Kim SJ. et al. A new functional scale and ambulatory functional classification of Duchenne muscular dystrophy: scale development and preliminary analyses of reliability and validity. Ann Rehabil Med 2018; 42 (05) 690-701
    CrossrefPubMedSearch in Google Scholar
  • 19 Parente L. Deflazacort: therapeutic index, relative potency and equivalent doses versus other corticosteroids. BMC Pharmacol Toxicol 2017; 18 (01) 1
    CrossrefPubMedSearch in Google Scholar
  • 20 Holick MF, Binkley NC, Bischoff-Ferrari HA. et al; Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 2011; 96 (07) 1911-1930
    CrossrefPubMedSearch in Google Scholar
  • 21 Genant HK, Wu CY, van Kuijk C, Nevitt MC. Vertebral fracture assessment using a semiquantitative technique. J Bone Miner Res 1993; 8 (09) 1137-1148
    CrossrefPubMedSearch in Google Scholar
  • 22 Dong H, Yan Y, Liu J. et al; China Child and Adolescent Cardiovascular Health (CCACH) collaboration members. Reference centiles for evaluating total body fat development and fat distribution by dual-energy x-ray absorptiometry among children and adolescents aged 3-18 years. Clin Nutr 2021; 40 (03) 1289-1295
    CrossrefPubMedSearch in Google Scholar
  • 23 Zemel BS, Shepherd JA, Grant SFA. et al. Reference ranges for body composition indices by dual energy X-ray absorptiometry from the Bone Mineral Density in Childhood Study Cohort. Am J Clin Nutr 2023; 118 (04) 792-803
    CrossrefPubMedSearch in Google Scholar
  • 24 Halton J, Gaboury I, Grant R. et al; Canadian STOPP Consortium. Advanced vertebral fracture among newly diagnosed children with acute lymphoblastic leukemia: results of the Canadian Steroid-Associated Osteoporosis in the Pediatric Population (STOPP) research program. J Bone Miner Res 2009; 24 (07) 1326-1334
    CrossrefPubMedSearch in Google Scholar
  • 25 Phung K, McAdam L, Ma J. et al. Risk factors associated with incident vertebral fractures in steroid-treated males with Duchenne muscular dystrophy. J Clin Endocrinol Metab 2024; 109 (02) 536-548
    CrossrefPubMedSearch in Google Scholar