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Osteoporosis International

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Open Access 18-05-2022 | Scoliosis | Original Article

Reduced bone mineral density in adolescents with Duchenne Muscular Dystrophy (DMD) and scoliosis

Authors: K. Tsaknakis, K. Jäckle, K. A. Lüders, H. M. Lorenz, L. Braunschweig, A. K. Hell

Published in: Osteoporosis International | Issue 9/2022

Abstract

Summary

Duchenne muscular dystrophy is a progressive disease usually associated with loss of ambulation and progressive scoliosis. Immobilisation and glucocorticoid treatment are predisposing factors for reduced bone mineral density (BMD). Analysis of quantitative computed tomography revealed low BMD in thoracic and lumbar vertebrae in comparison to age- and sex-matched healthy controls.

Introduction

Evaluation of vertebral bone mineral density (BMD) in Duchenne Muscular Dystrophy (DMD) adolescents with untreated advanced scoliosis and comparison with the BMD values of healthy age-matched controls, based on quantitative computer tomography.

Methods

Thirty-seven DMD adolescents (age 15.6 ± 2.5 years) with spinal deformity were evaluated clinically and radiologically prior to definite spinal fusion and compared to 31 male and age-matched healthy individuals (age 15.7 ± 2.3 years). Data related to previous medical treatment, physiotherapy and ambulatory status was also analysed. Scoliotic curves were measured on plain sitting radiographs of the spine. The BMD Z-scores of the thoracic and lumbar vertebrae were calculated with QCTpro® (Mindways Software Inc., USA), based on data sets of preoperative, phantom pre-calibrated spinal computed tomography scans.

Results

A statistically significant lower BMD could be found in DMD adolescents, when compared to healthy controls, showing an average value for the lumbar spine of 80.5 ± 30.5 mg/cm³. Z-scores deteriorated from the upper thoracic towards the lower lumbar vertebrae. All but the uppermost thoracic vertebrae had reduced BMD values, with the thoracolumbar and lumbar region demonstrating the lowest BMD. No significant correlation was observed between BMD and the severity of the scoliotic curve, previous glucocorticoid treatment, cardiovascular impairment, vitamin D supplementation, non-invasive ventilation or physiotherapy.

Conclusion

DMD adolescents with scoliosis have strongly reduced BMD Z-scores, especially in the lumbar spine in comparison to healthy controls. These findings support the implementation of a standardised screening and treatment protocol.

Level of evidence/clinical relevance: therapeutic level III

Bell JM, Shields MD, Watters J et al (2017) Interventions to prevent and treat corticosteroid-induced osteoporosis and prevent osteoporotic fractures in Duchenne muscular dystrophy. Cochrane Database Syst Rev 1:CD010899. https://doi.org/10.1002/14651858.CD010899.pub2CrossRefPubMed

Ward LM, Hadjiyannakis S, McMillan HJ et al (2018) Bone health and osteoporosis management of the patient with Duchenne Muscular Dystrophy. Pediatrics 142:S34–S42. https://doi.org/10.1542/peds.2018-0333ECrossRefPubMed

Joseph S, Wang C, Bushby K et al (2019) Fractures and linear growth in a nationwide cohort of boys with Duchenne Muscular Dystrophy with and without glucocorticoid treatment: results from the UK NorthStar Database. JAMA Neurol 76:701. https://doi.org/10.1001/jamaneurol.2019.0242CrossRefPubMedPubMedCentral

Tian C, Wong BL, Hornung L et al (2016) Bone health measures in glucocorticoid-treated ambulatory boys with Duchenne muscular dystrophy. Neuromuscul Disord 26:760–767. https://doi.org/10.1016/j.nmd.2016.08.011CrossRefPubMed

Singh A, Schaeffer EK, Reilly CW (2018) Vertebral fractures in Duchenne Muscular Dystrophy patients managed with deflazacort. J Pediatr Orthop 38:320–324. https://doi.org/10.1097/BPO.0000000000000817CrossRefPubMed

Oda T, Shimizu N, Yonenobu K et al (1993) Longitudinal study of spinal deformity in Duchenne Muscular Dystrophy. J Pediatr Orthop 13:478–488. https://doi.org/10.1097/01241398-199307000-00012CrossRefPubMed

Gupta A, Cha T, Schwab J et al (2021) Osteoporosis increases the likelihood of revision surgery following a long spinal fusion for adult spinal deformity. Spine J Off J North Am Spine Soc 21:134–140. https://doi.org/10.1016/j.spinee.2020.08.002CrossRef

Yuan L, Zhang X, Zeng Y, et al (2021) Incidence, risk, and outcome of pedicle screw loosening in degenerative lumbar scoliosis patients undergoing long-segment fusion. Glob Spine J 21925682211017476. https://doi.org/10.1177/21925682211017477

Bauer JS, Henning TD, Müeller D et al (2007) Volumetric quantitative CT of the spine and hip derived from contrast-enhanced MDCT: conversion factors. Am J Roentgenol 188:1294–1301. https://doi.org/10.2214/AJR.06.1006CrossRef

10.

Carey JJ, Delaney MF (2010) T-scores and Z-scores. Clin Rev Bone Miner Metab 8:113–121. https://doi.org/10.1007/s12018-009-9064-4CrossRef

11.

Zemel BS, Leonard MB, Kelly A et al (2010) Height adjustment in assessing dual energy X-ray absorptiometry measurements of bone mass and density in children. J Clin Endocrinol Metab 95:1265–1273. https://doi.org/10.1210/jc.2009-2057CrossRefPubMedPubMedCentral

12.

Cann CE, Genant HK, Kolb FO, Ettinger B (1985) Quantitative computed tomography for prediction of vertebral fracture risk. Bone 6:1–7. https://doi.org/10.1016/8756-3282(85)90399-0CrossRefPubMed

13.

Cann CE (1988) Quantitative CT for determination of bone mineral density: a review. Radiology 166:509–522. https://doi.org/10.1148/radiology.166.2.3275985CrossRefPubMed

14.

Block JE, Smith R, Glueer CC et al (1989) Models of spinal trabecular bone loss as determined by quantitative computed tomography. J Bone Miner Res Off J Am Soc Bone Miner Res 4:249–257. https://doi.org/10.1002/jbmr.5650040218CrossRef

15.

Gilsanz V, Varterasian M, Senac MO, Cann CE (1986) Quantitative spinal mineral analysis in children. Ann Radiol (Paris) 29:380–382

16.

Gilsanz V, Gibbens DT, Roe TF et al (1988) Vertebral bone density in children: effect of puberty. Radiology 166:847–850. https://doi.org/10.1148/radiology.166.3.3340782CrossRefPubMed

17.

(2018) ACR–SPR–SSR practice guideline for the performance of quantitative computed tomography (QCT) bone, Available online: https://www.acr.org/-/media/ACR/Files/Practice-Parameters/qct.pdf. Accessed 02 Feb 2022

18.

Writing Group for the ISCD Position Development Conference (2004) Nomenclature and decimal places in bone densitometry. J Clin Densitom Off J Int Soc Clin Densitom 7:45–50. https://doi.org/10.1385/jcd:7:1:45CrossRef

19.

Weltgesundheitsorganisation (2003) Prevention and management of osteoporosis: report of a WHO scientific group; [WHO Scientific Group Meeting on Prevention and Management of Osteoporosis, Geneva, 7–10 April]. World Health Organization, Geneva

20.

Ward RJ, Roberts CC, Bencardino JT et al (2017) ACR Appropriateness Criteria ® osteoporosis and bone mineral density. J Am Coll Radiol 14:S189–S202. https://doi.org/10.1016/j.jacr.2017.02.018CrossRefPubMed

21.

2019 ISCD Official Positions Pediatric; Available online: https://iscd.org/learn/official-positions/pediatric-positions/. Accessed 29 Mar 2022

22.

Morgenroth VH, Hache LP, Clemens PR (2012) Insights into bone health in Duchenne Muscular Dystrophy. BoneKEy Rep 1: https://doi.org/10.1038/bonekey.2012.5

23.

Archer JE, Gardner AC, Roper HP et al (2016) Duchenne Muscular Dystrophy: the management of scoliosis. J Spine Surg 2:185–194. https://doi.org/10.21037/jss.2016.08.05CrossRefPubMedPubMedCentral

24.

King WM, Ruttencutter R, Nagaraja HN et al (2007) Orthopedic outcomes of long-term daily corticosteroid treatment in Duchenne Muscular Dystrophy. Neurology 68:1607–1613. https://doi.org/10.1212/01.wnl.0000260974.41514.83CrossRefPubMed

25.

Nakhla M, Scuccimarri R, Duffy KNW et al (2009) Prevalence of vertebral fractures in children with chronic rheumatic diseases at risk for osteopenia. J Pediatr 154:438–443. https://doi.org/10.1016/j.jpeds.2008.09.023CrossRefPubMed

26.

Mäkitie O, Doria AS, Henriques F et al (2005) Radiographic vertebral morphology: a diagnostic tool in pediatric osteoporosis. J Pediatr 146:395–401. https://doi.org/10.1016/j.jpeds.2004.10.052CrossRefPubMed

27.

Catalano A, Vita GL, Russo M et al (2016) Effects of teriparatide on bone mineral density and quality of life in Duchenne Muscular Dystrophy related osteoporosis: a case report. Osteoporos Int J Establ Result Coop Eur Found Osteoporos Natl Osteoporos Found USA 27:3655–3659. https://doi.org/10.1007/s00198-016-3761-xCrossRef

28.

Birnkrant DJ, Bushby K, Bann CM et al (2018) Diagnosis and management of Duchenne Muscular Dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol 17:347–361. https://doi.org/10.1016/S1474-4422(18)30025-5CrossRefPubMedPubMedCentral

29.

Crabtree NJ, Adams JE, Padidela R et al (2018) Growth, bone health & ambulatory status of boys with DMD treated with daily vs. intermittent oral glucocorticoid regimen. Bone 116:181–186. https://doi.org/10.1016/j.bone.2018.07.019CrossRefPubMed

30.

Herbelet S, Rodenbach A, De Paepe B, De Bleecker JL (2020) Anti-inflammatory and general glucocorticoid physiology in skeletal muscles affected by Duchenne Muscular Dystrophy: exploration of steroid-sparing agents. Int J Mol Sci 21:4596. https://doi.org/10.3390/ijms21134596CrossRefPubMedCentral

31.

Ward LM, Weber DR (2019) Growth, pubertal development, and skeletal health in boys with Duchenne Muscular Dystrophy. Curr Opin Endocrinol Diabetes Obes 26:39–48. https://doi.org/10.1097/MED.0000000000000456CrossRefPubMedPubMedCentral

32.

Zatz M, Rapaport D, Vainzof M et al (1988) Relation between height and clinical course in Duchenne Muscular Dystrophy. Am J Med Genet 29:405–410. https://doi.org/10.1002/ajmg.1320290223CrossRefPubMed

33.

Ricotti V, Ridout DA, Scott E et al (2013) Long-term benefits and adverse effects of intermittent versus daily glucocorticoids in boys with Duchenne Muscular Dystrophy. J Neurol Neurosurg Psychiatry 84:698–705. https://doi.org/10.1136/jnnp-2012-303902CrossRefPubMed

34.

Griggs RC, Miller JP, Greenberg CR et al (2016) Efficacy and safety of deflazacort vs prednisone and placebo for Duchenne Muscular Dystrophy. Neurology 87:2123–2131. https://doi.org/10.1212/WNL.0000000000003217CrossRefPubMedPubMedCentral

35.

Alman BA, Raza SN, Biggar WD (2004) Steroid treatment and the development of scoliosis in males with Duchenne Muscular Dystrophy. J Bone Jt Surg 86:519–524. https://doi.org/10.2106/00004623-200403000-00009CrossRef

36.

Lebel DE, Corston JA, McAdam LC et al (2013) Glucocorticoid treatment for the prevention of scoliosis in children with Duchenne Muscular Dystrophy: long-term follow-up. J Bone Jt Surg-Am 95:1057–1061. https://doi.org/10.2106/JBJS.L.01577CrossRef

37.

Quinlivan R, Shaw N, Bushby K (2010) 170th ENMC international workshop: bone protection for corticosteroid treated Duchenne Muscular Dystrophy. 27–29 November 2009, Naarden. The Netherlands Neuromuscul Disord 20:761–769. https://doi.org/10.1016/j.nmd.2010.07.272CrossRefPubMed

38.

Catalano A, Vita GL, Bellone F et al (2022) Bone health in Duchenne Muscular Dystrophy: clinical and biochemical correlates. J Endocrinol Invest 45:517–525. https://doi.org/10.1007/s40618-021-01676-4CrossRefPubMed

39.

Dovio A, Perazzolo L, Osella G et al (2004) Immediate fall of bone formation and transient increase of bone resorption in the course of high-dose, short-term glucocorticoid therapy in young patients with multiple sclerosis. J Clin Endocrinol Metab 89:4923–4928. https://doi.org/10.1210/jc.2004-0164CrossRefPubMed

Title: Reduced bone mineral density in adolescents with Duchenne Muscular Dystrophy (DMD) and scoliosis
Authors: K. Tsaknakis
K. Jäckle
K. A. Lüders
H. M. Lorenz
L. Braunschweig
A. K. Hell
Publication date: 18-05-2022
Publisher: Springer London
Keywords: Scoliosis
Muscular Dystrophy
Muscular Dystrophy
Glucocorticoid
Osteoporosis
Osteoporosis
Non-Invasive Ventilation
Duchenne Muscular Dystrophy
Published in: Osteoporosis International / Issue 9/2022
Print ISSN: 0937-941X
Electronic ISSN: 1433-2965
DOI: https://doi.org/10.1007/s00198-022-06416-9

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Reduced bone mineral density in adolescents with Duchenne Muscular Dystrophy (DMD) and scoliosis

Abstract

Summary

Introduction

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Summary

Introduction

Methods

Results

Conclusion

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