Skeletal disproportion in glucocorticoid-treated boys with Duchenne muscular dystrophy

Kao, K.-T., Joseph, S., Capaldi, N., Brown, S., Di Marco, M., Dunne, J., Horrocks, I., Shepherd, S., Ahmed, S. F. and Wong, S. C. (2019) Skeletal disproportion in glucocorticoid-treated boys with Duchenne muscular dystrophy. European Journal of Pediatrics, 178(5), pp. 633-640. (doi: 10.1007/s00431-019-03336-5) (PMID:30762116) (PMCID:PMC6459782)

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Abstract

We aimed to compare body segment and bone lengths in glucocorticoid-treated boys with Duchenne muscular dystrophy (DMD) with healthy controls using dual-energy absorptiometry (DXA) images. Total height (Ht), sitting height (SH), leg length (LL) and bone lengths (femur, tibia) in boys with DMD and age-matched control boys were measured using DXA. Thirty boys with DMD (median age 10.0 years (6.1, 16.8)) were compared with 30 controls. SH in DMD was 3.3 cm lower (95% CI − 6.1, − 0.66; p = 0.016). LL in DMD was 7.3 cm lower (95% CI − 11.2, − 3.4; p < 0.0001). SH:LL of boys with DMD was higher by 0.08 (95% CI 0.04, 0.12; p < 0.0001). Femur length in DMD was 2.4 cm lower (95% CI − 4.6, − 0.12; p = 0.04), whereas tibial length in DMD was 4.8 cm lower (95% CI − 6.7, − 2.9; p < 0.0001). SH:LL was not associated with duration of glucocorticoid use (SH:LL β = 0.003, 95% CI − 0.01 to 0.002, p = 0.72). Conclusion: Glucocorticoid-treated boys with DMD exhibit skeletal disproportion with relatively shorter leg length and more marked reduction of distal long bones. As glucocorticoid excess is not associated with such disproportion, our findings raise the possibility of an intrinsic disorder of growth in DMD.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Wong, Dr Jarod and Horrocks, Dr Iain and Shepherd, Dr Sheila and Ahmed, Professor Syed Faisal and Joseph, Dr Shuko and Kao, Dr Jeff
Authors: Kao, K.-T., Joseph, S., Capaldi, N., Brown, S., Di Marco, M., Dunne, J., Horrocks, I., Shepherd, S., Ahmed, S. F., and Wong, S. C.
College/School:College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
Journal Name:European Journal of Pediatrics
Publisher:Springer
ISSN:0340-6199
ISSN (Online):1432-1076
Published Online:14 February 2019
Copyright Holders:Copyright © 2019 The Authors
First Published:First published in European Journal of Pediatrics 178(5): 633-640
Publisher Policy:Reproduced under a Creative Commons License

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Project CodeAward NoProject NamePrincipal InvestigatorFunder's NameFunder RefLead Dept
676381Secondary Osteoporosis & Its Therapy in Duchenne Muscular Dystrophy - ScOT-DMDSyed Faisal AhmedChief Scientist office (CSO)CAF/DMD/14/01SCHOOL OF MEDICINE, DENTISTRY & NURSING