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Bone. 2018 Nov;116:181-186. doi: 10.1016/j.bone.2018.07.019. Epub 2018 Jul 25.

Growth, bone health & ambulatory status of boys with DMD treated with daily vs. intermittent oral glucocorticoid regimen.

Author information

1
Department of Endocrinology and Diabetes, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK. Electronic address: Nicola.crabtree@nhs.net.
2
Radiology and Manchester Academic Health Science Centre, Manchester Royal Infirmary, Central Manchester University Hospitals NHS Foundation Trust and Centre for Imaging Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, UK.
3
Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester, UK.
4
Department of Endocrinology and Diabetes, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK; Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK.
5
Department of Paediatrics, Heartlands Hospital, Birmingham, UK.
6
Department of Paediatric Neurology, Royal Manchester Children's Hospital, Manchester, UK.

Abstract

Oral glucocorticoids (GC) preserve muscle strength and prolong walking in boys with Duchenne muscular dystrophy (DMD). Although vertebral fractures have been reported in boys taking GC, fracture rates for different GC regimes have not been investigated. The aim of this pragmatic longitudinal study was to compare growth, body mass, bone mineral density (BMD), vertebral fractures (VF) and ambulatory status in boys with DMD on daily (DAILY) or intermittent (INTERMITTENT), oral GC regimens. A convenience sample of 50 DMD boys from two centres was included in the study; 25 boys each were on the DAILY or INTERMITTENT regimen. Size adjusted lumbar spine BMD (LS BMAD), total body less head BMD (TBLH), by DXA and distal forearm bone densities by pQCT, GC exposure, VF assessment and ambulatory status were analysed at three time points; baseline, 1 and 2 years. At baseline, there were no differences in age, GC duration or any bone parameters. However, DAILY boys were shorter (height SDS DAILY = -1.4(0.9); INTERMITTENT = -0.8(1.0), p = 0.04) with higher BMI (BMI SDS DAILY = 1.5(0.9); INTERMITTENT = 0.8(1.0), p = 0.01). Over 2 years, DAILY boys got progressively shorter (delta height SDS DAILY = -0.9(1.1); INTERMITTENT = +0.1(0.6), p < 0.001). At their 2 year assessment, 5 DAILY and 10 INTERMITTENT boys were non-ambulant. DAILY boys had more VFs than INTERMITTENT boys (10 versus 2; χ2 p = 0.008). BMAD SDS remained unchanged between groups. TBLH and radius BMD declined significantly but the rate of loss was not different. In conclusion, there was a trend for more boys on daily GCs to remain ambulant but at the cost of more VFs, greater adiposity and markedly diminished growth. In contrast, boys on intermittent GCs had fewer vertebral fractures but there was a trend for more boys to loose independent ambulation.

KEYWORDS:

Ambulation; Bone density; Glucocorticoids; Growth; Muscular dystrophy; Vertebral fracture

PMID:
30055340
DOI:
10.1016/j.bone.2018.07.019

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