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

Research output: Contribution to journalArticlepeer-review

Authors

  • Nicola Crabtree
  • Judith E. Adams
  • Raja Padidela
  • N. J. Shaw
  • Wolfgang Högler
  • And 4 others
  • H. Roper
  • I. Hughes
  • A. Daniel
  • M. Z. Mughal

Colleges, School and Institutes

External organisations

  • Manchester University Hospitals NHS Foundation Trust

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.

Details

Original languageEnglish
Pages (from-to)181-186
JournalBone
Volume116
Early online date25 Jul 2018
Publication statusPublished - Nov 2018

Keywords

  • Glucocorticoids, Muscular dystrophy, Vertebral fracture, Ambulation, Bone density, Growth