Searchable abstracts of presentations at key conferences on calcified tissues
Bone Abstracts (2015) 4 OP9 | DOI: 10.1530/boneabs.4.OP9

ICCBH2015 Oral Posters (1) (12 abstracts)

Growth, body mass index, bone health and ambulatory status of boys with Duchenne Muscular Dystrophy treated with daily vs intermittent oral glucocorticoid regimen

Nicola Crabtree 1 , Raja Padidela 2 , Nicholas Shaw 1 , Wolfgang Högler 1 , Helen Roper 3 , Imelda Hughes 2 , Judith Adams 4 , Anjali Daniel 2 & Zulf Mughal 2

1Birmingham Children’s Hospital, Birmingham, UK; 2Royal Manchester Children’s Hospital, Manchester, UK; 3Heart of England Hospital, Birmingham, UK; 4Manchester Royal Infirmary, Manchester, UK.

Background: Oral glucocorticoids (GC; prednisolone dose of 0.75 mg/kg per day), help to preserve muscle strength and prolong independent walking in boys with DMD. This study compared longitudinal growth, body mass index (BMI), bone mineral density (BMD), vertebral fractures (VFs) and ambulatory status in boys with DMD on daily (DAILY) or intermittent (INTER; 10 days on & 10 days off), oral GC regimens.

Methods: 50 DMD boys from two UK centres were included in the study; 25 boys each were on the DAILY or the INTER regimen. Size adjusted lumbar spine BMD (LS BMAD), total body less head BMD (TBLH), VF assessment and forearm pQCT data were analysed in all boys at three time points; baseline, 1 and 2 years.

Results: At their first (baseline) assessment, there were no significant differences in mean (S.D.) age (8.3 (2.5) years) or any of the bone parameters, but DAILY boys were already shorter (P=0.013) with higher BMI (P=0.014). There were no documented VFs; however 1 DAILY and three INTER boys had suffered long bone fractures. All DAILY boys were still ambulant whereas five INTER boys had already stopped walking. Prior to the 2 year assessment, six of the DAILY boys had sustained symptomatic VFs and were subsequently commenced on IV bisphosphonate therapy; these boys were excluded from further follow-up bone parameter comparisons. At 2 years, six DAILY boys and ten INTER boys had lost ambulation and the difference in height between DAILY and INTER boys had increased significantly (P<0.001). The DAILY boys also had significantly higher BMI SDS but lower BMAD and TBLH z-scores. Most notably, significantly more DAILY boys had VFs compared to INTER boys (13 DAILY vs four INTER; P=0.015). Only two of the 16 boys with VFs also had low BMAD (z<−2); in contrast 14 had low TBLH BMD z-scores and 11 had low trabecular BMD z-scores.

Conclusion: Boys on a daily GC regimen appear to remain ambulant longer but at the cost of significantly greater VFs, greater adiposity and markedly diminished growth. In contrast, the boys on the intermittent GC regimen had fewer fractures but lost ambulation earlier. In both groups, LS BMAD was a poor predictor of VFs.

Disclosure: The authors declared no competing interests.

Volume 4

7th International Conference on Children's Bone Health

Salzburg, Austria
27 Jun 2015 - 30 Jun 2015


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