Exploring the natural history of bone mineral density in Duchenne muscular dystrophy: a systematic literature review
Authors' conclusion
Understanding the natural history of BMD and disease progression relating to bone fragility will help to support clinical trial design to assess and improve bone strength in DMD. To our knowledge, this is the first SLR investigating BMD Z-score trajectories in individuals with DMD receiving GCs and highlights associations between age and GC treatment duration with declining LS aBMD, TBLH, and LDF Z-scores.
Study context
- Population
- Duchenne muscular dystrophy (DMD) patients aged 5–15 years receiving glucocorticoid (GC) treatment; peer-reviewed articles 2000–2023, PubMed and Embase
- Outcome
- Natural course of BMD and factors associated with its progression
Methodological detailscorrelation analyses, generalized linear mixed models
- Correlation analyses
- Generalized linear mixed models
Main finding
As reported in the abstract
Lumbar spine (LS) areal BMD (aBMD) Z-score change with age (p < 0.001, n = 1527)
−0.25 SDS/year
LS aBMD Z-score change with longer GC use (p < 0.001, n = 817)
−0.24 SDS/year
Total body less head (TBLH) bone mineral density (BMD) Z-score change with age
−0.26 SDS/year
Lateral distal femur (LDF) BMD Z-score change with age
- 0.41 SDS/year
Further findings
Fractures vs LS aBMD Z-score
- Increase in % with fractures per 1-SDS decrease in LS aBMD Z-score (p < 0.01, n = 1066)
- 21%
LS aBMD decline by age
- LS aBMD Z-score reductions significantly accelerated after 11 years of age
Original publication
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