Myofibre Density Reveals a Critical Threshold Around Age 6 in Steroid-Naïve Duchenne Muscular Dystrophy: A Retrospective Observational Study - PubMed
5 hours ago
- #histopathology
- #Duchenne muscular dystrophy
- #myofibre density
- Myofibre density (MFD) is a key histological marker for early disease progression in Duchenne muscular dystrophy (DMD).
- A retrospective study of 35 steroid-naïve DMD muscle biopsies identified a critical threshold in MFD decline around age 6.25 years.
- After age 6, MFD plateaus at lower levels, indicating a phase of rapid myofibre loss up to this age.
- Bayesian modelling and Monte Carlo simulations supported the breakpoint at approximately 6.37 years with 80% detection power.
- MFD cut-offs (>596 and <426 fibres/mm²) can predict age groups (<6.25 years) with high probability.
- Significant shifts in myofibre size, area, and fat replacement were observed between early (<5 years) and late (≥7.5 years) age bands.
- The study highlights MFD as a simple yet effective metric for assessing early-stage DMD progression.