Table 2.
Changes in muscle MRI parameters in untreated and ERT-treated LOPD patients.
| Pichiecchio et al. [24] | Pichiecchio et al. [24] | Ravaglia et al. [26] | This study | |
|---|---|---|---|---|
| N | 4 | 9 | 11 | 3 |
| Duration | 5 years | 6 months | 18/24 months | 5 years |
| ERT with rhGAA | No | Yes | Yes | Yes |
| Changes in muscle volume [%] | ||||
| Lower limb | − 18.5 (p = 0.07) | 5.7 (p = 0.003) | 2.5 (p = 0.078) | − 0.6 (p = 0.76) |
| Thigh | − 24.8 (p = 0.07) | 8.1 (p = 0.051) | 5.3 (p = 0.035) | − 6.7 (p < 0.001) |
| Lower leg | − 1.3 (p = 0.14) | 2.7 (p = 0.008) | − 2.4 (p = 0.178) | 8.2 (p = 0.049) |
| Changes in intra-muscular fat [%] | ||||
| Lower limb | 8.4 (p = 0.068) | 2.8 (p = 0.021) | 3.3 (p = 0.001) | – |
| Thigh | 11.1 (p = 0.068) | 3.3 (p = 0.028) | 3.7 (p = 0.009) | – |
| Lower leg | 1.8 (p = 0.068) | 1.6 (p = 0.008) | 1.3 (p = 0.006) | – |