Table 2:
Descriptive statistics for PT tests and assessments in children and adults with GSD IIIa
| Children | Adults | |||||
|---|---|---|---|---|---|---|
| n | Median | 25th-75th Percentile | n | Median | 25th-75th Percentile | |
| mMRC Total Score | 16 | 8.00 | 6.45–8.75 | 5 | 8.00 | 6.75–8.20 |
| Grip Strength (lbs) | 12 | 37.32 | 23.56–41.87 | 5 | 37.16 | 25.92–49.88 |
| Grip Percentile | 9.09 | 3.46–16.48 | 1.77 | 0.15–13.92 | ||
| Lateral/Key Pinch (lbs) | 12 | 7.30 | 4.55–9.66 | 5 | 4.83 | 1.24–9.76 |
|
Lateral/Key Pinch
Percentile |
1.27 | 0.12–5.67 | 0.00 | 0.00–0.50 | ||
| GMFM Total Score % | 16 | 98.75 | 97.245–99.72 | 3 | 95.26 | 71.95–100.00 |
| GSGC Total Score | 17 | 5.94 | 2.21 | 3 | 15.67 | 7.51 |
| 6MWT Distance (m) | 16 | 490.5 | 60.57 | 3 | 421.5 | 92.82 |
|
6MWT Percent
Predicted |
76.67 | 17.35 | 68.09 | 6.09 | ||
|
BOT-2 Strength and
Agility Composite Standard Score |
15 | 39.00 | 31.00–42.00 | NA | NA | NA |
|
BOT-2 Strength and
Agility Composite Percentile Rank |
8.00 | 3.00–21.00 | NA | NA | ||
With regards to mMRC the normal value for the scale utilized in this study is 12.