Comparator 0.5 mg/kg/week (N) | Idursulfase beta 0.5 mg/kg/week (N) | Idursulfase beta 1.0 mg/kg/week (N) | |
---|---|---|---|
6MWT, m | -9.1±35.2 (8) | 61.6±32.2 (6) | 38.4±36.4 (7) |
6MWT,% change | -2.7±9.2 (8) | +23.5±16.9 (6) | +12.7±11.9 (7) |
FVC, L | 0±0.1 (5) | 0±0.1 (3) | 0.2±0.1 (4) |
FVC,% change | +1.2±8.4 (5) | +7.9±11.3 (3) | +15.8±7 (4) |
LVMI,% change | -1.7±18 (10) | -5.1±18.5 (10) | -5.9±25.1 (10) |
LVEF,% change | +2.9±11.9 (9) | +2.5±10.8 (10) | -1.4±7.4 (10) |
Joint range of motion,% changes | |||
Shoulder flexion | 23.4±26.6 (10) | 6.1±10.7 (10) | 6.7±11.2 (10) |
Shoulder extension | 1.8±43 (10) | -20.4±31.8 (10) | -1.2±19.8(10) |
Elbow flexion | 7.2±10.7 (10) | 1.1±9.4 (10) | -1.6±11 (10) |
Elbow extension | 25±78.8 (9) | 3.8±41.6 (10) | 11.9±94.5 (10) |
Hip flexion | 4.5±14 (10) | -4.1±14.9 (10) | -1.3±10.9 (10) |
Hip extension | 22.6±41.5 (10) | 47.3±98.9 (10) | 4.2±34.2 (8) |
Knee flexion | 4.5±7.9 (10) | -0.7±12.5 (10) | 1.3±8.2 (10) |
Knee extension | 7.4±40.1 (9) | -28.1±59.1(10) | 0±43.6 (8) |