Table 1 Patient demographic and baseline characteristics
Comparator 0.5 mg/kg/week (N=11) | Idursulfase beta 0.5 mg/kg/week (N=10) | Idursulfase beta 1.0 mg/kg/week (N=10) | |
|---|---|---|---|
Age, years | 10.8(±4.6) | 11.7(±9) | 11.5(±4.7) |
Height, cm | 124.7(±11.5) | 119.6(±10) | 122.2(±12.9) |
Weight, cm | 34.1(±9) | 28.6(±7.8) | 29.3(±8.9) |
Duration of previous ERT, month | 14.8(±0.8) | 14.3(±0.6) | 15.9(±0.7) |
Urine GAG*, CPCunit/g Cr | 129.1(±59.9) | 164(±53.1) | 124.7(±36.1) |
6MWT, m | 368(±76.8) | 277.5(±100.2) | 328(±70.1) |
FVC, L | 1.6(±0.5) (N=5) | 1.5(±1) (N=3) | 1.3(±0.4) (N=4) |
LVMI, g/m 2.7 | 55.6(±15.6) | 59.5(±16.3) | 54.4(±13.2) |
LVEF,% | 73.4(±6.7) | 68.6(±3.8) | 69(±4.1) |