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Table 2 Urine GAG* excretion

From: Phase I/II clinical trial of enzyme replacement therapy with idursulfase beta in patients with mucopolysaccharidosis II (Hunter Syndrome)

  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 )
Baseline 129.1(±59.9) 164(±53.1) 124.7(±36.1)
4 weeks 105.6(±56) 112.3(±56.7) 73.5(±35.7)
8 weeks 109.8(±67.8) 103.4(±56.4) 67.1(±21.7)
12 weeks 102.5(±55) 110.9(±44.1) 71.9(±24.4)
16 weeks 111.1(±69.7) 107(±46.2) 63(±24.6)
20 weeks 104.6(±62.6) 111.6(±46.5) 70.7(±24.5)
24 weeks 105.3(±59.6) 114.4(±45) 74.5(±27.3)
  1. All values are menas ± SD.
  2. *reference range: < 175 CPC unit/g Cr for ≤ 9 years, < 85 CPC unit/g Cr for > 9 years.