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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.