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Table 1 The β -glucosidase activity of bone removed from patients with and without Gaucher disease (GD) before and after infusion with alglucerase

From: Failure of Alglucerase Infused into Gaucher Disease Patients to Localize in Marrow Macrophages

  β-Glucosidase Activity (U/g DNA) Chitotriosidase (U/g DNA)
  Untreated Treated Expecteda
Normal Controls (4) 9.3 ± 5.3 10.0 ± 7.00
GD Patient 1 0.8 16
GD Patient 2 1.5 64
GD Patient 3 0.8 7.7b >300 (48, 76)c
GD Patient 4 0.7 3.2d >11.5 (110, 129)
GD Patient 5 1.5 2.6e >600 (215, N/A)
GD Patient 6 2.0f >4.7 (N/A, 70)
  1. Normal values are mean ± 1 standard deviation. N/A, not available.
  2. aExpected values are those that would be achieved if the infused enzyme were uniformly distributed to all body cells and remained with a t1/2 of 8 hr.
  3. bBone taken 8 hr after infusion of 60 U alglucerase/kg body wt.
  4. cValues in parentheses are pre- and postinfusion values, respectively.
  5. dBone taken at end of 2-hr infusion of 1.15 U alglucerase/kg body wt.
  6. eBone taken at end of 2-hr infusion of 60 U alglucerase/kg body wt.
  7. fBone taken 48 hr after infusion of 60 U alglucerase/kg body wt.