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G-CSF = Filgrastim

Neutropenia in GSD 1b

2.5 mcg/kg once daily. If the mean absolute neutrophil count (ANC) in the subsequent 2-week period was less than 1 × 109 cells/L, the dose should be escalated to 5 mcμg/kg/day for the next 2 weeks. Subsequent dose escalations to 10, 20, and 30 mcgg/kg/day after 2 weeks of therapy if the mean ANC remained at less than 1 × 109 cells/L. Maximum dose 30 mcg/kg/day.

Therapy could be continued for up to 6 weeks in patients with an ANC remaining at less than 0.5 × 109 cells/L.

If after 6 weeks of therapy at the highest dose level, the ANC failed to increase more than 0.5×109cells/L; then G-CSF should be discontinued (8, 80).

300 mcg/mL vial, 600 mcg/mL prefilled-syringe

SC

Nausea and vomiting (57% ) and bone pain ( (24% to 33%).

4

8. Centre for Evidence-Based Medicine. Secondary Centre for Evidence-Based Medicine  2009. http://www.cebm.net/index.aspx?o=1025.

80. Product information for Naglazyme. Secondary Product information for Naglazyme.    http://www.naglazyme.com/en/HCP/HCP.aspx.

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