Class: Endocrine and metabolic agent
- Solution for injection 2 mg/mL
Provides exogenous enzyme for uptake into cellular lysosomes.
C max is about 1.5 mcg/mL after 1 wk and about 1.1 mcg/mL after 27 wk. The AUC after 1 wk and 27 wk is about 206 and 169 min•mcg/mL, respectively.
The t ½ after 1 wk and 27 wk is approximately 44 and 48 min, respectively. Total body Cl after 1 wk and 27 wk is about 3 and 3.4 mL/min/kg, respectively.
Indications and Usage
Treatment of patients with Hunter syndrome (mucopolysaccharidosis [MPS] ΙΙ).
Dosage and AdministrationAdults and Children 5 yr of age and older
IV 0.5 mg/kg every week by IV infusion.
- Dilute in 100 mL of sodium chloride 0.9%.
- Each vial is for single use only.
- Use of an infusion with a 0.2 micrometer filter is recommended.
- Administer total volume over a period of 1 to 3 h. If a longer infusion time is required because of infusion reactions, the infusion time should not exceed 8 h. However, the infusion rate may be slowed, temporarily stopped, or discontinued for that visit if infusion reactions occur.
- The initial infusion rate should be 8 mL/h for the first 15 min. Then, if infusion is tolerated, the rate may be increased by 8 mL/h at 15-min intervals.
- At no time should the infusion rate exceed 100 mL/h.
- Do not infuse with other products in the infusion tubing.
Refrigerate at 36° to 46°F. Protect from light. Do not freeze or shake. If immediate use after reconstitution is not possible, the diluted solution can be refrigerated at 36° to 46°F for up to 48 h or must be administered within 8 h if held at room temperature.
None well documented.
Laboratory Test Interactions
None well documented.
Hypertension (25%); atrial abnormality (13%).
Headache (59%); malaise (22%); anxiety, irritability (13%).
Pruritus (28%); urticaria (16%); pruritic rash, skin disorder (13%).
Visual disturbances (22%).
Infusion site edema (13%).
Arthralgia (31%); limb pain (28%); chest wall musculoskeletal pain, musculoskeletal dysfunction (16%).
Pyrexia (63%); anti-idursulfase immunoglobulin G antibodies (51%); abscess (16%); superficial injury (13%).
Anaphylactoid reactions, which may be life-threatening, have been observed during infusions. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious, acute exacerbation of respiratory compromise caused by infusion reactions.
Category C .
Safety and efficacy not established in children younger than 5 yr of age.
Consider delaying infusion in patients with concomitant acute respiratory and/or febrile illness.
No experience with overdosage in humans is available.
- Encourage patient to participate in the Hunter Outcome Survey.
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