Trade Names:Myozyme- Powder for injection 50 mg
Exogenous source of alpha-glucosidase (GAA).
Following a single IV infusion of 20 mg/kg, C max and AUC were 162 mcg/mL and 811 mcg•h/mL, respectively.
Following a single IV infusion of 20 mg/kg, Cl and t ½ were 25 mL/h/kg and 2.3 h, respectively.
Treatment of Pompe disease (GAA deficiency).
IV 20 mg/kg administered by IV infusion over approximately 4 h every 2 wk. Administer in a step-wise manner, starting with no more than 1 mg/kg/h. The infusion rate may be increased by 2 mg/kg/h every 30 min, after tolerance to infusion rate is established (max rate, 7 mg/kg/h).
Refrigerate between 36° and 46°F. Administer reconstituted and diluted solution without delay. If immediate use is not possible, reconstituted and diluted solution is stable for up to 24 h at 36° to 46°F. Protect from light. Do not freeze or shake.
None well documented.
None well documented.
Tachycardia (23%); bradycardia (21%).
Rash (54%); diaper dermatitis (36%); urticaria (23%); flushing (21%).
Otitis media (44%); pharyngitis (36%); ear infection (33%); nasopharyngitis (23%).
Diarrhea (62%); vomiting (49%); gastroenteritis (41%); oral candidiasis (31%); gastroesophageal reflux disease (26%); constipation (23%).
Positive test for immunoglobulin G antibodies (89%); decreased oxygen saturation (41%).
Catheter-related infection (28%).
Cough, pneumonia (46%); upper respiratory tract infection (44%); respiratory distress (33%); respiratory failure (31%); rhinorrhea (28%); bronchiolitis, tachypnea (23%).
Infusion reactions (51%); postprocedural pain (26%).
Life-threatening anaphylactic reactions, including anaphylactic shock, have been observed in patients during alglucosidase alfa infusion. Appropriate medical support measures should be readily available.
Category B .
Safety and efficacy not established in juvenile-onset Pompe disease population.
Cardiac arrhythmia, including bradycardia, ventricular fibrillation, and ventricular tachycardia, resulting in cardiac arrest or defibrillation has occurred. These events were in infantile-onset Pompe disease patients with cardiac hypertrophy, associated with general anesthesia used for placement of central venous catheter for alglucosidase infusion.
Acute cardiorespiratory failure requiring intubation and inotropic support has been observed after infusion with alglucosidase.
Severe infusion reactions may occur.
No reports of overdosage.
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