Active Ingredient: Alglucosidase alfa
Alglucosidase alfa is indicated for long-term enzyme replacement therapy (ERT) in patients with a confirmed diagnosis of Pompe disease (acid α-glucosidase deficiency). It is indicated in adults and paediatric patients of all ages.
For this indication, competent medicine agencies globally authorize below treatments:
Intravenous
20 - 20 mg per kg of body weight
From 20 To 20 mg per kg of body weight once every 14 day(s)
The recommended dose regimen of alglucosidase alfa is 20 mg/kg of body weight administered once every 2 weeks.
Patient response to treatment should be routinely evaluated based on a comprehensive evaluation of all clinical manifestations of the disease.
Infusions should be administered incrementally. It is recommended that the infusion begin at an initial rate of 1 mg/kg/h and be gradually increased by 2 mg/kg/h every 30 minutes if there are no signs of infusion associated reactions (IARs) until a maximum rate of 7 mg/kg/h is reached.
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