Active Ingredient: Sebelipase alfa
Sebelipase alfa is indicated for long-term enzyme replacement therapy (ERT) in patients of all ages with lysosomal acid lipase (LAL) deficiency.
For this indication, competent medicine agencies globally authorize below treatments:
Intravenous
1 - 3 mg per kg of body weight
From 1 To 3 mg per kg of body weight once every 7 day(s)
The recommended starting dose in infants (<6 months of age) presenting with rapidly progressive LAL deficiency is 1 mg/kg administered as an intravenous infusion once weekly. Dose escalation to 3 mg/kg once weekly should be considered based on clinical response.
The total volume of the infusion should be administered over approximately 2 hours. A 1-hour infusion may be considered after patient tolerability is established. The infusion period may be extended in the event of dose escalation.
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