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ALIMENTARY TRACT AND METABOLISM › OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS › OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS › Enzymes
cipaglucosidase alfa
A16AB23
Forms & Strengths
- Lyophilized powder for solution for infusion: 105 mg per vial
Adult Dosing
- 20 mg/kg of cipaglucosidase alfa administered intravenously every 2 weeks, in combination with miglustat
Pediatric Dosing
- Safety and efficacy in pediatric patients have not been established
Indications
- Treatment of late-onset Pompe disease (acid alpha-glucosidase deficiency) in adult patients in combination with miglustat
Mechanism of Action
- Recombinant human acid alpha-glucosidase (rhGAA) that provides an exogenous source of GAA to hydrolyze glycogen to glucose in lysosomes, reducing glycogen accumulation in muscle tissues
Contraindications
- Life-threatening hypersensitivity reactions to the active substance or excipients, when not manageable
Adverse Reactions
- Infusion-associated reactions including chills, pyrexia, dizziness, and pruritus
- Upper respiratory tract infection
- Headache
- Arthralgia
- Fatigue
- Diarrhea
- Nausea
Drug Interactions
- Must be co-administered with miglustat; refer to miglustat prescribing information for interacting drugs
- No formal clinical drug-drug interaction studies have been conducted with cipaglucosidase alfa alone
Curated Content: Needs Vetting Before Put to Clinical Use