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avalglucosidase alfa-ngpt (Nexviazyme)
Indications:
- enzyme replacement therapy for Pompe disease
* avalglucosidase alfa-ngpt (Nexviazyme) indicated for late-onset disease >= 1 year of age (FDA-approved)
* alglucosidase alfa (Lumizyme) indicated for patients >= 8 years of age
Dosage:
- IV infusion every 2 weeks
Adverse effects:
- hypersensitivity reactions including anaphylaxis
- infusion-associated reactions
- arthralgia, nausea, vomiting, diarrhea
- headache, myalgia, paresthesia, dizziness, fatigue
- erythema, pruritus, urticaria
- persons susceptible to fluid volume overload or with compromised cardiac or respiratory function may be at risk for serious acute cardiorespiratory failure
Mechanism of action:
- target the mannose-6-phosphate (M6P) receptor
General
metabolic agent (metabolic modifier)
References
- Ingram I
New Enzyme Therapy OK'd for Pompe Disease.
IV treatment improved lung function, 6-minute walk test in later-onset disease.
MedPage Today August 6, 2021
https://www.medpagetoday.com/publichealthpolicy/fdageneral/93944
- Brooks M
FDA Clears New Enzyme Replacement Therapy for Pompe Disease
Medscape - Aug 06, 2021.
https://www.medscape.com/viewarticle/956179
- HIGHLIGHTS OF PRESCRIBING INFORMATION
LUMIZYME (alglucosidase alfa). for injection, for intravenous use
https://www.accessdata.fda.gov/drugsatfda_docs/label/2010/125291lbl.pdf