• Inborn errors of metabolism

Nexviazyme Generic Name & Formulations

General Description

Avalglucosidase alfa-ngpt 100mg/vial; lyophilized pwd for IV infusion after reconstitution and dilution.

Pharmacological Class

Hydrolytic lysosomal glycogen-specific enzyme.

How Supplied

Single-dose vial—1


Generic Availability


Nexviazyme Indications


Late-onset Pompe disease (GAA deficiency).

Nexviazyme Dosage and Administration

Adults and Children

<1yr: not established. Consider premedication with antihistamines, antipyretics, and/or corticosteroids prior to initiation. ≥1yr (<30kg): 40mg/kg every 2 weeks; (≥30kg): 20mg/kg every 2 weeks. Give by IV infusion incrementally, based on response. Duration: between 4–7hrs. Initial infusion rate: 1mg/kg/hr; may gradually increase every 30mins as tolerated; see full labeling. Dosage and administration modifications: see full labeling.

Nexviazyme Contraindications

Not Applicable

Nexviazyme Boxed Warnings

Boxed Warning

Severe hypersensitivity reactions or infusion-associated reactions. Risk of acute cardiorespiratory failure in susceptible patients.

Nexviazyme Warnings/Precautions


Risk of severe hypersensitivity reactions (including anaphylaxis); discontinue immediately if occurs. Have appropriate medical support and resuscitative equipment readily available. Monitor for severe infusion-associated reactions (IARs); consider immediate discontinuation if occurs. Withhold temporarily or slow the infusion rate if mild or moderate hypersensitivity reactions or moderate IARs occur. Increased risk of severe complications from IARs in those with advanced Pompe disease. Fluid volume overload, acute underlying respiratory illness, or compromised cardiac/respiratory function (with fluid restriction): risk of acute cardiorespiratory failure; monitor vitals frequently. Pregnancy. Nursing mothers.

Nexviazyme Pharmacokinetics

See Literature

Nexviazyme Interactions

Not Applicable

Nexviazyme Adverse Reactions

Adverse Reactions

Headache, fatigue, diarrhea, nausea, arthralgia, dizziness, myalgia, pruritus, vomiting, dyspnea, erythema, paresthesia, urticaria; hypersensitivity reactions, IARs.

Nexviazyme Clinical Trials

See Literature

Nexviazyme Note

Not Applicable

Nexviazyme Patient Counseling

See Literature