• Inborn errors of metabolism

Mepsevii Generic Name & Formulations

General Description

Vestronidase alfa-vjbk 2mg/mL; soln for IV infusion after dilution; preservative-free.

Pharmacological Class

Recombinant human lysosomal beta glucuronidase.

How Supplied

Single-dose vial (5mL)—1

Generic Availability


Mepsevii Indications


Treatment of Mucopolysaccharidosis VII (MPS VII, Sly syndrome).

Limitations of Use

Effect on the CNS manifestations of MPS VII has not been determined.

Mepsevii Dosage and Administration

Adults and Children

Premedicate with a non-sedating antihistamine with or without an antipyretic 30–60mins prior to infusion. Give as IV infusion over 4hrs (infuse first 2.5% of total volume over 1hr, then increase rate as tolerated over next 3hrs); see full labeling. 4mg/kg every 2 weeks.

Mepsevii Contraindications

Not Applicable

Mepsevii Boxed Warnings

Boxed Warning


Mepsevii Warnings/Precautions


Have appropriate medical support readily available. Should be administered under supervision of healthcare professional. Monitor during and for ≥60mins post-infusion for anaphylaxis; discontinue immediately if a severe systemic reaction occurs. Elderly. Pregnancy. Nursing mothers.

Mepsevii Pharmacokinetics

See Literature

Mepsevii Interactions

Mepsevii Adverse Reactions

Adverse Reactions

Infusion site extravasation, diarrhea, rash, anaphylaxis, infusion site swelling, peripheral swelling, pruritus.

Mepsevii Clinical Trials

See Literature

Mepsevii Note

Not Applicable

Mepsevii Patient Counseling

See Literature