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Inborn errors of metabolism
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Generic Name and Formulations:

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

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Indications for MEPSEVII:

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

Limitations Of use:

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

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.

Boxed Warning:



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.

Pharmacological Class:

Recombinant human lysosomal beta glucuronidase.

Adverse Reactions:

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

Generic Availability:


How Supplied:

Single-dose vial (5mL)—1