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Vestronidase Alfa-Vjbk


(ves TRON i dase AL fa vjbk)

Index Terms

  • Mepsevii
  • Recombinant Human Beta-Glucuronidase (rhGUS)

Pharmacologic Category

  • Enzyme

Use: Labeled Indications

Mucopolysaccharidosis VII: Treatment of mucopolysaccharidosis VII (MPS VII, Sly syndrome) in pediatric and adult patients.

Limitations of use: The effect of vestronidase alfa-vjbk on the CNS manifestations of MPS VII has not been determined.


There are no contraindications listed in the manufacturer's labeling.

Adverse Reactions


Dermatologic: Skin rash (≤25%)

Gastrointestinal: Diarrhea (≤25%)

Hypersensitivity: Anaphylaxis (10% to 17%)

Immunologic: Antibody development (≤78%; neutralizing: ≤56%)

1% to 10%:

Cardiovascular: Peripheral edema (≤8%)

Dermatologic: Pruritus (≤8%)

Local: Swelling at injection site (≤8%)

Frequency not defined: Miscellaneous: Febrile seizures

ALERT: U.S. Boxed Warning


Anaphylaxis has occurred with vestronidase alfa-vjbk administration, as early as the first dose, therefore appropriate medical support should be readily available when vestronidase alfa-vjbk is administered. Closely observe patients during and for 60 minutes after vestronidase alfa-vjbk infusion. Immediately discontinue the vestronidase alfa-vjbk infusion if the patient experiences anaphylaxis.