Mepsevii Generic Name & Formulations
Vestronidase alfa-vjbk 2mg/mL; soln for IV infusion after dilution; preservative-free.
Recombinant human lysosomal beta glucuronidase.
Single-dose vial (5mL)—1
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 Boxed Warnings
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 Adverse Reactions
Infusion site extravasation, diarrhea, rash, anaphylaxis, infusion site swelling, peripheral swelling, pruritus.
Mepsevii Clinical Trials
Mepsevii Patient Counseling