Beta Glucuronidase
Synonym :
Vestronidase alfa-vjbk
Mechanism :
Vestronidase alfa-vjbk is a recombinant human beta-glucuronidase (GUS), which provides exogenous GUS enzyme for uptake into cellular lysosomes.
Indication :
- Mucopolysaccharidosis VII
Contraindications :
No contraindications.
Dosing :
IV:
4 mg/kg every 2 weeks.
Limitations of use:
The effect of vestronidase alfa-vjbk on the CNS manifestations of MPS VII has not been determined.
Note: Premedicate with non-sedating antihistamines with/without antipyretics 30 to 60 minutes prior to the start of the infusion.
Adverse Effect :
Skin rash, diarrhea, anaphylaxis, antibody development, peripheral edema, pruritus, swelling at injection site, febrile seizures.
Interaction :
No known significant interactions.
Hepatic Dose :
No dosage adjustments are recommended.