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vestronidase alfa-vjbk (Mepsevii)
Indications:
- mucopolysaccharidosis VII
Dosage:
- IV infusion 10 mg (compatible with normal saline)
- premedicate with a nonsedating antihistamine 30-60 minutes before IV infusion
- infuse over 4 hours, 2.5% 1st hour
vial: 5 mL, 2 mg/mL (store at 2-8 degrees C)
Monitor:
- observe during infusion & for at least 60 minutes after for anaphylaxis
Pharmacokinetics:
- peak plasma level: 20 ug/mL
- volume of distribution: 260 mL:/kg
- eliminated by proteolytic degradation
- clearance: 1.3 mL/min/kg
- 1/2life: 155 minutes
Adverse effects:
- anaphylaxis (< 1%)
- diarrhea, rash, pruritus, edema (< 1%)
Mechanism of action:
- recombinant human lysosomal beta glucuronidase (GUSB)
General
recombinant protein; chimer
beta glucuronidase (GUSB, beta-G1, MPS7)
metabolic agent (metabolic modifier)
References
- RxNorm
- Medscape: vestronidase alfa-vjbk (Rx)
https://reference.medscape.com/drug/mepsevii-vestronidase-alfa-vjbk-1000214