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)

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

Monitor

• observe during infusion & for at least 60 minutes after for anaphylaxis

Adverse effects

• anaphylaxis (< 1%)
• diarrhea, rash, pruritus, edema (< 1%)

Mechanism of action

• recombinant human lysosomal beta glucuronidase (GUSB)

More general terms

• recombinant protein; chimer
• beta glucuronidase (GUSB, beta-G1, MPS7)
• metabolic agent (metabolic modifier)