• Inborn errors of metabolism

Inborn errors of metabolism:

Indications for VPRIV:

Long-term enzyme replacement therapy (ERT) in patients with Type 1 Gaucher disease.

Adults and Children:

<4yrs: not established. Give by IV infusion over 60 minutes. Individualize. ≥4yrs: 60 Units/kg every other week; adjust dose based on therapeutic goals. Begin treatment with Vpriv at the same dose when switching from imiglucerase.


Monitor for hypersensitivity reactions; immediately discontinue and treat appropriately if occur. Temporarily discontinue and resume with increased infusion time, slowing infusion rate, or administer antihistamines, antipyretics, and/or corticosteroids if infusion-related reactions occur. Elderly. Pregnancy (Cat.B). Nursing mothers.

Pharmacologic Class:

Lysosomal glucocerebroside-specific enzyme.

Adverse Reactions:

Infusion-related reactions, headache, dizziness, abdominal pain, nausea, back pain, joint pain, upper respiratory tract infection, activated PTT prolonged, fatigue/asthenia, pyrexia.

Generic Availability:


How Supplied:

Single-use vials—1