Formulary
Pegzilarginase: Indications, Dosing, Side Effects and Interactions
Pegzilarginase is an enzyme produced by recombinant DNA technology that is administered as enzyme replacement therapy for the treatment of arginase-1...
MedNext Academy | 3 min read
Pegzilarginase: Indications, Dosing, Side Effects and Interactions
Pegzilarginase is an enzyme produced by recombinant DNA technology that is administered as enzyme replacement therapy for the treatment of arginase-1...
Drug action
Pegzilarginase is an enzyme produced by recombinant DNA technology that is administered as enzyme replacement therapy for the treatment of arginase-1 deficiency (hyperargininaemia).
Indications and dose
**Arginase-1 deficiency [hyperargininaemia] (under expert supervision)**
- **Adult** (By Intravenous Infusion, Or By Subcutaneous Injection): Initially 0.1 mg/kg once weekly, dose should then be adjusted in steps of 0.05 mg/kg according to plasma-arginine concentrations-consult product literature, doses exceeding 0.2 mg/kg once weekly have not been studied.
**Arginase-1 deficiency [hyperargininaemia] (under expert supervision) for pegzilarginase**
- **Child 2-17 years** (By intravenous infusion, or by subcutaneous injection): Initially 0.1 mg/kg once weekly, dose should then be adjusted in steps of 0.05 mg/kg according to plasma-arginine concentrations-consult product literature, doses exceeding 0.2 mg/kg once weekly have not been studied.
Cautions
Hypersensitivity reactions Cautions, further information Hypersensitivity reactions With intravenous use: Reactions, including facial swelling, rash, and flushing, have occurred usually with the first few doses; initial doses of pegzilarginase should be administered, under medical observation, by appropriately trained staff. These reactions may be managed by temporarily interrupting or reducing the rate of the infusion, and/or treatment with antihistamines and/or corticosteroids. Pre-medication with an antihistamine and/or corticosteroid should be considered on re-administration. M
Side effects
Common or very common Hypersensitivity
Interactions
**Other interactions (1):**
- Pegzilarginase is a recombinant human enzyme and therefore no cytochrome P450 mediated drug-drug interactions are expected.
Pregnancy
Avoid (toxicity in animal studies). M
Breast feeding
Avoid (no information available). M
Medicinal forms
Clinical governance
**Author:** MedNext Clinical Team. **Clinical reviewer:** Dr Shameer Deen, MBBS, MS, MRCS. **Sources:** BNF, Indian Pharmacopoeia, CIMS India. **Correction:** Report errors at support@mednext.academy.
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