Pegzilarginase

How it works

It supplements deficient arginase activity, catalysing the breakdown of arginine and thereby lowering elevated plasma arginine concentrations.

Prescribing in practice

• Hypersensitivity reactions, including anaphylaxis, can occur; administer with appropriate supervision and monitoring.
• It is used alongside dietary protein and arginine restriction as part of overall metabolic management of the disorder.
• Plasma arginine should guide therapy, with caution to avoid excessive lowering; refer to current prescribing references.

Monitoring

Plasma arginine levels are monitored to guide therapy alongside clinical assessment and tolerability.

Counselling the patient

• This medicine is used together with your prescribed low-protein diet to control arginine levels.
• Report any allergic-type symptoms such as rash, swelling or breathing difficulty.
• Do not change your diet or treatment without specialist advice.

Evidence & guidelines

Use is supported by clinical trials in arginase-1 deficiency demonstrating reductions in plasma arginine.