Description
What is Palynziq (pegvaliase-pqpz) for?
Palynziq (pegvaliase-pqpz) is a PEGylated phenylalanine ammonia lyase (PAL) enzyme indicated for the treatment of phenylketonuria (PKU) in adults and adolescents from 16 years of age and who have uncontrolled blood phenylalanine concentrations greater than 600 micromol/L on existing management. It is available in single-dose prefilled syringe form each containing 2.5 mg/0.5 mL, 10 mg/0.5 mL and 20 mg/1 mL of pegvaliase-pqpz[1].
How does Palynziq (pegvaliase-pqpz) work?
Palynziq (pegvaliase-pqpz) converts phenylalanine to ammonia and trans-cinnamic acid.
It substitutes for the deficient phenylalanine hydroxylase (PAH) enzyme activity in patients with PKU and reduces blood phenylalanine concentrations[1].
Where has Palynziq (pegvaliase-pqpz) been approved?
Palynziq (pegvaliase-pqpz) was approved by:
- The Food and Drug Administration (FDA), USA, on May 24, 2018, for the treatment of adult patients with phenylketonuria (PKU) who have inadequate blood phenylalanine control (blood phenylalanine levels greater than 600 micromol/l) despite prior management with available treatment options[1].
- European Medicines Agency (EMA), European Union (EU), on May 03, 2019, for the treatment of patients with phenylketonuria (PKU) aged 16 years and older who have inadequate blood phenylalanine control (blood phenylalanine levels greater than 600 micromol/l) despite prior management with available treatment options[2].
Please note that this medicine may have also been approved in other regions than the ones we’ve listed. If you have a question about its approval in a specific country feel free to contact our support team.
How is Palynziq (pegvaliase-pqpz) taken?
Treatment with Palynziq (pegvaliase-pqpz) should be directed by physicians experienced in the management of phenylketonuria (PKU). The standard dosage consists of several phases and is as follows[2]:
Posology
Before initiating treatment, blood phenylalanine level must be obtained. Monitoring of blood phenylalanine level is recommended once a month.
Dietary phenylalanine intake should remain consistent until a maintenance dose is established.
Dosing regimens
Induction
The recommended starting dose of Palynziq (pegvaliase-pqpz) is 2.5 mg administered once per week for 4 weeks.
Titration
The dose should be escalated gradually based on tolerability to the daily maintenance dose required to achieve blood phenylalanine level of 120 to 600 micromol/l.
Maintenance
The maintenance dose is individualised to achieve the patient’s blood phenylalanine control (i.e., a phenylalanine level between 120 to 600 micromol/l) taking into account the patient tolerability to Palynziq (pegvaliase-pqpz) and the dietary protein intake.
Dosage may be different for special populations. Reduction in dose may be required based on symptoms and experienced side effects.
Complete information about Palynziq (pegvaliase-pqpz) dosage and administration, including a specific recommended dosing regimen table, can be found in the official prescribing information listed in our references section[1].
Note: Please consult with your treating doctor for personalised dosing and potential drug interactions.
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