Methods of therapeutic monitoring of phenylacetic acid prodrugs
View Patent ↗The present disclosure provides methods for adjusting the dosage of PAA prodrugs (e.g., HPN-100, PBA) based on measurement of PAA and PAGN in plasma and calculating the PAA:PAGN ratio so as to determine whether PAA to PAGN conversion is saturated.
1. A method of treating a urea cycle disorder in a subject comprising administering to a subject having a plasma PAA to PAGN ratio outside the target range of 1 to 2, a dosage of glyceryl tri-[4-phenylbutyrate] (HPN-100) effective to achieve a plasma PAA to PAGN ratio within the target range of 1 to 2.
2. A method of treating a urea cycle disorder in a subject comprising administering to a subject having a plasma PAA to PAGN ratio outside the target range of 1 to 2.5, a dosage of glyceryl tri-[4-phenylbutyrate] (HPN-100) effective to achieve a plasma PAA to PAGN ratio within the target range of 1 to 2.5.