METHODS OF THERAPEUTIC MONITORING OF PHENYLACETIC ACID PRODRUGS
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 - 13 . (canceled)
14 . A method of treating a urea cycle disorder in a subject experiencing symptoms of nausea, headache, somnolence, or confusion, having a plasma ammonia level within normal limits, and having a plasma PAA to PAGN ratio exceeding 2.5, the method comprising:
administering to said subject, a dosage of glyceryl tri-[4-phenylbutyrate] effective to achieve a plasma PAA to PAGN ratio below 2.5.
15 . The method of claim 14 , wherein the subject has moderate hepatic impairment.
16 . The method of claim 14 , wherein the subject has a low body surface area.
17 . The method of claim 14 , wherein the dosage of glyceryl tri-[4-phenylbutyrate] is administered orally.
18 . The method of claim 14 , further comprising measuring the plasma ammonia level of the subject.