GB2470310A — Dosing and monitoring patients on nitrogen scavenging drugs

USPTO Abstract

A method for determining a dose of an orally administered nitrogen scavenging drug that contains or is metabolized to produce phenyl acetic acid (PAA) is disclosed for a patient having a nitrogen retention disorder comprising determining the amount of the PAA prodrug needed to produce a target amount of phenylacetylglutamine (PAGN) based on a conversion efficiency of about 40-70% of the nitrogen scavenging drug being converted into urinary PAGN. The nitrogen scavenging drug may be phenylbutyric acid (PBA) or glyceryl tri-[4-phenylbutyrate] (HPN-100). Methods of transitioning patients to a HPN-100-based treatment and methods of determining a suitable dietary protein level for a patient having a nitrogen retention disorder are also claimed, along with the use of PBA prodrugs such as HPN-100 to treat urinary cycle disorders (UCD), chronic renal failure (CRF) or hepatic encephalopathy (HE). The PBA prodrug may be administered at a daily dose in excess of 19 g/day.