Metabolite Testing Kit
Determine your patient’s plasma
PAA:PAGN ratio and urinary PAGN level
Look over the patient treatment profile and important biomarkers, and order a metabolite testing kit
Periodic reassessment of body surface area should be performed to ensure accurate dosing, particularly in pediatric patients, who will experience significant changes in this parameter over time.1
Be sure to consider any changes in hepatic capacity; patients with moderate to severe hepatic impairment should receive the lowest dosage necessary to control ammonia levels.1
Changes in a patient’s protein intake—either prescribed or through nonadherence to diet—may require altering the dosage to compensate for greater amounts of nitrogen that need to be excreted as waste.1
aHypothetical case study.
bInstruct patients to abstain from eating or drinking any foods or liquids before plasma ammonia levels are to be measured.1
cConsider a patient’s use of concomitant medications, such as probenecid, when making dosing adjustment decisions based on U-PAGN and PAA. Probenecid may result in decrease of the urinary excretion of PAGN.1
Determine your patient’s plasma
PAA:PAGN ratio and urinary PAGN level
This assessment tool can help evaluate treatment of your patient and ensure management of their UCD
References: 1. RAVICTI (glycerol phenylbutyrate) Oral Liquid [prescribing information] Horizon. 2. Mokhtarani M, Diaz GA, Rhead W, et al. Urinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders. Mol Genet Metab. 2012;107(3):308-314. doi:10.1016/j.ymgme.2012.08.006 3. Mokhtarani M, Diaz GA, Rhead W, et al. Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio. Mol Genet Metab. 2013;110(4):446-453. doi:10.1016/j.ymgme.2013.09.017
RAVICTI (glycerol phenylbutyrate) Oral Liquid is indicated for use as a nitrogen-binding agent for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or supplementation alone. RAVICTI must be used with dietary protein restriction and, in some cases, dietary supplements (e.g. essential amino acids, arginine, citrulline, protein-free calorie supplements).
The most common adverse reactions reported in clinical trials (at least 10% of patients) were:
Please see Full Prescribing Information.
RAVICTI (glycerol phenylbutyrate) Oral Liquid is indicated for use as a nitrogen-binding agent for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or supplementation alone. RAVICTI must be used with dietary protein restriction and, in some cases, dietary supplements (e.g. essential amino acids, arginine, citrulline, protein-free calorie supplements).
The most common adverse reactions reported in clinical trials (at least 10% of patients) were:
Please see Full Prescribing Information.