Ravicti Oral Liquid
Ravicti Oral Liquid Uses, Dosage, Side Effects, Food Interaction and all others data.
Ravicti Oral Liquid is a nitrogen-binding agent. Chemically, it is a triglyceride in which three molecules of phenylbutyrate are linked to a glycerol backbone. FDA approved on February 1, 2013.
Ravicti Oral Liquid prolongs the QTc interval.
| Trade Name | Ravicti Oral Liquid |
| Generic | Glycerol phenylbutyrate |
| Glycerol phenylbutyrate Other Names | Glycerol phenylbutyrate, GT4P |
| Type | |
| Formula | C33H38O6 |
| Weight | Average: 530.6512 Monoisotopic: 530.266838948 |
| Protein binding | PBA = 80.6% to 98.0%; PAA = 37.1% to 65.6%; PAGN = 7% to 12%. |
| Groups | Approved |
| Therapeutic Class | |
| Manufacturer | |
| Available Country | USA |
| Last Updated: | September 19, 2023 at 7:00 am |
Uses
Ravicti Oral Liquid is a nitrogen-binding agent used to manage patients with urea cycle disorders who cannot be sufficiently managed amino acid supplementation and/or dietary restrictions.
Ravicti Oral Liquid is a nitrogen-binding agent for the chronic management of adult and pediatric patients ≥2 years of age with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone.
Ravicti Oral Liquid is also used to associated treatment for these conditions: Deficiencies in enzymes of the urea cycle
How Ravicti Oral Liquid works
The toxic accumulation of ammonia in the blood and brain arise from urea cycle disorders in which patients are deficient in critical enzymes or transporters that are involved in the synthesis of urea from ammonia. Ravicti Oral Liquid is a prodrug - the major metabolite, phenylacetate (PAA) is the molecule that binds to nitrogen. PAA conjugates with glutamine (which contains 2 molecules of nitrogen) via acetylation in the liver and kidneys to form phenylacetylglutamine (PAGN), which is excreted by the kidneys. PAGN, like urea, contains 2 moles of nitrogen and provides an alternate vehicle for waste nitrogen excretion.
Toxicity
Most common adverse reactions in ≥10% of patients are diarrhea, flatulence, and headache.
Food Interaction
- Take with food.
Elimination Route
Ravicti Oral Liquid is a prodrug in which phenylbutyrate (PBA) is released from the glycerol backbone by lipases in the gastrointestinal tract. PBA then undergoes beta-oxidtion to form PAA. When a single oral dose of 2.9 mL/m2 of Ravicti Oral Liquid is given to fasting adult subjects, the pharmacokinetic parameters are as follows: Tmax: PBA = 2 hours; PAA = 4 hours; PAGN = 4 hours. Cmax: PBA = 37.0 µg/mL; PAA = 14.9 µg/mL; PAGN = 30.2 µg/mL. In healthy subjects, the hydrolysis of glycerol phenylbutyrate is incomplete, but to what extent is unknown. When glycerol phenylbutyrate is given to adult UCD patients, maximum plasma concentrations at steady state (Cmaxss) of PBA, PAA, and PAGN occurred at 8 h, 12 h, and 10 h, respectively, after the first dose in the day. Intact glycerol phenylbutyrate was not detectable in plasma in UCD patients.
Elimination Route
Ravicti Oral Liquid is mainly excreted as PAGN in the urine (68.9% in adults and 66.5% in pediatric UCD patients). PAA and PBA represented minor urinary metabolites, each accounting for <1% of the administered dose of PBA.
Innovators Monograph
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