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RAVICTI (glycerol phenylbutyrate)
- Ornithine carbamoyltransferase deficiency
- Carbamoyl phosphate synthetase deficiency
- Citrullinemia
1.1 gram/mL oral liquid
- Dosage information is not available
- None
Contraindicated
- None
Severe
Moderate
- None
- None
Contraindicated
- Severe hepatic disease
Severe
Moderate
- Acute cognitive impairment
- Kidney disease with reduction in GFR
- Memory impairment
RAVICTI (glycerol phenylbutyrate)
- Ornithine carbamoyltransferase deficiency
- Carbamoyl phosphate synthetase deficiency
- Citrullinemia
- None
- Diarrhea
- Dysgeusia
- Flatulence
- Headache disorder
More Frequent
Severe
Less Severe
- None
- Acute abdominal pain
- Anorexia
- Dyspepsia
- Fatigue
- Nausea
- Vomiting
Less Frequent
Severe
Less Severe
Rare / Very Rare
Severe
- Hearing loss
- Peripheral neuropathy
- Seizure disorder
Less Severe
- Acute cognitive impairment
- Body odor
- Dizziness
- Drowsy
- Gagging
- Memory impairment
- Tremor
Contraindicated
None
Severe Precaution
None
Management or Monitoring Precaution
Glycerol Phenylbutyrate
Monitor phenylacetate (PAA) levels and PAA to phenylacetylglutamine (PAGN) ratio to guide dosing adjustment as necessary. Check for symptoms of vomiting, nausea, headache, somnolence, confusion, or sleepiness.
- 1 Day – 18 Years
- Monitor phenylacetate (PAA) levels and PAA to phenylacetylglutamine (PAGN) ratio to guide dosing adjustment as necessary. Check for symptoms of vomiting, nausea, headache, somnolence, confusion, or sleepiness.
Phenylbutyrates
- Severity Level:
2
- Additional Notes: Insuff human data avail
Contraindicated
None
| General | Excretion Potential | Effect on Infant | Notes |
| None |
Precaution Exists
Phenylbutyrates
Insufficient human data
| General | Excretion Potential | Effect on Infant | Notes |
| Evaluate use carefully | Unknown | Not known; no or inclusive data | Insufficient human data |
No Known Risk
None
| General | Excretion Potential | Effect on Infant | Notes |
| None |
Contraindicated
None
Precaution Exists
None
No Known Risk
None
- None
| Carbamoyl phosphate synthetase deficiency | |
| E72.29 | Other disorders of urea cycle metabolism |
| Citrullinemia | |
| E72.23 | Citrullinemia |
| Ornithine carbamoyltransferase deficiency | |
| E72.4 | Disorders of ornithine metabolism |
| 0-9 | A-Z |
|---|---|
| E72.23 | Citrullinemia |
| E72.29 | Other disorders of urea cycle metabolism |
| E72.4 | Disorders of ornithine metabolism |
Formulary Reference Tool