Référentiel des médicaments
Glycerol phenylbutyrate
Glycerol phenylbutyrate is a medicine in the class miscellaneous.
- Classe médicamenteuse: Miscellaneous
Dernière mise à jour Sources: US FDA label, EMA SmPC
Réservé aux professionnels de santé — à titre de référence uniquement. Cette page est un bref résumé, pas un avis médical, et ne remplace ni la notice officielle ni le jugement clinique. Vérifiez toujours avant de prescrire ou de délivrer.
Le contenu clinique de cette page n'est disponible qu'en anglais.
L'essentiel
- Classe médicamenteuse
- Miscellaneous
- Code ATC
- A16AX09
- Formes courantes
- solution
- Dosages courants
- 1.1 g/ml
- Interactions connues
- 28 (Majeure: 3 · Modérée: 12 · Mineure: 13)
- Noms commerciaux
- 2
- Pays et marchés
- 2
Mécanisme d'action
RAVICTI is a triglyceride containing 3 molecules of PBA. Its active moiety PAA conjugates with glutamine in the liver and kidneys to form PAGN, which the kidneys excrete. PAGN contains 2 moles of nitrogen, like urea, and provides an alternate route for waste nitrogen excretion.
Posologie adulte habituelle (résumé de la notice)
UCD, phenylbutyrate-naive patients: 4.5 to 11.2 mL/m2/day (5 to 12.4 g/m2/day) orally, given in 3 equally divided doses in patients 2 years and older; starting dose 4.5 mL/m2/day for patients with some residual enzyme activity; maximum total daily dosage 17.5 mL
Grossesse et allaitement
- Grossesse
- Unknown Limited human data are insufficient to inform risk. Animal studies showed maternal toxicity and, at higher doses in rats, reduced fetal weights and skeletal variations.
- Allaitement
- Unsafe No human milk data. Because of potential for serious adverse reactions in the infant, breastfeeding is not recommended.
Effets indésirables signalés
- Diarrhea
- Flatulence
- Headache
- Abdominal pain
- Vomiting
- Decreased appetite
Contre-indications
- Known hypersensitivity to phenylbutyrate.
Précautions particulières
- Neurotoxicity: PAA may be toxic; reduce dosage for neurotoxicity symptoms.
- Pancreatic insufficiency or intestinal malabsorption may reduce drug absorption and ammonia control; monitor ammonia closely.
- Not indicated for acute hyperammonemia.
Informez votre pharmacien ou votre médecin en cas de grossesse ou d'allaitement, de maladie rénale ou hépatique, d'allergies, ainsi que de tout autre médicament ou complément que vous prenez.
Principales interactions (28)
- Dexamethasone + Glycerol phenylbutyrate Major
- Glycerol phenylbutyrate + Sodium valproate Major
- Glycerol phenylbutyrate + Valproic acid Major
- Cyclosporin + Glycerol phenylbutyrate Moderate
- Dexamethasone-neomycin-polymyxin + Glycerol phenylbutyrate Moderate
- Dexamethasone-tobramycin + Glycerol phenylbutyrate Moderate
- Fludrocortisone + Glycerol phenylbutyrate Moderate
- Glycerol phenylbutyrate + Haloperidol Moderate
- Glycerol phenylbutyrate + Hydrocortisone Moderate
- Glycerol phenylbutyrate + Methylprednisolone Moderate
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- Dosing by age, kidney and liver function, and further dose lines
- Renal and hepatic dose adjustments
- Paediatric dosing
- Dosing in older adults
- How to give it
- Pharmacokinetics
- Patient counselling points
- Food interactions
- IV preparation and dilution
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Questions fréquentes
What is Glycerol phenylbutyrate used for?
Glycerol phenylbutyrate is a medicine in the class miscellaneous.
What side effects have been reported with Glycerol phenylbutyrate?
Diarrhea, Flatulence, Headache, Abdominal pain, Vomiting, Decreased appetite.
Can Glycerol phenylbutyrate be taken in pregnancy or while breastfeeding?
Pregnancy: Unknown. Limited human data are insufficient to inform risk. Animal studies showed maternal toxicity and, at higher doses in rats, reduced fetal weights and skeletal variations. Breastfeeding: Unsafe. No human milk data. Because of potential for serious adverse reactions in the infant, breastfeeding is not recommended.
What does Glycerol phenylbutyrate interact with?
MediHelp lists 28 known interactions for Glycerol phenylbutyrate. Major: 3 · Moderate: 12 · Minor: 13. The most serious include Dexamethasone, Sodium valproate, Valproic acid.
Monographies complètes, posologies et vérificateur d'interactions dans l'application MediHelp
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