Referência de medicamentos
Glycerol phenylbutyrate
Glycerol phenylbutyrate is a medicine in the class miscellaneous.
- Classe do medicamento: Miscellaneous
Última atualização Fontes: US FDA label, EMA SmPC
Para profissionais de saúde — apenas referência. Esta página é um resumo breve, não é aconselhamento médico nem substitui a bula oficial ou o julgamento clínico. Verifique sempre antes de prescrever ou dispensar.
O conteúdo clínico desta página está disponível apenas em inglês.
Pontos-chave
- Classe do medicamento
- Miscellaneous
- Código ATC
- A16AX09
- Formas farmacêuticas comuns
- solução
- Concentrações comuns
- 1.1 g/ml
- Interações conhecidas
- 28 (Grave: 3 · Moderada: 12 · Leve: 13)
- Nomes comerciais
- 2
- Países e mercados
- 2
Como funciona
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.
Dose habitual em adultos (resumo da bula)
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
Gravidez e amamentação
- Gravidez
- 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.
- Amamentação
- Unsafe No human milk data. Because of potential for serious adverse reactions in the infant, breastfeeding is not recommended.
Efeitos adversos relatados
- Diarrhea
- Flatulence
- Headache
- Abdominal pain
- Vomiting
- Decreased appetite
Contraindicações
- Known hypersensitivity to phenylbutyrate.
Quem deve ter cuidado
- 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.
Informe ao farmacêutico ou ao médico sobre gravidez ou amamentação, doença renal ou hepática, alergias e todos os outros medicamentos ou suplementos que você usa.
Principais interações (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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Perguntas frequentes
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.
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