Sodium phenylbutyrate


Concise Prescribing Info
Indications/Uses
Adjunct for hyperammonaemia in patients w/ urea cycle disorders.
Dosage/Direction for Use
Adult : PO <20 kg: 450-600 mg/kg/day; ≥20 kg: 9.9-13.0 g/m2/day. Take in equally divided doses 3-6 times/day. Max: 20 g/day.
Dosage Details
Oral
Adjunct for hyperammonaemia in patients with urea cycle disorders
Adult: <20 kg: 450-600 mg/kg/day; ≥20 kg: 9.9-13.0 g/m2/day. To be taken in equally divided doses 3-6 times daily. Max: 20 g/day.
Child: <20 kg: 450-600 mg/kg/day; ≥20 kg: 9.9-13.0 g/m2/day. To be taken in equally divided doses 3-6 times daily. Max: 20 g/day.
Administration
Should be taken with food.
Contraindications
Acute hyperammonaemia.
Special Precautions
Renal and hepatic impairment. CHF and conditions whereby there is sodium retention with oedema. Monitor serum levels of phenylbutyrate and its metabolites, phenylacetate and phenylacetylglutamine regularly. Dietary protein restriction is recommended. Pregnancy and lactation.
Adverse Reactions
Amenorrhoea/menstrual dysfunction, decreased appetite, body odour, taste aversion, abdominal pain, gastritis, nausea, vomiting, peptic ulcer disease and pancreatitis. May cause metabolic acidosis, alkalosis, hyperchloraemia, hypophosphataemia, hyperuricaemia, hypernatraemia and hypokalaemia.
Overdosage
Discontinue drug and institute supportive measures.
Drug Interactions
Excretion may be affected by concurrent admin of probenecid. Corticosteroids may increase protein breakdown and serum levels of ammonia.
Action
Description: Sodium phenylbutyrate is a prodrug for sodium phenylacetate. It is used as an adjunct in the treatment of hyperammonaemia in patients with urea cycle disorders.
Pharmacokinetics:
Metabolism: Conjugates with glutamine via acetylation to form phenylacetylglutamine.
Excretion: Mainly excreted via the kidneys within 24 hr as the conjugate product.
Storage
Store at 15-30°C.
Disclaimer: This information is independently developed by MIMS based on Sodium phenylbutyrate from various references and is provided for your reference only. Therapeutic uses, prescribing information and product availability may vary between countries. Please refer to MIMS Product Monographs for specific and locally approved prescribing information. Although great effort has been made to ensure content accuracy, MIMS shall not be held responsible or liable for any claims or damages arising from the use or misuse of the information contained herein, its contents or omissions, or otherwise. Copyright © 2020 MIMS. All rights reserved. Powered by MIMS.com
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