Sodium Phenylbutyrate
- Atc Codes:A16AX03
- CAS Codes:1716-12-7
- PHARMGKB ID:1716-12-7
Table of contents
- Brand Names
- Chemistry
- Pharmacologic Category
- Mechanism of Action
- Therapeutic Use
- Pregnancy and Lactation Implications
- Contraindications
- Warnings and Precautions
- Adverse Reactions
- Caution and personalized dose adjustment in patients with the following genotypes
- Other genes that may be involved
- Drug Interactions
- Nutrition/Nutraceutical Interactions
- Dosage
- Pharmacokinetics and Pharmacodynamics
- Special Considerations
Brand Names
Europe
Austria: Ammonaps; Bulgaria: Ammonaps; Czech Republic: Ammonaps; Denmark: Ammonaps; Estonia: Ammonaps; Finland: Ammonaps; France: Ammonaps; Germany: Ammonaps; Greece: Ammonaps; Hungary: Ammonaps; Finland: Ammonaps; France: Ammonaps; Ireland: Ammonaps; Italy: Ammonaps; Latvia: Ammonaps; Lithuania: Ammonaps; Luxembourg: Ammonaps; Malta: Ammonaps; Netherlands: Ammonaps; Poland: Ammonaps; Portugal: Ammonaps; Romania: Ammonaps; Slovakia: Ammonaps; Slovenia: Ammonaps; Spain: Ammonaps; Sweden: Ammonaps; UK: Ammonaps.
North America
USA: Buphenyl.
Drug combinations
Chemistry
Sodium Phenylbutyrate: C~10~H~11~NaO~2~. Mw: 186.18. Benzenebutanoic acid, sodium salt. CAS-1716-12-7 (1995).
Pharmacologic Category
Ammonia Detoxicants. (ATC-Code: A16AX03).
Mechanism of action
A prodrug which, when given orally, is rapidly converted to phenylacetate, in turn conjugated with glutamine to form active compound phenylacetylglutamine. Phenylacetylglutamine serves as substitute for urea and is excreted in urine whereby it carries with it 2 moles of nitrogen per mole of phenylacetylglutamine and can thereby assist in clearance of nitrogenous waste in urea cycle disorders.
Therapeutic use
Adjunctive therapy in chronic management of urea cycle disorder involving deficiencies of carbamoylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase.
Pregnancy and lactiation implications
Animal reproduction studies not conducted. Excretion in breast milk unknown (use with caution in nursing women).
Unlabeled use
Contraindications
Acute hyperammonemia.
Warnings and precautions
May cause sodium and fluid retention (caution where fluid accumulation may be poorly tolerated). Hyperammonemia and hyperammonemic encephalopathy may still occur while on therapy. Use with caution in hepatic/renal impairment. Contains sodium 125 mg/g of sodium phenylbutyrate (caution, if at all, in patients who must maintain low sodium intake). Use of sodium phenylbutyrate tablets in children weighing <20 kg not recommended.