Each gram of granules contains 483 mg of sodium phenylbutyrate. Each bottle contains 174 g of granules.
Pheburane is indicated as adjunctive therapy in the chronic management of urea cycle disorders, involving deficiencies of carbamylphosphate
synthetase, ornithine transcarbamylase or argininosuccinate synthetase.
It is indicated in all patients with neonatal-onset disease (complete enzyme deficiencies, presenting within the first 28 days of life). It is also indicated in patients with late-onset disease (partial enzyme deficiencies, presenting after the first month of life) who have a history of hyperammonaemic encephalopathy.
Pheburane is a taste-masked formulation of sodium phenylbutyrate achieved via a specific tasteless coating.
Each gram (1000 mg) of Pheburane contains 483 mg of sodium phenylbutyrate.
The dedicated dosing device is specifically calibrated in grams of sodium phenylbutyrate.
No need to calculate and write the dose in grams of Pheburane.
Adapted for paediatric use.
No specific storage requirements.
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The page was last updated on 21/09/2020