Sodium phenylbutyrate

Therapeutic indications

Sodium phenylbutyrate is indicated for:

Urea cycle disorders

Population group: only children (1 year - 12 years old) , adolescents (12 years - 18 years old) , adults (18 - 65 years old)

Sodium phenylbutyrate 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.

For this indication, competent medicine agencies globally authorize below treatments (click for details):

Urea cycle disorders

Population group: only newborns (0 - 40 days old) , infants (40 days - 1 year old)

Sodium phenylbutyrate 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.

For this indication, competent medicine agencies globally authorize below treatments (click for details):

Contraindications

Sodium phenylbutyrate is contraindicated in the following cases:

Pregnancy

Pregnancy

Lactation

Lactation

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Factors such as age, gender, and health history are evaluated to create a personalized medication regimen.

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