Advice

following an abbreviated submission

sodium phenylbutyrate granules (Pheburane®) are accepted for use within NHS Scotland.

Indication under review: 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 presentation (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.

Sodium phenylbutyrate granules (Pheburane®) provide an alternative to sodium phenylbutyrate tablets at no additional cost but are more expensive than an existing brand of sodium phenylbutyrate granules. 

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Medicine details

Medicine name:
sodium phenylbutyrate (Pheburane)
SMC ID:
914/13
Indication:
Adjunctive therapy in the chronic management of urea cycle disorders, involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase or argininosuccinate synthetase.
Pharmaceutical company
Lucane Pharma
BNF chapter
Nutrition and blood
Submission type
Abbreviated
Status
Accepted
Date advice published
11 November 2013