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sodium phenylbutyrate (Pheburane®)


Reference No. 2227

Publication date:
13/12/2013


Last review date:
01/12/2016

Appraisal information

sodium phenylbutyrate (Pheburane®) 483 mg/g granules


Company: Lucane Pharma
BNF category: Nutrition and blood
NMG meeting date: 23/10/2013
AWMSG meeting date: 20/11/2013
   
   
Submission Type: Limited Submission
Status: Recommended
Advice No: 3413
Ministerial ratification: 06/12/2013

Current Progress


Submission
received
NMG
meeting
AWMSG
meeting
Ministerial
Ratification

AWMSG advice

Sodium phenylbutyrate (Pheburane®) is recommended as an option for use within NHS Wales as adjunctive therapy in the chronic management of urea-cycle disorders, involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase or argininosuccinate synthetase; in all patients with neonatal-onset presentation (complete enzyme deficiencies, presenting within the first 28 days of life) and in patients with late-onset disease (partial enzyme deficiencies, presenting after the first month of life) who have a history of hyperammonaemic encephalopathy.
Final Appraisal Recommendation (FAR)
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AWMSG Secretariat Appraisal Report (ASAR)
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Clinical Expert (CE) Summary
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