Sodium phenylbutyrate (Pheburane®)
All Wales Medicines Strategy Group (AWMSG)
Record ID 32013001032
English
Authors' recommendations:
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.
Details
Project Status:
Completed
Year Published:
2013
URL for published report:
http://www.awmsg.org/awmsgonline/app/appraisalinfo/2227
English language abstract:
An English language summary is available
Publication Type:
Not Assigned
Country:
Wales, United Kingdom
MeSH Terms
- Humans
- Phenylbutyrates
- Urea Cycle Disorders, Inborn
Contact
Organisation Name:
All Wales Medicines Strategy Group
Contact Address:
All Wales Therapeutics and Toxicology Centre, Academic Centre, University Hospital Llandough, Penlan Road, Penarth, Vale of Glamorgan CF64 2XX
Contact Name:
AWTTC@wales.nhs.uk
Contact Email:
AWTTC@wales.nhs.uk
Copyright:
AWTTC
This is a bibliographic record of a published health technology assessment from a member of INAHTA or other HTA producer. No evaluation of the quality of this assessment has been made for the HTA database.