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Pheburane (sodium phenylbutyrate)

About Pheburane® and Prescribing Information

Pheburane (sodium phenylbutyrate) 483 mg/g granules

Flag of Ireland Proveca only market Pheburane in Ireland

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.

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Prescribing Information (Ireland)

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Summary of Product Characteristics (Ireland)

Benefits of Pheburane®

  • Pheburane® is a taste-masked formulation of sodium phenylbutyrate achieved via a taste-masking coating1,2
  • Each gram (1000 mg) of Pheburane® contains 483 mg of sodium phenylbutyrate1
  • A calibrated measuring spoon is provided. It dispenses up to 3g of sodium phenylbutyrate by graduation of 250 mg1
  • No specific storage requirements1

A MULTI-COATED GRANULE FORMULATION2

NUMBER OF HYPERAMMONEMIC EPISODES UNDER MARKETED NAPB AND PHEBURANE®3

Patient No Age (years) Before PHEBURANE®
(in previous 6 months)		 Under PHEBURANE®
(exposure duration)
5 11 3 None (6 months)
9 24 1 None (11 months)
10 4 2 None (11 months)
11 3 2 None (10 months)
17 15 1 None (8 months)
20 22 1 None (6 months)
22 64 3 None (3 months)
23 18 2 None (3 months)
24 4 1 None (3 months)
25 4 3 None (3 months)

NaPB sodium phenylbutyrate
In the cohort ATU, the number of hyperammonemic episodes decreased from 20 reported in 10 patients in the previous 6 months under the licensed NaPB to zero in the same patients over a period of 3–11 months treatment with Pheburane.3

A BOTTLE OF PHEBURANE® CONTAINS 174 G OF COATED GRANULES CORRESPONDING WITH 84 G OF SODIUM PHENYLBUTYRATE1

Calibrated dosing spoon graduated in 250 mg increments of sodium phenylbutyrate1

Please refer to the SmPC for full details on the product, safety information and dosing schedule.

 

Please see information below to report adverse events in the Republic of Ireland

Adverse events should be reported. Reporting forms and information can be found at: www.hpra.ie.
Adverse events should also be reported to Proveca Limited.
Phone: +44 333 200 1866 E-mail: medinfo@proveca.com

References:
1.Summary of Product Characteristics PHEBURANE® (MHRA: 1 January, 2021).
2. Guffon N, Kibleur Y, Copalu W, et al. Developing a new formulation of sodium phenylbutyrate. Archives of Disease in
Childhood 2012; 97:1081-1085
3.Kibleur Y, Dobbelaere D, Barth M, et al. Results from a Nationwide Cohort Temporary Utilization Authorization (ATU)
Survey of Patients in France Treated with Pheburane (Sodium Phenylbutyrate) Taste-Masked Granules. Pediatric
Drugs 2014; 16(5): 407-415.

IE-PHB-23-0001
Updated June 2023