Pheburane

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.

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.

PCOM-LUC-PH-V1
Date created April 2022