About Pheburane® and Prescribing Information
Pheburane (sodium phenylbutyrate) 483 mg/g granules
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.
Prescribing Information (Ireland)
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