Pharmacy Guide
Patient leaflets and SmPCs, drug interaction checker, official dosages and NHS pharmacy opening hours — all in one place.
Pharmacy Guide
Patient leaflets and SmPCs, drug interaction checker, official dosages and NHS pharmacy opening hours — all in one place.
Pheburane 483 mg/g granules
⚠ This medicine appears to have been discontinued
The electronic medicines compendium (emc) no longer publishes a Summary of Product Characteristics for this product, which usually means it is no longer marketed in the UK. The patient leaflet below is kept for reference, but the product may not be available.
If you were prescribed this medicine, other products containing Sodium phenylbutyrate may still be available. Do not stop your treatment — ask your pharmacist or GP what to use instead.
What it is and what it is used for
for
PHEBURANE contains the active substance sodium phenylbutyrate which is used to treat patients of all ages with urea cycle disorders. These rare disorders are due to a deficiency of certain liver enzymes which are necessary to eliminate waste nitrogen in the form of ammonia. Nitrogen is a building block of proteins, which are an essential part of the food we eat. As the body breaks down protein after eating, waste nitrogen, in the form of ammonia, accumulates because the body cannot eliminate it. Ammonia is especially toxic for the brain and leads, in severe cases, to reduced levels of consciousness and to coma. PHEBURANE helps the body to eliminate waste nitrogen, reducing the amount of ammonia in your body. However PHEBURANE must be used along with a diet reduced in proteins, designed especially for you by the doctor and the dietician. You must follow this diet carefully. 2.
What you need to know before you take it
e PHEBURANE
Do not take PHEBURANE if you:
- are allergic to sodium phenylbutyrate or any of the other ingredients of this medicine (listed in section 6).
- are pregnant.
- are breast-feeding. Warnings and precautions Talk to your doctor or pharmacist before taking PHEBURANE if you:
- suffer from congestive heart failure (a type of heart disease where the heart cannot pump enough blood around the body) or a decrease in your kidney function.
- have decreased kidney or liver function, since PHEBURANE is eliminated from the body through the kidney and liver. 1
PHEBURANE will not prevent the occurrence of an acute excess of ammonia in the blood, a condition which usually constitutes a medical emergency. If this happens you will develop symptoms such as feeling sick (nausea), being sick (vomiting), confusion and will need to get urgent medical help. If you need laboratory tests, it is important to remind your doctor that you are taking PHEBURANE, since sodium phenylbutyrate may interfere with certain laboratory test results (such as blood electrolytes or protein, or liver function tests) In case of any doubt, ask your doctor or pharmacist. Other medicines and PHEBURANE Tell your doctor or pharmacist if you are taking, have recently taken or might take any other medicines. It is especially important to tell your doctor if you are taking medicines containing:
- valproate (an antiepileptic medicine),
- haloperidol (used in certain psychotic disorders),
- corticosteroids (medicines that are used to provide relief for inflamed areas of the body),
- probenecid (for treatment of hyperuricaemia, high levels of uric acid in the blood, associated with gout) These medicines may change the effect of PHEBURANE and you will need more frequent blood tests. If you are uncertain if your medicines contain these substances, you should check with your doctor or pharmacist. Pregnancy and breast-feeding Do not use PHEBURANE if you are pregnant, because this medicine can harm your unborn baby. If you are a woman who could get pregnant, you must use reliable contraception, during treatment with PHEBURANE. Talk to your doctor for the details. Do not use PHEBURANE if you are breast-feeding, because this medicine can pass into the breast milk and may harm your baby. Driving and using machines PHEBURANE is unlikely to affect your ability to drive and use machines. PHEBURANE contains sodium and sucrose This medicine contains 124 mg (5.4 mmol) of sodium per 1 g of sodium phenylbutyrate. Talk to your doctor or pharmacist if you need 3 or more grams daily for a prolonged period, especially if you have been advised to follow a low salt (sodium) diet. This medicine contains 768 mg of sucrose per 1 g of sodium phenylbutyrate. This should be taken into account if you have diabetes mellitus. If you have been told by your doctor that you have an intolerance to some sugars, contact your doctor before taking this medicine. 3.
How to take it
PHEBURANE
Always take this medicine exactly as your doctor has told you. Check with your doctor or pharmacist if you are not sure. 2
Dosage The daily dose of PHEBURANE will be based on your body weight or body surface and adjusted according to your protein tolerance and diet. You will need regular blood tests to determine the correct daily dose. Your doctor will tell you the amount of granules you should take. Method of administration You should take PHEBURANE by mouth. Because it dissolves slowly, PHEBURANE should not be administered through a gastrostomy (tube that goes through the abdomen to the stomach) or through a nasogastric tube (tube that goes through the nose to the stomach). PHEBURANE must be taken with a special diet reduced in protein. You should take PHEBURANE with each meal or feeding. In small children this can be 4 to 6 times per day. The doses of PHEBURANE prescribed by your doctor are expressed in grams of sodium phenylbutyrate. A calibrated measuring spoon which dispenses up to 3 g of sodium phenylbutyrate at a time is provided with this medicine. Only use this measuring spoon to measure out the dose of PHEBURANE. The measuring spoon must not be used for any other medicine. To measure the dose:
- Lines on the spoon indicate the amount of PHEBURANE in gram of sodium phenylbutyrate. Take the correct amount as prescribed by your doctor.
- Pour granules directly into the spoon as shown by the picture (on the outer carton and in this leaflet)
- Tap the spoon once on a table to give a horizontal level of granules and continue filling if necessary The granules can be directly swallowed with a drink (water, fruit juices, protein-free infant formulas) or sprinkled on to a spoonful of solid foods (mashed potatoes or apple sauce). If you mix them with food, it is important that you take it immediately. This will keep the granules from producing any taste. You will need to take this medicine and to follow a diet throughout your life. If you take more PHEBURANE than you should Patients who have taken very high doses of sodium phenylbutyrate experienced:
- sleepiness, tiredness, light-headedness and less frequently confusion,
- headache,
- changes in taste (taste disturbances),
- decrease in hearing,
- disorientation,
- impaired memory,
- worsening of existing neurological conditions. If you experience any of these symptoms, you should immediately contact your doctor or the nearest hospital emergency department for supportive treatment. If you forget to take PHEBURANE You should take a dose as soon as possible with your next meal. Make sure that there are at least 3 hours between two doses. Do not take a double dose to make up for a forgotten dose. If you have any further questions on the use of this medicine, ask your doctor or pharmacist. 3
4.
Possible side effects
Like all medicines, this medicine can cause side effects, although not everybody gets them. If persistent vomiting occurs, you should contact your doctor immediately. Very common side effects (may affect more than 1 in 10 people): irregular menstrual periods and stopping of menstrual periods in fertile women. If you are sexually active and your period stops altogether, do not assume that this is caused by PHEBURANE. If this occurs, please discuss it with your doctor, because the absence of your period may be caused by pregnancy (see 'Pregnancy and breast-feeding' section above) or by menopause. Common side effects (may affect more than 1 in 100 people): changes in number of blood cells (red cells, white cells and platelets), changes in the amount of bicarbonate in the blood, reduced appetite, depression, irritability, headache, fainting, fluid retention (swelling), changes in taste (taste disturbances), stomach ache, vomiting, nausea, constipation, abnormal skin odor, rash, abnormal kidney function, weight gain, altered laboratory test values. Uncommon side effects (may affect more than 1 in 1,000 people): deficiency in red blood cells due to failure of the bone marrow, bruising, altered heart rhythm, rectal bleeding, inflammation of the stomach, stomach ulcer, inflammation of the pancreas. Reporting of side effects If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via Yellow Card Scheme Website: www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store Ireland: HPRA Pharmacovigilance Website: www.hpra.ie
5.
How to store it
PHEBURANE
Keep this medicine out of the sight and reach of children. Do not use PHEBURANE after the expiry date which is stated on the carton and the bottle label after EXP. The expiry date refers to the last day of that month. After the first opening, PHEBURANE can be used within 45 days. Do not throw away any medicines via wastewater or household waste. Ask your pharmacist how to throw away medicines you no longer use. These measures will help protect the environment. 6.
Contents of the pack and other information
4
What PHEBURANE contains The active substance is sodium phenylbutyrate. Each gram of granules contains 483 mg of sodium phenylbutyrate. The other ingredients are: sugar spheres (sucrose and maize starch, see section 2 'PHEBURANE contains sucrose), hypromellose, ethylcellulose N7, macrogol 1500, povidone K25. What PHEBURANE looks like and contents of the pack PHEBURANE granules are white to off-white. The granules are packaged in a plastic bottle with child-resistant closure and a dessicant. Each bottle contains 174g of granules. Each carton contains 1 bottle. A calibrated measuring spoon is provided. Marketing Authorisation Holder and Manufacturer Eurocept International BV Trapgans 5 1244 RL Ankeveen The Netherlands For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder: Lucane Pharma Tel: + 33 153 868 750 [email protected] This leaflet was last revised in September 2024. There are also links to other websites about rare diseases and treatments.
5
Frequently asked questions about Pheburane 483 mg/g granules
How do I take Pheburane 483 mg/g granules?
Pheburane 483 mg/g granules comes as granules containing 483mg. Always follow the dose your doctor or pharmacist has given you, and read the leaflet that comes with the medicine.
What is the active substance in Pheburane 483 mg/g granules?
The active substance in Pheburane 483 mg/g granules is sodium phenylbutyrate.
Where does this information come from?
This leaflet reproduces the patient information leaflet approved for Pheburane 483 mg/g granules, as published on the electronic medicines compendium (emc). The version printed inside your medicine’s packaging is the one that applies to you.
Can I get Pheburane 483 mg/g granules without a prescription?
Whether a medicine is available over the counter or on prescription only depends on its licence. Check the leaflet, or ask your pharmacist — they can tell you straight away.
About this leaflet
The text above reproduces the patient information leaflet approved for this medicine, restructured for easier reading.
- Source: electronic medicines compendium (emc), Datapharm
- Active substance: sodium phenylbutyrate
- Official document: view the original leaflet on emc →
⚕For healthcare professionals — Summary of Product Characteristics (SmPC)Full SmPC: dosage, interactions, contraindications, warnings+
4.1. Therapeutic indications
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.
4.2. Posology and method of administration
PHEBURANE treatment should be supervised by a physician experienced in the treatment of urea cycle disorders.
Posology
The daily dose should be individually adjusted according to the patient's protein tolerance and the daily dietary protein intake needed to promote growth and development.
The usual total daily dose of sodium phenylbutyrate in clinical experience is:
• 450 - 600 mg/kg/day in neonates, infants and children weighing less than 20 kg
• 9.9 - 13.0 g/m2/day in children weighing more than 20 kg, adolescents and adults.
The safety and efficacy of doses in excess of 20 g/day have not been established.
Therapeutic monitoring
Plasma levels of ammonia, arginine, essential amino acids (especially branched chain amino acids), carnitine and serum proteins should be maintained within normal limits. Plasma glutamine should be maintained at levels less than 1,000 μmol/L.
Nutritional management
PHEBURANE must be combined with dietary protein restriction and, in some cases, essential amino acid and carnitine supplementation.
Citrulline or arginine supplementation is required for patients diagnosed with neonatal-onset form of carbamyl phosphate synthetase or ornithine transcarbamylase deficiency at a dose of 0.17 g/kg/day or 3.8 g/m2/day.
Arginine supplementation is required for patients diagnosed with deficiency of argininosuccinate synthetase at a dose of 0.4 - 0.7 g/kg/day or 8.8 - 15.4 g/m2/day.
If caloric supplementation is indicated, a protein-free product is recommended.
Special populations
Renal and hepatic impairment
Since the metabolism and excretion of sodium phenylbutyrate involves the liver and kidneys, PHEBURANE should be used with caution in patients with hepatic or renal insufficiency.
Method of administration
PHEBURANE should be administered orally. Because of its slow dissolution, PHEBURANE should not be administered by nasogastric or gastrostomy tubes.
The total daily dose should be divided into equal amounts and given with each meal or feeding (e.g. 4-6 times per day in small children). The granules can be directly swallowed with a drink (water, fruit juices, protein-free infant formulas) or sprinkled on to a spoonful of solid foods (mashed potatoes or apple sauce); in this case, it is important that it is taken immediately in order to preserve the taste-masking.
A calibrated dosing spoon is provided which dispenses up to 3g of sodium phenylbutyrate by graduation of 250 mg.
4.3. Contraindications
• Hypersensitivity to the active substance or to any of the excipients listed in section 6.1.
• Pregnancy.
• Breast-feeding.
4.4. Special warnings and precautions for use
Content of clinically important electrolytes
• PHEBURANE contains 124 mg (5.4 mmol) of sodium per gram of sodium phenylbutyrate, corresponding to 2.5 g (108 mmol) of sodium per 20 g of sodium phenylbutyrate, which is the maximum daily dose. PHEBURANE should therefore be used with caution in patients with congestive heart failure or severe renal insufficiency, and in clinical conditions where there is sodium retention with oedema.
• Serum potassium should be monitored during therapy since renal excretion of phenylacetylglutamine may induce a urinary loss of potassium.
General considerations
• Even on therapy, acute hyperammonaemic encephalopathy may occur in a number of patients.
• PHEBURANE is not recommended for the management of acute hyperammonaemia, which is a medical emergency.
Excipients with known effect
• This medicinal product contains 124 mg sodium per gram, equivalent to 6.2% of the WHO recommended maximum daily intake for sodium.
The maximum daily dose of this medicinal product is equivalent to 125% of the WHO recommended maximum daily intake for sodium.
• PHEBURANE is considered high in sodium. This should be particularly taken into account for those on a low salt diet.
• This medicinal product contains 768 mg sucrose per gram. This should be taken into account in patients with diabetes mellitus. Patients with rare hereditary problems of fructose intolerance, glucose-galactose malabsorption or sucrase-isomaltase insufficiency should not take this medicinal product.
4.5. Interaction with other medicinal products and other forms of interaction
Concurrent administration of probenecid may affect renal excretion of the conjugation product of sodium phenylbutyrate. There have been published reports of hyperammonaemia being induced by haloperidol and by valproate. Corticosteroids may cause the breakdown of body protein and thus increase plasma ammonia levels. More frequent monitoring of plasma ammonia levels is advised when these medicinal products have to be used.
4.6. Fertility, pregnancy and lactation
Women of childbearing potential/Contraception in males and females
Effective contraceptive measures must be taken by women of child-bearing potential.
Pregnancy
There are no or limited amount of data from the use of sodium phenylbutyrate in pregnant women. Studies in animals have shown reproductive toxicity (see section 5.3).
Pheburane is contra-indicated during pregnancy (see section 4.3). Women of childbearing potential must use effective contraception during treatment.
Breast-feeding
Available pharmacodynamic/toxicological data in animals have shown excretion of sodium phenylbutyrate/metabolites in milk (see section 5.3). It is unknown whether sodium phenylbutyrate/metabolites are excreted in human milk. A risk to the newborns/infants cannot be excluded. Pheburane is contra-indicated during breast-feeding (see section 4.3).
Fertility
There is no evidence available on the effect of sodium phenylbutyrate on fertility.
4.7. Effects on ability to drive and use machines
PHEBURANE has negligible influence on the ability to drive and use machines.
4.8. Undesirable effects
Summary of safety profile
In clinical trials with sodium phenylbutyrate, 56 % of the patients experienced at least one adverse event and 78 % of these adverse events were considered as not related to sodium phenylbutyrate.
Adverse reactions mainly involved the reproductive and gastrointestinal system.
Tabulated list of adverse reactions
In the table below all adverse reactions are listed below, by system organ class and by frequency. Frequency is defined as very common (≥1/10), common (≥1/100 to <1/10), uncommon (≥1/1,000 to <1/100), rare (≥1/10,000 to <1/1,000), very rare (<1/10,000), not known (cannot be estimated from the available data). Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness.
System Organ Class
Frequency
Adverse reaction
Blood and lymphatic system disorders
Common
anaemia, thrombocytopenia, leukopenia, leukocytosis, thrombocytosis
Uncommon
aplastic anaemia, ecchymosis
Metabolism and nutrition disorders
Common
metabolic acidosis, alkalosis, decreased appetite
Psychiatric disorders
Common
depression, irritability
Nervous system disorders
Common
syncope, headache
Cardiac disorders
Common
oedema
Uncommon
arrhythmia
Gastrointestinal disorders
Common
abdominal pain, vomiting, nausea, constipation, dysgeusia
Uncommon
pancreatitis, peptic ulcer, rectal haemorrhage, gastritis
Skin and subcutaneous tissue disorders
Common
rash, abnormal skin odor
Renal and urinary disorders
Common
renal tubular acidosis
Reproductive system and breast disorders
Very common
amenorrhea, irregular menstruation
Investigations
Common
Decreased blood potassium, albumin, total protein and phosphate. Increased blood alkaline phosphatase, transaminases, bilirubin, uric acid, chloride, phosphate and sodium. Increased weight
Description of selected adverse reactions
A probable case of toxic reaction to sodium phenylbutyrate (450 mg/kg/d) was reported in an 18-year old anorectic female patient who developed a metabolic encephalopathy associated with lactic acidosis, severe hypokalaemia, pancytopaenia, peripheral neuropathy, and pancreatitis. She recovered following dose reduction except for recurrent pancreatitis episodes that eventually prompted treatment discontinuation.
Reporting of suspected adverse reactions
Reporting suspected adverse reactions after authorisation of the medicinal product is important. It allows continued monitoring of the benefit/risk balance of the medicinal product. Healthcare professionals are asked to report any suspected adverse reactions via Yellow Card Scheme Website: www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store.
4.9. Overdose
One case of overdose occurred in a 5-month old infant with an accidental single dose of 10 g (1370 mg/kg). The patient developed diarrhea, irritability and metabolic acidosis with hypokalaemia. The patient recovered within 48 hours after symptomatic treatment.
These symptoms are consistent with the accumulation of phenylacetate, which showed dose-limiting neurotoxicity when administered intravenously at doses up to 400 mg/kg/day. Manifestations of neurotoxicity were predominantly somnolence, fatigue and light-headedness. Less frequent manifestations were confusion, headache, dysgeusia, hypoacusis, disorientation, impaired memory and exacerbation of a pre-existing neuropathy.
In the event of an overdose, the treatment should be discontinued and supportive measures be instituted. Haemodialysis or peritoneal dialysis may be beneficial.
🇷🇴 Known in Romania as
Medicines sold in Romania with the same active substance: Cunoscut în România ca
⚠ Not the same combination. This medicine contains Sodium phenylbutyrate. The products below do not contain exactly the same set of active substances — they are not direct substitutes.
- AMMONAPS 500mg prescription partial — not the same combinationNATRII PHENYLBUTYRATE · taken by mouth
Some of these do not contain exactly the same active substances — check each one. The strength, the form and whether you need a prescription can differ. Always ask a pharmacist before you switch. Romanian medicines in the UK →
💬 Ask about this leaflet
Ask anything about Pheburane 483 mg/g granules. The assistant answers only from this leaflet — if the leaflet does not cover it, it will say so. It does not give medical advice.
Answers come from the patient leaflet published on the electronic medicines compendium (emc). They are not medical advice. Ask a pharmacist or your GP if you are unsure. For urgent help call NHS 111, or 999 in an emergency.