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 Oral Solution 350 mg Oral solution
⚠ 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 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 oral solution 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.
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•
have decreased kidney or liver function, since PHEBURANE oral solution is eliminated from the body through the kidney and liver.
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 oral solution, 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 oral solution is unlikely to affect your ability to drive and use machines. PHEBURANE oral solution contains sodium This medicine contains 124 mg (5.4 mmol) sodium (main component of cooking/table salt) in each g dose of sodium phenylbutyrate. This is equivalent to 6.2% of the recommended maximum daily dietary intake of sodium for an adult. The maximum daily dose of this medicine contains 2.5 g sodium This is equivalent to 125% of the recommended maximum daily dietary intake of sodium for an adult. 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. PHEBURANE oral solution contains aspartame This medicine contains 5.7 mg of aspartame per g dose of sodium phenylbutyrate. Aspartame is a source of phenylalanine. It may be harmful if you have phenylketonuria (PKU), a rare genetic disorder in which phenylalanine builds up because the body cannot remove it properly.
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Blackcurrant flavour topping contains propylene glycol This medicine contains 26.55 mg of propylene glycol per drop. If your baby is less than 4 weeks old, talk to your doctor or pharmacist before giving them this medicine, in particular if the baby is given other medicines that contain propylene glycol or alcohol.
3.
How to take it
PHEBURANE oral solution
Always take this medicine exactly as your doctor has told you. Check with your doctor or pharmacist if you are not sure. Dose The daily dose of PHEBURANE oral solution 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 liquid you should take. Method of administration PHEBURANE oral solution should be taken with meals. Use only the dosing syringe provided with PHEBURANE oral solution to measure a dose of PHEBURANE oral solution. Do not use other devices/spoons/syringes to administer a dose. The syringe is ranging from 0.5 g to 3 g with 0.25 increments. The graduation of the dosing syringe reflects the grams of sodium phenylbutyrate. Follow the instructions below to administer PHEBURANE oral solution: Administration for oral use 1. Open the bottle of PHEBURANE oral solution by pushing down on the cap and twisting to the left.
2.
Take the CE marked dosing syringe with attached bottle adapter from the sachet.
3.
Place (push) the adapter in the neck of the bottle while the syringe is in the adapter.
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4.
Invert the bottle.
5.
Take the required quantity of PHEBURANE oral solution from the bottle (equivalent to the number of grams sodium phenyl butyrate as prescribed by your doctor) with use of the dosing syringe.
6.
Take the dosing syringe with PHEBURANE oral solution out of the adapter and pour the quantity of PHEBURANE oral solution in the dosing syringe into a glass with minimum 20 ml water.
7.
Close the bottle with PHEBURANE oral solution without removing the bottle adapter inserted in the neck of the bottle.
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8.
Add one drop of the flavour topping of your like (blackcurrant or lemon-mint) to the content of the glass of water; swirl gently, and then drink (If one drop of flavour topping would not provide the taste intensity of your like, you could use 2 drops).
9.
After each administration, wash the syringe with cold to lukewarm water only.
PHEBURANE oral solution must be taken with a special diet reduced in protein. You should take PHEBURANE oral solution with each meal or feeding. In small children this can be 4 to 6 times per day. PHEBURANE oral solution can also be administered by nasogastric or gastrostomy tubes. PHEBURANE oral solution can be administered with tubes of a diameter of 2 mm (7-8 French) and larger. Use the provided oral syringe to measure your dose and follow the instructions below: Preparation for nasogastric tube or gastrostomy tube administration 1. 2. 3. 4.
Follow Step 1 to Step 5 of the Method of administration for oral use; The oral solution of Pheburane is ready-to use and no dilution is needed; When used via nasogastric/gastrostomy the flavour topping should not be added; Insert the tip of the syringe filled with the medicine onto the tip of the nasogastric/gastrostomy tube; 5. Use the plunger of the dosing syringe to administer the prescribed dose of PHEBURANE oral solution into the nasogastric/gastrostomy tube; 6. After each administration, the tube should be flushed once with the adequate volume of lukewarm water and allowed to drain. For adults, 20 ml of lukewarm water should be used. For children weighing less than 20 kg and neonates use 3 mL of water. You will need to take this medicine and to follow a diet throughout your life. If you take more PHEBURANE oral solution 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;
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• • •
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 oral solution 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.
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 oral solution. 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), stomachache, vomiting, nausea, constipation, abnormal skin door, 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 the Yellow Card Scheme at www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store. By reporting side effects you can help provide more information on the safety of this medicine.
5.
How to store it
PHEBURANE oral solution
Keep this medicine out of the sight and reach of children. Do not use PHEBURANE oral solution 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. Once the PHEBURANE oral solution bottle is first open, you must use your medicine within 4 weeks of opening. The bottle should be discarded even if it is not empty.
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Once the flavour bottle is first open, you must use it within 4 weeks of opening. The bottle should be discarded even if it is not empty. This medicine does not require any special storage conditions. 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
What PHEBURANE oral solution contains The active substance is sodium phenylbutyrate. Each mL of liquid contains 350 mg of sodium phenylbutyrate. The other ingredients are: purified water, aspartame (E951), sucralose (E955), glycerol (E422), hydroxyethylcellulose (E1525) (See section 2 "PHEBURANE oral solution contains aspartame"). Flavour toppings: • Blackcurrant flavour topping consisting of blackcurrant and mint flavouring, containing propylene glycol (E1520). • Lemon-mint flavour topping consisting of lemon and mint flavouring. What PHEBURANE oral solution looks like and contents of the pack PHEBURANE oral solution is clear, colourless to pale yellow liquid. Each pack contains: One amber glass bottle containing 100 mL of the oral solution and closed with a plastic childresistant cap; One dosing syringe with ranging from 0.5 g to 3 g with 0.25 increments to measure the dose in grams of sodium phenylbutyrate; Bottle Adapter; One amber glass bottle containing 3 mL of lemon-mint flavour topping; One amber glass bottle containing 3 mL of blackcurrant flavour topping. 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: United Kingdom Lucane Pharma Tel: + 33 153 868 750 [email protected] This leaflet was last revised in September/2024
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Frequently asked questions about Pheburane Oral Solution 350 mg Oral solution
How do I take Pheburane Oral Solution 350 mg Oral solution?
Pheburane Oral Solution 350 mg Oral solution comes as oral solution containing 350mg. 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 Oral Solution 350 mg Oral solution?
The active substance in Pheburane Oral Solution 350 mg Oral solution is sodium phenylbutyrate.
Where does this information come from?
This leaflet reproduces the patient information leaflet approved for Pheburane Oral Solution 350 mg Oral solution, 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 Oral Solution 350 mg Oral solution 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 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 of sodium phenylbutyrate 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.
Paediatric population
• The usual total daily dose of sodium phenylbutyrate in paediatric patients in clinical experience is:
• 450 - 600 mg/kg/day in neonates, infants and children weighing less than 20 kg.
• 9.9 - 13 g/m2/day in children weighing more than 20 kg.
Method of administration
PHEBURANE oral solution is for oral use.
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).
A dosing syringe with a press in bottle adapter (PIBA) is provided for accurate measurement of the prescribed dose of the oral solution. The PIBA allows to connect the dosing syringe to the bottle and to dose PHEBURANE oral solution.
Only the dosing syringe provided with PHEBURANE oral solution should be used to measure a dose of PHEBURANE oral solution. No other devices/spoons/syringes should be used to administer PHEBURANE oral solution.
The syringe is graduated in grams of sodium phenylbutyrate (from 0.5 g to 3 g of sodium phenylbutyrate.
PHEBURANE oral solution can also be administered by nasogastric or gastrostomy tubes.
Instructions for oral administration and administration via nasogastric or gastrostomy tube are provided in section 6.6.
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
• Each gram of PHEBURANE oral solution (2.86 mL of PHEBURANE oral solution) contains 124 mg (5.4 mmol) of sodium. The maximum daily dose of sodium phenylbutyrate is 20 g (57.14 mL of PHEBURANE oral solution), this would bring an associated amount of: 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 oral solution is not recommended for the management of acute hyperammonaemia, which is a medical emergency.
Excipients with known effect
PHEBURANE 350 mg/mL oral solution
• This medicinal product contains 124 mg (5.4 mmol) sodium per g dose of sodium phenylbutyrate, equivalent to 6.2% of the WHO recommended maximum daily intake of 2 g sodium for an adult.
The maximum daily dose of this medicinal product contains 2.5 g sodium , equivalent to 125% of the WHO recommended maximum daily intake of 2 g of sodium for an adult. PHEBURANE oral solution is considered high in sodium. This should be particularly taken into account for those on a low salt diet.
• This medicinal product contains 5.7 mg aspartame per g dose of sodium phenylbutyrate. Aspartame is a source of phenylalanine. It may be harmful for persons who have phenylketonuria (PKU), a rare genetic disorder in which phenylalanine builds up because the body cannot remove it properly. Neither non-clinical nor clinical data are available to assess aspartame (E951) use in infants below 12 weeks of age.
Blackcurrant flavour topping
The blackcurrant flavour topping contains 26.55 mg propylene glycol per drop.
If your baby is less than 4 weeks old, talk to your doctor or pharmacist before giving them this medicine, in particular if the baby is given other medicines that contain propylene glycol or alcohol.
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 door
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/day) 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 the Yellow Card Scheme at 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 (1,370 mg/kg). The patient developed diarrhoea, 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 Oral Solution 350 mg Oral solution. 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.