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.
Ucedane 200 mg dispersible tablets
⚠ 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 Carglumic acid 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
Ucedane can help eliminating excessive ammonia plasma levels (elevated ammonia level in the blood). Ammonia is especially toxic for the brain and leads, in severe cases, to reduced levels of consciousness and to coma. Hyperammonaemia may be due to
- the lack of a specific liver enzyme N- acetylglutamate synthase. Patients with this rare disorder are not able to eliminate nitrogen waste, which builds up after eating protein. This disorder persists during the entire life of the affected patient and therefore the need for this treatment is lifelong.
- isovaleric acidaemia, methylmalonic acidaemia or propionic acidaemia. Patients suffering from one of these disorders need treatment during the hyperammonaemia crisis.
2.
What you need to know before you take it
e Ucedane
Do not take Ucedane: if you are allergic to carglumic acid or any of the other ingredients of Ucedane (listed in section 6); Do not take Ucedane during breast-feeding. Warnings and precautions Talk to your doctor or pharmacist before taking Ucedane. Ucedane treatment should be initiated under the supervision of a physician experienced in the treatment of metabolic disorders. Your doctor will evaluate your individual responsiveness to carglumic acid before initiating any long term treatment. The dose should be individually adjusted in order to maintain normal ammonia plasma levels. Your doctor may prescribe supplemental arginine or restrict your protein intake. 1
PL Ucedane UK July 2024
In order to follow-up your condition and your treatment, your doctor may examine your liver, your kidneys, your heart and your blood on a regular basis. Other medicines and Ucedane Tell your doctor or pharmacist if you are using, have recently used or might use any other medicines. Ucedane with food and drink Ucedane must be taken orally before meals or feedings. The tablets must be dispersed in a minimum of 5 to 10 mL of water and taken immediately. Pregnancy and Breast-feeding The effects of Ucedane on pregnancy and the unborn child are not known. If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine. The excretion of carglumic acid into breast milk has not been studied in women. Nevertheless, as carglumic acid has been shown to be present in the milk of lactating rats with potential toxic effects for their fed pups, you should not breast-feed your baby if you are taking Ucedane. Driving and using machines Effects on the ability to drive and use machines are not known. Ucedane contains sodium This medicine contains less than 1 mmol sodium (23 mg) per maximum daily dose that is to say essentially 'sodium-free'
3.
How to take it
Ucedane
Always take this medicine exactly as your doctor has told you. Check with your doctor or pharmacist if you are not sure. The usual dose: The initial daily dose is usually 100 mg per kilogram of body weight, up to a maximum of 250 mg per kilogram of body weight (for example, if you weight 10 kg, you should take 1 g per day, or 5 tablets). For patients suffering from N-acetylglutamate synthase deficiency, in the long term, the daily dose usually ranges from 10 mg to 100 mg per kilogram of body weight. Your doctor will determine the dose suitable to you in order to maintain normal ammonia levels in your blood. Ucedane should ONLY be administered by mouth or via a feeding tube into the stomach (using a syringe, if necessary). When the patient is in hyperammonaemic coma, Ucedane is administered by fast push through a syringe via the tube set up and used to feed you. Tell your doctor in case you are suffering from renal impairement. Your daily dose should be reduced. If you take more Ucedane than you should Ask your doctor or pharmacist for advice. If you forget to take Ucedane 2
Do not take a double dose to make up for forgotten doses. If you stop taking Ucedane Do not stop Ucedane without informing your doctor. 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. The following side effects were reported as follows: very common (may affect more than 1 in 10 people), common (may affect up to 1 in 10 people), uncommon (may affect up to 1 in 100 people), rare (may affect up to 1 in 1,000 people), very rare (may affect up to 1 in 10,000 people) and not known (frequency cannot be estimated from the available data). • • •
Common: increased sweating Uncommon: bradycardia (decreased frequency of the heart), diarrhoea, fever, increased transaminases, vomiting Not known: rash
If any of the side effects gets serious, or if you notice any side effects not listed in this leaflet, please tell your doctor or pharmacist. 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. By reporting side effects you can help provide more information on the safety of this medicine.
5.
How to store it
Ucedane
Keep this medicine out of the sight and reach of children. Do not use this medicine after the expiry date which is stated on the blister and carton after EXP. The expiry date refers to the last day of that month. 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 Ucedane contains The active substance is carglumic acid. Each tablet contains 200 mg of carglumic acid. The other ingredients are microcrystalline cellulose, colloidal anhydrous silica, sodium stearyl fumarate (see section 2 "Ucedane contains sodium"), mannitol, copovidone K28, crospovidone type B.
3
What Ucedane looks like and contents of the pack Ucedane dispersible tablets are rod-shaped, white, and biconvex with three score lines on both sides and engraving "L/L/L/L" on one side. Approximate tablet dimensions are 17 mm in length and 6 mm in width. The tablet can be divided into four equal doses. The tablets are presented in aluminium/aluminium blister packed in cartons. Pack size of 12 or 60 dispersible tablets. Not all pack sizes may be marketed. Marketing Authorisation Holder Eurocept International BV Trapgans 5 1244 RL Ankeveen The Netherlands 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 (Northern Ireland) Lucane Pharma Tel: + 33 153 868 750 [email protected]
This leaflet was last revised in September 2024.
4
Frequently asked questions about Ucedane 200 mg dispersible tablets
How do I take Ucedane 200 mg dispersible tablets?
Ucedane 200 mg dispersible tablets comes as tablet containing 200mg. 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 Ucedane 200 mg dispersible tablets?
The active substance in Ucedane 200 mg dispersible tablets is carglumic acid.
Are there equivalent medicines to Ucedane 200 mg dispersible tablets?
Medicines with the same active substance, strength and form include: Carbaglu 200 mg dispersible tablets, Carglumic Acid 200 mg Dispersible Tablets. They are interchangeable only if your prescriber or pharmacist says so.
Where does this information come from?
This leaflet reproduces the patient information leaflet approved for Ucedane 200 mg dispersible tablets, 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 Ucedane 200 mg dispersible tablets 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: carglumic acid
- 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
Ucedane is indicated in treatment of
• hyperammonaemia due to N-acetylglutamate synthase primary deficiency.
• hyperammonaemia due to isovaleric acidaemia.
• hyperammonaemia due to methymalonic acidaemia.
• hyperammonaemia due to propionic acidaemia.
4.2. Posology and method of administration
Ucedane treatment should be initiated under the supervision of a physician experienced in the treatment of metabolic disorders.
Posology
• For N-acetylglutamate synthase deficiency:
Based on clinical experience, the treatment may be started as early as the first day of life. The initial daily dose should be 100 mg/kg, up to 250 mg/kg if necessary.
It should then be adjusted individually in order to maintain normal ammonia plasma levels (see section 4.4).
In the long term, it may not be necessary to increase the dose according to body weight as long as adequate metabolic control is achieved; daily doses range from 10 mg/kg to 100 mg/kg.
Carglumic acid responsiveness test
It is recommended to test individual responsiveness to carglumic acid before initiating any long term treatment. As examples:
- In a comatose child, start with a dose of 100 to 250 mg/kg/day and measure ammonia plasma concentration at least before each administration. It should normalise within a few hours after starting Ucedane.
- In a patient with moderate hyperammonaemia, administer a test dose of 100 to 200 mg/kg/day for 3 days with a constant protein intake and perform repeated determinations of ammonia plasma concentration (before and 1 hour after a meal); adjust the dose in order to maintain normal ammonia plasma levels.
• For isovaleric acidaemia, methylmalonic acidaemia and propionic acidaemia: The treatment should start upon hyperammonaemia in organic acidaemia patients. The initial daily dose should be 100 mg/kg, up to 250 mg/kg if necessary.
It should then be individually adjusted in order to maintain normal ammonia plasma levels (see section 4.4).
Renal impairment:
Caution is advised when administering Ucedane to patients with impaired renal function.
Dosage adjustment is required according to GFR.
• Patients with moderate renal impairment (GFR 30 59 mL/min)
o the recommended initial dose is 50 mg/kg/day to 125 mg/kg/day for patients presenting an hyperammonemia due to NAGS deficiency or organic acidaemia,
o In the long term use the daily dose will be in the range of 5 mg/kg/day to 50 mg/kg/day and should be adjusted individually in order to maintain normal ammonia plasma levels.
• Patients with severe renal impairment (GFR ≤ 29 mL/min)
o the recommended initial dose is 15 mg/kg/day to 40 mg/kg/day for patients presenting an hyperammonaemia due to NAGS deficiency or organic acidaemia,
o In the long term use the daily dose will be in the range of 2 mg/kg/day to 20 mg/kg/day and should be adjusted individually in order to maintain normal ammonia plasma levels.
Method of administration
This medicine is for oral use ONLY (ingestion or via nasogastric tube using a syringe, if necessary).
Based on pharmacokinetic data and clinical experience, it is recommended to divide the total daily dose into two to four doses to be given before meals or feedings. The breaking of the tablets in halves allows most of the required posology adjustments. Occasionally, the use of quarter tablets may also be useful to adjust the posology prescribed by the physician.
The tablets must be dispersed in a minimum of 5-10 mL of water and ingested immediately or administered by fast push through a syringe via a nasogastric tube.
4.3. Contraindications
Hypersensitivity to the active substance or to any of the excipients listed in section 6.1.
Breast-feeding during the use of carglumic acid is contraindicated (see sections 4.6 and 5.3).
4.4. Special warnings and precautions for use
Therapeutic monitoring
Plasma levels of ammonia and amino acids should be maintained within normal limits.
As very few data on the safety of carglumic acid are available, systematic surveillance of liver, renal, cardiac functions and haematological parameters is recommended.
Nutritional management
Protein restriction and arginine supplementation may be indicated in case of low protein tolerance.
Use in patients with renal impairment
The dose of Ucedane must be reduced in patients with renal impairment (see section 4.2).
Ucedane contains sodium
This medicinal product contains less than 1 mmol sodium (23 mg) per maximum daily dose that is to say essentially 'sodium-free'
4.5. Interaction with other medicinal products and other forms of interaction
No specific interaction studies have been performed.
4.6. Fertility, pregnancy and lactation
Pregnancy
For carglumic acid no clinical data on exposed pregnancies are available.
Animal studies have revealed minimal developmental toxicity (see section 5.3).
Caution should be exercised when prescribing to pregnant women.
Breast-feeding
Although it is not known whether carglumic acid is secreted into human milk, it has been shown to be present in the milk of lactating rats (see section 5.3). Therefore, breast-feeding during the use of carglumic acid is contraindicated (see section 4.3).
4.7. Effects on ability to drive and use machines
No studies on the effects on the ability to drive and use machines have been performed.
4.8. Undesirable effects
Reported adverse reactions are listed below, by system organ class and by frequency. Frequencies are 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, undesirable effects are presented in order of decreasing seriousness.
- Undesirable effects in N-acetylglutamate synthase deficiency
Investigations
Uncommon: increased transaminases
Skin and subcutaneous tissue disorders
Common: increased sweating
Not known: rash
- Undesirable effects in organic acidaemia
Cardiac disorders
Uncommon: bradycardia
Gastrointestinal disorders
Uncommon: diarrhoea, vomiting
General disorders and Administration site conditions
Uncommon: pyrexia
Skin and subcutaneous tissue disorders
Not known: rash
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
In one patient treated with carglumic acid, where the dose was increased up to 750 mg/kg/day, symptoms of intoxication occurred which can be characterised as a sympathomimetic reaction: tachycardia, profuse sweating, increased bronchial secretion, increased body temperature and restlessness. These symptoms resolved once the dose was reduced
💬 Ask about this leaflet
Ask anything about Ucedane 200 mg dispersible tablets. 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.