Ország: Európai Unió
Nyelv: angol
Forrás: EMA (European Medicines Agency)
Sodium phenylbutyrate
Immedica Pharma AB
A16AX03
sodium phenylbutyrate
Other alimentary tract and metabolism products,
Ornithine Carbamoyltransferase Deficiency Disease; Citrullinemia; Carbamoyl-Phosphate Synthase I Deficiency Disease
Ammonaps is indicated as adjunctive therapy in the chronic management of urea cycle disorders, involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase orargininosuccinate synthetase.It is indicated in all patients with neonatal-onset presentation (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.
Revision: 21
Authorised
1999-12-07
24 B. PACKAGE LEAFLET 25 PACKAGE LEAFLET: INFORMATION FOR THE USER AMMONAPS 500 MG TABLETS Sodium phenylbutyrate READ ALL OF THIS LEAFLET CAREFULLY BEFORE YOU START TAKING THIS MEDICINE BECAUSE IT CONTAINS IMPORTANT INFORMATION FOR YOU. - Keep this leaflet. You may need to read it again. - If you have any further questions, ask your doctor or pharmacist. - This medicine has been prescribed for you only. Do not pass it on to others. It may harm them, even if their signs of illness are the same as yours. - If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not listed in this leaflet. See section 4. WHAT IS IN THIS LEAFLET 1. What AMMONAPS is and what it is used for 2. What you need to know before you take AMMONAPS 3. How to take AMMONAPS 4. Possible side effects 5. How to store AMMONAPS 6. Contents of the pack and other information 1. WHAT AMMONAPS IS AND WHAT IT IS USED FOR AMMONAPS is prescribed to patients with urea cycle disorders. Patients with these rare disorders have a deficiency of certain liver enzymes and are therefore unable to eliminate nitrogen waste. Nitrogen is a building block of proteins, because of this, there is a build up of nitrogen in the body after eating protein. Nitrogen waste, in the form of ammonia, is especially toxic for the brain and leads, in severe cases, to reduced levels of consciousness and to coma. AMMONAPS helps the body to eliminate nitrogen waste, reducing the amount of ammonia in your body. 2. WHAT YOU NEED TO KNOW BEFORE YOU TAKE AMMONAPS DO NOT TAKE AMMONAPS - if you are pregnant. - if you are breast-feeding. - if you are allergic to sodium phenylbutyrate or any of the other ingredients of this medicine (listed in section 6). WARNINGS AND PRECAUTIONS Talk to your doctor before taking AMMONAPS - if you have difficulty swallowing. AMMONAPS tablets can get stuck in the oesophagus and cause ulcers. If you have difficulty swallowing it is recommended to use AMMONAPS granules instead. - if you suffer from heart failure, a decr Olvassa el a teljes dokumentumot
1 ANNEX I SUMMARY OF PRODUCT CHARACTERISTICS 2 1. NAME OF THE MEDICINAL PRODUCT AMMONAPS 500 mg tablets. 2. QUALITATIVE AND QUANTITATIVE COMPOSITION Each tablet contains 500 mg sodium phenylbutyrate. Excipient(s) with known effect Each tablet contains 2.7 mmol (62 mg) of sodium. For the full list of excipients, see section 6.1. 3. PHARMACEUTICAL FORM Tablet. The tablets are off-white, oval and embossed with “UCY 500”. 4. CLINICAL PARTICULARS 4.1 THERAPEUTIC INDICATIONS AMMONAPS 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_ presentation (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 AMMONAPS treatment should be supervised by a physician experienced in the treatment of urea cycle disorders. The use of AMMONAPS tablets is indicated for adults and children who are able to swallow tablets. AMMONAPS is also available as granules for infants, children who are unable to swallow tablets and for patients with dysphagia. 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 children weighing less than 20 kg • 9.9 - 13.0 g/m 2 /day in children weighing more than 20 kg, adolescents and adults. The safety and efficacy of doses in excess of 20 g/day (40 tablets) have not been established. _Therapeutic monitoring:_ Plasma levels of ammonia, arginine, essential amino acids (especially branched chain amino acids), carnitine and serum proteins sho Olvassa el a teljes dokumentumot