Страна: Европейски съюз
Език: английски
Източник: EMA (European Medicines Agency)
Sodium phenylbutyrate
Eurocept International B. V.
A16AX03
sodium phenylbutyrate
Various alimentary tract and metabolism products
Carbamoyl-Phosphate Synthase I Deficiency Disease
Treatment of chronic management of urea-cycle disorders.
Revision: 10
Authorised
2013-07-30
32 B. PACKAGE LEAFLET 33 PACKAGE LEAFLET: INFORMATION FOR THE PATIENT PHEBURANE 483 MG/G GRANULES 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 PHEBURANE is and what it is used for 2. What you need to know before you take PHEBURANE 3. How to take PHEBURANE 4. Possible side effects 5. How to store PHEBURANE 6. Contents of the pack and other information 1. WHAT PHEBURANE IS AND WHAT IT IS USED 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. This medicine 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 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). Прочетете целия документ
1 ANNEX I SUMMARY OF PRODUCT CHARACTERISTICS 2 1. NAME OF THE MEDICINAL PRODUCT PHEBURANE 483 mg/g granules 2. QUALITATIVE AND QUANTITATIVE COMPOSITION Each gram of granules contains 483 mg of sodium phenylbutyrate. Excipient(s) with known effect Each gram of sodium phenylbutyrate contains 124 mg (5.4 mmol) of sodium and 768 mg of sucrose. For the full list of excipients, see section 6.1. 3. PHARMACEUTICAL FORM Granules. White to off-white granules. 4. CLINICAL PARTICULARS 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/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 of sodium phenylbutyrate have not been established. _Therapeutic monitoring _ 3 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 Прочетете целия документ