Nchi: Umoja wa Ulaya
Lugha: Kiingereza
Chanzo: EMA (European Medicines Agency)
nitisinone
Swedish Orphan Biovitrum International AB
A16AX04
nitisinone
Other alimentary tract and metabolism products,
Tyrosinemias
Hereditary tyrosinemia type 1 (HT 1)Orfadin is indicated for the treatment of adult and paediatric (in any age range) patients with confirmed diagnosis of hereditary tyrosinemia type 1 (HT 1) in combination with dietary restriction of tyrosine and phenylalanine.Alkaptonuria (AKU)Orfadin is indicated for the treatment of adult patients with alkaptonuria (AKU).
Revision: 21
Authorised
2005-02-21
34 B. PACKAGE LEAFLET 35 PACKAGE LEAFLET: INFORMATION FOR THE USER ORFADIN 2 MG HARD CAPSULES ORFADIN 5 MG HARD CAPSULES ORFADIN 10 MG HARD CAPSULES ORFADIN 20 MG HARD CAPSULES nitisinone 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, pharmacist or nurse. - This medicine has been prescribed for you. 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, pharmacist or nurse. This includes any possible side effects not listed in this leaflet. See section 4. WHAT IS IN THIS LEAFLET 1. What Orfadin is and what it is used for 2. What you need to know before you take Orfadin 3. How to take Orfadin 4. Possible side effects 5. How to store Orfadin 6. Contents of the pack and other information 1. WHAT ORFADIN IS AND WHAT IT IS USED FOR Orfadin contains the active substance nitisinone. Orfadin is used to treat: - a rare disease called hereditary tyrosinemia type 1 in adults, adolescents and children (in any age range) - a rare disease called alkaptonuria (AKU) in adults. In these diseases your body is unable to completely break down the amino acid tyrosine (amino acids are building blocks of our proteins), forming harmful substances. These substances are accumulated in your body. Orfadin blocks the breakdown of tyrosine and the harmful substances are not formed. For the treatment of hereditary tyrosinemia type 1, you must follow a special diet while you are taking this medicine, because tyrosine will remain in your body. This special diet is based on low tyrosine and phenylalanine (another amino acid) content. For the treatment of AKU, your doctor may advise you to follow a special diet. 2. WHAT YOU NEED TO KNOW BEFORE YOU TAKE ORFADIN DO NOT TAKE ORFADIN - if you are allergic to nitisinone or any of the other ingredients of this medicine (listed Soma hati kamili
1 ANNEX I SUMMARY OF PRODUCT CHARACTERISTICS 2 1. NAME OF THE MEDICINAL PRODUCT Orfadin 2 mg hard capsules Orfadin 5 mg hard capsules Orfadin 10 mg hard capsules Orfadin 20 mg hard capsules 2. QUALITATIVE AND QUANTITATIVE COMPOSITION Each capsule contains 2 mg nitisinone. Each capsule contains 5 mg nitisinone. Each capsule contains 10 mg nitisinone. Each capsule contains 20 mg nitisinone. For the full list of excipients, see section 6.1. 3. PHARMACEUTICAL FORM Hard capsule. White, opaque capsules (6x16 mm) imprinted “NTBC 2mg” in black on the body of the capsule. White, opaque capsules (6x16 mm) imprinted “NTBC 5mg” in black on the body of the capsule. White, opaque capsules (6x16 mm) imprinted “NTBC 10mg” in black on the body of the capsule. White, opaque capsules (6x16 mm) imprinted “NTBC 20mg” in black on the body of the capsule. The capsules contain a white to off white powder. 4. CLINICAL PARTICULARS 4.1 THERAPEUTIC INDICATIONS Hereditary tyrosinemia type 1 (HT-1) Orfadin is indicated for the treatment of adult and paediatric (in any age range) patients with confirmed diagnosis of hereditary tyrosinemia type 1 (HT-1) in combination with dietary restriction of tyrosine and phenylalanine. Alkaptonuria (AKU) Orfadin is indicated for the treatment of adult patients with alkaptonuria (AKU). 4.2 POSOLOGY AND METHOD OF ADMINISTRATION Posology HT-1: Nitisinone treatment should be initiated and supervised by a physician experienced in the treatment of HT-1 patients. Treatment of all genotypes of the disease should be initiated as early as possible to increase overall survival and avoid complications such as liver failure, liver cancer and renal disease. Adjunct to the nitisinone treatment, a diet deficient in phenylalanine and tyrosine is required and should be followed by monitoring of plasma amino acids (see sections 4.4 and 4.8). _ _ _Starting dose HT-1 _ The recommended initial daily dose in the paediatric and adult population is 1 mg/kg body weight administered orally. The dose of nitisinone shou Soma hati kamili