Country: Unjoni Ewropea
Lingwa: Ingliż
Sors: EMA (European Medicines Agency)
Sapropterin dihydrochloride
BioMarin International Limited
A16AX07
sapropterin
Other alimentary tract and metabolism products,
Phenylketonurias
Kuvan is indicated for the treatment of hyperphenylalaninaemia (HPA) in adults and paediatric patients of all ages with phenylketonuria (PKU) who have been shown to be responsive to such treatment.Kuvan is also indicated for the treatment of hyperphenylalaninaemia (HPA) in adults and paediatric patients of all ages with tetrahydrobiopterin (BH4) deficiency who have been shown to be responsive to such treatment.
Revision: 20
Authorised
2008-12-02
38 B. PACKAGE LEAFLET 39 PACKAGE LEAFLET: INFORMATION FOR THE PATIENT KUVAN 100 MG SOLUBLE TABLETS Sapropterin dihydrochloride 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 Kuvan _ _ is and what it is used for 2. What do you need to know before you take Kuvan 3. How to take Kuvan _ _ 4. Possible side effects 5. How to store Kuvan _ _ 6. Contents of the pack and other information 1. WHAT KUVAN IS AND WHAT IT IS USED FOR Kuvan contains the active substance sapropterin which is a synthetic copy of a body’s own substance called tetrahydrobiopterin (BH4). BH4 is required by the body to use an amino acid called phenylalanine in order to build another amino acid called tyrosine. Kuvan is used to treat hyperphenylalaninaemia (HPA) or phenylketonuria (PKU) in patients of all ages. HPA and PKU are due to abnormally high levels of phenylalanine in the blood which can be harmful. Kuvan reduces these levels in some patients who respond to BH4 and can help increase the amount of phenylalanine that can be included in the diet. This medicine is also used to treat an inherited disease called BH4 deficiency in patients of all ages, in which the body cannot produce enough BH4. Because of very low BH4 levels phenylalanine is not used properly and its levels rise, resulting in harmful effects. By replacing the BH4 that the body cannot produce, Kuvan reduces the harmful excess of phenylalanine in the blood and increases the dietary tolerance to phenylalanine. 2. WHAT YOU NEED TO KNOW BEFORE YOU TAKE KUVAN DO NOT T Aqra d-dokument sħiħ
1 ANNEX I SUMMARY OF PRODUCT CHARACTERISTICS 2 1. NAME OF THE MEDICINAL PRODUCT Kuvan 100 mg soluble tablets _ _ 2. QUALITATIVE AND QUANTITATIVE COMPOSITION Each soluble tablet contains 100 mg of sapropterin dihydrochloride (equivalent to 77 mg of sapropterin). For the full list of excipients, see section 6.1. 3. PHARMACEUTICAL FORM Soluble tablet Off-white to light yellow soluble tablet with “177” imprinted on one face. 4. CLINICAL PARTICULARS 4.1 THERAPEUTIC INDICATIONS Kuvan is indicated for the treatment of hyperphenylalaninaemia _ _ (HPA) in adults and paediatric patients of all ages with phenylketonuria (PKU) who have been shown to be responsive to such treatment (see section 4.2). Kuvan is also indicated for the treatment of hyperphenylalaninaemia (HPA) in adults and paediatric patients of all ages with tetrahydrobiopterin (BH4) deficiency who have been shown to be responsive to such treatment (see section 4.2). 4.2 POSOLOGY AND METHOD OF ADMINISTRATION Treatment with Kuvan must be initiated and supervised by a physician experienced in the treatment of PKU and BH4 deficiency. Active management of dietary phenylalanine and overall protein intake while taking this medicinal product is required to ensure adequate control of blood phenylalanine levels and nutritional balance. _ _ As HPA due to either PKU or BH4 deficiency is a chronic condition, once responsiveness is demonstrated, Kuvan is intended for long-term use (see section 5.1). Posology _PKU _ The starting dose of Kuvan in adult and paediatric patients with PKU is 10 mg/kg body weight once daily. The dose is adjusted, usually between 5 and 20 mg/kg/day, to achieve and maintain adequate blood phenylalanine levels as defined by the physician. _BH4 deficiency _ The starting dose of Kuvan in adult and paediatric patients with BH4 deficiency is 2 to 5 mg/kg body weight total daily dose. Doses may be adjusted up to a total of 20 mg/kg per day. _ _ _ _ Kuvan is provided as 100 mg tablets. The calculated daily dose based on body weight should be rounded to Aqra d-dokument sħiħ