سعر كيوفان 100 مجم 30 قرص 2026

Sapropterin dihydrochloride 100 mg (soluble (dispersible) tablets), a synthetic form of tetrahydrobiopterin (BH4) used to lower blood phenylalanine in BH4-responsive phenylketonuria and BH4 deficiency.

• Treatment of hyperphenylalaninaemia (HPA) in adults and children of all ages with phenylketonuria (PKU) shown to be responsive to treatment.Population: Adults and paediatric patients of all agesEuropean Medicines Agency (EMA)
• Treatment of HPA in adults and children of all ages with tetrahydrobiopterin (BH4) deficiency shown to be responsive to treatment.Population: Adults and paediatric patients of all agesEuropean Medicines Agency (EMA)
• To reduce blood phenylalanine in patients one month of age and older with HPA due to BH4-responsive PKU, in conjunction with a Phe-restricted diet (US label does not list BH4 deficiency).Population: Patients 1 month of age and olderDailyMed (U.S. National Library of Medicine)

• Very common: headache, rhinorrhoea. Common: hypophenylalaninaemia, pharyngolaryngeal pain, nasal congestion, cough, diarrhoea, vomiting, abdominal pain, dyspepsia, nausea.International label, same product · European Medicines Agency (EMA)

• Hypersensitivity to the active substance or any excipient.International label, same product · European Medicines Agency (EMA)
• US label: no contraindications listed.International label, same product · DailyMed (U.S. National Library of Medicine)

• Patients must continue a phenylalanine-restricted diet with regular monitoring of blood phenylalanine and tyrosine, nutrient intake and psychomotor development; prolonged low phenylalanine/tyrosine in infancy is associated with impaired neurodevelopment.International label, same product · European Medicines Agency (EMA)
• Rebound increase in blood phenylalanine may occur on stopping treatment.International label, same product · European Medicines Agency (EMA)
• Hypersensitivity reactions including anaphylaxis, upper GI mucosal inflammation, hypophenylalaninaemia and hyperactivity have been reported (US label warnings).International label, same product · DailyMed (U.S. National Library of Medicine)

• Dihydrofolate reductase inhibitors (e.g. methotrexate, trimethoprim)May interfere with BH4 metabolism; caution.European Medicines Agency (EMA)
• Nitric-oxide-affecting vasodilators (GTN, isosorbide dinitrate, sodium nitroprusside, molsidomine), PDE-5 inhibitors, minoxidilBH4 is a cofactor of nitric oxide synthetase; caution with concomitant use.European Medicines Agency (EMA)
• LevodopaConvulsions, exacerbation of convulsions, excitability and irritability observed with co-administration in BH4-deficient patients; caution.European Medicines Agency (EMA)

• Limited data in pregnancy; maternal blood phenylalanine must be strictly controlled before and during pregnancy. Use only if strict dietary management does not adequately reduce phenylalanine.International label, same product · European Medicines Agency (EMA)
• EU: not known whether excreted in human milk; Kuvan should not be used during breastfeeding.International label, same product · European Medicines Agency (EMA)
• US: insufficient data on presence in human milk; 13 infants exposed through breastfeeding in post-marketing registries without reported lactation-related safety concerns; weigh benefits of breastfeeding against maternal need.International label, same product · DailyMed (U.S. National Library of Medicine)

• Sapropterin dihydrochloride: Synthetic BH4, the natural cofactor of phenylalanine hydroxylase; enhances residual enzyme activity and phenylalanine oxidation, lowering blood phenylalanine in responsive patients (EU SmPC 5.1).