Monograph Details
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Nutrition
> Inborn errors of metabolism
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KUVAN |
| Manufacturer |
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BioMarin Pharmaceuticals |
| Legal Classification |
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Rx
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| Pharmacological Class |
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Enzyme cofactor (BH4). |
| Generic Name |
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Sapropterin dihydrochloride 100mg; tabs. |
| Indications |
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In conjunction with a phenylalanine (Phe)-restricted diet: To reduce blood Phe levels in hyperphenylalaninemia (HPA) in responsive phenylketonuria (PKU). |
| Adults and Children |
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<4 years: consult manufacturer. ≥4 years: Take with food. Dissolve tablet in 4–8oz water or apple juice (may crush tab) and drink within 15 minutes (tablet may not completely dissolve). Initially 10mg/kg once daily; if inadequate response may increase to 20mg/kg once daily; if inadequate response after 1 month, discontinue. Usual range 5–20mg/kg per day; adjust based on blood Phe levels. |
| Precautions |
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Monitor blood levels of Phe after 1 week to assess effect, then periodically. Maintain Phe-restricted diet. Hepatic or renal impairment. Pregnancy (Cat.C). Nursing mothers: not recommended. |
| Interactions |
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Caution with drugs that can inhibit folate metabolism (eg, methotrexate), PDE-5 inhibitors (eg, sildenafil, tadalafil, vardenafil), other nitric oxide donors, levodopa. |
| Adverse Reactions |
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Headache, GI upset, upper respiratory infection/symptoms, throat pain, cough, fever, contusion, abdominal pain, rash; others (see literature). |
| How Supplied |
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Tabs—120 |
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