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KUVAN
Inborn errors of metabolism
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Drug Name:

KUVAN Rx

Generic Name and Formulations:
Sapropterin dihydrochloride 100mg; tabs.

Company:
BioMarin Pharmaceuticals

Therapeutic Use:

Indications for KUVAN:

In conjunction with a phenylalanine (Phe)-restricted diet: To reduce blood Phe levels in hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive phenylketonuria (PKU).

Adults and Children:

<1 month: consult manufacturer. Take with food at same time each day. Dissolve tab in 4–8oz water or apple juice (may crush tab) and drink within 15 mins (tab may not completely dissolve). 1 month–6yrs: Initially 10mg/kg once daily; if inadequate response may increase to 20mg/kg once daily; if inadequate response after 1 month, discontinue. ≥7yrs: 10–20mg/kg once daily; if inadequate response after 1 month, discontinue. Usual range 5–20mg/kg per day; adjust based on blood Phe levels.

Warnings/Precautions:

Monitor blood levels of Phe after 1 week to assess effect, then periodically. Discontinue if anaphylaxis occurs. Maintain Phe-restricted diet. Monitor BP and change in neurologic status. Hepatic or renal impairment; monitor. Pregnancy (Cat.C). Nursing mothers.

Interactions:

Caution with drugs that can inhibit folate metabolism (eg, methotrexate), PDE-5 inhibitors (eg, sildenafil, tadalafil, vardenafil), other nitric oxide donors, levodopa; monitor. May potentiate drugs that are P-gp or BCRP substrates.

See Also:

KUVAN POWDER

Pharmacological Class:

Enzyme cofactor (BH4).

Adverse Reactions:

Headache, rhinorrhea, pharyngolaryngeal pain, diarrhea, vomiting, cough, nasal congestion, gastritis, hypophenylalaninemia, hyperactivity (monitor).

Generic Availability:

NO

How Supplied:

Tabs—120; Packet—1, 30

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