KUVAN

उत्पाद अवलोकन

About KUVAN

KUVAN is a prescription medication containing the active ingredient Sapropterin dihydrochloride, used to treat phenylketonuria (PKU) in patients with the E298K, A298D, or A298V mutations in the PAH gene. It is a synthetic form of tetrahydrobiopterin (BH4), a cofactor necessary for the normal functioning of the enzyme phenylalanine hydroxylase.

Key Benefits & Uses

• Treatment of phenylketonuria (PKU) in patients with the E298K, A298D, or A298V mutations in the PAH gene.
• Reduction of phenylalanine levels in the blood.
• Supports the normal functioning of the enzyme phenylalanine hydroxylase.

Active Ingredient & Action

Sapropterin dihydrochloride works by replenishing the body's stores of tetrahydrobiopterin (BH4), a cofactor necessary for the normal functioning of the enzyme phenylalanine hydroxylase. This enzyme is responsible for converting the amino acid phenylalanine into tyrosine. In patients with PKU, the enzyme is deficient or non-functional, leading to elevated levels of phenylalanine in the blood. By replenishing BH4, KUVAN helps to restore normal enzyme function, reducing phenylalanine levels and alleviating symptoms associated with PKU.

Important Notes

KUVAN is a prescription medication and should only be used under the guidance of a qualified healthcare professional. It is essential to follow the recommended dosage and administration instructions carefully. Patients with PKU should also adhere to a strict diet that limits phenylalanine intake. Regular monitoring of blood phenylalanine levels is necessary to ensure the effectiveness of treatment and to prevent adverse effects.