Drug Index


Synonym :

Sapropterin Dihydrochloride

Mechanism :

Tetrahydrobiopterin or BH4 is a cofactor in the synthesis of nitric oxide. It is also essential in the conversion of phenylalanine to tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine to L-dopa by the enzyme tyrosine hydroxylase; and conversion of tryptophan to 5-hydroxytryptophan via tryptophan hydroxylase.

Indication :

  • Tetrahydrobiopterin synthesis defects to control phenylalanine levels in patients with phenylketonuria.

Contraindications :


Dosing :

1-3 mg/kg once daily, oral. Some may require upto 5-20 mg/kg/day.

Adverse Effect :

Headache, diarrhea, abdominal pain, vomiting, and nausea, dizziness, gastrointestinal bleeding, respiratory failure, joint pain, increased urination, agitation.

Interaction :

Drug Interactions Not Available.

Hepatic Dose :

No dosage adjustments are recommended.
02/16/2024 18:00:20 Tetrahydrobiopterin
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