Tetrahydrobiopterin
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 :
Hypersensitivity
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.