 sapropterin dihydrochloride |
| Kuvan |
Pharmaceutical
company:
BioMarin Pharmaceuticals, Inc.
www.biomarinpharm.com |
Pharmacologic classification: enzyme cofactor
Therapeutic classification: phenylalanine reducer
Pregnancy risk category: C
AVAILABLE FORMS
Tablets: 100 mg
INDICATIONS AND DOSAGES
In conjunction with diet, to reduce blood phenylalanine (Phe) levels in patients with hyperphenylalaninemia caused by tetrahydrobiopterin-responsive phenylketonuria--
Adults and children age 4 years and older: Starting dose 10 mg/kg P.O. taken once daily; may be adjusted in the range of 5 to 20 mg/kg/day, based on patient’s serum Phe levels.
CONTRAINDICATIONS AND CAUTIONS
There are no known contraindications.
Use cautiously in patients with liver impairment; with use of medications known to inhibit folate metabolism, such as methotrexate; with drugs known to affect nitric-oxide–mediated vasorelaxation, such as sildenafil, vardenafil, tadalafil; with levodopa; and in pregnant or breast-feeding women.
INTERACTIONS
Drug-drug.
Drugs that inhibit folate metabolism such as methotrexate: May decrease effectiveness of sapropterin. Use together cautiously.
Levodopa: May increase risk of CNS overstimulation and seizures if used with levodopa. Monitor patient closely and use cautiously together.
PDE-5 inhibitors (sildenafil, vardenafil, tadalafil): May severely reduce blood pressure. Monitor patient closely and use cautiously together.
ADVERSE REACTIONS
CNS: headache, asthenia, pyrexia.
EENT: pharyngolaryngeal pain, nasal congestion.
GI: diarrhea, vomiting, abdominal pain.
Respiratory: rhinorrhea, URI, cough.
Other: rash, contusion.
Reactions may be common, uncommon, life-threatening,
or COMMON AND LIFE-THREATENING.
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