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Nitisinone Dosage

Medically reviewed on April 4, 2017.

Applies to the following strengths: 2 mg; 5 mg; 10 mg; 20 mg; 4 mg/mL

Usual Adult Dose for Hereditary Tyrosinemia Type 1

Initial dose: 1 mg/kg/day divided into a morning and evening dose
Maintenance dose: 1 to 2 mg/kg/day divided into two daily doses
Maximum dose: 2 mg/kg/day

Comments: Requires dietary restriction of tyrosine and phenylalanine

Usual Pediatric Dose for Hereditary Tyrosinemia Type 1

Initial dose: 1 mg/kg/day divided into a morning and evening dose
Maintenance dose: 1 to 2 mg/kg/day divided into two daily doses
Maximum dose: 2 mg/kg/day

Comments: Requires dietary restriction of tyrosine and phenylalanine

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

In patients whose erythrocyte PBG-synthase activity and urine 5-ALA and urine succinylacetone are not normalized within one month after starting treatment, the dose may be increased to 1.5 mg/kg/day.

In patients receiving 1.5 mg/kg/day, whose erythrocyte PBG-synthase activity and urine 5-ALA and urine succinylacetone remain elevated and whose plasma succinylacetone is not normalized after three months, the dose may be increased up to a maximum dose of 2 mg/kg/day.

Precautions

Consult WARNINGS section for dosing related precautions

Dialysis

Data not available

Other Comments

Administration advice:
-Take one hour before or two hours after a meal
-Capsules may be opened and the contents suspended in a small amount of water immediately before use

Storage requirements: Refrigerate

General: Treatment should be initiated by a physician experienced in the treatment of Hereditary Tyrosinemia Type 1 (HT-1)

Monitoring: Plasma nitisinone concentration, plasma succinylacetone, urine 5-ALA and erythrocyte PBG-synthase activity, if not available, clinical laboratory assessments should include urine succinylacetone, liver function tests, alpha-fetoprotein, and serum tyrosine and phenylalanine level.

Patient advice:
-Maintain dietary restriction of tyrosine and phenylalanine during treatment
-Promptly report unexplained eye symptoms, rash, jaundice or excessive bleeding

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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