Drug Index


Mechanism :

Nitisinone competitively inhibits 4-hydroxyphenyl-pyruvate dioxygenase, an enzyme present early in the tyrosine degradation pathway, thereby preventing the build-up of the toxic metabolites.

Indication :

  • Hereditary tyrosinemia type 1

Contraindications :

Hypersensitivity to nitisinone or any component of the formulation; breastfeeding.

Dosing :

0.5 mg/kg twice daily. Increase to 0.75 mg/kg twice daily if succinylacetone is detectable 4 weeks after initiation.

Adverse Effect :

Increased plasma tyrosine, alopecia, exfoliative dermatitis, porphyria, leukopenia, thrombocytopenia, granulocytopenia, hepatic failure, hepatic neoplasm, conjunctivitis, corneal opacity, keratitis. Photophobia, epistaxis.

Interaction :

No known significant interactions.
Tyrosine toxicity can occur without proper dietary restriction of tyrosine and phenylalanine.

Hepatic Dose :

No dose adjustments are recommended.
11/03/2023 12:36:09 Nitisinone
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