Drug Index

Nitisinone

 
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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 :

Initial: 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.


12/09/2019 11:09:11 Nitisinone
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