Nitisinone 5mg Capsules

Product Introduction

Nitisinone 5mg treats hereditary tyrosinaemia type 1, an inherited disorder that produces toxic breakdown products capable of damaging the liver and kidneys. Treatment is combined with a diet restricting tyrosine and phenylalanine.

How to use

Take the capsules on an empty stomach, at least one hour before or two hours after a meal, at consistent daily times. The dose is based on body weight and adjusted using succinylacetone results. Continue the tyrosine- and phenylalanine-restricted diet alongside treatment; the capsules do not replace dietary management.

How it works

Nitisinone inhibits 4-hydroxyphenylpyruvate dioxygenase early in the tyrosine-breakdown pathway. This prevents formation of toxic downstream substances such as succinylacetone, but can increase blood tyrosine levels.

Side effects

Raised tyrosine, reduced platelets or white cells, rash and nosebleeds can occur. Eye pain, redness, light sensitivity or blurred vision may be associated with high tyrosine and need prompt assessment.

Important information

Monitoring includes tyrosine, phenylalanine, succinylacetone, blood counts and liver assessment. Liver surveillance continues even when metabolic control is good. Warfarin and other sensitive CYP2C9 substrates can require closer monitoring because nitisinone can increase their exposure.