Nitisinone (By mouth)


Nitisinone (nye-TIS-i-none)

Treats a hereditary condition called tyrosinemia, which causes your body to produce too much of an amino acid called tyrosine. This condition can cause unwanted effects on the liver and kidneys. This medicine will not cure tyrosinemia, but can be used together with diet to control the amount of tyrosine in your body.

Brand Name(s)


When This Medicine Should Not Be Used

How to Use This Medicine


  • Your doctor will tell you how much of this medicine to take and how often. Do not take more medicine or take it more often than your doctor tells you to.
  • Carefully follow your doctor's instructions about any special diet. You may need to restrict or avoid dairy products, meats, fish, eggs, almonds, avocados, bananas, soy, pumpkin seeds, sesame seeds, and many other foods that contain tyrosine.
  • It is best to take this medicine on an empty stomach, at least one hour before a meal.
  • If you cannot swallow the capsule, you may open it and pour the medicine into a small amount of water or applesauce. Stir this mixture well and swallow it without chewing.
  • If you are giving this medicine to an infant, you may open the capsule and mix the medicine with a small amount of liquid baby formula.
  • Never share your medicine with anyone.

If a dose is missed:

  • If you miss a dose or forget to take your medicine, take it as soon as you can. If it is almost time for your next dose, wait until then to take the medicine and skip the missed dose.
  • Do not use extra medicine to make up for a missed dose.

How to Store and Dispose of This Medicine

  • Store the medicine in the refrigerator. Do not freeze.
  • Keep all medicine out of the reach of children.

Drugs and Foods to Avoid

Ask your doctor or pharmacist before using any other medicine, including over-the-counter medicines, vitamins, and herbal products.

Warnings While Using This Medicine

  • Make sure your doctor knows if you are pregnant or breastfeeding.
  • Your doctor will need to check your blood or urine at regular visits while you are using this medicine. You may also need to have regular eye examinations and ultrasounds of your liver. Be sure to keep all appointments.

Possible Side Effects While Using This Medicine

Call your doctor right away if you notice any of these side effects:

  • Allergic reaction: Itching or hives, swelling in face or hands, swelling or tingling in the mouth or throat, tightness in chest, trouble breathing
  • Black, tarry stools
  • Dark-colored urine or pale stools
  • Fast heartbeat
  • Extreme thirst or hot, dry skin
  • Itchy, flaky, or peeling skin rash
  • Muscle weakness, mental changes, confusion, disorientation
  • Nausea, vomiting, loss of appetite, pain in the upper stomach
  • Problems with vision, or eyes feeling more sensitive to light
  • Raised or thickened skin areas on the palms of your hands or soles of your feet
  • Red, sore, painful, puffy, or irritated eyes
  • Seizures
  • Sudden stomach pain, constipation, vomiting
  • Unusual bleeding, bruising, or weakness
  • Yellow eyes or skin

If you notice these less serious side effects, talk with your doctor:

  • Changes in your menstrual periods
  • Feeling nervous or tired
  • Mild diarrhea, stomach pain

If you notice other side effects that you think are caused by this medicine, tell your doctor

Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088

Version Info

  • Last reviewed on 12/4/2015

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