DIETARY RESTRICTION

  • NITISINONE TABLETS must be used in conjunction with a diet restricted in the amino acids tyrosine and phenylalanine.
  • Food to avoid include, fish, eggs, milk, meat, bread, cheese and nuts.
  • Treatment with NITISINONE TABLETS and dietary management should begin as soon as possible after the diagnosis is confirmed.
  • A restricted diet alone is not effective in preventing liver, kidney, and neurologic problems associated with HT-1.

ENSURE PATIENTS WITH HT-1:

1- Maintain a low protein diet

2- Take NITISINONE TABLETS as prescribed

DISCLAIMER:
THIS INFORMATION DOES NOT PROVIDE MEDICAL ADVICE. All content (“Content”), including text, graphics, images and information are aimed a Healthcare Professionals in Canada and it is provided for general informational purposes only.  The Content is not intended to be a substitute for professional medical advice, diagnosis or treatment.

TOP 10 FOODS HIGHEST IN TYROSINE

milligrams of Tyrosine in 100 grams of food

CHEESE 1995mg/100g
SOY FOODS 1497mg/100g
LEAN BEEF & LAMB 1386mg /100g
LEAN PORK 1228mg /100g
FISH & SEAFOOD 1157mg/100g
CHICKEN & TURKEY 1155mg/100g
SEEDS & NUTS 1093mg/100g
EGGS & DAIRY 499mg/100g
BEANS & LENTILS 274mg/100g
WHOLEGRAINS 169mg/100g

NITISINONE TABLETS INDICATIONS

NITISINONE TABLETS, also known as NTBC tablets, are indicated for the treatment of hereditary tyrosinemia type 1 (HT-1) in combination with dietary restriction of tyrosine and phenylalanine.

Treatment with NITISINONE TABLETS should be initiated and supervised by a physician experienced in the treatment of HT-1.

IMPORTANT SAFETY INFORMATION ABOUT NITISINONE TABLETS

Contraindications:

  • Do not prescribe NITISINONE TABLETS to a patient that is hypersensitive to nitisinone or to any ingredient in the formulation.
  • Do not prescribe NITISINONE TABLETS if the patient is breast-feeding. Patients should not breast feed while taking NITISINONE TABLETS.

Warning and Precautions:

Endocrine and Metabolism: Elevated plasma tyrosine levels. Hematologic: Leukopenia and thrombocytopenia. Hepatic/Biliary/Pancreatic: Liver status should be assessed regularly through liver function tests. Neurologic: Variable degrees of intellectual disability and developmental delay have been observed in HT-1 patients treated with nitisinone. In patients treated with nitisinone who exhibit a change in neurologic status, a clinical laboratory assessment including plasma tyrosine should be performed. Ophthalmologic: Ocular signs and symptoms including corneal ulcers, corneal opacities, keratitis, conjunctivitis, eye pain, and photophobia have been reported in patients treated with nitisinone. Patients who develop photophobia, eye pain, or signs of inflammation such as redness, swelling, or burning of the eyes during treatment with NITISINONE TABLETS should undergo slit-lamp re-examination and immediate measurement of plasma tyrosine concentration.

Special Populations:

  • Pregnant women: NITISINONE TABLETS should be used in pregnancy only when the benefits of continued treatment are judged to outweigh the risks.
  • Nursing women: Because of the potential serious adverse reactions to nitisinone in nursing infants, mothers taking NITISINONE TABLETS should not breast-feed.
  • Pediatrics (< 18 years of age): Patients under 18 years should be monitored to ensure adequate control. It is recommended that a dietitian experienced in managing children with inborn errors of metabolism is consulted to design a low-protein diet restricted in tyrosine and phenylalanine.
  • Geriatrics (>65 years of age): Clinical studies of nitisinone did not include subjects over the age of 65 years, and no pharmacokinetic studies have been conducted in geriatric subjects.

Common Adverse Reactions:

The most common adverse reactions (≥1%) reported in patients treated with nitisinone (NTBC) are: Eye Disorders: conjunctivitis, corneal opacity, keratitis, photophobia, blepharitis and eye pain. Blood and lymphatic System Disorders: thrombocytopenia, leukopenia and granulocytopenia. Skin and subcutaneous tissue disorders: pruritis, exfoliative dermatitis and maculopapular rash. Investigations: elevated tyrosine levels.

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