CYCLE NITISINONE TABLETS F.A.Q.

NITISINONE TABLETS, also known as NTBC tablets, are used for the treatment of hereditary tyrosinemia type 1 (HT-1), in combination with dietary restriction of tyrosine and phenylalanine.
NITISINONE TABLETS stop the build-up of toxic substances which cause the severe liver and kidney problems in patients with HT-1. By doing that, they also prevent the porphyric crises associated with HT-1.
Medicinal ingredients: Nitisinone (NTBC). Non-medicinal ingredients: Glyceryl dibehenate, Lactose monohydrate. Do not prescribe NITISINONE TABLETS if the patient is allergic to nitisinone (NTBC) or other ingredients of this medicine.
NITISINONE TABLETS may be taken with or without food.

For infants, as the tablets are uncoated, these can be disintegrated in water using an oral syringe with a cap. Do not administer the suspension using a baby bottle.

For patients that can swallow semi-solid food, the tablets may be crushed and mixed with applesauce for administration.

The full administration instructions are available from the Patient Medication Information document.

The recommended initial dose of NITISINONE TABLETS in the pediatric and adult population is 1 mg/Kg body weight/day divided in two doses administered orally. The dose of nitisinone should be adjusted individually.

In patients whose plasma and urine succinylacetone (SA) are still detectable one month after starting treatment with NITISINONE TABLETS, the dose should be increased to 1.5 mg/kg/day. A maximun dosage of 2 mg/kg/day may be needed, based on the evaluation of all biochemical parameters. If the biochemical response is satisfactory, the dosage of NITISINONE TABLETS should be adjusted only according to body weight gain.

For management of a suspected drug overdose, contact your regional Poison Control Centre.

Accidental ingestion of nitisinone by individuals eating normal diets not restricted in tyrosine and phenylalanine will result in elevated tyrosine levels. Elevated tyrosine levels have been associated with toxicity to the eyes, skin, and nervous system. Restriction of tyrosine and phenylalanine int he diet should limit toxicity associated with this type of tyrosinemia. No information about specific treatment of overdose is available.

If patient taking NITISINONE TABLETS forgets a dose, they should take the next dose as planned. The patient should not take a double dose to make up for a forgotten dose.
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.

Keep NITISINONE TABLETS out of reach and sight of children. Store the tablets at room temperature (15 – 30°C), in a dry place. Keep the container tightly closed. There is an expiry date on the label. NITISINONE TABLETS should not be used after this date.

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.
  • Paediatrics (: 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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