Nityr

— THERAPEUTIC CATEGORIES —
  • Inborn errors of metabolism
PAGE CONTENTS
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  • Generic Name & Formulations
  • Indications
  • Dosage and Administration
  • Contraindications
  • Boxed Warnings
  • Warnings/Precautions
  • Pharmacokinetics
  • Interactions
  • Adverse Reactions
  • Clinical Trials
  • Note
  • Patient Counseling

    • Nityr Generic Name & Formulations

    Legal Class

    Rx

    General Description

    Nitisinone 2mg, 5mg, 10mg; tabs.

    Pharmacological Class

    4-hydroxyphenylpyruvate dioxygenase inhibitor.

    How Supplied

    Tabs—60

    Storage

    Store at room temperature between 20°C to 25°C (68°F to 77°F); excursions permitted between 15°C and 30°C (59°F and 86°F).

    Manufacturer

    Cycle Pharma

    Generic Availability

    NO

    Mechanism of Action

    Nitisinone competitively inhibits 4-hydroxyphenyl-pyruvate dioxygenase, therefore preventing the accumulation of maleylacetoacetate and fumarylacetoacetate, both catabolic intermediates that get converted to toxic metabolites succinylacetone and succinylacetoacetate in patients with hereditary tyrosinemia type 1.

    Nityr Indications

    Indications

    Treatment of hereditary tyrosinemia type 1 (HT-1) in combination with dietary restriction of tyrosine and phenylalanine.

    Nityr Dosage and Administration

    Adults and Children

    May crush tabs and mix with applesauce or disintegrate in water if difficulty swallowing tab; see full labeling. Initially 0.5mg/kg twice daily. Maintenance (in patients ≥5yrs with undetectable serum and urine succinylacetone levels after a minimum of 4 weeks on stable dose): may give 1–2mg/kg once daily. Titrate individually based on response. Monitor plasma and/or urine succinylacetone levels, liver function, and alpha-fetoprotein levels. Increase to 0.75mg/kg twice daily if succinylacetone is still detectable 4 weeks after initiation. Max: 2mg/kg/day.

    Nityr Contraindications

    Not Applicable

    Nityr Boxed Warnings

    Not Applicable

    Nityr Warnings/Precautions

    Warnings/Precautions

    Maintain dietary restriction of tyrosine and phenylalanine during therapy. Do not adjust dose to lower plasma tyrosine levels (maintain levels at <500μmol/L). Perform baseline eye exam (including slit-lamp test) prior to starting therapy and regularly thereafter; re-evaluate and assess plasma tyrosine levels if ocular symptoms develop or tyrosine >500μmol/L during therapy. Perform lab tests (including plasma tyrosine levels) if abrupt change in neurologic status occurs. Hyperkeratotic plaques (on the soles & palms). Monitor platelets, WBC counts during therapy. Elderly. Pregnancy. Nursing mothers.

    Nityr Pharmacokinetics

    Distribution

    Plasma protein bound: >95% at 50 micromolar concentration. Apparent volume of distribution (SD): 8.2 (1.6) L.

    Metabolism

    Hepatic.

    Elimination

    Renal. Half-life (SD): 59.3 (8.9) hours. Renal clearance: 0.003 L/h.

    Nityr Interactions

    Interactions

    May potentiate CYP2C9 substrates (eg, celecoxib, tolbutamide, phenytoin, warfarin); reduce dose of substrates by ½; drugs with narrow therapeutic index may need additional dose adjustments. May potentiate OAT1/OAT3 substrates (adefovir, ganciclovir, methotrexate); monitor.

    Nityr Adverse Reactions

    Adverse Reactions

    Elevated tyrosine levels, thrombocytopenia, leukopenia, conjunctivitis, corneal opacity, keratitis, photophobia, eye pain, blepharitis, cataracts, granulocytopenia, epistaxis, pruritus, exfoliative dermatitis, dry skin, maculopapular rash, alopecia.

    Nityr Clinical Trials

    See Literature

    Nityr Note

    Not Applicable

    Nityr Patient Counseling

    See Literature

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