Generic Name and Formulations:
Nitisinone 2mg, 5mg, 10mg; tabs.
Indications for NITYR:
Treatment of hereditary tyrosinemia type 1 (HT-1) in combination with dietary restriction of tyrosine and phenylalanine.
Adults and Children:
May crush tabs and mix with applesauce or disintegrate in water if difficulty swallowing tabs. Initially 0.5mg/kg twice daily. Titrate individually as needed 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 1 month after treatment initiation; max 1mg/kg twice daily dose may be needed.
Maintain dietary restriction of tyrosine and phenylalanine during therapy. Do not adjust dose to lower plasma tyrosine levels (maintain levels at <500μmol/L). Perform eye exams (including slit-lamp test) prior to starting therapy; re-evaluate and assess plasma tyrosine levels if ocular symptoms develop. Perform lab tests (including plasma tyrosine levels) if abrupt change in neurologic status occurs. Monitor platelets, WBC counts during therapy. Pregnancy. Nursing mothers.
May potentiate CYP2C9 substrates; monitor.
4-hydroxyphenylpyruvate dioxygenase inhibitor.
Elevated tyrosine levels, thrombocytopenia, leukopenia, conjunctivitis, corneal opacity, keratitis, photophobia, eye pain, blepharitis, cataracts, granulocytopenia, epistaxis, pruritus, exfoliative dermatitis, dry skin, maculopapular rash, alopecia.
Sign Up for Free e-newsletters
Regimen and Drug Listings
GET FULL LISTINGS OF TREATMENT Regimens and Drug INFORMATION
|Head and Neck Cancer||Regimens||Drugs|
|Renal Cell Carcinoma||Regimens||Drugs|
Cancer Therapy Advisor Articles
- Clostridium Difficile Infection in Patients With Cancer — In the Clinic
- Targeted and Immunotherapies for Metastatic Renal Cell Carcinoma
- Atezolizumab, Carboplatin, Nab-Paclitaxel Combination Prolongs PFS in NSCLC
- Nicotinamide and Cancer
- FDA Approves Front-Line Brentuximab Vedotin Plus Chemotherapy for Hodgkin Lymphoma
- Opioid Prescription Protocol Decreases Opioid Use After Gynecologic Surgery
- Everolimus Plus Letrozole May Be Active in Recurrent Endometrial Carcinoma
- Affordable Care Act Increased Percentage of Insured Patients With Gynecologic Cancers
- Everolimus Plus Letrozole: An Effective First-Line Therapy for Advanced Breast Cancer
- FDA Approves Nilotinib for Pediatric Patients With CML