Drug reference
Hereditary tyrosinaemia type I (in combination with dietary restriction of tyrosine and phenylalanine) (specialist use only) ▶BY MOUTH ▶Adult: Initially 500 micrograms/kg twice daily, adjusted according to response; maximum 2 mg/kg per day
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Slit-lamp examination of eyes recommended before treatment.
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→Appendix 1: nitisinone
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▶Common or very common Corneal opacity. eye inflammation. eye pain. granulocytopenia. leucopenia. photophobia. thrombocytopenia ▶Uncommon Leucocytosis. skin reactions
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Manufacturer advises avoid unless potential benefit outweighs risk—toxicity in animal studies.
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Manufacturer advises avoid—adverse effects in animal studies.
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▶Monitor liver function regularly. ▶Monitor platelet and white blood cell count every 6 months.
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Capsules can be opened and the contents suspended in a small amount of water or formula diet and taken immediately.
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There can be variation in the licensing of different medicines containing the same drug. Oral suspension ▶Orfadin (Swedish Orphan Biovitrum Ltd) Nitisinone 4 mg per 1 ml Orfadin 4mg/1ml oral suspension sugar- free | 90 ml P £1,692.00 DT = £1,692.00 Capsule ▶Nitisinone (Non-proprietary) Nitisinone 2 mg Nitisinone 2mg capsules | 60 capsule P £423.00 Nitisinone 5 mg Nitisinone 5mg capsules | 60 capsule P £845.25 Nitisinone 10 mg Nitisinone 10mg capsules | 60 capsule P £1,546.50 ▶Orfadin (Swedish Orphan Biovitrum Ltd) Nitisinone 2 mg Orfadin 2mg capsules | 60 capsule P £564.00 Nitisinone 5 mg Orfadin 5mg capsules | 60 capsule P £1,127.00 Nitisinone 10 mg Orfadin 10mg capsules | 60 capsule P £2,062.00 Nitisinone 20 mg Orfadin 20mg capsules | 60 capsule P £4,512.00 DT = £4,512.00 2.12 Urea cycle disorders AMINO ACIDS AND DERIVATIVES
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Initially 500 micrograms/kg twice daily, adjusted
First-line fragment. Read the complete Indications and Dose section above.
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