Aixiding®Nitisinone

Aixiding®Nitisinone Capsules

Brand Name:爱喜丁®(Aixiding)

Generic Name:Nitisinone Capsules

Strength:5mg(60 Capsules)/bottle

Manufacturer:Mendelikabs Inc

Marketing Authorization Holder:Guangzhou Han Guang Pharmaceutical Co., Ltd.

Approval Date in China:June 16th, 2021

Registration Number:国药准字HJ20210044

Storage:Store refrigerated at 2-8℃；protect from light and freezing

Price&Cost:Contact Us now for the best price of Nitisinone（Aixiding）

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  1. Indications and Usage
    • Hereditary Tyrosinemia Type 1 (HT-1)：Nitisinone is a hydroxyphenylpyruvate dioxygenase inhibitor indicated for the treatment of adult and pediatric patients with confirmed hereditary tyrosinemia type 1 (HT-1), in combination with dietary restriction of tyrosine and phenylalanine.
    • Alkaptonuria (AKU)：Nitisinone (Harliku) is indicated for the reduction of urine homogentisic acid (HGA) in adult patients with alkaptonuria (AKU).
  2.  Dosage and Administration
    2.1 Recommended Dosage (HT-1)
    • Starting Dose：0.5 mg/kg orally twice daily (actual body weight).
    • Maintenance Dose (≥5 years old)：After ≥4 weeks of stable dosing with undetectable serum/urine succinylacetone, the total daily dose (1–2 mg/kg) may be administered once daily.
    • Maximum Dose：2 mg/kg total daily dose.
    • AKU (Harliku)：2 mg orally once daily.
    2.2 Administration
    • Diet Requirement：Strictly maintain tyrosine/phenylalanine-restricted diet during treatment.
    • Timing：
    Capsules: Take 1 hour before or 2 hours after meals.
    Tablets/suspension: May be taken with or without food.
    • Missed Dose：Do not double the dose; take the next scheduled dose.
  3.  Mechanism of Action
    • Nitisinone is a competitive, reversible inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD).
    • HT-1 Pathway：HPPD acts upstream of fumarylacetoacetate hydrolase (FAH), the deficient enzyme in HT-1. Inhibition of HPPD blocks tyrosine catabolism, preventing accumulation of toxic metabolites (succinylacetone, succinylacetoacetate) that cause liver/kidney damage and neurotoxicity.
    • AKU Pathway：In AKU, HPPD inhibition reduces homogentisic acid (HGA) production, decreasing tissue deposition and urinary HGA excretion.
  4.  Safety and Warnings
    4.1 Boxed Warnings (HT-1)
    • Hematologic Toxicity：Risk of leukopenia, thrombocytopenia; monitor CBC regularly.
    • Ocular Toxicity：Elevated tyrosine causes corneal opacities, keratitis, photophobia; monitor eye function.
    • Hepatic Monitoring：Risk of liver failure/neoplasms; monitor liver function, alpha-fetoprotein (AFP).
    4.2 Contraindications
    • Hypersensitivity to nitisinone or excipients.
    4.3 Precautions
    • Tyrosine Elevation：Diet non-compliance causes severe hypertyrosinemia (eye/skin/neurotoxicity).
    • Pregnancy/Lactation：Use only if benefit outweighs risk; avoid breastfeeding.
    • Renal Impairment：No dose adjustment, but monitor succinylacetone.
  5.  Adverse Reactions
    5.1 Common (≥1%, HT-1)
    • Hematologic：Thrombocytopenia, leukopenia, anemia.
    • Ocular：Conjunctivitis, corneal opacity, photophobia, eye pain.
    • Hepatic：Elevated transaminases, liver failure, hepatic neoplasmsFDA Access Data.
    • Metabolic：Hypertyrosinemia.
    • Gastrointestinal：Vomiting, diarrhea, abdominal pain.
    5.2 Rare (<1%)
    • Skin rash, alopecia, headache, seizures, renal dysfunction.
    5.3 AKU (Harliku)
    • Mild: Headache, nausea, diarrhea, arthralgia.
  6.  Drug Interactions
    • CYP2C9 Inhibition：Nitisinone is a moderate CYP2C9 inhibitor; reduce dose of CYP2C9 substrates (e.g., warfarin, phenytoin) by 50%.
    • OAT1/OAT3 Inhibition：Inhibits organic anion transporters; avoid co-administration with OAT1/OAT3 substrates (e.g., methotrexate, penicillin).
    • No Effect on CYP1A2, CYP2D6, CYP3A4：No dose adjustment for substrates of these enzymes.
  7.  Pharmaceutical Information
    7.1 Active Ingredient
    • Nitisinone：Chemical name: 2-(2-nitro-4-trifluoromethylbenzoyl)cyclohexane-1,3-dione; Molecular formula: C₁₄H₁₀F₃NO₅; Molecular weight: 329.23.
    7.2 Dosage Forms & Strengths
    • Capsules ：2 mg, 5 mg, 10 mg, 20 mg (white, opaque hard gelatin capsules).
    7.3 Excipients
    • Capsules：Pregelatinized starch, citric acid anhydrous, hypromellose, mannitol, stearic acid; capsule shell: gelatin, titanium dioxide.
    7.4 Storage
    • Capsules：Store at 2°C–8°C (refrigerate) in original container, protected from light.

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