{"id":2814,"date":"2026-05-26T05:08:42","date_gmt":"2026-05-26T12:08:42","guid":{"rendered":"https:\/\/xpharma.com.cn\/?post_type=product&p=2814"},"modified":"2026-05-26T05:08:42","modified_gmt":"2026-05-26T12:08:42","slug":"aixidingnitisinone-capsules","status":"publish","type":"product","link":"https:\/\/xpharma.com.cn\/index.php\/product\/aixidingnitisinone-capsules\/","title":{"rendered":"Aixiding\u00aeNitisinone Capsules"},"content":{"rendered":"
    \n
  1. Indications and Usage
    \n\u2022 Hereditary Tyrosinemia Type 1 (HT-1)\uff1aNitisinone 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.
    \n\u2022 Alkaptonuria (AKU)\uff1aNitisinone (Harliku) is indicated for the reduction of urine homogentisic acid (HGA) in adult patients with alkaptonuria (AKU).<\/li>\n
  2. \u00a0Dosage and Administration
    \n2.1 Recommended Dosage (HT-1)
    \n\u2022 Starting Dose\uff1a0.5 mg\/kg orally twice daily (actual body weight).
    \n\u2022 Maintenance Dose (\u22655 years old)\uff1aAfter \u22654 weeks of stable dosing with undetectable serum\/urine succinylacetone, the total daily dose (1\u20132 mg\/kg) may be administered once daily.
    \n\u2022 Maximum Dose\uff1a2 mg\/kg total daily dose.
    \n\u2022 AKU (Harliku)\uff1a2 mg orally once daily.
    \n2.2 Administration
    \n\u2022 Diet Requirement\uff1aStrictly maintain tyrosine\/phenylalanine-restricted diet during treatment.
    \n\u2022 Timing\uff1a
    \nCapsules: Take 1 hour before or 2 hours after meals.
    \nTablets\/suspension: May be taken with or without food.
    \n\u2022 Missed Dose\uff1aDo not double the dose; take the next scheduled dose.<\/li>\n
  3. \u00a0Mechanism of Action
    \n\u2022 Nitisinone is a competitive, reversible inhibitor of 4-hydroxyphenylpyruvate dioxygenase (HPPD).
    \n\u2022 HT-1 Pathway\uff1aHPPD 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.
    \n\u2022 AKU Pathway\uff1aIn AKU, HPPD inhibition reduces homogentisic acid (HGA) production, decreasing tissue deposition and urinary HGA excretion.<\/li>\n
  4. \u00a0Safety and Warnings
    \n4.1 Boxed Warnings (HT-1)
    \n\u2022 Hematologic Toxicity\uff1aRisk of leukopenia, thrombocytopenia; monitor CBC regularly.
    \n\u2022 Ocular Toxicity\uff1aElevated tyrosine causes corneal opacities, keratitis, photophobia; monitor eye function.
    \n\u2022 Hepatic Monitoring\uff1aRisk of liver failure\/neoplasms; monitor liver function, alpha-fetoprotein (AFP).
    \n4.2 Contraindications
    \n\u2022 Hypersensitivity to nitisinone or excipients.
    \n4.3 Precautions
    \n\u2022 Tyrosine Elevation\uff1aDiet non-compliance causes severe hypertyrosinemia (eye\/skin\/neurotoxicity).
    \n\u2022 Pregnancy\/Lactation\uff1aUse only if benefit outweighs risk; avoid breastfeeding.
    \n\u2022 Renal Impairment\uff1aNo dose adjustment, but monitor succinylacetone.<\/li>\n
  5. \u00a0Adverse Reactions
    \n5.1 Common (\u22651%, HT-1)
    \n\u2022 Hematologic\uff1aThrombocytopenia, leukopenia, anemia.
    \n\u2022 Ocular\uff1aConjunctivitis, corneal opacity, photophobia, eye pain.
    \n\u2022 Hepatic\uff1aElevated transaminases, liver failure, hepatic neoplasmsFDA Access Data.
    \n\u2022 Metabolic\uff1aHypertyrosinemia.
    \n\u2022 Gastrointestinal\uff1aVomiting, diarrhea, abdominal pain.
    \n5.2 Rare (<1%)
    \n\u2022 Skin rash, alopecia, headache, seizures, renal dysfunction.
    \n5.3 AKU (Harliku)
    \n\u2022 Mild: Headache, nausea, diarrhea, arthralgia.<\/li>\n
  6. \u00a0Drug Interactions
    \n\u2022 CYP2C9 Inhibition\uff1aNitisinone is a moderate CYP2C9 inhibitor; reduce dose of CYP2C9 substrates (e.g., warfarin, phenytoin) by 50%.
    \n\u2022 OAT1\/OAT3 Inhibition\uff1aInhibits organic anion transporters; avoid co-administration with OAT1\/OAT3 substrates (e.g., methotrexate, penicillin).
    \n\u2022 No Effect on CYP1A2, CYP2D6, CYP3A4\uff1aNo dose adjustment for substrates of these enzymes.<\/li>\n
  7. \u00a0Pharmaceutical Information
    \n7.1 Active Ingredient
    \n\u2022 Nitisinone\uff1aChemical name: 2-(2-nitro-4-trifluoromethylbenzoyl)cyclohexane-1,3-dione; Molecular formula: C\u2081\u2084H\u2081\u2080F\u2083NO\u2085; Molecular weight: 329.23.
    \n7.2 Dosage Forms & Strengths
    \n\u2022 Capsules \uff1a2 mg, 5 mg, 10 mg, 20 mg (white, opaque hard gelatin capsules).
    \n7.3 Excipients
    \n\u2022 Capsules\uff1aPregelatinized starch, citric acid anhydrous, hypromellose, mannitol, stearic acid; capsule shell: gelatin, titanium dioxide.
    \n7.4 Storage
    \n\u2022 Capsules\uff1aStore at 2\u00b0C\u20138\u00b0C (refrigerate) in original container, protected from light.<\/li>\n<\/ol>\n","protected":false},"excerpt":{"rendered":"

    Brand Name:<\/span>\u7231\u559c\u4e01\u00ae(<\/span>Aixiding)<\/span><\/p>\n

    Generic Name:Nitisinone Capsules<\/span><\/p>\n

    Strength:5mg(60 Capsules)\/bottle<\/span><\/p>\n

    Manufacturer:Mendelikabs Inc<\/span><\/p>\n

    Marketing Authorization Holder:Guangzhou Han Guang Pharmaceutical Co., Ltd.<\/span><\/p>\n

    Approval Date in China:June 16th, 2021<\/span><\/p>\n

    Registration Number:<\/span>\u56fd\u836f\u51c6\u5b57HJ20210044<\/span><\/p>\n

    Storage:Store refrigerated at 2-8\u2103<\/span>\uff1bprotect from light and freezing<\/span><\/p>\n

    Price&Cost:Contact Us now for the best price of Nitisinone<\/span>\uff08Aixiding<\/span>\uff09<\/p>\n","protected":false},"featured_media":2816,"comment_status":"open","ping_status":"closed","template":"","meta":[],"product_brand":[],"product_cat":[26],"product_tag":[],"class_list":["post-2814","product","type-product","status-publish","has-post-thumbnail","product_cat-capsules","first","instock","shipping-taxable","product-type-simple"],"yoast_head":"\nAixiding\u00aeNitisinone Capsules Latest Cost and Price in China- Xpharma China<\/title>\n<meta name=\"description\" content=\"Looking for the best way to buyAixiding\u00aeNitisinone Capsules ?saving tips?Buying from Xpharma China may be a better way. 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