GNH USA

Europe — EMA

Nitisinone Dipharma

GNH USA supplies Nitisinone Dipharma (Nitisinone), a 4-hydroxyphenylpyruvate dioxygenase inhibitor for hereditary tyrosinemia type I and investigational

Nitisinone

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What is Nitisinone?

Nitisinone, the active ingredient in Nitisinone Dipharma, is a 4-hydroxyphenylpyruvate dioxygenase (HPPD) inhibitor presented as an oral tablet for oral use. It treats hereditary tyrosinemia type I and is being investigated for alkaptonuria in affected patients.

GNH USA supplies Nitisinone Dipharma as a licensed, GDP-compliant international pharmaceutical supplier serving hospitals, transplant centres, oncology centres, pharmacies and clinics worldwide.

What is Nitisinone used for?

Nitisinone acts on the tyrosine degradation pathway and is indicated for specific inherited metabolic disorders.

Hereditary tyrosinemia type I: reduces accumulation of toxic metabolites and improves liver function. Alkaptonuria (investigational): may lower homogentisic acid levels, under clinical investigation.

What are the side effects of Nitisinone?

Adverse reactions may vary in frequency and severity.

Common Side Effects: - Nausea - Vomiting - Abdominal pain - Headache - Fatigue - Skin rash

Serious or Rare Side Effects: - Hepatotoxicity (elevated liver enzymes, liver failure) - Severe hypoglycemia - Peripheral neuropathy - Severe skin reactions (Stevens‑Johnson syndrome, toxic epidermal necrolysis) - Anaphylactic reactions - Acute liver failure

What precautions apply to Nitisinone?

Before initiating therapy, clinicians should consider several safety measures.

Monitor liver function: perform baseline and periodic liver enzyme tests. Assess tyrosine levels: avoid excessive dietary tyrosine intake. Avoid in patients with known hypersensitivity to nitisinone. Use caution during pregnancy and breastfeeding: safety data are limited. Adjust dosing in renal impairment: monitor renal function. Store at below 25°C: keep in the original pack, protect from moisture.

What does Nitisinone interact with?

Nitisinone may interact with other medicines, requiring attention to potential effects.

Major Interactions (Avoid): - Co‑administration with other HPPD inhibitors: risk of additive toxicity. - High‑protein supplements or amino‑acid formulas that increase tyrosine levels.

Moderate Interactions (Monitor Closely): - Warfarin: possible alteration of INR, monitor coagulation. - Phenobarbital or other enzyme‑inducing agents: may affect nitisinone metabolism. - Oral contraceptives: monitor for hormonal changes or reduced efficacy. - Antacids containing aluminum or magnesium: may affect absorption, separate administration by at least 2 hours.

Frequently asked questions

What is Nitisinone Dipharma used for?+
Nitisinone Dipharma is used to treat hereditary tyrosinemia type I, a rare inherited disorder that affects the liver and kidneys. It is also being studied as a potential therapy for alkaptonuria, another metabolic condition.
How does nitisinone work in the body?+
Nitisinone blocks the enzyme 4‑hydroxyphenylpyruvate dioxygenase (HPPD) in the tyrosine degradation pathway. This inhibition prevents the formation of toxic downstream metabolites that cause organ damage in hereditary tyrosinemia type I.
What is the usual dose of Nitisinone Dipharma?+
The prescribed dose of Nitisinone Dipharma is determined by the treating physician based on the patient’s age, weight, disease severity, and laboratory results. Dosage adjustments are made according to clinical response and safety monitoring.
Is Nitisinone safe during pregnancy or breastfeeding?+
Safety data for nitisinone in pregnancy and lactation are limited. The drug should only be used if the potential benefit justifies the potential risk to the fetus or infant, and the decision must be made by a qualified healthcare professional.
How do I order Nitisinone Dipharma from GNH USA?+
To place an order, submit an enquiry on the product page at gnh-usa.com specifying the required quantity. GNH USA works with hospitals, pharmacies and procurement teams worldwide, verifies trade or institutional credentials, and then provides pricing, availability, shipping details and any necessary import documentation.
How does Nitisinone compare with other treatments for hereditary tyrosinemia type I?+
Nitisinone is the primary disease‑modifying therapy for hereditary tyrosinemia type I, directly targeting the metabolic defect. Alternative options such as liver transplantation address organ failure but do not correct the underlying metabolic pathway. Nitisinone therefore offers a less invasive, targeted approach.
What are the storage and handling requirements for Nitisinone Dipharma?+
Nitisinone Dipharma tablets should be stored at temperatures below 25 °C, in their original packaging, and protected from moisture and direct sunlight. Keep the product out of reach of children.
How is Nitisinone Dipharma administered?+
The medication is supplied as oral tablets and is taken by mouth with water. It is usually administered once daily, but the exact schedule is set by the prescribing clinician based on individual patient needs.
What are the most serious risks associated with Nitisinone Dipharma?+
Serious risks include hepatotoxicity, severe hypoglycemia, peripheral neuropathy, and rare but life‑threatening skin reactions such as Stevens‑Johnson syndrome. Patients should be monitored regularly for liver function and any signs of severe adverse reactions.
Who should not use Nitisinone Dipharma?+
Patients with known hypersensitivity to nitisinone or any of its excipients should avoid the product. Caution is advised in individuals with severe liver disease, uncontrolled diabetes, or those taking interacting medications listed under major interactions.
Information provided on this page is for educational use by procurement and clinical professionals. Always consult a licensed healthcare provider for medical advice and prescribing decisions. GNH USA supplies this product subject to applicable regulatory and licensing requirements.
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Product details

Drug
Nitisinone
Route
Oral Use
Manufacturer
Dipharma Arzneimittel Gmbh
Origin
EU

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