Product image of Nitisinona Dipharma (Nitisinone) supplied by GNH India

Nitisinona Dipharma

Active Ingredient:
Nitisinone
Origin:
EU

Nitisinona Dipharma (Nitisinone)

Nitisinone, the active ingredient in Nitisinona Dipharma, is a p‑Hydroxyphenylpyruvate dioxygenase (HPPD) inhibitor presented as an oral tablet for oral use. It blocks the formation of toxic metabolites in the tyrosine degradation pathway and is indicated for hereditary tyrosinemia type I, with off‑label use for alkaptonuria.

GNH India supplies Nitisinona Dipharma as a licensed, GDP‑compliant international pharmaceutical supplier serving hospitals, pharmacies and clinics worldwide, providing reliable sourcing for rare‑disease therapies across EU and global markets, and ensuring compliance with local regulatory requirements.

Manufacturer / TM Owner

Dipharma Arzneimittel Gmbh

1
Pack/s
This product is added to your enquiry list

Request a Quote

Please fill out the form below and our team will get back to you shortly with pricing and further information.

A minimum order value applies. Our team will confirm the exact amount or quantity after reviewing your requirements.

Indications & Clinical Uses

Nitisinona Dipharma (nitisinone) targets disorders of tyrosine metabolism, providing disease‑modifying therapy.

  • Hereditary tyrosinemia type I: reduces accumulation of toxic metabolites, improving liver function and survival.
  • Alkaptonuria (off‑label): lowers homogentisic acid production, potentially slowing ochronotic pigment deposition.

How It Works

  • Nitisinone selectively inhibits the enzyme p‑hydroxyphenylpyruvate dioxygenase (HPPD), a key catalyst in the catabolic pathway of the amino acid tyrosine. By blocking HPPD, the drug prevents conversion of p‑hydroxyphenylpyruvate to maleylacetoacetate, thereby halting the downstream formation of toxic metabolites such as succinylacetone. This interruption reduces hepatic and renal toxicity associated with hereditary tyrosinemia type I and decreases homogentisic acid generation in alkaptonuria.

Side Effects

Nitisinona Dipharma may be associated with several adverse reactions, which are categorized by frequency and severity.

Common Side Effects

  • Nausea
  • Headache
  • Fatigue
  • Skin rash

Serious or Rare Side Effects

  • Hepatic dysfunction
  • Severe allergic reaction
  • Neutropenia

Precautions & Warnings

When prescribing Nitisinona Dipharma, clinicians should observe the following precautions to ensure patient safety.

  • Liver function monitoring: assess hepatic enzymes regularly during therapy.
  • Hematologic monitoring: perform complete blood counts to detect neutropenia.
  • Renal function assessment: evaluate kidney parameters before and during treatment.
  • Hypersensitivity risk: discontinue if signs of severe allergic reaction appear.
  • Pregnancy and lactation: safety not established; avoid use unless benefits outweigh risks.
  • Store at below 25°C: keep in the original packaging, protect from moisture and light.

Avoid Interactions

Nitisinona Dipharma can interact with other medicines; awareness of major and moderate interactions helps prevent adverse outcomes.

Major Interactions (Avoid)

  • Warfarin: may potentiate anticoagulant effect and increase INR.
  • Strong CYP2C9 inhibitors (e.g., fluconazole): can raise nitisinone plasma concentrations.

Moderate Interactions (Monitor Closely)

  • Antacids: may reduce oral absorption of nitisinone.
  • Vitamin K supplements: could counteract potential coagulopathy.
  • High‑protein diet: may influence tyrosine metabolism and drug efficacy.

Frequently Asked Questions

Nitisinona Dipharma contains nitisinone, an HPPD inhibitor indicated for the treatment of hereditary tyrosinemia type I. It is also used off‑label to manage alkaptonuria, a condition characterized by excess homogentisic acid accumulation.

Nitisinone blocks the enzyme p‑hydroxyphenylpyruvate dioxygenase, interrupting the tyrosine degradation pathway. This prevents formation of downstream toxic metabolites such as succinylacetone in tyrosinemia and reduces homogentisic acid production in alkaptonuria.

The dosage of Nitisinona Dipharma is individualized. A qualified prescriber determines the appropriate strength, frequency, and duration based on the patient’s age, disease severity, and clinical response.

Safety data for nitisinone during pregnancy and lactation are limited. The product should be avoided unless the potential benefit justifies the potential risk to the fetus or infant, and the decision should be made by the treating physician.

To order, submit an enquiry on the product page at gnhindia.com, specifying the required quantity. GNH India 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.

Nitisinone (also known as NTBC) is the primary pharmacologic option for hereditary tyrosinemia type I, offering a disease‑modifying effect that dietary restriction alone cannot achieve. Compared with supportive care alone, nitisinone markedly reduces liver failure risk and improves long‑term survival.

Store the tablets at temperatures below 25 °C in their original container. Protect them from moisture, excessive heat and direct sunlight to maintain potency throughout the shelf life.

Nitisinona Dipharma is taken orally as a tablet with a glass of water. It may be taken with or without food, but consistent administration with respect to meals helps maintain stable absorption.

Clinicians should monitor for hepatic dysfunction, severe allergic reactions (including rash, swelling or breathing difficulty), and neutropenia. Prompt evaluation and discontinuation are advised if any of these serious adverse events occur.

Patients with known hypersensitivity to nitisinone or any tablet excipients should avoid the product. It is also contraindicated in individuals with severe liver impairment where alternative management is required.

Published by

GNH India Pharmaceuticals Limited

Licensed pharmaceutical supplier

Last updated:

Disclaimer

At GNH India, we aim to provide information that is expert-reviewed, accurate, and reliable. However, all content on this platform is intended for general informational purposes only and does not constitute professional medical advice, diagnosis, or treatment. While efforts are made to maintain accuracy and currency, the information may contain errors, omissions, or may not reflect the latest developments. Always consult a qualified healthcare professional regarding any medical concerns.

All product names, brand names, trademarks and registered trademarks displayed on this website are the property of their respective owners. This website and its operator are not affiliated with, endorsed by, or authorized by any such trade mark owners unless expressly stated. Any reference to product names or trademarks is made solely for descriptive and informational purposes. No commercial relationship, sponsorship, approval, or association with any trademark owner should be inferred. Information provided herein does not constitute promotion, advertising, or an offer for sale of any medicinal product and is intended solely for general informational purposes.

All trademarks and brand names displayed on this website are used strictly in accordance with the provisions of the Trade Marks Act, 1999, specifically Sections 30 and 30(1) governing fair use. Trademark ownership has been accurately attributed under the "Manufacturer/ TM Owner" section in compliance with statutory requirements.

Related Products

Other formulations containing Nitisinone

A

A
A

A
A

A
A

A

Browse all products →

Ordering Process

Simple, Secure, and Seamless

Learn how our streamlined ordering process guarantees fast, compliant, and reliable access to essential medicines worldwide.

Quote request form

01

Submit Quote Request Form

Tell us what medications you need by filling out our simple form and uploading a valid document.

Price estimate review

02

We Review & Send Estimate

Our licensed pharmacists will verify your details and provide you with a detailed cost estimate.

Secure payment

03

Confirm & Make Payment

Discuss any queries, approve the estimate, and complete your payment.

Worldwide delivery

04

Get Delivered

Your medicines, safely packed and delivered to your door or nearest airport — hassle-free.

Regulatory recognitions

Quality You Can Trust

Our internationally recognized certifications underscore our commitment to safety, quality, and excellence.

SGS GSDP Certification

GSDP Compliant from SGS

URS ISO 9001

URS ISO 9001 Certification

FDA Maharashtra

Licensed by FDA Maharashtra

IEC License DGFT

IEC License issued by DGFT