ATC Group: A16AX04 Nitisinone

The World Health Organization's ATC classification organizes medical drugs based on therapeutic properties, chemical composition, and anatomy. It helps make essential medicines readily available globally and is widely used in the pharmaceutical industry.

Position of A16AX04 in the ATC hierarchy

Level Code Title
1 A Alimentary tract and metabolism
2 A16 Other alimentary tract and metabolism products
3 A16A Other alimentary tract and metabolism products
4 A16AX Various alimentary tract and metabolism products
5 A16AX04 Nitisinone

Defined daily dose

The DDD is the assumed average maintenance dose per day for a drug used for its main indication in adults. The DDD is a unit of measurement and does not necessarily reflect the recommended or Prescribed Daily Dose. Therapeutic doses for individual patients and patient groups will often differ from the DDD as they will be based on individual characteristics (such as age, weight, ethnic differences, type and severity of disease) and pharmacokinetic considerations.

Code Route Amount Notes
ORAL Oral 20 mg

Active ingredients in A16AX04

Active Ingredient

Nitisinone is a competitive inhibitor of 4-hydroxyphenylpyruvate dioxygenase, an enzyme which precedes fumarylacetoacetate hydrolase in the tyrosine catabolic pathway. By inhibiting the normal catabolism of tyrosine in patients with hereditary tyrosinemia type 1 (HT-1), nitisinone prevents the accumulation of the toxic intermediates maleylacetoacetate and fumarylacetoacetate.

Related product monographs

Document Type Information Source  
 NITYR Tablet MPI, US: SPL/PLR FDA, National Drug Code (US)
 ORFADIN Capsule / Oral suspension MPI, US: SPL/PLR FDA, National Drug Code (US)

United States medicines

Medicines classified under this ATC code globally

Austria (AT)

Brazil (BR)

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Croatia (HR)

Estonia (EE)

Finland (FI)

France (FR)

Ireland (IE)

Israel (IL)

Italy (IT)

Japan (JP)

Lithuania (LT)

Netherlands (NL)

New Zealand (NZ)

Poland (PL)

Romania (RO)

Spain (ES)

Tunisia (TN)

Turkey (TR)

United Kingdom (UK)

Note the following: The list of brand names is continuously updated, and thus does not include the total of products circulating worldwide.