Nityr (Nitisinone)

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According to the European Medicines Agency, brand name Nityr originating from Europe is manufactured at the following site(s):Millmount Healthcare Limited Block-7, City North Business Campus Stamullen, Co. Meath, K32 YD60, IrelandSciensus International B.V. Bijsterhuizen 3142 6604 LV Wijchen Netherlands

Information about Nityr (Nitisinone)

Nityr is the brand name for nitisinone, an oral medication primarily used to treat tyrosinaemia type 1, a rare genetic disorder that affects the breakdown of the amino acid tyrosine. In this condition, the body cannot properly break down tyrosine, leading to the accumulation of toxic substances that can cause severe liver, kidney, and neurological damage. Nitisinone works by inhibiting the enzyme 4-hydroxyphenylpyruvate dioxygenase (HPPD), which is involved in the breakdown of tyrosine. By blocking this enzyme, nitisinone prevents the production of toxic metabolites, helping to manage the symptoms of tyrosinaemia type 1.

Product Highlights

• Nitisinone is indicated for the treatment of hereditary tyrosinaemia type 1, a metabolic disorder caused by a deficiency in the enzyme fumarylacetoacetate hydrolase (FAH). This condition leads to the accumulation of toxic metabolites that can result in liver failure, renal tubular dysfunction, and neurological impairment.
• Nitisinone helps manage the life-threatening complications of tyrosinaemia type 1 by reducing the production of toxic metabolites, improving liver function, and preventing neurological damage.

Key Ingredient

• Nitisinone

Key Benefits

• By inhibiting the HPPD enzyme, nitisinone prevents the buildup of toxic metabolites (such as succinylacetone) that can damage the liver, kidneys, and nervous system.
• It helps prevent severe liver dysfunction, liver failure, and kidney damage in patients with tyrosinaemia type 1.
• Nitisinone, when combined with a low-tyrosine diet, significantly improves long-term outcomes in patients with tyrosinaemia type 1, reducing the risk of complications such as cirrhosis and liver transplant.
• The medication may reduce the risk of neurological problems, including developmental delays and movement disorders, which are common in untreated tyrosinaemia type 1.

Direction of Use

• Nitisinone is taken orally, either as a capsule or an oral suspension.
• Capsules: Typically, the dose for adults and children over 2 years of age is based on body weight, usually starting at 1 mg/kg per day divided into two doses.
• Oral Suspension: For patients who cannot swallow capsules, the oral suspension form may be used.
• The dosage may be adjusted depending on the patient's response to treatment and the results of laboratory tests, such as monitoring of tyrosine levels in the blood.
• Nitisinone should be used as part of a comprehensive treatment plan that includes dietary restrictions to limit the intake of tyrosine and phenylalanine, as these amino acids contribute to the disease's progression.

Safety Concerns

• Nitisinone may cause retinopathy (damage to the retina), which can lead to visual disturbances. Regular eye exams are recommended to monitor for early signs of retinal damage, particularly for long-term users.
• Liver function should be closely monitored, as tyrosinaemia type 1 can lead to liver dysfunction, and nitisinone's effects on liver function should be assessed regularly.
• In some cases, nitisinone can cause elevated tyrosine levels, which may lead to adverse effects like corneal clouding and other potential long-term health concerns. Close monitoring of blood tyrosine levels is essential during treatment.
• Nitisinone treatment should be carefully monitored in growing children to ensure proper growth and development, as the disease itself and treatment might impact these aspects.

Avoid Nityr (Nitisinone) If

• Avoid nitisinone if you have a known allergy to the drug or any of its components.
• If you have a pre-existing condition of the eye, particularly severe retinopathy, nitisinone may exacerbate the condition, and its use should be avoided or closely monitored.
• Nitisinone is classified as a Category C drug in pregnancy. It should be used during pregnancy only if the benefits are deemed greater than the potential risks to the fetus. It is important to consult a healthcare provider to discuss these risks.
• The safety of nitisinone during breastfeeding has not been fully established, so it should be used with caution, and a healthcare provider should be consulted to determine if it is appropriate.
• Nitisinone should be avoided in patients with severe liver impairment unless closely monitored, as it may worsen liver function in some individuals.