Prospecto:Information for the User

Nitisinona Dipharma 2 mg Hard Capsules EFG

Nitisinona Dipharma 20 mg Hard Capsules EFG

nitisinona

Read this prospect carefully before starting to take this medicine, because it contains important information for you.

• Keep this prospect, as you may need to read it again.
• If you have any doubts, consult your doctor, pharmacist, or nurse.
• This medicine has been prescribed only for you, and you should not give it to other people even if they have the same symptoms as you, as it may harm them.
• If you experience any adverse effects, consult your doctor, pharmacist, or nurse, even if they are not listed in this prospect. See section 4.

1.What is Nitisinona Dipharma and for what it is used

2.What you need to know before starting to take Nitisinona Dipharma

3.How to take Nitisinona Dipharma

4.Possible adverse effects

5Storage of Nitisinona Dipharma

6.Contents of the package and additional information

Nitisinona Dipharma containsthe active ingredient nitisinona.Nitisinonais used to treat:

• a rare disease called hereditary tyrosinemia type 1 in adults, adolescents, and children (of any age group).
• a rare disease called alkaptonuria (AKU) in adults.

In these diseases, your body cannot fully break down the amino acid tyrosine (amino acids are the fundamental elements of proteins), forming toxic substances. These substances accumulate in your body.Nitisinonablocks the breakdown of tyrosine, and toxic substances do not form.

For the treatment of hereditary tyrosinemia type I, you must follow a special diet while taking this medication, because tyrosine will still be in your body. This diet is based on a low content of tyrosine and phenylalanine (another amino acid).

For the treatment of AKU, your doctor may advise you to follow a special diet.

Do not takeNitisinona Dipharma

• if you are allergic to nitisinona or any of the other ingredients of this medication (listed in section 6).

Do not breastfeed while taking this medication (see section “Pregnancy and lactation”).

Warnings and precautions

Consult your doctor or pharmacist before starting to take Nitisinona Dipharma.

• An ophthalmologist will examine your eyes before treatment and regularly during treatment with nitisinona. If you notice redness of the eyes or any other eye effect, contact your doctor immediately for an eye examination. Eye problems (see section 4) may be a sign of inadequate dietary control.

Blood samples will be taken during treatment to control whether the treatment is adequate and to ensure that there are no side effects causing blood alterations.

If you are taking Nitisinona Dipharma for the treatment of hereditary tyrosinemia type I, you will have periodic liver checks because the disease affects the liver.

Your doctor should follow up every 6 months. If you experience any adverse effect, it is recommended to use shorter time intervals.

Other medicationsand Nitisinona Dipharma

Inform your doctor or pharmacist if you are using, have used recently or may have to use any other medication.

Nitisinona Dipharma may interfere with the effect of other medications, such as:

• Medications for epilepsy (such as phenytoin)
• Medications against blood clot formation (such as warfarin)

Taking Nitisinona Dipharmawith food

If you start treatment taking it with food, it is recommended to follow this regimen throughout the entire treatment.

Pregnancy andlactation

The safety of this medication has not been studied in pregnant women and in women who are breastfeeding.

If you are pregnant or breastfeeding, or if you think you may be pregnant or intend to become pregnant, consult your doctor or pharmacist before using this medication.Do not breastfeed while taking this medication (see section “Do not take Nitisinona Dipharma”).

Driving and use of machines

The influence of this medication on the ability to drive and use machines is small. However, if you experience adverse effects that affect vision, you should not drive or use machines until your vision has returned to normal (see section 4 “Possible adverse effects”).

Follow exactly the administration instructions for this medication as indicated by your doctor.In case of doubt, consult your doctor or pharmacist again.

For hereditary tyrosinemia type I, treatment with this medication should be initiated and supervised by a doctor experienced in treating the disease.

The recommended daily dose for hereditary tyrosinemia type I is 1 mg/kg of body weight administered orally. Your doctor will adjust the dose individually.

It is recommended to administer the dose once a day.

However, due to limited data in patients with a body weight of less than 20 kg

The recommended dose for AKU is 10 mg once a day.

If you have trouble swallowing the capsules, you can open the capsules and mix the powder with a small amount of water or dietary supplement before taking it.

If you take more Nitisinona Dipharma than you should

If you have taken more of this medication than you should, inform your doctor or pharmacist immediately.

In case of overdose or accidental ingestion, consult your doctor or pharmacist immediately or call the Toxicological Information Service, phone: 91 562 04 20, indicating the medication and the amount ingested.

If you forget to take Nitisinona Dipharma

Do not take a double dose to compensate for the missed doses. If you have forgotten to take a dose, inform your doctor or pharmacist.

If you interrupt treatment with Nitisinona Dipharma

If you believe the medication's action is not adequate, inform your doctor. Do not change the dose or suspend treatment without first speaking with your doctor.

If you have any other questions about the use of this medication, ask your doctor or pharmacist.

Like all medications, this medication may cause adverse effects, although not everyone will experience them.

If you notice any eye-related adverse effects, inform your doctor immediately so they can perform an ophthalmological examination. Treatment with nitisinone increases blood tyrosine levels, which can cause eye-related symptoms. In patients with hereditary tyrosinemia type 1, frequently reported eye adverse effects (affecting more than 1 in 100 people) due to higher tyrosine levels include eye inflammation (conjunctivitis), corneal opacity and inflammation (keratitis), light sensitivity (photophobia), and eye pain. Blepharitis is a rare adverse effect (affecting up to 1 in 100 people).

In patients with AKU, eye irritation (keratopathy) and eye pain are frequently reported adverse effects (affecting more than 1 in 10 people).

The following are other adverse effects reported in patients with hereditary tyrosinemia type 1:

Other frequent adverse effects

Other rare adverse effects

• • Increased white blood cell count (leucocytosis),
  • itching (pruritus), skin inflammation (dermatitis exfoliativa), rash.
• The following are other adverse effects reported in patients with AKU:
• Other frequent adverse effects
• • bronchitis,
  • pneumonia,
  • itching (pruritus), rash.

Reporting Adverse Effects

If you experience any type of adverse effect, consult your doctor, pharmacist, or nurse, even if it is a possible adverse effect not listed in this prospectus. You can also report them directly through the Spanish System for Pharmacovigilance of Medicinal Products for Human Use (www.notificaRAM.es). By reporting adverse effects, you can contribute to providing more information on the safety of this medication.

Keepthis medicationout of the sight and reach of children.

Do not usethis medicationafter the expiration date that appears on the blister pack after “EXP” and onthebottle and box after “CAD”.The expiration date is the last day of the month indicated.

Store at a temperature below 30°C.

Medicines should not be disposed of through drains or in the trash. Ask your pharmacisthow to disposeofthepackaging and of themedicines that you no longerneed.By doing so, you will help protect the environment.

Nitisinona Dipharma Composition

• The active ingredient is nitisinona.

Nitisinona Dipharma 2 mg:Each hard capsule contains 2 mg of nitisinona.

Nitisinona Dipharma 20 mg:Each hard capsule contains 20 mg of nitisinona.

• The other components(excipients)are:

Hard capsule content:

Pregelatinized cornstarch

Stearic acid

Capsule coating:

Gelatin

Titanium dioxide (E171)

Printing ink:

Shellac

Propylene glycol

Aluminum lake Indigotina (E132)

Product appearance and packaging contents

The capsules are hard, white, opaque, made of gelatin, and have the company logo and the dose “2” or “20” printed in dark blue. The capsule contains a powder that may be white or off-white.

Nitisinona Dipharma is available in plastic bottles with a tamper-evident closure of 60 capsules and pre-cut, single-dose OPA/Alu/PVC-Alu blister packs of 60 capsules.

Only some package sizes may be marketed.

Marketing Authorization Holder

Dipharma Arzneimittel GmbH

Offheimer Weg 33

65549 Limburg a.d. Lahn

Germany

Manufacturer

Doppel Farmaceutici S.r.l.

Via Volturno 48

20089 Quinto dé Stampi - Rozzano (MI), Italy

For more information about this medication, please contact the local representative of the marketing authorization holder:

Aurovitas Spain, S.A.U.

Avda. de Burgos, 16-D

28036 Madrid

Spain

Tel.: 91 630 86 45

This medication is authorized in the member states of the European Economic Area with the following names:

GermanyNitisinone Dipharma

AustriaNitisinon Dipharma

BelgiumNitisinone Dipharma

DenmarkNitisinone Dipharma

SpainNitisinona Dipharma

FranceNitisinone Dipharma

IrelandNitisinone Dipharma

ItalyNitisinone Dipharma

NorwayNitisinone Dipharma

NetherlandsNitisinone Dipharma

PortugalNitisinona Dipharma

United KingdomNitisinone Dipharma

SwedenNitisinone Dipharma

Last review date of thisleaflet:february 2022.

For detailed information about this medication, please visit the website of the Spanish Agency for Medicines and Medical Devices (AEMPS) (http://www.aemps.gob.es/).