natgillin/translations-raw huggingface dataset:
The final study results will be submitted to NCAs/RMS by:
Končni rezultati študije bodo predloženi nacionalnim agencijam držav(e) članic(e) ali referenčne(ih) držav(e) članic(e):
Adults (including the elderly)
Odrasli (vključno s starejšimi)
The study should be conducted according to an agreed protocol.
Študijo je treba izvesti v skladu z dogovorjenim protokolom.
Date of first authorisation: 25 July 2019
Datum prve odobritve: 25. julij 2019
Frequency of monitoring is recommended to be at least twice a year.
Priporočljiva pogostost spremljanja je vsaj dvakrat letno.
However, a risk to the newborns/infants cannot be excluded.
Toda tveganja za novorojenčke/dojenčke ni mogoče izključiti.
Do not stop or change your treatment without speaking with your doctor even if you feel better.
Ne ustavite ali spremenite zdravljenja, ne da bi se pogovorili z zdravnikom, tudi če se počutite bolje.
Take this medicine with water only.
To zdravilo vzemite samo z vodo.
Do not take it with other drinks or food because they may reduce the medicine's effect.
Ne vzemite ga z drugimi pijačami ali hrano, ker lahko zmanjšajo učinek zdravila.
Do not use if the capsules become sticky or wet.
Ne uporabljajte, če kapsule postanejo lepljive ali mokre.
Exposure seems to be highly variable between subjects.
Izpostavljenost je med posamezniki zelo variabilna.
Electrocardiography was also unaffected.
Tudi elektrokardiografija je ostala nespremenjena.
Capsules should be swallowed whole with water.
Kapsule je treba pogoltniti cele z vodo.
You and your doctor should fully discuss the potential benefits of treatment whilst considering any possible risks that there may be.
Vi in vaš zdravnik se morate v celoti pogovoriti o možnih koristih zdravljenja, pri čemer upoštevajte morebitna tveganja.
It is possible that this effect may be more evident in patients with pre-existing neurological symptoms.
Možno je, da je ta učinek bolj očiten pri bolnikih z obstoječimi nevrološkimi simptomi.
Therefore, monitoring for manifestations of overtreatment should be undertaken.
Zato je treba spremljati manifestacije prekomernega zdravljenja.
Moreover, there was some doubt about the relationship to trientine, as the lung findings were also observed in most of the control dogs in the 26 week study.
Poleg tega je obstajal dvom glede povezave s trientinom, saj so bile ugotovitve glede pljuč opažene tudi pri večini kontrolnih psov v 26-tedenski študiji.
Final report: Q4 2025 (main study)
Končno poročilo: 4. trimesečje 2025 (glavna študija)
The OECD SIDS triethylenetetramine 2002 classifies the genotoxic profile of trientine as low priority/concern.
OECD SIDS trietilentetramin 2002 razvršča genotoksični profil trientina kot nizko prioriteto/skrb.
The doctor may need to increase or decrease your dose of Cufence.
Zdravnik bo morda moral povečati ali zmanjšati odmerek zdravila Cufence.
At the start of treatment the dose varies between 2 and 5 capsules per day.
Na začetku zdravljenja se odmerek giblje med 2 in 5 kapsulami na dan.
After first opening the bottle: 3 months.
Po prvem odprtju steklenice: 3 meseci.
If you forget to take a dose take your next dose at its usual scheduled time.
Če ste pozabili vzeti odmerek, vzemite naslednji odmerek ob običajnem času.
Therefore, iron supplementation may be necessary in some cases.
Zato so morda v nekaterih primerih potrebni dodatki železa.
However it was not possible to determine if the reaction was due to trientine or to previous penicillamine treatment.
Vendar pa ni bilo mogoče ugotoviti, ali je bila reakcija posledica trientina ali predhodnega zdravljenja s pencilaminom.
Neurological deterioration can occur at the start of the treatment.
Nevrološko poslabšanje se lahko pojavi na začetku zdravljenja.
You must contact your doctor or another health care provider immediately.
Nemudoma se morate obrniti na zdravnika ali drugega zdravstvenega delavca.
Cufence with food and drink
Zdravilo Cufence skupaj s hrano in pijačo
Some functional neurological abnormalities were also identified, particularly in severly affected animals, however no associated nerve damage was observed.
Opredeljene so bile tudi nekatere funkcionalne nevrološke anomalije, zlasti pri močno prizadetih živalih, vendar niso bile opažene nobene poškodbe živcev.
Use within 3 months after first opening the bottle.
Porabite v 3 mesecih po prvem odprtju steklenice.
This medicine is for long-term use because Wilson's disease is a life-long condition.
To zdravilo je namenjeno za dolgotrajno uporabo, ker je Wilsonova bolezen vseživljenjsko stanje.
The total daily dose can be divided into 2 to 4 smaller doses, as indicated by your doctor.
Celotni dnevni odmerek lahko razdelimo v 2 do 4 manjše odmerke, kot je predpisal zdravnik.
- The other ingredients are
- Ostale sestavine zdravila so
If you are allergic to trientine or any of the other ingredients of this medicine (listed in section 6).
Če ste alergični na trientin ali katero koli sestavino tega zdravila (navedeno v poglavju 6).
Your doctor will need to regularly check for symptoms of the disease and copper levels in your blood and urine.
Zdravnik bo moral redno preverjati simptome bolezni in ravni bakra v krvi in urinu.
Your doctor will decide the correct dose for you.
Zdravnik bo določil ustrezen odmerek za vas.
Iron supplementation may be necessary in some cases.
V nekaterih primerih so morda potrebni dodatki železa.
The dose is lower than for adults and depends on age and body weight.
Odmerek je manjši kot pri odraslih in je odvisen od starosti in telesne mase.
< 1/10); uncommon (≥ 1/1,000 to < 1/100); rare (≥ 1/10,000 to < 1/1,000); very rare (< 1/10,000); not known (cannot be estimated from the available data).
Pogostnost je opredeljena kot: zelo pogosti (≥ 1/10), pogosti (≥ 1/100 do < 1/10), občasni (≥ 1/1.000 do < 1/100), redki (≥ 1/10.000 do < 1/1.000), zelo redki (< 1/10.000), neznana pogostnost (ni mogoče oceniti iz razpoložljivih podatkov).
Occasionally (frequency unknown; cannot be estimated from available data), treatment with this medicine can cause inflammation of the small intestine or colon.
Včasih (pogostost ni znana; ni je mogoče oceniti na podlagi razpoložljivih podatkov) lahko zdravljenje s tem zdravilom povzroči vnetje tankega ali debelega črevesa.
In cases up to 20 g of trientine base there were no apparent adverse effects reported.
V primerih do 20 g trientinske baze niso poročali o vidnih neželenih učinkih.
There is no antidote.
Protistrupa ni.
This suggests a reduced carcinogenic risk for Wilson's disease patients as a result of trientine therapy.
To kaže na zmanjšano tveganje karcinogenosti za bolnike Wilsonove bolezni kot posledica terapije s trientinom.
Printing ink:
Tiskarsko črnilo:
Moreover, there is evidence to suggest that trientine actually reduces endogenous DNA damage in a strain of rat (Long-Evans Cinnamon) considered to represent an appropriate model of Wilson's disease.
Poleg tega obstajajo dokazi, ki kažejo, da trientin dejansko zmanjšuje endogeno poškodbo DNA v sevu podgan (Long-Evans Cinnamon), za katerega velja, da predstavlja ustrezen model Wilsonove bolezni.
EXP After first opening the bottle can be kept for 3 months.
EXP Po prvem odprtju lahko steklenico shranjujete 3 mesece.
What you need to know before you take Cufence Do not take Cufence:
Kaj morate vedeti, preden boste vzeli zdravilo Cufence Ne jemljite zdravila Cufence:
The safety and efficacy of Cufence in children aged 0 to 5 years have not yet been established.
Otroci, mlajši od 5 let Varnost in učinkovitost zdravila Cufence pri otrocih med 0 in 5 let še nista bili dokazani.
The limited information available suggests that Cufence does not pass into breast milk, but it is not certain that there is no risk to the baby.
Omejene razpoložljive informacije kažejo, da zdravilo Cufence ne prehaja v materino mleko, vendar ni zagotovljeno, da ni tveganja za otroka.
Do not use after the expiry date which is stated on the bottle label and outer carton.
Zdravila ne smete uporabljati po datumu izteka roka uporabnosti, ki je naveden na etiketi steklenice in na zunanji škatli.
Store in a refrigerator (2ºC-8ºC).
Shranjujte v hladilniku (2 do 8 ºC).
The determination of serum free copper, calculated using the difference between the total copper and the ceruloplasmin-bound copper (normal level of free copper in the serum is usually 100 to 150 microgram/L), can be a useful index for monitoring therapy.
Koncentracija prostega bakra v serumu, izračunana na podlagi razlike med skupnim bakrom in bakrom, vezanim na ceruloplazmin (normalna koncentracija prostega bakra v serumu običajno znaša 100 do 150 mikrogramov/l), je lahko uporaben pokazatelj za spremljanje zdravljenja.
The use of appropriate copper parameter target ranges is described in clinical practice guidelines related to Wilson's disease.
Uporaba ustreznih parametrov, kar zadeva ciljne vrednosti bakra, je opisana v smernicah za klinično prakso, povezanih z Wilsonovo boleznijo.
Signs of an allergic reaction include rash, itching, swelling of the face, fainting and breathing problems.
Znaki alergijske reakcije vključujejo izpuščaj, srbenje, otekanje obraza, omedlevico in težave z dihanjem.
If you are taking iron tablets or medicines that neutralise the acid in your stomach, leave at least 2 hours before or after you have taken Cufence because they may reduce Cufence's effect.
Če jemljete tablete železa ali zdravila za nevtralizacijo kisline v želodcu, jih vzemite vsaj 2 uri pred ali po jemanju zdravila Cufence, saj lahko zmanjšajo učinek zdravila Cufence.
Patients who have difficulties swallowing should contact their doctor.
Bolniki, ki imajo težave s požiranjem, naj se obrnejo na zdravnika.
The study will also contain a PK/PD sub-study to evaluate the doseresponse relationship especially during the up-titration phase.
Študija mora vsebovati tudi FK/FD podštudijo za oceno razmerja med odmerkom in odzivom, zlasti v fazi zviševanja odmerka.
These effects were observed only at exposures sufficiently in excess of maximum human exposure to indicate little relevance to clinical use.
Ti učinki so bili opaženi le pri izpostavljenosti, ki presega največjo izpostavljenost pri človeku, kar kaže na majhen pomen za klinično uporabo.
Thus, the pharmacodynamic action of trientine is dependent on its chemical property of chelating copper and not on its interaction with receptors, enzyme systems or any other biological system that might differ between species.
Farmakodinamično delovanje trientina je torej odvisno od njegove kemijske lastnosti keliranja bakra in ne od njegove interakcije z receptorji, encimskimi sistemi ali katerim koli drugim biološkim sistemom, ki bi se lahko razlikoval med vrstami.
No fertility data are available but estrous cyclicity was unaffected and reproductive organs were not identified as target organs in general repeat dose toxicity studies.
Podatki o plodnosti niso na voljo, vendar estrična cikličnost ni bila prizadeta in reproduktivni organi niso bili opredeljeni kot ciljni organi v splošnih študijah toksičnosti pri ponavljajočih odmerkih.
Detailed information on this medicine is available on the European Medicines Agency web site: http://www.ema.europa.eu.There are also links to other websites about rare diseases and treatments.
Podrobne informacije o zdravilu so objavljene na spletni strani Evropske agencije za zdravila http://www.ema.europa.eu, kjer so na voljo tudi povezave do drugih spletnih strani o redkih boleznih in zdravljenju.
There are insufficient data to support the concomitant use of zinc and trientine.
Ni dovolj podatkov, ki bi podpirali sočasno uporabo cinka in trientina.
The aim of maintenance treatment is to maintain free copper levels in plasma (also known as nonceruloplasmin plasma copper) and the urinary copper excretion within the acceptable limits.
Cilj vzdrževalnega zdravljenja je vzdrževanje ravni prostega bakra v plazmi (znan tudi kot neceruloplazminski plazemski baker) ter izločanje bakra z urinom v sprejemljivih mejah.
There are only limited data on concomitant use available and no specific dose recommendations can be made.
Na voljo je le malo podatkov o njuni sočasni uporabi in ni mogoče dati priporočil glede odmerjanja.
All effects were reversible with the exception of the lung findings; however the dose levels where these effects were observed are far in excess of those used clinically.
Vsi učinki so bili reverzibilni, razen sprememb pljuč; vendar pa so odmerki, pri katerih so bili ti učinki opaženi, veliko večji od tistih, ki se uporabljajo klinično.
It is recommended to monitor patients closely for such signs and symptoms and to consider careful titration to reach the recommended therapeutic dose and to reduce dose when necessary.
Priporočljivo je skrbno spremljanje bolnikov glede takšnih znakov in simptomov ter razmisliti o skrbni titraciji, da je dosežen priporočeni terapevtski odmerek, in po potrebi zmanjšati odmerek.
Swallow the capsules whole with a drink of water on an empty stomach, at least 1 hour before or 2 hours after food.
Kapsule pogoltnite cele z vodo na prazen želodec oz. vsaj 1 uro pred ali 2 uri po hrani.
Like with all anti-copper agents, overtreatment carries the risk of copper deficiency, which is especially harmful for children and pregnant women (see section 4.6) since copper is required for proper growth and mental development.
Tako kot pri vseh sredstvih proti bakru predstavlja prekomerno zdravljenje tveganje za pomanjkanje bakra, kar je še posebej škodljivo za otroke in nosečnice (glejte poglavje 4.6), ker je baker potreben za pravilno rast in duševni razvoj.
When trientine doses are adjusted this should be done in small steps.
Odmerke trientina je treba prilagoditi v majhnih korakih.
Trientine is metabolised by acetylation via spermidine/spermine N-acetyltransferase and not via N-acetyltransferase 2.
Trientin se presnavlja z acetilacijo preko spermidin/spermin N-acetiltransferaze in ne preko N-acetiltransferaze 2.
However as trientine is poorly absorbed following oral intake, food may inhibit absorption.
Ker pa se trientin po peroralnem vnosu slabo absorbira, lahko hrana zavira absorpcijo.
Clinical trials with Cufence including a limited number of children in the age range of 5 to 17 years at the start of treatment indicate that frequency, type and severity of adverse reactions in children are expected to be the same as in adults.
Glede na podatke iz kliničnih preskušanja z zdravilom Cufence, vključno z omejenim številom otrok v starostnem razponu od 5 do 17 let na začetku zdravljenja, se pričakuje, da bodo pogostnost, vrsta in resnost neželenih učinkov pri otrocih enake kot pri odraslih.
The recommended dose is 800-1,600 mg (4-8 capsules) daily in 2 to 4 divided doses.
Priporočeni odmerek je med 800 in 1.600 mg (4 do 8 kapsul) na dan, razdeljen v 2 do 4 odmerke.
There is insufficient clinical information available for Cufence to determine whether there exist differences in responses between the elderly and younger patients.
Za zdravilo Cufuence ni na voljo dovolj podatkov za ugotovitev razlik v odzivu med starejšimi in mlajšimi bolniki.
Dose adjustment may be required (see section 4.2).
Morda bo potrebna prilagoditev odmerka (glejte poglavje 4.2).
Your doctor will advise you which treatment and which dose is best in your situation.
Zdravnik vam bo svetoval, katero zdravljenje in kateri odmerek je najboljši za vašo situacijo.
Regular monitoring is especially important at the start of your treatment or when your dose is changed, in growing children and pregnant women to ensure that copper levels are maintained at a suitable level.
Redno spremljanje je še posebej pomembno na začetku zdravljenja ali pri spremembi odmerka pri odraščajočih otrocih in nosečnicah, da je zagotovljeno ohranjanje ustrezne ravni bakra.
If you are pregnant and taking Cufence, you will be monitored throughout your pregnancy for any effects on the baby or changes in your copper levels.
Če ste noseči in jemljete zdravilo Cufence, vas bodo med nosečnostjo spremljali glede morebitnih učinkov na otroka ali spremembe ravni bakra.
It is very important to continue treatment to maintain normal copper levels during pregnancy.
Zelo pomembno je, da se med nosečnostjo nadaljuje zdravljenje, da se ohranijo običajne ravni bakra med nosečnostjo.
If you notice these whilst taking Cufence, you must tell your doctor immediately.
Če opazite te težave med jemanjem zdravila Cufence, morate o tem nemudoma obvestiti zdravnika.
When switching a patient from another trientine formulation, caution is advised because different trientine salts are available which may have a different trientine content (base) and a different bioavailability.
Kadar bolnik preide z zdravljenja z drugo obliko trientina, se priporoča previdnost, ker so na voljo različne trientinijeve soli, ki imajo lahko drugačno vsebnost trientina (baza) in drugačno biološko uporabnost.
Factors to consider include the known risks associated with untreated or undertreated Wilson's disease, risks associated with the stage of disease, the risk of those alternative treatments which are available and the possible effects of trientine (see section 5.3).
Dejavniki, ki jih je treba upoštevati, vključujejo znana tveganja, povezana z nezdravljeno ali neustrezno zdravljeno Wilsonovo boleznijo, tveganja, povezana s stopnjo bolezni, tveganja drugih možnosti zdravljenja, ki so na voljo, in možne učinke trientina (glejte poglavje 5.3).
Your doctor will decide which treatment and which dose is best in your situation.
Zdravnik se bo odločil, katero zdravljenje in kateri odmerek je najboljši za vašo situacijo.
The trientine dose may also be reduced in case of side effects of trientine, such as gastrointestinal complaints and haematological changes.
Odmerek trientina je lahko zmanjšan tudi v primeru neželenih učinkov trientina, kot so gastrointestinalne težave ter hematološke spremembe.
Some positive in vitro mutagenicity data were obtained but in vivo test systems showed no mutagenic activity.
Pridobljeni so bili nekateri pozitivni podatki o in vitro mutagenosti, vendar testni sistemi in vivo niso pokazali mutagenega delovanja.
Trientine doses should be reduced to a more tolerable dose and may be increased again, once side effects have been resolved.
Odmerke trientina je treba zmanjšati na sprejemljivejši odmerek in ga je mogoče znova povečati, ko neželeni učinki izginejo.
Fertility It is unknown whether trientine has an effect on human fertility.
Ni znano, ali ima trientin učinke na plodnost pri človeku.
Pharmacotherapeutic group: Other alimentary tract and metabolism, various alimentary tract and metabolism products, ATC code: A16AX12
Farmakoterapevtska skupina: Druga zdravila za bolezni prebavil in presnove, razna zdravila za bolezni prebavil in presnove, oznaka ATC: A16AX12
The extent of MAT and DAT's contribution to the overall effect of Cufence on copper levels in Wilson's Disease patients remains to be determined.
Obseg prispevka MAT in DAT k skupnemu učinku zdravila Cufence na ravni bakra pri bolnikih z Wilsonovo boleznijo je treba še določiti.
Avoid eating or drinking for 1 hour before, or 2 hours after taking Cufence.
Izogibajte se hrani ali pijači 1 uro pred ali 2 uri po zaužitju zdravila Cufence.
Chronic overtreatment can lead to copper deficiency and reversible sideroblastic anaemia.
Kronično prekomerno zdravljenje lahko privede do pomanjkanja bakra in reverzibilne straroblastične anemije.
Blood and lymphatic system disorders:
Bolezni krvi in limfnega sistema:
It is advised, however, to monitor patients presenting with hepatic symptoms every two to three weeks after initiation of treatment with Cufence.
Kljub temu je priporočljivo spremljati bolnike s simptomi okvare jeter vsaka dva do tri tedne po začetku zdravljenja z zdravilom Cufence.
It is important that Cufence is given on an empty stomach, at least one hour before meals or two hours after meals, and at least one hour apart from any other medicinal product, food or milk (see section 4.5).
Pomembno je, da se zdravilo Cufence jemlje na prazen želodec vsaj eno uro pred obrokom ali dve uri po njem in v razmiku vsaj ene ure od jemanja drugega zdravila, hrane ali mleka (glejte poglavje 4.5).
There is limited clinical data suggesting that trientine is not excreted in breast milk.
Omejeni klinični podatki kažejo, da se trientin ne izloča v materino mleko.
This leaflet was last revised in <{MM/YYYY}><{month YYYY}>.
Navodilo je bilo nazadnje revidirano dne <{MM/LLLL}><{mesec LLLL}> Drugi viri informacij
The recommended doses of Cufence are expressed as mg of trientine base (i.e. not in mg of the trientine dihydrochloride salt) (see section 4.4).
Priporočeni odmerki zdravila Cufence so izraženi v mg baze trientina (ne pa v mg soli trientinijevega diklorida) (glejte poglavje 4.4).
Nervous system problems can occur (for example, shaking, lack of coordination, slurred speech, muscle stiffness and worsening of muscle spasms), especially in patients just starting treatment with Cufence.
Pojavijo se lahko težave z živčevjem (npr. tresenje, pomanjkanje koordinacije, nerazumljiv govor, togost mišic in poslabšanje mišičnih krčev), zlasti pri bolnikih, ki šele začenjajo zdravljenje z zdravilom Cufence.
The main toxicological findings associated with trientine, which were generally seen across all species examined, included body weight loss or lower body weight gain, altered urinary electrolytes, low plasma copper levels and various histopathological changes in the lungs (mainly interstitial pneumonitis).
Glavne toksikološke ugotovitve, povezane s trientinom, ki so bile običajno opažene pri vseh preučevanih vrstah, so bile izguba telesne mase ali zmanjšaoe pridobivanje telesne mase, spremenjeni elektroliti v urinu, nizke ravni bakra v plazmi in različne histopatološke spremembe v pljučih (predvsem intersticijski pnevmonitis).
No specific food interaction studies in humans have been performed with trientine.
Specifičnih študij medsebojnega delovanja s hrano pri ljudeh s trientinom niso izvedli.
The combination of trientine with zinc is not recommended.
Kombiniranje trientina s cinkom se ne priporoča.
Pregnancy There is a limited amount of data from the use of trientine in pregnant women.
Nosečnost Podatki o uporabi trientina pri nosečnicah so omejeni.
A large overdose of 40 g of trientine base resulted in selflimiting dizziness and vomiting with no other clinical sequelae or significant biochemical abnormalities reported.
Veliko preveliko odmerjanje 40 g baze trientina je povzročilo samoomejevalno omotico in bruhanje, brez kakršnih koli drugih kliničnih sekula ali pomembnih biokemijskih nepravilnosti.
Since chelation therapy leads to an increase in urinary copper levels, this may/will not give an accurate reflection of the excess copper load in the body but may be a useful measure of treatment compliance.
Ker kelacijska terapija poveča izločanje bakra v urinu, tak način morda ne odraža/ne bo natančno pokazal presežka bakra v telesu, je pa lahko uporaben za preverjanje, ali se bolnik drži zdravljenja.
Other medicines and Cufence
Druga zdravila in zdravilo Cufence
The principal mechanism of action of trientine requires its systemic exposure (see section 5.1) so it is recommended that trientine be taken at least 1 hour before meals or 2 hours after meals and at least one hour apart from any other medicinal product, food, or milk to allow for maximum absorption and reduce the likelihood of the formation of complexes by metal binding in the gastrointestinal tract (see section 4.2).
Glavni mehanizem delovanja trientina zahteva njegovo sistemsko izpostavljenost (glejte poglavje 5.1), zato je priporočljivo, da bolnik trientin vzame vsaj 1 uro pred obrokom ali 2 uri po obroku in vsaj z enournimi presledki pri jemanju katerega koli drugega zdravila, hrane ali mleka, da je omogočena maksimalna absorpcija in zmanjšana verjetnost nastanka kompleksov z vezavo kovin v prebavnem traktu (glejte poglavje 4.2).
MedDRA- system organ class database
Razvrstitev po organskih sistemih glede na podatkovno zbirko MedDRA
Studies in animals have shown reproductive toxicity, which was probably a result of trientine-induced copper deficiency (see section 5.3).
Študije na živalih so pokazale škodljiv vpliv na sposobnost razmnoževanja, ki je bila verjetno posledica s trientinom povzročenega pomanjkanja bakra (glejte poglavje 5.3).