natgillin/translations-raw huggingface dataset:
What measures are being taken to ensure the safe and effective use of Orphacol?
Kateri ukrepi se izvajajo za zagotovitev varne in učinkovite uporabe zdravila Orphacol?
Orphacol is available as capsules and should be taken every day at approximately the same time, with a meal.
Zdravilo Orphacol je na voljo v obliki kapsul in ga je treba jemati vsak dan z obrokom ob približno istem času.
Because cholic acid is a well-known substance, and its use in these enzyme deficiencies is well established, the applicant presented data from the scientific literature.
Ker je holna kislina dobro znana snov in je njena uporaba pri pomanjkanju tega encima dobro uveljavljena, je predlagatelj predstavil podatke iz znanstvene literature.
The daily dose is between 5 and 15 mg per kg body weight, adjusted for each patient according to the state of their bile acid, with a minimum daily dose of 50 mg and a maximum of
Dnevni odmerek je med 5 in 15 mg na kg telesne mase, ki se pri posameznem bolniku prilagodi glede na stanje žolčne kisline, pri čemer je najmanjši dnevni odmerek 50 mg, največji pa 500 mg.
It is used to treat adults and children from one month of age who have a genetic abnormality that makes them unable to produce bile.
Uporablja se za zdravljenje odraslih in otrok, starejših od enega meseca, z genetsko okvaro, zaradi katere niso zmožni tvoriti žolča.
A risk management plan has been developed to ensure that Orphacol is used as safely as possible.
Za zagotovitev čim varnejše uporabe zdravila Orphacol je bil pripravljen načrt obvladovanja tveganj.
Treatment should be stopped if liver function does not improve within three months.
Če se delovanje jeter ne izboljša v treh mesecih, je treba zdravljenje prekiniti.
It decided that based on the evidence from the scientific literature Orphacol's benefits are greater than its risks and recommended that it be given marketing authorisation.
Na podlagi dokazov iz znanstvene literature je zaključil, da so koristi zdravila Orphacol večje od z njim povezanih tveganj, in priporočil, da se zanj odobri dovoljenje za promet.
The summary of the opinion of the Committee for Orphan Medicinal Products for Orphacol can be found on the Agency's website: ema.europa.eu/Find medicine/Human medicines/Rare disease designation.
Povzetek mnenja Odbora za zdravila sirote o zdravilu Orphacol je na voljo na spletni strani agencije: ema.europa.eu/Find medicine/Human medicines/Rare disease designation.
Orphacol cholic acid
Orphacol holna kislina
Other information about Orphacol
Druge informacije o zdravilu Orphacol
The Committee for Medicinal Products for Human Use (CHMP) noted that the use of cholic acid to treat inborn errors in primary bile acid synthesis was well established in medical practice and documented in the scientific literature, although the number of documented cases was low due to the rarity of the condition.
Odbor za zdravila za uporabo v humani medicini (CHMP) je upošteval, da je uporaba holne kisline za zdravljenje dednih napak v sintezi primarnih žolčnih kislin dobro uveljavljena v zdravstveni praksi in dokumentirana v znanstveni literaturi, čeprav je število dokumentiranih primerov majhno zaradi redkosti bolezni.
500 mg. For small children who cannot swallow capsules, the contents can be mixed in with infant formula or juice.
Pri majhnih otrocih, ki ne morejo požirati kapsul, se lahko vsebina zmeša z začetno formulo za dojenčke ali sokom.
Because the number of patients with inborn errors in primary bile acid synthesis is low, the condition is considered ‘rare', and Orphacol was designated an ‘orphan medicine' (a medicine used in rare diseases) on 18 December 2002.
Ker je bolnikov z dednimi napakami v sintezi primarnih žolčnih kislin malo, velja ta bolezen za redko, zato je bilo zdravilo Orphacol 18. decembra 2002 določeno kot „zdravilo sirota“ (zdravilo za zdravljenje redkih bolezni).
The company that makes Orphacol will also provide doctors in all Member States who will use Orphacol with a pack containing product literature and information regarding the correct diagnosis of these disease conditions, the risks of side effects and how to use the medicine properly.
Družba, ki izdeluje zdravilo Orphacol, bo zdravnikom v vseh državah članicah, ki uporabljajo zdravilo Orphacol, prav tako priskrbela literaturo o zdravilu in informacije o pravilnem diagnosticiranju teh bolezni, tveganjih za neželene učinke ter pravilni uporabi zdravila.
What are the risks associated with Orphacol?
Kakšna tveganja so povezana z zdravilom Orphacol?
Cholic acid is the main primary bile acid produced by the liver.
Holna kislina je glavna primarna žolčna kislina, ki jo tvorijo jetra.
The cholic acid contained in Orphacol replaces the patient's missing cholic acid.
Holna kislina v zdravilu Orphacol nadomesti manjkajočo holno kislino pri bolniku.
The side effects seen with Orphacol were diarrhoea, pruritus (itching), increases in transaminases (liver enzymes) and possibly gallstones, although their frequency could not be reliably estimated from the available limited data.
Neželeni učinki, ki so jih opazili pri uporabi zdravila Orphacol, so bili driska, pruritus (srbenje), povišane ravni transaminaz (jetrnih encimov) in morebitni žolčni kamni, čeprav njihove pogostnosti ni bilo mogoče zanesljivo oceniti na podlagi razpoložljivih omejenih podatkov.
What benefits of Orphacol have been shown in studies?
Kakšne koristi je zdravilo Orphacol izkazalo v študijah?
What is Orphacol and what is it used for?
Kaj je zdravilo Orphacol in za kaj se uporablja?
It is not intended to provide practical advice on how to use Orphacol.
Povzetek ni namenjen zagotavljanju praktičnih nasvetov o njegovi uporabi.
Orphacol is a medicine containing cholic acid, a substance found in the bile which is used to digest fats.
Orphacol je zdravilo, ki vsebuje holno kislino, tj. snov, ki jo najdemo v žolču in se uporablja za presnavljanje maščob.
The applicant presented data on 49 patients with inborn errors in primary bile acid synthesis, taken from the scientific literature (38 patients with 3β-Hydroxy-Δ5-C27-steroid oxidoreductase deficiency and 11 patients with Δ4-3-Oxosteroid-5β-reductase deficiency).
Predstavil je tudi podatke 49 bolnikov z dednimi napakami v sintezi primarnih žolčnih kislin iz znanstvene literature (38 bolnikov s pomanjkanjem 3β-hidroksi-Δ5-C27-steroid-oksidoreduktaze in 11 bolnikov s pomanjkanjem Δ4-3oksosteroid-5β-reduktaze).
It compared the outcomes of 28 patients who received cholic acid with others who were given different bile acids or did not receive bile acid treatment.
Izide 28 bolnikov, ki so prejeli holno kislino, je primerjal z drugimi bolniki, ki so prejeli druge žolčne kisline ali niso prejeli zdravljenja z žolčno kislino.
The full EPAR for Orphacol can be found on the Agency's website: ema.europa.eu/Find medicine/Human medicines/European Public Assessment Reports.
Celotno evropsko javno poročilo o oceni zdravila (EPAR) za zdravilo Orphacol je na voljo na spletni strani agencije: ema.europa.eu/Find medicine/Human medicines/European Public Assessment Reports.
Orphacol should not be used in people who may be hypersensitive (allergic) to cholic acid or any of the other ingredients.
Zdravila Orphacol ne smejo uporabljati osebe, ki so preobčutljive za (alergične na) holno kislino ali katero koli drugo sestavino zdravila.
For more information about treatment with Orphacol, read the package leaflet (also part of the EPAR) or contact your doctor or pharmacist.
Za več informacij o zdravljenju z zdravilom Orphacol preberite navodilo za uporabo (ki je prav tako del EPAR) ali se posvetujte z zdravnikom ali farmacevtom.
Orphacol is used in patients who do not have enough of two specific liver enzymes (3β-Hydroxy-Δ5-C27-steroid oxidoreductase or Δ4-3-Oxosteroid-5β-reductase).
Zdravilo Orphacol se uporablja pri bolnikih s pomanjkanjem dveh specifičnih jetrnih encimov (3β-hidroksi-Δ5-C27-steroid-oksidoreduktaze ali Δ4-3-oksosteroid-5βreduktaze).
Why is Orphacol approved?
Zakaj je bilo zdravilo Orphacol odobreno?
Based on this plan, safety information has been included in the summary of product characteristics and the package leaflet for Orphacol, including the appropriate precautions to be followed by healthcare professionals and patients.
V skladu s tem načrtom so bile v povzetek glavnih značilnosti zdravila in navodilo za uporabo za zdravilo Orphacol vključene informacije o varnosti, vključno s previdnostnimi ukrepi, ki jih morajo upoštevati zdravstveni delavci in bolniki.
Since Orphacol has been approved under exceptional circumstances, the company that markets Orphacol will establish a database of patients treated with Orphacol to monitor the safety and effectiveness of the treatment, and will submit the results to the CHMP at regular, specified intervals.
Ker je zdravilo Orphacol pridobilo dovoljenje za promet v izjemnih okoliščinah, bo družba, ki ga trži, vzpostavila podatkovno zbirko bolnikov, ki se zdravijo z njim, s katero se bosta spremljali varnost ter učinkovitost zdravljenja, rezultate pa bo predložila odboru CHMP v rednih, vnaprej določenih obdobjih.
What information is still awaited for Orphacol?
Katere informacije o zdravilu Orphacol še pričakujemo?
This makes their liver unable to produce enough of the main components of bile, called primary bile acids, such as cholic acid.
Zaradi tega pomanjkanja jetra niso zmožna tvoriti dovolj glavnih sestavin žolča, imenovanih primarne žolčne kisline, kot je holna kislina.
This helps to decrease the production of abnormal bile acids and contributes to the normal activity of bile in the digestive system, thereby relieving the symptoms of the condition.
To pomaga zmanjšati tvorbo nenormalnih žolčnih kislin in prispeva k normalnemu delovanju žolča v prebavilih ter s tem lajša simptome te bolezni.
In the scientific literature, cholic acid treatment was shown to reduce the amount of abnormal bile acids in patients, to restore the normal functions of the liver and to help delay or prevent the need for a liver transplant.
V znanstveni literaturi so pokazali, da zdravljenje s holno kislino zmanjša količino nenormalnih žolčnih kislin pri bolnikih, kar vzpostavi normalno delovanje jeter in pomaga odložiti ali preprečiti potrebo po presaditvi jeter.
When these primary bile acids are lacking, the body produces abnormal bile acids instead which can damage the liver, potentially leading to life-threatening liver failure.
Pri pomanjkanju teh primarnih žolčnih kislin telo tvori nenormalne žolčne kisline, ki lahko poškodujejo jetra in povzročijo tudi smrtno nevarno odpoved jeter.
For practical information about using Orphacol, patients should read the package leaflet or contact their doctor or pharmacist.
Za praktične informacije o uporabi zdravila Orphacol naj bolniki preberejo navodilo za uporabo ali se posvetujejo z zdravnikom ali farmacevtom.
The medicine can only be obtained with a prescription and treatment with Orphacol should be started and supervised by a doctor specialised in liver disease.
Zdravljenje z zdravilom Orphacol mora uvesti in nadzirati zdravnik, specializiran za jetrne bolezni.
How does Orphacol work?
Kako zdravilo Orphacol deluje?
How is Orphacol used?
Kako se zdravilo Orphacol uporablja?
The effects last for at least 12 hours.
Ti učinki trajajo vsaj 12 ur.
Relative Risk (95% confidence intervals)
Relativno tveganje (95-odstotni intervali zaupanja)
This could make your breathing worse.
To lahko poslabša dihanje.
It is not known if this was due to pharmacogenetic or other factors.
Ni znano, ali je to posledica farmakogenetike ali drugih dejavnikov.
This study was not designed to investigate the effect on exacerbations.
Ta študija ni bila zasnovana za raziskovanje učinka na poslabšanja.
Breathe in through your mouth as deeply and as forcefully as you can.
Vdihnite skozi usta tako globoko in močno kot lahko.
• Tuberculosis (TB) now or have had in the past, or have other lung infections
• tuberkulozo (TB) sedaj ali ste jo imeli v preteklosti, ali če imate drugo okužbo pljuč
A 4-unit decrease is considered clinically relevant.
Zmanjšanje za 4 enote velja za klinično pomembno.
There are no data in humans.
Podatkov pri ljudeh ni.
Do not bite the mouthpiece.
Ustnika ne grizite.
Frequencies were derived from clinical trial data.
Pogostnosti izvirajo iz podatkov kliničnih preskušanj.
A risk to breastfed newborns/infants cannot be excluded.
Tveganja za dojenega novorojenca/dojenčka ne moremo izključiti.
It is not known whether this medicine can pass into breast milk.
Ni znano, ali lahko to zdravilo prehaja v materino mleko.
These side effects may include any of the following:
Ti neželeni učinki lahko vključujejo kar koli od naslednjega:
You may notice your heart beating faster than usual and that you feel shaky.
Opazili bi lahko, da vaše srce bije hitreje kot običajno in da drhtite.
Treatment was stepped up every 12 weeks until **total control was achieved or the highest dose of study drug was reached.
Zdravljenje so vsakih 12 tednov titrirali navzgor, dokler ni bila dosežena **popolna nadzorovanost ali pa največji odmerek proučevanega zdravila.
The secondary endpoints in the table above reached statistical significance in the whole population.)
Sekundarni opazovani dogodki, navedeni v zgornji tabeli, so v celotni populaciji dosegli statistično značilnost.)
• Headache - this usually gets better as treatment continues.
• glavobol - običajno se izboljša z nadaljevanjem zdravljenja;
(For Poland only) Teva Operations Poland Sp. z o.o.
(Samo za Poljsko) Teva Operations Poland Sp. z o.o.
Mogilska 80 Str. 31-546 Kraków, Poland
Mogilska 80 Str. 31-546 Kraków, Poljska
Number of primary endpoint events /number of patients
Število sekundarnih opazovanih dogodkov/število bolnikov
After opening the foil wrap: 3 months.
Po odprtju ovoja iz folije: 3 mesece.
Lactose is a type of sugar found in milk.
Laktoza je vrsta sladkorja v mleku.
However, these animal experimental results do not seem to be relevant for man given recommended doses.
Vendar kaže, da ti eksperimentalni izsledki pri živalih niso pomembni za ljudi pri uporabi priporočenih odmerkov.
Keep taking it until your doctor tells you to stop.
Nadaljujte z uporabo, dokler vam zdravnik ne naroči, da prenehate.
Survival status at 3 years was determined for all patients regardless of withdrawal from study medication.
Status preživetja po 3 letih je bil določen za vse bolnike, ne glede na to, če so študijsko zdravilo morda prenehali jemati.
Hold your breath for 10 seconds or as long as you comfortably can.
Dih zadržite 10 sekund ali dokler ga lahko brez težav zadržite.
Do not take more than the recommended dose.
Ne uporabljajte večjega odmerka od priporočenega.
If this happens you need to use a fast-acting ‘reliever' (‘rescue') inhaler, such as salbutamol.
Če se to zgodi, morate uporabiti hitrodelujoče "olajševalno" ("rešilno") zdravilo, kot je salbutamol.
To prevent these symptoms, your doctor may prescribe extra corticosteroids in tablet form (such as prednisolone).
Za preprečevanje teh simptomov vam lahko zdravnik predpiše dodatne kortikosteroide v obliki tablet (kot je prednizolon).
Non-significant p value after adjustment for 2 interim analyses on the primary efficacy comparison from a log-rank analysis stratified by smoking status
1. p-vrednost je neznačilna po prilagoditvi za 2 vmesni analizi za primerjavo primarne učinkovitosti iz analize log-rank, stratificirane glede na kadilski status
The respective mechanisms of action of both active substances are discussed below.
Mehanizma delovanja obeh zdravilnih učinkovin sta obravnavana spodaj.
If your asthma or breathing gets worse tell your doctor straight away.
Če se vaša astma ali dihanje poslabša, takoj obiščite zdravnika.
This includes other medicines for asthma or any medicines obtained without a prescription.
To vključuje druga zdravila za astmo, pa tudi vsa zdravila, ki so na voljo brez recepta.
For pharmacokinetic purposes each component can be considered separately.
S stališča farmakokinetike je mogoče farmakokinetiko vsake sestavine obravnavati posebej.
This training is important to ensure you receive the dose you require.
Ta vadba je zelo pomembna, saj zagotovi, da je odmerek, ki ga potrebujete, pravilen.
Caution is recommended and long-term treatment with such drugs should if possible be avoided.
Potrebna je previdnost; če je možno, se je treba dolgotrajnemu zdravljenju s takšnimi zdravili izogniti.
Fluticasone propionate is cleared very rapidly from the systemic circulation.
Flutikazonpropionat se zelo hitro očisti iz sistemskega obtoka.
Consideration should be given to referring the child or adolescent to a paediatric respiratory specialist.
Treba je razmisliti o tem, da bi otroka ali mladostnika napotili k pediatričnemu specialistu za dihalne poti.
• Tiredness and loss of appetite, feeling sick
• utrujenost in izgubo apetita, občutek siljenja na bruhanje
See section 4.4: “Adrenal function”.
Glejte poglavje 4.4: "Delovanje nadledvičnih žlez".
After 12 weeks no children in either treatment arm had abnormally low 24-hour urinary cortisol excretion.
Po 12 tednih nihče od otrok v kateri koli skupini zdravljenja po 24 urah ni imel neobičajno nizkega očistka kortizola.
• Low levels of sugar in your blood
• nizko raven sladkorja v krvi
• For inhalations remaining from 20 downwards to ‘8', ‘6', ‘4', ‘2' the numbers are displayed in red on a white background.
• Za inhalacije, ki so preostale, so števila od 20 do 8, 6, 4, 2 napisana z rdečo pisavo na beli podlagi.
Tell your doctor or pharmacist if you are taking or, have recently taken or might use any other medicines.
Obvestite zdravnika ali farmacevta, če jemljete, ste pred kratkim jemali ali pa boste morda začeli jemati katero koli drugo zdravilo.
Place the mouthpiece between your teeth.
Ustnik namestite med zobe.
• Salmeterol is a long-acting bronchodilator.
• Salmeterol je dolgodelujoči bronhodilatator.
Additionally, hypokalaemia can occur and therefore serum potassium levels should be monitored.
Pojavi se lahko tudi hipokaliemija, zato je treba spremljati ravni kalija v serumu.
Your doctor, nurse or pharmacist should also check how you use your Spiromax device from time to time to ensure that you are using the device properly and as prescribed.
Zdravnik, medicinska sestra ali farmacevt morajo od časa do časa tudi preveriti, kako uporabljate svoj pripomoček Spiromax, da se prepričajo, da ga uporabljate pravilno in kot je predpisano.
However, it should be noted that children and adolescents less than 16 years taking high doses of fluticasone propionate (typically ≥ 1000 micrograms/day) may be at particular risk.
Vendar je treba opozoriti, da je tveganje pri otrocih in mladostnikih, mlajših od 16 let, ki uporabljajo velike odmerke flutikazonpropionata (običajno ≥ 1.000 mikrogramov/dan) posebno veliko.
• Check the dose indicator to see that there are 60 inhalations in the inhaler.
• Preverite kazalnik odmerkov, da preverite, če je v inhalatorju 60 inhalacij.
Therefore these patients should be treated with special care and adrenocortical function regularly monitored.
Zato je treba te bolnike obravnavati s posebno pozornostjo in redno spremljati adrenokortikalno delovanje.
• Aerivio Spiromax may affect the normal production of steroid hormones in the body, particularly if you have taken high doses for long periods of time.
• Zdravilo Aerivio Spiromax lahko vpliva na normalno nastajanje steroidnih hormonov v telesu, zlasti če dalj časa jemljete visoke odmerke.
Beta adrenergic blockers may weaken or antagonise the effect of salmeterol.
Antagonisti adrenergičnih receptorjev beta lahko oslabijo ali izničijo učinek salmeterola.
Ritonavir can greatly increase the concentration of fluticasone propionate in plasma.
Ritonavir lahko močno zviša koncentracijo flutikazonipropionata v plazmi.
• Other bronchodilators (such as salbutamol).
• Drugi bronhodilatatorji (kot je salbutamol).
If you are not using Aerivio Spiromax properly and/or you are not breathing in forcefully enough, this may mean that you are not getting enough of the medicine into your lungs.
Če zdravila Aerivio Spiromax ne uporabljate pravilno in/ali če ne vdihujete dovolj močno, lahko to pomeni, da ne dobite dovolj zdravila v pljuča.
Some of these medicines may increase the amount of salmeterol or fluticasone propionate in your body.
Nekatera od teh zdravil lahko povečajo količino salmeterola ali flutikazonpropionata v telesu.
• Diuretics, also known as ‘water tablets' used to treat high blood pressure.
• Diuretiki, znani tudi kot "tablete za odvajanje vode", ki se uporabljajo za zdravljenje visokega
Lactose contains small amounts of milk protein which may cause allergic reactions.
Laktoza vsebuje majhne količine mlečnih beljakovin, ki lahko povzročijo alergijske reakcije.
Front panel: Not for use in children and adolescents.
Sprednji del: Ni za uporabo pri otrocih in mladostnikih.
• The dose indicator on the rear of the device tells you how many doses (inhalations) are left in your inhaler, starting with 60 inhalations when it is full.
• Kazalnik odmerkov na hrbtni strani pripomočka vam pove, koliko odmerkov (inhalacij) je še ostalo v inhalatorju, začenši s 60 inhalacijami, ko je poln.
Some medicines may increase the effects of Aerivio Spiromax and your doctor may wish to monitor you carefully if you are taking these medicines (including some medicines for HIV: ritonavir, cobicistat).
Nekatera zdravila lahko povečajo učinek zdravila Aerivio Spiromax in zdravnik vas bo morda želel skrbno spremljati, če jemljeta ta zdravila (vključno z nekaterimi zdravili za HIV: ritonavirjem,
There have been very rare reports of increases in blood glucose levels (see section 4.8) and this should be considered when prescribing to patients with a history of diabetes mellitus.
Zelo redko so poročali o zvišanih vrednostih glukoze v krvi (glejte poglavje 4.8), kar je treba upoštevati pri predpisovanju zdravila bolnikom, ki imajo v anamnezi sladkorno bolezen.
Frequency not known, but may also occur:
Neželeni učinki, katerih pogostnost ni znana, vendar se lahko pojavijo:
It should not be used to treat acute asthma symptoms for which a fast- and short-acting bronchodilator is required.
Ne sme se ga uporabljati za zdravljenje akutnih simptomov astme, za katere je treba uporabiti hitro- in kratkodelujoči bronhodilatator.
Bronchodilators help the airways in the lungs to stay open.
Bronhodilatatorji pomagajo, da ostanejo dihalne poti v pljučih odprte.
Health Related Quality of Life, as measured by the St George's Respiratory Questionnaire (SGRQ) was improved by all active treatments in comparison with placebo.
Kakovost življenja, povezana z zdravjem, izmerjena po vprašalniku St. George Respiratory Questionnaire (SGRQ), se je v primerjavi s placebom izboljšala pri vseh zdravljenjih z zdravilno učinkovino.
Note that it is important that you breathe in forcefully.
Pomembno je, da vdihnete silovito.
• Heart disease, including an irregular or fast heart beat
• bolezen srca, vključno z nerednim ali hitrim srčnim utripom
Animal studies with salmeterol have shown embryofetal toxicity only at high exposure levels.
Študije pri živalih s salmeterolom so pokazale embriofetalne toksične učinke le pri visoki izpostavljenosti.
The renal clearance of fluticasone propionate is negligible.
Ledvični očistek flutikazonpropionata je zanemarljiv.
Remove your inhaler from your mouth.
Inhalator vzemite iz ust.
Both non-selective and selective β blockers should be avoided unless there are compelling reasons for their use.
Izogniti se je treba neselektivnim in selektivnim antagonistom β, razen če je njihova uporaba nujna.
Concomitant use of other β adrenergic-containing medicinal products can have a potentially additive effect.
Sočasna uporaba drugih zdravil, ki vsebujejo β adrenergike, ima lahko aditiven učinek.
1/10), uncommon (1/1000 to 1/100), rare (1/10,000 to <1/1000) and not known (frequency cannot be estimated from the available data).
Pogostnosti so opredeljene 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), neznana pogostnost (ni mogoče oceniti iz razpoložljivih podatkov).
However, do not change the number of inhalations your doctor has prescribed without talking to your doctor first.
Vendar pa števila inhalacij, ki vam ga je predpisal zdravnik, ne spreminjajte brez predhodnega posveta z