FrancophonIA/ELRC3.0 huggingface dataset:
English: Treatment with cholic acid in patients with pre-existing hepatic impairment should be given under close monitoring and, for all patients should be stopped in case of abnormal hepatocellular function, as measured by prothrombin time, if hepatocellular function does not improve within 3 months of the initiation of cholic acid treatment.
Slovenian: Zdravljenje s holno kislino pri bolnikih z obstoječo jetrno okvaro mora potekati pod skrbnim nadzorom, pri vseh bolnikih pa je treba zdravljenje prekiniti pri nenormalni hepatocelularni funkciji, ki se izmeri s protrombinskim časom, če se ne izboljša v treh mesecih po uvedbi zdravljenja s holno kislino.
English: Phenobarbital has been shown to increase the pool size and turnover of cholic acid and therefore has an antagonistic effect to the desired action of cholic acid in patients.
Slovenian: Pokazali so, da fenobarbital poveča velikost zaloge in premeno holne kisline, zato ima antagonistični učinek na želeno delovanje holne kisline pri bolnikih.
English: Adverse reactions are ranked according to system organ class, using the following convention: very common (≥ 1/10), common (≥1/100 to <1/10), uncommon (≥1/1,000 to <1/100), rare (≥1/10,000 to
Slovenian: pogosti (≥ 1/100 do < 1/10), občasni (≥ 1/1000 do < 1/100), redki (≥ 1/10.000 do < 1/1000), zelo redki
English: If your doctor considers it necessary that you keep taking ciclosporin, he will closely monitor the levels of bile acids in your blood and urine and adjust the dose of cholic acid accordingly.
Slovenian: Če zdravnik meni, da je nujno, da še naprej jemljete ciklosporin, bo skrbno spremljal ravni žolčnih kislin v krvi ter urinu in ustrezno prilagodil odmerek holne kisline.
English: It is recommended that the capsules are taken with food, as this may help to make the cholic acid more effective, and also reduces the likelyhood of diarrhoea.
Slovenian: Priporočljivo je, da kapsule vzamete skupaj s hrano, saj lahko to pomaga pri učinkovitejšem delovanju holne kisline in zmanjša verjetnost driske.
English: Cholic acid is not mutagenic; however, co-administration of cholic acid with known carcinogens has shown increased tumour formation compared to the known carcinogen alone.
Slovenian: Holna kislina ni mutagena, vendar so pokazali, da sočasna uporaba holne kisline z znanimi kancerogeni poveča tvorbo tumorjev v primerjavi z uporabo samega kancerogena.
English: INFORMATION IN BRAILLE Kolbam 50 mg
Slovenian: PODATKI V BRAILLOVI PISAVI Kolbam 50 mg
English: Transient elevations of transaminases at the initiation of cholic acid treatment have been observed and do not indicate the need for a dose reduction if Gamma GT is not elevated and if serum bile acid levels are falling or in the normal range.
Slovenian: Opazili so prehodno povečanje vrednosti transaminaz med uvajanjem zdravljenja s holno kislino, kar pa ne pomeni, da je treba zmanjšati odmerek, če ravni GGT niso zvišane in če ravni žolčnih kislin v serumu padajo oziroma so v normalnem razponu.
English: Among the subgroup of patients with CTX (n=8), 3 transitioned from CAC-91-10-10 and were on cholic acid treatment at study start.
Slovenian: V podskupini bolnikov s CTX (n = 8) so 3 prešli s CAC-91-10-10 in so se na začetku študije zdravili s holno kislino.
English: In the body cholic acid is naturally produced in the liver and is part of the bile, a fluid that helps digestion and absorbs fat and vitamins from the food.
Slovenian: V telesu holno kislino naravno tvorijo jetra in je del žolča, tekočine, ki pomaga pri prebavi in absorbira maščobe ter vitamine iz hrane.
English: Medicines for lowering cholesterol levels in the blood, such as cholestyramine, colestipol or colesevelam, and certain antacids which contain aluminium (e.g. indigestion relief products) may affect the absorption of cholic acid.
Slovenian: Zdravila za zniževanje ravni holesterola v krvi, kot so holestiramin, holestipol ali holesevelam, in nekateri antacidi, ki vsebujejo aluminij (npr. zdravila za lajšanje zgage), lahko vplivajo na absorpcijo holne kisline.
English: A total of 53 patients took part in the clinical study and received at least one dose of cholic acid; 22 (42%) were treatment naive, i.e., received their first dose of cholic acid during study CAC002-01.
Slovenian: V klinični študiji je sodelovalo skupno 53 bolnikov, ki so prejeli vsaj en odmerek holne kisline; 22 (42 %) je bilo predhodno nezdravljenih, tj. med študijo CAC-002-01 so prejeli svoj prvi odmerek holne kisline.
English: In repeated dose studies, frequently reported effects of cholic acid have included decreased body weight, diarrhoea and liver damage with elevated transaminases although are considered to be associated with the pharmacological effects of bile acid metabolism.
Slovenian: V študijah z večkratnimi odmerki so pogosti učinki holne kisline vključevali zmanjšano telesno maso, diarejo in jetrno okvaro s povišanimi ravnmi transaminaz, čeprav menijo, da so ti povezani s farmakološkimi učinki presnove žolčnih kislin.
English: Concurrent elevation of serum gamma glutamyltransferase (Gamma GT), alanine aminotransferase (ALT) and/or serum bile acids above normal levels may indicate overdose.
Slovenian: Sočasno zvišanje serumskih vrednosti gama-glutamiltransferaze (GGT), alanin-aminotransferaze (ALT) in/ali serumskih vrednosti žolčnih kislin nad normalne ravni lahko kaže na preveliko odmerjanje.
English: • CYP7A1 deficiency (n=1)
Slovenian: • pomanjkanje CYP7A1 (n = 1).
English: In growing infants, treatment with cholic acid helps with the normal development of the liver and bile circulation system.
Slovenian: Pri rastočih dojenčkih zdravljenje s holno kislino pomaga pri normalnem razvoju jetrnega in žolčnega obtoka.
English: Adverse reactions reported in the literature are pruritus and increased serum transaminases in one or two children treated with high doses of cholic acid; however, these adverse reactions disappeared with a reduced dosage.
Slovenian: Neželena učinka, o katerih so poročali v literaturi, sta pruritus in povišana raven transaminaz v serumu pri enem ali dveh otrocih, zdravljenih z velikimi odmerki holne kisline, vendar sta ta neželena učinka izzvenela z zmanjšanjem odmerka.
English: Concomitant use with phenobarbital (see section 4.5).
Slovenian: Sočasna uporaba fenobarbitala (glejte poglavje 4.5).
English: Pharmacotherapeutic group: Bile and liver therapy, bile acid preparations; ATC code: A05AA03
Slovenian: Farmakoterapevtska skupina: zdravila za bolezni žolča in jeter, žolčne kisline, oznaka ATC: A05AA03
English: Distribution and pharmacological effects of bile acids such as cholic acid are mainly limited to the enterohepatic circulation, which includes the intestine, portal vein, liver and biliary tract.
Slovenian: Razporeditev in farmakološki učinki žolčnih kislin, kot je holna kislina, so večinoma omejeni na enterohepatični obtok, ki vključuje črevo, portalno veno, jetra in žolčevod.
English: There are no data on the effects of cholic acid on fertility.
Slovenian: Podatkov o učinkih holne kisline na plodnost ni.
English: • Increase liver enzymes (serum transaminases)
Slovenian: • povišana raven jetrnih encimov (transaminaz v serumu),
English: Patients with certain types of conditions known as inborn errors of bile synthesis cannot produce cholic acid and bile normally, which leads to the production and build-up of abnormal substances that are potentially damaging to the liver.
Slovenian: Bolniki z nekaterimi stanji, imenovanimi prirojene okvare sinteze žolča, niso sposobni normalno tvoriti holne kisline in žolča, kar povzroča tvorbo in kopičenje nenormalnih snovi, ki lahko poškodujejo jetra.
English: Serum transaminases were below the ULN for most patients (71-100%) at baseline and for most patients (86%) at the worst post-baseline assessment and for all patients (100%) at the best post-baseline assessment.
Slovenian: Transaminaze v serumu so bile pod vrednostjo ULN za večino bolnikov (71-100 %) ob izhodišču in za večino bolnikov (86 %) v oceni najslabše vrednosti po izhodišču ter za vse bolnike (100 %) v oceni najboljše vrednosti po izhodišču.
English: There is insufficient information on the excretion of cholic acid and its metabolites in human milk.
Slovenian: Ni dovolj podatkov o izločanju holne kisline in njenih presnovkov v materino mleko.
English: Deconjugated cholic acid and deoxycholic acid, the product of 7-dehydroxylation, are passively absorbed in the lower intestine and carried back to the liver in portal blood, where reconjugation will take place.
Slovenian: Dekonjugirana holna kislina in deoksiholna kislina, produkt 7-dehidroksilacije, se pasivno absorbirata v debelo črevo in s portalno krvjo potujeta nazaj v jetra, kjer bo prišlo do ponovne konjugacije.
English: In case of persistent lack of therapeutic response to cholic acid monotherapy, other treatment options should be considered, see section 4.4.
Slovenian: V primeru perzistentnega neodzivanja na monoterapijo s holno kislino je treba razmisliti o drugih možnostih zdravljenja; glejte poglavje 4.4.
English: These increase cholesterol production in the liver and as a result prevent cholic acid from working properly.
Slovenian: Ta povečajo tvorbo holesterola v jetrih, kar onemogoči pravilno delovanje holne kisline.
English: In Study CAC-002-01 for patients overall with single-enzyme defects, urinary bile acids and serum transaminases did not change significantly from baseline to worst post-baseline value.
Slovenian: V študiji CAC-002-01 za bolnike na splošno z okvaro enega encima, se vrednosti žolčnih kislin v urinu in transaminaz v serumu niso značilno spremenile od izhodišča v primerjavi z najslabšimi vrednostmi po izhodišču.
English: In this manner the vast majority of the bile acid pool is conserved and will cycle multiple times during feeding.
Slovenian: Tako se ohrani večina zaloge žolčnih kislin, ki bodo med hranjenjem opravile več ciklov.
English: The lowest dose of cholic acid that effectively reduces the bile acid metabolites to as close to zero as possible should be chosen.
Slovenian: Izbrati je treba najmanjši odmerek holne kisline, ki učinkovito zmanjša presnovke žolčnih kislin čim bližje vrednosti nič.
English: Packs containing 90 capsules.
Slovenian: Pakiranje vsebuje 90 kapsul.
English: In these cases, serum/bile levels of cholic acid should be closely monitored and the dose adjusted accordingly.
Slovenian: V teh primerih je treba skrbno spremljati ravni holne kisline v serumu/žolču in ustrezno prilagoditi odmerek.
English: INFORMATION IN BRAILLE Kolbam 250 mg
Slovenian: PODATKI V BRAILLOVI PISAVI Kolbam 250 mg
English: For infants and children who cannot swallow capsules, the capsules may be opened and the content added to infant formula or juice.
Slovenian: Za dojenčke in otroke, ki ne morejo pogoltniti kapsul, se lahko kapsule odprejo in vsebina doda otroški formuli ali soku.
English: The adverse reactions reported in the literature with an unknown frequency are also reported in the following table.
Slovenian: V spodnji preglednici so navedeni tudi neželeni učinki z neznano pogostnostjo, o katerih so poročali v literaturi.
English: Skin and subcutaneous tissue disorders Skin lesion Pruritus
Slovenian: Bolezni kože in podkožja kožne lezije pruritus pogosti neznana
English: Phenobarbital may stop cholic acid from working.
Slovenian: Fenobarbital lahko prepreči delovanje holne kisline.
English: The purpose of substituting missing cholic acid is to restore the main functions of this bile acid consisting of lipid transport in the form of mixed micelles, the activation of co-lipase and fat digestion and absorption, the absorption of fat-soluble vitamins, and the induction of bile flow, thus preventing cholestasis.
Slovenian: Namen nadomeščanja pomanjkanja holne kisline je ponovno vzpostaviti glavne funkcije te žolčne kisline, ki vključujejo prenos lipidov v obliki mešanih micelov, aktivacijo kolipaze in presnovo ter absorpcijo maščob, absorpcijo v maščobi topnih vitaminov in indukcijo žolčnega pretoka, kar preprečuje holestazo.
English: There are a limited number of studies that have demonstrated that cholic acid administered orally for up to 26 weeks at doses significantly greater than the therapeutic dose, was well tolerated in animals with no mortalities, no effects on bodyweight or food consumption and no evidence of significant macroscopic or microscopic findings in the liver.
Slovenian: 26 tednov v odmerkih, pomembno večjih od terapevtskega odmerka, dobro prenašale brez smrti, brez učinkov na telesno maso ali uživanje hrane ter brez dokazov pomembnih makro- ali mikroskopskih ugotovitev v jetrih.
English: Therefore use of phenobarbital in patients treated with cholic acid is contraindicated (see section 4.3).
Slovenian: Uporaba fenobarbitala pri bolnikih, zdravljenih s holno kislino, je zato kontraindicirana (glejte poglavje 4.3).
English: Administration of a single dose of cholic acid intravenously to pregnant ewes in late gestation demonstrated systemic exposure of cholic acid in the foetus with no effect on either the mother or the foetuses other than an increase in early deliveries.
Slovenian: Uporaba enkratnega intravenskega odmerka holne kisline pri ovcah v pozni brejosti je pokazala sistemsko izpostavljenost ploda holni kislini, pri čemer ni bilo učinkov na mater ali plode, razen povečanja pojavnosti prezgodnjih skotitev.
English: If you forget to take Kolbam
Slovenian: Če ste pozabili vzeti zdravilo Kolbam
English: Biliary bile alcohols and bile acids show remarkable structural diversity across animal species.
Slovenian: Alkoholi žolčnih kislin in žolčne kisline se strukturno izjemno razlikujejo med različnimi vrstami.
English: • Slight tingling (pruritus)
Slovenian: • rahlo srbenje (pruritus).
English: Any conjugated cholic acid not absorbed in the ileum will pass into the lower intestine where it may be subject to bacterial metabolism, principally deconjugation and 7-dehydroxylation.
Slovenian: Konjugirana holna kislina, ki se ne absorbira v ileumu, bo potovala v debelo črevo, kjer je lahko izpostavljena bakterijski presnovi, predvsem dekonjugaciji in 7-dehidroksilaciji.
English: Cholic acid is unlikely to cause serious side effects but you should contact your doctor for advice if you or your child have taken more than the amount prescribed.
Slovenian: Če ste vzeli večji odmerek zdravila Kolbam, kot bi smeli Ni verjetno, da bi holna kislina povzročila resne neželene učinke, vendar se posvetujte z zdravnikom, če ste vi ali vaš otrok vzeli večjo količino od predpisane.
English: Laboratory tests showed elevation of serum gamma glutamyltransferase (Gamma GT) transaminases and serum bile acid concentrations.
Slovenian: Laboratorijske preiskave so pokazale povečane serumske vrednosti gama-glutamiltransferaze (GGT), transaminaz in koncentracij žolčnih kislin v serumu.
English: Do not take any phenobarbital while you are on cholic acid.
Slovenian: Med jemanjem holne kisline ne uporabljajte fenobarbitala.
English: Within the liver, cholic acid is amidated in species-specific proportions, with glycine and/or taurine, into a more hydrophilic, conjugated form.
Slovenian: Holna kislina se v jetrih amidira v za vrsto specifičnih deležih (z glicinom in/ali tavrinom) v bolj hidrofilno, konjugirano obliko.
English: Cholic acid has no or negligible influence on the ability to drive and use machines.
Slovenian: Holna skupina nima vpliva ali ima zanemarljiv vpliv na sposobnost vožnje in upravljanja strojev.
English: Inborn errors of primary bile acid synthesis involve congenital defects in the primary enzymes responsible for catalysing key reactions in the synthesis of cholic and chenodeoxycholic acids.
Slovenian: Prirojene okvare sinteze primarnih žolčnih kislin vključujejo kongenitalne okvare primarnih encimov, odgovornih za katalizacijo ključnih reakcij pri sintezi holne in henodeoksiholne kisline.
English: Affected patients often present with significant comorbidities, including CNS impairment, which would not be treated by addressing the bile defect effects.
Slovenian: Prizadeti bolniki imajo pogosto pomembne sočasne bolezni, vključno z okvaro živčevja, ki jih ni mogoče zdraviti z obravnavo učinkov okvare žolčnih kislin.
English: Adverse reactions in patients (both adults and children) receiving cholic acid are generally mild to moderate in severity; the main reactions observed are given in the table below.
Slovenian: Resnost neželenih učinkov pri bolnikih (odraslih in otrocih), ki prejemajo holno kislino, je navadno blaga do zmerna; glavni opaženi neželeni učinki so navedeni v spodnji preglednici.
English: How to store Kolbam 6.
Slovenian: Shranjevanje zdravila Kolbam 6.
English: • Summary of product characteristics
Slovenian: • povzetek glavnih značilnosti zdravila;
English: In each of the primary bile acid deficiencies due to enzyme defects in the biosynthetic pathway, absence of primary bile acids leads to cholestasis and unregulated accumulation of toxic bile acid precursors.
Slovenian: Pri vseh pomanjkanjih primarnih žolčnih kislin, ki so posledica encimskih okvar v poti biosinteze, odsotnost primarnih žolčnih kislin povzroča holestazo in neuravnovešeno kopičenje prekurzorjev toksičnih žolčnih kislin.
English: Kolbam is indicated for the treatment of inborn errors in primary bile acid synthesis due to Sterol 27hydroxylase (presenting as cerebrotendinous xanthomatosis, CTX) deficiency, 2- (or α-) methylacylCoA racemase (AMACR) deficiency or Cholesterol 7α-hydroxylase (CYP7A1) deficiency in infants, children and adolescents aged 1 month to 18 years and adults.
Slovenian: Zdravilo Kolbam je indicirano za zdravljenje prirojenih napak v sintezi primarnih žolčnih kislin, do katerih pride zaradi pomanjkanja sterol 27-hidroksilaze (ki se kaže kot cerebrotendinozna ksantomatoza, CTX), pomanjkanja 2- (ali α-) metilacil-CoA racemaze (AMACR) ali pomanjkanja holesterol 7α-hidroksilaze (CYP7A1), pri dojenčkih, otrocih in mladostnikih, starih od enega meseca do 18 let, ter pri odraslih.
English: The study was an open-label, single arm, non-randomized design.
Slovenian: Študija je imela odprt, nerandomiziran načrt z eno skupino.
English: • skin lesions
Slovenian: • kožne lezije,
English: Statistically significant changes were seen in the baseline to the best post-baseline analysis of urinary bile acids, with substantial decreases in marked, significant, and slight abnormalities as well as increases in normal spectra.
Slovenian: Statistično značilne spremembe žolčnih kislin v urinu so opazili v analizi primerjave izhodiščnih vrednosti in najboljših vrednosti po izhodišču, z znatnim zmanjšanjem izrazitih, značilnih in blagih nenormalnostih, ter povečanjem normalnih spektrov.
English: For babies and children who cannot swallow capsules, open the capsule by gently twisting it and add the contents to infant formula, expressed breast milk or fruit puree in a suitable clean container.
Slovenian: Pri dojenčkih in otrocih, ki ne morejo pogoltniti kapsul, odprite kapsulo, tako da jo nežno zavrtite, in njeno vsebino dodajte otroški formuli, iztisnjenemu materinemu mleku ali sadni kašici v ustrezni čisti posodi.
English: Treatment must be initiated and monitored by physicians, including paediatricians, experienced in the management of the specific deficiencies.
Slovenian: 4.2 Odmerjanje in način uporabe Zdravljenje mora uvesti in spremljati zdravnik ali pediater z izkušnjami z obvladovanjem določenih oblik pomanjkanj.
English: Patients with hepatic impairment who are treated with cholic acid should be monitored closely (see section 4.4).
Slovenian: Bolnike z jetrno okvaro, ki se zdravijo s holno kislino, je treba pozorno spremljati (glejte poglavje 4.4).
English: How to use Kolbam
Slovenian: Kako uporabljati zdravilo Kolbam
English: NAME AND ADDRESS OF THE MARKETING AUTHORISATION HOLDER Retrophin Europe Limited, Palmerston House, Fenian Street Dublin 2, Ireland
Slovenian: IME IN NASLOV IMETNIKA DOVOLJENJA ZA PROMET Z ZDRAVILOM Retrophin Europe Limited, Palmerston House, Fenian Street Dublin 2, Irska
English: After the initiation period, serum and urine bile acids (using suitable analytical techniques) and liver parameters should be determined annually, at a minimum, and the dose adjusted accordingly.
Slovenian: Po obdobju uvajanja je treba vsaj enkrat na leto določiti žolčne kisline v serumu in urinu (z ustreznimi analitičnimi metodami) in jetrne parametre ter ustrezno prilagoditi odmerek.
English: Aluminium-based antacids have been shown to adsorb bile acids in vitro and may be expected to reduce the levels of cholic acid in the same manner as the bile acid sequestering agents.
Slovenian: Pokazali so, da antacidi na podlagi aluminija in vitro adsorbirajo žolčne kisline, zato se lahko pričakuje, da bodo znižali ravni holne kisline enako kot vezalci žolčnih kislin.
English: Concurrent with the disappearance of atypical bile acid metabolites, there is a consistent reduction and normalization in serum liver enzymes.
Slovenian: Sočasno z izginotjem netipičnih presnovkov žolčnih kislin pride do doslednega znižanja in normalizacije ravni jetrnih encimov v serumu.
English: • Sterol 27-hydroxylase deficiency (presenting as CTX)
Slovenian: • pomanjkanje sterol 27-hidroksilaze (ki se kaže kot CTX);
English: It followed an open-label, single arm, non-randomized design and included eligible subjects who had previously received cholic acid through CAC-91-10-10 and CAC-001-01, and newly diagnosed subjects.
Slovenian: Imela je odprt, nerandomiziran načrt z eno skupino in je vključila primerne bolnike, ki so predhodno prejemali holno kislino v študijah CAC-91-10-10 in CAC-001-01, in bolnike z novo postavljeno diagnozo.
English: For infants and children who cannot swallow capsules, the capsule may be opened gently and the contents mixed with food.
Slovenian: Za dojenčke in otroke, ki ne morejo pogoltniti kapsul, se lahko kapsule previdno odprejo in vsebina doda hrani.
English: The safety and efficacy of cholic acid in elderly patients has not been established.
Slovenian: Varnosti in učinkovitosti holne kisline pri starejših bolnikih niso ugotovili.
English: Any medicinal products implicated in drug-induced cholestasis through inhibition of transporters could reduce the effectiveness of cholic acid treatment on coadministration.
Slovenian: Katera koli zdravila, ki povzročajo holestazo zaradi zaviranja prenašalcev, lahko ob sočasni uporabi zmanjšajo učinkovitost zdravljenja s holno kislino.
English: Treatment with exogenous cholic acid is intended to replace physiological bile acid in cases of inborn errors of bile acid synthesis.
Slovenian: Zdravljenje z eksogeno holno kislino je namenjeno zamenjavi fiziološke žolčne kisline pri primerih prirojenih okvar sinteze žolčnih kislin.
English: Of all the patients treated in Study CAC-91-10-10, 49 patients presented with a single enzyme defect.
Slovenian: Od vseh bolnikov, zdravljenih v študiji CAC-91-10-10, jih je imelo 49 okvaro posamičnega encima.
English: Study CAC-91-10-10, (Investigation in the pathogenesis of liver disease in patients with inborn errors of bile acid metabolism) was conducted from 1992-2009 to evaluate the therapeutic efficacy and safety of cholic acid to treat patients with identified inborn errors of bile acid metabolism.
Slovenian: Med letoma 1992 in 2009 so izvedli študijo CAC-91-10-10 (Preučevanje patogeneze jetrne bolezni pri bolnikih s prirojenimi okvarami presnove žolčnih kislin - Investigation in the pathogenesis of liver disease in patients with inborn errors of bile acid metabolism), s katero so ocenili terapevtsko učinkovitost in varnost holne kisline pri zdravljenju bolnikov z ugotovljenimi prirojenimi okvarami presnove žolčnih kislin.
English: The efficacy analysis also demonstrated that treatment with cholic acid significantly improved ALT and AST values for patients stratified by single enzyme defects.
Slovenian: Analiza učinkovitosti je prav tako pokazala, da je zdravljenje s holno kislino pomembno izboljšalo vrednosti ALT in AST pri bolnikih, stratificiranih glede na okvare posamičnega encima.