FrancophonIA/ELRC3.0 huggingface dataset:
English: Mild and transient effects may not require medical treatment or discontinuation of the infusion.
Slovenian: Blagi in prehodni učinki morda ne bodo zahtevali zdravniške oskrbe ali prekinitve infuzije.
English: The experience in children 0-6 years old is limited and therefore no dose can be recommended for this age group.
Slovenian: Z uporabo pri otrocih, starih od 0 do 6 let, je malo izkušenj, zato ni mogoče priporočiti odmerka za to starostno skupino.
English: Twenty-five patients completed the first study and entered an extension study.
Slovenian: 25 bolnikov je zaključilo prvo študijo in te so nato vključili v nadaljevalno študijo.
English: The adverse reactions categorized as incidence “not known (cannot be estimated from the available data)” are derived from post-marketing spontaneous reports.
Slovenian: O neželenih reakcijah, razvrščenih kot pogostnost »neznana« (ni mogoče oceniti iz razpoložljivih podatkov), so spontano poročali v obdobju trženja .
English: Pharmacokinetic parameters were not significantly different between male and female patients.
Slovenian: Farmakokinetični parametri se niso bistveno razlikovali med bolnicami in bolniki.
English: Antibodies to the protein
Slovenian: Protitelesa proti proteinu
English: • For single use only.
Slovenian: • Le za enkratno uporabo.
English: It is expected that its metabolic degradation will follow the pathways of other proteins, i.e. peptide hydrolysis.
Slovenian: Pričakuje se, da bo njena presnovna razgradnja potekala po istih poteh kot razgradnja drugih proteinov, tj. s hidrolizo peptidov.
English: Children and adolescents may be more likely than adults to experience an infusion related reaction.
Slovenian: V primerjavi z odraslimi je pri otrocih in mladostnikih verjetneje, da se bo pojavila z infuzijo povezana reakcija.
English: If treatment with Replagal makes your body produce antibodies this will not stop Replagal from working and the antibodies may disappear with time.
Slovenian: Če zaradi zdravljenja z zdravilom Replagal začne vaše telo proizvajati protitelesa, to delovanja zdravila ne bo preprečilo, protitelesa pa lahko s časom izginejo.
English: The onset of infusion related reactions has generally occurred within the first 2-4 months after initiation of treatment with Replagal although later onset (after 1 year) has been reported as well.
Slovenian: Reakcije, povezane z infundiranjem, so se na splošno pojavljale v prvih 2 do 4 mesecih po začetku zdravljenja z zdravilom Replagal, vendar so poročali o njihovem začetku tudi pozneje (po enem letu).
English: Agalsidase alfa is a protein.
Slovenian: Agalzidaza alfa je protein.
English: Following six months of Replagal treatment 12 of 28 male patients showed altered pharmacokinetics including an apparent increase in clearance.
Slovenian: Po šestih mesecih zdravljenja z zdravilom Replagal so se pri 12 od 28 bolnikov pokazale spremembe v farmakokinetiki, vključno z navideznim povečanjem očistka.
English: Most of the time you can still be given Replagal even if these symptoms occur.
Slovenian: Večinoma vam bodo zdravilo Replagal dajali tudi, če se pojavijo ti simptomi.
English: These IgG antibodies appeared to develop following approximately 3-12 months of treatment.
Slovenian: Kaže, da so se ta protitelesa IgG razvila po približno 3-12
English: • Calculate the dose and number of Replagal vials needed.
Slovenian: • Izračunajte odmerek in potrebno število vial zdravila Replagal.
English: About 1 out of 7 patients (frequency “very common”) may have a reaction during or following an infusion of Replagal (infusion related reaction).
Slovenian: Približno 1 od 7 bolnikov (»zelo pogost« učinek) ima lahko reakcijo med infuzijo zdravila Replagal ali po njej (reakcija, povezana z infuzijo).
English: This was associated with a significant reduction in chronic neuropathic pain medication use and number of days on pain medication.
Slovenian: To je bilo povezano s pomembnim zmanjšanjem uporabe zdravil proti kronični nevropatični bolečini in števila dni jemanja zdravil proti bolečini.
English: It is used as long-term enzyme replacement therapy when the level of enzyme in the body is absent or lower than normal as in Fabry Disease.
Slovenian: Uporabljamo ga kot dolgotrajno encimsko nadomestno zdravljenje, kadar encim v telesu ni prisoten ali kadar je njegova koncentracija manjša od normalne, kot pri Fabryjevi bolezni.
English: B. CONDITIONS OR RESTRICTIONS REGARDING SUPPLY AND USE Medicinal product subject to restricted medical prescription (See Annex I: Summary of Product Characteristics, Section 4.2)
Slovenian: B. POGOJI ALI OMEJITVE GLEDE OSKRBE IN UPORABE Predpisovanje in izdaja zdravila je le s posebnim režimom (glejte Prilogo I: Povzetek glavnih značilnosti zdravila, poglavje 4.2).
English: Your doctor may also treat the symptoms with other medicines (antihistamines or corticosteroids).
Slovenian: Zdravnik lahko tudi zdravi te simptome z drugimi zdravili (antihistaminiki ali kortikosteroidi).
English: List of other side effects:
Slovenian: Seznam drugih neželenih učinkov:
English: DATE OF FIRST AUTHORISATION/RENEWAL OF THE AUTHORISATION Date of first authorisation: 03/08/2001
Slovenian: Datum prve odobritve: 03/08/2001 Datum zadnjega podaljšanja:03/08/2006
English: In a second study, in 14 patients ≥ 7 years of age, the results regarding heart rate variability were consistent with previous findings.
Slovenian: V drugi študiji, pri 14 bolnikih, starih ≥ 7 let, so se rezultati glede variabilnosti srčnega utripa ujemali s predhodnimi ugotovitvami.
English: What Replagal is and what it is used for What you need to know before you are given Replagal How Replagal is given
Slovenian: Kaj je zdravilo Replagal za kaj ga uporabljamo Kaj morate vedeti, preden boste dobili zdravilo Replagal Kako se daje zdravilo Replagal
English: There is a theoretical risk of decreased agalsidase alfa activity.
Slovenian: Obstaja teoretično tveganje, da se bo aktivnost agalzidaze alfa zmanjšala.
English: An analysis showed that serious adverse events were reported in 39.4% of those patients who switched from agalsidase beta compared to 31.0% in those who were naïve to therapy prior to study entry.
Slovenian: Analiza je pokazala, da so o resnih neželenih učinkih poročali pri 39,4 % bolnikov, ki so prešli z agalzidaze beta, v primerjavi z 31,0 % tistih, ki se pred vstopom v študijo niso zdravili.
English: Very limited clinical data on pregnancies exposed to Replagal have shown no adverse effects on the mother and newborn child.
Slovenian: Zelo omejeni klinični podatki o nosečnicah, izpostavljenih zdravilu Replagal, so pokazali, da ni neželenih učinkov na mater in novorojenca.
English: Pharmacodynamic data suggest that at a dose of 0.2 mg/kg Replagal, the reductions in plasma Gb3 are more or less comparable between adolescents and young children (see section 5.1).
Slovenian: Farmakodinamični podatki kažejo, da je pri odmerku 0,2 mg/kg zdravila Replagal zmanjšanje ravni Gb3 v plazmi so pri mladostnikih in mladi otrocih bolj ali manj primerljivi (glejte poglavje 5.1).
English: For single use only.
Slovenian: Le za enkratno uporabo.
English: Antibodies to agalsidase alfa have not been shown to be associated with any clinically significant effects on safety (e.g. infusion reactions) or efficacy.
Slovenian: Ni podatkov, da bi protitelesa proti agalzidazi alfa kakorkoli klinično pomembno vplivala na varnost (npr. infuzijske reakcije) ali učinkovitost zdravila.
English: For children and adolescents 7-18 years old a dose of 0.2 mg/kg every other week may be used.
Slovenian: Pri otrocih in mladostnikih, starih od 7 do 18 let, se lahko uporablja odmerek 0,2 mg/kg vsak drugi teden.
English: PHARMACEUTICAL FORM AND CONTENTS 1 x 3.5 ml concentrate for solution for infusion 4 x 3.5 ml concentrate for solution for infusion 10 x 3.5 ml concentrate for solution for infusion
Slovenian: FARMACEVTSKA OBLIKA IN VSEBINA 1 x 3,5 ml koncentrata za raztopino za infundiranje 4 x 3,5 ml koncentrata za raztopino za infundiranje 10 x 3,5 ml koncentrata za raztopino za infundiranje
English: Care must be taken to ensure the sterility of the prepared solutions since Replagal does not contain any preservative or bacteriostatic agent; aseptic technique must be observed.
Slovenian: Skrbno pazite, da boste zagotovili sterilnost pripravljene raztopine, ker zdravilo Replagal ne vsebuje konzervansov ali bakteriostatičnih sredstev - upoštevajte aseptično tehniko.
English: Your treatment will be supervised by a doctor who specialises in the treatment of Fabry Disease.
Slovenian: Zdravljenje bo nadzoroval zdravnik, ki je specialist za zdravljenje Fabryjeve bolezni.
English: These changes were associated with the development of low titre antibodies to agalsidase alfa but no clinically significant effects on safety or efficacy were observed in the patients studied.
Slovenian: Te spremembe so bile povezane z nastankom majhnega titra protiteles proti agalzidazi alfa, vendar pa pri preučevanih bolnikih niso opazili klinično pomembnih učinkov na varnost in učinkovitost zdravila.
English: - swelling in your hands, feet, ankles, face, lips, mouth or throat which may cause difficulty in swallowing or breathing.
Slovenian: - otekanje dlani, podplatov, gležnjev, obraza, ustnic, ust ali grla, ki lahko povzroča težave pri požiranju ali dihanju.
English: If you have advanced renal disease, you may find that your Replagal treatment has a limited effect on your kidneys.
Slovenian: Če imate napredovalo bolezen ledvic, bo morda imelo zdravljenje z zdravilom Replagal omejen učinek na vaše ledvice.
English: Replagal is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry Disease (α-galactosidase A deficiency).
Slovenian: Zdravilo Replagal je indicirano za dolgoročno encimsko nadomestno zdravljenje pri bolnikih s potrjeno diagnozo Fabryjeve bolezni (pomanjkanje α-galaktozidaze A).
English: It is not known whether Replagal is excreted in human milk.
Slovenian: Ni znano, ali se zdravilo Replagal izloča v materino mleko.
English: A low titre IgG antibody response has been observed in approximately 24% of the male patients treated with Replagal.
Slovenian: Odziv z majhnim titrom protiteles IgG so opazili pri približno 24 % moških bolnikov, ki so jih zdravili z zdravilom Replagal.
English: Table 1 System organ class
Slovenian: Preglednica 1 Organski sistem
English: In clinical studies of children (7-18 years) who received Replagal 0.2 mg/kg every other week, no unexpected safety issues were encountered (see section 5.1).
Slovenian: V kliničnih študijah pri otrocih, starih od 7 do 18 let, ki so dobivali odmerke zdravila Replagal 0,2 mg/kg vsak drugi teden, ni bilo nepričakovanih varnostnih težav (glejte poglavje 5.1).
English: In a second study, fifteen patients with left ventricular hypertrophy completed a 6 month placebocontrolled study and entered an extension study.
Slovenian: V drugi študiji je 15 bolnikov s hipertrofijo levega prekata zaključilo šestmesečno, s placebom kontrolirano študijo in so jih nato vključili v nadaljevalno študijo.
English: Calculate the dose and number of Replagal vials needed.
Slovenian: Izračunajte odmerek in potrebno število vial zdravila Replagal.
English: Replagal should not be co-administered with chloroquine, amiodarone, benoquin or gentamicin since these substances have the potential to inhibit intra-cellular α-galactosidase activity.
Slovenian: Zdravila Replagal ne smete dajati sočasno s klorokvinom, amiodaronom, benokvinom ali gentamicinom, saj lahko te snovi zavirajo intracelularno delovanje α-galaktozidaze.
English: Study in patients switching from agalsidase beta to Replagal (agalsidase alfa) 100 patients [(naïve (n=29); or previously treated with agalsidase beta who switched to Replagal (n=71)) were treated for up to 30 months in an open label, uncontrolled study.
Slovenian: Študija pri bolnikih, ki so prešli z agalzidaze beta na zdravilo Replagal (agalzidaza alfa) 100 bolnikov [predhodno nezdravljenih (n = 29) ali predhodno zdravljenih z agalzidazo beta, ki so prešli na zdravilo Replagal (n = 71)] se je do 30 mesecev zdravilo v odprti nenadzorovani študiji.
English: A single patient could be affected by several adverse reactions.
Slovenian: Pri posameznem bolniku se je lahko pojavilo tudi več neželenih reakcij.
English: 4.7 Undesirable effects
Slovenian: 4.8 Neželeni učinki
English: In clinical trials doses up to 0.4 mg/kg weekly were used, and their safety profile was not different from the recommended dose of 0.2 mg/kg biweekly.
Slovenian: V kliničnih preskušanjih so uporabljali odmerke do 0,4 mg/kg tedensko, vendar varnostni profil ni bil drugačen kot pri priporočenem odmerku 0,2 mg/kg na vsaka dva tedna.
English: Replagal is used to treat adult patients, as well as adolescents and children from the age of 7, with confirmed diagnosis of Fabry Disease.
Slovenian: Zdravilo Replagal je namenjeno zdravljenju odraslih bolnikov ter mladostnikov in otrok, starih 7 let ali več, s potrjeno diagnozo Fabryjeve bolezni.
English: Infusion related reactions have been experienced by 9 patients of the naïve population (31.0%) compared to 27 patients of the switched population (38.0%).
Slovenian: Z infuzijo povezane reakcije so se pojavile pri 9 predhodno nezdravljenih bolnikih (31,0 %) v primerjavi s 27 bolniki, ki so prešli na zdravilo Replagal (38,0 %).
English: A review of cardiac events showed that infusion reactions may be associated with hemodynamic stress triggering cardiac events in patients with pre-existing cardiac manifestations of Fabry disease.
Slovenian: Pregled srčnih dogodkov je pokazal, da so lahko reakcije na infundiranje povezane s hemodinamičnim stresom, ki sproži srčne dogodke pri bolnikih z obstoječimi srčnimi manifestacijami
English: Agalsidase alfa has been produced in a human cell line to provide for a human glycosylation profile that can influence uptake by mannose-6-phosphate receptors on the surface of target cells.
Slovenian: Agalzidazo alfa so proizvedli v humani celični liniji zaradi priprave na humani profil glikozilacije, ki lahko vpliva na privzem preko manoza-6 fosfatnih receptorjev na površini ciljnih celic.
English: Patients received the recommended dosage of 0.2 mg/kg of Replagal.
Slovenian: Bolniki so dobili priporočeni odmerek 0,2 mg/kg zdravila Replagal.
English: No increase in the incidence of adverse events was detected for this patient.
Slovenian: Pri tem bolniku ni bilo opaziti nobenega povečanja incidence neželenih učinkov.
English: The metabolic effects were also associated with clinically significant weight gain, increased sweating and increased energy.
Slovenian: Presnovni učinki so bili povezani tudi s klinično pomembnim povečanjem telesne mase, močnejšim potenjem in povečano količino energije.
English: There is very limited data on pregnancies exposed to Replagal.
Slovenian: Podatki o nosečnicah, izpostavljenih zdravilu Replagal so zelo omejeni.
English: Compared with placebo, treatment with Replagal also reduced accumulation of Gb3.
Slovenian: V primerjavi s placebom je zdravljenje z zdravilom Replagal zmanjšalo tudi kopičenje Gb3.
English: Common: may affect up to 1 in 10 people: - tingling or numbness or pain in fingers or toes, change in the taste of food, eyes tearing, blink reflex abnormal, ears ringing, shakes, prolonged sleep - palpitations, increased heart rate, increased blood pressure - cough, chest pain or tightness, hoarseness, sore or tight throat, sticky throat secretions, runny nose, cold symptoms - vomiting, abdominal pain or discomfort, diarrhoea - acne, red or itchy or mottled skin, rash at the infusion site - back or limb pain, muscle pain, joint pain, muscle and bone discomfort, swelling of the extremities or joints - feeling cold or hot, flu-like symptoms, feeling sick, feeling lack of energy
Slovenian: - ščemenje ali otrplost ali bolečina v prstih na rokah ali nogah, spremembe okusa hrane, solzenje oči, nenormalen refleks mežikanja, zvenenje v ušesih, tresenje, podaljšano spanje - palpitacije, zvišan srčni utrip, zvišan krvni tlak - kašelj, bolečina ali stiskanje v prsnem košu, hripavost, bolečina ali stiskanje v grlu, vlažen izloček iz grla, izcedek iz nosu, simptomi prehlada - bruhanje, bolečina ali neprijeten občutek v trebuhu, driska - akne, rdeča, srbeča ali lisasta koža, izpuščaj na mestu infundiranja - bolečina v hrbtu ali okončinah, bolečina v mišicah, bolečina v sklepih, neprijeten občutek v mišicah in kosteh, otekanje okončin ali sklepov - občutek vročine ali mraza, simptomi, podobni gripi, slabo počutje, pomanjkanje energije
English: *agalsidase alfa is the human protein α-galactosidase A produced in a human cell line by genetic engineering technology.
Slovenian: * agalzidaza alfa je humani protein α-galaktozidaza A, izdelan s tehnologijo genskega inženiringa v humani celični liniji.
English: After 12 to 54 months of therapy, 17% of Replagal treated patients were still antibody positive whereas 7% showed evidence for the development of immunologic tolerance, based on the disappearance of IgG antibodies over time.
Slovenian: Po 12 do 54 mesecih zdravljenja je bilo 17% bolnikov, ki so jih zdravili z zdravilom Replagal, še vedno pozitivnih na protitelesa, pri 7 % pa so se kazali znaki imunske tolerance, kar se je kazalo kot izginjanje protiteles IgG s časom.
English: Elimination half-lives were 108 ± 17 minutes in males compared to 89 ± 28 minutes in females and volume of distribution was approximately 17% body weight in both sexes.
Slovenian: 108 ± 17 minut pri bolnikih v primerjavi z 89 ± 28 minut pri bolnicah, porazdelitveni volumen pa je bil približno 17 % telesne mase pri obeh spolih.
English: Patients switched from agalsidase beta to Replagal had a safety profile consistent with that observed in other clinical experience.
Slovenian: Varnostni profil pri bolnikih, ki so prešli z algazidaze beta na zdravilo Replagal, je bil skladen z opaženim v drugih kliničnih izkušnjah.
English: Agalsidase alfa catalyses the hydrolysis of Gb3, cleaving a terminal galactose residue from the molecule.
Slovenian: Agalzidaza alfa katalizira hidrolizo Gb3 z odcepitvijo terminalnega ostanka galaktoze iz molekule.
English: The enzyme is then removed from the cells and made into a sterile concentrate for solution for infusion.
Slovenian: Encim se nato odstrani iz celic in predela v sterilni koncentrat za pripravo raztopine za infundiranje.
English: Other infusion-related symptoms may include dizziness and hyperhidrosis.
Slovenian: Med drugimi simptomi, povezanimi z infundiranjem, sta lahko omotica in hiperhidroza.
English: Since no preservative is present, it is recommended that administration is started as soon as possible.
Slovenian: Ker raztopina ne vsebuje konzervansov, je priporočljivo, da začnete z dajanjem čim prej.
English: As metabolism is expected to occur by peptide hydrolysis, impaired liver function is not expected to affect the pharmacokinetics of agalsidase alfa in a clinically significant manner.
Slovenian: Ker je pričakovati, da bo presnova potekala s hidrolizo peptidov, ni verjetno, da bi jetrna okvara klinično pomembno vplivalo na farmakokinetiko agalzidaze alfa.
English: Based on the similarity of pharmacokinetic properties of agalsidase alfa in both males and females, tissue distribution in major tissues and organs is also expected to be comparable in male and female patients.
Slovenian: Zaradi podobnosti farmakokinetičnih lastnosti agalzidaze alfa pri bolnicah in bolnikih je pričakovati, da je tudi porazdelitev encima po poglavitnih tkivih in organih podobna pri bolnicah in bolnikih.
English: Data with patients indicates that at least 0.1mg/kg is required to achieve a pharmacodynamics response.
Slovenian: Podatki, pridobljeni pri bolnikih kažejo, da je za farmakodinamični odziv potreben vsaj odmerek 0,1 mg/kg.
English: Kidney biopsy specimens revealed a significant increase in the fraction of normal glomeruli and a significant decrease in the fraction of glomeruli with mesangial widening in patients treated with Replagal in contrast to the patients treated with placebo.
Slovenian: so vzorci biopsije ledvic pokazali pomembno povečanje deleža normalnih glomerulov in pomembno zmanjšanje deleža glomerulov z mezangialno razširitvijo v primerjavi z bolniki, ki so prejemali placebo.
English: The presence of extensive renal damage (eGFR <60mL/min) may limit the renal response to enzyme replacement therapy.
Slovenian: Hude poškodbe ledvic (eGFR [receptor za epidermalni rastni faktor] < 60 ml/min) lahko omejijo odziv ledvic na encimsko nadomestno zdravljenje.
English: The selection of 0.2 mg/kg dose (infused over 40 minutes) for the registration clinical studies was intended to temporarily saturate the ability of the mannose-6phosphate receptors to internalize the agalsidase alfa in the liver and allow distribution of enzyme to other relevant organ tissues.
Slovenian: Izbira odmerka 0,2 mg/kg (40-minutna infuzija) za klinične študije za pridobitev dovoljenja za promet z zdravilom je bila namenjena začasnemu nasičenju sposobnosti manoza-6-fosfatnih receptorjev za internalizacijo agalzidaze alfa v jetrih in omogočanju porazdelitve encima v druga pomembna organska tkiva.
English: In male paediatric Fabry patients ≥ 7 years of age, hyperfiltration can be the earliest manifestation of renal involvement in the disease.
Slovenian: Pri moških pediatričnih bolnikih ≥ 7 let s Fabryjevo boleznijo je lahko hiperfiltracija najzgodnejši znak težav z ledvicami.
English: Uncommon: may affect up to 1 in 100 people: - severe allergic (anaphylactic-type) reaction
Slovenian: Občasni (pojavijo se lahko pri največ 1 od 100 bolnikov): - huda alergijska reakcija (anafilaktičnega tipa).
English: Instructions for use, handling and disposal
Slovenian: Navodila za uporabo in ravnanje z zdravilom ter odstranjevanje
English: Adverse drug reactions reported in patients with history of end stage renal disease were similar to those reported in the general patient population.
Slovenian: Neželeni učinki, o katerih so poročali pri bolnikih z anamnezo ledvične bolezni v zadnji fazi, so bili podobni tistim, o katerih so poročali pri splošni populaciji bolnikov.
English: Renal elimination of agalsidase alfa is considered to be a minor clearance pathway since pharmacokinetic parameters are not altered by impaired renal function.
Slovenian: Ledvično izločanje agalzidaze alfa velja za manj pomembno pot izločanja, saj ledvična okvara ne vpliva na spremembo farmakokinetičnih parametrov.
English: Chemical and physical in use stability has been demonstrated for 24 hours at 25°C.
Slovenian: Vendar pa je razredčena raztopina dokazano kemično in fizikalno stabilna 24 ur pri temperaturi 25 °C.
English: Do not use Replagal if you notice that there is discolouration or other foreign particles present.
Slovenian: Ne uporabljajte zdravila Replagal če opazite, da je razbarvan ali če vsebuje tuje delce.
English: It is not known whether Replagal crosses the placenta.
Slovenian: Ni znano, ali zdravilo Replagal prehaja skozi placento.
English: • Since no preservative is present, it is recommended that administration is started as soon as possible after dilution.
Slovenian: • Ker raztopina ne vsebuje konzervansov je priporočljivo, da začnete z dajanjem čim prej po redčenju.
English: No studies have been conducted with respect to parturition or peri/post-natal development.
Slovenian: Študije vpliva na kotenje oziroma peri in postnatalni razvoj niso bile opravljene.
English: Administer the infusion solution over a period of 40 minutes using an intravenous line with an integral filter.
Slovenian: Raztopino infundirajte 40 minut z uporabo intravenskega kanala z vgrajenim filtrom.
English: The overall incidence of SAEs by treatment group did not show any obvious effect of treatment regimen on the SAE profile of the different treatment groups.
Slovenian: Skupna incidenca hudih neželenih učinkov v zdravljenih skupinah ni pokazala, da bi režim zdravljenja kakor koli očitno vplival na profil hudih neželenih učinkov v skupinah z različnimi zdravljenji.