FrancophonIA/ELRC3.0 huggingface dataset:
English: Efficacy and safety of Fabrazyme was evaluated in two studies with children, one dose-finding study, two double-blind placebo-controlled studies, and one open-label extension study in both male and female patients.
Danish: Fabrazymes virkning og sikkerhed blev vurderet i to studier med børn, et dosis-bestemmende studie, to dobbeltblinde placebokontrollerede studier og en åben udvidelse med både mandlige og kvindelige patienter.
English: This clearance was achieved in 69% (20/29) of the Fabrazyme treated patients, but in none of the placebo patients (p<0.001).
Danish: Denne clearance blev opnået hos 69 % (20/29) af de patienter, der blev behandlet med Fabrazyme, men ikke hos nogen af placebopatienterne (p<0,001).
English: Genzyme Ireland Limited, IDA Industrial Park, Old Kilmeaden Road, Waterford, Ireland
Danish: Genzyme Ireland Limited, IDA Industrial Park, Old Kilmeaden Road, Waterford, Irland
English: The occurrence of an adverse reaction in a single patient is defined as uncommon in light of the relatively small number of patients treated.
Danish: Forekomsten af en bivirkning hos en enkelt patient er defineret som "ikke almindelig", set i lyset af det relativt begrænsede antal behandlede patienter.
English: No interaction studies and no in vitro metabolism studies have been performed.
Danish: Der er ikke udført interaktionsundersøgelser samt in-vitro metabolisme-undersøgelser.
English: • red eyes
Danish: • røde øjne
English: Another double-blind, placebo-controlled study of 82 patients was performed to determine whether Fabrazyme would reduce the rate of occurrence of renal, cardiac, or cerebrovascular disease or death.
Danish: Et andet dobbeltblindt placebokontrolleret studie med 82 patienter blev foretaget for at undersøge, om Fabrazyme kunne reducere forekomsten af nyre-, hjerte- eller cerebrovaskulær sygdom eller død.
English: Doses up to 3 mg/kg body weight have shown to be safe.
Danish: Doser på op til 3 mg/kg legemsvægt har vist sig at være sikre.
English: If severe allergic or anaphylactic-type reactions occur, immediate discontinuation of the administration of Fabrazyme should be considered and appropriate treatment initiated.
Danish: Hvis der forekommer alvorlige allergiske eller anafylaktiske reaktioner, skal det overvejes, omgående at ophøre med behandlingen med Fabrazyme, og en egnet behandling skal påbegyndes.
English: Genzyme Corporation 45, 51, 68, 74, 76 and 80 New York Avenue Framingham
Danish: 45, 51, 68, 74, 76 and 80 New York Avenue Framingham
English: A reduction in GL-3 was observed in kidney, heart, skin and plasma at all doses.
Danish: En reduktion i GL-3 blev observeret i nyrer, hjerte, hud og plasma ved alle doser.
English: The decision to have a patient move to home infusion should be made after evaluation and recommendation by the treating physician.
Danish: Beslutningen om at overflytte en patient til hjemmebehandling skal træffes efter evaluering og anbefaling af den behandlende læge.
English: Use Aseptic Technique
Danish: Anvend aseptisk teknik
English: The initial infusion rate should be no more than 0.25 mg/min (15 mg/hour) to minimise the potential occurrence of infusion-associated reactions.
Danish: Den initiale infusionshastighed bør ikke være hurtigere end 0,25 mg/min (15 mg/time) for at minimere den potentielle forekomst af infusionsrelaterede reaktioner.
English: The amino acid sequence of the recombinant form, as well as the nucleotide sequence which encoded it, are identical to the natural form of α-galactosidase A.
Danish: Aminosyresekvensen i den rekombinante form er lige som nukleotidsekvensen, der har kodet den, identisk med den naturlige form af α-galaktosidase A.
English: In addition, infusion-associated reactions were dose dependent.
Danish: Desuden var de infusionsrelaterede reaktioner dosisafhængige.
English: Because there are no data available on effects in neonates exposed to agalsidase beta via breast milk, it is recommended to stop breast-feeding when Fabrazyme is used.
Danish: Da der ikke foreligger nogen tilgængelige data om virkningerne hos neonatale, der udsættes for agalsidase beta via modermælk, anbefales det at ophøre med amningen, mens der anvendes Fabrazyme.
English: Talk to your doctor or pharmacist before using Fabrazyme.
Danish: Kontakt lægen eller apotekspersonalet, før du bruger Fabrazyme.
English: The elimination half-life was dose independent and ranged from 45 to 100 minutes.
Danish: Eliminationshalveringstiden var ikke dosisafhængig og lå på mellem 45 og 100 minutter.
English: Severe life-threatening allergic reactions (“anaphylactoid reactions”) have been reported in some patients.
Danish: Alvorlige, livstruende allergiske reaktioner (”anafylaktoide reaktioner”) er blevet indberettet for visse patienter.
English: If not used immediately, in-use storage and conditions are the responsibility of the user.
Danish: Hvis det ikke anvendes øjeblikkeligt, er brugeren ansvarlig for opbevaring og opbevaringsforhold.
English: Plasma GL-3 was cleared in a dose dependent manner, but was less consistent at the dose of 0.3 mg/kg.
Danish: Plasma GL-3 clearance var dosisafhængig, men var mindre konsekvent ved en dosis på 0,3 mg/kg.
English: The pH of the reconstituted solution is approximately 7.0.
Danish: pH i det rekonstituerede koncentrat er cirka 7,0.
English: If you suffer from Fabry disease a fat substance, called globotriaosylceramide (GL-3), is not removed from the cells of your body and starts to accumulate in the walls of the blood vessels of your organs.
Danish: Hvis man lider af Fabrys sygdom, vil et fedtstof kaldet globotriaosylceramid (GL-3) ikke blive fjernet fra kroppens celler, og det vil begynde at ophobe sig i væggene i blodårerne i kroppens organer.
English: Animal studies do not indicate direct or indirect harmful effects with respect to embryonal/foetal development (see section 5.3).
Danish: Dyrestudier viser ikke direkte eller indirekte skadelige virkninger i forbindelse med embryoets/fostrets udvikling.
English: • feeling hot and cold
Danish: • varme- og kuldefølelse
English: The majority of patients developed IgG antibodies to r-hαGAL, typically within 3 months of the first infusion with Fabrazyme.
Danish: Hovedparten af patienterne udviklede typisk IgG antistoffer mod r-hαGAL indenfor 3 måneder efter den første infusion af Fabrazyme.
English: White to off-white lyophilised cake or powder.
Danish: Hvid til off-white lyofiliseret substans eller pulver.
English: The rationale for enzyme replacement therapy is to restore a level of enzymatic activity sufficient to clear the accumulating substrate in the organ tissues; thereby, preventing, stabilizing or reversing the progressive decline in function of these organs before irreversible damage has occurred.
Danish: Begrundelsen for enzymsubstituerende behandling er at etablere et enzymatisk aktivitetsniveau, som er tilstrækkeligt til at fjerne det akkumulerende substrat i organvævene og derved forebygge, stabilisere eller vende den progressive forværring af disse organers funktion, før der er opstået irreversible skader.
English: Patients experiencing adverse events during the home infusion need to immediately stop the infusion process and seek the attention of a healthcare professional.
Danish: Patienter, der oplever bivirkninger under hjemmebehandlingen, skal øjeblikkeligt stoppe infusionen og søge lægehjælp.
English: Roll and tilt each vial gently.
Danish: Rul og vip hvert hætteglas forsigtigt.
English: Fabrazyme is indicated in adults, children and adolescents aged 8 years and older.
Danish: Fabrazyme er indiceret til voksne og børn og unge i alderen fra 8 år og derover.
English: - if you are allergic to agalsidase beta or any of the other ingredients of this medicine (listed in section 6).
Danish: - Hvis du er allergisk over for agalsidase beta eller et af de øvrige indholdsstoffer i Fabrazyme (angivet i punkt 6).
English: • sudden swelling of the face or throat
Danish: • pludselig hævelse i ansigt og hals
English: Clearance of GL-3 was also achieved in several other cell types from the kidney.
Danish: Der blev også opnået clearance af GL-3 i adskillige andre celletyper fra nyrerne.
English: This finding was further supported by a statistically significant decrease in GL-3 inclusions in kidney, heart and skin combined and in the individual organs in patients treated with agalsidase beta compared to placebo patients (p<0.001).
Danish: Dette resultat blev yderligere understøttet ved en statistisk signifikant reduktion af GL-3 inklusioner i nyrer, hjerte og hud samlet og i de enkelte organer hos patienter, der blev behandlet med agalsidase beta, sammenlignet med placebopatienter (p<0,001).
English: The risks and benefits of Fabrazyme in children aged 5 to 7 years have not yet been established and therefore no dose can be recommended for this age group.
Danish: Risici og fordele ved brug af Fabrazyme hos børn i alderen 5-7 år er endnu ikke klarlagt, og der kan derfor ikke anbefales nogen dosis til denne aldersgruppe.
English: The safety and efficacy of Fabrazyme in patients older than 65 years have not been established and no dosage regimen can presently be recommended in these patients.
Danish: Sikkerhed og effekt af Fabrazyme er ikke påvist hos patienter, som er ældre end 65 år, og ingen dosisplan kan endnu anbefales for disse patienter.
English: Fabrazyme is given through a drip into a vein (by intravenous infusion).
Danish: Fabrazyme indgives gennem drop i en vene (intravenøs infusion).
English: In the dose finding study, the effects of 0.3, 1.0 and 3.0 mg/kg once every 2 weeks and 1.0 and 3.0 mg/kg once every 2 days were evaluated.
Danish: I det dosis-bestemmende studie blev virkningen af 0,3, 1,0 og 3,0 mg/kg én gang hver anden uge og 1,0 og 3,0 mg/kg én gang hver anden dag vurderet.
English: Pregnancy There are no adequate data from the use of agalsidase beta in pregnant women.
Danish: Graviditet Der foreligger ikke tilstrækkelige data om brugen af agalsidase beta hos gravide kvinder.
English: Renal function, as measured by glomerular filtration rate and serum creatinine, as well as proteinuria, remained stable in the majority of the patients.
Danish: Både nyrefunktion, målt ved glomerulær filtrationshastighed og serum-kreatinin, og proteinindhold i urinen forblev stabile hos hovedparten af patienterne.
English: PARTICULARS TO APPEAR ON THE OUTER PACKAGING OUTER CARTON (1 VIAL, 5 VIALS, 10 VIALS)
Danish: MÆRKNING, DER SKAL ANFØRES PÅ DEN YDRE EMBALLAGE YDRE EMBALLAGE (1 HÆTTEGLAS, 5 HÆTTEGLAS, 10 HÆTTEGLAS)
English: In clinical trials doses up to 3 mg/kg body weight were used.
Danish: Ved kliniske studier blev der anvendt op til 3 mg/kg legemsvægt.
English: A. MANUFACTURER(S) OF THE BIOLOGICAL ACTIVE SUBSTANCE(S) AND MANUFACTURER(S) RESPONSIBLE FOR BATCH RELEASE
Danish: A. FREMSTILLER AF DET BIOLOGISK AKTIVE STOF OG FREMSTILLERE ANSVARLIGE FOR BATCHFRIGIVELSE
English: List of other side effects:
Danish: Liste over bivirkninger:
English: Incidence of adverse reactions with Fabrazyme treatment System organ class
Danish: Forekomsten af bivirkninger under Fabrazyme-behandling Systemorganklasse
English: In clinical studies side effects were mainly seen while patients were being given the medicine or shortly after (“infusion related reactions”).
Danish: I kliniske studier er bivirkningerne hovedsagelig set, mens patienterne fik medicinen, eller kort tid efter (”infusionsrelaterede reaktioner”).
English: Over time, the majority of seropositive patients in clinical trials demonstrated either a downward trend in titers (based on a ≥ 4-fold reduction in titer from the peak measurement to the last measurement) (40% of the patients), tolerised (no detectable antibodies confirmed by 2 consecutive radioimmunoprecipitation (RIP) assays) (14% of the patients) or demonstrated a plateau (35% of the patients).
Danish: Med tiden oplevede størsteparten af de seropositive patienter i kliniske studier en faldende tendens i antistofniveau (baseret på en ≥ 4 gange reduktion i antistofniveau fra den højeste værdi til den seneste værdi)(40 % af patienterne) tolerans (ingen påviselige antistoffer bekræftet ved to på hinanden følgende radioimmunfældningsassays (RIP)) (14 % af patienterne) eller opnåelse af plateau (35 % af patienterne).
English: In some of these patients, an increase of some of the following symptoms was spontaneously reported: pain, paraesthesia and diarrhoea, as well as cardiac, central nervous system and renal manifestations.
Danish: Hos nogle af disse patienter blev følgende symptomer rapporteret spontant: smerte, parestæsi og diarré samt manifestationer fra hjerte, centralnervesystem og nyrer.
English: Anaphylactoid reactions have been reported in the postmarketing setting.
Danish: Anafylaktoide reaktioner er blevet indberettet efter markedsføringen.
English: Consequently, impaired liver function is not expected to affect the pharmacokinetics of agalsidase beta in a clinically significant way.
Danish: Følgelig forventes nedsat leverfunktion ikke at påvirke farmakokinetikken for agalsidase beta på nogen klinisk signifikant måde.
English: The infusion-associated reactions were managed by a reduction in the infusion rate together with the administration of non-steroidal anti-inflammatory medicinal products, antihistamines and/or corticosteroids.
Danish: De infusionsrelaterede reaktioner blev afhjulpet gennem en reduktion af infusionshastigheden sammen med indgivelse af non-steroide antiinflammatoriske lægemidler, antihistaminer og/eller kortikosteroider.
English: Then slowly inject the reconstituted solution directly into the 0.9% sodium chloride intravenous solution (not in any remaining airspace) to a final concentration between 0.05 mg/ml and 0.7 mg/ml.
Danish: Dernæst injiceres det rekonstituerede koncentrat langsomt direkte i 0,9 % natriumchloridinfusionsvæske (ikke ind i et eventuelt resterende luftrum) til en slutkoncentration mellem 0,05 mg/ml og 0,7 mg/ml.
English: Determine the total volume of sodium chloride 0.9% solution for infusion (between 50 and 500 ml) based on the individual dose.
Danish: Bestem det totale volumen af 0,9 % natriumchlorid-infusionsvæske (mellem 50 og 500 ml) på basis af den individuelle dosis.
English: The rate of clinical events was substantially lower among Fabrazyme-treated patients compared to placebo-treated patients (risk reduction = 53% intent-to-treat population (p=0.0577); risk reduction = 61 % per-protocol population (p=0.0341)).
Danish: Frekvensen for kliniske hændelser var betydelig lavere blandt patienter, der blev behandlet med Fabrazyme, end hos de patienter, der blev behandlet med placebo (risikoreduktion = 53 % intent-totreat population (p=0.0577); Risikoreduktion = 61 % pr. protokol population (p=0.0341).
English: The reconstituted solution must be diluted further (see section 6.6).
Danish: Det rekonstituerede koncentrat skal fortyndes yderligere (se pkt.
English: In an additional study, 21 male patients were enrolled to follow GL3 clearance in kidney and skin tissues at an alternative dosing regimen.
Danish: I et supplerende studie blev 21 mandlige patienter inkluderet for at følge GL3-clearance i nyre- og hudvæv ved et alternativt doseringsregime.
English: After intravenous infusion, agalsidase beta is rapidly removed from the circulation and taken up by vascular endothelial and parenchymal cells into lysosomes, likely through the mannose-6 phosphate, mannose and asialoglycoprotein receptors.
Danish: Efter intravenøs infusion fjernes agalsidase beta hurtigt fra kredsløbet og optages af vaskulære endoteliale og parenkymale celler i lysosomer, sandsynligvis gennem mannose-6-fosfat-, mannose- og asialoglykoprotein-receptorer.
English: For instructions on reconstitution and dilution of the medicinal product before administration, see section 6.6.
Danish: Administration For instruktioner om rekonstitution og fortynding af lægemidlet før administration, se pkt.
English: The results of these studies indicate that Fabrazyme treatment at 1 mg/kg every other week provides clinical benefit on key clinical outcomes in patients with early and advanced Fabry disease.
Danish: Resultaterne af disse studier viser, at behandling med Fabrazyme med 1 mg/kg hver anden uge er klinisk fordelagtigt i forhold til centrale kliniske parametre hos patienter med tidlig eller fremskreden Fabrys sygdom.
English: Agalsidase beta is a recombinant form of human α-galactosidase A and is produced by recombinant DNA technology using a mammalian Chinese Hamster Ovary (CHO) cell culture.
Danish: Agalsidase beta er en rekombinant form af human α-galaktosidase og fremstilles ved rekombinant DNA-teknologi i en kultur af ovarieceller fra kinesiske hamstere (CHO).
English: These patients should be treated with caution when re-administering agalsidase beta (see section 4.8).
Danish: Disse patienter skal behandles med forsigtighed, når agalsidase beta genadministreres (se pkt.
English: In the postmarketing setting, experience was gained in patients who initiated treatment at a dose of 1 mg/kg every 2 weeks and subsequently received a reduced dose for an extended period.
Danish: Efter markedsføring blev erfaring indsamlet fra patienter, der indledte behandling med en dosis på 1 mg/kg hver 2. uge og efterfølgende fik en reduceret dosis i en længere periode.
English: After IgG seroconversion, there was no apparent change in CL (+24% and +6%, resp.), while Vss was 1.8 and 2.2 fold higher, with the net effect being a small decrease in Cmax (up to -34% and -11%, resp.) and no change in AUC (-19% and6%, resp.).
Danish: Efter IgGserokonvertering sås ingen tydelig ændring i CL (henholdsvis +24 % og +6 %), hvorimod Vss var 1,8 og 2,2 gange højere, og nettoeffekten var en lidt lavere Cmax (op til henholdsvis -34 % og -11 %) og ingen ændring i AUC (henholdsvis -19 % og -6 %).
English: For doses lower than 35 mg use a minimum of 50 ml, for doses 35 to 70 mg use a minimum of 100 ml, for doses 70 to 100 mg use a minimum of 250 ml and for doses greater than 100 mg use only 500 ml.
Danish: For doser under 35 mg bruges mindst 50 ml, for doser mellem 35 og 70 mg bruges mindst 100 ml, for doser mellem 70 og 100 mg bruges mindst 250 ml, og for doser over 100 mg bruges altid 500 ml.
English: Renal elimination of agalsidase beta is considered to be a minor pathway for clearance.
Danish: Renal udskillelse af agalsidase beta betragtes som en mindre væsentlig clearancevej.
English: Currently available data are described in sections 5.1 and 5.2 but no recommendation on posology can be made in children aged 5 to 7 years.
Danish: 5.1 og 5.2, men der kan ikke gives nogen anbefalinger vedrørende dosering hos børn i alderen 5-7 år.
English: After reconstitution it is a clear, colourless liquid, free from foreign matter.
Danish: Efter rekonstitution er det en klar, farveløs væske uden fremmede partikler.
English: • pain at the extremities
Danish: • smerter i ekstremiteter
English: The frequency of IARs decreased over time.
Danish: Hyppigheden af IAR'er faldt med tiden.
English: Patients experiencing mild or moderate infusion-associated reactions when treated with agalsidase beta during clinical trials have continued therapy after a reduction in the infusion rate (~0.15 mg/min; 10 mg/hr) and/or pre-treatment with antihistamines, paracetamol, ibuprofen and/or corticosteroids.
Danish: Patienter i kliniske studier som oplevede milde eller moderate infusionsrelaterede reaktioner under behandling med agalsidase beta fortsatte behandlingen efter en reduktion af infusionshastigheden (~0,15 mg/min; 10 mg/t) og/eller forbehandling med antihistaminer, paracetamol, ibuprofen og/eller kortikosteroider.
English: If you forget to use Fabrazyme
Danish: Hvis du har glemt at bruge Fabrazyme
English: Remove the airspace within the infusion bag to minimize the air/liquid interface.
Danish: Fjern luften inden i infusionsposen for at mindske luft/væske berøringsfladen.
English: In the first placebo-controlled clinical trial, Fabrazyme was effective in clearing GL-3 from the vascular endothelium of the kidney after 20 weeks of treatment.
Danish: I et placebokontrolleret klinisk studie var Fabrazyme effektivt til clearance af GL-3 fra det vaskulære endotel i nyrerne efter 20 ugers behandling.
English: Patients with antibodies to r-hαGAL have a greater potential to experience infusion-associated reactions (IARs), which are defined as any related adverse event occurring on the infusion day.
Danish: Patienter med antistoffer mod r-hαGAL har en større risiko for at udvikle infusionsrelaterede reaktioner (IAR'er), hvilket defineres som enhver relateret uønsket hændelse som opstår på infusionsdagen.
English: Reduced or absent αgalactosidase activity results in the accumulation of GL-3 in the lysosomes of many cell types including the endothelial and parenchymal cells, ultimately leading to life-threatening clinical deteriorations as a result of renal, cardiac and cerebrovascular complications.
Danish: Reduceret eller helt manglende α-galaktosidase-aktivitet medfører akkumulation af GL-3 i lysosomerne af mange celletyper, herunder endotel- og parenkymale celler, hvilket i sidste ende forårsager livstruende, klinisk forværring som følge af nyre-, hjerte- og cerebrovaskulære komplikationer.
English: Fabrazyme may have a minor influence on the ability to drive or use machines on the day of Fabrazyme administration because dizziness, somnolence, vertigo and syncope may occur (see section
Danish: På administrationsdagen kan Fabrazyme i mindre grad påvirke evnen til at føre motorkøretøj eller betjene maskiner, da der kan optræde svimmelhed, døsighed, vertigo og synkope (se pkt.
English: If you experience any serious side effect, you should contact your doctor immediately.
Danish: Hvis du oplever en alvorlig bivirkning, skal du kontakte din læge med det samme.
English: Due to the limitations of the study design (small number of patients), no definitive conclusion regarding the dose maintenance regimen can be drawn, but these findings suggest that, after an initial debulking dose of 1.0 mg/kg every 2 weeks, 0.3 mg/kg every 2 weeks may be sufficient in some patients to maintain clearance of GL-3.
Danish: På grund af studiedesignets begrænsninger (få patienter) kan der ikke uddrages nogen definitiv konklusion med hensyn til dosisvedligeholdelsesregimet, men disse resultater antyder, at efter en første dosis på 1,0 mg/kg hver anden uge kan 0,3 mg/kg hver anden uge måske være tilstrækkeligt hos nogle patienter til at opretholde GL-3 clearance.
English: Non-clinical data reveal no special hazard for humans based on studies of safety pharmacology, single dose toxicity, repeated dose toxicity and embryonal/foetal toxicity.
Danish: De prækliniske data viser ingen særlig risiko for mennesker vurderet ud fra konventionelle undersøgelser af sikkerhedsfarmakologi, enkeltdosis-toksikologi, toksicitet efter gentagne doser, samt reproduktionstoksicitet.
English: In this trial, the initial rechallenge administration was at a low dose and a lower infusion rate (1/2 the therapeutic dose at 1/25 the initial standard recommended rate).
Danish: I dette studie blev Fabrazyme readministreret med en lav dosis og en nedsat infusionshastighed (1/2 af den terapeutiske dosis med 1/25 af den anbefalede indledende standardhastighed).
English: • decreased mouth sensitivity
Danish: • nedsat følsomhed i munden
English: The closure consists of a siliconised butyl stopper and an aluminium seal with a plastic flip-off cap.
Danish: Lukningen består af silikonerede butylpropper og en aluminiumforsegling med en plasthætte, der kan vippes af.
English: Uncommon (may affect up to 1 in 100 people): • tremor
Danish: Ikke almindelig (kan forekomme hos op til 1 ud af 100 personer): • rysten
English: The educational material for patients should include information on the following key elements: - The prescribing physician may decide that Fabrazyme may be administered at home.
Danish: Uddannelsesmaterialet til patienter skal omfatte oplysninger om følgende væsentlige elementer: - Den ordinerende læge kan bestemme, at Fabrazyme kan gives som hjemmebehandling.