FrancophonIA/ELRC3.0 huggingface dataset:
English: • Since no preservative is present, it is recommended that administration is started as soon as possible after dilution.
Danish: • Da der ikke er tilsat konserveringsmiddel, anbefales det, at indgift påbegyndes så hurtigt som muligt efter fortynding.
English: Clearance normalised for body weight was 2.66 and 2.10 ml/min/kg for males and females, respectively.
Danish: Clearance normaliseret for legemsvægt var 2,66 og 2,10 ml/min/kg for henholdsvis mænd og kvinder.
English: Tell your doctor if you experience any side effects whilst having the infusion.
Danish: Fortæl det til lægen, hvis De får bivirkninger under infusionen.
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.
Danish: Replagal bør ikke indgives sammen med chloroquin, amiodaron, benoquin eller gentamicin, da disse stoffer kan hæmme intracellulær aktivitet af α-galactosidase.
English: This was associated with a significant reduction in chronic neuropathic pain medication use and number of days on pain medication.
Danish: Dette blev sammenkædet med en signifikant reduktion i medicin forbrug for kronisk neuropatisk smerte og antal dage med smertemedicinering.
English: The presence of extensive renal damage may limit the renal response to enzyme replacement therapy, possibly due to underlying irreversible pathological changes.
Danish: Forekomsten af omfattende nyreskade kan begrænse det renale respons på enzym-substitutionsterapi, eventuelt på grund af basale, irreversible patologiske forandringer.
English: In such cases, the loss of renal function remains within the expected range of the natural progression of disease.
Danish: I sådanne tilfælde vil forringelsen af nyrefunktionen forblive af samme størrelsesorden som ved den naturlige sygdomsprogression.
English: Replagal is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry Disease (α-galactosidase A deficiency).
Danish: Replagal er indiceret til langtids-enzym-substitutionsterapi i patienter med en bekræftet diagnose af Fabrys sygdom (α-galactosidase A-mangel).
English: Following six months of Replagal treatment 12 of 28 male patients showed altered pharmacokinetics including an apparent increase in clearance.
Danish: Efter seks måneders behandling med Replagal udviste 12 af 28 mandlige patienter en ændret farmakokinetik, herunder en åbenlyst hurtigere clearance.
English: In clinical studies, neuropathic pain medicinal products (such as carbamazepine, phenytoin and gabapentin) were administered concurrently to most patients without any evidence of interaction.
Danish: I kliniske studier blev lægemidler mod neuropatisk smerte (såsom carbamazepin, phenytoin og gabapentin) indgivet samtidig til de fleste patienter uden tegn på interaktion.
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.
Danish: Det anvendes som langtidsvarende enzymsubstitueringsterapi, når mængden af enzym i kroppen er lavere end normalt, således som det ses ved Fabrys sygdom.
English: After dilution Replagal is given in a vein.
Danish: Efter fortynding indgives Replagal i en vene.
English: It is produced by switching on the gene for α-galactosidase A in cells.
Danish: Det fremstilles ved at tænde for genet for α-galaktosidase A i celler.
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.
Danish: Antistoffer mod agalsidase alfa er aldrig vist at være associeret med nogen form for klinisk signifikante effekter på sikkerhed (f.eks. infusionsreaktioner) eller virkning.
English: Agalsidase alfa is a form of the human enzyme α-galactosidase.
Danish: Agalsidase alfa er en form af det humane enzym α-galactosidase.
English: 13.7% of adult patients treated with Replagal in clinical trials have experienced idiosyncratic infusion related reactions.
Danish: 13,7% af voksne patienter, der blev behandlet med Replagal i kliniske studier, har oplevet idiosynkratiske infusionsrelaterede reaktioner.
English: Hypersensitivity reactions have been reported.
Danish: Overfølsomhedsreaktioner er blevet rapporteret.
English: Administer the infusion solution over a period of 40 minutes using an intravenous line with an integral filter.
Danish: Indgiv infusionsvæsken over en periode på 40 minutter via en intravenøs adgang med et integreret filter.
English: In a second study, fifteen patients with left ventricular hypertrophy completed a 6 month placebocontrolled study and entered an extension study.
Danish: I et andet studie afsluttede 15 patienter med hypertrofi af venstre ventrikel et 6 måneders placebokontrolleret studie og indgik i et vedligeholdelsesstudie.
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%).
Danish: Infusionsrelaterede reaktioner er set hos 9 patienter (31,0 %) i den naive population sammenlignet med 27 patienter (38,0 %) i den population, der skiftede behandling.
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).
Danish: De farmakokinetiske data tyder på, at ved en dosis på 0,2 mg/kg Replagal er reduktionerne i plasma-Gb3 mere eller mindre sammenlignelige blandt unge og små børn (se pkt.
English: What you need to know before Replagal is given
Danish: Det skal De vide, før De får Replagal
English: Following a single intravenous dose of 0.2 mg/kg, agalsidase alfa had a biphasic distribution and elimination profile from the circulation.
Danish: Efter en enkelt intravenøs dosis på 0,2 mg/kg havde agalsidase alfa en bifasisk fordelings- og elimineringsprofil fra kredsløbet.
English: No increase in the incidence of adverse events was detected for this patient.
Danish: Der sås ingen stigning i forekomsten af bivirkninger hos denne patient.
English: Uncommon: may affect up to 1 in 100 people: - severe allergic (anaphylactic-type) reaction
Danish: Ikke almindelige: forekommer hos op til 1 ud af 100 behandlede personer: - alvorlig allergisk (anafylaktisk) reaktion
English: Since no preservative is present, it is recommended that administration is started as soon as possible.
Danish: Da der ikke er noget konserveringsmiddel i, anbefales det, at indgift påbegyndes så hurtigt som muligt.
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.
Danish: Disse ændringer blev sammenkædet med udviklingen af lave antistoftitre imod agalsidase alfa, men der observeredes ingen klinisk signifikant effekt hvad angår sikkerhed eller virkning hos de undersøgte patienter.
English: The metabolic effects were also associated with clinically significant weight gain, increased sweating and increased energy.
Danish: De metaboliske effekter var også associeret med klinisk signifikant vægtøgning, øget svedsekretion og mere energi.
English: However, the chemical and physical stability of the diluted solution has been demonstrated for 24 hours at 25°C.
Danish: Kemisk og fysisk stabilitet af den fortyndede opløsning er imidlertid demonstreret i 24 timer ved 25oC.
English: Your treatment will be supervised by a doctor who specialises in the treatment of Fabry Disease.
Danish: De vil blive behandlet under vejledning af en læge, som er specialiseret i behandling af Fabrys sygdom.
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.
Danish: Baseret på lighederne i farmakokinetiske egenskaber af agalsidase alfa hos både mænd og kvinder formodes vævsfordelingen i større væv og organer også at være sammenlignelig hos mandlige og kvindelige patienter.
English: Patients received the recommended dosage of 0.2 mg/kg of Replagal.
Danish: Patienterne fik den anbefalede dosis af 0,2 mg/kg Replagal.
English: The most commonly reported adverse reactions were infusion associated reactions, which occurred in 13.7% of adult patients treated with Replagal in clinical trials.
Danish: De hyppigst rapporterede bivirkninger var infusionsrelaterede reaktioner, som optrådte hos 13,7% af voksne patienter, der blev behandlet med Replagal i kliniske studier.
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.
Danish: Da metaboliseringen forventes at ske ved peptidhydrolyse, forventes nedsat leverfunktion ikke at påvirke farmakokinetikken af agalsidase alfa klinisk signifikant.
English: In the absence of compatibility studies this medicinal product must not be mixed with other medicinal products.
Danish: Da der ikke foreligger studier af eventuelle uforligeligheder, må Replagal ikke blandes med andre lægemidler.
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).
Danish: I kliniske studier med børn (7-18 år), der fik Replagal 0,2 mg/kg hver anden uge, blev der ikke konstateret nogen uventede sikkerhedsmæssige problemer (se pkt.
English: In this study, only one patient had LVH at baseline and remained stable over time.
Danish: I dette studie havde kun én patient venstre ventrikel hypertrofi ved baseline, og vedkommende forblev stabil over tid.
English: Table 1 lists adverse reactions reported for the 177 patients treated with Replagal in clinical trials, including 21 patients with history of end stage renal disease, 24 paediatric patients (7 to 17 years of age) and 17 female patients, and from post-marketing spontaneous reports.
Danish: Tabel 1 angiver bivirkninger rapporteret hos 177 patienter behandlet med Replagal i kliniske studier, herunder 21 patienter med tidligere tilfælde af nyresygdom i sidste stadie, 24 pædiatriske patienter (7 til 17 år) og 17 kvindelige patienter, og spontant indberettet efter markedsføring.
English: Agalsidase alfa is unlikely to be a candidate for drug-drug interactions.
Danish: Agalsidase alfa er sandsynligvis ikke kandidat for lægemiddel-lægemiddel-interaktioner.
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.
Danish: Replagal anvendes til behandling af voksne patienter samt børn og unge i alderen 7 år og opefter med en bekræftet diagnose på Fabrys sygdom.
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.
Danish: Studie hos patienter, der skifter fra agalsidase beta til Replagal (agalsidase alfa) 100 patienter [behandlingsnaive (n = 29) eller tidligere behandlet med agalsidase beta, som skiftede til Replagal (n = 71)] blev behandlet i op til 30 måneder i et åbent, ikke-kontrolleret studie.
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.
Danish: Valget af dosen på 0,2 mg/kg (indgivet over 40 min.) i de kliniske registreringsstudier var beregnet på midlertidigt at mætte mannose-6-fosfat-receptorernes evne til at internalisere agalsidase alfa i leveren og muliggøre fordelingen af enzymet til andre relevante organvæv.
English: The safety and efficacy of Replagal was assessed in two randomised, double blind, placebo controlled studies and open label extension studies, in a total of forty patients with a diagnosis of Fabry Disease based on clinical and biochemical evidence.
Danish: Sikkerheden og virkningen af Replagal blev vurderet i to randomiserede, dobbeltblindede, placebokontrollerede studier og åbne ekstensionsstudier i 40 patienter i alt med en diagnosticeret Fabrys sygdom baseret på klinisk og biokemisk evidens.
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.
Danish: En analyse viste, at der blev indberettet alvorlige bivirkninger hos 39,4 % af de patienter, der skiftede fra agalsidase beta, sammenlignet med hos 31,0 % af de patienter, der var behandlingsnaive før deltagelse i studiet.
English: Two patients with right bundle branch block in the studies conducted reverted to normal following therapy with Replagal.
Danish: To patienter med højre grenblok under studierne vendte tilbage til normaltilstanden efter behandling med Replagal.
English: Renal elimination of agalsidase alfa is considered to be a minor clearance pathway since pharmacokinetic parameters are not altered by impaired renal function.
Danish: Renal elimination af agalsidase alfa antages at være en mindre væsentlig clearancevej, da de farmakokinetiske parametre ikke ændres ved nedsat nyrefunktion.
English: Hypersensitivity, including anaphylaxis, has been reported.
Danish: Overfølsomhed, herunder anafylaksi, er blevet rapporteret.
English: Treatment with the enzyme has been shown to reduce accumulation of Gb3 in many cell types including endothelial and parenchymal cells.
Danish: Behandling med enzymet er vist at reducere akkumulation af Gb3 i mange celletyper inklusive endoteliale og parenkymale celler.
English: Replagal was also associated with improved myocardial contractility, a decrease in mean QRS duration and a concomitant decrease in septal thickness on echocardiography.
Danish: Replagal blev også associeret med forbedret myokardiel kontraktion, en reduktion i den gennemsnitlige QRSvarighed og en samtidig reduktion i septumtykkelse målt ved ekkokardiografi.
English: The presence of extensive renal damage (eGFR <60mL/min) may limit the renal response to enzyme replacement therapy.
Danish: Forekomst af omfattende nyrebeskadigelse (estimeret GFR (eGFR) <60 ml/min) kan begrænse nyrernes reaktion på enzym-substitutionsterapi.
English: Metabolism and nutrition disorders peripheral oedema
Danish: Metabolisme og ernæring perifert ødem
English: Fabry Disease is a glycosphingolipid storage disorder that is caused by deficient activity of the lysosomal enzyme α-galactosidase A, resulting in accumulation of globotriaosylceramide (Gb3 or GL-3, also known as ceramidetrihexoside (CTH)), the glycosphingolipid substrate for this enzyme.
Danish: Fabrys sygdom er en forstyrrelse i glycosphingolipid-deponering, som er forårsaget af en mangelfuld aktivitet af det lysosomale enzym α-galactosidase A, resulterende i akkumulation af globotriaosylceramid (Gb3 eller GL-3, også betegnet ceramidtrihexosid (CTH)), glycosphingolipid substratet for dette enzym.
English: *agalsidase alfa is the human protein α-galactosidase A produced in a human cell line by genetic engineering technology.
Danish: *agalsidase alfa er det humane protein α-galaktosidase A fremstillet ved genteknologi i en human cellelinje.
English: In male paediatric Fabry patients ≥ 7 years of age, hyperfiltration can be the earliest manifestation of renal involvement in the disease.
Danish: Hos mandlige pædiatriske Fabry-patienter ≥7 år kan hyperfiltrering være den tidligste manifestation af, at nyrerne er involveret i sygdommen.
English: Agalsidase alfa catalyses the hydrolysis of Gb3, cleaving a terminal galactose residue from the molecule.
Danish: Agalsidase alfa katalyserer hydrolysen af Gb3, idet det fjerner en terminalgalaktose-rest fra molekylet.
English: Replagal treatment should be supervised by a physician experienced in the management of patients with Fabry Disease or other inherited metabolic diseases.
Danish: Behandling med Replagal skal ske under supervision af en læge med erfaring i behandling af patienter med Fabrys sygdom eller andre arvelige metaboliske sygdomme.
English: After 12 to 18 months treatment a reduction of 5080% was observed in plasma and urine sediment.
Danish: Efter 12 til 18 måneders behandling sås en reduktion på 50-80% i plasma og urinsediment.
English: Talk to your doctor or pharmacist before using Replagal.
Danish: Tal med lægen eller apotekspersonalet, før De tager Replagal.
English: If you notice any of these effects during or after an infusion you should tell your doctor immediately: - high fever, chills, sweating, fast heart rate; - vomiting; - light-headedness; - hives;
Danish: Hvis De bemærker nogen af nedenstående bivirkninger under eller efter en infusion, skal De straks give Deres læge besked: - høj feber, kulderystelser, øget svedtendens, hurtig hjerterytme (puls); - opkastning;
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.
Danish: Efter 12 til 54 måneders terapi var 17% af de Replagal-behandlede patienter stadig antistof-positive, hvorimod 7% udviste tegn på udvikling af immunologisk tolerance vurderet på baggrund af reduktionen af IgGantistoffer med tiden.
English: Ear and labyrinth disorders tinnitus, tinnitus aggravated
Danish: Øre og labyrint tinnitus, forværring af tinnitus
English: Other medicines and Replagal
Danish: Brug af anden medicin sammen med Replagal
English: For patients between 0 and 7 years of age, limited data indicate no specific safety issues.
Danish: Begrænsede data tyder ikke på nogen specifikke sikkerhedsmæssige problemer hos patienter mellem 0 og 7 år.
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.
Danish: Nyrebiopsiprøver viste en signifikant stigning i andelen af normale glomeruli og et signifikant fald i andelen af glomeruli med mesangial udvidelse hos patienter behandlet med Replagal i modsætning til patienterne behandlet med placebo.
English: In a second study, in 14 patients ≥ 7 years of age, the results regarding heart rate variability were consistent with previous findings.
Danish: I et andet studie med deltagelse af 14 patienter i alderen 7 år og derover stemte resultaterne vedrørende variabilitet i hjertefrekvens overens med tidligere fund.
English: Each ml of Replagal contains 1 mg of agalsidase alfa.
Danish: Hver ml Replagal indeholder 1 mg agalsidase alfa.
English: No studies have been conducted with respect to parturition or peri/post-natal development.
Danish: Der er ikke gennemført studier med hensyn til påvirkning af fødslen eller den peri-/postnatale udvikling.
English: The following adverse reactions have been identified for agalsidase alfa:
Danish: Følgende bivirkninger er identificeret for agalsidase alfa:
English: Chemical and physical in use stability has been demonstrated for 24 hours at 25°C.
Danish: Den kemiske og fysiske stabilitet efter fortynding er blevet påvist i 24 timer ved 25oC.
English: Three of 8 (37.5%) paediatric patients <7 years of age experienced at least one infusion related reaction over a mean observation time of 4.2 years.
Danish: Tre ud af 8 (37,5%) pædiatriske patienter <7 år oplevede mindst én infusionsrelateret reaktion i en gennemsnitlig observationstid på 4,2 år.
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.
Danish: Agalsidase alfa er fremstillet i en human cellelinje for at opnå en glycosyleringsprofil som hos mennesker, hvilket kan influere på optagelsen via mannose-6-fosfat-receptorer på overfladen af målcellerne.
English: Infusion related reactions involving the heart including heart rhythm problems, heart muscle ischemia and heart failure, may occur in patients with Fabry disease involving the heart structures (frequency “not known” (cannot be estimated from the available data)).
Danish: Infusionsrelaterede reaktioner, der berører hjertet, herunder problemer med hjerterytmen, manglende blodtilførsel til hjertemusklen og hjertesvigt, kan opstå hos patienter med Fabrys sygdom, hvor hjertet er påvirket (hyppigheden er ikke kendt (kan ikke vurderes ud fra tilgængelige data).
English: - if you are allergic to agalsidase alfa or any of the other ingredients of this medicine (listed in section 6).
Danish: -hvis De er allergisk over for agalsidase alfa eller et af de øvrige indholdsstoffer angivet i punkt 6.
English: Other infusion-related symptoms may include dizziness and hyperhidrosis.
Danish: Andre infusionsrelaterede reaktioner kan omfatte svimmelhed og hyperhidrose.
English: Mean clearance of Replagal in children aged (7-11 years), in adolescents (aged 12-18 years), and adults was 4.2 ml/min/kg, 3.1 ml/min/kg, and 2.3 ml/min/kg, respectively.
Danish: Den gennemsnitlige clearance af Replagal hos børn fra 7-11 år, unge fra 12-18 år og voksne var henholdsvis 4,2 ml/min/kg, 3,1 ml/min/kg og 2,3 ml/min/kg.
English: In each treatment group, LVMI remained stable over the treatment period of the study.
Danish: VVMI var stabilt i studiets behandlingsperiode i alle behandlingsgrupper.
English: Reproduction toxicity studies in female rats and rabbits have shown no effect on pregnancy or the developing foetus.
Danish: Studier på hunrotter og kaniner af reproduktionstoksicitet viste ingen effekt på graviditet eller fosterudvikling.
English: Longer term therapy (48-54 months) resulted in stabilisation of GFR in male patients with normal baseline GFR (≥ 90 mL/min/1.73 m2) and with mild to moderate renal dysfunction (GFR 60 to < 90 mL/min/1.73 m2), and in slowing of the rate of decline in renal function and progression to end-stage renal disease in male Fabry patients with more severe renal dysfunction (GFR 30 to < 60 mL/min/1.73 m2).
Danish: Længerevarende behandling (48-54 måneder) resulterede i en stabilisering af GFR hos mandlige patienter med normal GFR ved baseline (≥ 90 ml/min/1,73 m2) og med mild til moderat nyredysfunktion (GFR 60 til < 90 ml/min/1,73 m2) og i et langsommere fald i nyrefunktion og progression til nyresygdom i sidste stadie hos mandlige patienter med Fabrys sygdom med mere alvorlig nyredysfunktion (GFR 30 til < 60 ml/min/1,73 m2).
English: Pack sizes of 1, 4 or 10 vials.
Danish: Pakningstørrelser på 1, 4 og 10 hætteglas.
English: Single doses ranging from 0.007 - 0.2 mg enzyme per kg body weight were administered to adult male patients as 20 - 40 minute intravenous infusions while female patients received 0.2 mg enzyme per kg body weight as 40 minute infusions.
Danish: Der blev indgivet enkeltdoser varierende fra 0,007 til 0,2 mg enzym per kg legemsvægt til voksne mandlige patienter som 20 - 40 minutters intravenøse infusioner, medens kvindelige patienter fik 0,2 mg enzym per kg legemsvægt som 40 minutters infusioner.
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
Danish: LÆGEMIDDELFORM OG INDHOLD (PAKNINGSSTØRRELSE) 1 x 3,5 ml koncentrat til infusionsvæske, opløsning 4 x 3,5 ml koncentrat til infusionsvæske, opløsning 10 x 3,5 ml koncentrat til infusionsvæske, opløsning
English: For children and adolescents 7-18 years old a dose of 0.2 mg/kg every other week may be used.
Danish: For børn og unge i alderen 7-18 år anbefales en dosis på 0,2 mg/kg hver anden uge.