natgillin/translations-raw huggingface dataset:
Hvis behandlingen med Replagal får Deres krop til at danne antistoffer, vil det ikke forhindre, at Replagal virker, og antistofferne kan forsvinde med tiden.
If treatment with Replagal makes your body produce antibodies this will not stop Replagal from working and the antibodies may disappear with time.
4.7 Virkninger på evnen til at føre motorkøretøj og betjene maskiner
4.6 Effects on ability to drive and use machines
Replagal påvirker ikke eller kun i ubetydelig grad evnen til at føre motorkøretøj og betjene maskiner.
Replagal has no or negligible influence on the ability to drive and use machines.
Meget almindelige: forekommer hos flere end 1 ud af 10 behandlede personer: - generel smerte eller ubehag.
Very common: may affect more than 1 in 10 people - general pain or discomfort.
Brug ikke Replagal, hvis De bemærker misfarvning eller tilstedeværelse af andre fremmede partikler.
Do not use Replagal if you notice that there is discolouration or other foreign particles present.
Det forventes, at dets metabolisering følger samme rute som andre proteiner, dvs. peptidhydrolyse.
It is expected that its metabolic degradation will follow the pathways of other proteins, i.e. peptide hydrolysis.
Enzymet fjernes dernæst fra cellen og omdannes til et sterilt koncentrat til opløsning til infusion.
The enzyme is then removed from the cells and made into a sterile concentrate for solution for infusion.
Der findes begrænsede data fra patienter i dialyse eller efter nyretransplantation; dosisjustering anbefales ikke.
Limited data are available in patients on dialysis or post-kidney transplantation, no dose adjustment is recommended.
Deres læge kan også behandle symptomerne med andre lægemidler (antihistaminer eller kortikosteroider).
Your doctor may also treat the symptoms with other medicines (antihistamines or corticosteroids).
Idiosynkratiske infusionsrelaterede reaktioner
Idiosyncratic infusion related reactions
Den samlede incidens af alvorlige bivirkninger (SAE'er) pr. behandlingsgruppe viste ingen tydelig virkning af behandlingsregimet på SAE-profilen i de forskellige behandlingsgrupper.
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.
Hvis produktet ikke anvendes straks, er opbevaringstid og -betingelser efter fortynding og inden anvendelse brugerens eget ansvar og vil normalt ikke overstige 24 timer ved 2oC til 8oC, medmindre fortyndingen er foretaget under kontrollerede og aseptiske betingelser.
If not used immediately, in-use storage times and conditions prior to use are the responsibility of the user and would normally not be longer than 24 hours at 2 to 8°C, unless dilution has taken place in controlled and validated aseptic conditions.
En reduktion i deres hypernormale eGFR'er blev observeret inden for 6 måneder efter iværksættelse af behandling med Replagal.
Reduction in their hypernormal eGFRs was observed within 6 months of initiating Replagal therapy.
12 til 18 måneders behandling med Replagal medførte en forbedring af livskvalitet (QoL) målt ved hjælp af validerede instrumenter.
Twelve to 18 months of treatment with Replagal resulted in improvement in quality of life (QoL), as measured by validated instruments.
Hvis De har fremskreden nyresygdom, kan det være, at Replagal-behandlingen har en begrænset virkning på Deres nyrer.
If you have advanced renal disease, you may find that your Replagal treatment has a limited effect on your kidneys.
Antistoffer mod proteinet
Antibodies to the protein
Dyrestudier indikerer hverken direkte eller indirekte skadelige virkninger hvad angår graviditeten eller den embryonale/føtale udvikling ved eksponering under organogenesen (se pkt.
Animal studies do not indicate direct or indirect harmful effects with respect to pregnancy or embryonic/fetal development when exposed during organogenesis (see Section 5.3).
Efter fortynding bør opløsningen blandes blidt, men uden omrystning.
Once diluted, the solution should be mixed gently but not shaken.
Fortæl det til lægen, hvis De tager medicin, der indeholder chloroquin, amiodaron, benoquin eller gentamicin.
Tell your doctor if you use any medicines containing chloroquine, amiodarone, benoquin or gentamicin.
I sammenligning med placebo reducerede behandling med Replagal også akkumulation af Gb3.
Compared with placebo, treatment with Replagal also reduced accumulation of Gb3.
Data fra patienter tyder på, at en dosis på mindst 0,1 mg/kg er nødvendig for at opnå et farmakodynamisk respons.
Data with patients indicates that at least 0.1mg/kg is required to achieve a pharmacodynamics response.
Der er meget begrænsede data fra graviditeter, hvor kvinden har været eksponeret for Replagal.
There is very limited data on pregnancies exposed to Replagal.
Elimineringshalveringstider var 108 ± 17 minutter hos mænd sammenlignet med 89 ± 28 minutter hos kvinder, og fordelingsvolumen var ca. 17% legemsvægt hos begge køn.
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.
Meget begrænsede kliniske data om graviditeter, der er blevet eksponeret for Replagal, har ikke vist nogen bivirkninger på moderen og det nyfødte barn.
Very limited clinical data on pregnancies exposed to Replagal have shown no adverse effects on the mother and newborn child.
Lette og forbigående symptomer kræver ikke nødvendigvis medicinsk behandling eller afbrydelse af infusionen.
Mild and transient effects may not require medical treatment or discontinuation of the infusion.
De kan som oftest stadig kunne få Replagal, selvom De får disse symptomer.
Most of the time you can still be given Replagal even if these symptoms occur.
Patienter, der skiftede fra agalsidase beta til Replagal, havde en sikkerhedsprofil, der stemmer overens med den, der er observeret i andre kliniske studier.
Patients switched from agalsidase beta to Replagal had a safety profile consistent with that observed in other clinical experience.
Prækliniske data viser ingen speciel risiko for mennesker vurderet ud fra undersøgelser af toksicitet efter gentagne doser.
Non-clinical data reveal no special hazard for humans based on studies of repeated dose toxicity.
Et lav-titret IgG-antistofsvar er observeret hos ca. 24% af mandlige patienter behandlet med Replagal.
A low titre IgG antibody response has been observed in approximately 24% of the male patients treated with Replagal.
Ca. 1 ud af 7 patienter (meget almindelig bivirkning) kan have en reaktion under eller efter en infusion med Replagal (infusionsrelateret reaktion).
About 1 out of 7 patients (frequency “very common”) may have a reaction during or following an infusion of Replagal (infusion related reaction).
• Da der ikke er tilsat konserveringsmiddel, anbefales det, at indgift påbegyndes så hurtigt som muligt efter fortynding.
• Since no preservative is present, it is recommended that administration is started as soon as possible after dilution.
Clearance normaliseret for legemsvægt var 2,66 og 2,10 ml/min/kg for henholdsvis mænd og kvinder.
Clearance normalised for body weight was 2.66 and 2.10 ml/min/kg for males and females, respectively.
Fortæl det til lægen, hvis De får bivirkninger under infusionen.
Tell your doctor if you experience any side effects whilst having the infusion.
Replagal bør ikke indgives sammen med chloroquin, amiodaron, benoquin eller gentamicin, da disse stoffer kan hæmme intracellulær aktivitet af α-galactosidase.
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.
Dette blev sammenkædet med en signifikant reduktion i medicin forbrug for kronisk neuropatisk smerte og antal dage med smertemedicinering.
This was associated with a significant reduction in chronic neuropathic pain medication use and number of days on pain medication.
Forekomsten af omfattende nyreskade kan begrænse det renale respons på enzym-substitutionsterapi, eventuelt på grund af basale, irreversible patologiske forandringer.
The presence of extensive renal damage may limit the renal response to enzyme replacement therapy, possibly due to underlying irreversible pathological changes.
I sådanne tilfælde vil forringelsen af nyrefunktionen forblive af samme størrelsesorden som ved den naturlige sygdomsprogression.
In such cases, the loss of renal function remains within the expected range of the natural progression of disease.
Replagal er indiceret til langtids-enzym-substitutionsterapi i patienter med en bekræftet diagnose af Fabrys sygdom (α-galactosidase A-mangel).
Replagal is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry Disease (α-galactosidase A deficiency).
Efter seks måneders behandling med Replagal udviste 12 af 28 mandlige patienter en ændret farmakokinetik, herunder en åbenlyst hurtigere clearance.
Following six months of Replagal treatment 12 of 28 male patients showed altered pharmacokinetics including an apparent increase in clearance.
I kliniske studier blev lægemidler mod neuropatisk smerte (såsom carbamazepin, phenytoin og gabapentin) indgivet samtidig til de fleste patienter uden tegn på interaktion.
In clinical studies, neuropathic pain medicinal products (such as carbamazepine, phenytoin and gabapentin) were administered concurrently to most patients without any evidence of interaction.
Det anvendes som langtidsvarende enzymsubstitueringsterapi, når mængden af enzym i kroppen er lavere end normalt, således som det ses ved Fabrys sygdom.
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.
Efter fortynding indgives Replagal i en vene.
After dilution Replagal is given in a vein.
Det fremstilles ved at tænde for genet for α-galaktosidase A i celler.
It is produced by switching on the gene for α-galactosidase A in cells.
Antistoffer mod agalsidase alfa er aldrig vist at være associeret med nogen form for klinisk signifikante effekter på sikkerhed (f.eks. infusionsreaktioner) eller virkning.
Antibodies to agalsidase alfa have not been shown to be associated with any clinically significant effects on safety (e.g. infusion reactions) or efficacy.
Agalsidase alfa er en form af det humane enzym α-galactosidase.
Agalsidase alfa is a form of the human enzyme α-galactosidase.
13,7% af voksne patienter, der blev behandlet med Replagal i kliniske studier, har oplevet idiosynkratiske infusionsrelaterede reaktioner.
13.7% of adult patients treated with Replagal in clinical trials have experienced idiosyncratic infusion related reactions.
Overfølsomhedsreaktioner er blevet rapporteret.
Hypersensitivity reactions have been reported.
Indgiv infusionsvæsken over en periode på 40 minutter via en intravenøs adgang med et integreret filter.
Administer the infusion solution over a period of 40 minutes using an intravenous line with an integral filter.
I et andet studie afsluttede 15 patienter med hypertrofi af venstre ventrikel et 6 måneders placebokontrolleret studie og indgik i et vedligeholdelsesstudie.
In a second study, fifteen patients with left ventricular hypertrophy completed a 6 month placebocontrolled study and entered an extension study.
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.
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%).
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.
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).
Det skal De vide, før De får Replagal
What you need to know before Replagal is given
Efter en enkelt intravenøs dosis på 0,2 mg/kg havde agalsidase alfa en bifasisk fordelings- og elimineringsprofil fra kredsløbet.
Following a single intravenous dose of 0.2 mg/kg, agalsidase alfa had a biphasic distribution and elimination profile from the circulation.
Der sås ingen stigning i forekomsten af bivirkninger hos denne patient.
No increase in the incidence of adverse events was detected for this patient.
Ikke almindelige: forekommer hos op til 1 ud af 100 behandlede personer: - alvorlig allergisk (anafylaktisk) reaktion
Uncommon: may affect up to 1 in 100 people: - severe allergic (anaphylactic-type) reaction
Da der ikke er noget konserveringsmiddel i, anbefales det, at indgift påbegyndes så hurtigt som muligt.
Since no preservative is present, it is recommended that administration is started as soon as possible.
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.
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.
De metaboliske effekter var også associeret med klinisk signifikant vægtøgning, øget svedsekretion og mere energi.
The metabolic effects were also associated with clinically significant weight gain, increased sweating and increased energy.
Kemisk og fysisk stabilitet af den fortyndede opløsning er imidlertid demonstreret i 24 timer ved 25oC.
However, the chemical and physical stability of the diluted solution has been demonstrated for 24 hours at 25°C.
De vil blive behandlet under vejledning af en læge, som er specialiseret i behandling af Fabrys sygdom.
Your treatment will be supervised by a doctor who specialises in the treatment of Fabry Disease.
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.
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.
Patienterne fik den anbefalede dosis af 0,2 mg/kg Replagal.
Patients received the recommended dosage of 0.2 mg/kg of Replagal.
De hyppigst rapporterede bivirkninger var infusionsrelaterede reaktioner, som optrådte hos 13,7% af voksne patienter, der blev behandlet med Replagal i kliniske studier.
The most commonly reported adverse reactions were infusion associated reactions, which occurred in 13.7% of adult patients treated with Replagal in clinical trials.
Da metaboliseringen forventes at ske ved peptidhydrolyse, forventes nedsat leverfunktion ikke at påvirke farmakokinetikken af agalsidase alfa klinisk signifikant.
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.
Da der ikke foreligger studier af eventuelle uforligeligheder, må Replagal ikke blandes med andre lægemidler.
In the absence of compatibility studies this medicinal product must not be mixed with other medicinal products.
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.
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).
I dette studie havde kun én patient venstre ventrikel hypertrofi ved baseline, og vedkommende forblev stabil over tid.
In this study, only one patient had LVH at baseline and remained stable over time.
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.
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.
Agalsidase alfa er sandsynligvis ikke kandidat for lægemiddel-lægemiddel-interaktioner.
Agalsidase alfa is unlikely to be a candidate for drug-drug interactions.
Replagal anvendes til behandling af voksne patienter samt børn og unge i alderen 7 år og opefter med en bekræftet diagnose på Fabrys sygdom.
Replagal is used to treat adult patients, as well as adolescents and children from the age of 7, with confirmed diagnosis of Fabry Disease.
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.
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.
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.
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.
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.
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.
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.
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.
To patienter med højre grenblok under studierne vendte tilbage til normaltilstanden efter behandling med Replagal.
Two patients with right bundle branch block in the studies conducted reverted to normal following therapy with Replagal.
Renal elimination af agalsidase alfa antages at være en mindre væsentlig clearancevej, da de farmakokinetiske parametre ikke ændres ved nedsat nyrefunktion.
Renal elimination of agalsidase alfa is considered to be a minor clearance pathway since pharmacokinetic parameters are not altered by impaired renal function.
Overfølsomhed, herunder anafylaksi, er blevet rapporteret.
Hypersensitivity, including anaphylaxis, has been reported.
Behandling med enzymet er vist at reducere akkumulation af Gb3 i mange celletyper inklusive endoteliale og parenkymale celler.
Treatment with the enzyme has been shown to reduce accumulation of Gb3 in many cell types including endothelial and parenchymal cells.
Replagal blev også associeret med forbedret myokardiel kontraktion, en reduktion i den gennemsnitlige QRSvarighed og en samtidig reduktion i septumtykkelse målt ved ekkokardiografi.
Replagal was also associated with improved myocardial contractility, a decrease in mean QRS duration and a concomitant decrease in septal thickness on echocardiography.
Forekomst af omfattende nyrebeskadigelse (estimeret GFR (eGFR) <60 ml/min) kan begrænse nyrernes reaktion på enzym-substitutionsterapi.
The presence of extensive renal damage (eGFR <60mL/min) may limit the renal response to enzyme replacement therapy.
Metabolisme og ernæring perifert ødem
Metabolism and nutrition disorders peripheral oedema
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.
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.
*agalsidase alfa er det humane protein α-galaktosidase A fremstillet ved genteknologi i en human cellelinje.
*agalsidase alfa is the human protein α-galactosidase A produced in a human cell line by genetic engineering technology.
Hos mandlige pædiatriske Fabry-patienter ≥7 år kan hyperfiltrering være den tidligste manifestation af, at nyrerne er involveret i sygdommen.
In male paediatric Fabry patients ≥ 7 years of age, hyperfiltration can be the earliest manifestation of renal involvement in the disease.
Agalsidase alfa katalyserer hydrolysen af Gb3, idet det fjerner en terminalgalaktose-rest fra molekylet.
Agalsidase alfa catalyses the hydrolysis of Gb3, cleaving a terminal galactose residue from the molecule.
Behandling med Replagal skal ske under supervision af en læge med erfaring i behandling af patienter med Fabrys sygdom eller andre arvelige metaboliske sygdomme.
Replagal treatment should be supervised by a physician experienced in the management of patients with Fabry Disease or other inherited metabolic diseases.
Efter 12 til 18 måneders behandling sås en reduktion på 50-80% i plasma og urinsediment.
After 12 to 18 months treatment a reduction of 5080% was observed in plasma and urine sediment.
Tal med lægen eller apotekspersonalet, før De tager Replagal.
Talk to your doctor or pharmacist before using Replagal.
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;
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;
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.
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.
Øre og labyrint tinnitus, forværring af tinnitus
Ear and labyrinth disorders tinnitus, tinnitus aggravated
Brug af anden medicin sammen med Replagal
Other medicines and Replagal
Begrænsede data tyder ikke på nogen specifikke sikkerhedsmæssige problemer hos patienter mellem 0 og 7 år.
For patients between 0 and 7 years of age, limited data indicate no specific safety issues.
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.
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.