FrancophonIA/ELRC3.0 huggingface dataset:
English: Determine the number of vials to be reconstituted based on the individual patient's weight and remove the required vials from the refrigerator in order to allow them to reach room temperature (in approximately 30 minutes).
Danish: Bestem det antal hætteglas, der skal rekonstitueres, på basis af den enkelte patients vægt, og tag det nødvendige antal hætteglas ud af køleskabet, for at de kan opnå stuetemperatur (på ca. 30 minutter).
English: Sixty seven percent (67%) of the patients experienced at least one infusion-associated reaction.
Danish: Syvogtres procent (67 %) af patienterne oplevede mindst en infusionsrelateret reaktion.
English: Reactions suggestive of immediate (Type I) hypersensitivity have been reported in a small number of patients (see section
Danish: Reaktioner, der tyder på akut (type I) overfølsomhed, er blevet indberettet for et lille antal patienter (se pkt.
English: The safety and efficacy of Fabrazyme in children aged 0 to7 years have not yet been established.
Danish: Fabrazymes sikkerhed og virkning hos børn i alderen 0-7 år er endnu ikke klarlagt.
English: Do not drive or use machines if you experience dizziness, sleepiness, vertigo or fainting during or shortly after administration of Fabrazyme (see section 4).
Danish: Kør ikke bil eller motorcykel og lad være med at cykle eller arbejde med værktøj eller maskiner, hvis du bliver svimmel eller søvnig, eller hvis du besvimer under eller kort tid efter indgivelse af Fabrazyme (se punkt 4).
English: With careful rechallenge Fabrazyme has been re-administered to all 6 patients who tested positive for IgE antibodies or had a positive skin test to Fabrazyme in a clinical trial.
Danish: Ved forsigtig readministration er Fabrazyme blevet readministreret til alle 6 personer som testedes positive for IgE-antistoffer, eller som havde en positiv hudreaktion overfor Fabrazyme i et klinisk studie.
English: For single use only.
Danish: Kun til éngangsbrug.
English: Following an intravenous administration of agalsidase beta to adults at doses of 0.3 mg, 1 mg and 3 mg/kg body weight, the AUC values increased more than dose proportional, due to a decrease in clearance, indicating a saturated clearance.
Danish: Efter intravenøs indgivelse af agalsidase beta i doser til voksne på 0,3 mg, 1 mg og 3 mg/kg legemsvægt steg AUC-værdierne mere end dosisproportionalt på grund af en sænkning i clearance, hvilket tyder på en mættet clearance.
English: Very common symptoms (may affect more than 1 in 10 people) include chills, fever, feeling cold, nausea, vomiting, headache and abnormal feelings in the skin such as burning or tingling.
Danish: Meget almindelige bivirkninger (kan forekomme hos flere end 1 ud af 10 personer) omfatter kulderystelser, feber, kuldefornemmelse, kvalme, opkastning, hovedpine og unormale fornemmelser i huden som f.eks. brænden eller prikken.
English: Instructions for use - reconstitution, dilution and administration
Danish: Brugsanvisning - rekonstitution, fortynding og indgivelse
English: Fabrazyme may get into breast milk.
Danish: Fabrazyme kan udskilles i brystmælk.
English: After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions.
Danish: Når patientens tolerance er konstateret, kan infusionshastigheden øges gradvist ved efterfølgende infusioner.
English: Name and address of the manufacturer of the biological active substance
Danish: Navn og adresse på fremstilleren af det biologisk aktive stof Genzyme Corporation
English: The educational material for healthcare professionals should include information on the following key elements: - Guidance regarding patient evaluation and selection and organisational requirements for home infusion.
Danish: Undervisningsmaterialet til sundhedspersonalet skal omfatte oplysninger om følgende væsentlige elementer: - Vejledning vedrørende patientevaluering og -udvælgelse og de organisatoriske krav i forbindelse med hjemmeinfusion.
English: - That it is the responsibility of the prescribing physician to determine which patients may be suitable for home or self-administration of Fabrazyme.
Danish: - At det er den ordinerende læges ansvar at bestemme, hvilke patienter der kan være egnede til hjemme- eller selvbehandling med Fabrazyme.
English: - That it is the responsibility of the prescribing physician to provide appropriate training to the non-healthcare professional, such as the patient for self-administration or a caregiver who will administer the treatment at home, if the treating physician decides that this is appropriate.
Danish: - At det er den ordinerende læges ansvar at sørge for passende uddannelse af ikkesundhedspersonale som f.eks. patienter, der skal behandle sig selv, eller plejepersonale, der skal administrere behandlingen hjemme, hvis den behandlende læge bestemmer, at dette er hensigtsmæssigt.
English: The reconstituted solution contains 5 mg agalsidase beta per ml, and appears as a clear colourless solution.
Danish: Det rekonstituerede koncentrat indeholder 5 mg agalsidase beta per ml og fremstår som en klar farveløs opløsning.
English: Agalsidase beta is a protein and is expected to be metabolically degraded through peptide hydrolysis.
Danish: Agalsidase beta er et protein, der antages at blive metaboliseret gennem peptidhydrolyse.
English: Fabrazyme should not be administered with chloroquine, amiodarone, benoquin or gentamycin due to a theoretical risk of inhibition of intra-cellular α-galactosidase A activity.
Danish: Fabrazyme bør ikke indgives sammen med chloroquin, amiodaron , benoquin eller gentamicin på grund af en teoretisk risiko for hæmning af den intracellulære α-galaktosidase A-aktivitet.
English: Do not use this medicine after the expiry date which is stated on the label after ‘EXP'.
Danish: Brug ikke lægemidlet efter den udløbsdato, der står på pakningen efter Exp. Udløbsdatoen er den sidste dag i den nævnte måned.
English: The reconstituted solution cannot be stored and should be promptly diluted; only the diluted solution can be held for up to 24 hours at 2°C-8°C.
Danish: Den rekonstituerede opløsning kan ikke opbevares og bør øjeblikkeligt fortyndes; kun den fortyndede opløsning kan gemmes i op til 24 timer ved 2 °C - 8 °C.
English: Agalsidase beta may be excreted in milk.
Danish: Agalsidase beta kan måske udskilles i modermælk.
English: Once a patient tolerates the infusion, the dose may be increased to reach the therapeutic dose of 1 mg/kg and the infusion rate may be increased by slowly titrating upwards, as tolerated.
Danish: Når patienten tolererer infusionen, kan dosen øges til en terapeutisk dosis på 1 mg/kg opnås, og infusionshastigheden kan øges ved langsom optitrering, efter toleranceevne.
English: Following treatment with 1 mg/kg every other week for 24 weeks, a dose regimen of 0.3 mg/kg every 2 weeks for 18 months was able to maintain the clearance of cellular GL-3 in the capillary endothelium of the kidney, other kidney cell types and skin (superficial skin capillary endothelium) in the majority of patients.
Danish: Efter behandling med 1 mg/kg hver anden uge i 24 uger kunne 0,3 mg/kg hver anden uge i 18 måneder opretholde clearance for cellulær GL-3 i det kapillære endotel i nyrerne, andre typer nyreceller og huden (det kapillære endotel i huden) hos de fleste patienter.
English: Sustained clearance of GL-3 from kidney vascular endothelium upon agalsidase beta treatment was demonstrated further in the open label extension of this trial.
Danish: Vedvarende clearance af GL-3 fra det vaskulære endotel i nyrerne ved behandling med agalsidase beta blev yderligere bekræftet gennem en åben udvidelse af dette studie.
English: Common (may affect up to 1 in 10 people): • chest pain
Danish: Almindelig (kan forekomme hos op til 1 ud af 10 personer): • brystsmerter
English: Both baseline and 5-year kidney biopsies were obtained in a subset of 6 patients: in all, kidney capillary endothelium GL-3 scores were reduced to zero but highly variable effects were observed in podocyte GL-3, with a reduction in 3 patients.
Danish: Nyrebiopsier ved både baseline og ved år 5 var tilgængelige for en undergruppe af 6 patienter: hos alle 6 blev GL-3-scoren for det kapillære endotel i nyrerne reduceret til nul, omend en yderst varierende effekt blev observeret for podocyt-GL-3 med en reduktion hos 3 patienter.
English: Based on its metabolism, agalsidase beta is an unlikely candidate for cytochrome P450 mediated drug-drug interactions.
Danish: Baseret på lægemidlets metabolisme er det usandsynligt, at agalsidase beta vil medføre cytochrom P450-relateret lægemiddelinteraktion.
English: Ten (10) patients met per protocol dose increase criteria, two (2) had a dose increase to the recommended dose of 1.0 mg/kg every 2 weeks.
Danish: 10 patienter opfyldte protokolkriterierne for dosisstigning, hos 2 blev dosis øget til den anbefalede dosis på 1,0 mg/kg hver 2. uge.
English: Do not use the solution if foreign particles are observed or if the solution is discoloured.
Danish: Anvend ikke koncentratet, hvis der ses fremmede partikler, eller hvis det er misfarvet.
English: Contents of the pack and other information What Fabrazyme contains
Danish: Pakningsstørrelser og yderligere oplysninger Fabrazyme indeholder
English: Vss ranged from 8.3-40.8 l, plasma clearance from 119-345 ml/min and the mean elimination half-life from 80-120 minutes.
Danish: Fordelingsvolumen ved steady state (Vss) lå mellem 8,3-40,8 l, plasma-clearance mellem 119 og 345 ml/min og den gennemsnitlige eliminationshalveringstid mellem 80 og 120 minutter.
English: Patients with antibodies to r-hαGAL have a greater potential to experience infusion-associated reactions (IARs).
Danish: Patienter med antistoffer mod r-hαGAL har en større risiko for at udvikle infusionsrelaterede reaktioner (IAR'er).
English: • burning sensation
Danish: • brændende følelse
English: However, the effect of Fabrazyme treatment on the kidney function was limited in some patients with advanced renal disease.
Danish: Imidlertid var effekten af Fabrazyme behandling på nyrefunktionen begrænset hos nogle patienter med fremskreden nyresygdom.
English: Fabrazyme contains the active substance agalsidase beta and is used as enzyme replacement therapy in Fabry disease, where the level of α-galactosidase enzyme activity is absent or lower than normal.
Danish: Fabrazyme indeholder det aktive stof agalsidase beta og anvendes til behandling af Fabrys sygdom, hvor enzymet α-galaktosidase enten helt mangler, eller aktiviteten af enzymet er lavere end normalt.
English: Genzyme Europe B.V., Paasheuvelweg 25, 1105 BP Amsterdam, The Netherlands
Danish: Genzyme Europe B.V., Paasheuvelweg 25, 1105 BP Amsterdam, Holland
English: Superficial skin capillary endothelium GL-3 scores were reduced to zero or maintained at zero at all time points post-baseline upon treatment in 19/27 patients completing the study without a dose increase.
Danish: Under behandlingen blev GL-3-scoren for det overfladiske kapillære endotel i huden reduceret til eller holdt sig på nul på alle tidspunkter postbaseline hos 19/27 patienter, der fuldførte studiet uden dosisstigning.
English: The effect of Fabrazyme treatment on the kidneys may be limited in patients with advanced renal disease.
Danish: Virkning på nyrerne af behandlingen med Fabrazyme kan være begrænset hos patienter med fremskreden nyresygdom.
English: Fabrazyme is only used under the supervision of a doctor who is knowledgeable in the treatment of Fabry disease.
Danish: Fabrazyme anvendes kun under vejledning af en læge, der har erfaring med behandlingen af Fabrys sygdom.
English: The recommended dose of Fabrazyme is 1 mg/kg body weight administered once every 2 weeks as an intravenous infusion.
Danish: Den anbefalede dosis Fabrazyme er 1 mg/kg legemsvægt indgivet én gang hver 2. uge som intravenøs infusion
English: Clearance of GL-3 in the superficial skin vascular endothelium was achieved in all patients who had accumulated GL-3 at baseline.
Danish: Clearance af GL-3 fra det overfladiske vaskulære endotel i huden blev opnået hos alle de patienter, som havde ophobet GL-3 ved studiestart.
English: Adverse reactions were mostly mild to moderate in severity:
Danish: Bivirkningerne var oftest lette til moderate i sværhedsgrad.
English: The 2 female patients had little or no GL-3 accumulation in the superficial skin vascular endothelium at baseline, making this conclusion applicable in male patients only.
Danish: De to piger havde kun lidt eller ingen GL-3-ophobning i det overfladiske vaskulære endotel i huden ved studiestart, hvilket gør, at denne konklusion kun er gældende for drenge.
English: • decreased blood pressure • hot flush
Danish: • sænket blodtryk • hedeture
English: Fabrazyme treatment should be supervised by a physician experienced in the management of patients with Fabry disease or other inherited metabolic diseases.
Danish: Behandling med Fabrazyme skal overvåges af en læge, der har erfaring med behandling af patienter med Fabrys sygdom eller andre nedarvede stofskiftesygdomme.
English: Adverse reactions reported from clinical trials with a total of 168 patients (154 males and 14 females) treated with Fabrazyme administered at a dose of 1 mg/kg every 2 weeks for a minimum of one infusion up to a maximum of 5 years are listed by System Organ Class and frequency (very common ≥ 1/10; common ≥ 1/100 to < 1/10 and uncommon ≥ 1/1000 to < 1/100) in the table below.
Danish: Bivirkninger rapporteret fra kliniske studier med i alt 168 patienter (154 mænd og 14 kvinder), der blev behandlet med Fabrazyme indgivet med en dosis på 1 mg/kg hver 14. dag, og som har fået mindst én infusion og maksimalt er blevet behandlet i 5 år, er angivet efter systemorganklasse og hyppighed (meget almindelig: ≥1/10, almindelig: ≥1/100 til <1/10 og ikke almindelig ≥1/1.000 til <1/100) i nedenstående tabel.
English: There is no experience with the use of Fabrazyme in pregnant women.
Danish: Der er ingen erfaringer med anvendelse af Fabrazyme til gravide.
English: Fabrazyme is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease (α-galactosidase A deficiency).
Danish: Fabrazyme er indiceret til langtids-enzymerstatningsterapi hos patienter med bekræftet diagnosticering af Fabrys sygdom (α-galaktosidase A-mangel).
English: No changes in dose are necessary for patients with kidney disease.
Danish: Det er ikke nødvendigt at ændre dosis til patienter med nyresygdom.
English: In a limited number of patients IgE antibodies were demonstrated (see section 4.4).
Danish: Hos et begrænset antal patienter blev der påvist IgE-antistoffer (se pkt.
English: - The treating physician will be responsible for determining which patients may be suitable for home or self-administration of Fabrazyme and for arranging for treatment at home and training the patient and/or caregiver in the appropriate skills necessary for this.
Danish: - Den behandlende læge er ansvarlig for at bestemme, hvilke patienter der kan være egnede til hjemme- eller selvbehandling med Fabrazyme og for at træffe de nødvendige foranstaltninger til hjemmebehandling og uddannelse af patienten og/eller plejepersonalet i de færdigheder, der er nødvendige til dette.
English: A small number of patients have experienced reactions suggestive of immediate (Type I) hypersensitivity.
Danish: Et lille antal patienter har oplevet reaktioner, der tyder på akut overfølsomhed (type I allergi).
English: It is recommended to administer the diluted solution through an in-line low protein-binding 0.2 µm filter to remove any protein particles which will not lead to any loss of agalsidase beta activity.
Danish: 10. For at fjerne alle proteinpartikler anbefales det at administrere den fortyndede infusionsvæske gennem et in-line 0,2 µm filter med lav proteinbinding, hvilket ikke vil medføre tab af agalsidase beta-aktivitet.
English: The diluted solution can be held for up to 24 hours at 2°C - 8°C.
Danish: Den fortyndede infusionsvæske kan opbevares i op til 24 timer ved 2-8 ºC.
English: After intravenous administration of agalsidase beta to adults with an infusion time of approximately 300 minutes and at a dose of 1 mg/kg body weight, biweekly, mean Cmax plasma concentrations ranged from 2000-3500 ng/ml, while the AUCinf ranged from 370-780 µg min/ml.
Danish: Efter intravenøs indgivelse af agalsidase beta til voksne med en infusionstid på cirka 300 minutter og en dosis på 1 mg /kg legemsvægt to gange ugentlig lå de gennemsnitlige Cmax plasmakoncentrationer mellem 200-3.500 ng/ml, mens AUCinf lå på mellem 370-780 µg min/ml.
English: What you need to know before you use Fabrazyme Do not use Fabrazyme
Danish: Det skal du vide, før du begynder at bruge Fabrazyme Brug ikke Fabrazyme:
English: Since agalsidase beta (r-hαGAL) is a recombinant protein, the development of IgG antibodies is expected in patients with little or no residual enzyme activity.
Danish: Da agalsidase beta (r-hαGAL) er et rekombinant protein, kan udvikling af IgG-antistoffer forventes hos patienter med ringe eller ingen enzymatisk residualaktivitet.
English: After reconstitution it is recommended to promptly dilute the vials, to minimise protein particle formation over time.
Danish: Efter rekonstitution anbefales det, at man omgående fortynder hætteglassene, for at mindske dannelse af proteinpartikler over tid.
English: The reconstituted solution must be further diluted.
Danish: Det rekonstituerede koncentrat skal fortyndes.
English: In an additional 5-year open-label paediatric study, 31 male patients aged 5 to 18 years were randomized prior to the onset of clinical symptoms involving major organs and treated with two lower dose regimens of agalsidase beta, 0.5 mg/kg every 2 weeks or 1.0 mg/kg every 4 weeks.
Danish: I et andet åbent pædiatrisk studie af 5 års varighed blev 31 drenge i alderen 5-18 år randomiseret, før der indtrådte kliniske symptomer i de vigtigste organer, og de blev behandlet med to lavdosis-regimer på 0,5 mg/kg agalsidase beta hver 2. uge eller 1,0 mg/kg agalsidase beta hver 4. uge.
English: In one of these studies, 15 paediatric patients with available pharmacokinetics data, aged 8.5 to 16 years weighing 27.1 to 64.9 kg were treated with 1.0 mg/kg every 2 weeks.
Danish: I et af disse studier blev 15 pædiatriske patienter i alderen 8,5-16 år med tilgængelige farmakokinetiske data og med en vægt mellem 27,1 og 64,9 kg behandlet med 1,0 mg/kg hver 2. uge.
English: In one open-label paediatric study, sixteen patients with Fabry disease (8-16 years old; 14 males, 2 females) had been treated for one year at 1.0 mg/kg every 2 weeks.
Danish: I et åbent pædiatrisk studie blev 16 patienter med Fabrys sygdom behandlet (8-16 år; 14 drenge, 2 piger) i et år med 1,0 mg/kg hver 2. uge.
English: Other medicines and Fabrazyme
Danish: Brug af anden medicin sammen med Fabrazyme
English: No data are available in children 0 to 4 years No dose adjustment is necessary for children 8-16 years
Danish: Dosisjustering er ikke nødvendig hos børn mellem 8-16 år.
English: If you experience a reaction like this, you should tell your doctor immediately.
Danish: Hvis du oplever sådanne bivirkninger, skal du fortælle det til lægen med det samme.
English: In another study with 30 paediatric patients with available pharmacokinetics data, aged 5 to 18 years, treated with two lower dose regimens of 0.5 mg/kg every 2 weeks and 1.0 mg/kg every 4 weeks, mean CL was 4.6 and 2.3 ml/min/kg, respectively, mean Vss was 0.27 and 0.22 l/kg, respectively, and mean elimination half-life was 88 and 107 minutes, respectively.
Danish: I et andet studie med farmakokinetiske data fra 30 pædiatriske patienter i alderen 5-18 år, som blev behandlet med to lavdosis-regimer på 0,5 mg/kg hver 2. uge og 1,0 mg/kg hver 4. uge, var gennemsnitlig CL henholdsvis 4,6 og 2,3 ml/min/kg, gennemsnitlig Vss var henholdsvis 0,27 og 0.22 l/kg, og gennemsnitlig eliminationshalveringstid var henholdsvis 88 og 107 minutter.
English: Each vial of Fabrazyme is intended for single use only.
Danish: Fabrazyme hætteglas er kun til engangsbrug.
English: The reconstituted solution cannot be stored and should be promptly diluted.
Danish: Den rekonstituerede opløsning må ikke opbevares, men skal straks fortyndes.
English: The marketing authorisation holder (MAH) shall agree the details of the educational programme for Fabrazyme home infusion with the National Competent Authorities prior to implementing the programme nationally.
Danish: infusion af Fabrazyme i hjemmet med den relevante nationale myndighed, før programmet implementeres nationalt.
English: Studies have not been performed to examine the effects of Fabrazyme on fertility.
Danish: Der er ikke udført undersøgelser til belysning af Fabrazymes effekt på frugtbarheden.
English: In one of these studies, performed in adult male patients, after an initial dose of 1.0 mg/kg every 2 weeks for 6 months, 0.3 mg/kg every 2 weeks may maintain clearance of GL-3 in certain cell types in some patients; however, the long term clinical relevance of these findings has not been established (see section 5.1).
Danish: I et af disse studier med voksne mandlige patienter kunne 0,3 mg/kg hver anden uge opretholde globotriaosylceramid (GL-3)clearance i visse celletyper hos nogle patienter efter en indledende dosis på 1,0 mg/kg hver anden uge i 6 måneder; den langsigtede kliniske relevans af disse resultater er imidlertid ikke klarlagt (se pkt.
English: In clinical trials, sixty seven percent (67 %) of the patients experienced at least one infusionassociated reaction (see section 4.8).
Danish: I kliniske studier oplevede syvogtres procent (67 %) af patienterne mindst en infusionsrelateret reaktion (se pkt.
English: Cardiac Disorders tachycardia, palpitations, bradycardia sinus bradycardia --
Danish: Takykardi, palpitationer, bradykardi
English: Additional symptoms included mild or moderate dyspnoea, hypoxia (oxygen saturation decreased), throat tightness, chest discomfort, flushing, pruritus, urticaria, face oedema, angioneurotic oedema, rhinitis, bronchospasm, tachypnoea, wheezing, hypertension, hypotension, tachycardia, palpitations, abdominal pain, nausea, vomiting, infusion-related pain including pain at the extremities, myalgia, and headache.
Danish: Blandt de øvrige symptomer var mild eller moderat dyspnø, hypoksi (nedsat oxygenmætning), strammen i halsen, utilpashed i brystet, rødme, pruritus, urticaria, ansigtsødem, angioødem, rhinitis, bronkospasmer, takypnø, hiven efter vejret, hypertension, hypotension, takykardi, palpitationer, abdominalsmerter, kvalme, opkastning, infusionsrelaterede smerter, herunder ekstremitetssmerter, myalgi og hovedpine.
English: Use of Fabrazyme during pregnancy is not recommended.
Danish: Anvendelse af Fabrazyme under graviditet kan ikke anbefales.
English: No dose adjustment is necessary for patients with renal insufficiency.
Danish: Dosisjustering er ikke nødvendig hos patienter med nyreinsufficiens.
English: The MAH shall ensure that all Healthcare Professional who are expected to prescribe/use Fabrazyme are provided with an educational pack aiming to facilitate the training of the patients/caregivers and also to guide prescribers regarding patient evaluation and selection and organisational requirements for home infusion.
Danish: Indehaveren af markedsføringstilladelsen skal sikre, at læger, som forventes at ordinere/administrere Fabrazyme, får udleveret undervisningsmateriale med det formål at lette uddannelsen af patienter/plejepersonale samt vejlede lægerne i patientevaluering og -udvælgelse og i de organisatoriske krav i forbindelse med hjemmeinfusion.
English: Slowly, withdraw 1.0 ml (equal to 5 mg) of the reconstituted solution from each vial up to the total volume required for the patient dose.
Danish: Træk langsomt 1,0 ml (svarende til 5 mg) rekonstitueret koncentrat ud fra hvert hætteglas op til det totale volumen, der er nødvendigt til patientens dosis.