FrancophonIA/ELRC3.0 huggingface dataset:
English: The vast majority of patients with infantile-onset Pompe disease treated with Myozyme demonstrate improvement in cardiac function as well as stabilisation or improvements in growth parameters.
Danish: Langt størstedelen af patienter med Pompes sygdom med debut i spædbørnsalderen, der blev behandlet med Myozyme, udviste forbedring i hjertefunktion samt stabilisering eller forbedring af vækstparametre.
English: Determine the number of vials to be reconstituted based on the individual patient's dose regimen (mg/kg) and remove the required vials from the refrigerator in order to allow them to reach room temperature (approximately 30 minutes).
Danish: Beregn det antal hætteglas, der skal rekonstitueres, på basis af den enkelte patients doseringsregimen (mg/kg) og tag det nødvendige antal hætteglas ud af køleskabet, så de kan komme op på stuetemperatur (cirka 30 minutter).
English: Add the water for injections by slow drop-wise addition down the side of the vial and not directly onto the lyophilised cake.
Danish: Tilsæt vand til injektionsvæske ved langsomt, dråbevist langs hætteglassets side og ikke direkte ned i den frysetørrede kage.
English: Comparison of survival curves from time of diagnosis versus the untreated historical cohort was made using a Cox proportional hazards regression analysis.
Danish: Sammenligning af overlevelseskurver fra diagnosetidspunktet versus den ubehandlede, historiske gruppe blev foretaget ved hjælp af en Cox proportionel risiko regressions analyse.
English: After 78 weeks, patients treated with Myozyme showed improvement in distance walked as measured by 6MWT and stabilization of pulmonary function as measured by FVC % predicted as compared to placebo-treated patients.
Danish: Efter 78 uger udviste de patienter, der blev behandlet med Myozyme, forbedring i gang distance (målt med 6MWT) og stabilisering af lungefunktionen (målt med FVC % forventet) sammenlignet med de placebobehandlede patienter.
English: There is no evidence for special considerations when Myozyme is administered to paediatric patients of all ages or older people.
Danish: Der er ingen dokumentation for særlige hensyn ved indgivelse af Myozyme til børn i alle aldre eller hos ældre patienter.
English: You may need to be given pre-treatment medicines to prevent an allergic reaction (e.g. antihistamines and/or corticosteroids) or to reduce fever (antipyretics).
Danish: De skal muligvis have medicin inden behandlingen for at forhindre en allergisk reaktion (f.eks. antihistaminer og/eller kortikosteroider) eller febernedsættende medicin (antipyretika).
English: Face edema Feeling hot Pyrexia
Danish: Ansigtsødem Varmefølelse Pyreksi
English: The probability of a poor outcome and of developing high and sustained IgG antibody titres appears higher among CRIM-negative patients (Cross Reactive Immunologic Material- negative patients in whom no endogenous GAA protein was detected by Western blot analysis) than among CRIM-positive patients in whom endogenous GAA protein was detected by Western blot analysis and/or predicted based on genotype.
Danish: Sandsynligheden for et dårligt resultat og for udvikling af høje og vedvarende IgGantistoftitre er tilsyneladende større hos CRIM-negative patienter (Cross Reactive Immunologic Material-negative patienter, hvor der ikke blev detekteret endogent GAA-protein med Western blotanalyse) end hos CRIM-positive patienter, hvor der blev detekteret endogent GAA-protein med Western blot-analyse og/eller det blev forudset baseret på genotype.
English: • Swelling of the arms and legs
Danish: • Hævelse af arme og ben
English: -The active substance is alglucosidase alfa.
Danish: -Det aktive stof er alglucosidase alfa.
English: What you need to know before you are given Myozyme Do not use Myozyme:
Danish: Det skal De vide, før De begynder at bruge Myozyme Brug ikke Myozyme:
English: Slightly more patients in the Myozyme group than in the placebo group experienced IARs (28% versus 23%).
Danish: Lidt flere patienter i Myozyme-gruppen end i placebogruppen fik infusionsrelaterede reaktioner (28 % vs. 23 %).
English: There was no difference in the pharmacokinetic profile of alglucosidase alfa in these juvenile lateonset patients compared to infantile-onset patients.
Danish: Der var ingen forskel på den farmakokinetiske profil for alglucosidase alfa hos disse unge patienter med sent sygdomsdebut sammenlignet med patienter med sygdom, der debuterede i spædbørnsalderen.
English: Subjects were aged 6 months or less at the onset of treatment.
Danish: Forsøgspersoner var 6 måneder eller yngre ved behandlingens start.
English: Method of administration Myozyme should be administered as an intravenous infusion.
Danish: Indgivelsesmetode Myozyme skal indgives som en intravenøs infusion.
English: After the first year (58 up to 168 weeks) of treatment LVM further decreased in 9 out of 12 patients with available data.
Danish: Efter det første år (58 til 168 uger) med behandling faldt LVM yderligere hos 9 af 12 patienter med tilgængelige data.
English: Patients with an acute illness (e.g. pneumonia, sepsis) at the time of Myozyme infusion appear to be at greater risk for IARs.
Danish: Patienter med en akut sygdom (f.eks. lungebetændelse, sepsis) på tidspunktet for Myozyme-infusionen har tilsyneladende større risiko for infusionsrelaterede reaktioner.
English: After 52 weeks all patients continued treatment for a total duration of more than 3 years (168 weeks with a median of 121 weeks).
Danish: Efter 52 uger fortsatte alle patienter med behandling med en samlet varighed på op til mere end 3 år (168 uger med en median på 121 uger).
English: Perform an immediate inspection of the reconstituted vials for particulate matter and discoloration.
Danish: Udfør straks en inspektion af de rekonstituerede hætteglas for partikler og misfarvning.
English: Intravenous use after reconstitution and dilution
Danish: Intravenøs brug efter rekonstituering og fortynding
English: Immunogenicity data from clinical trials and published literature in CRIM-negative infantile-onset patients (IOPD) suggests that the administration of immune tolerance induction (ITI) regimen given to alglucosidase alfa naive patients (prophylactic ITI) may be effective in preventing or reducing the development of High Sustained Antibody Titer (HSAT) against alglucosidase alfa.
Danish: Immunogenicitetsdata fra kliniske studier og publiceret litteratur for CRIM-negative patienter med sygdomsdebut i spædbørnsalderen (IOPD) tyder på, at administration af regime til immuntoleranceinduktion (ITI) til alglucosidase alfa-naive patienter (profylaktisk ITI) kan være effektivt til at forebygge og reducere udviklingen af HSAT (High Sustained Antibody Titer) mod alglucosidase alfa.
English: Therefore, these patients should be monitored more closely during administration of Myozyme.
Danish: Disse patienter skal derfor overvåges tættere under indgivelse af Myozyme.
English: Adverse reactions reported in at least 2 patients are listed in Table 1 by System Organ Class.
Danish: Bivirkninger, der blev indberettet fra mindst 2 patienter, er anført i Tabel 1 efter systemisk organklasse.
English: • Nephrotic Syndrome: swelling of lower limbs, generalized swelling and protein loss in urine
Danish: • Nefrotisk syndrom: hævede ben, generel hævelse og proteintab via urinen
English: • Narrowing of the blood vessels causing blood flow to be decreased
Danish: • Forsnævring af blodkarrene med nedsat blodgennemstrømning til følge
English: Urticaria Rash papular Pruritus Hyperhidrosis not known
Danish: Urticaria Papuløst udslæt Pruritus Hyperhidrose ikke kendt
English: Peripheral coldness Infusion site pain Infusion site reaction Infusion site swelling Infusion site induration
Danish: Perifer kuldefølelse Smerte på infusionsstedet Reaktion på infusionsstedet Hævelse på infusionsstedet Induration af infusionsstedet Ekstravasation på infusionsstedet
English: There does not appear to be a correlation between the onset of IARs and the time of IgG antibody formation.
Danish: Der er tilsyneladende ingen sammenhæng mellem forekomst af infusionsrelaterede reaktioner og IgG-antistofdannelsen.
English: Patients with moderate to severe or recurrent IARs have been evaluated for alglucosidase alfa specific IgE antibodies; some patients tested positive including some who experienced an anaphylactic reaction.
Danish: Patienter med moderate til svære eller recidiverende infusionsrelaterede reaktioner er vurderet for specifikke IgE-antistoffer mod alglucosidase alfa; nogle patienter blev testet positive, herunder nogle, der oplevede en anafylaktisk reaktion.
English: Serious adverse reactions reported in 4 patients treated with Myozyme were: angioedema, chest discomfort, throat tightness, non-cardiac chest pain and supraventricular tachycardia.
Danish: Alvorlige bivirkninger, der blev indberettet fra 4 patienter, som blev behandlet med Myozyme, var: angioødem, ubehag i brystet, kvælningsfornemmelser, ikke-kardiale brystsmerter samt supraventrikulær takykardi.
English: At the end of the study (with individual patient treatment durations ranging from 60 to 150 weeks; mean follow-up period of 119 weeks) 14 of 16 patients were alive and 9 of 16 patients were alive and free of invasive ventilatory support.
Danish: Ved afslutningen af studiet (med individuelle patientbehandlingsvarigheder fra 60 til 150 uger: gennemsnitlig opfølgningsperiode på 119 uger) var 14 af 16 patienter i live, og 9 af 16 patienter var i live og fri for invasiv ventilationssbehandling.
English: Patients presented with a constellation of signs and symptoms, primarily respiratory, cardiovascular, edematous and/or cutaneous in nature (see section 4.4).
Danish: Patienterne udviste en række tegn og symptomer, primært respiratoriske, kardiovaskulære, ødematøse og/eller kutane af natur (se pkt.
English: Patients were randomized in a 2:1 ratio and received 20 mg/kg Myozyme (n=60) or placebo (n=30) once every two weeks for 78 weeks (18 months).
Danish: Patienterne blev randomiseret i forholdet 2:1 og fik 20 mg/kg Myozyme (n=60) eller placebo (n=30) én gang hver anden uge i 78 uger (18 måneder).
English: Genzyme Europe B.V.-NL
Danish: Genzyme Europe B.V.
English: Sodium dihydrogen phosphate monohydrate (E339) Disodium phosphate heptahydrate (E339) Polysorbate 80 (E433)
Danish: Natriumdihydrogenphosphatmonohydrat (E339) Dinatriumphosphatheptahydrat (E339) Polysorbat 80 (E433)
English: The time course of disease progression is extremely variable and not predictable, with some patients experiencing a rapid deterioration in skeletal and respiratory muscle function leading to loss of ambulation and respiratory failure, others progressing less rapidly, and yet others presenting with a dissociation in the progression of skeletal and respiratory muscle involvement.
Danish: Tidsforløbet for sygdommens udvikling varierer meget og er ikke forudsigelig, da nogle patienter oplever en hurtig forringelse i skeletal- og åndedrætsmuskelfunktion, som medfører tab af mobilitet og respirationssvigt, andre progredierer mindre hurtigt, og atter andre præsenterer sig med en adskillelse mellem progressionen af skeletal- og åndedrætsmuskel involvering.
English: Preclinical data did not reveal any significant adverse findings (see section 5.3).
Danish: Prækliniske data påviste ikke nogen signifikante bivirkninger (se pkt.
English: 1 Reactions reported in 39 infantile-onset patients in 2 clinical trials.
Danish: 1 Bivirkninger, der er indberettet fra 39 patienter med debut i spædbørnsalderen i 2 kliniske forsøg.
English: After the first and sixth infusion of Myozyme, mean maximum plasma concentrations (Cmax) ranged from 178.2 to 263.7 µg/ml for the 20 mg/kg and 40 mg/kg dose groups respectively.
Danish: Efter første og sjette infusion af Myozyme, varierede gennemsnitlige plasmakoncentrationer (Cmax) fra 178,2 til 263,7 µg/ml for grupperne, der fik henholdsvis 20 mg/kg og 40 mg/kg doser.
English: The untreated historical cohort was matched to the pivotal study population and was derived from a retrospective natural history study (n=42) in patients with infantile-onset Pompe disease.
Danish: Den ubehandlede, historiske patientgruppe var i overensstemmelse med pivotal studiets population og var afledt fra et retrospektivt naturligt anamnesestudie (n=42) omhandlende patienter med Pompes sygdom med debut i spædbørnsalderen.
English: Because dizziness has been reported as an infusion associated reaction, this may affect the ability to drive and use machines on the day of the infusion.
Danish: Svimmelhed er indberettet som en infusionsrelateret reaktion, og evnen til at føre motorkøretøj eller betjene maskiner kan derfor muligvis påvirkes på infusionsdagen.
English: After 52 weeks of treatment, 3 out of 8 patients with available data made gains in motor function over baseline as measured by raw scores and age-equivalent scores from baseline in the AIMS.
Danish: Efter 52 ugers behandling opnåede 3 af 8 patienter en forbedret motorisk funktion i forhold til baseline målt ved rå scorer og aldersækvivalente scorer fra baseline på AIMS.
English: Body temperature increased
Danish: Forhøjet legemstemperatur
English: Median Week 78/Last Observation Mean ± s.d.
Danish: Uge 78/sidste observation
English: Flushing common Hypertension Pallor
Danish: Flushing almindelig Hypertension Bleghed
English: Myozyme will be given to you under the supervision of a doctor who is experienced in the treatment of Pompe disease.
Danish: Myozyme gives til Dem under overvågning fra en læge, der har kendskab til behandlingen af Pompes sygdom.
English: There is no experience with overdose of alglucosidase alfa.
Danish: Der er ingen erfaring med overdosering af alglucosidase alfa.
English: Approximately half of the patients treated with Myozyme in infantile-onset clinical studies and 28% of the patients treated with Myozyme in a late-onset clinical study developed infusion associated reactions (IARs).
Danish: Ca. halvdelen af patienterne behandlet med Myozyme i kliniske studier med patienter med sygdomsdebut i spædbørnsalderen og 28 % af patienterne behandlet med Myozyme i kliniske studier med patienter med sent debuterende sygdom udviklede infusionsrelaterede reaktioner.
English: Infusions should be administered incrementally.
Danish: Infusionerne skal indgives trinvis.
English: A small number of patients (<1%) in clinical trials and in the commercial setting developed anaphylactic shock and/or cardiac arrest during Myozyme infusion that required life-support measures.
Danish: Et lille antal patienter (<1 %) fra kliniske undersøgelser og post markedsføring udviklede anafylaktisk chok og/eller hjertestop under infusion med Myozyme, som krævede genoplivningstiltag.
English: • One study in Italy enrolled 74 late-onset patients with up to 48 months follow up.
Danish: • Et studie i Italien inkluderede 74 sent debuterende patienter med op til 48 måneders opfølgning.
English: These proportions were maintained up to the end of the study (with individual patient treatment durations ranging from 1 to 168 weeks; mean follow-up period of 109 weeks).
Danish: Disse andele blev opretholdt indtil afslutningen af studiet (med individuelle patientbehandlingsvarigheder mellem 1 og 168 uger: gennemsnitlig opfølgningsperiode på 109 uger).
English: Deficiency of this enzyme leads to glycogen accumulation in various tissues, particularly cardiac, respiratory and skeletal muscle, leading to the development of hypertrophic cardiomyopathy and progressive muscle weakness, including impairment of respiratory function.
Danish: Mangel på dette enzym medfører glykogen-ophobning i forskellige væv, især hjerte-, åndedræts- og skeletalmuskler, hvilket medfører udvikling af hypertrofisk kardiomyopati og progressiv muskelsvækkelse, herunder svækkelse af åndedrætsfunktionen.
English: There are no data from the use of alglucosidase alfa in pregnant women.
Danish: Der foreligger ikke data om brugen af alglucosidase alfa hos gravide kvinder.
English: • One study in Germany enrolled 38 late-onset patients with 36 months follow up.
Danish: • Et studie i Tyskland inkluderede 38 sent debuterende patienter med 36 måneders opfølgning.
English: Contents of the pack and other information What Myozyme contains
Danish: Pakningsstørrelser og yderligere oplysninger Myozyme indeholder:
English: For storage conditions after dilution of the medicinal product, see section 6.3.
Danish: Opbevaringforhold efter fortynding af lægemidlet, se pkt.
English: The % predicted FVC changed by a median of 0.0 for Myozyme-treated patients and decreased by a median of 3% for placebo-treated patients, indicating a statistically significant treatment effect (p=0.0026).
Danish: Den forventede procentvise FVC ændredes med en median på 0,0 for Myozyme-behandlede patienter og faldt med en median på 3 % for placebobehandlede patienter, hvilket tyder på en statistisk signifikant behandlingseffekt (p=0,0026).
English: Patients with Pompe disease treated with immunosuppressive agents maybe at further increased risk of developing severe infections and vigilance is recommended.
Danish: Patienter med Pompes sygdom, som behandles med immunsuppressiva, kan have en yderligere øget risiko for at udvikle alvorlige infektioner, og overvågning anbefales.
English: MINIMUM PARTICULARS TO APPEAR ON SMALL IMMEDIATE PACKAGING UNITS VIAL LABEL
Danish: MINDSTEKRAV TIL MÆRKNING PÅ SMÅ INDRE EMBALLAGER Etiket på hætteglas
English: For single use only Read the package leaflet before use.
Danish: Kun til engangsbrug Læs indlægssedlen før brug Intravenøs anvendelse
English: Pompe disease
Danish: Pompes sygdom
English: Tremor Headache
Danish: Tremor Hovedpine
English: If severe hypersensitivity or anaphylactic reactions occur, immediate discontinuation of Myozyme infusion should be considered and appropriate medical treatment should be initiated.
Danish: I tilfælde af svære overfølsomhedsreaktioner eller anafylaktiske reaktioner skal omgående seponering af Myozyme-infusionen overvejes og passende medicinsk behandling igangsættes.
English: At 104 weeks of treatment, all 13 patients with available data had maintained or improved weight-for-age percentiles (above the 3rd percentile), all 12 patients with available data were above the 3rd percentile for length and all 12 patients with available data were above the 3rd percentile for head circumference.
Danish: Efter 104 ugers behandling havde alle 13 patienter med tilgængelige data opnået vedligeholdelse eller forbedring af vægt/alder-percentilen (over 3. percentil), alle 12 patienter med tilgængelige data var over den 3. percentil for højde, og alle 12 patienter med tilgængelige data var over den 3. percentil for hovedomkreds.
English: Serious infusion reactions including urticaria, rales, tachycardia, decreased oxygen saturation, bronchospasm, tachypnea, periorbital edema and hypertension have been reported.
Danish: Alvorlige infusionsreaktioner så som urticaria, rallen, takykardi, reduceret iltmætning, bronkospasme, takypnø, periorbitalt ødem og hypertension er blevet rapporteret.
English: Patients received 20 mg/kg Myozyme once every two weeks for 52 weeks except for 8 patients who received 40 mg/kg after at least 26 weeks of treatment.
Danish: Patienterne fik 20 mg/kg Myozyme én gang hver anden uge i 52 uger med undtagelse af 8 patienter, der fik 40 mg/kg efter mindst 26 ugers behandling.
English: Higher mean clearance, lower mean AUC∞, and lower mean Cmax were observed in 5 patients who tested positive for inhibition of cellular uptake of enzyme.
Danish: Højere middelclearance, lavere middel-AUC∞ og lavere middel-Cmax blev observeret hos 5 patienter, der blev testet positive for hæmning af den cellulære optagelse af enzym.
English: The distance walked in 6 minutes increased by a median of 15.0 meters for Myozyme-treated patients and decreased by a median of 7.5 meters for placebo-treated patients, indicating a statistically significant Myozyme treatment effect compared to placebo (p=0.0283).
Danish: Gang distancen på 6 minutter steg med en median på 15,0 meter for Myozyme-behandlede patienter og faldt med en median på 7,5 meter for placebobehandlede patienter, hvilket tyder på en statistisk signifikant behandlingseffekt af Myozyme sammenlignet med placebo (p=0,0283).
English: Your doctor should consider discontinuation of the administration of Myozyme and initiate appropriate medical treatment.
Danish: Deres læge bør overveje at afbryde behandlingen med Myozyme og iværksætte passende behandling.
English: The reconstituted volume is 10.5 ml containing 5 mg/ml, and appears as a clear, colourless to pale yellow solution which may contain particles in the form of thin white strands or translucent fibres.
Danish: Den rekonstituerede mængde er 10,5 ml indeholdende 5 mg/ml, og fremstår som en klar, farveløs til lysegul opløsning, der kan indeholde partikler i form af tynde, hvide tråde eller gennemsigtige fibre.
English: Skin and subcutaneous tissue disorders very common
Danish: Hud og subkutane væv meget almindelig
English: An open-label clinical trial assessed the safety and efficacy of Myozyme in 5 patients with late-onset Pompe disease who ranged in age from 5 to 15 years at initiation of treatment (AGLU02804).
Danish: En open-label klinisk undersøgelse vurderede sikkerhed og effekt for Myozyme hos 5 patienter med sent debuterende Pompes sygdom, der var fra 5 til 15 år ved behandlingens start (AGLU02804).
English: The majority of patients were successfully re-challenged with alglucosidase alfa using lower doses and/or pretreatment with antiinflammatory drugs and/or corticosteroids and have continued to receive treatment under close clinical supervision.
Danish: Størstedelen af patienterne blev med godt resultat reeksponeret for alglucosidase alfa ved lavere doser og/eller efter præmedicinering med antiinflammatorisk medicin og/eller kortikosteroider, og de kunne fortsætte med at få behandling under nøje overvågning.
English: If immune-mediated reactions occur, discontinuation of the administration of alglucosidase alfa should be considered and appropriate medical treatment initiated.
Danish: Hvis der forekommer immunmedierede reaktioner, bør ophør af behandling med alglucosidase alfa overvejes, og passende medicinsk behandling startes.
English: Pyrexia common Irritability Chills
Danish: Pyreksi almindelig Irritabilitet Kulderystelser
English: Such a reaction comprises different symptoms like low blood pressure, chest discomfort, throat tightness, face, lips or tongue swelling (angioedema), hives (urticaria), dizziness, rash, itchy skin, nausea, vomiting, cough and bronchospasm (see section 4 for an overview of all infusion-associated reactions).
Danish: En sådan reaktion indbefatter forskellige symptomer såsom lavt blodtryk, ubehag i brystet, strammende fornemmelse omkring halsen, hævelse af ansigtet, læberne eller tungen (angioødem), nældefeber (urticaria), svimmelhed, udslæt, hudkløe, kvalme, opkastning, hoste og bronkospasme (se punkt 4 for at se en oversigt over alle infusionsrelaterede reaktioner).
English: Gently invert or massage the infusion bag to mix the diluted solution.
Danish: Vend eller masser forsigtigt infusionsposen for at blande den fortyndede opløsning.
English: If you experience swelling of your lower limbs or generalized swelling, please inform your doctor.
Danish: Hvis De oplever, at Deres ben hæver, eller De får generelle hævelser, skal De fortælle det til Deres læge.
English: Reconstitute each 50 mg vial of Myozyme with 10.3 ml water for injections using a syringe with a needle diameter not larger than 20 gauge.
Danish: Hvert 50 mg hætteglas med Myozyme rekonstitueres med 10,3 ml vand til injektionsvæske ved hjælp af en sprøjte med en nålediameter, der ikke overstiger 20 gauge.
English: Patients were randomized to receive either 20 mg/kg or 40 mg/kg once every two weeks for a period of 52 weeks.
Danish: Patienterne blev randomiseret til at få enten 20 mg/kg eller 40 mg/kg én gang hver anden uge i en periode på 52 uger.
English: For instructions on reconstitution and dilution of the medicinal product before administration, see section 6.6.
Danish: For anvisninger om rekonstituering og fortynding af lægemidlet før indgivelse, henvises til pkt.