FrancophonIA/ELRC3.0 huggingface dataset:
English: The t½ of approximately 2-3 hours was also similar in this group of patients.
Danish: En t½ på ca. 2-3 timer var også tilsvarende i denne gruppe patienter.
English: The total time between reconstitution and completion of the infusion should not exceed 24 hours.
Danish: Den samlede tid mellem rekonstituering og gennemførelse af infusionen bør ikke overstige 24 timer.
English: An infusionassociated reaction can sometimes be very severe.
Danish: En infusionsrelateret reaktion kan sommetider være meget alvorlig.
English: It will be given to you once every 2 weeks.
Danish: Lægemidlet gives til Dem hver anden uge.
English: In this group of patients a variable response has also been seen with respect to motor function.
Danish: I denne gruppe patienter er et variabelt respons også set med hensyn til motorisk funktion.
English: Evaluation of motor function gave disparate results.
Danish: Vurdering af motorisk funktion gav uensartede resultater.
English: The reconstituted solution must be further diluted.
Danish: Den rekonstituerede opløsning skal fortyndes yderligere.
English: One additional patient died after study end and another one after withdrawal from the study.
Danish: Endnu en patient døde efter studiets afslutning og endnu en efter udtræden fra studiet.
English: However, motor and respiratory responses to treatment have been more variable.
Danish: Det motoriske og respiratoriske respons på behandling har imidlertid varieret mere.
English: Investigations very common
Danish: Undersøgelser meget almindelig
English: Two patients died and did not enter into the extension study.
Danish: To patienter døde og deltog ikke i det forlængede studie.
English: Patient response to treatment should be routinely evaluated based on a comprehensive evaluation of all clinical manifestations of the disease.
Danish: Patientens respons på behandlingen bør vurderes rutinemæssigt på basis af en omfattende vurdering af alle sygdommens kliniske manifestationer.
English: The results are shown in Table 4.
Danish: Resultaterne er vist i Tabel 4.
English: • Chest pain (not in the heart)
Danish: • Brystsmerter (ikke i hjertet)
English: Kaplan-Meier survival rates at 12, 24 and 36 months of age were 26%, 9% and 7%, respectively.
Danish: Kaplan-Meier overlevelsesrater ved 12, 24 og 36 måneders alderen var henholdsvis 26 %, 9 % og 7 %.
English: Adverse reaction (Preferred Term Level)
Danish: Bivirkning (foretrukken term)
English: Not known: frequency cannot be estimated from the available data
Danish: Ikke kendt (hyppigheden kan ikke vurderes ud fra tilgængelige data):
English: Only one patient became wheelchair-free.
Danish: Kun 1 patient blev fri for kørestol.
English: Treated Patients
Danish: Behandlede patienter
English: Some of these infusion related side effects were serious or life-threatening.
Danish: Nogle af disse infusionsrelaterede bivirkninger var alvorlige eller livstruende.
English: Motor development is often completely arrested, or if motor milestones are achieved, they are subsequently lost.
Danish: Motorisk udvikling ophører ofte helt, og hvis der opnås motoriske milepæle, går de senere tabt.
English: It is recommended to start the administration of the diluted solution within three hours.
Danish: Det anbefales at påbegynde indgivelsen af den fortyndede opløsning inden for tre timer.
English: 1105 BP Amsterdam The Netherlands
Danish: 1105 BP AmsterdamHolland
English: • Temporary suspension or sudden cessation of breathing
Danish: • Midlertidigt ophør eller pludselig standsning af vejrtrækning
English: Reactions in 2 of these patients were IgE-mediated hypersensitivity reactions.
Danish: Reaktionerne hos to af disse patienter var IgE-medierede overfølsomhedsreaktioner.
English: The primary endpoint of the pivotal trial was the proportion of patients who were alive.
Danish: Det primære endepunkt for den pivotale undersøgelse var andelen af patienter, som var i live.
English: It is supplied as a powder which will be mixed with sterile water before it is given.
Danish: Det leveres som et pulver, der blandes med sterilt vand, inden det gives.
English: IgG antibody titres should be regularly monitored.
Danish: IgG-antistoftitre bør monitoreres regelmæssigt.
English: Death typically occurs due to cardiac and/or respiratory failure before the age of one year.
Danish: Død indtræder typisk som følge af hjertestop og/eller respirationssvigt inden 1 års alderen.
English: In the absence of compatibility studies, this medicinal product must not be mixed with other medicinal products.
Danish: Da der ikke foreligger undersøgelser vedrørende eventuelle uforligeligheder, bør dette lægemiddel ikke blandes med andre lægemidler.
English: Cmax (µg/ml) AUC∞ (µg•h/ml) CL (ml/h/kg) Vss (ml/kg) Effective half-life (h)
Danish: Cmax (µg/ml) AUC∞ (µg•t/ml) CL (ml/t/kg) Vss (ml/kg)
English: The 9 remaining patients either died (5 patients) or became invasive-ventilator dependent (4 patients).
Danish: De 9 resterende patienter enten døde (5 patienter) eller blev afhængige af invasiv ventilationsbehandling (4 patienter).
English: The majority of these reactions were non-serious, mild to moderate in intensity and resolved spontaneously.
Danish: Størstedelen af disse reaktioner var ikke-alvorlige, milde til moderate i styrke og svandt spontant.
English: Manufacturer Genzyme Ireland Limited, IDA Industrial Park, Old Kilmeaden Road, Waterford, Ireland
Danish: Genzyme Ireland Limited, IDA Industrial Park, Old Kilmeaden Road, Waterford, Irland
English: Change from Baseline to Week 78/Last Observation*
Danish: Ændring fra baseline til uge 78/sidste observation*
English: Reactions generally occurred shortly after initiation of the infusion.
Danish: Reaktionerne forekom generelt kort tid efter starten på infusionen.
English: Fatal and life-threatening respiratory infections have been observed in some of these patients.
Danish: Der er observeret dødelige og livstruende luftvejsinfektioner hos nogle af disse patienter.
English: Glycogen provides the body with energy, but in Pompe disease the levels of glycogen can get too high.
Danish: Glykogen giver kroppen energi, men ved Pompes sygdom kan glykogenniveauerne blive for høje.
English: • polysorbate 80 (E433).
Danish: • Polysorbat 80 (E433).
English: Linearity/non linearity Pharmacokinetics were dose proportional and did not change over time.
Danish: Liniaritet/non-liniaritet Farmakokinetikken var dosisproportional og ændrede sig ikke over tid
English: Any unused product should be discarded.
Danish: Alt ubrugt produkt skal kasseres
English: Patients should be closely monitored and all cases of IARs, delayed reactions and possible immunological reactions should be reported to the marketing authorisation holder.
Danish: Patienterne bør overvåges nøje, og alle tilfælde af infusionsrelaterede reaktioner, forsinkede reaktioner og eventuelle immunologiske reaktioner bør indberettes til indehaveren af markedsføringstilladelsen.
English: The final infusion solution should be administered as close to preparation time as possible.
Danish: Den endelige infusionsopløsning bør indgives så tæt på klargøringstidspunktet som muligt.
English: This effect was partly attributable to maternal toxicity, as a significant decrease in feed consumption and body weight gain was observed.
Danish: Denne effekt kunne delvist tilskrives maternel toksicitet, da der blev observeret et markant fald i indtagelsen af føde og en stigning i legemsvægt.
English: Wilcoxon-Mann-Whitney Test p-value Forced Vital Capacity (Percent of predicted normal) Pre-treatment Baseline
Danish: Wilcoxon-Mann-Whitney-test p-værdi Forced vital capacity (procentdel af forventet normalværdi) Baseline inden behandling
English: Thus seroconversion is expected to occur in most patients treated with Myozyme.
Danish: Serokonvertering forventes således at optræde hos de fleste patienter, der behandles med Myozyme.
English: *Human acid α-glucosidase is produced in Chinese hamster ovary cells (CHO) by recombinant DNA technology.
Danish: *Human sur α-glucosidase fremstilles ud fra CHO-celler (Chinese Hamster Ovaries) ved hjælp af rekombinant DNA-teknologi.
English: Overall, the numbers of patients experiencing adverse reactions and serious adverse reactions were comparable between the two groups.
Danish: Overordnet var antallet af patienter, der fik bivirkninger og alvorlige bivirkninger, sammenlignelig i de to grupper.
English: Benefits of treatment on motor function including the regaining of lost motor skills were observed in some patients.
Danish: Behandlingens fordele på motorisk funktion omfattede generhvervelse af tabte motoriske færdigheder, blev observeret hos nogle patienter.
English: Very common: may affect more than 1 in 10 people
Danish: Meget almindelig (kan forekomme hos flere end 1 ud af 10 personer):
English: In studies doctors have used medicines to suppress the immune system to reduce the production of antibodies.
Danish: I flere forsøg har læger brugt medicin til at undertrykke immunsystemet med henblik på at reducere dannelsen af antistoffer.
English: After dilution, an immediate use is recommended.
Danish: Efter fortynding anbefales øjeblikkelig brug.
English: The totality of the data suggests that early diagnosis and treatment at an early stage of disease may be critical to achieve the best outcomes in these infantile onset patients.
Danish: Betragtet som helhed tyder data på, at tidlig diagnose og behandling på et tidligt stadium af sygdommen kan være kritisk for at opnå det bedste resultat hos disse patienter med debut i spædbørnsalderen.
English: Data from a small number of patients with HSAT, with or without inhibitory activity, showed limited ITI treatment effect.
Danish: Data fra et lille antal patienter med HSAT, med eller uden hæmmende aktivitet, viste begrænset effekt af ITI-behandling.
English: Due to the small patient population, an adverse reaction reported in 2 patients is classified as common.
Danish: På grund af den lille patientpopulation er en bivirkning, der blev indberettet fra 2 patienter, klassificeret som almindelig.
English: You can also report side effects directly via the national reporting system listed in Appendix V. By reporting side effects you can help provide more information on the safety of this medicine
Danish: De eller Deres pårørende kan også indberette bivirkninger direkte til Lægemiddelstyrelsen via det nationale rapporteringssystem anført i Appendiks V. Ved at indrapportere bivirkninger kan De hjælpe med at fremskaffe mere information om sikkerheden af dette lægemiddel.
English: In clinical studies doses up to 40 mg/kg body weight were used.
Danish: I kliniske undersøgelser blev der brugt doser på op til 40 mg/kg kropsvægt.
English: Pompe disease is a rare, progressive and fatal metabolic myopathy with an estimated global incidence of 1 in 40,000 births.
Danish: Pompes sygdom er en sjælden, progressiv og dødelig stofskifte-myopati med en anslået global forekomst på 1 ud af 40.000 fødsler.
English: This medicine contains less than 1 mmol sodium (23 mg) per vial, i.e. essentially ‘sodium free'.
Danish: Dette lægemiddel indeholder under 1 mmol natrium (23 mg) pr. hætteglas, dvs. i væsentlig grad ”natriumfri”.
English: This enzyme helps the body control levels of glycogen (a type of carbohydrate).
Danish: Dette enzym hjælper kroppen med at regulere niveauerne af glykogen (en form for kulhydrat).
English: Each pack contains 1, 10 or 25 vials.
Danish: Hver pakning indeholder 1, 10 eller 25 hætteglas).
English: ITI regimens may need to be tailored to individual patient needs (see section 5.1).
Danish: Det kan være nødvendigt at tilpasse ITI-regimet til den enkelte patients behov (se pkt.
English: Medical or healthcare professionals are encouraged to register patients who are diagnosed with Pompe disease at www.PompeRegistry.com.
Danish: Læger og sundhedspersonale opfordres til at registrere patienter, der får diagnosen Pompes sygdom på www.PompeRegistry.com.
English: These three studies with Myozyme (with a follow up of at least 3 years in two studies and a median of 23 months in the other study) suggested stabilisation or improvement of motor function and stabilisation of pulmonary function.
Danish: Disse tre studier med Myozyme (med en opfølgning på mindst 3 år i to undersøgelser og en median på 23 måneder i den sidste undersøgelse) viste stabilisering eller forbedring af motoriske funktioner og stabilisering af lungefunktionen.
English: • Increased or high blood pressure
Danish: • Forøget eller for højt blodtryk
English: • Swelling of the face, swelling of the throat or severe combined swelling of the face, throat and tongue due to a severe allergic reaction
Danish: • Hævelse i ansigtet eller halsen eller kraftig kombineret hævelse i ansigt, hals og tunge på grund af en kraftig allergisk reaktion
English: Flushing not known
Danish: Flushing ikke kendt
English: Due to the bloodbrain barrier effect and the enzyme's size, uptake of alglucosidase alfa in the central nervous system is unlikely.
Danish: På grund af blodhjernebarrierens virkning og enzymets størrelse er optagelse af alglucosidase alfa i centralnervesystemet usandsynlig.
English: • Fever or increased body temperature
Danish: • Feber eller forhøjet kropstemperatur
English: • Abnormal breathing sounds, including a whistling sound
Danish: • Unormale vejrtrækningslyde, herunder en fløjtende lyd
English: Echocardiographic indices of cardiomyopathy improved as measured by a decrease in left ventricular mass (LVM).
Danish: Ekkokardiografiske tegn på kardiomyopati blev forbedret som målt ved et fald i venstre ventrikelmasse (LVM).
English: Mild and transient effects may not require medical treatment or discontinuation of the infusion.
Danish: Milde og forbigående reaktioner kræver måske ikke lægebehandling eller seponering af infusionen.
English: Non-clinical data reveal no special hazard for humans based on conventional studies of safety pharmacology, single and repeated dose toxicity.
Danish: Prækliniske data viser ingen speciel risiko for mennesker vurderet ud fra konventionelle studier af sikkerhedsfarmakologi og toksicitet efter enkeltdosis og gentagne doser.
English: Some patients have experienced infusion related side effects in the form of flu-like symptoms, which lasted for a few days after completion of the infusion.
Danish: Nogle patienter har oplevet infusionsrelaterede bivirkninger i form af influenza-lignende symptomer, som varede nogle få dage efter indgivelse af infusionen.
English: If upon immediate inspection foreign particles other than those described above are observed, or if the solution is discoloured, do not use.
Danish: Hvis der ved omgående inspektion observeres andre fremmedlegemer end dem, der er beskrevet ovenfor, eller hvis opløsningen er misfarvet, må den ikke bruges.
English: Blood pressure increased not known
Danish: Forhøjet blodtryk ikke kendt
English: The current medical standards for emergency treatment of anaphylactic reactions are to be observed.
Danish: De gældende medicinske retningslinjer for akut behandling af anafylaktiske reaktioner skal overholdes.
English: Instructions for proper use
Danish: Anvisninger vedrørende korrekt anvendelse
English: All 5 patients who were receiving invasive ventilation at baseline continued to require ventilation throughout the study (4 patients survived beyond week 104 and one patient died).
Danish: Alle 5 patienter, der modtog invasiv ventilationsbehandling ved baseline, havde fortsat brug for ventilationsbehandling igennem hele studiet (4 patienter overlevede efter uge 104, og en patient døde).
English: Some reactions were severe (see section 4.8).
Danish: Nogle reaktioner var alvorlige (se pkt.
English: Myozyme contains sodium
Danish: Myozyme indeholder natrium
English: The objectives of the “Pompe Registry” are to enhance the understanding of Pompe disease and to monitor patients and their response to enzyme replacement therapy over time, with the ultimate goal of improving clinical outcomes for these patients.
Danish: Formålet med dette ”Pompe Registry” er at forbedre forståelsen af Pompes sygdom og overvåge patienterne og deres respons på enzymerstatningsbehandling over tid med det endelige mål at forbedre de kliniske resultater for disse patienter.
English: • Low blood pressure
Danish: • Lavt blodtryk
English: Side effects were mainly seen while patients were being given the medicine or shortly after (“infusion related effects”).
Danish: Bivirkninger blev hovedsageligt observeret, mens patienterne fik medicinen eller kort tid derefter (”infusionsrelaterede reaktioner”).
English: Usually, it is characterised by the presence of sufficient residual GAA activity to preclude the development of cardiomyopathy, however some cardiac involvement has been reported in up to approximately 4% of patients with late-onset Pompe disease.
Danish: Som regel kendetegnes den ved tilstedeværelsen af tilstrækkelig resterende GAA-aktivitet, hvilket forhindrer udviklingen af kardiomyopati, men nogen involvering af hjertet er rapporteret hos op til ca. 4 % af patienter med sent debuterende Pompes sygdom.
English: It was demonstrated that the use of a 0.2 micron in-line filter removes visible particles and does not result in an apparent loss of protein or activity.
Danish: Det er påvist, at brug af et 0,2 mikron in-line filter fjerner synlige partikler og ikke medfører betydende tab af protein eller aktivitet.
English: • Swelling and thickening of the skin at infusion site in case of leakage of the product outside blood vessels
Danish: Hævelse og fortykkelse af huden på infusionsstedet i tilfælde af lækage af produktet uden for blodåren.
English: The primary endpoint was the proportion of patients who were alive and free of invasive ventilator support.
Danish: Det primære endepunkt var andelen af patienter, som var i live og fri for invasiv ventilations/respiratorbehandling.
English: No significant adverse findings on embryofoetal development were observed in a mouse and a rabbit embryofoetal study and no significant adverse findings were observed in a mouse fertility and early embryonic development study.
Danish: Der blev ikke observeret nogen betydelige negative fund for så vidt angår den embryoføtale udvikling i et embryoføtal studie af mus og kaniner, og der blev ikke observeret nogen signifikante negative fund i en undersøgelse af fertiliteten og den tidlige embryonale udvikling hos mus.
English: Reconstitution and dilution should be performed in accordance with good practice rules, particularly for the respect of asepsis.
Danish: Rekonstituering og fortynding skal udføres i overensstemmelse med reglerne for god praksis, især af hensyn til det aseptiske aspekt.
English: You are recommended to stop breast-feeding when you are given Myozyme.
Danish: De anbefales at standse amningen, når De gives Myozyme.
English: Because you have Pompe disease, there is a risk that you get a severe infection of your airways or lungs.
Danish: Da De har Pompes sygdom, er der en risiko for, at De får en svær infektion i luftvejene eller lungerne.
English: • Low level of oxygen in the blood
Danish: • Lavt iltniveau i blodet
English: If you forget to use Myozyme
Danish: Hvis De har glemt at bruge Myozyme
English: The cause of a poor clinical outcome and of developing high and sustained IgG antibody titres is thought to be multi-factorial.
Danish: Årsagen til et dårligt klinisk resultat og udvikling af høje og vedvarende IgG-antistoftitre menes dog at være multi-faktoriel.
English: However, high and sustained IgG antibody titres also occur in some CRIM-positive patients.
Danish: Der ses dog også høje og vedvarende IgGantistoftitre hos nogle CRIM-positive patienter.
English: It is noteworthy that a higher proportion of patients with better motor outcomes show stability or improvement in growth parameters (weight), while the large majority of patients, regardless of their motor outcomes or baseline features, show reversal of cardiomyopathy as measured by changes in LVM Z-score.
Danish: Det er bemærkelsesværdigt, at en højere andel af patienter med bedre motoriske resultater viser stabilitet eller forbedring i vækstparametre (vægt), medens størstedelen af patienter uafhængigt af deres motoriske resultater eller baseline-funktioner udviser reversibilitet af kardiomyopati som målt ved ændringer i LVM Z-score.
English: The recommended dosage of Myozyme in children and adolescents is the same as in adults.
Danish: Den anbefalede dosis Myozyme til børn og unge er den samme som til voksne.
English: Class, presented by frequency categories: very common (≥1/10), common (≥1/100 to<1/10), uncommon (≥1/1,000 to <1/100), rare (≥1/10,000 to <1/1,000), very rare (<1/10,000) and not known
Danish: (≥1/100 til <1/10), ikke almindelig (≥1/1.000 to <1/100), sjælden (≥1/10.000 to <1/1.000), meget sjælden (<1/10.000) og ikke kendt (kan ikke estimeres ud fra forhåndenværende data).
English: Use aseptic technique
Danish: Brug aseptisk teknik
English: The pulmonary benefits observed in patients included a clinically meaningful improvement in FVC of 35% in one patient, and significant reductions in the number of hours of ventilator support needed in 2 patients.
Danish: De pulmonale fordele, der blev observeret hos patienter, omfattede en klinisk betydelig forbedring af FVC på 35 % hos 1 patient og signifikante reduktioner i antal timer med ventilationsstøtte hos 2 patienter.
English: Other names for Pompe disease include glycogen storage disease type II (GSDII), acid maltase deficiency (AMD) and glycogenosis type II.
Danish: Andre navne for Pompes sygdom er ”glycogen storage disease type II” (GSD-II), ”sur maltase deficiency” (AMD) og ”glykogonesis type II”.
English: It is postulated that Myozyme will restore lysosomal GAA activity resulting in stabilisation or restoration of cardiac and skeletal muscle function (including respiratory muscles).
Danish: Det påstås, at Myozyme vil genoprette lysosomal GAA-aktivitet, hvilket medfører stabilisering eller genoprettelse af hjerte- og skeletalmuskelfunktion (herunder åndedrætsmuskler).
English: Tell your doctor or pharmacist if you are using, have recently used or might use any other medicines.
Danish: Fortæl det altid til lægen eller apotekspersonalet, hvis De bruger anden medicin eller har gjort det for nylig.
English: IARs may occur at any time during the infusion of Myozyme or generally up to 2 hours after, and are more likely with higher infusion rates.
Danish: Infusionsrelaterede reaktioner kan forekomme på ethvert tidspunkt under infusion af Myozyme eller generelt op til 2 timer efter og er mere sandsynlige ved høje infusionshastigheder.
English: The reconstituted volume allows accurate withdrawal of 10.0 ml (equal to 50 mg) from each vial.
Danish: Den rekonstituerede mængde muliggør nøjagtig udtagning af 10,0 ml (svarende til 50 mg) fra hvert hætteglas.
English: Subjects ranged in age from 6 months to 3.5 years at initiation of treatment.
Danish: Forsøgspersoner var fra 6 måneder til 3,5 år gamle ved behandlingens start.
English: Patients improved following treatment interruption.
Danish: Patienternes tilstand bedredes efter seponering af behandlingen.
English: Nephrotic syndrome Proteinuria
Danish: Nefrotisk syndrom Proteinuri
English: All patients were freely ambulatory and all but one patient did not require any form of ventilator support (1 patient required nocturnal non-invasive ventilation).
Danish: Alle patienter var fuldt oppegående, og kun en af patienterne havde behov for respiratorstøtte (1 patient krævede ikke-invasiv ventilation om natten).
English: Because there are no data available on effects in neonates exposed to alglucosidase alfa via breast milk, it is recommended to stop breast-feeding when Myozyme is used.
Danish: Da der ikke findes nogen data vedrørende effekt på nyfødte, der udsættes for alglucosidase alfa via modermælk, anbefales det at afbryde amningen ved anvendelse af Myozyme.
English: Pack sizes of 1, 10 or 25 vials.
Danish: Pakningsstørrelser á 1, 10 eller 25 hætteglas.
English: Myozyme should not be used during pregnancy unless clearly necessary.
Danish: Myozyme bør ikke anvendes under graviditet, med mindre det er klart nødvendigt.
English: MANUFACTURERS OF THE BIOLOGICAL ACTIVE SUBSTANCE AND MANUFACTURERS RESPONSIBLE FOR BATCH RELEASE
Danish: FREMSTILLERE AF DET BIOLOGISK AKTIVE STOF OG FREMSTILLERE ANSVARLIG FOR BATCHFRIGIVELSE