FrancophonIA/ELRC3.0 huggingface dataset:
English: Infusion should be avoided if the patient has an acute febrile or respiratory illness at the time.
Danish: Infusion skal undgås, hvis patienten har en akut feber- eller åndedrætssygdom på det pågældende tidspunkt.
English: Other Investigations
Danish: Andre undersøgelser
English: Adverse reactions are presented by System Organ Class and frequency.
Danish: Bivirkningerne er angivet i henhold til systemorganklasse og hyppighed.
English: As end organ damage progresses over time, it is more difficult for the treatment to reverse the damage or to show improvements.
Danish: Efterhånden som endeorganskader skrider frem med tiden, er det vanskeligere i behandlingen at revertere skaderne eller fremvise forbedringer.
English: This medicine has been authorised under ‘exceptional circumstances'.
Danish: Dette lægemiddel er godkendt under ”betingede omstændigheder”.
English: Frequencies are defined as very common (≥ 1/10), common (≥ 1/100 to < 1/10), uncommon (≥ 1/1000 to < 1/100), rare (≥ 1/10,000 to < 1/1,000), and very rare (< 1/10,000).
Danish: Hyppighed er defineret som meget almindelig (≥ 1/10), almindelig (≥ 1/100 til < 1/10), ikke almindelig (≥ 1/1.000 til < 1/100), sjælden (≥ 1/10.000 til < 1/1.000) og meget sjælden (< 1/10.000).
English: This belongs to a group of medicines called enzyme replacement therapies.
Danish: Det tilhører en gruppe lægemidler, der kaldes enzymsubstitutionsterapier.
English: Based on mechanism of action, rhGUS is not expected to be tumorigenic.
Danish: Baseret på virkningsmekanismen forventes rhGUS ikke at være tumorigen.
English: Treatment with Mepsevii should be started and monitored by your doctor.
Danish: Behandling med Mepsevii skal påbegyndes og overvåges af din læge.
English: - If you have an allergic reaction during your infusion your doctor may slow down, or stop your infusion.
Danish: - Hvis du får en allergisk reaktion under din infusion, kan din læge sænke hastigheden eller stoppe infusionen.
English: Patients should be informed of the signs and symptoms of hypersensitivity reactions and instructed to seek immediate medical care should such signs and symptoms occur.
Danish: Patienter skal informeres om tegn og symptomer på overfølsomhedsreaktioner og instrueres i omgående at søge lægehjælp, hvis sådanne tegn og symptomer forekommer.
English: A sufficiently large needle (18 gauge) should be used to minimise bubbles in the solution.
Danish: Der skal anvendes en tilstrækkelig stor kanyle (18 gauge) for at minimere luftbobler i opløsningen.
English: Mepsevii is given to you and monitored by your doctor.
Danish: Mepsevii gives til dig og overvåges af din læge.
English: If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor for advice before using this medicine.
Danish: Hvis du er gravid eller ammer, har mistanke om, at du er gravid eller planlægger at blive gravid, skal du spørge din læge eller apotekspersonalet til råds, før du bruger dette lægemiddel.
English: The extremely small population of patients with MPS VII globally necessitated the enrolment of all patients able to participate in this clinical trial, resulting in a highly variable group.
Danish: Den ekstremt lille patientpopulation med MPS VII globalt nødvendiggjorde tilmelding af alle patienter, der var i stand til at deltage i dette kliniske forsøg, hvilket resulterede i en yderst varierende gruppe.
English: Take this into account if you are on a controlled sodium diet.
Danish: Tag højde for dette, hvis du er på natriumfattig diæt.
English: This being a marketing authorisation under exceptional circumstances and pursuant to Article 14(8) of Regulation (EC) No 726/2004, the MAH shall complete, within the stated timeframe, the following measures:
Danish: Dette er en betinget markedsføringstilladelse, og i henhold til artikel 14, stk. 8, i forordning (EF) nr. 726/2004 skal indehaveren af markedsføringstilladelsen inden for den fastsatte tidsramme gennemføre følgende foranstaltninger:
English: - Your doctor or nurse will give Mepsevii to you by an infusion (drip) into a vein.
Danish: - Din læge eller sygeplejerske vil give dig Mepsevii ved en infusion (et drop) i en vene.
English: Administration of vestronidase alfa should be carried out by an appropriately trained healthcare professional with the ability to manage medical emergencies.
Danish: Administration af vestronidase alfa bør udføres af en behørigt uddannet sundhedsfaglig person med evnen til at håndtere medicinske nødsituationer.
English: Side effects were mainly seen while patients were being given the medicine or within a day after the infusion (infusion reactions).
Danish: Bivirkninger sås hovedsagelig, mens patienterne fik indgivet medicinen eller inden for en dag efter infusionen (infusionsreaktioner).
English: Other symptoms to look out for
Danish: Andre symptomer, du skal være på vagt over for
English: - Treatment may improve various signs and symptoms of illness, like walking difficulties and tiredness.
Danish: - Behandling kan forbedre forskellige tegn og symptomer på sygdommen, som f.eks. gangbesvær og træthed.
English: Nineteen patients were younger than 18 years of age.
Danish: Nitten patienter var under 18 år.
English: Total Infusion Volume (infused over 4 hours) (mL)
Danish: Samlet infusionsvolumen (infunderet over 4 timer) (ml)
English: Nine patients were younger than 18 years of age.
Danish: Ni patienter var under 18 år.
English: You will not be given Mepsevii if you are pregnant unless treatment is clearly necessary.
Danish: Brug ikke Mepsevii, hvis du er gravid, medmindre det tydeligvis er nødvendigt.
English: Patients should be observed for a minimum of 60 minutes after completing the infusion of vestronidase alfa.
Danish: Patienter skal overvåges i mindst 60 minutter efter afslutning på infusion med vestronidase alfa.
English: In addition, uGAG response (% change from study week 0) shows a similar magnitude of reduction in uGAG in all groups after crossover to active treatment.
Danish: Desuden viser uGAG-respons (procentuel ændring fra studieuge 0) en lignende reduktionsstørrelse i uGAG i alle grupper efter skift til aktiv behandling.
English: Summary of safety profile
Danish: Oversigt over sikkerhedsprofil
English: All 12 patients were responders, pre-specified as ≥ 50% reduction in uGAG on at least one visit during the first 24 weeks of treatment.
Danish: Alle 12 patienter var respondenter, præspecificerede som ≥ 50 % reduktion i uGAG ved mindst et besøg i løbet af de første 24 ugers behandling.
English: Spinal or cervical cord compression is a known and serious complication of MPS VII.
Danish: Kompression af medulla spinalis eller cervicalis er en kendt og alvorlig komplikation ved MPS VII.
English: Most adverse reactions were mild to moderate in severity.
Danish: De fleste bivirkninger var milde til moderate i sværhedsgrad.
English: Colourless glass vial (Ph. Eur.
Danish: Farveløst hætteglas (Ph. Eur.
English: Infusion Rate for Subsequent 3 Hours
Danish: Infusionshastighed for efterfølgende 3 timer
English: After the first hour, the rate can be increased to infuse the remainder of the solution for infusion over 3 hours as tolerated according to the recommended rate guidelines in Table 2.
Danish: Efter den første time kan hastigheden øges for at infundere den resterende infusionsopløsning over 3 timer, som det tolereres i henhold til retningslinjerne for anbefalet hastighed i tabel 2.
English: Ultragenyx Netherlands B. V. Evert van de Beekstraat 1, Unit 104 1118 CL Schiphol The Netherlands
Danish: Ultragenyx Netherlands B. V. Evert van de Beekstraat 1, Unit 104 1118 CL Schiphol Nederlandene
English: The colourless to slightly yellow concentrate must be free of visible particles.
Danish: Farveløst til let gult koncentrat skal være fri for synlige partikler.
English: - The dose is given every two weeks through a drip into a vein (intra-venous infusion).
Danish: - Dosen gives hver anden uge via et drop i en vene (intravenøs infusion).
English: - The recommended dose is 4 mg for each kg of body weight.
Danish: - Den anbefalede dosis er 4 mg for hver kg kropsvægt.
English: There is no definitive correlation between the antibody titre and neutralizing antibody development.
Danish: Der er ingen definitiv korrelation mellem antistoftiteren og udviklingen af neutraliserende antistoffer.
English: - Without enough beta-glucuronidase, parts of these sugars build up in cells, leading to damage in the body.
Danish: - Uden tilstrækkelig beta-glucuronidase opbygges dele af sukkerstofferne i cellerne og resulterer i skader i kroppen.
English: Treatment should be supervised by a healthcare professional experienced in the management of patients with MPS VII or other inherited metabolic disorders.
Danish: Behandling skal overvåges af sundhedspersonale med erfaring i behandling af patienter med MPS VII eller andre arvelige metaboliske sygdomme.
English: Do not infuse Mepsevii with other medicinal products in the infusion tubing.
Danish: Infunder ikke Mepsevii sammen med andre lægemidler i infusionsslangen.
English: Do not shake the solution.
Danish: Undlad at ryste opløsningen.
English: Key Secondary Endpoint: Multi-Domain Clinical Responder Index (MDRI)
Danish: Sekundært nøgleendepunkt: MDRI (Multi-Domain Clinical Responder Index)
English: Patients with MPS VII who received Hematopoietic Stem Cell Transplant therapy were excluded in this study.
Danish: Patienter med MPS VII, som modtog hæmatopoietisk stamcelletransplantationsterapi, var udelukket fra dette studie.
English: The presence of ADA (non-NAb and NAb) does not appear to affect reduction in the pharmacodynamic marker, urinary glycosaminoglycans (uGAGs).
Danish: Forekomsten af ADA (ikke-NAb og NAb) synes ikke at påvirke reduktionen i den farmakodynamiske markør, urin-glycosaminoglycaner (uGAG'er).
English: These side effects are called infusion reactions because they are caused by the infusion (drip) of the medicine.
Danish: Disse bivirkninger kaldes infusionsreaktioner, fordi de forårsages af infusionen af medicinen (via droppet).
English: Infusion Rate for 1st Hour
Danish: Infusionshastighed for 1. time
English: Two other patients with baseline hepatosplenomegaly had reduction in liver volume (24% and 53%) and spleen volume (28% and 47%) after 36 weeks of treatment.
Danish: To andre patienter med hepatosplenomegali ved baseline havde en reduktion i levervolumen (24 % og 53 %) og miltvolumen (28 % og 47 %) efter 36 ugers behandling.
English: The effects of treatment with vestronidase alfa should be periodically evaluated and discontinuation of treatment should be considered in cases where clear benefits (including stabilisation of disease manifestations) are not observed.
Danish: Virkningerne af behandling med vestronidase alfa skal evalueres jævnligt, og det bør overvejes at seponere behandling i tilfælde, hvor der ikke observeres klare fordele (herunder stabilisering af sygdomsmanifestationer).
English: SPECIFIC OBLIGATION TO COMPLETE POST-AUTHORISATION MEASURES FOR THE MARKETING AUTHORISATION UNDER EXCEPTIONAL CIRCUMSTANCES
Danish: SÆRLIG FORPLIGTELSE TIL AT AFSLUTTE FORANSTALTNINGER EFTER UDSTEDELSE AF MARKEDSFØRINGSTILLADELSE TIL LÆGEMIDLER GODKENDT UNDER SÆRLIGE VILKÅR
English: For the clinical (secondary) endpoints, beneficial responses were observed although not in all patients.
Danish: Der observeredes gavnlige reponser for de kliniske (sekundære) endepunkter, dog ikke hos alle patienter.
English: The primary endpoint was the percent reduction in urinary GAG excretion (Dermatan Sulfate, DS) before and after 24 weeks of treatment with vestronidase alfa.
Danish: Det primære endepunkt var procentreduktionen i urinær GAG-udskillelse (dermatansulfat, DS) før og efter 24 ugers behandling med vestronidase alfa.
English: It is important to administer vestronidase alfa according to the recommended infusion rate schedule (see Table 2 in section 6.6).
Danish: Det er vigtigt at administrere vestronidase alfa i henhold til det anbefalede skema for infusionshastighed (se tabel 2 i pkt.
English: - This enzyme helps to break down sugars in the body called mucopolysaccharides.
Danish: - Dette enzym hjælper med til at nedbryde sukkerstoffer i kroppen, der kaldes mukopolysakkarider.
English: MedDRA Preferred Term
Danish: Foretrukken term i henhold til MedDRA
English: The infusion was stopped, the patient received anticonvulsants, antipyretics and antibiotics, and the febrile convulsion resolved.
Danish: Infusionen blev stoppet, patienten fik anticonvulsiva, antipyretika og antibiotika, og feberkrampen faldt til ro.
English: The safety and efficacy of vestronidase alfa in patients older than 65 years have not been established.
Danish: Virkningen og sikkerheden af vestronidase alfa hos patienter, der er ældre end 65 år, er ikke fastlagt.
English: **One adverse reaction of Peripheral swelling is included within the frequency of Infusion site swelling as the event is classified as intravenous catheter issue.
Danish: **En bivirkning i form af perifer hævelse er medtaget i hyppigheden af hævelser på infusionsstedet, da hændelsen er klassificeret som et problem med et intravenøst kateter.
English: Because it is a recombinant human protein and its enzyme action is within the lysosome, vestronidase alfa is not expected to interact with other medicinal products.
Danish: Da det er et rekombinant humant protein, og dets enzymaktion er inden for lysosomet, forventes vestronidase alfa ikke at interagere med andre lægemidler.
English: Do not flush the line containing vestronidase alfa to avoid a rapid bolus of infused enzyme.
Danish: Skyl ikke slangen med vestronidase alfa for at undgå en hurtig bolus af infunderet enzym.
English: It is not known whether Mepsevii passes into breast milk, but transfer of the medication to your baby is not expected.
Danish: Det vides ikke, om Mepsevii overføres til modermælk, men lægemidlet forventes ikke at blive overført til dit barn.
English: • Periodic safety update reports
Danish: • Periodiske opdaterede sikkerhedsindberetninger (PSUR'er)
English: This medicine replaces beta-glucuronidase - this helps to break down the sugars that collect in the tissues of people with MPS VII.
Danish: Dette lægemiddel erstatter beta-glucuronidase - der er med til at nedbryde de sukkerstoffer, som samler sig i vævet på personer med MPS VII.
English: In order to obtain long-term data on effectiveness and safety of treatment with Mepsevii and to characterize the entire mucopolysaccharidosis VII, including variability of clinical manifestation, progression and natural history, the MAH is requested to submit the results of a study based on adequate source of data deriving from a Disease Monitoring Program of patients with mucopolysaccharidosis VII.
Danish: For at indhente langsigtede data om effekten og sikkerheden af behandling med Mepsevii og for at karakterisere hele mucopolysakkaridose VII, herunder variabiliteten af den kliniske manifestation, progression og naturlige historik, anmodes indehaveren af markedsføringstilladelsen om at fremsende resultaterne af forsøget baseret på tilstrækkelige data, der er afledt fra et sygdomsmonitoreringsprogram med patienter med mucopolysakkaridose VII.
English: Talk to your doctor before you are given Mepsevii.
Danish: Kontakt lægen, før du får Mepsevii.
English: Clinical endpoints were not assessable in some patients due to their extent of disease, age or level of cognition (23 out of 72 assessments [~32%] in 6 domains for 12 patients were non-assessable at baseline).
Danish: De kliniske endepunkter kunne ikke vurderes hos alle patienter på grund af omfanget af sygdom, alder eller kognitionsniveau (23 ud af 72 vurderinger [~32 %] på 6 domæner for 12 patienter kunne ikke vurderes ved baseline).
English: Gently rock the infusion bag to ensure proper distribution of Mepsevii.
Danish: Bevæg infusionsposen forsigtigt for at sikre en korrekt fordeling af Mepsevii.
English: The total infusion volume is based on the total Mepsevii dose and volume (refer to Table 2).
Danish: Det samlede infusionsvolumen er baseret på den samlede dosis Mepsevii og volumenet (se Tabel 2).
English: This event was assessed as possibly related to vestronidase alfa due to the temporal association with the infusion.
Danish: Denne hændelse blev vurderet til muligvis at være relateret til vestronidase alfa på grund af den tidsmæssige forbindelse med infusionen.
English: Mepsevii contains an enzyme called vestronidase alfa.
Danish: Mepsevii indeholder et enzym, der kaldes vestronidase alfa.
English: Pharmacotherapeutic group: Other alimentary tract and metabolism products, enzymes, ATC code: A16AB18
Danish: Farmakoterapeutisk klassifikation: Andre produkter til fordøjelseskanalen og stofskiftet, enzymer, ATC-kode: A16AB18
English: - Mucopolysaccharides are made in the body and they help build bones, cartilage, skin, and tendons.
Danish: - Mukopolysakkarider dannes i kroppen og er med til at opbygge knogler, brusk, hud og sener.
English: Each vial dosed is therefore equivalent to 1.8% of the recommended maximum daily dietary intake of sodium for an adult.
Danish: Hvert hætteglas svarer derfor til 1,8 % af det anbefalede maksimale daglige indtag af natrium for en voksen.
English: Nonclinical data reveal no special hazard for humans based on conventional studies of safety pharmacology, single-dose toxicity in rats, repeated dose toxicity in MPS VII mice and juvenile monkeys, fertility and embryo-foetal development in rats or rabbits, and pre- and postnatal development in rats.
Danish: Non-kliniske data viser ingen speciel fare for mennesker baseret på konventionelle studier af sikkerhedsfarmakologi, enkeltdosistoksicitet hos rotter, gentagen dosistoksicitet hos MPS VII-mus og unge aber, fertilitets- og embryo-føtal udvikling hos rotter og kaniner, og præ- og postnatal udvikling hos rotter.
English: This should be taken into consideration during dilution of the medicinal product for patients on a controlled sodium diet or for those patients with congestive heart failure needing to restrict sodium and total water intake.
Danish: Det bør tages i betragtning under fortynding af lægemidlet for patienter på en kontrolleret natriumdiæt eller for patienter med kongestiv hjerteinsufficiens, der skal begrænse indtaget af natrium og vand.
English: If not used immediately, in-use storage times and conditions prior to use are the responsibility of the user, but should normally not be longer than 36 hours at 2°C - 8°C followed by up to 6 hours at room temperature up to a maximum of 25°C.
Danish: Hvis det ikke anvendesstraks, er brugsopbevaringstider og forhold før anvendelse brugerens ansvar, men må normalt ikke være mere end 36 timer ved 2 °C - 8 °C efterfulgt af op til 6 timer ved stuetemperatur i op til maksimalt 25 °C.
English: The lowest rate administered to a patient in the clinical development program was 0.5 mL/hour during the first 30 minutes of infusion, followed by 1 mL/hour over the next 30 minutes, equalling 0.75 mL as the lowest total volume infused during the first hour.
Danish: Den laveste hastighed, der blev administreret til en patient i det kliniske udviklingsprogram, var 0,5 ml/time i løbet af de første 30 minutters infusion efterfulgt af 1 ml/time over de næste 30 minutter, hvilket svarer til 0,75 ml som den laveste samlede mængde, der blev infunderet i løbet af den første time.
English: Look out for side effects
Danish: Vær på vagt over for bivirkninger
English: The total diluted volume of the solution for infusion should be administered with a rate titration regimen over approximately 4 hours.
Danish: Den samlede fortyndede infusionsopløsning skal administreres ifølge et regime med en titreringshastighed over ca. 4 timer.
English: Discuss with your doctor if the benefits of using Mepsevii are greater than the possible risks to your unborn baby.
Danish: Drøft med din læge, om fordelene ved at bruge Mepsevii er større end de mulige risici for dit ufødte barn.
English: Block-7, City North Business Campus Stamullen, Co. Meath, Ireland
Danish: Millmount Healthcare Ltd. Block-7, City North Business Campus Stamullen, Co. Meath, Irland
English: Do not use if the solution is discoloured or if there is particulate matter in the solution.
Danish: Anvend ikke opløsningen, hvis den er misfarvet, eller der er partikler i opløsningen.
English: After 120 weeks of exposure to vestronidase alfa, one patient demonstrated a 21% improvement over baseline in forced vital capacity (FVC% predicted) on pulmonary function testing in addition to a 105 meter improvement in the 6MWT.
Danish: Efter 120 ugers eksponering for vestronidase alfa viste en patient en forbedring på 21 % over baseline i FVC (forced vital capacity) (FVC% forudsagt) på lungefunktionstestning udover en forbedring på 105 meter i 6MWT.
English: Each vial of Mepsevii is intended for single use only.
Danish: Hvert hætteglas med Mepsevii er beregnet til engangsbrug.
English: Vestronidase alfa is a recombinant human enzyme and is therefore eliminated by proteolytic degradation into small peptides and amino acids.
Danish: Vestronidase alfa er et rekombinant humant enzym og elimineres derfor ved proteolytisk degradering i små peptider og aminosyrer.
English: It is not known whether vestronidase alfa is excreted in human milk, but systemic exposure via breast-milk is not expected.
Danish: Det er ukendt, om vestronidase alfa udskilles i human mælk, men systemisk eksponering via human mælk forventes ikke.
English: No alternative dose regimen is recommended in these patients (see section 5.1).
Danish: Intet alternativt dosisregimen anbefales til disse patienter (se pkt.
English: It is used in adults and children of all ages with MPS VII to treat nonneurological manifestations of the disease (mucopolysaccharidosis VII, also known as Sly Syndrome).
Danish: Det anvendes hos voksne og børn i alle aldre med MPS VII til at behandle ikke-neurologiske manifestationer af sygdommen (mukopolysakkaridose VII, også kendt som Sly-syndrom).
English: Vestronidase alfa, at the exposure observed in humans, is not expected to cross the blood-brain-barrier and therefore it is not likely to impact the neurological manifestations of the disease.
Danish: Ved den observerede eksponering hos mennesker forventes vestronidase alfa ikke at krydse blodhjerne-barrieren, og det er derfor ikke sandsynligt, at det vil indvirke på sygdommens neurologiske manifestationer.
English: The infusion rate may be slowed, temporarily interrupted or discontinued in the event of hypersensitivity reactions (see section 4.4).
Danish: Infusionshastigheden kan sænkes, afbrydes midlertidigt eller helt i tilfælde af overfølsomhedsreaktioner (se pkt.
English: The rate of infusion should be as follows: in the first hour, 2.5% of the total volume will be infused, with the balance infused over the subsequent three hours.
Danish: Infusionshastigheden skal være som følger: I den første time infunderes 2,5 % af den samlede mængde, og resten infunderes over de efterfølgende tre timer.
English: Table 1 Adverse reactions reported in patients treated with Mepsevii MedDRA System Organ Class
Danish: Table 1 Bivirkninger indberettet af patienter, der er blevet behandlet med Mepsevii Systemorganklasse i henhold til
English: The recommended dose of vestronidase alfa is 4 mg/kg of body weight administered by intravenous infusion every two weeks.
Danish: Den anbefalede dosis vestronidase alfa er 4 mg/kg legemsvægt, som administreres ved intravenøs infusion hver anden uge.
English: - Store in a refrigerator (2°C to 8°C).
Danish: - Opbevares i køleskab (2 °C - 8 °C).
English: Sodium dihydrogen phosphate dihydrate Sodium chloride
Danish: Natriumhydrogenphosphatdihydrat Natriumchlorid
English: Round to the next whole vial and remove the required number of vials (refer to Table 2) from the refrigerator to allow them to reach room temperature up to a maximum of 25°C.
Danish: Rund op til det næste hele hætteglas, og tag det påkrævede antal hætteglas (se Tabel 2) ud af køleskabet for at lade dem nå stuetemperatur op til højst 25 °C.
English: Table 1 lists the adverse reactions reported from 4 clinical trials in 23 patients treated with Mepsevii.
Danish: Tabel 1 angiver de bivirkninger, der blev indberettet fra 4 kliniske forsøg med 23 patienter, der blev behandlet med Mepsevii.