FrancophonIA/ELRC3.0 huggingface dataset:
English: Sustained improvements in efficacy measures and reductions in urine keratan sulphate (KS) over time were observed across studies, despite the presence of anti elosulfase alfa antibodies.
Danish: Vedvarende forbedringer af effektmål og reduktioner i urin-keratansulfat (KS) over tid blev observeret over flere studier, trods tilstedeværelsen af anti elosulfase alfa-antistoffer.
English: If you have any further questions on the use of this medicine, ask your doctor or nurse.
Danish: Er du i tvivl om brugen af denne medicin, så spørg lægen eller sygeplejersken.
English: It is used to treat adults and children with mucopolysaccharidosis type IVA (MPS IVA disease, also known as Morquio A Syndrome).
Danish: Det bruges til at behandle voksne og børn med mukopolysakkaridose type IVA (sygdommen MPS IVA, også kendt som Morquie A syndrom).
English: The infusion bag is gently rotated to ensure proper distribution of Vimizim.
Danish: Infusionsposen roteres forsigtigt for at sikre en korrekt fordeling af Vimizim.
English: Animal studies do not indicate direct or indirect harmful effects with respect to pregnancy or embryofoetal development (see section 5.3).
Danish: Dyreforsøg indikerer hverken direkte eller indirekte skadelige virkninger, hvad angår graviditet reproduktionstoksicitet (se pkt.
English: The number of vials to be diluted based on the individual patient's weight and the recommended dose of 2 mg/kg is determined, using the following calculation:
Danish: Antallet af hætteglas, der skal fortyndes, baseret på den enkelte patients vægt og den anbefalede dosis på 2 mg/kg bestemmes ved hjælp af følgende beregning:
English: Despite the alteration of the pharmacokinetics profile, presence of neutralizing antibodies did not affect pharmacodynamics, efficacy, or safety of the patients who were treated with elosulfase alfa.
Danish: På trods af ændringen af den farmakokinetiske profil påvirkede forekomsten af neutraliserende antistoffer ikke farmakodynamik, effekt eller sikkerhed for de patienter, der blev behandlet med elosulfase alfa.
English: Common side effects observed was muscle pain.
Danish: Almindeligt observerede bivirkninger var muskelsmerter.
English: Anaphylaxis and severe allergic reactions have been reported in clinical studies.
Danish: Anafylaksi og svære allergiske reaktioner er blevet rapporteret i kliniske studier.
English: You should not receive Vimizim during pregnancy unless clearly necessary.
Danish: Du må ikke få Vimizim under graviditet, medmindre det er klart nødvendigt.
English: Vimizim has minor influence on the ability to drive and use machines.
Danish: Vimizim påvirker i mindre grad evnen til at føre motorkøretøj og betjene maskiner.
English: The total volume of the infusion is determined by the patient's body weight.
Danish: Det samlede infusionsvolumen bestemmes af patientens kropsvægt.
English: Preparation of the Vimizim infusion (Use aseptic technique)
Danish: Forberedelse af Vimiziminfusion (brug aseptisk teknik)
English: Tell your doctor if you feel dizzy after your infusion, especially before driving or using any machine where dizziness might be dangerous.
Danish: Fortæl din læge, hvis du føler dig svimmel efter infusionen, især før du fører motorkøretøj eller betjener nogen maskiner, hvor svimmelhed kan være farlig.
English: Do not use Vimizim if the solution is discoloured or it contains visible particles.
Danish: Brug ikke Vimizim, hvis opløsningen er misfarvet eller den indeholder synlige partikler.
English: • the calculation of the dose and of the volume of infusion
Danish: • beregning af dosis og infusionsvolumenet
English: If you experience any difficulty swallowing, talking, severe shortness of breath or wheezing, swelling of the face or lips, dizziness or weak pulse; these may represent symptoms of a severe allergic reaction and you should tell your doctor immediately.
Danish: Hvis du oplever besvær med at synke eller tale, alvorlig stakåndethed eller pibende åndedræt, hævelse af ansigt eller læber, svimmehed eller svag puls: Dette kan være symptomer på en alvorlig allergisk reaktion, og du skal straks fortælle det til din læge.
English: The primary endpoint was the change from baseline in the 6 MWT distance compared to placebo at Week 24.
Danish: Det primære endepunkt var ændringen fra baseline i 6 MWT-afstanden sammenlignet med placebo i uge 24.
English: No association was apparent between the total antibody titre and elosulfase clearance.
Danish: Der var ingen synlig sammenhæng mellem den totale antistoftiter og elosulfase clearance.
English: Available reproductive data in animals have shown excretion of elosulfase alfa in milk.
Danish: De foreliggende reproduktionsdata hos dyr har vist udskillelse af elosulfase alfa i mælk.
English: Vimizim is indicated for the treatment of mucopolysaccharidosis, type IVA (Morquio A Syndrome, MPS IVA) in patients of all ages.
Danish: Vimizim er indiceret til behandling af mukopolysakkaridose, type IVA (Morquio A syndrom, MPS IVA) hos patienter i alle aldre.
English: In clinical studies, doses of elosulfase alfa were explored up to 4 mg/kg per week and no specific signs or symptoms were identified following the higher doses.
Danish: I kliniske studier blev doser med elosulfase alfa på op til 4 mg/kg per uge undersøgt, og der blev ikke identificeret nogen specifikke tegn eller symptomer efter de høje doser.
English: If these reactions occur, immediately stop the infusion and initiate appropriate medical treatment.
Danish: Hvis sådanne reaktioner forekommer, skal infusionen straks stoppes, og der skal indledes passende medicinsk behandling.
English: Your doctor may also give you additional medicines to manage any allergic reactions (e.g. antihistamines and/or corticosteroids).
Danish: Din læge kan også give dig ekstra medicin til at håndtere eventuelle allergiske reaktioner (f.eks. antihistaminer og/eller kortikosteroider).
English: IRs may include allergic reactions.
Danish: Infusionsreaktioner kan omfatte allergiske reaktioner.
English: As a precautionary measure, it is preferable to avoid the use of Vimizim during pregnancy, unless clearly necessary.
Danish: Som en forebyggende foranstaltning bør brug af Vimizim under graviditet undgås, medmindre det er klart nødvendigt.
English: The calculated volume of Vimizim from the appropriate number of vials is slowly withdrawn using caution to avoid excessive agitation.
Danish: Den beregnede mængde Vimizim fra det passende antal hætteglas trækkes langsomt ud med forsigtighed for at undgå overdreven omrystning.
English: The most serious side effects were severe allergic reactions (seen uncommonly - may affect up to 1 in 100 people) and mild to moderate vomiting (seen very commonly - may affect more than 1 in 10 people).
Danish: De mest alvorlige bivirkninger var svære allergiske reaktioner (ikkealmindelig - kan påvirke op til 1 ud af 100 personer) og lette til moderate opkastninger (meget almindelig - kan påvirke mere end 1 ud af 10 personer).
English: This medicine replaces the natural enzyme N-acetylgalactosamine-6-sulfatase which is lacking in MPS IVA patients.
Danish: Dette lægemiddel erstatter det naturlige enzym N-acetylgalaktosamin-6-sulfatase, som mangler hos MPS IVA patienter.
English: Table 2: Adverse reactions in patients treated with Vimizim
Danish: Tabel 2: Bivirkninger hos patienter behandlet med Vimizim
English: • Summary of Product Characteristics and Patient Information Leaflet
Danish: • Produktresumé og patientinformationsark
English: Patients should be monitored for signs and symptoms of SCC (including back pain, paralysis of limbs below the level of compression, urinary and faecal incontinence) and given appropriate clinical care.
Danish: Patienterne bør overvåges for tegn og symptomer på SCC (herunder rygsmerter, lammelse af lemmer under kompressionsniveauet, urin og fækal inkontinens) og gives relevant klinisk pleje.
English: Clinical studies performed with Vimizim assessed the impact of treatment on the systemic manifestations of MPS IVA in various domains including endurance, respiratory function, growth velocity, and mobility, as well as urine KS.
Danish: Kliniske studier, som er udført med Vimizim, vurderede virkningen af behandling på de systemiske manifestationer af MPS IVA inden for forskellige områder, herunder udholdenhed, respiratorisk funktion, væksthastighed og mobilitet samt KS i urinen.
English: Patients with hereditary fructose intolerance (HFI) must not be given this medicinal product unless strictly necessary.
Danish: Må ikke anvendes til patienter med hereditær fructoseintolerans (HFI), medmindre det er strengt nødvendigt.
English: Each infusion will be given over approximately 4 hours.
Danish: Hver infusion gives i løbet af ca. 4 timer.
English: No accumulation of elosulfase alfa in plasma was evident following weekly dosing
Danish: Der var ingen tydelig ophobning af elosulfase alfa i plasma efter den ugentlige dosering.
English: In additional extension studies, patients receiving elosulfase alfa 2 mg/kg every week, showed maintenance of initial improvement in endurance and sustained reduction of urinary KS up to 156 weeks.
Danish: I yderligere udvidede studier viste det sig, at patienter, der fik elosulfase alfa 2 mg/kg hver uge, opretholdt den indledende forbedring i udholdenhed og vedvarende reduktion af KS i urinen i op til 156 uger.
English: Symptoms of allergic reaction include rash, itching or hives on the skin (seen commonly - may affect up to 1 in 10 people).
Danish: Symptomer på allergisk reaktion omfatter udslæt, kløe eller nældefeber på huden (almindeligt forekommende - kan påvirke op til 1 ud af 10 personer).
English: Approximately 80% of patients developed neutralizing antibodies capable of inhibiting the elosulfase alfa from binding to the cationindependent mannose-6-phosphate receptor.
Danish: Cirka 80 % af patienterne udviklede neutraliserende antistoffer, som var i stand til at hindre elosulfase alfa i at binde sig til den kation-uafhængige mannose-6-phosphat-receptor.
English: Step 1 Initial infusion rate 0-15 minutes (ml/hr)
Danish: Trin 1 Indledende infusionshasti ghed 0-15 minutter (ml/t)
English: (CI95, -2.1, 4.4) (p = 0.4935)
Danish: (CI95, -2,1 og (p = 0,4935)
English: Any unused product or waste material is to be disposed of in accordance with local requirements.
Danish: Alle ubrugte produkter eller affald skal bortskaffes i overensstemmelse med lokale retningslinjer.
English: • Obligation to conduct post-authorisation measures
Danish: • Forpligtigelse til at gennemføre foranstaltninger efter udstedelse af markedsføringstilladelsen
English: Administration of Vimizim should be carried out by an appropriately trained healthcare professional with the ability to manage medical emergencies.
Danish: Vimizim skal administreres af en behørigt uddannet sundhedsfaglig person, som kan håndtere kliniske nødsituationer.
English: Pharmacokinetic parameter
Danish: Farmakokinetiske parametre
English: Sodium acetate trihydrate;
Danish: Natriumacetat trihydrat;
English: All patients developed antibodies to elosulfase alfa in clinical studies.
Danish: Alle patienter udviklede antistoffer mod elosulfase alfa i kliniske studier.
English: MPS IVA is characterised by the absence or marked reduction in N-acetylgalactosamine-6-sulfatase activity.
Danish: MPS IVA er karakteriseret ved fravær eller markant reduktion i N-acetylgalaktosamin-6sulfatase-aktivitet.
English: Each vial of Vimizim is intended for single use only.
Danish: Hvert hætteglas med Vimizim er kun beregnet til engangsbrug.
English: The data in Table 2 below describes adverse reactions from clinical studies in patients treated with Vimizim.
Danish: Dataene i tabel 2 nedenfor beskriver bivirkninger fra kliniske studier hos patienter behandlet med Vimizim.
English: Tmax, minute¶ * AUC0-t, area under the plasma concentration-time curve from time zero to the time of last measurable concentration; † Cmax, observed maximum plasma concentration; ‡ CL, total clearance of elosulfase alfa after intravenous administration; § t1/2, elimination half-life; ¶ Tmax, time from zero to maximum plasma concentration
Danish: * AUC0-t, areal under plasmakoncentrationstids-kurven fra tid nul til tiden for den sidste målbare koncentration; † Cmax, højest observerede plasmakoncentration; ‡ CL, total clearance af elosulfase alfa efter intravenøs indgivelse; § t1/2, halveringstid for eliminering; ¶ Tmax tid fra nul til maksimal plasmakoncentration
English: The majority of patients who received Vimizim during clinical studies were in the paediatric and adolescent age range (5 to 17 years).
Danish: Hovedparten af de patienter, der fik Vimizim i løbet af de kliniske studier, var børn og unge i aldersgruppen 5 til 17 år.
English: Each 5 ml vial contains 8 mg sodium and 100 mg sorbitol (E420).
Danish: Hvert 5 ml hætteglas indeholder 8 mg natrium og 100 mg sorbitol (E420).
English: The calculated total number of vials is rounded up to next whole vial.
Danish: Det beregnede samlede antal hætteglas rundes op til næste hele hætteglas.
English: - If you experience back pain, numbness in your arms or legs, or lack of control over passing urine or stools, you should immediately contact your doctor.
Danish: - Hvis du oplever rygsmerter, følelsesløshed i arme eller ben eller manglende kontrol over urin eller afføring, skal du straks kontakte din læge.
English: The other ingredients are: sodium acetate trihydrate, sodium dihydrogen phosphate monohydrate, arginine hydrochloride, sorbitol, polysorbate 20, and water for injections (see section 2 under ‘Vimizim contains sodium and sorbitol (E420)').
Danish: Øvrige indholdsstoffer: natriumacetattrihydrat, natriumdihydrogenphosphatmonohydrat, argininhydrochlorid, sorbitol, polysorbat 20 og vand til injektioner (se punkt 2 under ”Dette lægemiddel indeholder natrium og sorbitol (E420)”).
English: Non-clinical data revealed no special hazard for humans based on conventional studies of safety pharmacology evaluating central nervous, respiratory and cardiovascular systems, single-dose and repeated-dose toxicity in rats and monkeys or fertility and embryo-foetal development in rats or rabbits.
Danish: Prækliniske data viser ingen speciel risiko for mennesker vurderet ud fra konventionelle studier af sikkerhedsfarmakologi, der evaluerer centralnervesystemet, luftvejs- og hjerte-kar-systemerne, toksicitet efter en enkelt dosis og efter gentagne doser hos rotter og aber eller reproduktions- og udviklingstoksicitet hos rotter eller kaniner.
English: For instructions for dilution of the medicinal product prior to administration, see section 6.6.
Danish: For instruktioner om fortynding af lægemidlet før administration, se pkt.
English: Impact of antibodies on elosulfase alfa pharmacokinetics was assessed.
Danish: Virkningen af antistoffer på elosulfase alfas farmakokinetik blev vurderet.
English: In patients < 5 years of age, the overall safety profile of Vimizim at 2 mg/kg/week was consistent with the safety profile of Vimizim observed in older children.
Danish: Hos patienter i alderen < 5 år stemte den samlede sikkerhedsprofil for Vimizim 2 mg/kg/uge overens med sikkerhedsprofilen for Vimizim hos ældre børn.
English: IgE antibodies against elosulfase alfa were detected in ≤ 10% of treated patients and have not consistently been related to anaphylaxis or other hypersensitivity reactions and/or treatment withdrawal.
Danish: IgE-antistoffer mod elosulfase alfa blev registreret hos ≤ 10 % af de behandlede patienter og har ikke konsekvent været forbundet med anafylakse eller andre overfølsomhedsreaktioner og/eller tilbagetrækning fra behandling.
English: The most common symptoms of IRs (occurring in ≥ 10% of patients treated with Vimizim and ≥ 5% more when compared to placebo) were headache, nausea, vomiting, pyrexia, chills and abdominal pain.
Danish: De mest almindelige symptomer på infusionsreaktioner (forekommer hos ≥ 10 % af patienterne behandlet med Vimizim og ≥ 5 % mere i forhold til placebo) var hovedpine, kvalme, opkastning, pyreksi, kuldegysninger og mavesmerter.
English: After the first 52 weeks of treatment the normalised standing height z-score was -1.9 (±1.62).
Danish: Efter de første 52 ugers behandling var den normaliserede ståhøjdes z-score -1,9 (±1,62).
English: Consequently, impaired liver function is not expected to affect the pharmacokinetics of elosulfase alfa.
Danish: Som følge heraf forventes nedsat leverfunktion ikke at påvirke farmakokinetikken for elosulfase alfa.
English: Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness.
Danish: Inden for hver hyppighedsgruppering anføres bivirkningerne efter faldende sværhedsgrad.
English: Chemical and physical in-use stability has been demonstrated for up to 24 hours at 2C - 8C followed by up to 24 hours at 23C - 27C.
Danish: Kemisk og fysisk stabilitet i brug er blevet påvist i op til 24 timer ved 2 C - 8 C efterfulgt af 24 timer ved 23 C - 27 C.
English: The pharmacokinetic parameters of elosulfase alfa were evaluated in 23 patients with MPS IVA who received weekly intravenous infusions of 2 mg/kg of elosulfase alfa over approximately 4 hours for 22 weeks and the parameters at Week 0 and Week 22 were compared.
Danish: De farmakokinetiske parametre for elosulfase alfa blev evalueret hos 23 patienter med MPS IVA, der modtog ugentlige, intravenøse infusioner med 2 mg/kg elosulfase alfa over ca. 4 timer i 22 uger, og parametrene i uge 0 og uge 22 blev sammenlignet.
English: Table 4: Pharmacokinetic properties
Danish: Tabel 4: Farmakokinetiske egenskaber
English: *Elosulfase alfa is a recombinant form of human N-acetylgalactosamine-6-sulfatase (rhGALNS) and is produced in Chinese Hamster Ovary cell culture by recombinant DNA technology.
Danish: * Elosulfase alfa er en rekombinant form for human N-acetylgalaktosamin-6-sulfatase (rhGALNS) og er fremstillet ved dyrkning af ovarieceller fra kinesiske hamstre ved hjælp af rekombinant DNAteknologi.
English: Reproduction studies have been performed in rats at doses up to 10 times the human dose and have revealed no evidence of impaired fertility or reproductive performance.
Danish: Reproduktionsforsøg er udført på rotter med doser på op til 10 gange den humane dosis og har ikke afsløret nogen evidens for forringet fertilitet eller forplantningsevne.
English: Total infusion volume (ml)
Danish: Samlet infusionsvolu men (ml)
English: The secondary endpoints were the change from baseline in the 3 Minute Stair Climb Test (MSCT) and urine KS levels at Week 24.
Danish: De sekundære endepunkter var ændringen fra baseline i 3-minutters trappetesten (MSCT) og niveauerne af KS i urinen i uge 24.
English: Patients should receive antihistamines with or without antipyretics prior to infusion (see section 4.2).
Danish: Patienter bør modtage antihistaminer med eller uden antipyretika forud for infusion (se pkt.
English: Week 0 Mean (SD)
Danish: Uge 0 Middel (SD)
English: Diarrhoea, vomiting, oropharyngeal pain, upper abdominal pain, abdominal pain, nausea
Danish: Diarré, opkastning, smerter i svælget, smerte i øvre abdomen, mavesmerter, kvalme
English: The accumulation leads to widespread cellular, tissue, and organ dysfunction.
Danish: Ophobningen fører til udbredt celle-, vævs- og organdysfunktion.
English: Week 22 Mean (SD)
Danish: Uge 22 Middel (SD)
English: What you need to know before you are given Vimizim 3.
Danish: Det skal du vide, før du begynder at tage Vimizim 3.
English: Mucopolysaccharidoses comprises a group of lysosomal storage disorders caused by the deficiency of specific lysosomal enzymes required for the catabolism of glycosaminoglycans (GAG).
Danish: Mukopolysakkaridoser består af en gruppe af lysosomale ophobningssygdomme forårsaget af mangel på specifikke lysosomale enzymer, der er nødvendige for katabolisme af glykosaminoglykaner (GAG).
English: What Vimizim contains
Danish: Vimizim indeholder
English: A total of 173 patients subsequently enrolled in an extension study in which patients received 2 mg/kg of elosulfase alfa every week or 2 mg/kg every other week, and then all were switched to 2 mg/kg every week upon availability of the Week 24 results.
Danish: I alt 173 patienter blev efterfølgende indskrevet i et udvidet studie, hvor patienterne fik 2 mg/kg elosulfase alfa hver uge eller 2 mg/kg hver anden uge, og dernæst blev alle ændret til 2 mg/kg hver uge, efter at resultaterne fra uge 24 var til rådighed.
English: The number of vials to be diluted is based on the individual patient's weight.
Danish: Antal hætteglas, der skal fortyndes, er baseret på den enkelte patients vægt.
English: They accumulate in the tissues interfering with their normal function and causing the symptoms of MPS IVA, such as difficulty walking, trouble breathing, short height, and hearing loss.
Danish: De hober sig op i mange væv, hvilket forstyrrer deres normale funktion forårsager symptomerne på MPS IVA, f.eks. besvær med at gå, besvær med vejrtrækningen, lav legemshøjde og nedsættelse hørelsen.
English: Management of IRs should be based on the severity of the reaction and include slowing or temporary interruption of the infusion and/or administration of additional antihistamines, antipyretics, and/or corticosteroids.
Danish: Håndtering af infusionsreaktioner bør være baseret på sværhedsgraden af reaktionen og omfatte opbremsning eller midlertidig afbrydelse af infusionen og/eller administration af yderligere antihistaminer, antipyretika og/eller kortikosteroider.
English: • Patient weight (kg) multiplied by 2 (mg/kg) = Patient dose (mg)
Danish: • Patientens vægt (kg) ganget med 2 (mg/kg) = patientdosis (mg)
English: Water for injections;
Danish: Vand til injektionsvæsker;
English: No correlations were found between higher antibody titres or neutralizing antibody positivity and reductions in efficacy measurements or occurrence of anaphylaxis or other hypersensitivity reactions.
Danish: Der blev ikke fundet nogen korrelationer mellem højere antistoftitere eller neutraliserende antistof-positivitet og reduktioner i effektmål eller forekomst af anafylakse eller andre overfølsomhedsreaktioner.
English: The sulfatase activity deficiency results in the accumulation of the GAG substrates, KS and chondroitin 6 sulphate (C6S), in the lysosomal compartment of cells throughout the body.
Danish: Mangel på sulfatase-aktivitet resulterer i ophobning af GAG-substrater, KS og kondroitin-6-sulfat (C6S), i det lysosomale rum i cellerne i hele kroppen.
English: The baseline mean (±SD) normalised standing height z-score was - 1.6 (±1.61).
Danish: Baselines gennemsnitlige, (±SD) normaliserede ståhøjdes z-score var -1,6 (±1,61).
English: Very common side effects include symptoms of infusion reactions such as headache, nausea, fever, chills and stomach ache.
Danish: Meget almindelige bivirkninger omfatter symptomer på infusionsreaktioner som f.eks. hovedpine, kvalme, feber, kuldegysninger og mavepine.
English: In an open-label study, 15 paediatric patients with MPS IVA under the age of 5 years (9 months to <5 years) received 2 mg/kg of Vimizim once a week for 52 weeks.
Danish: I et ikke-blindet studie fik 15 pædiatriske patienter med MPS IVA i alderen under 5 år (9 måneder til < 5 år) 2 mg/kg Vimizim 2 mén gang ugentlig i 52 uger.
English: Clear glass vial (Type I) with a butyl rubber stopper and a flip-off crimp seal (aluminium) with a plastic cap.
Danish: Klart hætteglas (type I) med butylgummiprop og flip-off krympepakning (aluminium) med en plastikhætte.
English: Because of the potential for hypersensitivity reactions with elosulfase alfa, patients should receive antihistamines with or without antipyretics 30 to 60 minutes prior to start of infusion (see section 4.4).
Danish: På grund af risikoen for overfølsomhedsreaktioner med elosulfase alfa bør patienterne medicineres med antihistaminer med eller uden antipyretika 30 til 60 minutter før, infusionen påbegyndes (se pkt.
English: • the calculation of the infusion rate
Danish: • beregning af infusionraten