FrancophonIA/ELRC3.0 huggingface dataset:
English: When this enzyme is missing or does not work properly, these sugar compounds build up inside cells and cause the signs and symptoms of the disease.
Danish: Når dette enzym mangler eller ikke virker korrekt, ophobes disse sukkerstoffer i cellerne, og forårsager tegn og symptomer på sygdommen.
English: (1) Preferred terms considered as IRR as described in the section below (2) Selected adverse reaction as described in the section below
Danish: (1) Foretrukken terminologi, der anses for at være en IRR, som beskrevet i punktet nedenfor (2) Udvalgt bivirkning, som beskrevet i afsnittet nedenfor
English: As no patients older than 41 years have been identified across Europe, no relevant use in elderly patients is expected.
Danish: Da der ikke er blevet identificeret patienter over 41 år i Europa, forventes det ikke, at det er relevant at bruge lægemidlet til ældre patienter.
English: In such a case, treatment with an antihistamine and corticosteroids should be considered as being appropriate.
Danish: I sådanne tilfælde skal behandling med et antihistamin og kortikosteroider anses for at være passende.
English: From the total reconstituted volume, only 22 mL (corresponding to 44 mg) should be administered.
Danish: Fra det totale rekonstituerede volumen, må kun 22 ml (svarende til 44 mg) administreres.
English: Filter needles must not be used during preparation.
Danish: Der må ikke anvendes filterkanyler under forberedelsen.
English: The development of antibodies has not been shown to affect clinical efficacy or safety.
Danish: Udviklingen af antistoffer har ikke vist sig at påvirke klinisk virkning eller sikkerhed.
English: Paediatric population No dose adjustment is necessary for the paediatric population.
Danish: Pædiatrisk population En dosisjustering er ikke nødvendig for den pædiatriske population.
English: No clinical data are available in children below the age of 6 years.
Danish: Der foreligger ingen kliniske data hos børn under 6 år.
English: Pre-treatment with antihistamines and/or corticosteroids may prevent subsequent reactions in those cases where symptomatic treatment was required.
Danish: Forbehandling med antihistaminer og/eller kortikosteroider kan forhindre efterfølgende reaktioner i de tilfælde, hvor en symptomatisk behandling var nødvendig.
English: Placebo 12 months
Danish: Placebo 12 måneder
English: The following information is intended for healthcare professionals only.
Danish: Nedenstående oplysninger er til læger og sundhedspersonale.
English: Rentschler Biopharma SE Erwin-Rentschler-Strasse 21 88471 Laupheim Germany
Danish: Rentschler Biopharma SE Erwin-Rentschler-Strasse 21 88471 Laupheim Tyskland
English: Instructions for reconstitution and administration
Danish: Instruktioner for rekonstitution og administration
English: Aseptic technique is to be used during preparation.
Danish: Der skal anvendes en aseptisk teknik under forberedelsen.
English: The total volume of infusion is determined by the patient's weight and should be administrated over a minimum of 50 minutes.
Danish: Det totale infusionsvolumen bestemmes ud fra patientens vægt, og bør administreres i løbet af mindst 50 minutter.
English: From a microbiological point of view, the medicinal product should be used immediately.
Danish: Fra et mikrobiologisk synspunkt bør lægemidlet anvendes umiddelbart.
English: Talk to your doctor before Lamzede is used.
Danish: Kontakt lægen, før Lamzede bruges.
English: An integrated database was created by pooling cumulative databases from all studies with velmanase alfa.
Danish: En integreret database blev dannet ved at samle kumulative databaser fra alle studier med velmanase alfa.
English: In case the patient's condition deteriorates during ERT, cessation of treatment should be considered.
Danish: I tilfælde af, at patientens tilstand forværres under ERT, skal det overvejes at seponere behandlingen.
English: The patient later experienced epileptic seizures that were considered not related.
Danish: Patienten oplevede senere epileptiske krampeanfald, der blev anset for ikke at være relateret.
English: - If you have an infusion-related reaction you may be given additional medicines to treat or help prevent future reactions.
Danish: - Hvis du har en infusionsrelateret reaktion, kan du få ekstra lægemidler til at behandle eller hjælpe med at forhindre fremtidige reaktioner.
English: All patients who experienced IRRs recovered.
Danish: Hos alle patienter, der oplevede IRR'er, forsvandt disse igen.
English: In total 15 patients (7 paediatrics and 8 adults) received active treatment and 10 patients received placebo (5 paediatrics and 5 adults).
Danish: I alt 15 patienter (7 pædiatriske og 8 voksne) fik aktiv behandling, og 10 patienter fik placebo (5 pædiatriske og 5 voksne).
English: As the accumulation of end organ damage progresses over time, it is more difficult for the treatment to reverse the damage or to show improvements.
Danish: Da tiltagende skader udvikler sig over tid på berørte organer, er det vanskeligere at revertere skaden eller at vise forbedringer med behandlingen.
English: Chemical and physical in-use stability has been demonstrated for 24 hours at 2°C - 8°C.
Danish: Der er blevet påvist kemisk og fysisk stabilitet ved brug i 24 timer ved 2 ºC - 8 ºC.
English: Animal studies do not indicate direct or indirect harmful effects with respect to pregnancy, embryonal/foetal development, parturition or postnatal development (see section 5.3).
Danish: Dyreforsøg indikerer hverken direkte eller indirekte skadelige virkninger hvad angår graviditet, embryonal/føtal udvikling, fødsel eller postnatal udvikling (se pkt.
English: Lamzede has no or negligible influence on the ability to drive and use machines.
Danish: Lamzede påvirker ikke eller kun i ubetydelig grad evnen til at føre motorkøretøj og betjene maskiner.
English: Minimum infusion time (min)
Danish: Minimal infusionstid (min)
English: These reactions were non-serious and mild to moderate in intensity.
Danish: Disse reaktioner var ikke alvorlige, og med en intensitet på let til moderat.
English: As expected for a protein of this size, the steady-state volume of distribution was low (0.27 L/kg), indicating distribution confined to plasma.
Danish: Som forventet for et protein af denne størrelse var fordelingsvoluminet ved steady-state lavt (0,27 l/kg), hvilket indikerer en fordeling, der er begrænset til plasma.
English: A pharmacodynamic effect with statistically significant decrease of serum oligosaccharides in comparison to placebo was demonstrated.
Danish: En farmakodynamisk virkning med en statistisk signifikant reduktion i oligosakkarider i serum, sammenlignet med placebo, blev vist.
English: Adverse reactions are classified by system organ class and preferred term according to the MedDRA frequency convention.
Danish: Bivirkninger er klassificeret i henhold til systemorganklasse og foretrukken terminologi i henhold til MedDRAs hyppighedskonvention.
English: Oligosaccharides 20.0% 100% 91.0%
Danish: Oligosakkarider 20,0% 100% 91,0%
English: There is no experience with patients younger than 6 years of age.
Danish: Der er ingen erfaring med patienter under 6 år.
English: Acute renal failure was of moderate severity leading to temporary discontinuation of the study treatment and fully resolved within 3 months.
Danish: Akut nyresvigt var af moderat sværhedsgrad, og førte til en midlertidig seponering af studiebehandlingen, og gik helt væk i løbet af 3 måneder.
English: Very common side effects (may affect more than 1 in 10 people) - diarrhoea - weight increase - fever/increased body temperature
Danish: Meget almindelige bivirkninger (kan forekomme hos flere end 1 ud af 10 personer) - diarré - vægtøgning - feber/øget legemstemperatur
English: The numerical improvement of most clinical endpoints over placebo (2 to 8 %) observed in the year of observation could be suggestive of the ability of velmanase alfa to slow down the existing disease progression.
Danish: Den numeriske forbedring af de fleste kliniske endepunkter over placebo (2 til 8 %), som blev observeret i løbet af observationsåret, kan være tegn på velmanase alfas evne til at nedsætte hastigheden af den eksisterende sygdomsprogression.
English: The infusion duration should be a minimum of 50 minutes.
Danish: Infusionen bør vare mindst 50 minutter.
English: Reflux gastritis
Danish: Refluks gastritis
English: Absolute change from baseline Mean
Danish: Absolut ændring fra baseline
English: Concomitant long-term treatment with high doses of ibuprofen was noted as a potentially causative contributor to the occurrence of the event.
Danish: Samtidig langvarig behandling med høje doser ibuprofen blev registreret som et muligt kausalt bidrag til, at hændelsen opstod.
English: Pack sizes of 1, 5 or 10 vials per carton.
Danish: Pakningsstørrelser på 1, 5 eller 10 hætteglas pr. karton.
English: Most side effects occur during the infusion or shortly after (“infusion-related reaction”, see section 2 Warnings and precautions).
Danish: De fleste bivirkninger opstår under infusionen eller kort efter (”infusionsrelateret reaktion”, se punkt 2 Advarsler og forsigtighedsregler).
English: rhLAMAN-10 study n=33
Danish: rhLAMAN-10 studie n = 33
English: Hypersensitivity reactions have been reported in patients in clinical studies.
Danish: Der er rapporteret overfølsomhedsreaktioner hos patienter i kliniske studier.
English: If you are treated with Lamzede, you may experience a side effect during or immediately following the drip (infusion) used to give the medicine (see section 4).
Danish: Hvis du bliver behandlet med Lamzede, kan du opleve en bivirkning under eller umiddelbart efter droppet (infusionen), der anvendes til at give lægemidlet (se punkt 4).
English: The effects of treatment with velmanase alfa should be periodically evaluated and discontinuation of treatment considered in cases where no clear benefits could be observed.
Danish: Virkninger af behandlingen med velmanase alfa skal evalueres periodisk, og seponering af behandlingen skal overvejes i tilfælde, hvor der ikke kan observeres nogen klare fordele.
English: Psychotic behaviour
Danish: Psykotisk adfærd
English: During the infusion with this higher dose, fever of mild intensity and short duration (5 hours) was observed in one patient.
Danish: Under infusionen med denne højere dosis, blev der observeret feber af en let intensitet og kort varighed (5 timer) hos en patient.
English: There were no apparent pharmacokinetic gender differences in patients with alpha-mannosidosis disease.
Danish: Der var ingen tilsyneladende farmakokinetiske kønsforskelle hos patienter med sygdommen alfa-mannosidose.
English: These medicines may include medicines used to treat allergies (antihistamines), medicines used to treat fever (antipyretics) and medicines to control inflammation (corticosteroids).
Danish: Disse lægemidler kan omfatte lægemidler, der anvendes til at behandle allergier (antihistaminer), lægemidler, der anvendes til at behandle feber (antipyretika) og lægemidler til at kontrollere betændelse (kortikosteroider).
English: Lamzede is administered through intravenous infusion.
Danish: Lamzede administreres via intravenøs infusion.
English: For patients weighing less than 18 kg, and receiving less than 9 mL reconstituted solution, the infusion rate should be calculated so that the infusion time is ≥50 minutes.
Danish: For patienter, der vejer under 18 kg, og som får under 9 ml rekonstitueret opløsning, skal infusionshastigheden beregnes, så infusionstiden er ≥ 50 minutter.
English: Infusion-related reaction
Danish: Infusionsrelateret reaktion
English: Functional Domain Response Combined
Danish: Funktionelt domænserespons Kombineret
English: Once it has been made up, the medicine will be given by infusion pump (drip) into a vein over a period of at least 50 minutes under your doctor's supervision.
Danish: Når det er blevet tilberedt, vil lægemidlet gives via en infusionspumpe (drop) i en vene over en periode på mindst 50 minutter under overvågning af din læge.
English: Non-clinical data reveal no special hazard for humans based on conventional studies of safety pharmacology, repeated dose toxicity, juvenile toxicity and toxicity to reproduction and development.
Danish: Prækliniske data viser ingen speciel risiko for mennesker vurderet ud fra konventionelle studier af sikkerhedsfarmakologi, toksicitet efter gentagne doser, juvenil toksicitet og reproduktions- og udviklingstoksicitet.
English: If the number of calculated vials includes a fraction, it should be rounded up to the next whole number.
Danish: Hvis det beregnede antal hætteglas ikke er et helt tal, skal det rundes op til det næste hele tal.
English: What you need to know before Lamzede is used Lamzede must not be used:
Danish: Det skal du vide, før Lamzede bruges Lamzede må ikke bruges:
English: Store and transport refrigerated (2°C - 8°C).
Danish: Opbevares i køleskab og transporteres nedkølet (2 °C - 8 °C).
English: Patients
Danish: Patienter
English: If not used immediately, the reconstituted solution should be stored in a refrigerator for no longer than 24 hours.
Danish: Hvis det ikke straks anvendes, må den rekonstituerede opløsning ikke opbevares i et køleskab i mere end 24 timer.
English: - Infusion-related reactions include dizziness, headache, nausea, low blood pressure, tiredness and fever.
Danish: - Infusionsrelaterede reaktioner omfatter svimmelhed, hovedpine, kvalme, lavt blodtryk, træthed og feber.
English: It should be considered by the treating physician that the administration of velmanase alfa does not affect the irreversible complications (i.e. skeletal deformities, disostosis multiplex, neurological manifestations and impaired cognitive function).
Danish: Den behandlende læge bør overveje, at administrationen af velmanase alfa ikke påvirker de irreversible komplikationer (dvs. skeletdeformiteter, disostosis multiplex, neurologiske manifestationer og nedsat kognitiv funktion).
English: In patients over 18 years old a stabilisation has been demonstrated.
Danish: En stabilisering er blevet vist hos patienter over 18 år.
English: Baseline actual value Mean (SD)
Danish: Baseline reel værdi Gennemsnit (SD)
English: As velmanase alfa aims at normalizing alpha-mannosidase in alpha-mannosidosis patients, Lamzede should be used during pregnancy only when strictly needed.
Danish: Da velmanase alfa har til formål at normalisere alfa-mannosidase hos patienter med alfa-mannosidose, må Lamzede kun anvendes under graviditeten, når det er strengt nødvendigt.
English: Treatment with placebo for 12 months (n=10)
Danish: Behandling med placebo i 12 måneder (n = 10)
English: A slower infusion rate may be prescribed when clinically appropriate according to the physician's judgment, for example at the beginning of the treatment or in case of previous infusion-related reactions (IRRs).
Danish: Der kan ordineres en langsommere infusionshastighed, når det er klinisk hensigtsmæssigt, i henhold til lægens skøn, for eksempel ved behandlingens start eller i tilfælde af tidligere infusionsrelaterede reaktioner (IRR'er).
English: All of these adverse reactions were non-serious.
Danish: Ingen af disse bivirkninger var alvorlige.
English: f) When the last syringe is empty, the dosage syringe is replaced with a 20 mL syringe filled with sodium chloride 9 mg/mL (0.9%) solution for injection.
Danish: f) Når den sidste sprøjte er tom, skal doseringssprøjten udskiftes med en 20 ml sprøjte fyldt med natriumchlorid 9 mg/ml (0,9 %) injektionsvæske, opløsning.
English: Treatment with velmanase alfa for 12 months (n=15)
Danish: Behandling med velmanase alfa i 12 måneder (n = 15)
English: The management of IRRs should be based on the severity of the reaction and includes slowing the infusion rate, treatment with medicinal products such as antihistamines, antipyretics and/or corticosteroids, and/or stopping and resuming treatment with increased infusion time.
Danish: Behandlingen af IRR'er bør baseres på sværhedsgraden af reaktionen og omfatter en reduktion af infusionshastigheden, behandling med lægemidler, såsom antihistaminer, antipyretika og/eller kortikosteroider, og/eller en afbrydelse og genstart af behandlingen med en øget infusionstid.
English: The patient received saline infusion in a hospital setting and was then discharged after 6-hour observation.
Danish: Patienten fik saltvandsinfusion på hospitalet, og blev udskrevet efter 6 timers observation.
English: The most common adverse reactions observed were weight increase (18%), IRRs (9%), diarrhoea (12%), headache (9%), arthralgia (9%), increased appetite (6%) and pain in extremity (6%).
Danish: De mest almindelige observerede bivirkninger var vægtøgning (18 %),IRR'er (9 %), diarré (12 %), hovedpine (9 %), artralgi (9 %), øget appetit (6 %) og smerter i ekstremiteter (6 %).
English: common (≥1/100 to <1/10), uncommon (≥1/1,000 to <1/100), rare (≥1/10,000 to <1/1,000), very rare
Danish: Hyppighederne 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), meget sjælden (<1/10.000) eller ikke kendt (kan ikke estimeres ud fra forhåndenværende data).
English: Antibodies may play a role in treatment-related reactions observed with the use of velmanase alfa.
Danish: Antistoffer kan spille en rolle i behandlingsrelaterede reaktioner, der observeres med anvendelsen af velmanase alfa.
English: Lamzede should be reconstituted and administrated by a healthcare professional.
Danish: Lamzede skal rekonstitueres og administreres af en læge eller sundhedspersonale.
English: Nevertheless, the absorption of any ingested milk-containing velmanase alfa in the breastfed child is considered to be minimal and no untoward effects are therefore anticipated.
Danish: Ikke desto mindre anses absorptionen af alt velmanase alfa indtaget via mælken hos det ammede barn for at være minimal, og der forventes derfor ingen uhensigtsmæssige virkninger.
English: The vials should reach ambient temperature (between 15°C and 25°C) prior to reconstitution.
Danish: Hætteglassene skal nå til stuetemperatur (fra 15 °C til 25 °C) før rekonstitution.
English: *Velmanase alfa is produced in mammalian Chinese Hamster Ovary (CHO) cells using recombinant DNA technology.
Danish: *Velmanase alfa er fremstillet i ovarieceller fra kinesiske hamstre (Chinese Hamster Ovary, CHO) ved hjælp af rekombinant dna-teknologi.
English: The infusion duration should be calculated individually considering a maximum infusion rate of 25 mL/hour to control the protein load.
Danish: Infusionens varighed skal beregnes individuelt ved at tage hensyn til en maksimal infusionshastighed på 25 ml/time for at kontrollere proteinbelastningen.
English: Dose (mL)
Danish: Dosis (ml)
English: Appropriate medical support should be readily available when velmanase alfa is administered.
Danish: Der skal være passende medicinsk støtte til umiddelbar rådighed, når velmanase alfa administreres.
English: Proteins larger than 50,000 Da, such as velmanase alfa, are not eliminated renally.
Danish: Proteiner over 50.000 Da, såsom velmanase alfa, elimineres ikke via nyrerne.
English: If the volume of the solution exceeds one syringe capacity, the required number of syringes should be prepared in order to replace the syringe quickly during the infusion.
Danish: Hvis opløsningens volumen overskrider kapaciteten af en sprøjte, skal det påkrævede antal sprøjter forberedes, så sprøjten hurtigt kan udskiftes under infusionen.
English: Due to the nature of the medicinal product, the reconstituted solution may occasionally contain some proteinaceous particles in form of thin white strands or translucent fibers which will be removed by the in-line filter during infusion (see point e).
Danish: På grund af arten af lægemidlet, kan den rekonstituerede opløsning til tider indeholde nogle proteinøse partikler i form af tynde hvide strenge eller gennemsigtige fibre, som vil blive fjernet af in-line-filtret under infusionen (se pkt.
English: A post-hoc multiparametric responders analysis supports the benefit of longer treatment with velmanase alfa in 87.9% of responders in at least 2 domains at last observation (table 4).
Danish: En post-hoc-, multiparametrisk analyse af personer, der responderede, understøtter fordelen ved længere behandling med velmanase alfa hos 87,9 % af personerne, der responderede, i mindst 2 domæner ved den sidste observation (tabel 4).
English: This medicine is only to be used under the supervision of a doctor experienced in the treatment of alpha-mannosidosis or other similar diseases and should only be given by a healthcare professional.
Danish: Dette lægemiddel må kun bruges under overvågning af en læge med erfaring i behandling af alfa-mannosidose eller andre tilsvarende sygdomme, og det må kun gives af læger eller sundhedspersonale.
English: For the calculation of the infusion rate and the infusion time based on body weight see the table in section 6.6.
Danish: For beregningen af infusionshastigheden og infusionstiden baseret på legemsvægten, se tabellen i pkt.
English: - If the infusion-related reaction is severe, your doctor will stop the infusion immediately and start giving you appropriate medical treatment.
Danish: - Hvis den infusionsrelaterede reaktion er svær, vil lægen straks stoppe infusionen, og begynde at give dig passende medicinsk behandling.
English: Data suggest that the beneficial effects of the treatment with velmanase alfa diminish with the increase of disease burden and disease-related respiratory infections.
Danish: Data tyder på, at de gavnlige virkninger af behandlingen med velmanase alfa falder med en øget sygdomsbyrde og sygdomsrelaterede infektioner i luftvejene.
English: The solution should be clear and not used if opaque particles are observed or if the solution is discoloured.
Danish: Opløsningen skal være klar og må ikke bruges, hvis der observeres uigennemsigtige partikler, eller hvis opløsningen er misfarvet.
English: In order to obtain long-term data on effectiveness and safety of treatment with Lamzede and to characterize the entire alpha-mannosidosis population, 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 registry of patients with alpha-mannosidosis.
Danish: Med henblik på at indhente langtidsdata om virkning og sikkerhed ved behandling med Lamzede, og for at karakterisere hele alfa-mannosidose-populationen, herunder variabilitet af klinisk manifestation, progression og naturhistorie, skal indehaveren af markedsføringstilladelsen indsende resultaterne af et studie baseret på tilstrækkelige kildedata fra et patientregister med alfa-mannosidose.
English: In the clinical studies, one patient experienced acute renal failure considered possibly related to the study treatment.
Danish: I de kliniske studier oplevede en patient akut nyresvigt, der blev anset for at være muligvis relateret til studiebehandlingen.
English: If severe allergic or anaphylactic-type reactions occur, immediate discontinuation of velmanase alfa is recommended and current medical standards for emergency treatment are to be followed.
Danish: Hvis der opstår alvorlige allergiske reaktioner eller reaktioner af anafylakse-typen, anbefales en umiddelbar seponering af velmanase alfa, og gældende medicinske standarder for nødbehandling skal følges.
English: - If the infusion-related reactions are severe and/or there is a loss of effect from this medicine, your doctor will perform a blood test to check for antibodies that might affect the outcome of your treatment.
Danish: - Hvis de infusionsrelaterede reaktioner er svære, og/eller der er en manglende virkning af lægemidlet, vil lægen tage en blodprøve for at kontrollere for antistoffer, som kan påvirke dit behandlingsudfald.
English: To further evaluate the relationship, in instances of development of severe IRRs or lack or loss of treatment effect, patients should be tested for the presence of anti-velmanase alfa antibodies.
Danish: For at evaluere forholdet yderligere, i tilfælde af, at der udvikles svære IRR'er, eller hvis der er en manglende eller udebleven virkning af behandlingen, skal patienterne testes for tilstedeværelse af anti-velmanase alfa-antistoffer.
English: 6MWT (metres)
Danish: 6MWT (meter)