FrancophonIA/ELRC3.0 huggingface dataset:
English: Patients were not routinely pre-medicated prior to infusion of velmanase alfa during clinical studies.
Danish: Patienterne blev ikke rutinemæssigt forbehandlet med lægemidler før infusionen af velmanase alfa i kliniske studier.
English: Last observation % change from baseline (SD)
Danish: Sidste observation % ændring fra baseline
English: The amino acid sequence of the monomeric protein is identical to the naturally occurring human enzyme, alpha-mannosidase.
Danish: Aminosyresekvensen for det monomere protein er identisk med det naturligt forekommende humane enzym, alfa-mannosidase.
English: The recommended dose of Lamzede is 1 mg/kg of body weight given once every week.
Danish: Den anbefalede dosis af Lamzede er 1 mg/kg legemsvægt, givet én gang ugentligt.
English: A total of 25 patients were enrolled, including 12 paediatric subjects (age range: 6 to 17 years; mean: 10.9 years) and 13 adult subjects (age range: 18 to 35 years; mean: 24.6).
Danish: I alt 25 patienter indgik, herunder 12 pædiatriske forsøgspersoner (aldersinterval: 6 til 17 år, gennemsnit: 10,9 år) og 13 voksne personer (aldersinterval: 18 til 35 år, gennemsnit: 24,6 år).
English: Confusional state
Danish: Forvirret tilstand
English: Thereafter each vial should be tilted and rolled gently for 15-20 seconds to enhance the dissolution process.
Danish: Derefter skal hvert hætteglas holdes på skrå, og indholdet skal rulles forsigtigt rundt i 15-20 sekunder, for at hjælpe med at opløse pulveret.
English: Administration of Lamzede should be carried out by a healthcare professional with the ability to manage ERT and medical emergencies.
Danish: Administrationen af Lamzede skal udføres af læger og sundhedspersonale med færdigheder i behandling med ERT og medicinske nødstilfælde.
English: As with other enzyme replacement therapies, velmanase alfa does not cross the blood-brain-barrier.
Danish: Som for andre enzymerstatningsbehandlinger, krydser velmanase alfa ikke blod-hjerne-barrieren.
English: There are no data from the use of velmanase alfa in pregnant women.
Danish: Der er ingen data fra anvendelse af velmanase alfa til gravide kvinder.
English: The results (serum oligosaccharide concentration, 3MSCT, 6MWT and FVC%) are presented in table 2.
Danish: Resultaterne (oligosakkerid-koncentration i serum, 3MSCT, 6MWT og FVC%) vises i tabel 2.
English: Overall response
Danish: Samlet respons
English: The maximum dose of velmanase alfa in clinical studies was a single administration of 100 units/kg (approximately corresponding to 3.2 mg/kg).
Danish: Den maksimale dosis velmanase alfa i kliniske studier var en enkelt administration på 100 enheder/kg (der ca. svarer til 3,2 mg/kg).
English: No clear correlation was found between antibody titres (velmanase alfa IgG antibody level) and reduction in efficacy or occurrence of anaphylaxis or other hypersensitivity reactions.
Danish: Der blev ikke fundet nogen klar korrelation mellem antistoftitre (velmanase alfa IgG-antistofniveau) og reduktion i virkning eller forekomst af anafylakse eller andre overfølsomhedsreaktioner.
English: How Lamzede is used
Danish: Sådan bruges Lamzede
English: Patient's weight (44 kg) × dose (1 mg/kg) = Patient dose (44 mg) 44 mg divided by 10 mg/vial = 4.4 vials, therefore, 5 vials should be reconstituted.
Danish: Patientens vægt (44 kg) × dosis (1 mg/kg) = Patientens dosis (44 mg) 44 mg delt med 10 mg/hætteglas = 4,4 hætteglas, derfor skal der rekonstitueres 5 hætteglas.
English: Lamzede is supplied in a vial as a powder for infusion which will be made up with water for injections before being given.
Danish: Lamzede leveres i et hætteglas som et pulver til infusionsvæske, som vil blive tilberedt med vand til injektionsvæsker, før det gives.
English: Package leaflet: Information for the patient Lamzede 10 mg powder for solution for infusion velmanase alfa This medicine is subject to additional monitoring.
Danish: Indlægsseddel: Information til patienten Lamzede 10 mg pulver til infusionsvæske, opløsning velmanase alfa Dette lægemiddel er underlagt supplerende overvågning.
English: Each carton contains 1, 5 or 10 vials.
Danish: Hver æske indeholder 1, 5 eller 10 hætteglas.
English: Lamzede requires reconstitution and is intended for intravenous infusion only.
Danish: Det er nødvendigt at rekonstituere Lamzede, og det er kun beregnet til intravenøs infusion.
English: <18 years(2) ≥18 years(2)
Danish: ≥18 år(2)
English: Do not use this medicine after the expiry date which is stated on the label and the carton after ‘EXP'.
Danish: Brug ikke lægemidlet efter den udløbsdato, der står på etiketten og æsken efter EXP. Udløbsdatoen er den sidste dag i den nævnte måned.
English: IRRs (including hypersensitivity, nausea, vomiting, pyrexia, chills, feeling hot, malaise, urticaria, anaphylactoid reaction and hyperhidrosis) were reported in 9% of the patients (3 out of 33 patients) in clinical studies.
Danish: IRR'er (herunder overfølsomhed, kvalme, opkastning, pyreksi, kulderystelser, varmefølelse, utilpashed, urticaria, anafylaktoid reaktion og hyperhidrose) blev rapporteret hos 9 % af patienterne (3 ud af 33 patienter) i kliniske studier.
English: Initial insomnia
Danish: Indledningsvis insomni
English: Animal studies do not show evidence of impaired fertility.
Danish: Dyreforsøg viser ikke evidens for nedsat fertilitet.
English: The safety profile of velmanase alfa in clinical studies involving children and adolescents was similar to that observed in adult patients.
Danish: Sikkerhedsprofilen af velmanase alfa i kliniske studier, der omfattede børn og unge, svarede til den observerede sikkerhedsprofil hos voksne patienter.
English: Combined
Danish: Kombineret
English: Paediatric Study rhLAMAN-08.
Danish: Pædiatrisk studie rhLAMAN-08.
English: IRRs include hypersensitivity in 3 patients and anaphylactoid reaction in 1 patient.
Danish: IRR'er omfatter overfølsomhed hos 3 patienter og anafylaktoid reaktion hos 1 patient.
English: The recommended dose regimen is 1 mg/kg of body weight administered once every week by intravenous infusion at a controlled speed.
Danish: Det anbefalede dosisprogram er 1 mg/kg legemsvægt, administreret én gang ugentligt ved en intravenøs infusion med en kontrolleret hastighed.
English: Velmanase alfa exhibited a linear (i.e. first-order) pharmacokinetic profile, and Cmax and AUC increased proportionally to the dose with doses ranging from 0.8 to 3.2 mg/kg (corresponding to 25 and 100 units/kg).
Danish: Velmanase alfa udviste en lineær (dvs. førsteordens) farmakokinetisk profil, og Cmax og AUC steg proportionalt med dosis i dosisintervallet fra 0,8 til 3,2 mg/kg (svarende til 25 og 100 enheder/kg).
English: A total of 2 serious adverse reactions (loss of consciousness in 1 patient and acute renal failure in 1 patient) were observed.
Danish: Der blev observeret i alt 2 alvorlige bivirkninger (bevidstløshed hos 1 patient og akut nyresvigt hos 1 patient).
English: Store and transport refrigerated.
Danish: Opbevares i køleskab og transporteres nedkølet.
English: If not used immediately, the reconstituted solution may be stored up to 24 hours at 2°C to 8°C.
Danish: Hvis det ikke bruges straks, kan den rekonstituerede opløsning opbevares i op til 24 timer ved 2 °C - 8 °C.
English: After the end of the infusion, velmanase alfa plasma concentrations fell in a biphasic fashion with a mean terminal elimination half-life of about 30 hours.
Danish: Efter endt infusion faldt plasmakoncentrationerne af velmanase alfa bifasisk med en gennemsnitlig terminal eliminationshalveringstid på omkring 30 timer.
English: Overall effects of treatment were evaluated in the domains of pharmacodynamics (reduction of serum oligosaccharides), functional (three-minute stair climbing test (3MSCT), six-minute walking test (6MWT), and forced vital capacity (FVC) % predicted) and quality of life (childhood health assessment questionnaire (CHAQ) disability index (DI) and CHAQ VAS pain (visual analogue scale)).
Danish: De samlede virkninger af behandlingen blev evalueret i domænerne for farmakodynamik (reduktion af oligosakkarider i serum), funktionalitet (tre minutters trappetest (three-minute stair climbing test, 3MSCT), 6 minutters gangtest (six-minute walking test, 6MWT) og forceret vitalkapacitet (FVC) % forventet), og livskvalitet (childhood health assessment questionnaire (CHAQ) disability index (DI) og CHAQ-VAS pain (visuel analog skala)).
English: The typical manifestations of the disease include distinctive facial features, mental retardation, difficulty in controlling movements, difficulties in hearing and speaking, frequent infections, skeletal problems, muscle pain and weakness.
Danish: De typiske tegn på denne sygdom omfatter karakteristiske ansigtstræk, mental retardering, problemer med at kontrollere bevægelser, høre- og talebesvær, hyppige infektioner, skeletproblemer, muskelsmerter og svaghed.
English: Based on this criteria mild to moderate patients, presenting heterogeneous severity with ability to perform endurance tests, large variability of clinical manifestations and age of onset were enrolled.
Danish: Baseret på dette kriterium indgik patienter med let til moderat sygdom, der præsenterede heterogen sværhedsgrad, men var i stand til at udføre udholdenhedstests, og med stor variabilitet af kliniske manifestationer og alder for sygdomsdebut.
English: This medicine must not be used if the reconstituted solution contains opaque particles or is discoloured.
Danish: Dette lægemiddel må ikke bruges, hvis den rekonstituerede opløsning indeholder uigennemsigtige partikler eller er misfarvet.
English: Chiesi Bulgaria EOOD
Danish: Chiesi Bulgaria EOOD Teл.
English: If you experience an infusion-related reaction, you must tell your doctor immediately.
Danish: Hvis du oplever en infusionsrelateret reaktion, skal du straks fortælle det til lægen.
English: The IRRs observed in clinical studies of velmanase alfa were characterised by a rapid onset of symptoms and were of mild to moderate severity.
Danish: De observerede IRR'er i kliniske studier med velmanase alfa blev karakteriseret af hurtigt opståede symptomer med en sværhedsgrad på let til moderat.
English: A volume of 10 mL sodium chloride solution should be administered through the infusion system to infuse the remaining fraction of Lamzede in the line to the patient.
Danish: Et volumen på 10 ml natriumchloridopløsning skal administreres gennem infusionssystemet for at infundere den resterende fraktion af Lamzede i patientens slange.
English: At steady-state after weekly infusion administration of 1 mg/kg of velmanase alfa, the mean maximum plasma concentration was about 8 µg/mL and was reached at 1.8 hours after the start of administration corresponding to the mean infusion duration time.
Danish: Ved steady-state efter administration af ugentlige infusioner af velmanase alfa på 1 mg/kg, var den gennemsnitlige maksimale plasmakoncentration omkring 8 µg/ml, og den blev nået 1,8 timer efter starten af administrationen, hvilket svarer til den gennemsnitlige infusionstid.
English: The treatment should be supervised by a physician experienced in the management of patients with alpha-mannosidosis or in the administration of other enzyme replacement therapies (ERT) for lysosomal storage disorder.
Danish: Behandlingen skal overvåges af en læge med erfaring i at behandle patienter med alfa-mannosidose eller i administration af andre enzymerstatningsbehandlinger (enzyme replacement therapies, ERT) for lysosomal aflejringssygdom.
English: All but one patient were naïve to the treatment with velmanase alfa.
Danish: Alle patienter, undtagen én, var behandlingsnaive med hensyn til velmanase alfa.
English: Alpha-mannosidosis disease is a rare genetic disorder caused by a lack of an enzyme named alpha-mannosidase, which is needed to break down certain sugar compounds (called ‘mannose-rich oligosaccharides') in the body.
Danish: Alfa-mannosidose er en sjælden genetisk sygdom, der forårsages af mangel på et enzym kaldet alfa-mannosidase, som er nødvendigt for at nedbryde visse sukkerstoffer (kaldet 'oligosakkarider med et højt mannoseindhold') i kroppen.
English: Use of velmanase alfa in the age group 6 to 17 years is supported by evidence from clinical studies in paediatric (19 out of 33 patients) and adult patients.
Danish: Brugen af velmanase alfa i aldersgruppen fra 6 til 17 år understøttes af evidens fra kliniske studier med pædiatriske (19 ud af 33 patienter) og voksne patienter.
English: The patient should be observed for IRRs for at least one hour after the infusion according to clinical conditions and the physician's judgment.
Danish: Patienten bør observeres for IRR'er i mindst en time efter infusionen, i henhold til kliniske betingelser og lægens skøn.
English: Table 1: Adverse reactions reported in clinical studies in patients with alpha-mannosidosis treated with velmanase alfa
Danish: Tabel 1: Bivirkninger rapporteret i kliniske studier hos patienter med alfa-mannosidose, behandlet med velmanase alfa
English: It is unknown whether velmanase alfa or its metabolites are excreted in human milk.
Danish: Det er ukendt, om velmanase alfa eller dets metabolitter udskilles i human mælk.
English: In case symptoms such as angioedema (tongue or throat swelling), upper airway obstruction or hypotension occur during or immediately after infusion, anaphylaxis or an anaphylactoid reaction should be suspected.
Danish: I tilfælde af, at symptomer som angioødem (hævelse af tunge eller hals), obstruktion af de øvre luftveje eller hypotension opstår under eller umiddelbart efter infusionen, skal anafylakse eller en anafylaktoid reaktion formodes.
English: The recommended dose of 1 mg/kg is determined using the following calculation: Patient's weight (kg) × dose (mg/kg) = Patient dose (in mg) Patient dose (in mg) divided by 10 mg/vial (content of one vial) = number of vials to reconstitute.
Danish: Den anbefalede dosis på 1 mg/kg bestemmes ud fra den følgende beregning: Patientens vægt (kg) × dosis (mg/kg) = Patientens dosis (i mg) Patientens dosis (i mg) delt med 10 mg/hætteglas (indholdet af et hætteglas) = antal hætteglas, der skal rekonstitueres.
English: The patient should be kept under observation for IRRs for one hour or longer after the infusion, according to the treating physician's judgement.
Danish: Patienten skal holdes under observation for IRR'er i en time eller længere efter infusionen, i henhold til den behandlende læges skøn.
English: - if you are allergic to velmanase alfa or any of the other ingredients of this medicine (listed in section 6).
Danish: - hvis du er allergisk over for velmanase alfa eller et af de øvrige indholdstoffer i Lamzede (angivet i punkt 6).
English: (1) For overall: adjusted mean change and adjusted mean difference estimated by ANCOVA model are presented (2) By age: unadjusted mean and SD are presented.
Danish: ≥18 år(2) (1) For samlet: justeret gennemsnitlig ændring og justeret gennemsnitlig forskel, estimeret ved ANCOVA-modellen, vises (2) Efter alder: ikke justeret gennemsnit og SD vises.
English: Lamzede contains sodium
Danish: Lamzede indeholder natrium
English: Velmanase alfa, the active substance of Lamzede, is a recombinant form of human alpha-mannosidase.
Danish: Velmanase alfa, det aktive stof i Lamzede, er en rekombinant form af humant alfa-mannosidase.
English: Pharmacodynamic
Danish: Farmakodynamik
English: The vial should not be inverted, swirled, or shaken.
Danish: Hætteglasset må ikke vendes på hovedet, slynges eller rystes.
English: The clearance of velmanase alfa from plasma (mean 6.7 mL/h/kg) is consistent with a rapid cellular uptake of velmanase alfa via mannose receptors.
Danish: Clearance af velmanase alfa fra plasma (gennemsnit 6,7 ml/t/kg) stemmer overens med en hurtig celleoptagelse af velmanase alfa via mannosereceptorer.
English: 10 mL vial (Type I glass) with a bromobutyl rubber stopper, an aluminium seal and a polypropylene flip off cap.
Danish: 10 ml hætteglas (type I-glas) med en brombutyl-gummiprop, en aluminiumsforsegling og et flip off-låg af polypropylen.
English: There is no experience with overdose of velmanase alfa.
Danish: Der er ingen erfaring med en overdosering af velmanase alfa.
English: p-value [95% CI]
Danish: p-værdi [95 % CI]
English: The maximum infusion rate is 25 mL/hour.
Danish: Den maksimale infusionshastighed er 25 ml/time.
English: c) An immediate visual inspection of the solution for particulate matter and discoloration should be performed after reconstitution.
Danish: c) Der skal straks efter rekonstitution udføres en visuel inspektion af opløsningen for partikler og misfarvning.
English: Lamzede is a white to off-white powder for solution for infusion, supplied in a glass vial.
Danish: Lamzede er et hvidt til off-white pulver til infusionsvæske, opløsning, der leveres i et hætteglas.
English: In the clinical studies at any time under treatment, 8 patients out of 33 (24%) developed IgG-class antibodies to velmanase alfa.
Danish: I de kliniske studier, uanset tidspunktet under behandlingen, udviklede 8 patienter ud af 33 (24 %) antistoffer af IgG-klassen over for velmanase alfa.
English: Velmanase alfa is intended to supplement or replace natural alpha-mannosidase, an enzyme that catalyses the sequential degradation of hybrid and complex high-mannose oligosaccharides in the lysosome, reducing the amount of accumulated mannose-rich oligosaccharides.
Danish: Velmanase alfa er beregnet som et tilskud eller en erstatning for naturligt alfa-mannosidase, et enzym, der katalyserer den sekventielle nedbrydning af hybride og komplekse oligosakkerider med et højt mannoseindhold i lysosomet, hvorved mængden af akkumulerede oligosakkarider med et højt mannoseindhold, reduceres.
English: The adverse reactions reflecting exposure of 33 patients treated with velmanase alfa in clinical studies are listed in the table 1 below.
Danish: Bivirkninger, der afspejlede en eksponering af 33 patienter i behandling med velmanase alfa i kliniske studier, er opstillet i den nedenstående tabel 1.
English: Velmanase alfa is designed to replace the missing enzyme in patients with alpha-mannosidosis disease.
Danish: Velmanase alfa er beregnet til at erstatte det manglende enzym hos patienter med alfa-mannosidose.
English: Table 3: Change of clinical endpoints from baseline to the last observation in rhLAMAN-10 study (source data: rhLAMAN-10)
Danish: Tabel 3: Ændring af kliniske endepunkter fra baseline til den sidste observation i rhLAMAN-10-studiet (kildedata: rhLAMAN-10)
English: A total of 33 patients (20 males and 13 females, ranging in age from 6 to 35 years) were exposed to velmanase alfa in five clinical studies.
Danish: I alt 33 patienter, (20 mænd og 13 kvinder, i alderen fra 6 til 35 år) blev eksponeret for velmanase alfa i fem kliniske studier.
English: FVC (% of predicted) Overall
Danish: FVC (% af forventet)
English: November 2020
Danish: Endelig studierapport: November 2020
English: b) The powder should be reconstituted in the vial by a slow drop-wise addition of the water for injections down the inside of the vial and not directly onto the lyophilised powder.
Danish: b) Pulveret skal rekonstitueres i hætteglasset ved langsomt at tilsætte dråber af vandet til injektionsvæsker langs indersiden af hætteglasset, og ikke direkte på det lyofiliserede pulver.
English: Enzyme replacement therapy for the treatment of non-neurological manifestations in patients with mild to moderate alpha-mannosidosis.
Danish: Enzymerstatningsbehandling til behandling af ikke-neurologiske manifestationer hos patienter med let til moderat alfa-mannosidose.
English: Administration of velmanase alfa may result in an IRR, including anaphylactoid reaction (see section 4.8).
Danish: Administration af velmanase alfa kan føre til en IRR, herunder anafylaktoid reaktion (se pkt.
English: The European Medicines Agency has deferred the obligation to submit the results of studies with Lamzede in one or more subsets of the paediatric population in the treatment of alpha-mannosidosis disease (see section 4.2 for information on paediatric use).
Danish: Det Europæiske Lægemiddelagentur har dispenseret fra kravet om at fremlægge resultaterne af studier med Lamzede i en eller flere undergrupper af den pædiatriske population ved behandling af sygdommen alfa-mannosidose (se pkt.
English: It is used to treat patients with mild to moderate alpha-mannosidosis disease.
Danish: Det anvendes til at behandle patienter med let til moderat alfa-mannosidose.
English: - The other ingredients are: disodium phosphate dihydrate, sodium dihydrogen phosphate dihydrate, mannitol and glycine.
Danish: - Øvrige indholdsstoffer: dinatriumphosphatdihydrat, natriumdihydrogenphosphatdihydrat, mannitol og glycin.
English: There are no clinical data on the effects of velmanase alfa on fertility.
Danish: Der er ingen kliniske data om velmanase alfas virkning på fertilitet.
English: Maximum infusion rate (mL/h)
Danish: Maksimal infusionshastighed (ml/t)
English: In the phase 3 pivotal multi-centre, double-blind, randomised, placebo-controlled, parallel group study rhLAMAN-05, the efficacy and safety of repeated administrations of velmanase alfa over 52 weeks at a dose of 1 mg/kg given weekly as intravenous infusion were investigated.
Danish: I det fase III pivotale, dobbeltblindede, randomiserede, placebokontrollerede multicenterstudie med parallelle grupper, rhLAMAN-05, blev virkningen og sikkerheden ved gentagne administrationer af velmanase alfa undersøgt i løbet af 52 uger ved en dosis på 1 mg/kg, der blev givet ugentligt som en intravenøs infusion.
English: Contents of the pack and other information What Lamzede contains
Danish: Pakningsstørrelser og yderligere oplysninger Lamzede indeholder:
English: Serum oligosaccharide concentration (µmol/L)
Danish: Oligosakkaridkoncentration i serum (µmol/l)
English: Hyperhidrosis(1)
Danish: Hyperhidrose(1)
English: No dose adjustment is necessary for patients with renal or hepatic impairment.
Danish: En dosisjustering er ikke nødvendig for patienter med nedsat nyre- eller leverfunktion.
English: Each vial is reconstituted by slowly injecting 5 mL of water for injections to the inside of the wall of each vial.
Danish: Hvert hætteglas rekonstitueres ved langsomt at injicere 5 ml vand til injektionsvæsker langs indersiden af hvert hætteglas.
English: Velmanase alfa is a protein and is predicted to be metabolically degraded into amino acids.
Danish: Velmanase alfa er et protein, og det forventes at blive metaboliseret til aminosyrer.
English: Disodium phosphate dihydrate Sodium dihydrogen phosphate dihydrate Mannitol
Danish: Dinatriumphosphatdihydrat Natriumdihydrogenphosphatdihydrat Mannitol
English: Adjusted mean difference
Danish: Justeret gennemsnitlig forskel
English: Lamzede contains the active substance velmanase alfa which belongs to a group of medicines known as enzyme replacement therapies.
Danish: Lamzede indeholder det aktive stof velmanase alfa, som tilhører en gruppe lægemidler, der kaldes enzymerstatningsbehandlinger.
English: Overall, 58% of patients (19 out of 33) with alpha-mannosidosis receiving velmanase alfa in clinical studies were aged 6 to 17 years at the start of the study.
Danish: I alt var 58 % af patienterne (19 ud af 33) med alfa-mannosidose, der fik velmanase alfa i kliniske studier, fra 6 til 17 år ved studiestart.
English: This medicinal product contains less than 1 mmol sodium (23 mg) per dose, that is to say essentially ‘sodium-free'.
Danish: Dette lægemiddel indeholder mindre end 1 mmol (23 mg) natrium pr. dosis, dvs. den er i det væsentlige natrium-fri.
English: Other medicines and Lamzede
Danish: Brug af anden medicin sammen med Lamzede
English: The long-term efficacy and safety of velmanase alfa was investigated in the uncontrolled, open label, phase 3 clinical study rhLAMAN-10 in 33 subjects (19 paediatrics and 14 adults, from 6 to 35 years at treatment initiation) who previously participated in velmanase alfa studies.
Danish: Den langvarige virkning og sikkerhed af velmanase alfa blev undersøgt i det ikke kontrollerede, åbne, fase III kliniske studie rhLAMAN-10 med 33 personer (19 pædiatriske og 14 voksne, fra 6 til 35 år ved påbegyndelse af behandlingen), som tidligere deltog i studier med velmanase alfa.