natgillin/translations-raw huggingface dataset:
VPRIV kan gives til ældre (personer over 65 år) med samme dosis og hyppighed som hos andre voksne.
VPRIV may be given to the elderly (aged over 65 years) at the same dose and frequency as in adults.
Uddannelsesmaterialet til infusion af VPRIV i hjemmet er beregnet som en vejledning i håndteringen af risikoen for infusionsrelaterede reaktioner, herunder overfølsomhedsreaktioner af allergitypen, i hjemlige omgivelser.
The educational materials for the use of VPRIV in home infusion are aimed at providing guidance on how to manage risk of infusion-related reactions including allergic-type hypersensitivity reactions in a home setting.
Behandlingen skal iværksættes med forsigtighed hos patienter, der har udvist symptomer på overfølsomhed over for velaglucerase alfa eller anden enzymerstatningsterapi.
Treatment should be approached with caution in patients who have exhibited symptoms of hypersensitivity to velaglucerase alfa or other enzyme replacement therapy.
Efter afslutningen af infusionen faldt serumkoncentrationen af velaglucerase alfa hurtigt på monofasisk eller bifasisk vis med en gennemsnitlig t1/2 fra 5 til 12 minutter for 15, 30, 45 og 60 enheder/kg-doserne.
After the end of the infusion, velaglucerase alfa serum concentrations fell rapidly in a monophasic or biphasic fashion with a mean t1/2 ranging from 5 to 12 minutes at doses of 15, 30, 45, and 60 Units/kg.
I dette ene tilfælde vurderedes antistofferne at være neutraliserende i en in vitroanalyse.
In this one event, the antibodies were determined to be neutralising in an in vitro assay.
I alt 95 patienter (73 voksne og 22 børn/unge), som deltog i studierne 032, 034 og 039, fortsatte i det åbne forlængelsesstudie og blev behandlet med velaglucerase alfa.
A total of 95 patients (73 adult and 22 paediatric) who participated in studies 032, 034, and 039 enrolled in the open label extension study and were treated with velaglucerase alfa.
• Vejledning til læger og sundhedspersonale, som behandler patienter med Gauchers sygdom,
• Guide for Healthcare Professionals Treating Patients with Gaucher Disease;
I denne lysosomale ophobningssygdom (lysosomal storage disease) er de kliniske karakteristika en afspejling af fordelingen af Gaucher-celler i leveren, milten, knoglemarven, skelettet og lungerne.
In this lysosomal storage disorder (LSD), clinical features are reflective of the distribution of Gaucher cells in the liver, spleen, bone marrow, skeleton, and lungs.
I studie 025EXT fik 7 ud af 10 patienter behandling i hjemmet mindst én gang i løbet af 60 måneders behandling.
In Study 025EXT, 7 of 10 patients received home therapy at least once during 60 months of treatment.
Behandlingsrespons
Response to treatment
Patienterne skulle lide af anæmi relateret til Gauchers sygdom og enten trombocytopeni eller organomegali.
Patients were required to have Gaucher disease-related anaemia and either thrombocytopenia or organomegaly.
I studie 034 fik 25 ud af 40 patienter behandling i hjemmet mindst én gang i løbet af det 12 måneder lange studie.
In Study 034, 25 of 40 patients received home therapy at least once during the 12-month study.
Ikke anvendte lægemidler samt affald heraf bør destrueres i henhold til lokale retningslinjer.
Any unused medicine or waste material should be disposed of in accordance with local requirements.
Yderligere information om VPRIV findes på Det Europæiske Lægemiddelagenturs hjemmeside http://www.ema.europa.eu/.
Detailed information on this medicinal product is available on the website of the European Medicines Agency http://www.ema.europa.eu
Der blev påvist fortsat klinisk aktivitet under behandlingen, observeret som forbedringer i hæmoglobinkoncentration og trombocyttal samt reduceret lever- og miltvolumen.
Sustained clinical activity continued to be demonstrated during treatment as observed by improvements in haemoglobin concentrations and platelet counts and reduced liver and spleen volumes.
Hvert hætteglas indeholder nu en volumen til udtagelse på 4,0 ml (100 enheder/ml).
Each vial will contain an extractable volume of 4.0 ml (100 Units/ml).
Vejledningen til læger og sundhedspersonale, der behandler patienter med Gauchers sygdom, bør indeholde følgende nøgleelementer:
The Guide for Healthcare Professionals Treating Patients with Gaucher Disease should contain the following key elements:
En infusionsrelateret reaktion defineres som en bivirkning, der opstår inden for 24 timer efter, infusionen med velaglucerase alfa er påbegyndt.
An infusion-related reaction is defined as any adverse drug reaction occurring within 24 hours after the initiation of velaglucerase alfa infusion.
Hvis du er i behandling for Gauchers sygdom med en anden enzymerstatningsterapi, og din læge ønsker at skifte dig over til VPRIV, kan du indledningsvis få VPRIV i samme dosis og med samme hyppighed, som gjaldt for den anden enzymerstatningsterapi.
If you are currently being treated for Gaucher disease with another ERT and your doctor wants to change you to VPRIV, you can initially receive VPRIV at the same dose and frequency you had been receiving the other ERT.
Velaglucerase alfa havde en nogenlunde lineær (dvs. førsteordens-) farmakokinetisk profil, og Cmax og AUC steg nogenlunde proportionalt til dosis i dosisområdet 15 til 60 enheder/kg.
Velaglucerase alfa exhibited an approximately linear (i.e. first-order) pharmacokinetic profile, and Cmax and AUC increased approximately proportional to the dose over the dose range 15 to 60 Units/kg.
En svær allergisk reaktion med vejrtrækningsbesvær, hævelse i ansigt, læber, tunge eller hals er set.
A severe allergic reaction, with difficulty breathing, swelling of the face, lips, tongue or throat occured.
Bivirkninger, der blev indberettet hos patienter med Gauchers sygdom, type 1, er opstillet i tabel 1.
Adverse reactions reported in patients with type 1 Gaucher disease are listed in Table 1.
Overfølsomhedsreaktioner (omfatter allergisk dermatitis og anafylaktiske/anafylaktoide reaktioner)
Immune system disorders hypersensitivity reactions (includes dermatitis allergic and anaphylactic/anaphylactoid reactions)
Hos den pædiatriske population (i alderen 4-17 år) steg den gennemsnitlige Z-score for højde i løbet af 60 måneders behandling i den samlede behandlingsnaive population, hvilket tyder på en positiv behandlingseffekt med velaglucerase alfa på lineær vækst.
In the paediatric population (ages 4 to 17 years studied), increases in the mean height Z-score were seen through 60 months of treatment in the overall treatment-naïve population, suggesting a beneficial treatment effect with velaglucerase alfa on linear growth.
Pakningsstørrelser: 1, 5 og 25 hætteglas.
Pack sizes of 1, 5 and 25 vials.
VPRIV påvirker ikke eller i ubetydelig grad evnen til at føre motorkøretøj eller betjene maskiner.
VPRIV has no or negligible influence on the ability to drive or use machines.
Infusionsrelateret reaktion, asteni/træthed, pyreksi/øget kropstemperatur
Infusion-related reaction, asthenia/fatigue, pyrexia/body temperature increased
Den gennemsnitlige absolutte stigning fra baseline i hæmoglobinkoncentrationer var 1,624 g/dl (± 0,223 SE) efter 9 måneders behandling med velaglucerase alfa.
The mean absolute increase from baseline in haemoglobin concentrations was 1.624 g/dl (±0.223 SE) following 9 months of treatment with velaglucerase alfa.
Brug ikke dette lægemiddel efter den udløbsdato, der står på pakningen efter Exp. Udløbsdatoen er den sidste dag i den nævnte måned.
Do not use this medicine after the expiry date which is stated on the carton and label after ‘EXP'.
Passende klinisk støtte, herunder personale med tilstrækkelig træning i nødhjælpsprocedurer, skal være umiddelbart tilgængelig, når velaglucerase alfa indgives.
Appropriate medical support, including adequately trained personnel in emergency measures, should be readily available when velaglucerase alfa is administered.
Tyve af de 94 patienter (21 %), der blev behandlet med velaglucerase alfa i kliniske studier, var børn og unge (4 til 17 år).
Twenty of the 94 patients (21%) who received velaglucerase alfa during clinical studies were in the paediatric and adolescent age range (4 to 17 years).
I dette studie blev hæmoglobinkoncentration, trombocyttal, levervolumen og miltvolumen vurderet hos de behandlingsnaive patienter efter 24 måneders behandling.
In this study, haemoglobin concentration, platelet count, liver volume and spleen volume were assessed in treatment-naïve patients after 24 months of treatment.
Studie 058 var et åbent klinisk sikkerhedsstudie hos 211 patienter, herunder 205 patienter, der tidligere var blevet behandlet med imiglucerase, 6 behandlingsnaive patienter og 57 patienter i alderen 65 år og derover (56/57 var skiftet fra imiglucerase til velaglucerase alfa).
Study 058 was an open-label clinical safety study in 211 patients including 205 patients previously treated with imiglucerase 6 treatment-naïve patients and 57 patients aged 65 years or older (56/57 had switched from imiglucerase to velaglucerase alfa).
I dette lille, eksplorative studie var fundene for ikke-neurologisk virkning og sikkerhedsprofilen ved intravenøs administration af velaglucerase alfa hos patienter med Gauchers sygdom, type 3 i overensstemmelse med det observerede hos patienter med Gauchers sygdom, type 1.
In this small, exploratory study, the non-neurological efficacy findings and the safety profile of intravenous velaglucerase alfa in type 3 Gaucher patients were consistent with those observed in patients with type 1 Gaucher disease.
Hvert hætteglas med 400 enheder af denne medicin indeholder 12,15 mg natrium.
Each 400 Units vial of this medicine contains 12.15 mg sodium.
Klinisk betydningsfulde forbedringer fra baseline blev observeret i hæmoglobinkoncentrationen og trombocyttallene allerede efter 3 måneder og i lever- og miltvolumen efter både 6 og 9 måneder efter start på behandling med velaglucerase alfa.
Clinically meaningful improvements from baseline were observed in haemoglobin concentration and platelet counts as early as 3 months and in liver and spleen volumes at both 6 months and 9 months following the initiation of treatment with velaglucerase alfa.
Sikkerhedsprofilen for VPRIV i kliniske studier med patienter i alderen 65 år og derover svarede til den, der blev observeret hos andre voksne patienter.
The safety profile of VPRIV in clinical studies involving patients aged 65 years and above was similar to that observed in other adult patients.
Patienter, der aktuelt behandles med enzymerstatningsterapi med imiglucerase for Gauchers sygdom, type 1, kan skiftes over til VPRIV under anvendelse af samme dosis og doseringshyppighed.
Patients currently treated with imiglucerase enzyme replacement therapy for type 1 Gaucher disease may be switched to VPRIV, using the same dose and frequency.
Der var ingen statistisk signifikante forskelle mellem velaglucerase alfa og imiglucerase i ændringer i trombocyttal samt lever- og miltvolumen efter 9 måneders velaglucerase alfa-behandling og i tiden til første hæmoglobinrespons (defineret som en stigning på 1 g/dl fra baseline).
There were no statistically significant differences between velaglucerase alfa and imiglucerase in changes in platelet counts and liver and spleen volumes after 9 months of velaglucerase alfa treatment, and in the time to first haemoglobin response (defined as 1 g/dl increase from baseline).
Det er nødvendigt at foretage nøje overvågning af graviditeten og de kliniske manifestationer af Gauchers sygdom med henblik på at tilpasse behandlingen til den enkelte patient.
Close monitoring of the pregnancy and clinical manifestations of Gaucher disease is necessary for the individualisation of therapy.
- Hvis reaktionerne er svære, og/eller virkningen af medicinen er nedsat, vil lægen tage en blodprøve for at tjekke for antistoffer, der kan påvirke behandlingsresultatet.
- If the 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 which may affect the outcome of your treatment - Most of the time you can still be given VPRIV even if you experience one of these reactions.
Brug ikke opløsningen, hvis den er misfarvet, eller hvis der ses fremmedpartikler.
Do not use if the solution is discoloured or if foreign particles are present.
De nedenfor beskrevne data afspejler eksponeringen af 94 patienter med Gauchers sygdom, type 1, som fik velaglucerase alfa i doser på 15 til 60 enheder/kg hver anden uge i 5 kliniske studier.
The data described below reflect exposure of 94 patients with type 1 Gaucher disease who received velaglucerase alfa at doses ranging from 15 to 60 Units/kg every other week in 5 clinical studies.
VPRIV indeholder et stof, der hedder velaglucerase alfa, og det er designet til at erstatte det manglende eller dårligt fungerende enzym, glucocerebrosidase, hos patienter med Gauchers sygdom.
VPRIV contains a substance called velaglucerase alfa which is designed to replace the missing or defective enzyme, glucocerebrosidase, in patients with Gaucher disease.
Disse reaktioner omfatter: - Hovedpine - Svimmelhed - Feber/øget kropstemperatur - Nedsat blodtryk eller forhøjet blodtryk - Kvalme og træthed - Brystgener - Vejrtrækningsbesvær - Kløe.
These reactions include; - headache - dizziness - fever/body temperature increased - decreased blood pressure or increased blood pressure - nausea and tiredness - chest discomfort - difficulty in breathing - itching
De pædiatriske patienter, der skiftede fra langvarig imiglucerase til velaglucerase alfa i studie 034, havde en højere gennemsnitlig Z-score for højde ved baseline, og deres gennemsnitlige Z-score for højde forblev stabil over tid.
Paediatric subjects who switched from long-term imiglucerase to velaglucerase alfa in study 034 had greater mean height Z-scores at baseline and their mean height Z-scores remained stable over time.
På grund af risikoen for overfølsomhedsreaktioner, herunder anafylaksi, skal passende klinisk støtte, herunder personale med tilstrækkelig træning i nødhjælpsprocedurer, være umiddelbart tilgængelig, når velaglucerase alfa indgives.
Due to the risk for hypersensitivity reactions including anaphylaxis appropriate medical support, including adequately trained personnel in emergency measures, should be readily available when velaglucerase alfa is administered.
Studie med patienter, der skifter fra imiglucerase-behandling til VPRIV Studie 034 var et 12-måneders, åbent sikkerhedsstudie med 40 patienter i alderen 2 år og opefter, som var blevet behandlet med imiglucerase i doser fra 15 til 60 enheder/kg i minimum 30 måneder i træk.
Study in patients switching from imiglucerase treatment to VPRIV Study 034 was a 12-month, open-label safety study in 40 patients aged 2 years and older who had been receiving treatment with imiglucerase at doses ranging from 15 to 60 Units/kg for a minimum of 30 consecutive months.
Intervallet for velaglucerase alfa-clearance hos pædiatriske patienter (N = 7; aldersinterval 4-17 år) lå inden for intervallet for clearanceværdier hos voksne patienter (N = 15; aldersinterval 19-62 år).
The range of velaglucerase alfa clearance in paediatric patients (N=7, age range 4 to 17 years) was contained within the range of clearance values in adult patients (N=15, age range 19 to 62 years).
9/l), levervolumen reduceredes fra 1,46 til 1,22 gange normalstørrelsen (gennemsnitlig reduktion på 17 %), og miltvolumen reduceredes fra 14,0 til 5,75 gange normalstørrelsen (gennemsnitlig reduktion på 50 %).
9/l), liver volume was reduced from 1.46 to 1.22 times normal (mean reduction of 17%) and spleen volume was reduced from 14.0 to 5.75 times normal (mean reduction of 50%).
Dette var et åbent multicenterstudie, hvor 60 E/kg velaglucerase alfa blev administreret ved intravenøs infusion hver anden uge i 12 måneder hos 6 patienter (i alderen 2 til 17 år ved inklusion) med en bekræftet diagnose på Gauchers sygdom, type 3.
This was a multicentre, open-label study in which 60 U/kg of velaglucerase alfa was administered by intravenous infusion every other week (EOW) over 12 months in 6 patients (2 to 17 years of age at enrolment) with a confirmed diagnosis of type 3 Gaucher disease.
Gauchers sygdom er en autosomal recessiv sygdom, der skyldes mutationer i GBA-genet, som medfører en mangel på det lysosomale enzym betaglucocerebrosidase.
Gaucher disease is an autosomal recessive disorder caused by mutations in the GBA gene which results in a deficiency of the lysosomal enzyme beta-glucocerebrosidase.
Hvis der opstår anafylaktiske eller andre akutte reaktioner på klinikken eller i hjemmet, skal infusionen straks afbrydes og passende medicinsk behandling påbegyndes.
If anaphylactic or other acute reactions occur, in the clinic or home setting, immediately discontinue the infusion and initiate appropriate medical treatment.
Velaglucerase alfa fremstilles på en måde, så det hovedsageligt indeholder højmannoseglykaner for at facilitere internaliseringen af enzymet ved de fagocytiske målceller gennem mannosereceptoren.
Velaglucerase alfa is manufactured to contain predominantly high-mannose-type glycans to facilitate internalisation of the enzyme by the phagocytic target cells via the mannose receptor.
Sådan skal VPRIV anvendes
How VPRIV is used
Ingen patienter udviklede IgE-antistoffer mod velaglucerase alfa.
No patients developed IgE antibodies to velaglucerase alfa.
- Hvis du oplever et eller flere af symptomerne, skal du fortælle det til lægen med det samme.
- If you experience any of the symptoms, you must tell your doctor immediately.
For de patienter, der tidligere var blevet behandlet med imiglucerase, var det mediane antal modtagne velaglucerase alfa-infusioner 8 med en median behandlingsvarighed på 15,1 uger.
Patients previously treated with imiglucerase received a median of 8 velaglucerase alfa infusions with median duration of treatment of 15.1 weeks.
Lignende behandlingseffekt sås i løbet af 48 måneder hos den pædiatriske population, der fik imiglucerase i 9 måneder efterfulgt af velaglucerase alfa.
Similar treatment effects were seen through 48 months in the paediatric population who received 9 months of imiglucerase followed by velaglucerase alfa.
Hvis du ammer eller overvejer at amme, skal du tale med lægen, før dette lægemiddel anvendes.
If you are breast feeding or considering breast-feeding, you should talk to your doctor before this medicine is used.
VPRIV skal rekonstitueres og fortyndes og er udelukkende beregnet til intravenøs infusion.
VPRIV requires reconstitution and dilution, and is intended for intravenous infusion only.
VPRIV er kun til engangsbrug og indgives gennem et 0,22 µm-filter.
VPRIV is for single-use only and is administered through a 0.22 µm filter.
Sikkerhedsprofilen af VPRIV i kliniske studier med børn og unge i alderen 4 til 17 år svarede til sikkerhedsprofilen hos voksne patienter.
The safety profile of VPRIV in clinical studies involving children and adolescents aged 4 to 17 years was similar to that observed in adult patients.
Volumen til udtagelse
Extractable volume
Velaglucerase alfa øger hæmoglobinkoncentrationen og trombocyttallet samt mindsker lever- og miltvolumen hos patienter med Gauchers sygdom, type 1.
Velaglucerase alfa increases haemoglobin concentration and platelet counts and reduces liver and spleen volumes in patients with type 1 Gaucher disease.
VPRIV leveres i et hætteglas som fast pulver, som skal blandes med sterilt vand og yderligere fortyndes i en 9 mg/ml (0,9 %)-natriumchloridopløsning før infusion i en blodåre.
VPRIV is supplied in a vial as a packed powder which is mixed with sterile water and further diluted in sodium chloride 9 mg/ml (0.9%) solution prior to intravenous infusion.
Gauchers sygdom er en genetisk lidelse, der skyldes et manglende eller dårligt fungerende enzym, der hedder glucocerebrosidase.
Gaucher disease is a genetic disorder caused by a missing or defective enzyme named glucocerebrosidase.
Hos 31 behandlingsnaive voksne patienter, der blev behandlet med velaglucerase alfa, var den gennemsnitlige Z-score for BMD i lændehvirvlerne ved baseline -1,820 (95 %-KI: -2,21; -1,43) og steg med 0,62 (95 %-KI: 0,39; 0,84) fra baseline efter 24 måneders behandling med velaglucerase alfa.
Among 31 treatment-naïve adult patients treated with velaglucerase alfa, the mean lumbar spine BMD Z-score at baseline was -1.820 (95% CI: -2.21, -1.43) and increased by 0.62 (95% CI: 0.39, 0.84) from baseline following 24 months of treatment with velaglucerase alfa.
Spørg på apoteket, hvordan du skal bortskaffe medicinrester.
Ask your pharmacist how to throwaway medicines you no longer use.
9/l), levervolumen reduceredes fra 1,40 til 1,24 gange normalstørrelsen (gennemsnitlig reduktion på 6 %), og miltvolumen reduceredes fra 14,5 til 9,50 gange normalstørrelsen (gennemsnitlig reduktion på 40 %).
9/l), liver volume was reduced from 1.40 to 1.24 times normal (mean reduction of 6%) and spleen volume was reduced from 14.5 to 9.50 times normal (mean reduction of 40%).
Velaglucerase alfa blev indledningsvis indgivet på en dosiseskalerende måde hos de første 3 patienter (15, 30 og 60 enheder/kg), og de sidste 9 patienter fik en startdosis på 60 enheder/kg.
Velaglucerase alfa was initially administered in a dose-escalating fashion in the first 3 patients (15, 30, 60 Units/kg) and the 9 remaining patients began treatment with 60 Units/kg.
Velaglucerase alfa supplerer eller erstatter betaglucocerebrosidase (det enzym, der katalyserer hydrolysen af glucocerebrosid til glukose og ceramid i lysosomet), hvorved mængden af ophobet glucocerebrosid mindskes, og hvorved patofysiologien for Gauchers sygdom korrigeres.
Velaglucerase alfa supplements or replaces beta-glucocerebrosidase, the enzyme that catalyzes the hydrolysis of glucocerebroside to glucose and ceramide in the lysosome, reducing the amount of accumulated glucocerebroside and correcting the pathophysiology of Gaucher disease.
Det gives som intravenøs infusion af en læge eller en sygeplejerske.
It is given by a doctor or nurse by intravenous infusion.
Den beregnede mængde lægemiddel udtages fra det antal hætteglas, der skal bruges.
The calculated volume of medicinal product is withdrawn from the appropriate number of vials.
Efter mindst 12 måneders løbende behandling med velaglucerase alfa var alle patienter kvalificerede til at få velaglucerase alfa-dosen reduceret gradvist fra 60 til 30 enheder/kg efter at have nået mindst 2 af de 4 terapeutiske mål for år 1 for enzymerstatningsterapien for Gauchers sygdom, type 1.
After a minimum of 12 months of continuous treatment with velaglucerase alfa, all patients qualified to have the dose of velaglucerase alfa reduced in a step-wise fashion from 60 to 30 Units/kg after achieving at least 2 of the 4 “Year 1” therapeutic goals of ERT for type 1 Gaucher disease.
Det blev påvist, at stigningen i hæmoglobinkoncentration var klinisk og statistisk noninferior over for imiglucerase (gennemsnitlig forskel i ændring mellem behandlingerne fra baseline til 9 måneder [velaglucerase alfaimiglucerase]: 0,135 g/dl).
This increase in haemoglobin concentration was demonstrated to be clinically and statistically non-inferior to imiglucerase (mean treatment difference of change from baseline to 9 months [velaglucerase alfa - imiglucerase]: 0.135 g/dl).
Få patienter oplevede dog en allergisk hudreaktion såsom svært udslæt eller kløe, som kan forekomme hos op til 1 ud af 10 patienter (almindelig hyppighed).
However a few patients experienced an allergic skin reaction which may affect up to 1 in 10 people (common) such as severe rash or itching.
Patienter, der skiftede fra langvarig imiglucerase-behandling til velaglucerase alfa (n
Patients who switched from long-term imiglucerase treatment to velaglucerase alfa (N=38)
Det er kun til engangsbrug og indgives gennem et 0,22 µm-filter.
It is for single-use only and is administered through a 0.22 µm filter.