FrancophonIA/ELRC3.0 huggingface dataset:
English: • non-ketotic hyperglycinaemia, where abnormally high levels of the amino acid glycine occur in the blood.
Danish: • ikke-ketotisk hyperglycinæmi, hvor der ses unormalt høje niveauer af aminosyren glycin i blodet.
English: Furthermore, as Prohippur was not used in clinical trials, the company should have provided data to show that Prohippur granules worked in the same way as the sodium benzoate used in the published studies.
Danish: Da Prohippur ikke var blevet anvendt i kliniske undersøgelser, burde virksomheden desuden have fremlagt data til påvisning af, at Prohippur-granulatet virker på samme måde som det natriumbenzoat, der blev anvendt i de offentliggjorte undersøgelser.
English: The company informed the CHMP that there are no ongoing clinical trials with Prohippur and that a compassionate use programme approved in France will continue as planned.
Danish: Virksomheden har oplyst til CHMP, at der for tiden ikke gennemføres kliniske undersøgelser af Prohippur, og at et program for anvendelse med særlig udleveringstilladelse, der er tilladt i Frankrig, vil blive gennemført som planlagt.
English: The company presented data from published studies on the use of sodium benzoate in patients with non-ketotic hyperglycinaemia and urea cycle disorders.
Danish: Virksomheden fremlagde data fra offentliggjorte undersøgelser om anvendelse af natriumbenzoat hos patienter med ikke-ketotisk hyperglycinæmi og defekt urincyklus.
English: • urea cycle disorders, a group of conditions where abnormally high levels of nitrogen occur in the body in the form of ammonia.
Danish: • defekt urinstofcyklus, der er en gruppe tilstande, hvor der ses unormalt høje niveauer af nitrogen i kroppen i form af ammoniak.
English: The CHMP noted that the published studies submitted by the company did not provide enough evidence of the effectiveness and safety of sodium benzoate in the various disorders.
Danish: CHMP bemærkede, at de offentliggjorte undersøgelser, som virksomheden havde fremlagt, ikke var tilstrækkelig dokumentation for effekten og sikkerheden ved natriumbenzoat til behandling af de nævnte sygdomme.
English: What did the company present to support its application?
Danish: Hvilken dokumentation fremlagde virksomheden til støtte for sin ansøgning?
English: 30 Churchill Place ● Canary Wharf ● London E14 5EU ● United Kingdom27 April 2018 EMA/258015/2018 EMEA/H/C/004150
Danish: EMA/258015/2018 EMEA/H/C/004150
English: Further information on the orphan designation can be found here.
Danish: Yderligere oplysninger om lægemidler til sjældne sygdomme findes her
English: How does Prohippur work?
Danish: Hvordan virker Prohippur?
English: Withdrawal of the marketing authorisation application for Prohippur (sodium benzoate) EMA/258015/2018
Danish: EMA/258015/2018
English: 5 years.
Danish: 5 år.
English: Date of first authorisation: 20 November 2002 Date of latest renewal: 20 November 2012
Danish: Dato for første markedsføringstilladelse: 20. november 2002 Dato for seneste fornyelse: 20. november 2012
English: Zavesca can be taken with or without food.
Danish: Zavesca kan tages sammen med eller uden mad.
English: Most serious side effects:
Danish: Mest alvorlige bivirkninger:
English: You should not take Zavesca if you are pregnant or thinking of becoming pregnant.
Danish: De må ikke tage Zavesca, hvis De er gravid, eller hvis De overvejer at blive gravid.
English: Do not store above 30 °C.
Danish: Må ikke opbevares ved temperaturer over 30 °C.
English: Don't stop taking Zavesca without talking to your doctor.
Danish: Stop ikke med at tage Zavesca uden at rådføre Dem med lægen.
English: Capsule shell Gelatin,
Danish: Kapselskallen Gelatine,
English: The other ingredients are:
Danish: Øvrige indholdsstoffer:
English: If you do lose some weight when you start treatment with Zavesca don't worry.
Danish: Hvis De taber i vægt, når De starter behandling med Zavesca, skal De ikke blive bekymret.
English: Miglustat crosses the placenta and should not be used during pregnancy.
Danish: Miglustat passerer placenta og Zavesca bør ikke anvendes under graviditet.
English: Among the 41 patients enrolled in the trial 14 patients were treated with Zavesca for more than 3 years.
Danish: Af de 41 patienter, der deltog i studiet, blev 14 patienter behandlet med Zavesca i mere end 3 år.
English: Tell your doctor if you are taking medicines containing imiglucerase, which are sometimes used at the same time as Zavesca.
Danish: Fortæl det til lægen, hvis De tager lægemidler, der indeholder imiglucerase, som nogle gange bruges samtidigt med Zavesca.
English: If you are pregnant, breast feeding, think you may be pregnant or planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.
Danish: Hvis De er gravid eller ammer, har mistanke om, at De er gravid, eller planlægger at blive gravid, skal De spørge Deres læge eller apotekspersonalet til råds, før De tager dette lægemiddel.
English: Your doctor will perform some tests before and during treatment with Zavesca to assess this (see section 2).
Danish: Deres læge vil gennemføre nogle prøver før og under behandling med Zavesca for at bedømme dette (se punkt 2).
English: He or she may reduce the dose of Zavesca or recommend other medicines to help control side effects.
Danish: Han eller hun kan nedsætte Deres dosis af Zavesca eller anbefale anden medicin, der kan hjælpe med at kontrollere bivirkningerne.
English: Name and address of the manufacturer responsible for batch release Actelion Manufacturing GmbH
Danish: Navn og adresse på den fremstiller, der er ansvarlig for batchfrigivelse Actelion Manufacturing GmbH
English: Titanium dioxide (E171).
Danish: Titandioxid (E171).
English: Do not give this medicine to children and adolescents (below 18 years old) with type 1 Gaucher disease because it is not known if it works in this disease.
Danish: Giv ikke denne medicin til børn og unge (under 18 år) med Gauchers sygdom, type 1, da det ikke vides, om den virker.
English: It is not known if miglustat is secreted in breast milk.
Danish: Det vides ikke, om miglustat udskilles i mælk.
English: They may lower the amount of Zavesca in your body.
Danish: Imiglucerase kan nedsætte mængden af Zavesca i kroppen.
English: Zavesca has been administered at doses of up to 3000 mg/day for up to six months in HIV positive patients during clinical trials.
Danish: Zavesca er indgivet i doser på op til 3000 mg/dag i op til seks måneder hos HIV-positive patienter under kliniske forsøg.
English: Eighteen patients then continued to receive Zavesca under an optional extended treatment protocol.
Danish: Atten patienter fortsatte dernæst med at modtage Zavesca under en frivillig forlængelse af behandlingsprotokollen.
English: Weight loss has been reported in 55% of patients.
Danish: Vægttab er rapporteret hos 55% af patienterne.
English: When compared to the measurements at 6 months, disease control was unchanged after 18 and 24 months of Zavesca monotherapy (20 and 6 patients, respectively).
Danish: Sammenlignet med målingerne efter 6 måneder var sygdomskontrollen uændret efter 18 og 24 måneders Zavesca-monoterapi (henholdsvis 20 og 6 patienter).
English: There are no pharmacokinetic data available in patients with liver impairment or in the elderly (> 70 years).
Danish: Der foreligger ingen tilgængelige farmakokinetiske data for patienter med leverinsufficiens eller ældre (>70 år).
English: Zavesca contains the active substance miglustat which belongs to a group of medicines that affect metabolism.
Danish: Zavesca indeholder det aktive stof miglustat, som tilhører en gruppe af lægemidler, som påvirker stofskiftet.
English: This study also indicated that Zavesca has no or limited effect on the pharmacokinetics of imiglucerase.
Danish: Denne undersøgelse tyder også på, at Zavesca ingen eller begrænset effekt har på imiglucerases farmakokinetik.
English: They could be signs of peripheral neuropathy, due to side effects of Zavesca or they could be due to existing conditions.
Danish: Dette kan være tegn på perifer neuropati, som skyldes bivirkninger af Zavesca, eller det kan være på grund af eksisterende lidelser.
English: Your doctor will perform the following tests before treatment and during treatment with Zavesca: - an examination to check the nerves in your arms and legs - measurement of vitamin B12 levels - monitoring growth if you are a child or adolescent with Niemann-Pick type C disease - monitoring of blood platelet counts
Danish: Deres læge vil gennemføre følgende tests, før og under behandling med Zavesca: - en undersøgelse for at kontrollere nerverne i Deres arme og ben - måling af vitamin B12-niveauerne - kontrollere Deres vækst, hvis De er barn eller ung med Niemann-Picks sygdom, type C. - kontrollere antallet af blodplader
English: This leaflet was last revised in:
Danish: Denne indlægsseddel blev senest ændret:
English: In some patients there were reductions in platelet count and increases in chitotriosidase activity indicating that Zavesca monotherapy may not maintain the same control of disease activity in all patients.
Danish: Hos nogle patienter var der reduktioner i trombocyttal og forhøjet chitotriosidaseaktivitet, hvilket tyder på, at Zavesca-monoterapi måske ikke opretholder samme kontrol med sygdomsaktiviteten hos alle patienter.
English: Package leaflet: Information for the user Zavesca 100 mg capsules
Danish: Indlægsseddel: Information til brugeren Zavesca 100 mg kapsler
English: If you stop taking Zavesca
Danish: Hvis De holder op med at tage Zavesca
English: There is no experience with the use of Zavesca in patients over the age of 70.
Danish: Der er ingen erfaring med Zavesca hos patienter over 70 år.
English: A total daily dose of 300 mg Zavesca administered in three divided doses was used in the above two studies.
Danish: Der blev benyttet en samlet daglig dosis på 300 mg Zavesca fordelt på tre doser i ovennævnte to undersøgelser.
English: Zavesca is only used when a patient is considered unsuitable for treatment with enzyme replacement therapy.
Danish: Zavesca anvendes kun, når en patient anses for uegnet til behandling med enzymsubstitutionsterapi.
English: A second open, controlled study randomised 36 patients who had received a minimum of 2 years of treatment with ERT, into three treatment groups: continuation with imiglucerase, imiglucerase in combination with Zavesca, or switch to Zavesca.
Danish: En anden åben, kontrolleret undersøgelse randomiserede 36 patienter, som havde modtaget minimum 2 års behandling med ERT, i tre behandlingsgrupper: fortsat behandling med imiglucerase, imiglucerase i kombination med Zavesca, eller skift til Zavesca.
English: For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder.
Danish: Hvis De ønsker yderligere oplysninger om dette lægemiddel, skal De henvende Dem til den lokale repræsentant for indehaveren af markedsføringstilladelsen:
English: Zavesca has negligible influence on the ability to drive and use machines.
Danish: Zavesca påvirker kun i ubetydelig grad evnen til at føre motorkøretøj eller betjene maskiner.
English: Zavesca has been used in clinical trials at doses ten times higher than the recommended dose: this caused decreases in white blood cells and other side effects similar to those described in section 4.
Danish: Zavesca er blevet anvendt i kliniske forsøg med doser, der var ti gange højere, end den anbefalede dosis: Dette medførte et fald i antallet af hvide blodlegemer og andre bivirkninger svarende til dem, der er beskrevet i pkt.
English: Limited data suggest that co-administration of Zavesca and enzyme replacement with imiglucerase in patients with type 1 Gaucher disease may result in decreased exposure to miglustat (approximate reductions of 22% in Cmax and 14% in AUC were observed in a small parallel-group study).
Danish: Begrænsede data tyder på, at samtidig indgivelse af Zavesca og enzymsubstitutionsterapi med imiglucerase hos patienter med Gauchers sygdom, type 1 kan medføre reduceret eksponering for miglustat (reduktioner på cirka 22% i Cmax og 14% i AUC blev observeret i en lille parallel-gruppeundersøgelse).
English: MINIMUM PARTICULARS TO APPEAR ON BLISTERS OR STRIPS BLISTERS
Danish: MINDSTEKRAV TIL MÆRKNING PÅ BLISTER ELLER STRIP BLISTER
English: In 11 clinical trials in different indications 247 patients were treated with Zavesca at dosages of 50200 mg t.i.d.
Danish: I 11 kliniske undersøgelser med forskellige indikationer blev 247 patienter behandlet med Zavesca i doser på 50-200 mg t.i.d.
English: This study was conducted over a 6-month randomised comparison period followed by 18 months extension where all patients received Zavesca monotherapy.
Danish: Denne undersøgelse blev gennemført over en 6-måneders randomiseret sammenligningsperiode, efterfulgt af 18 måneders forlængelse, hvor alle patienter fik Zavesca monoterapi.
English: The tests will help the doctor decide whether these effects are due to your disease or other existing conditions, or due to side effects of Zavesca (see section 4 for further details).
Danish: Prøverne vil hjælpe lægen til at beslutte, om disse virkninger skyldes Deres sygdom eller eksisterende tilstande eller er bivirkninger af Zavesca (se pkt.
English: Small mean reductions in haemoglobin [0.95 g/dL (95% CI: -1.38, -0.53)] and platelet count [-44.1 × 109/L (95% CI: -57.6, -30.7)] were observed between baseline and end of study.
Danish: Små gennemsnitlige reduktioner i hæmoglobin [-0,95 g/dl (95% CI: -1,38; -0,53)] og trombocyttal [-44,1 × 109/l (95% CI: -57,6; -30,7)] sås mellem baseline og studieafslutning.
English: The reason for these tests is that some patients have had tingling or numbness in the hands and feet, or a decrease in body weight, while taking Zavesca.
Danish: Årsagen til disse prøver er, at visse patients har snurren eller følelsesløshed i hænder og fødder, eller taber i vægt, når de tager Zavesca.
English: Sometimes your doctor will need to reduce the dose or stop Zavesca treatment to stop the tremor.
Danish: Af og til vil det være nødvendigt for lægen at nedsætte dosis eller stoppe behandlingen med Zavesca for at stoppe denne rysten.
English: Miglustat affected embryo/foetal survival in rats and rabbits, dystocia was reported, post-implantation losses were increased, and an increased incidence of vascular anomalies occurred in rabbits.
Danish: Miglustat påvirkede embryo-/føtal overlevelse hos rotter og kaniner, dystoci blev indberettet, postimplantationstab blev forøget, og der var en øget forekomst af vaskulære anomalier hos kaniner.
English: - if you are allergic to miglustat or any of the other ingredients of this medicine (listed in section 6)
Danish: - hvis De er allergisk over for miglustat eller et af de øvrige indholdsstoffer i Zavesca (angivet i punkt 6).
English: If you have diarrhoea, your doctor may ask you to change your diet to reduce your lactose and carbohydrate intake such as sucrose (cane sugar), or not to take Zavesca together with food, or to temporarily reduce your dose.
Danish: Hvis De har diarré, vil Deres læge måske bede Dem om følgende: At De ændrer Deres kost for at reducere indtagelsen af lactose og kulhydrater såsom saccharose (sukker), at De ikke tager Zavesca sammen med måltider, eller at De nedsætter Deres dosis midlertidigt.
English: ACLAR/ALU blister strips supplied as a box of 4 blister strips, each blister strip containing 21 capsules providing a total of 84 capsules.
Danish: ACLAR/ALU-blisterstrips leveres som en æske med 4 blisterstrips, hvor hver blisterstrip indeholder 21 kapsler, hvilket giver i alt 84 kapsler.
English: After 3 years of continuous Zavesca treatment, mean reductions in liver and spleen organ volume were 17.5% and 29.6%, respectively.
Danish: Efter 3 års kontinuerlig behandling med Zavesca var de gennemsnitlige reduktioner i lever- og miltorganvolumen på henholdsvis 17,5% og 29,6%.
English: Management In case of overdose general medical care is recommended.
Danish: Behandling I tilfælde af overdosering anbefales almen, understøttende behandling.
English: Talk to your doctor or pharmacist before taking Zavesca - if you suffer from kidney disease
Danish: Kontakt lægen eller apotekspersonalet, før De tager Zavesca, - hvis De lider af en nyresygdom - hvis De lider af en leversygdom
English: In the first 6 months in patients who were switched to Zavesca, liver and spleen organ volumes and haemoglobin levels were unchanged.
Danish: I de første 6 måneder hos patienter, der skiftede til Zavesca, var lever- og miltorganvolumina og hæmoglobinniveauer uændrede.
English: Data to support safety and efficacy of Zavesca in Niemann-Pick type C disease come from a prospective open-label clinical trial and a retrospective survey.
Danish: Data, der understøtter Zavescas sikkerhed og virkning ved Niemann-Picks sygdom, type C, stammer fra et prospektivt, åbent klinisk studie og en retrospektiv undersøgelse.
English: No patient showed rapid deterioration of type 1 Gaucher disease following the switch to Zavesca monotherapy.
Danish: Ingen patienter udviste hurtig forværring af Gauchers sygdom type 1 efter skift til Zavescamonoterapi.
English: Due to limited experience, Zavesca should be used with caution in patients with renal or hepatic impairment.
Danish: På grund af begrænset erfaring bør Zavesca anvendes med forsigtighed hos patienter med nyre- eller leverinsufficiens.
English: Use of Zavesca in patients with severe renal impairment (creatinine clearance < 30 ml/min/1.73 m2) is not recommended.
Danish: Anvendelse af Zavesca til patienter med svær nyreinsufficiens (kreatinin-clearance <30 ml/min/1,73 m2) kan ikke anbefales.
English: Contents of the pack and other information What Zavesca contains
Danish: Pakningsstørrelser og yderligere oplysninger Zavesca indeholder:
English: Other medicines and Zavesca
Danish: Brug af anden medicin sammen med Zavesca
English: Although no direct comparisons with Enzyme Replacement Therapy (ERT) have been performed in treatment-naive patients with type 1 Gaucher disease, there is no evidence of Zavesca having an efficacy or safety advantage over ERT. ERT is the standard of care for patients who require treatment for type 1 Gaucher disease (see section 5.1).
Danish: Der er ikke udført direkte sammenligninger med enzymsubstitutionsterapi (ERT) hos behandlingsnaive patienter med Gauchers sygdom, type 1, og der er intet bevis for, at Zavesca har fordele med hensyn til effektivitet eller sikkerhed i forhold til ERT. ERT er standardbehandling for patienter, der kræver behandling for Gauchers sygdom type 1 (se pkt.
English: The benefit to the patient of treatment with Zavesca should be evaluated on a regular basis (see section
Danish: Patienternes gavn af behandling med Zavesca skal evalueres regelmæssigt (se pkt.
English: An open-label, non comparative, 2-year study enrolled 42 patients with type 1 Gaucher disease, who had received a minimum of 3 years of ERT and who fulfilled criteria of stable disease for at least 2 years.
Danish: Et åbent, non-komparativt studie af 2 års varighed inkluderede 42 patienter med Gauchers sygdom, type 1, som i mindst 3 år havde fået ERT, og som opfyldte kriteriet stabil sygdom i mindst 2 år.
English: What you need to know before you take Zavesca Do not take Zavesca
Danish: Det skal De vide, før De begynder at tage Zavesca Tag ikke Zavesca
English: Zavesca has been studied in indications where certain events reported as adverse reactions, such as neurological and neuropsychological symptoms/signs, cognitive dysfunction and thrombocytopenia could also be due to the underlying conditions.
Danish: Zavesca er undersøgt ved indikationer, hvor visse hændelser rapporteret som bivirkninger, som f.eks. neurologiske og neuropsykologiske symptomer/tegn, kognitiv dysfunktion og trombocytopeni, også kunne skyldes de underliggende sygdomme.
English: PARTICULARS TO APPEAR ON THE OUTER PACKAGING OUTER CARTON
Danish: MÆRKNING, DER SKAL ANFØRES PÅ DEN YDRE EMBALLAGE YDRE ÆSKE
English: You must use effective birth control while taking Zavesca.
Danish: De skal benytte effektiv prævention, mens De tager Zavesca.
English: If you forget to take Zavesca
Danish: Hvis De har glemt at tage Zavesca
English: The survey included a case series of 66 patients treated with Zavesca outside of the clinical trial for a mean duration of 1.5 years.
Danish: Undersøgelsen omfattede et patientmateriale på 66 patienter, som blev behandlet med Zavesca uden for den kliniske undersøgelse med en gennemsnitlig behandlingsvarighed på 1,5 år.
English: Box of 4 blister strips, each blister strip containing 21 capsules providing a total of 84 capsules.
Danish: Æske med 4 blisterpakninger hver indeholdende 21 kapsler, hvilket giver 84 kapsler i alt.
English: This can result in liver and spleen enlargement, changes in the blood, and bone disease.
Danish: Dette kan medføre forstørrelse af lever og milt, forandringer i blodet og knoglesygdom.
English: Do not take this medicine after the expiry date which is stated on the carton after ‘EXP'.
Danish: Brug ikke lægemidlet efter den udløbsdato, der står på pakningen efter Exp. Udløbsdatoen er den sidste dag i den nævnte måned.
English: In clinical practice, miglustat-induced gastrointestinal events have been observed to respond to individualised diet modification (for example reduction of sucrose, lactose and other carbohydrate intake), to taking Zavesca between meals, and/or to anti-diarrhoeal medicinal products such as loperamide.
Danish: I klinisk praksis er det observeret, at miglustatinducerede gastrointestinale bivirkninger reagerer på individuelle kostændringer (for eksempel nedsat indtagelse af saccharose, lactose og andre kulhydrater), på indtagelse af Zavesca mellem måltiderne og/eller på lægemidler mod diarré, såsom loperamid.
English: Overall the data show that treatment with Zavesca can reduce the progression of clinically relevant neurological symptoms in patients with Niemann-Pick type C disease.
Danish: Generelt viste data, at behandling med Zavesca kan reducere progressionen af klinisk relevante neurologiske symptomer hos patienter med Niemann-Picks sygdom, type C.
English: The efficacy of Zavesca in children and adolescents aged 0-17 years with type 1 Gaucher disease has not been established.
Danish: Zavescas virkning hos børn og unge i alderen 0-17 år med Gauchers sygdom, type 1, er ikke klarlagt.
English: The efficacy and safety of Zavesca has not been specifically evaluated in patients with severe Gaucher disease.
Danish: Zavescas virkning og sikkerhed er ikke specifikt evalueret hos patienter med svær Gauchers sygdom.
English: Male patients should use reliable birth control methods during their treatment with Zavesca, and for 3 months after finishing treatment.
Danish: Mandlige patienter bør anvende pålidelige præventionsmidler under behandling med Zavesca og i 3 måneder efter, at behandlingen er afsluttet.
English: Approximately 37% of patients in clinical trials in type 1 Gaucher disease, and 58% of patients in a clinical trial in Niemann-Pick type C disease reported tremor on treatment.
Danish: Cirka 37% af patienterne i kliniske studier med Gauchers sygdom, type 1 og 58% af patienterne i kliniske studier med Niemann-Picks sygdom, type C, rapporterede om tremor ved behandling.
English: Ask your pharmacist how to throw away medicines you no longer take.
Danish: Spørg på apoteket, hvordan De skal bortskaffe medicinrester.
English: Zavesca is a white 100 mg capsule with “OGT 918” printed in black on the cap and “100” printed in black on the body.
Danish: Zavesca er en hvid 100 mg kapsel med ”OGT 918” trykt i sort på kapslens overdel og ”100” trykt i sort på underdelen.
English: Black iron oxide (E172) Shellac.
Danish: Sort jernoxid (E172) Shellac.
English: Sodium starch glycollate, Povidone (K30), Magnesium stearate.
Danish: Natriumstivelsesglycolat, Povidon (K30), Magnesiumstearat.
English: Detailed information on this medicinal product is available on the website of the European Medicines Agency http://www.ema.europa.eu.
Danish: Yderligere oplysninger om Zavesca findes på Det Europæiske Lægemiddelagenturs hjemmeside http://www.ema.europa.eu.