FrancophonIA/ELRC3.0 huggingface dataset:
English: A higher intestinal concentration of Kuvan is needed to inhibit BCRP than P-gp, as inhibitory potency in intestine for BCRP (IC50=267 µM) is lower than P-gp (IC50=158 µM).
Danish: Der er brug for en højere intestinalkoncentration af Kuvan for at hæmme BCRP end P-gp, da hæmningsevnen i tarmen for BCRP (IC50=267 µM) er lavere end P-gp (IC50=158 µM).
English: See leaflet for further information.
Danish: Se indlægssedlen for flere oplysninger.
English: Treatment with Kuvan must be initiated and supervised by a physician experienced in the treatment of PKU and BH4 deficiency.
Danish: Behandling med Kuvan skal initieres og superviseres af læger med erfaring i behandling af PKU og BH4-mangel.
English: BH4 deficiency is a group of disorders arising from mutations or deletions in the genes encoding for one of the five enzymes involved in the biosynthesis or recycling of BH4.
Danish: BH4-mangel er en gruppe sygdomme der skyldes mutationer eller deletioner i de gener, der koder for et af de 5 enzymer, som er involveret i biosyntesen eller regenerationen af BH4.
English: The rate and extent of absorption of sapropterin is influenced by food.
Danish: Hastighed og omfang af absorptionen af sapropterin er afhængig af fødeindtaget.
English: The calculated daily dose based on body weight should be rounded to the nearest multiple of 100.
Danish: Den daglige beregnede dosis på basis af kropsvægt bør afrundes til nærmeste multiplum af 100.
English: Contents of the pack and other information
Danish: Pakkens indhold og yderligere oplysninger
English: Kuvan contains the active substance sapropterin which is a synthetic copy of a body's own substance called tetrahydrobiopterin (BH4).
Danish: Kuvan indeholder det aktive stof sapropterin, som er en kunstig kopi af et af kroppens egne stoffer, som kaldes tetrahydrobiopterin (BH4).
English: If the strict diet does not adequately reduce phenylalanine amount in your blood your doctor will consider whether you must take this medicine.
Danish: Hvis den begrænsede kost ikke nedsætter fenylalalin i blodet tilstrækkeligt, vil din læge overveje, om du kan tage denne medicin.
English: Dosing for BH4 deficiency
Danish: Dosering ved BH4-mangel
English: Polyethylene terephthalate, aluminum, polyethylene laminate sachet, heat sealed on four sides.
Danish: Brev af polyetylentereftalat, aluminium, polyetylenlaminat, varmeforseglet på alle fire sider.
English: Keep the bottle tightly closed in order to protect from moisture.
Danish: Hold tabletbeholderen tæt tillukket for at beskytte mod fugt.
English: Doses may be adjusted up to a total of 20 mg/kg per day.
Danish: Dosis kan justeres op til i alt 20 mg/kg pr. dag.
English: Do not swallow the tube or the contents.
Danish: Slug hverken røret eller dets indhold.
English: If you forget to take Kuvan
Danish: Hvis du har glemt at tage Kuvan
English: Taking the medicine • Drink the solution within 30 minutes.
Danish: Sådan tages lægemidlet • Drik opløsningen inden for 30 minutter.
English: If you have red, itchy, raised areas (hives), runny nose, fast or uneven pulse, swelling of your tongue and throat, sneezing, wheezing, serious difficulty in breathing or dizziness, you may be having a serious allergic reaction to the medicine.
Danish: Hvis du får røde, kløende, hævede hudområder (nældefeber), løbenæse, hurtig eller uregelmæssig puls, hævelse af tunge eller svælg, nysen, hvæsende åndedræt, alvorligt vejrtrækningsbesvær eller svimmelhed, kan det skyldes en allergisk reaktion på lægemidlet.
English: Response to this medicinal product is determined by a decrease in blood phenylalanine.
Danish: Respons på dette lægemiddel bestemmes ved et fald i blodfenylalanin.
English: Emesis is considered to be related to the pH of the solution containing sapropterin.
Danish: Emesis menes at være relateret til sapropterinopløsningens pH.
English: In the pivotal clinical trials and in the post-marketing experience for Kuvan, the following adverse reactions have been identified.
Danish: I de pivotale kliniske forsøg med Kuvan og ud fra erfaringerne efter markedsføringen blev nedenstående bivirkninger identificeret.
English: Available disease-associated maternal and/or embryofoetal risk data from the Maternal Phenylketonuria Collaborative Study on a moderate amount of pregnancies and live births (between 300-1,000) in PKU-affected women demonstrated that uncontrolled phenylalanine levels above 600 μmol/l are associated with a very high incidence of neurological, cardiac, facial dysmorphism, and growth anomalies.
Danish: Tilgængelige data vedrørende den maternelle og/eller embryoføtale risiko forbundet med sygdommen fra Maternal Phenylketonuria Collaborative Study fra et begrænset antal graviditeter og levendefødsler (mellem 300-1.000) hos kvinder med PKU viste, at ukontrollerede fenylalaninniveauer over 600 μmol/l er forbundet med en meget stor incidens af neurologiske anomalier, hjerte- og vækstanomalier samt ansigtsdysmorfi.
English: Draw up the prescribed volume from the solution prepared in the medicine cup and place the tip of the oral syringe into your baby's mouth.
Danish: Træk det ordinerede volumen op fra opløsningen, der blev tilberedt i medicinbægret, og anbring spidsen af den orale sprøjte i barnets mund.
English: Pharmacotherapeutic group: Other alimentary tract and metabolism products, Various alimentary tract and metabolism products, ATC code: A16AX07
Danish: Farmakoterapeutisk klassifikation: Andre midler til fordøjelsesorganer og stofskifte, diverse midler til fordøjelsesorganer og stofskifte, ATC-kode: A16AX07
English: Volume of solution to be administered
Danish: Volumen af opløsning, der skal administreres
English: Since sapropterin dihydrochloride is a synthetic version of the naturally occurring 6R-BH4, it can be reasonably anticipated to undergo the same metabolism, including 6R-BH4 regeneration.
Danish: Da sapropterindihydrochlorid er en syntetisk version af det naturligt forekommende 6R-BH4, må det rimeligvis kunne antages at metaboliseres på samme vis, inklusive 6R-BH4-regeneration.
English: For instance, a calculated dose of 401 to 450 mg should be rounded down to 400 mg corresponding to 4 tablets.
Danish: Eksempelvis bør en beregnet dosis fra 401 til 450 mg rundes ned til 400 mg svarende til 4 tabletter.
English: Supplies needed to prepare and give your child's dose of Kuvan
Danish: Nødvendige remedier til at tilberede og give dit barn Kuvan-dosen.
English: This medicine is also used to treat an inherited disease called BH4 deficiency in patients of all ages, in which the body cannot produce enough BH4.
Danish: Denne medicin anvendes også til at behandle en nedarvet sygdom hos patienter i alle aldre, der kaldes BH4-mangel, hvor kroppen ikke selv kan danne tilstrækkeligt med BH4.
English: Kuvan has no or negligible influence on the ability to drive and use machines.
Danish: Kuvan påvirker ikke eller kun i ubetydelig grad evnen til at føre motorkøretøj og betjene maskiner.
English: Date of revision of the text: MM/YYYY
Danish: Dato for ændring af teksten:{MM/ÅÅÅÅ}
English: It is of primary importance to initiate treatment as early as possible to avoid the appearance of non-reversible clinical manifestations of neurological disorders in paediatric patients and cognitive deficits and psychiatric disorders in adults due to sustained elevations of blood phenylalanine.
Danish: Det er vigtigt at initiere behandling så tidligt som muligt for at forebygge irreversible neurologiske skader hos pædiatriske patienter og tab af kognitive funktioner og psykiske sygdomme hos voksne patienter på grund af vedvarende forhøjet fenylalanin i blodet.
English: HPA and PKU are due to abnormally high levels of phenylalanine in the blood which can be harmful.
Danish: HPA og PKU skyldes et unormalt højt indhold af fenylalanin i blodet, hvilket kan være skadeligt.
English: High-density polyethylene (HDPE) bottle with child-resistant closure.
Danish: High-density polyethylen (HDPE)-tabletbeholder med børnesikret lukke.
English: Based on an in vitro study, there is potential for sapropterin dihydrochloride to inhibit p-glycoprotein (P-gp) and breast cancer resistance protein (BCRP) in the gut at the therapeutic doses.
Danish: På baggrund af et in vitro-studie er der potentiale for, at sapropterindihydrochlorid hæmmer pglykoprotein (P-gp) og brystcancer-resistensprotein (BCRP) i tarmen ved terapeutiske doser.
English: Volume of solution to be administered (ml)*
Danish: Volumen af opløsningen der skal administreres (ml)*
English: In both cases, phenylalanine cannot be effectively transformed into the amino acid tyrosine, leading to increased phenylalanine levels in the blood.
Danish: I begge tilfælde bliver fenylalanin ikke tilstrækkeligt omdannet til aminosyren tyrosin, hvilket fører til øget niveau af fenylalanin i blodet.
English: - if you are allergic to sapropterin or any of the other ingredients of this medicine (listed in section 6).
Danish: - hvis du er allergisk over for sapropterin eller et af de øvrige indholdsstoffer i dette lægemiddel (angivet i punkt 6).
English: Push on the plunger slowly, a small amount at a time, until all of the solution in the oral syringe is given.
Danish: Tryk langsomt stemplet ned, lidt af gangen, indtil hele opløsningen i den orale sprøjte er givet.
English: Discontinuation of treatment should be done only under the supervision of a physician.
Danish: Behandlingen må kun seponeres under lægelig overvågning.
English: More frequent monitoring may be required, as blood phenylalanine levels may increase.
Danish: Det kan være nødvendigt med hyppigere overvågning, idet fenylalaninniveauet i blodet muligvis stiger.
English: If after approximately 4 weeks, a patient's phenylalanine tolerance had not increased by >20% versus baseline, the Kuvan dose was increased in a single step to 20 mg/kg/day.
Danish: Hvis en patients tolererede fenylalaninindtagelse efter ca. 4 uger ikke var øget med >20 % versus baseline, blev Kuvan-dosis øget til 20 mg/kg/dag i et enkelt trin.
English: If you are not able to give your child's dose within 30 minutes after dissolving the powder, you will need to prepare a new solution as the unused solution should not be used beyond 30 minutes.
Danish: Hvis du ikke er i stand til at give dit barn dosen inden for 30 minutter efter opløsning af pulveret, skal du tilberede en ny opløsning, da den ubrugte opløsning ikke må bruges efter 30 minutter.
English: A calculated dose of 451 mg to 499 mg should be rounded up to 500 mg corresponding to 5 tablets.
Danish: En beregnet dosis fra 451 til 499 mg bør rundes op til 500 mg svarende til 5 tabletter.
English: The oral syringe and the cup should be stored for next use.
Danish: Den orale sprøjte og bægret skal opbevares indtil næste anvendelse.
English: Both parts of the oral syringe and the cup should be washed with warm water and air dry.
Danish: Begge den orale sprøjtes dele og bægret bør vaskes med varmt vand og lufttørres.
English: Prolonged exposure to low blood phenylalanine and tyrosine levels during infancy has been associated with impaired neurodevelopmental outcome.
Danish: Langvarig eksponering for lave fenylalanin- og tyrosinniveauer i blodet i barndommen er associeret med nedsat udvikling af nervesystemet.
English: Kuvan 500 mg is for use in patients above 25 kg body weight only.
Danish: Kuvan 500 mg er kun til brug for patienter med en kropsvægt på over 25 kg.
English: If maternal phenylalanine levels are not strictly controlled before and during pregnancy, this could be harmful to the mother and the foetus.
Danish: Hvis det maternelle fenylalaninniveau ikke kontrolleres tæt før og under graviditet, kan det skade såvel den gravide som fosteret.
English: If you are allergic to sapropterin or any of the other ingredients of this medicine (listed in section 6).
Danish: Hvis du er allergisk over for sapropterin eller et af de øvrige indholdsstoffer i dette lægemiddel (angivet i punkt 6).
English: Sustained or recurrent dysfunction in the phenylalanine-tyrosine-dihydroxy-L-phenylalanine (DOPA) metabolic pathway can result in deficient body protein and neurotransmitter synthesis.
Danish: Vedholdende eller tilbagevendende dysfunktion af fenylalanin-tyrosin-dihydroxy-L-fenylalanin (DOPA) metabolismen kan medføre utilstrækkelig protein- og neurotransmittersyntese.
English: Once responsiveness to the medicinal product has been established, the dose may be adjusted within the range of 5 to 20 mg/kg/day according to response to therapy.
Danish: Når det én gang er påvist, at patienten responderer på lægemidlet, kan dosis justeres inden for 5 til 20 mg/kg/dag i overensstemmelse med behandlingsrespons.
English: Maternal blood phenylalanine levels must therefore be strictly controlled before and during pregnancy.
Danish: Det maternelle fenylalaninniveau i blodet skal derfor holdes under tæt kontrol før og under graviditet.
English: Excretion of total biopterin in milk was demonstrated in rats by intravenous route.
Danish: Udskillelsen af total biopterin i mælk sås hos rotter efter intravenøs indgift.
English: If you are not able to give your child's dose within 15 to 20 minutes after dissolving the tablets, you will need to prepare a new solution as the unused solution should not be used beyond 20 minutes.
Danish: Hvis du ikke er i stand til at give dit barn dosen inden for 15 til 20 minutter efter, at du har opløst tabletterne, skal du tilberede en ny opløsning, da den ubrugte opløsning ikke må bruges efter 20 minutter.
English: Do not stop taking Kuvan without prior discussion with your doctor, as phenylalanine levels in your blood may increase.
Danish: Du må ikke holde op med at tage Kuvan uden at have talt med din læge, da indholdet af fenylalanin i dit blod kan stige.
English: Depending on the dose (in mg/kg/day) the appropriate number of tablets should be dissolved in a volume of water as depicted in Tables 1-4, whereby the volume of the solution to be administered is calculated according to the prescribed total daily dose.
Danish: Afhængigt af dosis (i mg/kg/dag) skal det passende antal tabletter opløses i et volumen af vand som angivet i tabel 1-4, hvor volumenet af opløsningen, der skal administreres, beregnes i henhold til den ordinerede samlede daglige dosis.
English: BH4 deficiency
Danish: BH4-mangel
English: • Place the prescribed number of tablets in the medicine cup.
Danish: • Kom det ordinerede antal tabletter i medicinbægret.
English: For PKU patients, the total daily dose is taken once a day at the same time each day, preferably in the morning.
Danish: For PKU-patienter skal den samlede daglige dosis tages én gang om dagen på samme tidspunkt hver dag, gerne om morgenen.
English: The most commonly reported adverse reactions are “amino acid level decreased” (hypophenylalaninaemia), vomiting and rhinitis.
Danish: De hyppigst rapporterede bivirkninger er ”nedsat aminosyreniveau” (hypofenylalaninæmi), opkastning og rhinitis.
English: What you need to know before you take Kuvan Do not take Kuvan
Danish: Det skal du vide, før du begynder at tage Kuvan Tag ikke Kuvan
English: BH4 is required by the body to use an amino acid called phenylalanine in order to build another amino acid called tyrosine.
Danish: BH4 er nødvendigt, for at kroppen kan udnytte en aminosyre kaldet fenylalanin til at omdanne dette stof til en anden aminosyre kaldet tyrosin.
English: A satisfactory response is defined as a ≥30 percent reduction in blood phenylalanine levels or attainment of the therapeutic blood phenylalanine goals defined for an individual patient by the treating physician.
Danish: Et tilfredsstillende behandlingsrespons er defineret som en ≥ 30 % reduktion af fenylalanin i blodet eller opnåelse af et fenylalaninniveau, der af den behandlende læge er sat som mål for den enkelte patient.
English: The safety, efficacy and population pharmacokinetics of Kuvan were studied in a multicentre, openlabel, randomised, controlled study in children <4 years old with a confirmed diagnosis of PKU.
Danish: Kuvans sikkerhed, virkning og populationsfarmakokinetik blev undersøgt i et åbent, randomiseret kontrolleret multicenterstudie hos børn <4 år med en bekræftet PKU-diagnose.
English: Take Kuvan as a single daily dose with a meal to increase the absorption, and at the same time each day, preferably in the morning.
Danish: Indtag Kuvan sammen med et måltid for at øge optagelsen og på samme tidspunkt hver dag, gerne om morgenen.
English: Not known side effects (frequency cannot be estimated from the available data) Gastritis (inflammation of the lining of the stomach), oesophagitis (inflammation of the lining of the gullet).
Danish: Ikke kendte bivirkninger (kan ikke estimeres ud fra forhåndenværende data) Gastritis (betændelse i mavens slimhinde), øsofagitis (betændelse i spiserørets slimhinde).
English: EU/1/08/481/004 100 mg sachet EU/1/08/481/005 500 mg sachet
Danish: EU/1/08/481/004 100 mg brev EU/1/08/481/005 500 mg brev
English: In healthy subjects, administration of a single dose of Kuvan at the maximum therapeutic dose of 20 mg/kg had no effect on the pharmacokinetics of a single dose of digoxin (P-gp substrate) administered concomitantly.
Danish: Administration af en enkelt dosis Kuvan i maksimal terapeutisk dosis på 20 mg/kg til raske forsøgspersoner havde ingen indvirkning på farmakokinetikken af en enkelt dosis digoxin (P-gpsubstrat), som blev administreret samtidig.
English: The most commonly reported adverse reactions are headache and rhinorrhoea.
Danish: De hyppigst rapporterede bivirkninger var hovedpine og rinoré.
English: In the clinical trial extension period, patients maintained dietary phenylalanine tolerance while on Kuvan treatment in conjunction with a Phe-restricted diet, demonstrating sustained benefit over 3.5 years.
Danish: I det kliniske forsøgs forlængelsesperiode opretholdt patienterne den tolererede fenylalaninindtagelse via kosten, mens de fik behandling med Kuvan sammen med en fenylalaninbegrænset diæt, og udviste dermed en vedvarende fordel over 3,5 år.
English: No tumorigenic activity was observed in an oral carcinogenicity study in mice at doses of up to 250 mg/kg/day (12.5 to 50 times the human therapeutic dose range).
Danish: Det blev ikke observeret tumorigen aktivitet i et karcinogenicitetsstudie hos mus ved en oral dosis på op til 250 mg/kg/dag (12,5 til 50 gange det terapeutiske dosisinterval hos mennesker).
English: Sapropterin is a synthetic version of the naturally occurring 6R-BH4, which is a cofactor of the hydroxylases for phenylalanine, tyrosine and tryptophan.
Danish: Sapropterin er en syntetisk udgave af det naturligt forekommende 6R-BH4, som er en cofaktor for fenylalanin-, tyrosin- og tryptophan hydroxylase.
English: Discard unused solution within 20 minutes for tablet solution.
Danish: Kassér ubrugt opløsning inden for 20 minutter til tabletopløsning.
English: Divide the total daily dose into 2 or 3 doses, taken over the day.
Danish: Del den samlede daglige dosis i 2 eller 3 doser, der skal administreres i løbet af dagen.
English: Headache and dizziness have been reported after the administration of sapropterin dihydrochloride above the recommended maximum dose of 20 mg/kg/day.
Danish: Hovedpine og svimmelhed er indberettet efter indgift af sapropterindihydrochlorid over den anbefalede maksimumdosis på 20 mg/kg/dag.
English: Caution must be exercised when prescribing to pregnant women.
Danish: Der bør udvises forsigtighed ved ordination til gravide.
English: Non-clinical data reveal no special hazard for humans based on conventional studies of safety pharmacology (CNS, respiratory, cardiovascular, genitourinary), and toxicity to reproduction.
Danish: Prækliniske data viser ingen speciel risiko for mennesker vurderet ud fra konventionelle studier af sikkerhedsfarmakologi (CNS, respiratorisk, kardiovaskulær, genitourinær) og reproduktionstoksicitet.
English: 30 soluble tablets 120 soluble tablets 240 soluble tablets
Danish: 30 opløselige tabletter 120 opløselige tabletter 240 opløselige tabletter
English: Kuvan 100 mg soluble tablets are off-white to light yellow with “177” imprinted on one face.
Danish: Kuvan 100 mg opløselige tabletter er offwhite til lysegule og præget med ”177” på en af fladerne.
English: Limited studies have been conducted in patients under 4 years of age with BH4 deficiency using another formulation of the same active substance (sapropterin) or an un-registered preparation of BH4.
Danish: Få studier er udført med patienter under 4 år med BH4-mangel med anvendelse af en anden sapropterinformulering med samme aktive substans eller med et andet, ikke-godkendt BH4-produkt.
English: No increase in total biopterin concentrations in either foetuses or milk was observed in rats after oral administration of 10 mg/kg sapropterin dihydrochloride.
Danish: Der sås ingen øgning af total biopterinkoncentrationer hverken i fostre eller i mælk hos rotter efter peroral indgift af 10 mg/kg sapropterindihydrochlorid.
English: There was a significant difference in dietary phenylalanine tolerance in the sapropterin treatment group as compared to the placebo group.
Danish: Der var en signifikant forskel i tolerancen over for fenylalaninindtaget via kosten i gruppen behandlet med sapropterindihydrochlorid sammenlignet ned placebogruppen.
English: Kuvan is provided as 100 mg tablets.
Danish: Kuvan findes som tabletter à 100 mg.
English: Your doctor will monitor the restriction of dietary phenylalanine intake prior and during pregnancy.
Danish: Din læge vil overvåge begrænsningen af indholdet af fenylalanin i kosten før og under graviditet.
English: Your doctor should continue to monitor your blood phenylalanine levels often during your treatment with Kuvan, to make sure that your blood phenylalanine levels are not too high or too low.
Danish: Din læge bør fortsætte med at overvåge fenylalaninniveauet i blodet hyppigt under din behandling med Kuvan for at sikre, at fenylalaninniveauet i blodet ikke er for højt eller for lavt.
English: If an unsatisfactory reduction in blood phenylalanine levels is observed, then the dose can be increased weekly to a maximum of 20 mg/kg/day, with continued weekly monitoring of blood phenylalanine levels over a one month period.
Danish: Hvis fenylalanin i blodet ikke reduceres tilstrækkeligt, kan dosis øges en gang om ugen til maksimalt 20 mg/kg/dag med fortsat ugentligt monitorering af fenylalanin i blodet gennem en periode på en måned.
English: It is available in bottles with child-resistant closure of 30, 120 or 240 soluble tablets.
Danish: Kuvan er tilgængelig i tabletbeholdere med børnesikret lukke og med 30, 120 eller 240 opløselige tabletter.
English: Give your child the prescribed amount of solution within 30 minutes after dissolving.
Danish: Giv dit barn den ordinerede mængde opløsning inden for 30 minutter efter tilberedningen.
English: These devices are supplied to the specialized paediatric centers for inborn errors of metabolism to be provided to the caregivers of the patients.
Danish: Disse remedier leveres til de specialiserede pædiatriske centre for medfødte metaboliske forstyrrelser og skal gives til patienternes omsorgspersoner.
English: Stir with the small spoon or clean utensil until the powder dissolves.
Danish: Omrør med en lille ske eller andet rent bestik, indtil pulveret er opløst.
English: You must continue your diet treatment as recommended by your doctor.
Danish: Du skal altid fortsætte din diæt, som lægen har anvist.
English: Store the oral syringe and the medicine cup for next use.
Danish: Opbevar den orale sprøjte og medicinbægret til den næste anvendelse.
English: Following intravenous administration in rats, sapropterin dihydrochloride is mainly excreted in the urine.
Danish: Efter intravenøs indgift i rotter af sapropterindihydrochlorid sker udskillelsen overvejende i urinen.
English: This medicine contains less than 1 mmol sodium (23 mg) per tablet, that is to say essentially ‘sodiumfree'.
Danish: Dette lægemiddel indeholder mindre end 1 mmol (23 mg) natrium pr. tablet, det vil sige, at det i det væsentlige er natriumfrit.