FrancophonIA/ELRC3.0 huggingface dataset:
English: An open-label, long-term study (Study 5) was conducted to assess ammonia control in paediatric patients with UCD.
Danish: Et åbent langtidsstudie (studie 5) blev udført for at vurdere ammoniakkontrol hos pædiatriske patienter med UCD.
English: No developmental abnormalities were observed in rats through day 92 postpartum following oral administration in pregnant rats, during organogenesis and lactation.
Danish: Der blev ikke set udviklingsforstyrrelser hos rotter til og med dag 92 postpartum efter oral administration til drægtige rotter under organogenese og amning.
English: Store bottle and oral syringe in a clean, dry place between doses.
Danish: Opbevar flasken og sprøjten på et rent, tørt sted mellem doserne.
English: • In urea cycle disorders, the body is not able to produce enough liver enzymes to remove the extra nitrogen.
Danish: • Ved urinstofcyklus-defekter er kroppen ikke i stand til at producere nok leverenzymer til at fjerne det ekstra kvælstof.
English: In particular, tell your doctor or pharmacist if you are taking any of the following medicines, which may be less effective when used with glycerol phenylbutyrate.
Danish: Fortæl især lægen eller apotekspersonalet, hvis du tager nogle af de følgende lægemidler, som kan være mindre effektive, når de bruges samtidig med glycerolphenylbutyrat.
English: It is unknown whether glycerol phenylbutyrate or its metabolites are excreted in human milk.
Danish: Det er ukendt, om glycerolphenylbutyrat eller dets metabolitter udskilles i human mælk.
English: You will need to take this medicine and follow a special low protein diet throughout your life.
Danish: Du skal tage lægemidlet og følge en særlig proteinfattig kostplan resten af livet.
English: Metrorrhagia Uncommon Amenorrhoea, irregular menstruation
Danish: Almindelig Metroragi Ikke almindelig Amenorré, uregelmæssig menstruation
English: Once the bottle is open, you must use your medicine within 14 days of opening.
Danish: Når en flaske er åbnet, skal medicinen anvendes inden for 14 dage.
English: In infants and young children (generally below 6 years of age) where obtaining fasting ammonia is problematic due to frequent feedings, the first ammonia of the morning should be kept below the ULN.
Danish: For spædbørn og små børn (generelt under 6 år), hvor det kan være vanskeligt at bestemme et fastende ammoniakniveau på grund af hyppige måltider/amning, skal det først målte ammoniakniveau om morgenen holdes under ULN.
English: Measuring the dose
Danish: Afmåling af dosen
English: This may be associated with increased risk of medicinal product interactions with lipase inhibitors and with lipase contained in pancreatic enzyme replacement therapies.
Danish: Dette kan være forbundet med øget risiko for lægemiddelinteraktioner med lipase-hæmmere og med lipase i pancreasenzympræparater til substitutionsterapi.
English: Glycerol phenylbutyrate given at the maximum recommended dose contains less than 0.5 g of glycerol per dose.
Danish: Glycerolphenylbutyrat, givet i den maksimale anbefalede dosis, indeholder mindre end 0,5 g glycerol pr. dosis.
English: Each bottle contains 25 ml of liquid.
Danish: Hver flaske indeholder 25 ml oral væske.
English: Tell a doctor straight away if: • you are sick (vomit), which is a common side effect, more than once and this does not stop.
Danish: Tal straks med en læge, hvis: • du kaster op mere end én gang, og det ikke stopper.
English: Although two patients had day 7 ammonia values of 150 micromol/L and 111 micromol/L respectively, neither had associated signs and symptoms of hyperammonemia.
Danish: Selvom to patienter havde ammoniakværdier på dag 7 på hhv. 150 mikromol/l og 111 mikromol/l, havde ingen af dem associerede tegn og symptomer på hyperammoniæmi.
English: N-acetylglutamate synthase (NAGS) and CITRIN (citrullinaemia type 2) deficiency The safety and efficacy of RAVICTI for the treatment of patients with N-acetylglutamate synthase (NAGS) and CITRIN (citrullinaemia type 2) deficiency have not been established.
Danish: N-acetylglutamat-syntetase- (NAGS) og CITRIN (citrullinæmi type 2-) defekt RAVICTIs sikkerhed og virkning til behandling af patienter med N-acetylglutamat-syntetase (NAGS) og CITRIN (citrullinæmi type 2-) defekt er ikke klarlagt.
English: Anaemia, Thrombocytosis
Danish: Anæmi, trombocytose
English: Venous ammonia levels were monitored at a minimum of every 6 months.
Danish: Det venøse ammoniakniveau blev målt mindst hver 6. måned.
English: The recommended daily dose schedule of 8.5 ml/m2/day - 11.2 mL/m2/day over a period of up to 24 hours for patients stabilised with no further hyperammonaemia is as follows:
Danish: Det anbefalede daglige dosisregime på 8,5 ml/m2/dag-11,2 ml/m2/dag over en periode på op til 24 timer for stabiliserede patienter uden yderligere hyperammoniæmi er som følger:
English: Clear, colourless to pale yellow liquid.
Danish: Klar, farveløs til svagt gul væske.
English: In 16 paediatric UCD patients aged less than 2 months, PAA clearance was 3.8 L/h.
Danish: Hos 16 pædiatriske UCD-patienter under 2 måneder var PAA-clearance 3,8 l/t.
English: Each gram of U-PAGN excreted over 24 hours covers waste nitrogen generated from 1.4 grams of dietary protein.
Danish: Hvert gram U-PAGN, der bliver udskilt i løbet af et døgn, redegør for den mængde restkvælstof, der dannes af 1,4 gram protein i kosten.
English: This process allows phenylbutyrate to be absorbed into the circulation.
Danish: Denne proces bevirker, at phenylbutyrat kan absorberes til kredsløbet.
English: Therefore, therapeutic effects and/or metabolite levels of medicinal products, including some oral contraceptives, that are substrates for this enzyme may be reduced and their full effects cannot be guaranteed, following coadministration with glycerol phenylbutyrate.
Danish: Den terapeutiske effekt og/eller metabolitniveauet af lægemidler, som er substrater for dette enzym, herunder visse orale kontraceptiva, kan derfor være reduceret, og der er ikke garanti for fuld effekt ved administration sammen med glycerolphenylbutyrat.
English: Venous ammonia levels were monitored monthly.
Danish: Det venøse ammoniakniveau blev målt månedligt.
English: It is a triglyceride containing 3 molecules of PBA linked to a glycerol backbone.
Danish: Det er et triglycerid, som indeholder 3 molekyler PBA bundet til et glycerol-skelet.
English: Do not use RAVICTI after the expiry date which is stated on the carton and the bottle label after the letters “EXP”.
Danish: Brug ikke RAVICTI efter den udløbsdato, der står på kartonen og flaskens etikette efter EXP. Udløbsdatoen er den sidste dag i den nævnte måned.
English: If RAVICTI is added to water the liquid will become cloudy and you may not get the full dose.
Danish: Hvis RAVICTI blandes med vand, vil væsken blive uklar, og du vil muligvis ikke få hele dosen.
English: After you take your full dose, have a drink of water to make sure no medicine is left in your mouth, or flush the gastrostomy or nasogastric tube with 10 ml of water using a new oral syringe.
Danish: Efter du har taget din fulde dosis, skal du drikke vand for at sikre, at der ikke er medicin tilbage i munden, eller du skal skylle sonden med 10 ml vand ved brug af en ny sprøjte.
English: If RAVICTI gets in contact with water, the liquid will become cloudy in appearance.
Danish: Hvis RAVICTI kommer i kontakt med vand vil væsken blive uklar.
English: Valproic Acid and Haloperidol
Danish: Valproinsyre og haloperidol
English: In general, dose selection for an elderly patient should be cautious, usually starting at the low end of the dosing range, reflecting the greater frequency of decreased hepatic, renal, or cardiac function and of concomitant disease or other medicinal product therapy.
Danish: Generelt skal der udvises forsigtighed ved valg af dosis til en ældre patient, og dosis skal sædvanligvis begynde i den lave ende af dosisintervallet for at tage højde for den højere hyppighed af nedsat lever-, nyre- og hjertefunktion såvel som af samtidig sygdom eller behandling med andre lægemidler.
English: Fill the oral syringe by pulling the plunger back until the syringe is filled with the amount of glycerol phenylbutyrate liquid that your doctor has told you to take.
Danish: Fyld sprøjten ved at trække stemplet tilbage, indtil sprøjten er fyldt med den mængde glycerolphenylbutyrat, som din læge har sagt, at du skal tage.
English: Other medicinal products such as corticosteroids, valproic acid, haloperidol and probenecid may have the potential to affect ammonia levels, see section 4.4.
Danish: Andre lægemidler, såsom kortikosteroider, valproinsyre, haloperidol og probenecid kan potentielt påvirke ammoniakniveauet, se pkt.
English: On a molar basis, PAGN, like urea, contains 2 moles of nitrogen and provides an alternate vehicle for waste nitrogen excretion.
Danish: På molær basis indeholder PAGN, ligesom urinstof, 2 mol kvælstof og er således et alternativt middel til udskillelse af restkvælstof.
English: The dose normalized mean AUC0-23h for PAA was 108% higher in females than in males.
Danish: Dosis-normaliseret gennemsnitlig AUC0-23t for PAA var 108 % højere hos kvinder end hos mænd.
English: There are limited data regarding the use of glycerol phenylbutyrate in pregnant women.
Danish: Der er utilstrækkelige data fra anvendelse af glycerolphenylbutyrat til gravide kvinder.
English: The plasma PAA to PAGN (both measured in mcg/ml) ratio has been observed to be generally less than 1 in patients without PAA accumulation.
Danish: PAA:PAGN i plasma (begge målt i mikrogram/ml) er observeret til generelt at være under 1 hos patienter uden PAA-akkumulation.
English: However, the formation of mono- or bis-esters was not studied in humans.
Danish: Dannelse af monoester- eller diester-metabolitter blev dog ikke undersøgt hos mennesker.
English: Multi-centre prospective non-interventional registry in patients with urea cycle disorders on treatment with glycerol phenylbutyrate to characterise patients` demographics, and to document long-term safety and clinical outcomes
Danish: Et prospektivt ikke-interventions-multicenterregister for patienter med urinstofcyklus-defekter, der behandles med glycerolphenylbutyrat, for at beskrive patienternes demografiske karakteristika og dokumentere sikkerheden og de kliniske resultater efter langvarig behandling
English: Glycerol phenylbutyrate This medicine is subject to additional monitoring.
Danish: Glycerolphenylbutyrat Dette lægemiddel er underlagt supplerende overvågning.
English: No precipitant trigger or missing dose was reported for the other 2 subjects (1 age < 1 month, 1 age 1-2- months).
Danish: Der blev ikke rapporteret nogen præcipitat-trigger eller manglende dosis for de andre 2 forsøgsdeltagere (1 forsøgsdeltager i alderen < 1 måned, 1 forsøgsdeltager i alderen 1-2 måneder).
English: • lipase in pancreatic replacement therapies
Danish: • pancreaslipase til substitutionsterapi
English: The recommended total daily dose of RAVICTI is based on body surface area and ranges from 4.5 ml/m2/day to 11.2 ml/m2/day [5.3 g/m2/day to 12.4 g/m2/day) and should take into account the following:
Danish: Den anbefalede samlede daglige dosis RAVICTI er baseret på kropsoverfladeareal og er fra 4,5 ml/m2/dag til 11,2 ml/m2/dag (5,3 g/m2/dag til 12,4 g/m2/dag), og følgende skal tages i betragtning:
English: After the first opening of the bottle, the medicinal product must be used within 14 days and the bottle and its contents discarded, even if not empty.
Danish: Efter anbrud af flasken skal lægemidlet anvendes inden for 14 dage, og flasken med indhold kasseres, selv om den ikke er tom.
English: The liquid is filled into a 25 ml clear glass bottle and capped with a plastic, child-resistant cap.
Danish: Den orale væske er fyldt i en 25 ml klar glasflaske med et børnesikkert plastiklåg.
English: • You should have the medicine with the reclosable bottle cap adapter along with an oral syringe to administer the correct amount of glycerol phenylbutyrate.
Danish: • Du skal bruge medicinen med den lukbare adapter sammen med en oral sprøjte for at administrere den rigtige mængde glycerolphenylbutyrat.
English: RAVICTI should not be used during pregnancy and in women of childbearing potential not using contraception unless the clinical condition of the woman requires treatment with glycerol phenylbutyrate, see section 4.6.
Danish: RAVICTI bør ikke anvendes under graviditet og til kvinder i den fertile alder, som ikke anvender sikker kontraception, medmindre kvindens kliniske tilstand kræver behandling med glycerolphenylbutyrat, se pkt.
English: Dose adjustment was made to 3 subjects age < 1 month.
Danish: Dosis blev justeret hos 3 forsøgsdeltagere < 1 måned.
English: Population pharmacokinetic modelling and dosing simulations suggest body surface area is the most significant covariate explaining the variability of PAA clearance.
Danish: Populationsfarmakokinetisk modellering og dosissimuleringer antyder, at kropsoverfladearealet er den mest signifikante kovariat til at forklare variabilitet af PAA-clearance.
English: In this case, glycerol phenylbutyrate will not stop the level of ammonia in your blood from becoming seriously high.
Danish: I disse tilfælde vil glycerolphenylbutyrat ikke hindre, at niveauet af ammoniak i blodet bliver alvorligt forhøjet.
English: Three patients reported a total of 7 hyperammonemic crises defined as having signs and symptoms consistent with hyperammonemia (such as frequent vomiting, nausea, headache, lethargy, irritability, combativeness, and/or somnolence) associated with high venous ammonia levels and requiring medical intervention.
Danish: Tre patienter rapporterede i alt 7 hyperammoniæmiske kriser defineret som tegn og symptomer overensstemmende med hyperammoniæmi (såsom hyppig opkastning, kvalme, hovedpine, letargi, irritabilitet, stridbarhed og/eller somnolens) forbundet med høje venøse ammoniakniveauer, som krævede medicinsk intervention.
English: One oral syringe should be used each day.
Danish: Der skal bruges en oral sprøjte per dag.
English: Contents of the pack and other information What RAVICTI contains
Danish: Pakningsstørrelser og yderligere oplysninger RAVICTI indeholder:
English: This corresponds to a density of 1.1 g/ml.
Danish: Det svarer til en massefylde på 1,1 g/ml.
English: Package leaflet: Information for the patient RAVICTI 1.1 g/ml oral liquid
Danish: Indlægsseddel: Information til patienten RAVICTI 1,1 g/ml oral væske
English: Although these have not been seen in clinical trials involving UCD patients, high PAA levels should be suspected in patients (particularly in children <2months) with unexplained somnolence, confusion, nausea and lethargy who have normal or low ammonia.
Danish: Selvom dette ikke er set i kliniske studier med UCDpatienter, skal et højt PAA-niveau mistænkes hos patienter (især hos børn < 2 måneder) med uforklarlig døsighed, forvirring, kvalme og apati, som har et normalt til lavt ammoniakniveau.
English: Assessment of adverse reactions was based on exposure in 114 UCD patients (65 adults and 49 children between the ages of 2 months and 17 years) with deficiencies in CPS, OTC, ASS, ASL, ARG, or HHH across 4 short term and 3 long term clinical studies, in which 90 patients completed 12 months duration (median exposure = 51 weeks).
Danish: Vurderingen af bivirkninger blev baseret på eksponering hos 114 UCD-patienter (65 voksne og 49 børn i alderen fra 2 måneder til 17 år) med defekter i CPS, OTC, ASS, ASL, ARG eller HHH på tværs af 4 kortvarige og 3 langvarige kliniske studier, i hvilke 90 patienter fuldførte 12 måneders deltagelse (gennemsnitlig eksponering = 51 uger).
English: Glycerol phenylbutyrate was not tumourigenic at doses up to 1000 mg/kg/day in a 26-week mouse study.
Danish: Glycerolphenylbutyrat var ikke tumorfremkaldende ved doser på op til 1000 mg/kg/dag i et 24 ugers studie med mus.
English: • tests show increased liver enzyme levels, imbalance of salts in the blood, low levels of a type of white blood cell (‘lymphocytes') or low levels of vitamin D
Danish: • tests, der viser forhøjet niveau af leverenzymer, forstyrrelser i blodets saltbalance, lavt niveau af en bestemt slags hvide blodlegemer (lymfocytter) eller lavt niveau af D-vitamin
English: In 7 paediatric patients aged 2 months to under 2 years of age who received RAVICTI for up to 12 months, the concentrations of PAA, PBA, and PAGN did not increase over the treatment period and the overall median PAA, PBA, and PAGN concentrations in these patients were similar to those observed in older paediatric age groups.
Danish: Hos 7 pædiatriske patienter i alderen 2 måneder til under 2 år, som fik RAVICTI i op til 12 måneder, steg koncentrationerne af PAA, PBA og PAGN ikke i behandlingsperioden, og de overordnede gennemsnitlige koncentrationer af PAA, PBA og PAGN hos disse patienter svarede til dem, som blev set hos de ældre pædiatriske aldersgrupper.
English: Further adjustment may be based on monitoring of plasma ammonia, glutamine, U-PAGN and/or plasma PAA and PAGN as well as the ratio of plasma PAA to PAGN (see section 4.2).
Danish: Yderligere justering kan være baseret på monitorering af ammoniak, glutamin, UPAGN og/eller PAA og PAGN i plasma såvel som plasma-PAA:PAGN-ratioen (se pkt.
English: Neurotoxicity
Danish: Neurotoksicitet
English: Do not add and stir the medicinal product into a large volume of other liquid, as glycerol phenylbutyrate is heavier than water and this may result in incomplete administration.
Danish: Bland ikke lægemidlet med en større mængde anden væske, da glycerolphenylbutyrat er tungere end vand, og det kan medføre ufuldstændig administration.
English: The following side effects have been observed in a clinical study including 16 patients less than 2 months of age:
Danish: Følgende bivirkninger er blevet observeret i et klinisk studie, der omfattede 16 patienter under 2 måneder:
English: RAVICTI should be used with caution in patients with severe renal impairment.
Danish: RAVICTI skal anvendes med forsigtighed hos patienter med svært nedsat nyrefunktion.
English: RAVICTI contains the active substance ‘glycerol phenylbutyrate' which is used to treat six known ‘urea cycle disorders' (UCDs) in adults and children.
Danish: RAVICTI indeholder det aktive stof glycerolphenylbutyrat, som bruges til at behandle seks kendte fejl i urinstofcyklussen (UCD'er) hos voksne og børn.
English: • tests show higher triglycerides, low density lipo-protein or white cells in your blood
Danish: • tests viser højere triglycerider, low density lipoprotein eller hvide blodlegemer i blodet
English: In patients with a PAA to PAGN ratio exceeding 2.5, a further increase in glycerol phenylbutyrate dose may not increase PAGN formation, even if plasma PAA concentrations are increased, due to saturation of the conjugation reaction.
Danish: Hos patienter med en PAA:PAGN-ratio over 2,5 vil en yderligere forhøjelse af glycerolphenylbutyrat-dosis muligvis ikke øge PAGN-dannelse, selvom PAA-plasmakoncentrationen stiger, på grund af mætning af konjugeringsreaktionen.
English: • haloperidol - used to treat some mental health problems
Danish: • haloperidol - anvendes til at behandle visse psykiske forstyrrelser
English: Clear, Type III glass, bottle with a high density polyethylene (HDPE) child-resistant closure.
Danish: Klar, type III-glasflaske med børnesikkert låg af polyethylen med høj densitet (HDPE).
English: Hyperammonemic crises were precipitated by vomiting, upper respiratory tract infection, gastroenteritis, decreased caloric intake or had no identified precipitating event (3 events).
Danish: Hyperammoniæmiske kriser blev fremskyndet af opkastning, infektion i de øvre luftveje, gastroenteritis, nedsat kalorieindtag, eller ingen identificerbar fremskyndende hændelse (3 hændelser).
English: Hyperammonaemia was reported in 5 (50%) subjects age < 1 month (all serious but non-fatal) and 1 subject (16.7%) age 1-2 months (non-serious), which is consistent with more severe disease types diagnosed in the neonatal period.
Danish: Hyperammoniæmi blev rapporteret hos 5 (50 %) forsøgsdeltagere < 1 måned (alle alvorlige, men ikke letale) og hos 1 forsøgsdeltager (16,7 %) på 1-2 måneder (ikke alvorlig), hvilket er overensstemmende med mere alvorlige sygdomstyper diagnosticeret i den neonatale periode.
English: Supplemental amino acid formulations may be necessary to maintain essential amino acids and branched chain amino acids within normal range.
Danish: Supplerende aminosyreformuleringer kan være nødvendige for at opretholde essentielle aminosyrer og forgrenede aminosyrer inden for normalområdet.
English: • if you are allergic to glycerol phenylbutyrate
Danish: • hvis du er allergisk over for glycerolphenylbutyrat
English: This is because it can pass into breast milk and may harm your baby.
Danish: Det skyldes, at RAVICTI kan udskilles i mælken og muligvis være skadelig for dit barn.
English: ANNEX III LABELING AND PACKAGE LEAFLET
Danish: BILAG III ETIKETTERING OG INDLÆGSSEDDEL
English: Glycerol phenylbutyrate has been tested in a range of in vitro and in vivo genotoxicity studies, and shown no genotoxic activity.
Danish: Glycerolphenylbutyrat er blevet testet i en række in vitro og in vivo genotoksicitetsstudier og udviste ingen genotoksisk aktivitet.
English: One additional patient developed hyperammonemia on day 3 of dosing and experienced surgical complications (bowel perforation and peritonitis) following jejunal tube placement on day 4.
Danish: En yderligere patient udviklede hyperammoniæmi på 3. doseringsdag og oplevede kirurgiske komplikationer (tarmperforation og peritonitis) efterfulgt af anlæggelse af jejunal sonde på dag 4.
English: What you need to know before you take RAVICTI Do not take RAVICTI:
Danish: Det skal du vide, før du begynder at tage RAVICTI Tag ikke RAVICTI:
English: Patients had to have no clinical evidence of hyperammonaemia at enrolment and were not allowed to receive medicinal products known to increase ammonia levels (e.g., valproate), increase protein catabolism (e.g., corticosteroids), or significantly affect renal clearance (e.g., probenecid).
Danish: Patienterne måtte ikke have kliniske tegn på hyperammoniæmi ved inklusion og måtte ikke få lægemidler, som vides at øge ammoniakniveauet (f.eks. valproat), øge proteinkatabolismen (f.eks. kortikosteroider) eller signifikant påvirke den renale clearance (f.eks. probenecid).
English: • if you have problems with your kidneys or liver - this is because glycerol phenylbutyrate is removed from your body through the kidneys and liver
Danish: • hvis du har problemer med dine nyrer eller lever - dette skyldes, at glycerolphenylbutyrat udskilles af kroppen gennem nyrerne og leveren
English: Further, glycerol phenylbutyrate was hydrolysed in vitro by esterases in human plasma.
Danish: Endvidere blev glycerolphenylbutyrat hydrolyseret in vitro af esteraser i human plasma.
English: RAVICTI may be added to a small amount of apple sauce, ketchup, or squash puree and should be used within 2 hours when stored at room temperature (25° C).
Danish: RAVICTI kan blandes med en lille smule æblemos, ketchup eller græskarmos, og blandingen skal anvendes inden for 2 timer ved opbevaring ved stuetemperatur (25° C).
English: PAA clearance was 7.1 L/h, 10.9 L/h, 16.4 L/h, and 24.4 L/h, respectively, for UCD patients ages ≤ 2, 3 to 5, 6 to 11, and 12 to 17 years.
Danish: PAA-clearance var hhv. 7,1 l/t, 10,9 l/t, 16,4 l/t og 24,4 l/t hos UCD-patienter i alderen ≤ 2 år, 3 til 5 år, 6 til 11 år og 12 til 17 år.
English: In these in vitro studies, a complete disappearance of glycerol phenylbutyrate did not produce molar equivalent PBA, suggesting the formation of mono- or bis-ester metabolites.
Danish: I disse in vitro studier medførte en fuldstændig forsvinden af glycerolphenylbutyrat ikke dannelse af en ækvivalent mængde PBA (på molær basis), hvilket antyder dannelse af monoester- eller diestermetabolitter.
English: If you stop taking RAVICTI
Danish: Hvis du holder op med at tage RAVICTI
English: However, for patients who cannot swallow liquids, a nasogastric tube or gastrostomy tube may be used to administer RAVICTI as follows:
Danish: For patienter, som ikke kan synke væsker, kan en nasogastrisk eller gastrostomisonde dog anvendes til at administrere RAVICTI på følgende måde: