FrancophonIA/ELRC3.0 huggingface dataset:
English: If you need laboratory tests, it is important to remind your doctor that you are taking PHEBURANE, since sodium phenylbutyrate may interfere with certain laboratory test results (such as blood electrolytes or protein, or liver function tests)
Danish: Hvis du skal have taget blodprøver, er det vigtigt, at du fortæller lægen, at du tager PHEBURANE, da natriumphenylbutyrat kan påvirke resultaterne af visse blodprøver (såsom blodets indhold af elektrolytter og protein samt leverfunktionsprøver).
English: This should be taken into account if you have diabetes mellitus.
Danish: Dette skal tages i betragtning, hvis du har sukkersyge.
English: In clinical trials with sodium phenylbutyrate, 56 % of the patients experienced at least one adverse event and 78 % of these adverse events were considered as not related to sodium phenylbutyrate.
Danish: I kliniske forsøg med natriumphenylbutyrat oplevede 56 % af patienterne mindst én uønsket hændelse, og det blev antaget, at 78 % af disse uønskede hændelser ikke var relateret til natriumphenylbutyrat.
English: Micronucleus test results indicate that sodium phenylbutyrate was considered not to have produced any clastogenic effect in rats treated at toxic or non-toxic dose levels (examined 24 and 48 hours after a single oral administration of 878 to 2800 mg/kg).
Danish: Resultaterne af mikronukleustesten viser, at natriumphenylbutyrat ikke fremkaldte nogen klastogen effekt hos rotter, der blev behandlet med toksiske eller non-toksiske doser (undersøgt 24 og 48 timer efter en enkelt oral administration af 878 til 2 800 mg/kg).
English: • Lines on the spoon indicate the amount (in gram of sodium phenylbutyrate).
Danish: • Linjerne på skeen angiver mængden (i gram natriumphenylbutyrat).
English: When high doses of phenylacetate (190 - 474 mg/kg) were given subcutaneously to rat pups, decreased proliferation and increased loss of neurons were observed, as well as a reduction in CNS
Danish: Når der blev givet høje doser af phenylacetat (190 - 474 mg/kg) subkutant til rotteunger, blev der observeret en nedsat proliferation og et øget tab af neuroner såvel som en reduktion af CNS myelin.
English: Patients who have taken very high doses of sodium phenylbutyrate experienced:
Danish: Patienter, der har taget meget store doser natriumphenylbutyrat, oplever:
English: It is also indicated in patients with late-onset disease (partial enzyme deficiencies, presenting after the first month of life) who have a history of hyperammonaemic encephalopathy.
Danish: Det er også indiceret til patienter med sent indsættende sygdom (partiel enzymmangel, som viser sig efter den første måned post partum), som har tidligere tilfælde af hyperammonæmisk encefalopati.
English: Less frequent manifestations were confusion, headache, dysgeusia, hypoacusis, disorientation, impaired memory and exacerbation of a pre-existing neuropathy.
Danish: Mindre hyppige manifestationer var konfusion, hovedpine, smagsforstyrrelser, hørenedsættelse, desorientering, svækket hukommelse og forværring af allerede eksisterende neuropati.
English: Talk to your doctor or pharmacist before taking PHEBURANE if you:
Danish: Kontakt lægen eller apoteket, før du tager PHEBURANE:
English: Tell your doctor or pharmacist if you are taking, have recently taken or might take any other medicines.
Danish: Fortæl det til lægen eller på apoteket, hvis du tager anden medicin eller for nylig har taget anden medicin.
English: These symptoms are consistent with the accumulation of phenylacetate, which showed dose-limiting neurotoxicity when administered intravenously at doses up to 400 mg/kg/day.
Danish: Disse symptomer er konsistente med akkumulering af phenylacetat, som viste dosisbegrænsende neurotoksicitet, når det blev administreret intravenøst ved doser på op til 400 mg/kg/dag.
English: • This medicinal product contains 768 mg sucrose per gram.
Danish: • Lægemidlet indeholder 768 mg saccharose pr. gram.
English: • PHEBURANE is not recommended for the management of acute hyperammonaemia, which is a medical emergency.
Danish: • PHEBURANE anbefales ikke til behandling af akut hyperammonæmi, som er en akut tilstand.
English: Description of selected adverse reactions
Danish: Beskrivelse af udvalgte uønskede hændelser
English: HDPE bottle, child-resistant closure with desiccant, containing 174 g of granules.
Danish: HDPE-flaske med børnesikker lukning og tørremiddel, indeholdende 174 g granulat.
English: Do not use PHEBURANE if you are breast-feeding, because this medicine can pass into the breast milk and may harm your baby.
Danish: Tag ikke PHEBURANE, hvis du ammer, da denne medicin kan passere over i modermælken og skade dit barn.
English: • haloperidol (used in certain psychotic disorders),
Danish: • haloperidol (der anvendes til behandling af visse psykotiske lidelser)
English: Results indicate that sodium phenylbutyrate did not induce any mutagenic effects in the Ames test with or without metabolic activation.
Danish: Resultaterne indikerer, at natriumphenylbutyrat ikke inducerer nogen mutagen effekt ved Ames test med eller uden metabolisk aktivering.
English: Plasma levels of ammonia, arginine, essential amino acids (especially branched chain amino acids), carnitine and serum proteins should be maintained within normal limits.
Danish: Plasmakoncentrationerne af ammoniak, arginin, essentielle aminosyrer (især aminosyrer med forgrenede sidegrupper), carnitin og serumproteiner skal holdes inden for normalområderne.
English: The usual total daily dose of sodium phenylbutyrate in clinical experience is:
Danish: Den normale daglige dosis natriumphenylbutyrat i kliniske undersøgelser er:
English: To measure the dose:
Danish: Sådan afmåles dosis:
English: In patients with impaired hepatic function the conversion of phenylacetate to phenylacetylglutamine may be relatively slower.
Danish: Hos patienter med svækket leverfunktion kan omdannelsen af phenylacetat til phenylacetylglutamin være relativt langsommere.
English: PHEBURANE will not prevent the occurrence of an acute excess of ammonia in the blood, a condition which usually constitutes a medical emergency.
Danish: PHEBURANE kan ikke forhindre, at blodets indhold af ammoniak akut bliver for højt, en tilstand, der som regel udgør en medicinsk nødsituation.
English: A probable case of toxic reaction to sodium phenylbutyrate (450 mg/kg/d) was reported in an 18year old anorectic female patient who developed a metabolic encephalopathy associated with lactic acidosis, severe hypokalaemia, pancytopaenia, peripheral neuropathy, and pancreatitis.
Danish: Et sandsynligt tilfælde af en toksisk reaktion mod natriumphenylbutyrat (450 mg/kg/dag) er indberettet for en 18-årig anorektisk kvinde, der udviklede metabolisk encefalopati associeret med lactacidose, alvorlig hypokalæmi, pancytopeni, perifer neuropati og pankreatitis.
English: PHEBURANE should be administered orally.
Danish: PHEBURANE administreres oralt.
English: If persistent vomiting occurs, you should contact your doctor immediately.
Danish: Hvis du får gentagne opkastninger, skal du straks kontakte lægen.
English: Previously, neonatal-onset presentation of urea cycle disorders was almost universally fatal within the first year of life, even when treated with peritoneal dialysis and essential amino acids or their nitrogen-free analogues.
Danish: Tidligere var den neonatalt indsættende form af urinstofcyklusdefekt næsten altid dødelig inden for det første leveår, selv når det blev behandlet med peritonealdialyse og essentielle aminosyrer eller disses kvælstoffrie analoger.
English: In normal volunteers gender differences were found in the pharmacokinetic parameters of phenylbutyrate and phenylacetate (AUC and Cmax about 30 - 50 % greater in females), but not phenylacetylglutamine.
Danish: Hos raske frivillige forsøgspersoner blev der fundet en forskel mellem kønnene i de farmakokinetiske parametre for phenylbutyrat og phenylacetat (AUC og Cmax omkring 30-50 % større for kvinder), men ikke for phenylacetylglutamin.
English: It is unknown whether sodium phenylbutyrate/metabolites are excreted in human milk.
Danish: Det vides ikke, om natriumphenylbutyrat/metabolitter udskilles i human modermælk.
English: Because of its slow dissolution, PHEBURANE should not be administered by nasogastric or gastrostomy tubes.
Danish: På grund af sin langsomme opløsning bør PHEBURANE ikke administreres gennem nasogastrisk sonde eller gastrostomisonde.
English: Do not use PHEBURANE after the expiry date which is stated on the carton and the bottle label after EXP.
Danish: Brug ikke PHEBURANE efter den udløbsdato, der står på pakningen og flaskeetiketten efter EXP. Udløbsdatoen er den sidste dag i den nævnte måned.
English: • corticosteroids (medicines that are used to provide relief for inflamed areas of the body),
Danish: • kortikosteroider (lægemidler, som anvendes til at lindre betændelsestilstande i kroppen)
English: • decrease in hearing,
Danish: • nedsat hørelse
English: • have decreased kidney or liver function, since PHEBURANE is eliminated from the body through the kidney and liver.
Danish: • hvis du har nedsat nyre- eller leverfunktion, da PHEBURANE udskilles af kroppen gennem nyrerne og leveren.
English: With haemodialysis, use of alternative waste nitrogen excretion pathways (sodium phenylbutyrate, sodium benzoate and sodium phenylacetate), dietary protein restriction, and, in some cases, essential amino acid supplementation, the survival rate in newborns diagnosed after birth (but within the first month of life) increased to almost 80 % with most deaths occurring during an episode of acute hyperammonaemic encephalopathy.
Danish: Med hæmodialyse, brug af alternative udskillelsesveje for kvælstofaffald (natriumphenylbutyrat, natriumbenzoat og natriumphenylacetat), restriktiv proteinindtagelse med føden og i nogle tilfælde tilskud af essentielle aminosyrer, steg overlevelsen for nyfødte, som blev diagnosticeret efter fødslen (men inden for den første levemåned), til næsten 80 %, hvor de fleste dødsfald skete under et tilfælde med akut hyperammonæmisk encefalopati.
English: In the event of an overdose, the treatment should be discontinued and supportive measures be instituted.
Danish: I tilfælde af overdosering seponeres behandlingen, og symptomatisk behandling gives.
English: PHEBURANE treatment should be supervised by a physician experienced in the treatment of urea cycle disorders.
Danish: Behandlingen med PHEBURANE bør kontrolleres af en læge med erfaring inden for behandling af urinstofcyklusdefekter.
English: In late-onset deficiency patients, including females heterozygous for ornithine transcarbamylase deficiency, who recovered from hyperammonaemic encephalopathy and were then treated chronically with dietary protein restriction and sodium phenylbutyrate, the survival rate was 98 %.
Danish: Hos patienter med sent indsættende mangel, herunder kvinder, der var heterozygote for ornithintranscarbamylase-mangel og var restitueret efter hyperammonæmisk encefalopati og derefter sat i kronisk behandling med proteinfattig kost og natriumfenylbutyrat, var overlevelsesprocenten 98 %.
English: Prenatal exposure of rat pups to phenylacetate (the active metabolite of phenylbutyrate) produced lesions in cortical pyramidal cells; dendritic spines were longer and thinner than normal and reduced in number (see section 4.6).
Danish: Prænatal eksponering af rotteunger for phenylacetat (den aktive metabolit af phenylbutyrat) producerede læsioner i kortikale pyramidale celler; dendritiske rygsøjler var længere og tyndere end normalt og reduceret i antal (se pkt.
English: Corticosteroids may cause the breakdown of body protein and thus increase plasma ammonia levels.
Danish: Kortikosteroider kan medføre nedbrydning af vævsprotein og på denne måde øge plasmakoncentrationen af ammoniak.
English: What you need to know before you take PHEBURANE 3.
Danish: Det skal du vide, før du begynder at tage PHEBURANE 3.
English: Therapeutic monitoring
Danish: Terapeutisk kontrol:
English: PHEBURANE granules are white to off-white.
Danish: PHEBURANE granulat er hvidt til gulligt.
English: If this occurs, please discuss it with your doctor, because the absence of your period may be caused by pregnancy (see ‘Pregnancy and breast-feeding' section above) or by menopause.
Danish: Fortæl det til din læge, hvis dette sker, da menstruationsophør enten kan skyldes, at du er gravid (se afsnittet herover om graviditet og amning), eller i overgangsalderen.
English: Sodium phenylbutyrate was negative in 2 mutagenicity tests, i.e. the Ames test and the micronucleus test.
Danish: Natriumphenylbutyrat var negativ i 2 mutagentest, dvs. Ames test og mikronukleustesten.
English: The active substance is sodium phenylbutyrate.
Danish: Det aktive stof er natriumphenylbutyrat.
English: Arginine supplementation is required for patients diagnosed with deficiency of argininosuccinate synthetase at a dose of 0.4 - 0.7 g/kg/day or 8.8 - 15.4 g/m2/day.
Danish: Arginintilskud er nødvendigt til patienter, som har fået diagnosticeret mangel på argininosuccinatsyresyntetase med en dosering på 0,4-0,7 g/kg/dag eller 8,8-15,4 g/m2/dag.
English: Women of childbearing potential/Contraception in males and females Effective contraceptive measures must be taken by women of child-bearing potential.
Danish: Kvinder i den fertile alder/Antikonception til mænd og kvinder Effektiv antikonception skal bruges af kvinder i den fertile alder.
English: Common side effects (may affect more than 1 in 100 people): changes in number of blood cells (red cells, white cells and platelets), changes in the amount of bicarbonate in the blood, reduced appetite, depression, irritability, headache, fainting, fluid retention (swelling), changes in taste (taste disturbances), stomach ache, vomiting, nausea, constipation, abnormal skin odor, rash, abnormal kidney function, weight gain, altered laboratory test values.
Danish: Almindelige bivirkninger (kan forekomme hos mere end 1 ud af hver 100 behandlede): Ændringer i antallet af blodlegemer (røde blodlegemer, hvide blodlegemer og blodplader), ændringer i mængden af bikarbonat i blodet, nedsat appetit, depression, irritabilitet, hovedpine, besvimelse, væskeophobning (hævelse), ændringer i smagen (smagsforstyrrelser), mavesmerter, opkastning, kvalme, forstoppelse, unormal kropslugt, udslæt, unormal nyrefunktion, vægtstigning, ændrede resultater af laboratorieanalyser.
English: Pheburane is contra-indicated during pregnancy (see section 4.3).
Danish: Pheburane er kontraindiceret under graviditet (se pkt.
English: The other ingredients are: sugar spheres (sucrose and maize starch, see section 2 ‘PHEBURANE contains sucrose), hypromellose, ethylcellulose N7, macrogol 1500, povidone K25.
Danish: Øvrige indholdsstoffer er saccharosekugler (saccharose og majsstivelse, se afsnit 2 “PHEBURANE indeholder saccharose”), hypromellose, ethylcellulose N7, macrogol 1500, povidon K25.
English: Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness.
Danish: Inden for hver enkelt hyppighedsgruppe skal bivirkningerne opstilles efter, hvor alvorlige de er.
English: • This medicinal product contains 124 mg sodium per gram, equivalent to 6.2% of the WHO recommended maximum daily intake for sodium.
Danish: • Dette lægemiddel indeholder 124 g natrium pr. gram, hvilket er ækvivalent med 6,2% af WHO's maksimalt anbefalede daglige indtag af natrium.
English: Phenylbutyrate is rapidly absorbed under fasting conditions.
Danish: Phenylbutyrat bliver hurtigt absorberet ved faste.
English: The granules are packaged in a plastic bottle with child-resistant closure and a dessicant.
Danish: Granulatet er pakket i plastflasker med børnesikret låg og tørremiddel.
English: • probenecid (for treatment of hyperuricaemia, high levels of uric acid in the blood, associated with gout)
Danish: • probenecid (til behandling af forhøjet indhold af urinsyre i blodet i forbindelse med gigt
English: The majority of the patients who were tested had an IQ in the average to low average/borderline mentally retarded range.
Danish: Hos størstedelen af de testede patienter var IQ middel eller i underkanten af middel/grænsende til retardering.
English: PHEBURANE should therefore be used with caution in patients with congestive heart failure or severe renal insufficiency, and in clinical conditions where there is sodium retention with oedema.
Danish: PHEBURANE skal derfor anvendes med forsigtighed til patienter med kongestiv hjerteinsufficiens eller svær nyreinsufficiens og ved kliniske tilstande, hvor der foreligger natriumretention med ødemdannelse.
English: The disposition of phenylbutyrate and its metabolites has also been studied in cancer patients following intravenous infusion of sodium phenylbutyrate (up to 2 g/m²) or phenylacetate.
Danish: Fordelingen af phenylbutyrat og dets metabolitter har også været undersøgt hos cancerpatienter efter intravenøs infusion af natriumphenylbutyrat (op til 2 g/m2) eller phenylacetat.
English: How to take PHEBURANE 4.
Danish: Sådan skal du tage PHEBURANE 4.
English: Fertility There is no evidence available on the effect of sodium phenylbutyrate on fertility.
Danish: Fertilitet Der foreligger ingen tilgængelige data vedrørende virkningen af natriumphenylbutyrat på fertiliteten.
English: PHEBURANE is unlikely to affect your ability to drive and use machines.
Danish: PHEBURANE forventes ikke at påvirke din evne til at køre bil og betjene maskiner.
English: PHEBURANE has negligible influence on the ability to drive and use machines.
Danish: PHEBURANE har ubetydelig indflydelse på evnen til at føre motorkøretøj og betjene maskiner.
English: Because it dissolves slowly, PHEBURANE should not be administered through a gastrostomy (tube that goes through the abdomen to the stomach) or through a nasogastric tube (tube that goes through the nose to the stomach).
Danish: Da PHEBURANE opløses langsomt, skal det ikke gives gennem en gastrostomi (en sonde, som går gennem maveskindet direkte ind i mavesækken) eller gennem en nasogastrisk sonde (en sonde, som går gennem næsen til mavesækken).
English: Haemodialysis or peritoneal dialysis may be beneficial.
Danish: Hæmodialyse og peritonealdialyse kan have effekt.
English: Studies with high intravenous doses of phenylacetate showed non-linear pharmacokinetics characterised by saturable metabolism to phenylacetylglutamine.
Danish: Forsøg med indgift af høje doser phenylacetat intravenøst viste ikke-lineær farmakokinetik, der var karakteriseret ved saturabel metabolisme til phenylacetylglutamin.
English: Three cirrhotic patients (out of 6) who received repeated oral administration of sodium phenylbutyrate (20 g/day in three doses) showed sustained plasma levels of phenylacetate on the third day that were five times higher than those achieved after the first dose.
Danish: Tre cirrhosepatienter (ud af 6), som fik gentagen peroral indgift af natriumphenylbutyrat (20 g/dag i tre doser), viste på tredjedagen vedvarende plasmakoncentrationer af phenylacetat som var fem gange højere end de, som blev opnået efter den første dosis.
English: PHEBURANE contains the active substance sodium phenylbutyrate which is used to treat patients of all ages with urea cycle disorders.
Danish: PHEBURANE indeholder det aktive stof phenylbutyrat, som anvendes til behandling af patienter i alle aldre, der lider af forstyrrelser i urinstofcyklus.
English: Since the metabolism and excretion of sodium phenylbutyrate involves the liver and kidneys, PHEBURANE should be used with caution in patients with hepatic or renal insufficiency.
Danish: Da metaboliseringen og udskillelsen af natriumphenylbutyrat inddrager leveren og nyrerne, bør der udvises forsigtighed ved anvendelse af PHEBURANE hos patienter med lever- eller nyreinsufficiens.
English: Contents of the pack and other information What PHEBURANE contains
Danish: Pakningsstørrelser og yderligere oplysninger PHEBURANE indeholder:
English: Common abdominal pain, vomiting, nausea, constipation, dysgeusia Uncommon pancreatitis, peptic ulcer, rectal haemorrhage, gastritis
Danish: Almindelig abdominalsmerter, opkastning, kvalme, konstipation, dysgeusi Ikke almindelig pankreatitis, ulcus pepticum, rektalblødning, gastritis
English: Uncommon side effects (may affect more than 1 in 1,000 people): deficiency in red blood cells due to failure of the bone marrow, bruising, altered heart rhythm, rectal bleeding, inflammation of the stomach, stomach ulcer, inflammation of the pancreas.
Danish: Ikke almindelige bivirkninger (kan forekomme hos mere end 1 ud af hver 1 000 behandlede): Mangel på røde blodlegemer som følge af knoglemarvssvigt, blå mærker, ændret hjerterytme, blødning fra endetarmen, inflammation i maven, mavesår, betændelse i bugspytkirtlen.
English: Available pharmacodynamic/toxicological data in animals have shown excretion of sodium phenylbutyrate/metabolites in milk (see section 5.3).
Danish: Tilgængelige farmakodynamiske/toksikologiske data hos dyr har vist udskillelse af natriumphenylbutyrat/metabolitter i modermælk (se pkt.
English: Mechanism of action and pharmacodynamic effects Sodium phenylbutyrate is a pro-drug and is rapidly metabolised to phenylacetate.
Danish: Virkningsmekanisme og farmakodynamiske virkninger Natriumphenylbutyrat er et prodrug og metaboliseres hurtigt til phenylacetat.
English: • Serum potassium should be monitored during therapy since renal excretion of phenylacetylglutamine may induce a urinary loss of potassium.
Danish: • Serum-kalium bør overvåges i behandlingsforløbet, idet den renale udskillelse af phenylacetylglutamin kan fremkalde tab af kalium via urinen.
English: Pharmacotherapeutic group: Other alimentary tract and metabolism products, various alimentary tract and metabolism products, ATC code: A16AX03.
Danish: Farmakoterapeutisk klassifikation: Fordøjelsesorganer og stofskifte, Andre stofskiftesygdomme, ATCkode: A16AX03.
English: PHEBURANE is indicated as adjunctive therapy in the chronic management of urea cycle disorders, involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase or argininosuccinate synthetase.
Danish: PHEBURANE er indiceret som adjuvant behandling til den kroniske kontrol af urinstofcyklusdefekter, som involverer mangel på carbamylphosphatsyntetase, ornithintranscarbamylase eller argininosuccinatsyntetase.
English: Concurrent administration of probenecid may affect renal excretion of the conjugation product of sodium phenylbutyrate.
Danish: Samtidig indgift af probenecid kan påvirke den renale udskillelse af konjugeringsproduktet af natriumphenylbutyrat.
English: • PHEBURANE contains 124 mg (5.4 mmol) of sodium per gram of sodium phenylbutyrate, corresponding to 2.5 g (108 mmol) of sodium per 20 g of sodium phenylbutyrate, which is the maximum daily dose.
Danish: • PHEBURANE indeholder 124 mg (5,4 mmol) natrium pr. gram natriumphenylbutyrat, hvilket svarer til 2,5 g (108 mmol) natrium pr. 20 g natriumphenylbutyrat, som er den maksimale daglige dosis.
English: Nutritional management PHEBURANE must be combined with dietary protein restriction and, in some cases, essential amino acid and carnitine supplementation.
Danish: PHEBURANE skal kombineres med indskrænkning i fødens indhold af protein og i nogle tilfælde suppleres med essentielle aminosyrer og carnitin.
English: After the first opening, PHEBURANE can be used within 45 days.
Danish: PHEBURANE skal anvendes inden 45 dage efter åbning af emballagen.
English: Only use the calibrated measuring spoon provided.
Danish: Brug kun den medfølgende inddelte måleske.
English: PHEBURANE must be taken with a special diet reduced in protein.
Danish: PHEBURANE skal tages sammen med en særlig lavproteindiæt.
English: The granules can be directly swallowed with a drink (water, fruit juices, protein-free infant formulas) or sprinkled on to a spoonful of solid foods (mashed potatoes or apple sauce).
Danish: Granulatet kan indtages straks med væske (vand, frugtsaft, proteinfri modermælkserstatning) eller drysses på en skefuld fast føde (kartoffelmos eller æblemos).
English: Sodium phenylbutyrate
Danish: Natriumphenylbutyrat
English: Carcinogenicity and fertility studies have not been conducted with sodium phenylbutyrate.
Danish: Der er ikke udført undersøgelser af karcinogenicitet og fertilitet med natriumphenylbutyrat.