natgillin/translations-raw huggingface dataset:
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.
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.
PHEBURANE indeholder det aktive stof phenylbutyrat, som anvendes til behandling af patienter i alle aldre, der lider af forstyrrelser i urinstofcyklus.
PHEBURANE contains the active substance sodium phenylbutyrate which is used to treat patients of all ages with urea cycle disorders.
Da metaboliseringen og udskillelsen af natriumphenylbutyrat inddrager leveren og nyrerne, bør der udvises forsigtighed ved anvendelse af PHEBURANE hos patienter med lever- eller nyreinsufficiens.
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.
Pakningsstørrelser og yderligere oplysninger PHEBURANE indeholder:
Contents of the pack and other information What PHEBURANE contains
Almindelig abdominalsmerter, opkastning, kvalme, konstipation, dysgeusi Ikke almindelig pankreatitis, ulcus pepticum, rektalblødning, gastritis
Common abdominal pain, vomiting, nausea, constipation, dysgeusia Uncommon pancreatitis, peptic ulcer, rectal haemorrhage, gastritis
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.
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.
Tilgængelige farmakodynamiske/toksikologiske data hos dyr har vist udskillelse af natriumphenylbutyrat/metabolitter i modermælk (se pkt.
Available pharmacodynamic/toxicological data in animals have shown excretion of sodium phenylbutyrate/metabolites in milk (see section 5.3).
Virkningsmekanisme og farmakodynamiske virkninger Natriumphenylbutyrat er et prodrug og metaboliseres hurtigt til phenylacetat.
Mechanism of action and pharmacodynamic effects Sodium phenylbutyrate is a pro-drug and is rapidly metabolised to phenylacetate.
• Serum-kalium bør overvåges i behandlingsforløbet, idet den renale udskillelse af phenylacetylglutamin kan fremkalde tab af kalium via urinen.
• Serum potassium should be monitored during therapy since renal excretion of phenylacetylglutamine may induce a urinary loss of potassium.
Farmakoterapeutisk klassifikation: Fordøjelsesorganer og stofskifte, Andre stofskiftesygdomme, ATCkode: A16AX03.
Pharmacotherapeutic group: Other alimentary tract and metabolism products, various alimentary tract and metabolism products, ATC code: A16AX03.
PHEBURANE er indiceret som adjuvant behandling til den kroniske kontrol af urinstofcyklusdefekter, som involverer mangel på carbamylphosphatsyntetase, ornithintranscarbamylase eller argininosuccinatsyntetase.
PHEBURANE is indicated as adjunctive therapy in the chronic management of urea cycle disorders, involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase or argininosuccinate synthetase.
Samtidig indgift af probenecid kan påvirke den renale udskillelse af konjugeringsproduktet af natriumphenylbutyrat.
Concurrent administration of probenecid may affect renal excretion of the conjugation product of sodium phenylbutyrate.
• 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.
• 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.
PHEBURANE skal kombineres med indskrænkning i fødens indhold af protein og i nogle tilfælde suppleres med essentielle aminosyrer og carnitin.
Nutritional management PHEBURANE must be combined with dietary protein restriction and, in some cases, essential amino acid and carnitine supplementation.
PHEBURANE skal anvendes inden 45 dage efter åbning af emballagen.
After the first opening, PHEBURANE can be used within 45 days.
Brug kun den medfølgende inddelte måleske.
Only use the calibrated measuring spoon provided.
PHEBURANE skal tages sammen med en særlig lavproteindiæt.
PHEBURANE must be taken with a special diet reduced in protein.
Granulatet kan indtages straks med væske (vand, frugtsaft, proteinfri modermælkserstatning) eller drysses på en skefuld fast føde (kartoffelmos eller æblemos).
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).
Natriumphenylbutyrat
Sodium phenylbutyrate
Der er ikke udført undersøgelser af karcinogenicitet og fertilitet med natriumphenylbutyrat.
Carcinogenicity and fertility studies have not been conducted with sodium phenylbutyrate.
Som følge heraf reducerer natriumphenylbutyrat forhøjede plasmaammoniak- og glutaminkoncentrationer hos patienter med urinstofcyklusdefekt.
As a consequence, sodium phenylbutyrate reduces elevated plasma ammonia and glutamine levels in patients with urea cycle disorders.
Citrullin- eller arginintilskud er nødvendigt til patienter, som har fået diagnosticeret neonatalt indsættende mangel på carbamylphosphatsyntetase eller ornithintranscarbamylase med en dosering på 0,17 g/kg/dag eller 3,8 g/m2/dag.
Citrulline or arginine supplementation is required for patients diagnosed with neonatal-onset form of carbamyl phosphate synthetase or ornithine transcarbamylase deficiency at a dose of 0.17 g/kg/day or 3.8 g/m2/day.
Det skal du vide, før du begynder at tage PHEBURANE Tag ikke PHEBURANE:
What you need to know before you take PHEBURANE Do not take PHEBURANE if you:
Hos størstedelen af patienter med urinstofdefekter eller hæmoglobinopatier, der fik forskellige doser phenylbutyrat (300-650 mg/kg/dag op til 20 g/dag), kunne plasmakoncentrationen af phenylacetat ikke påvises efter faste til dagen efter.
In the majority of patients with urea cycle disorders or haemoglobinopathies receiving various doses of phenylbutyrate (300 - 650 mg/kg/day up to 20 g/day) no plasma level of phenylacetate could be detected after overnight fasting.
Almindelig synkope, hovedpine,
Common syncope, headache
Er du i tvivl, så spørg lægen eller på apoteket.
In case of any doubt, ask your doctor or pharmacist.
PHEBURANE skal imidlertid bruges sammen med en lavproteindiæt, som udarbejdes specielt til dig af en læge eller en kostvejleder.
However PHEBURANE must be used along with a diet reduced in proteins, designed especially for you by the doctor and the dietician.
Plasma og urinkoncentrationer af phenylbutyrat og dets metabolitter er blevet målt på fastende raske voksne, efter at de havde fået en enkelt dosis på 5 g natriumphenylbutyrat og fra patienter med urinstofcyklusdefekter, hæmoglobinopatier og levercirrhose, som fik enkelte og gentagne doser peroralt på op til 20 g/dag (ukontrollerede forsøg).
Plasma and urine concentrations of phenylbutyrate and its metabolites have been obtained from fasting normal adults who received a single dose of 5 g of sodium phenylbutyrate and from patients with urea cycle disorders, haemoglobinopathies and cirrhosis receiving single and repeated oral doses up to 20 g/day (uncontrolled studies).
Phenylacetat er en metabolisk aktiv substans, som konjugeres med glutamin ved acetylering for at danne phenylacetylglutamin, som derefter udskilles af nyrerne.
Phenylacetate is a metabolically active compound that conjugates with glutamine via acetylation to form phenylacetylglutamine which is then excreted by the kidneys.
Hvert gram natriumfenylbutyrat indeholder 124 mg (5,4 mmol) natrium og 768 mg saccharose.
Each gram of sodium phenylbutyrate contains 124 mg (5.4 mmol) of sodium and 768 mg of sucrose.
PHEBURANE 483 mg/g {Kun karton}
PHEBURANE 483 mg/g {For carton only}
PHEBURANE indeholder natrium og saccharose.
PHEBURANE contains sodium and sucrose
Patienternes kognitive præstationer forblev relativt uændrede under behandlingen med phenylbutyrat.
Their cognitive performance remained relatively stable during phenylbutyrate therapy.
Hver flaske indeholder 174 g granulat.
Each bottle contains 174g of granules.
Kvinder i den fertile alder skal bruge en effektiv antikonception.
Women of childbearing potential must use effective contraception during treatment.
Hvis du har glemt at tage PHEBURANE:
If you forget to take PHEBURANE
Blod og lymfesystem Almindelig anæmi, trombocytopeni, leukopeni, leukocytose, trombocytose
Common anaemia, thrombocytopenia, leukopenia, leukocytosis, thrombocytosis
Efter en enkelt oral dosis på 5 g natriumphenylbutyrat i form af granulat blev der påvist målbare koncentrationer i plasma af phenylbutyrat 15 minutter efter indgift.
After a single oral dose of 5 g of sodium phenylbutyrate, in the form of granules, measurable plasma levels of phenylbutyrate were detected 15 minutes after dosing.
Karton: 1 flaske med 174 g granulat.
Carton: One bottle with 174 g granules.
Efter en enkelt dosis på 5 g natriumphenylbutyrat i granulatform blev der påvist målelige plasmakoncentrationer af phenylacetat og phenylacetylglutamin henholdsvis 30 og 60 minutter efter indgift.
After a single dose of 5 g of sodium phenylbutyrate, in the form of granules, measurable plasma levels of phenylacetate and phenylacetylglutamine were detected 30 and 60 minutes respectively after dosing.
Der medfølger en måleske, som afmåler op til 3 g natriumphenylbutyrat med en graduering på 250 mg.
A calibrated dosing spoon is provided which dispenses up to 3g of sodium phenylbutyrate by graduation of 250 mg.
Man ved, at phenylbutyrat bliver oxyderet til phenylacetat, som enzymatisk konjugeres med glutamin for at danne phenylacetylglutamin i leveren og nyren.
Phenylbutyrate is known to be oxidised to phenylacetate which is enzymatically conjugated with glutamine to form phenylacetylglutamine in the liver and kidney.
Saccharosekugler (saccharose og majsstivelse), hypromellose, ethylcellulose N7, macrogol 1500, povidon K25.
PHARMACEUTICAL PARTICULARS 6.1 List of excipients sugar spheres (sucrose and maize starch), hypromellose, ethylcellulose N7, macrogol 1500, povidone K25.
Flaske: 174 g granulat.
Bottle: 174 g granules.
Den daglige dosis PHEBURANE beregnes på baggrund af din legemsvægt eller legemsoverflade og justeres efter din proteinbalance og kost.
The daily dose of PHEBURANE will be based on your body weight or body surface and adjusted according to your protein tolerance and diet. You will need regular blood tests to determine the correct daily dose.
Dette lægemiddel indeholder 124 mg (5,4 mmol) natrium pr. g natriumphenylbutyrat.
This medicine contains 124 mg (5.4 mmol) of sodium per 1 g of sodium phenylbutyrate.
• hovedpine
• headache,
Indlægsseddel: Information til brugeren PHEBURANE granulat, 483 mg/g
Package leaflet: Information for the patient PHEBURANE 483 mg/g granules
Brug af anden medicin sammen med PHEBURANE
Other medicines and PHEBURANE
Ikke almindelig arytmi
Uncommon arrhythmia
og den gennemsnitlige maksimumkoncentration var 195 µg/ml.
The mean time to peak concentration was 1 hour and the mean peak concentration 195 μg/ml.
Baseret på undersøgelser af udskillelsen af phenylacetylglutamin hos patienter med urinstofcyklusdefekter er det muligt at skønne, at der dannes mellem 0,12 og 0,15 g phenylacetylglutaminkvælstof, for hvert gram natriumphenylbutyrat, der er indgivet.
Clinical efficacy and safety Based on studies of phenylacetylglutamine excretion in patients with urea cycle disorders it is possible to estimate that, for each gram of sodium phenylbutyrate administered, between 0.12 and 0.15 g of phenylacetylglutamine nitrogen are produced.
Pheburane er kontraindiceret under amning (se pkt.
Pheburane is contra-indicated during breast-feeding (see section 4.3).
Der medfølger en inddelt måleske.
A calibrated measuring spoon is provided.
Dette lægemiddel indeholder 768 mg saccharose pr. g natriumphenylbutyrat.
This medicine contains 768 mg of sucrose per 1 g of sodium phenylbutyrate.
På molær basis er natriumphenylbutyrat sammenlignelig med urinstof (indeholder begge 2 mol nitrogen).
On a molar basis, phenylacetylglutamine is comparable to urea (each containing 2 moles of nitrogen) and therefore provides an alternate vehicle for waste nitrogen excretion.
Granulat Hvidt til let gulligt granulat.
White to off-white granules.
Almindelig nedsat indhold i blodet af kalium, albumin, total protein og fosfat, forhøjet indhold i blodet af alkalisk fosfatase, transaminaser, bilirubin, urinsyre, chlorid, fosfat og natrium, vægtøgning
Increased blood alkaline phosphatase, transaminases, bilirubin, uric acid, chloride, phosphate and sodium.
• hvis du er overfølsom (allergisk) over for phenylbutyrat eller et af de øvrige indholdsstoffer
• are allergic to sodium phenylbutyrate or any of the other ingredients of this medicine (listed in section 6).
Hvert gram af granulatet indeholder 483 mg natriumphenylbutyrat.
Each gram of granules contains 483 mg of sodium phenylbutyrate.
Sådan skal du tage PHEBURANE
How to take PHEBURANE
EXP Skal anvendes inden for 45 dage efter anbrud af emballagen.
EXP After the first opening, to be used within 45 days.
Ikke almindelig aplastisk anæmi, ekkymose Metabolisme og ernæring Almindelig metabolisk acidose, alkalose, nedsat appetit Psykiske lidelser
Common metabolic acidosis, alkalosis, decreased appetite
Navn og adresse på fremstilleren (fremstillerne) ansvarlig(e) for batchfrigivelse
Name and address of the manufacturer(s) responsible for batch release Lucane Pharma
• svækket hukommelse
• impaired memory,
Tabel over uønskede hændelser
Tabulated list of adverse reactions
Uønsket hændelse
Adverse reaction
Skal anvendes inden 45 dage efter anbrud af emballagen.
After the first opening, to be used within 45 days.
Hvis du har taget for meget PHEBURANE
If you take more PHEBURANE than you should
PHEBURANE granulat, 483 mg/g
PHEBURANE 483 mg/g granules
• smagsforstyrrelser
• changes in taste (taste disturbances),
Der medfølger en inddelt måleske, som kan afmåle op til 3 gram natriumphenylbutyrat.
A calibrating measuring spoon which dispenses up to 3 g of sodium phenylbutyrate is provided with the medicine.
• PHEBURANE anses for at have et højt natrium-indhold, Dette bør især tages i betragtning hos patienter på en diæt med lavt saltindhold.
• PHEBURANE is considered high in sodium.
• Spørg lægen eller på apoteketspersonalet, hvis der er mere, du vil vide.
• If you have any further questions, ask your doctor or pharmacist.
Spørg din læge eller på apoteket, hvis du er usikker på, om din medicin indeholder disse stoffer.
If you are uncertain if your medicines contain these substances, you should check with your doctor or pharmacist.
Mave-tarmkanalen
Common oedema
Relevansen for mennesker er dog ukendt.
However, the relevance to humans is unknown.
*Repræsenterer en sammenlægning af foretrukne termer
*Represents a pooling of preferred terms
Responsen blev vurderet hver 4. uge indtil sygdomsprogression.
Response was assessed every 4 weeks until disease progression.
Lægen undersøger dig, og du vil blive overvåget nøje under behandlingen.
Your doctor will examine you and you will be monitored closely during treatment.
Lægen kan også justere dosis af de to andre lægemidler.
The doctor may also adjust the doses of the other medicines.
• Lad ikke indholdet i kapslen komme i kontakt med din hud.
• Do not let the contents of the capsule come into contact with your skin.
Alder, køn og race
Age, gender, race
Resultaterne for PFS er opsummeret i tabel 4 og figur 1.
PFS results are summarised in Table 4 and Figure 1.
Den gennemsnitlige absolutte orale biotilgængelighed er 58%.
The mean absolute oral bioavailability is 58%.
Anden reduktion til
Second reduction to
‡ORR = CR+VGPR+PR §Baseret på responders i den respons-evaluerbare population
‡ORR = CR+VGPR+PR §Based on responders in the response-evaluable population
Transportørbaserede interaktioner
Transporter-based interactions
Lenalidomid er strukturelt beslægtet med thalidomid.
Lenalidomide is structurally related to thalidomide.
Tid til respons, måneder Median
Time to Response, months Median
En risiko for nyfødte/spædbørn kan ikke udelukkes, og derfor skal amning ophøre.
A risk to newborns/infants cannot be excluded and therefore breast-feeding should be discontinued.
85% af populationen var hvide, 9% asiater og 2% sorte.
Eighty five percent of the population was White, 9% Asian and 2% Black.
NINLARO tages én gang om ugen (på samme ugedag) i de første 3 uger i denne cyklus.
NINLARO is taken once a week (on the same day of the week) for the first 3 weeks of this cycle.
Hvert blisterkort indeholder én kapsel).
Each blister contains one capsule).
Primær refraktær, defineret som bedste respons af stabil sygdom eller sygdomsprogression på alle tidligere behandlinger, blev dokumenteret hos 6% af patienterne.
Primary refractory, defined as best response of stable disease or disease progression on all prior therapies, was documented in 6% of patients.
Medianalderen var 66 år (38-91 år); 58% af patienterne var over 65 år.
The median age was 66 years, range 38-91 years; 58% of patients were older than 65 years.
Medianen for samlet overlevelse (OS) blev ikke nået i nogen af grupperne.
Median overall survival was not reached in either regimen.
Varighed af behandlingen med NINLARO Du skal fortsætte behandlingen, indtil lægen siger, du skal stoppe.
Duration of the treatment with NINLARO You should continue treatment until your doctor tells you to stop.
Der foreligger ingen data fra dyrestudier.
No animal data are available.
Patienten skal vurderes med henblik på underliggende årsager og eventuelt gives understøttende pleje.
The patient should be evaluated for underlying causes and provide supportive care, as necessary.
Du skal læse indlægssedlerne for de to andre lægemidler for at få yderligere oplysninger om deres anvendelse og virkning.
You should read the Package Leaflets of these other medicines for further information on their use and effects.
Bemærk: De bivirkninger, der er angivet som foretrukne termer, er baseret på MedDRA-version 16.0.
Note: ADRs included as preferred terms are based on MedDRA version 16.0.
Før du begynder at tage NINLARO og under behandlingen, vil der blive taget blodprøver for at sikre, at du har blodceller nok.
Before starting NINLARO and during treatment, you will have blood tests to check that you have enough blood cells.
Du kan føle dig træt og svimmel, mens du tager NINLARO.
You may feel tired and dizzy while taking NINLARO.
Ixazomibs indvirkning på andre lægemidler
Effect of ixazomib on other medicinal products
Anbefalede handlinger
Recommended actions
Undgå direkte kontakt med kapslens indhold.
Direct contact with the capsule contents should be avoided.
En hazard ratio under 1 indikerer en fordel for ixazomib-regimet.
A hazard ratio less than 1 indicates an advantage for the ixazomib regimen.
Reproduktiv og embryo-føtal udvikling
Reproductive and embryo-foetal development
De kliniske data er begrænsede, men der er rapporteret om doser på op til 12 mg i det randomiserede kontrollerede studie.
Clinical data is limited but doses up to 12 mg have been reported in the randomised controlled trial.
Overvej at tilføje G-CSF i henhold til de kliniske retningslinjer.
Consider adding G-CSF as per clinical guidelines.
Da der foreligger begrænsede data for disse patienter, anbefales en omhyggelig risk/benefit-vurdering, før et ixazomib-regime påbegyndes.
As data are limited in these patients, a careful risk-benefit assessment is recommended before initiating the ixazomib regimen.
Disse lægemidler skal undgås, da de kan nedsætte virkningen af NINLARO.
These medicines should be avoided as they may reduce the effectiveness of NINLARO.
• Slug kapslen hel sammen med vand.
• Swallow the capsule whole with water.
Figur 2: Forest-plot af progressionsfri overlevelse i alle undergrupper
Figure 2: Forest plot of progression-free survival in subgroups
Tag ikke NINLARO hvis du er allergisk over for ixazomib eller et af de øvrige indholdsstoffer i dette lægemiddel (angivet i punkt 6).
Do not take NINLARO if you are allergic to ixazomib or to any of the other any of the other ingredients of this medicine (listed in section 6).
Børn og unge NINLARO anbefales ikke til børn og unge på under 18 år.
Children and adolescents NINLARO is not recommended for use in children and adolescents aged under 18 years.
Af de 5 patienter i placebogruppen havde 2 bekræftet meget god delvis respons.
Of the 5 patients in the placebo regimen, 2 had a confirmed very good partial response.
I dette studie blev refraktær sygdom defineret som sygdomsprogression under behandling eller progression inden for 60 dage efter den sidste dosis af lenalidomid eller en proteasomhæmmer.
For the purposes of this study, refractory disease was defined as disease progression on treatment or progression within 60 days after the last dose of lenalidomide or a proteasome inhibitor.
Den anbefalede dosis er én 4 mg kapsel, der skal indtages gennem munden.
The recommended dose is one 4 mg capsule taken by mouth.
Forbedringen i PFS i ixazomib-gruppen blev understøttet af forbedringer i den samlede responsrate.
The improvement in PFS in the ixazomib regimen was supported by improvements in overall response rate.
Det skyldes, at anden medicin kan påvirke den måde, NINLARO fungerer på.
This is because other medicines can affect the way NINLARO works.
Det er ukendt, om ixazomib eller dets metabolitter udskilles i human mælk.
It is unknown whether ixazomib or its metabolites are excreted in human milk.
De demografiske data og sygdomskarakteristika ved baseline var afbalancerede og sammenlignelige mellem de to forsøgsgrupper.
The baseline demographics and disease characteristics were balanced and comparable between the study regimens.
Patienten skal overvåges for symptomer på perifer neuropati.
The patient should be monitored for symptoms of peripheral neuropathy.
En plasmacelle er en blodcelle, der normalt danner proteiner for at bekæmpe infektioner.
A plasma cell is a blood cell that normally produces proteins to fight infections.
Fortæl straks lægen, hvis du eller din partner bliver gravid under behandlingen med NINLARO.
Tell doctor right away if you or your partner becomes pregnant while receiving NINLARO.
Betingelserne i graviditetsforebyggelsesprogrammet for lenalidomid skal overholdes hos alle patienter, medmindre der er pålidelig evidens for, at patienten ikke er fertil.
The conditions of the Pregnancy Prevention Programme for lenalidomide must be fulfilled for all patients unless there is reliable evidence that the patient does not have childbearing potential.
I tilfælde af alvorlige gastrointestinale bivirkninger anbefales monitorering af serum-kalium.
In case of severe gastrointestinal events, monitoring of serum potassium level is recommended.
Hvis pulveret ved et uheld kommer i kontakt med din hud, skal du vaske huden grundigt med vand og sæbe.
If the powder accidentally comes into contact with your skin, wash it off thoroughly with soap and water.
Dette er en betinget markedsføringstilladelse, og i henhold til artikel 14a, stk. 4, i forordning (EF) nr. 726/2004 skal indehaveren af markedsføringstilladelsen inden for den fastsatte tidsramme gennemføre følgende foranstaltninger:
FOR THE CONDITIONAL MARKETING AUTHORISATION This being a conditional marketing authorisation and pursuant to Article 14a(4) of Regulation (EC) No 726/2004, the MAH shall complete, within the stated timeframe, the following measures: Description
Hvis du kaster op efter at have taget en dosis, skal du ikke tage en ekstra dosis.
If you vomit after taking a dose, do not take an extra dose.