natgillin/translations-raw huggingface dataset:
Studie med børn under 2 måneder
Study in Children Less Than 2 Months of Age
4-ugers, non-inferioritet, blindet overkrydsningsstudie med aktiv kontrol (studie 1) Et randomiseret, dobbeltblindt, aktivt kontrolleret non-inferioritetsstudie (studie 1) sammenlignede ækvivalente doser af glycerolphenylbutyrat og natriumphenylbutyrat ved at evaluere det venøse ammoniakniveau i 24 timer hos patienter med UCD'er, som var blevet behandlet med natriumphenylbutyrat inden inklusion med henblik på regulering af deres UCD.
Active-Controlled, 4-Week, Noninferiority, Blinded Crossover Study (Study 1) A randomised, double-blind, active-controlled, crossover, noninferiority study (Study 1) compared equivalent doses of glycerol phenylbutyrate to sodium phenylbutyrate by evaluating 24-hour venous ammonia levels in patients with UCDs who had been on sodium phenylbutyrate prior to enrolment for control of their UCD.
5.1 og 5.2 for oplysninger vedrørende farmakodynamiske og farmakokinetiske egenskaber hos denne aldersgruppe.
For data regarding pharmacodynamic and pharmacokinetic properties in this age group, see sections 5.1 and 5.2.
• hvis du har problemer med bugspytkirtlen, maven eller tarmene, da disse organer er ansvarlige for absorption af glycerolphenylbutyrat.
• if you have problems with your pancreas, stomach or gut (‘intestines') - these organs are responsible for absorption of glycerol phenylbutyrate into the body.
Glycerolphenylbutyrats indvirkning på CYP2C9 og potentialet for interaktion med celecoxib er blevet undersøgt hos mennesker uden noget bevis for interaktion.
The effects of glycerol phenylbutyrate on cytochrome P450 (CYP) 2C9 isoenzyme and potential for interaction with celecoxib has been studied in humans with no evidence of an interaction observed.
Du skal følge en særlig proteinfattig kostplan under behandling med glycerolphenylbutyrat.
You must follow a special low protein diet during treatment with glycerol phenylbutyrate.
• sultfornemmelse, vægttab eller vægtstigning
• feeling hungry, weight loss or weight gain
Forberedelse til administration gennem nasogastrisk sonde eller gastrostomisonde: In vitro undersøgelser til evaluering af den procentvise genfinding af den samlede dosis givet gennem en nasogastrisk, nasojejunal eller gastrostomisonde påviste, at dosis genfundet var > 99 % for doser > 1 ml og 70 % for en dosis på 0,5 ml.
Preparation for nasogastric tube or gastrostomy tube administration: In vitro studies evaluating the percent recovery of total dose delivered with nasogastric, nasojejunal or gastrostomy tubes demonstrated the percent of dose recovered was > 99% for doses > 1 ml and 70% for a 0.5 ml dose.
Givet at ca. 47 % af kvælstoffet i kosten udskilles som overskud, og at ca. 70 % af en administreret dosis af 4-phenylbutansyre (PBA) vil blive udskilt som phenylacetylglutamin i urinen (U-PAGN), er en estimeret begyndelsesdosis af glycerolphenylbutyrat per døgn 0,6 ml pr. gram indtaget protein i kosten pr. døgn, idet det antages, at alt restkvælstof omdannes af glycerolphenylbutyrat og udskilles som phenylacetylglutamin (PAGN).
Given that approximately 47% of dietary nitrogen is excreted as waste and approximately 70% of an administered 4phenylbutyric acid (PBA) dose will be converted to urinary phenylacetylglutamine (U-PAGN), an initial estimated glycerol phenylbutyrate dose for a 24-hour period is 0.6 ml glycerol phenylbutyrate per gram of dietary protein ingested per 24 hour period assuming all the waste nitrogen is covered by glycerol phenylbutyrate and excreted as phenylacetylglutamine (PAGN).
Det skal besluttes, om amning eller behandling med glycerolphenylbutyrat skal ophøre, idet der tages højde for fordelene ved amning for barnet i forhold til de terapeutiske fordele for moderen.
A decision must be made whether to discontinue breastfeeding or to discontinue/abstain from glycerol phenylbutyrate therapy taking into account the benefit of breast-feeding for the child and the benefit of therapy for the woman.
• Samlet daglig dosis RAVICTI (ml) = samlet daglig dosis natriumphenylbutyrat-tabletter (g) x
• Total daily dosage of RAVICTI (ml) = total daily dosage of sodium phenylbutyrate tablets (g) x
Efter administration af en enkelt dosis glycerolphenylbutyrat havde 15 ud af 22 deltagere en kvantificerbar PBA-plasmakoncentration i den første prøve udtaget efter dosering (0,25 time).
Upon singledose administration of glycerol phenylbutyrate, plasma concentrations of PBA were quantifiable in 15 of 22 participants at the first sample time post dose (0.25 h).
En plasma-PAA:PAGN-ratio på over 2,5 kan indikere mætning af kapaciteten til at omdanne PAA til PAGN, og det kan være nødvendigt at reducere dosis og/eller øge doseringshyppigheden.
A plasma PAA to PAGN ratio exceeding 2.5 may indicate saturation of PAA to PAGN conversion capacity and the need for reduced dosing and/or increased frequency of dosing.
RAVICTI skal anvendes i kombination med proteinrestriktion i kosten og i visse tilfælde med kosttilskud (f.eks. essentielle aminosyrer, arginin, citrullin, proteinfri kalorietilskud).
RAVICTI must be used with dietary protein restriction and, in some cases, dietary supplements (e.g., essential amino acids, arginine, citrulline, protein-free calorie supplements).
Hvis det er nødvendigt at administrere en dosis på 0,5 ml eller mindre via en nasogastrisk, nasojenunal eller gastrostomisonde, skal den lave genfinding af lægemiddel tages i betragtning ved fastlæggelse af dosis.
If there is a need to administer a dose of 0.5 ml or less with such nasogastric, gastrostomy or nasojejunal tubes, consideration should be given to the low drug recovery in dosing.
En ny lukbar adapter skal anvendes, når en ny flaske åbnes.
A new reclosable bottle cap adapter should be used when a new bottle is opened.
UCD'erne inkluderer defekter i carbamoylfosfatsyntetase I (CPS), ornithintranscarbamylase (OTC), argininoravsyre-syntetase (ASS), argininoravsyrelyase (ASL), arginase I (ARG), og ornithin-translokase-mangel hyperammoniæmihyperornithiniæmi homocitrullinuri-syndrom (HHH).
The UCDs include deficiencies of carbamoyl phosphate synthetase I (CPS), ornithine carbamoyltransferase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase I (ARG) and ornithine translocase deficiency hyperornithinaemia-hyperammonaemia homocitrullinuria syndrome (HHH).
Dosisjustering baseret på phenylacetat og phenylacetylglutamin i plasma Opkastning, kvalme, hovedpine, døsighed, forvirring eller søvnighed ved fravær af et højt ammoniakniveau eller interkurrent sygdom kan være tegn på phenyleddikesyre- (PAA) toksicitet (se pkt.
Adjustment based on plasma phenylacetate and phenylacetylglutamine Symptoms of vomiting, nausea, headache, somnolence, confusion, or sleepiness in the absence of high ammonia or intercurrent illness may be signs of phenylacetic acid (PAA) toxicity (see section 4.4, PAA toxicity).
• tests viser højere eller lavere kalium i blodet
• tests show higher or lower potassium in your blood
Der er et muligt potentiale for interaktion mellem glycerolphenylbutyrat som CYP3A4induktor og lægemidler, som hovedsagligt metaboliseres af CYP3A4.
The potential for interaction of glycerol phenylbutyrate as a CYP3A4 inducer and those products predominantly metabolised by the CYP3A4 pathway is possible.
Probenecid kan hæmme den renale udskillelse af glycerolphenylbutyrats metabolitter, herunder PAGN.
Probenecid Probenecid may inhibit the renal excretion of metabolites of glycerol phenylbutyrate including PAGN.
Forekomsten af følgende tumorer var også øget hos hunrotter: adenom, karcinom og kombineret adenom eller karcinom i tyroid follikelcelle, kombineret adenom eller karcinom i binyrebarken, cervikalt neurinom, stromale polypper og kombineret polyp eller sarkom i livmoderens endometrie.
The incidence of the following tumours was also increased in female rats: thyroid follicular cell adenoma, carcinoma and combined adenoma or carcinoma, adrenal cortical combined adenoma or carcinoma, cervical schwannoma, uterine endometrial stromal polyp, and combined polyp or sarcoma.
De gennemsnitlige maksimale koncentrationer (Cmax) af PBA, PAA og PAGN var hhv. 37,0 mikrogram/ml, 14,9 mikrogram/ml og 30,2 mikrogram/l.
Mean maximum concentration (Cmax) for PBA, PAA, and PAGN was 37.0 micrograms/ml, 14.9 micrograms/ml, and 30.2 micrograms/ml, respectively.
Hos raske, fastende voksne forsøgspersoner, som fik en enkelt oral dosis på 2,9 ml/m2 glycerolphenylbutyrat, forekom spidsniveauer af PBA, PAA, og PAGN i plasma efter hhv. 2 timer, 4 timer og 4 timer.
In healthy, fasting adult subjects receiving a single oral dose of 2.9 ml/m2 of glycerol phenylbutyrate, peak plasma levels of PBA, PAA, and PAGN occurred at 2 h, 4 h, and 4 h, respectively.
RAVICTI 1,1 g/ml oral væske Glycerolphenylbutyrat
RAVICTI 1.1 g/ml oral liquid Glycerol phenylbutyrate
Farmakokinetikken af glycerolphenylbutyrat er ikke blevet undersøgt hos patienter med nedsat nyrefunktion, herunder patienter med nyresygdom i slutstadiet (end-stage renal disease, ESRD) eller patienter i hæmodialyse.
The pharmacokinetics of glycerol phenylbutyrate in patients with impaired renal function, including those with end-stage renal disease (ESRD) or those on haemodialysis, have not been studied.
I et yderligere langtids- (24 måneder), ukontrolleret, åbent klinisk studie blev RAVICTIs sikkerhed evalueret hos 16 UCD-patienter under 2 måneder og hos 10 pædiatriske patienter med UCD'er i alderen fra 2 måneder til under 2 år.
In an additional long term (24 month), uncontrolled, open-label clinical study the safety of RAVICTI has been evaluated in 16 UCD patients less than 2 months of age and 10 paediatric patients with UCDs aged 2 months to less than 2 years.
• hævede led, muskelsammentrækninger, smerter i hænder og fødder, hælsmerter
• joint swelling, muscle spasms, pain in the hands and feet, heel pain
RAVICTI 1,1 g/ml oral væske
RAVICTI 1.1 g/ml oral liquid
Dosisjustering baseret på phenylacetylglutamin i urinen U-PAGN-målinger kan anvendes som vejledning ved dosisjustering af glycerolphenylbutyrat og til at bedømme komplians.
Adjustment based on urinary phenylacetylglutamine U-PAGN measurements may be used to help guide glycerol phenylbutyrate dose adjustment and assess compliance.
• virusinfektion i tarmene
• viral infection in the gut
Hvis noget af ovenstående gælder for dig (eller hvis du er i tvivl), skal du spørge lægen eller apotekspersonalet, før du tager RAVICTI.
If any of the above apply to you (or you are not sure), talk to your doctor or pharmacist before taking RAVICTI.
Ammoniakniveauet skal monitoreres tæt, når glycerolphenylbutyrat-dosis ændres.
Ammonia levels must be monitored closely when changing the dose of glycerol phenylbutyrate.
• stikkende og prikkende fornemmelse, udpræget rastløshed, besvær med at vågne, døsighed, talebesvær, forvirret eller deprimeret tilstand
• feeling of pins and needles, feeling very restless, difficulty in waking up, feeling drowsy, problems with speech, feeling confused, depressed
Yderligere bivirkninger hos børn under 2 måneder
Additional side effects in children less than 2 months of age
Begyndelsesdosis til patienter, som skifter fra natriumphenylbutyrat til RAVICTI:
Initial dosage in patients switching from sodium phenylbutyrate to RAVICTI:
Hos voksne UCD-patienter, som fik flere doser glycerolphenylbutyrat, optrådte maksimale steady state plasmakoncentrationer (Cmaxss) af PBA, PAA og PAGN hhv. 8 timer, 12 timer og 10 timer efter dagens første dosis.
In adult UCD patients receiving multiple doses of glycerol phenylbutyrate, maximum plasma concentrations at steady state (Cmaxss) of PBA, PAA, and PAGN occurred at 8 h, 12 h, and 10 h, respectively, after the first dose in the day.
Yderligere bivirkninger er blevet evalueret i et klinisk studie, der inkluderede 16 UCD-patienter under 2 måneder.
Additional adverse reactions have been evaluated in a clinical study including 16 UCD patients less than 2 months of age.
Monitorer ammoniakniveauet nøje, når kortikosteroider og glycerolphenylbutyrat anvendes samtidig.
Monitor ammonia levels closely when corticosteroids and glycerol phenylbutyrate are used concomitantly.
I alt 51 voksne patienter med defekter i CPS, OTC, ASS, ASL, ARG og HHH blev inkluderet i forsøget, og alle med undtagelse af 6 var blevet omstillet fra natriumphenylbutyrat til ækvivalente doser glycerolphenylbutyrat.
A total of 51 adult patients involving deficiencies of CPS, OTC, ASS, ASL, ARG, and HHH were enrolled in the study and all but 6 had been converted from sodium phenylbutyrate to equivalent doses of glycerol phenylbutyrate.
Brug af anden medicin sammen med RAVICTI
Other medicines and RAVICTI
I alt 16 pædiatriske patienter med UCD'er i alderen under 2 måneder deltog i et langtids(24 måneder), ukontrolleret, åbent studie, hvor 10 patienter konverterede fra natriumphenylbutyrat til RAVICTI.
A total of 16 paediatric patients with UCDs aged less than 2 months participated in a long-term (24 months), uncontrolled, open-label study, of which 10 patients converted from sodium phenylbutyrate to RAVICTI.
Hvis noget af ovenstående gælder for dig (eller hvis du er i tvivl), skal du spørge lægen, før du tager RAVICTI.
If any of the above apply to you (or you are not sure), talk to your doctor before taking RAVICTI.
Ikke almindelig: kan påvirke op til 1 ud af 100 personer mundtørhed, ændret smagssans
Uncommon: may affect up to 1 in 100 people
Den gennemsnitlige eksponering var 10 måneder (interval 2-20 måneder) og den gennemsnitlige eksponering hos patienter i alderen 2 måneder til under 2 år var 9 måneder (interval 0,2-20,3 måneder).
The median exposure was 10 months (range 2 to 20 months) and median exposure in the 2 months to less than 2 years of age was 9 months (range 0.2 to 20.3 months).Adverse drug reactions (ADR) are summarized below.
Ikke almindelig Ventrikulær arytmi
Uncommon Ventricular arrhythmia
Flasken er pakket sammen med en genlukkelig lågadapter.
The bottle is packed with a reclosable bottle cap adapter.
• 7 ml/m2/dag (8 g/m2/dag) hos patienter med BSA ≥ 1,3 m2
• 7 ml/m2/day (8 g/m2/day) in patients with a BSA ≥ 1.3 m2
Vend flasken på hovedet med den orale sprøjte siddende i.
Turn the bottle upside down with the oral syringe still inserted.
Synk væsken fra den orale sprøjte eller fastgør sprøjten til en gastrostomisonde eller nasogastrisk sonde.
Swallow the liquid from the oral syringe or attach the oral syringe to a gastrostomy or nasogastric tube.
RAVICTI er indiceret som tillægsbehandling med henblik på kronisk behandling af patienter med urinstofcyklus-defekter (UCD'er), herunder defekter i carbamylfosfat-syntetase I (CPS), ornithintranscarbamylase (OTC), argininoravsyre-syntetase (ASS), argininoravsyrelyase (ASL), arginase I (ARG) og ornithin-translokase-mangel hyperornithinæmi-hyperammoniæmi homocitrullinuri-syndrom (HHH), som ikke alene kan behandles ved begrænsning af protein i kosten og/eller tilskud af aminosyrer.
RAVICTI is indicated for use as adjunctive therapy for chronic management of patients with urea cycle disorders (UCDs) including deficiencies of carbamoyl phosphate synthetase I (CPS), ornithine carbamoyltransferase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase I (ARG) and ornithine translocase deficiency hyperornithinaemia-hyperammonaemia homocitrullinuria syndrome (HHH) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone.
• oralt (gennem munden)
• by mouth
Glycerolphenylbutyrat bør ikke anvendes under graviditet og til kvinder i den fertile alder, som ikke anvender sikker kontraception.
Glycerol phenylbutyrate is not recommended during pregnancy and in women of childbearing potential not using contraception.
Den samme adapter kan bruges, indtil flasken er tom.
The same reclosable bottle cap adapter can be used until a bottle is empty.
Brug én oral sprøjte til alle doserne samme dag.
Use one oral syringe for all doses taken each day.
RAVICTI skal bruges med proteinrestriktion i kosten og undertiden kosttilskud (f.eks. essentielle aminosyrer, arginin, citrullin, proteinfrit kalorietilskud) afhængigt af, hvor stor en daglige indtagelse af protein i kosten der skal til for at fremme vækst og udvikling.
RAVICTI must be used with dietary protein restriction and sometimes dietary supplements (e.g., essential amino acids, arginine, citrulline, protein-free calorie supplements) depending on the daily dietary protein intake needed to promote growth and development.
Vend flasken på hovedet med den orale sprøjte siddende i. Fyld sprøjten ved at trække stemplet tilbage, indtil sprøjten er fyldt med den ønskede mængde lægemiddel.
Fill the oral syringe by pulling the plunger back until the syringe is filled with the amount of medicinal product.
RAVICTIs udseende og pakningsstørrelser
What RAVICTI looks like and contents of the pack
Tre patienter var behandlingsnaive, og hos tre yderligere patienter blev intravenøs natriumbenzoat og natriumphenylacetat gradvist seponeret, mens RAVICTI blev påbegyndt.
Three patients were treatment naïve and three additional patients were gradually discontinued from intravenous sodium benzoate and sodium phenylacetate while RAVICTI was initiated.
• 8,5 ml/m2/dag (9,4 g/m2/dag) hos patienter med kropsoverfladeareal (BSA) < 1,3 m2
• 8.5 ml/m2/day (9.4 g/m2/day) in patients with a body surface area (BSA) < 1.3 m2
• Trin 2: 50 % dosis af natriumphenylacetat/natriumbenzoat og 100 % RAVICTI i 4-8 timer
• Step 2: 50% dose sodium phenylacetate/sodium benzoate and 100% RAVICTI for 4-8 hours;
Kemisk kompatibilitet af glycerolphenylbutyrat med nasogastriske, nasojejunale eller gastrostomisonder af silikone af medicinsk kvalitet er blevet påvist.
Chemical compatibility of glycerol phenylbutyrate with medical grade silicone nasogastric, gastrostomy, and nasojejunal tubes has been demonstrated.
Glycerolphenylbutyrat var non-inferiort i forhold til natriumphenylbutyrat med hensyn til AUC0-24t for ammoniak.
Glycerol phenylbutyrate was non-inferior to sodium phenylbutyrate with respect to the 24-hour AUC for ammonia.
Høje niveauer af ammoniak forårsager kvalme, opkastning eller forvirring.
High levels of ammonia leads to feeling sick (nausea), being sick (vomiting) or feeling confused.
Når patienter, som skifter fra natriumphenylacetat/natriumbenzoat til RAVICTI, er stabile med kontrolleret ammoniak bør de få en dosis RAVICTI i den højere ende af behandlingsområdet (11,2 ml/m2/dag) med måling af ammoniak i plasma som vejledning for yderligere dosering.
Initial dosage in patients switching from sodium phenylacetate/sodium benzoate injection to RAVICTI: Once stable with controlled ammonia, patients switching from sodium phenylacetate/sodium benzoate to RAVICTI should receive a dose of RAVICTI at the higher end of the treatment range (11.2 ml/m2/day) with measurements of plasma ammonia to guide further dosing.
In vitro blev 80,6 % til 98,0 % af 14C-mærkede metabolitter af PBA (i intervallet 1-250 mikrogram/ml) bundet til plasmaprotein, og 37,1 % til 65,6 % af 14C-mærkede metabolitter af PAA (i intervallet 5-500 mikrogram/ml).
In vitro, the extent of human plasma protein binding for 14C-labeled metabolites was 80.6% to 98.0% for PBA (over 1-250 microgram/ml), and 37.1% to 65.6% for PAA (over 5-500 microgram /ml).
Ud af 49 pædiatriske patienter, som deltog i disse forlængelsesforsøg, rapporterede 12 patienter (25 %) i alt 17 hyperammoniæmiske kriser under behandling med glycerolphenylbutyrat sammenlignet med 38 kriser hos 21 patienter (43 %) under behandling med natriumphenylbutyrat i de 12 måneder forud for studieinklusion.
Of the 49 paediatric patients who participated in these extension studies, 12 patients (25 %) reported a total of 17 hyperammonaemic crises during treatment with glycerol phenylbutyrate as compared with 38 crises in 21 patients (43 %) in the preceding 12 months prior to study entry, while they were being treated with sodium phenylbutyrate.
Glycerolphenylbutyrat hydrolyseres af pancreaslipaser til PBA, som ved beta-oxidation omdannes til PAA, den aktive del af glycerolphenylbutyrat.
Glycerol phenylbutyrate is hydrolysed by pancreatic lipases to yield, PBA, which is converted by beta oxidation to PAA, the active moiety of glycerol phenylbutyrate.
Den daglige dosis skal justeres individuelt i henhold til patientens proteintolerance og den daglige mængde protein, der er behov for at indtage gennem kosten.
The daily dose should be individually adjusted according to the patient's protein tolerance and the daily dietary protein intake needed.
Studie med børn i alderen 2 måneder til under 2 år I alt 10 pædiatriske patienter med UCD'er i alderen 2 måneder til under 2 år deltog i et langtids(24 måneder), ukontrolleret, åbent studie, hvoraf 6 patienter konverterede fra natriumphenylbutyrat til RAVICTI, og 1 patient konverterede fra natriumphenylbutyrat og natriumbenzoat.
Study in Children 2 Months to Less than 2 Years of Age A total of 10 paediatric patients with UCDs aged 2 months to less than 2 years participated in a long term (24 months) uncontrolled, open label study, of which 6 patients converted from sodium phenylbutyrate to RAVICTI and 1 patient converted from sodium phenylbutyrate and sodium benzoate.
I et studie hos patienter med klinisk dekompenseret cirrose og hepatisk encefalopati (Child-Pugh B og C), var den gennemsnitlige Cmax af PAA 144 mikrogram/ml (14-358 mikrogram/ml) efter en daglig dosis på 6 ml glycerolphenylbutyrat to gange dagligt, og den gennemsnitlige Cmax af PAA var 292 mikrogram/ml (57-655 mikrogram/ml) efter en daglig dosis på 9 ml glycerolphenylbutyrat to gange dagligt.
In a study in patients with clinically decompensated cirrhosis and hepatic encephalopathy (Child-Pugh B and C), mean Cmax of PAA was 144 mcg/ml (range: 14-358 mcg /ml) after daily dosing of 6 ml of glycerol phenylbutyrate twice daily, while mean Cmax of PAA was 292 mcg /ml (range: 57-655 mcg/ml) after daily dosing of 9 ml of glycerol phenylbutyrate twice daily.
I alt 49 børn i alderen fra 2 måneder til 17 år med defekter i OTC, ASS, ASL og ARG blev inkluderet, og alle med undtagelse af 1 var blevet omstillet fra natriumphenylbutyrat til glycerolphenylbutyrat.
A total of 49 children ages 2 months to 17 years with deficiencies of OTC, ASS, ASL, and ARG were enrolled, and all but 1 had been converted from sodium phenylbutyrate to glycerol phenylbutyrate.
Monitorer ammoniakniveauet nøje, når det er nødvendigt at behandle UCD-patienter med valproinsyre eller haloperidol.
Monitor ammonia levels closely when use of valproic acid or haloperidol is necessary in UCD patients.
Samtidig brug af lægemidler, der vides at hæmme lipase, skal gives med forsigtighed, da glycerolphenylbutyrat hydrolyseres til phenylbutansyre og glycerol af fordøjelsesenzymet lipase.
Concomitant use of medicinal products known to inhibit lipase should be given with caution as glycerol phenylbutyrate is hydrolysed by digestive lipase into phenylbutyrate acid and glycerol.
Ud af 51 voksne patienter, som deltog i studie 2, rapporterede 7 patienter (14 %) i alt 10 hyperammoniæmiske kriser under behandling med glycerolphenylbutyrat sammenlignet med 9 patienter (18 %), som havde rapporteret i alt 15 kriser under behandling med natriumphenylbutyrat i de 12 måneder forud for studieinklusion.
Of 51 adult patients participating in Study 2, 7 patients (14%) reported a total of 10 hyperammonaemic crises during treatment with glycerol phenylbutyrate as compared with 9 patients (18 %) who had reported a total of 15 crises in the 12 months prior to study entry while they were being treated with sodium phenylbutyrate.
Reduceret absorption af phenylbutyrat ved pancreasinsufficiens eller intestinal malabsorption Eksokrine pancreasenzymer hydrolyserer glycerolphenylbutyrat i tyndtarmen og spalter den aktive del, phenylbutyrat, fra glycerol.
Reduced phenylbutyrate absorption in pancreatic insufficiency or intestinal malabsorption Exocrine pancreatic enzymes hydrolyse glycerol phenylbutyrate in the small intestine, separating the active moiety, phenylbutyrate, from glycerol.
• kortikosteroider - anvendes til behandling af inflammation (betændelseslignende tilstande) i kroppen
• corticosteroids - used to treat inflamed areas of the body
Farmakologisk virkning
Pharmacological effects