natgillin/translations-raw huggingface dataset:
Ikke almindelig: Ændret hårfarve, striae i huden, skrøbelig hud (molluskoide pseudotumorer på albuerne)
Uncommon: Hair colour changes, skin striae, skin fragility (molluscoid pseudotumour on elbows)
Ved oral og parenteral indgift af høje doser cysteamin opstår der ulcus duodeni hos rotter og mus, men ikke hos aber.
High doses of cysteamine, either by oral or parenteral routes, produce duodenal ulcers in rats and mice but not in monkeys.
Dette lægemiddel kan administreres ved at sluge de intakte kapsler samt drysse kapselindholdet (enterokorn) på mad eller det kan administreres via en mavesonde.
This medicinal product can be administered by swallowing the intact capsules as well as sprinkling the capsule contents (enteric coated beads) on food or delivery through a gastric feeding tube.
Dette lægemiddel indeholder mindre end 1 mmol (23 mg) natrium pr. dosis, dvs. det er i det væsentlige natrium-frit.
This medicine contains less than 1 mmol sodium (23 mg) per dose, that is to say essentially “sodiumfree.”
Bivirkningerne omfatter hovedsagelig mave-tarmsystemet og centralnervesystemet.
These mainly involve the gastrointestinal and central nervous systems.
Prøverne skal tages 12,5 timer efter den foregående dags aftendosis, dvs. 30 minutter efter den påfølgende morgendosis.
These tests must be obtained 12.5 hours after the evening dose the day before, and therefore 30 minutes after the following morning dose is given.
Hele cysteaminkapsler bør ikke gives til børn under seks år på grund af risikoen for, at de får dem i den gale hals (se afsnit 3 Sådan skal du tage PROCYSBI - Administration).
Whole cysteamine capsules should not be given to children under the age of 6 years due to the risk of choking (refer to section 3 How to take PROCYSBI - Method of administration).
Administration af cysteamin til nyfødte rotter medførte katarakt.
Administration of cysteamine in neonate rats induced cataracts.
Behandlingsvarighed Behandlingen med PROCYSBI er livslang og skal følge lægens anvisninger.
Duration of treatment Treatment with PROCYSBI should continue life-long, as instructed by your doctor.
• Mulighed 1 / Sprøjte: Der omrøres forsigtigt i 5 minutter, hvorefter blandingen af korn af cysteamin og sur frugtjuice eller vand suges op i en doseringssprøjte.
• Option 1 / Syringe: Mix gently for 5 minutes, then aspirate the mixture of cysteamine granules and acidic fruit juice or water into a dosing syringe.
Cystinose er en stofskiftesygdom, der påvirker kroppens funktioner og medfører unormal ophobning af aminosyren cystin i en række organer, såsom nyrerne, øjnene, musklerne, bugspytkirtlen og hjernen.
Cystinosis is a disease affecting how the body functions, with an abnormal accumulation of the amino acid cystine in various organs of the body such as the kidney, eye, muscle, pancreas, and brain.
For lavt antal hvide blodlegemer.
Low white blood cell count.
- Der skal regelmæssigt tages blodprøver til bestemmelse af cystin i de hvide blodlegemer og/eller cysteamin i blodet, for at den rigtige dosis af PROCYSBI kan fastsættes.
- Regular blood tests to measure the amount of cystine inside white blood cells and/or the concentration of cysteamine in the blood are necessary to help determine the correct dose of PROCYSBI.
Fødeindtagelse nedsætter absorptionen af PROCYSBI: Fødeindtagelse 30 minutter før dosering medfører således ca. 35 % lavere eksponering, og fødeindtagelse 30 minutter efter dosering medfører ca. 16 og 45 % lavere eksponering for henholdsvis hele og åbnede kapsler).
Food intake reduces the absorption of PROCYSBI at 30 minutes pre-dose (approximately 35% decrease in exposure) and at 30 min post-dose (approximately 16 or 45% decrease in exposure for intact and open capsules respectively).
Spørg på apoteket, hvordan du skal bortskaffe medicinrester.
Ask your pharmacist how to throw away the medicines you no longer use.
Reproduktionsundersøgelser viste embryoføtale toksiske effekter (resorptioner og postimplantationstab) hos rotter ved et dosisniveau på 100 mg/kg/dag og hos kaniner ved en cysteamindosis på 50 mg/kg/dag.
Reproduction studies showed embryo-foetotoxic effects (resorptions and post-implantation losses) in rats at the 100 mg/kg/day dose level and in rabbits receiving cysteamine 50 mg/kg/day.
Desuden skal der regelmæssigt tages prøver af blod og urin for at bestemme vigtige elektrolytter, så lægen kan tilpasse doserne af disse kosttilskud.
Regular blood and urine tests to measure the levels of the body's important electrolytes are also necessary to help you or your doctor correctly adjust the doses of these supplements.
Læger og sundhedspersonale skal referere til analysespecifikke behandlingsmål fra de individuelle testlaboratorier, når de tager beslutninger om diagnose og PROCYSBI-dosering for patienter med cystinose.
Healthcare professionals should refer to the assay-specific therapeutic targets provided by individual testing laboratories when making decisions regarding diagnosis and PROCYSBI dosing for cystinosis patients.
DATO FOR FØRSTE MARKEDSFØRINGSTILLADELSE/FORNYELSE AF TILLADELSEN Dato for første markedsføringstilladelse: 06.09.2013 Dato for seneste fornyelse: 26.07.2018
DATE OF FIRST AUTHORISATION/RENEWAL OF THE AUTHORISATION Date of first authorisation: 06 September 2013
Almindelig: Leverfunktionsprøver abnorme
Common: Liver function tests abnormal
Vigtig information om nogle af hjælpestofferne i PROCYSBI Dette lægemiddel indeholder mindre end 1 mmol (23 mg) natrium pr. dosis, dvs. det er i det væsentlige natrium-frit.
Important information about some of the excipients of PROCYSBI This medicinal product contains less than 1 mmol sodium (23 mg) per dose, i.e. essentially sodiumfree.
Administration af bikarbonat bør finde sted mindst en time før eller en time efter PROCYSBI for at undgå potentiel hurtigere udløsning af cysteamin.
Bicarbonate should be administered at least one hour before or one hour after PROCYSBI to avoid potential earlier release of cysteamine.
Hvis du har glemt at tage PROCYSBI
If you forget to take PROCYSBI
Stoffet medfører depletion af somatostatin ved eksperimentel indgift hos flere dyrearter.
Experimental administration of the drug causes depletion of somatostatin in several animal species.
Forandringerne består i mærker i huden (som ligner strækmærker), knogleskader (såsom brud), knogledeformiteter og ledproblemer.
These include skin striae (which are like stretch marks), bone injuries (such as fractures), bone deformities, and joint problems.
Behandlingen med PROCYSBI bør initieres under ledelse af en læge med erfaring i behandling af cystinose.
PROCYSBI treatment should be initiated under the supervision of a physician experienced in the treatment of cystinosis.
Låget er børnesikret og har folietætning.
The cap is child resistant and has a foil seal.
Hvis der opstår svært hududslæt såsom erythema multiforme bullosa eller toksisk epidermal nekrolyse, bør behandlingen med cysteamin ikke genoptages (se pkt.
If a severe skin rash develops such as erythema multiforme bullosa or toxic epidermal necrolysis, cysteamine should not be re-administered (see sections 4.8).
I kliniske undersøgelser med raske forsøgspersoner var de hyppigste bivirkninger gastrointestinale symptomer (16 %), hovedsageligt som enkeltepisoder af let eller moderat sværhed.
In clinical studies with healthy volunteers, the most frequent adverse reactions were very common GI symptoms (16%) and occurred primarily as single episodes that were mild or moderate in severity.
PROCYSBI er et lægemiddel, der reagerer med cystin og nedsætter cellernes indhold af cystin.
PROCYSBI is a medicine that reacts with cystine to decrease its level within the cells.
Cylindrene kan kasseres sammen med flasken efter brug.
The cylinders may be discarded with the bottle after use.
Farmakoterapeutisk klassifikation: Andre stofskiftesygdomme, midler mod cystinose, ATC-kode: A16AA04.
Pharmacotherapeutic group: Other alimentary tract and metabolism product, ATC code: A16AA04.
Hvis børn og patienter har problemer med at synke, henvises til afsnit 3 Sådan skal du tage PROCYSBI - Administration.
For children and patients who have problems to swallow, please refer to section 3 How to take PROCYSBIMethod of administration.
Informationsmaterialet til læger skal indeholde sikkerhedstjeklisten, produktresuméet og indlægssedlen.
The physician education pack should contain the Safety Checklist, the Summary of Product Characteristics and Package Leaflet.
Administration sammen med et måltid
Administration with food
• Mulighed 2 / Kop: Blandingen omrøres forsigtigt i 5 minutter i en kop eller rystes forsigtigt i fem minutter i en kop med låg (f.eks. med drikketud).
• Option 2 / Cup: Mix gently for 5 minutes in a cup or shake gently for 5 minutes in a covered cup (e.g., “sippy” cup).
• Overfølsomhed over for det aktive stof, enhver form for cysteamin (mercaptamin) eller over for et eller flere af hjælpestofferne anført i pkt.
• Hypersensitivity to the active substance, any form of cysteamine (mercaptamine), or to any of the excipients listed in section 6.1.
Der må ikke tages en dobbelt dosis.
Do not double the dose.
Hvis der konstateres eller planlægges graviditet, bør behandlingen tages op til nøje overvejelse, og patienten skal oplyses om den teratogene risiko ved cysteamin.
If a pregnancy is diagnosed or planned, the treatment should be carefully reconsidered and the patient must be advised of the possible teratogenic risk of cysteamine.
• Anvisninger vedrørende risikoen for fibroserende colonsygdom og relevante anvisninger for risikominimering: patienterne skal informeres om den potentielle risiko for fibroserende colonsygdom patienterne skal informeres om tegn og symptomer på fibroserende colonsygdom og om at kontakte den behandlende læge, hvis sådanne tegn eller symptomer opstår.
• The risk of fibrosing colonopathy and relevant risk minimisation advice: Patients should be informed about the potential risk of fibrosing colonopathy; Patients should be advised of the signs and symptoms of fibrosing colonopathy and to alert the treating physician if they develop any.
Kapselindhold cellulose, mikrokrystallinsk methacrylsyre-ethylacrylat copolymer (1:1) hypromellose talcum triethylcitrat natriumlaurilsulfat
Capsule content microcrystalline cellulose methacrylic acid - ethyl acrylate copolymer (1:1) hypromellose talc triethyl citrate sodium lauryl sulphate
Dette kan efterfølges af 250 ml af en acceptabel sur drikfrugtjuice (f.eks. appelsinjuice eller enhver anden sur frugtjuice) eller vand.
This may be followed by 250 mL of an acceptable acidic liquid - fruit juice (e.g., orange juice or any acidic fruit juice) or water.
Der kræves jævnlig øjenundersøgelse for at diagnosticere denne tilstand tidligt og i givet fald behandle den rettidigt, så synstab undgås.
A periodic eye examination is needed to identify this condition early and timely treatment should be provided when it occurs to prevent vision loss.
Patienter, der overgår fra cysteaminbitartrat med hurtig udløsning til PROCYSBI, bør have kontrolleret leukocytternes cystinindhold inden for 2 uger og efterfølgende hver tredje måned for at vurdere, om dosis er optimal som ovenfor beskrevet.
Patients being transferred from immediate-release cysteamine bitartrate to PROCYSBI should have their WBC cystine levels measured in 2 weeks, and thereafter every 3 months to assess optimal dose as described above.
Ved indledende dårlig tolerabilitet af cysteamin på grund af gastrointestinale symptomer eller forbigående hududslæt bør behandlingen midlertidigt seponeres for derefter at genoptages med en lavere dosering, der gradvis øges til det hensigtsmæssige niveau (se pkt.
If cysteamine is initially poorly tolerated due to gastrointestinal (GI) tract symptoms or transient skin rashes, therapy should be temporarily stopped, then re-instituted at a lower dose and gradually increased to the appropriate dose (see section 4.4).
Gastrointestinale risici
Gastrointestinal disorders
Cysteaminbitratrat kan administreres sammen med en syrlig frugtjuice eller vand.
Cysteamine bitartrate can be administered with an acidic fruit juice or water.
Blandingen gives derefter gennem gastrostomisonde, nasogastrisk sonde eller gastrostomi-jejunostomi-sonde.
The mixture should then be administered via gastrostomy tube, nasogastric tube or gastrostomy-jejunostomy tube.
Derfor skal læger og sundhedspersonale referere til analysespecifikke behandlingsmål fra de individuelle testlaboratorier.
Therefore, healthcare professionals should refer to the assay-specific therapeutic targets provided by individual testing laboratories.
Indtagelse sammen med appelsinjuice eller anden sur frugtjuice eller vand Kapslerne til den pågældende morgen- eller aftendosis åbnes, og indholdet drysses i 100-150 ml sur frugtjuice eller vand.
Sprinkling in orange juice or any acidic fruit juice or water Capsules for either the morning or evening dose should be opened and the contents sprinkled into 100 to 150 mL of acidic fruit juice or water.
Den tilsigtede vedligeholdelsesdosis er 1,3 g/m2/dag, fordelt på to doser med 12 timers mellemrum.
The targeted maintenance dose is 1.3 g/m2/day, in two divided doses, given every 12 hours.
Indtagelse ved drysning på maden
Sprinkling on food
Til patienter, der ikke kan synke hele kapsler, kan enterokapslerne åbnes, og indholdet drysses over maden (æblemos eller bærgelé) eller blandes i en sur drik (appelsinjuice eller anden sur juice) eller vand.
For patients who cannot swallow the whole capsule, the gastro-resistant hard capsule may be opened and the contents may be sprinkled on food (such as apple sauce or berry jelly) or mixed in with an acidic drink (such as orange juice or any acidic juice) or water.
Der er indberettet enkelte tilfælde, der minder om Ehlers-Danlos syndrom, på albuerne af børn i kronisk behandling med høje doser af forskellige cysteaminformuleringer (cysteaminhydrochlorid, cysteamin eller cysteaminbitartrat), hovedsageligt i højere dosis end den maksimale dosis på 1,95 g/m2/dag.
A few cases of Ehlers-Danlos-like syndrome on elbows have been reported in children chronically treated with high doses of different cysteamine preparations (cysteamine chlorhydrate or cystamine or cysteamine bitartrate) mostly above the maximal dose 1.95 g/m2/day.
Ved en eventuel overdosering bør der gives passende støttende behandling til opretholdelse af respiration og kredsløb.
Should overdosing occur, the respiratory and cardiovascular systems should be supported appropriately.
* målt med den blandede leukocytanalyse
* measured using the mixed leukocyte assay
De øvrige bivirkninger med PROCYSBI er anført nedenfor sammen med et skøn over deres hyppighed.
The other side effects listed below are given with an estimation of the frequency with which they may occur with PROCYSBI.
Bestemmelsen af cystin i leukocytter og/eller cysteamin i plasma skal ske 12,5 timer efter den foregående aftendosis, dvs. 30 minutter efter den påfølgende morgendosis.
The determination of WBC cystine and/or plasma cysteamine must be obtained 12.5 hours after the evening dose the day before, and therefore 30 minutes after the following morning dose is given.
Brug af PROCYSBI sammen med mad og drikke Du skal forsøge at undgå måltider med højt indhold af fedt eller proteiner i mindst 1 time før og 1 time efter, du tager PROCYSBI, samt madvarer eller væsker, der kan sænke syreindholdet i maven, som f.eks. mælk eller yoghurt.
For at least 1 hour before and 1 hour after taking PROCYSBI try to avoid meals, which are rich in fat or proteins as well as any food or liquid that could decrease the acidity in your stomach, like milk or yogurt.
Fortæl det straks til lægen, hvis du får mavesmerter, kvalme, opkastning, appetitløshed eller blodigt opkast.
Tell your doctor right away if you get stomach ache, nausea, vomiting, loss of appetite, or throw up blood.
Hos disse patienter anbefales tættere overvågning af leukocytternes cystinniveau.
A closer monitoring of the WBC cystine levels is recommended in these patients.
Strikturer på ileocøkum og colon (fibroserende colonsygdom) blev først beskrevet hos patienter med cystisk fibrose, der fik høje doser af pankreasenzymer som tabletter med enterocoating af methacrylsyre-ethylacrylat copolymer (1:1), der er et af hjælpestofferne i PROCYSBI.
Strictures of the ileo-caecum and large bowel (fibrosing colonopathy) was first described in cystic fibrosis patients who were given high doses of pancreatic enzymes in the form of tablets with an enteric coating of methacrylic acid - ethyl acrylate copolymer (1:1), one of the excipients in PROCYSBI.
Ikke almindelig: Nervøsitet, hallucinationer Almindelig: Hovedpine, encefalopati
Uncommon: Nervousness, hallucination Common: Headache, encephalopathy
Hvis dette ikke er muligt, kan du spise et lille måltid (ca. 100 g, fortrinsvis kulhydrater, f.eks. brød, pasta, frugt) i tidsrummet fra en time før til en time efter indtagelse af PROCYSBI.
If this is not possible, you can eat a small amount (about 100 grams) of food (preferably carbohydrates e.g. bread, pasta, fruits) during the hour before and after taking PROCYSBI.
Du vil regelmæssigt få undersøgt øjnene med henblik på denne tilstand, da rettidig behandling kan forebygge synstab.
You will receive regular eye examinations to identify this condition as early treatment can prevent vision loss.
Dosisplan
Dosing schedule
PROCYSBI 75 mg hård kapsel EU/1/13/861/002
PROCYSBI 75 mg hard capsule EU/1/13/861/002
EXP Skal kasseres 30 dage efter anbrud af folielukningen.
EXP Discard 30 days after opening the foil seal.
Ikke almindelig: Somnolens, kramper Meget almindelig: Opkastning, kvalme, diarré Almindelig: Abdominalsmerter, dårlig ånde, dyspepsi, gastroenteritis
Uncommon: Somnolence, convulsions Very common: Vomiting, nausea, diarrhoea Common: Abdominal pain, breath odour, dyspepsia, gastroenteritis
Overdosering med cysteamin kan medføre tiltagende letargi.
An overdose of cysteamine may cause progressive lethargy.
Cysteaminbitartrat hæmmer ikke OAT1, OAT3 eller OCT2.
Cysteamine bitartrate is not an inhibitor of OAT1, OAT3 and OCT2.
Cysteaminbitartrat i formuleringen med hurtig udløsning forventes at give bivirkninger hos ca. 35 % af patienterne.
For the immediate-release formulation of cysteamine bitartrate, approximately 35% of patients can be expected to experience adverse reactions.
•Behovet for at instruere patienten om: indtagelsesmåde og tidspunkt for indtagelse af lægemidlet, at patienten skal kontakte lægen: ved hudproblemer eller hudforandringer ved forstyrrelser i afføringsvanerne ved sløvhed, øget søvnbehov, depression eller krampeanfald ved enhver mistanke om at være gravid
•The need to advise patients about: The method of administration and timing of medicine intake The need to contact the treating physician if they experience the following events: Problems or changes with their skin Upset in their normal bowel habit, Lethargy, somnolence depression, fits Any suspicion that they might be pregnant
Hvis der konstateres eller planlægges graviditet, bør behandlingen tages op til nøje overvejelse, og patienten skal oplyses om den teratogene risiko ved cysteamin (se pkt.
If a pregnancy is diagnosed or planned, the treatment should be carefully reconsidered and the patient must be advised of the possible teratogenic risk of cysteamine (see section 4.6).
Hyppighed: bivirkning Meget almindelig: Letargi, pyreksi
Uncommon: Nephrotic syndrome Very common: Lethargy, pyrexia
Der er beskrevet teratogene virkninger hos rotter ved indgift af cysteamin i en dosis på 100 mg/kg/dag i hele organogeneseperioden.
Teratogenic effects have been described in rats when cysteamine is administered over the period of organogenesis at a dose of 100 mg/kg/day.
Histologisk sås i det ene tilfælde membranøs glomerulonefritis af det renale allotransplantat, og i det andet interstitiel nefritis som følge af hypersensitivitet.
Histology showed a membranous glomerulonephritis of the renal allograft in one case and hypersensitivity interstitial nephritis in the other.
Det er ikke påvist, at oral indtagelse af cysteamin forhindrer aflejringer af cystinkrystaller i øjet.
Oral cysteamine has not been shown to prevent eye deposition of cystine crystals.
INFORMATION I BRAILLESKRIFT PROCYSBI 25 mg
INFORMATION IN BRAILLE PROCYSBI 25 mg
Det kan ikke udelukkes, at cysteamin medfører klinisk relevant induktion af CYP-enzymer, hæmning af P-gp og BCRP i tarmen og hæmning af transportproteiner for optagelse i leveren (OATP1B1, OATP1B3 and OCT1).
It cannot be excluded that cysteamine is a clinically relevant inducer of CYP enzymes, inhibitor of P-gp and BCRP at the intestinal level and inhibitor of liver uptake transporters (OATP1B1, OATP1B3 and OCT1).
PROCYSBI 25 mg hård kapsel EU/1/13/861/001
PROCYSBI 25 mg hard capsule EU/1/13/861/001
Erfaringer fra kliniske undersøgelser med PROCYSBI I kliniske undersøgelser, der sammenlignede PROCYSBI med cysteaminbitartrat med hurtig udløsning, fik en tredjedel af patienterne meget hyppige gastrointestinale forstyrrelser (kvalme, opkastning, abdominalsmerter).
In clinical studies comparing PROCYSBI to the immediate-release cysteamine bitartrate, one third of the patients exhibited very common GI disorders (nausea, vomiting, abdominal pain).
In vitro: Cysteaminbitartrat er et substrat for P-gp og OCT2, men ikke for BCRP, OATP1B1, OATP1B3, OAT1, OAT3 eller OCT1.
In vitro: Cysteamine bitartrate is a substrate of P-gp and OCT2, but not a substrate of BCRP, OATP1B1, OATP1B3, OAT1, OAT3 and OCT1.
En randomiseret farmakokinetisk og farmakodynamisk afgørende fase 3-PK og PD overkrydsningsundersøgelse (som også var den første randomiserede undersøgelse nogensinde med cysteaminbitartrat med hurtig udløsning) viste, at patienter i steady-state behandling med PROCYSBI hver 12. time (Q12H) vedvarende var relativt depleteret for cystin i leukocytterne i forhold til cysteaminbitartrat med hurtig udløsning hver 6. time (Q6H).
A pivotal phase 3 randomized, crossover PK and PD study (which was also the first ever randomized study with immediate-release cysteamine bitartrate) demonstrated that at steady-state, patients receiving PROCYSBI every 12 hours (Q12H) maintained a comparable depletion of WBC cystine levels compared to immediate-release cysteamine bitartrate every 6 hours (Q6H).
Hos raske forsøgspersoner var bivirkningsprofilen den samme som hos patienter hvad angår gastrointestinale forstyrrelser (diarré og abdominalsmerter).
The adverse reactions profile for healthy subjects was similar to the adverse reactions profile in patients relative to GI disorders (diarrhoea and abdominal pain).
Cystinkoncentrationen i leukocytterne kan for eksempel måles ved flere forskellige teknikker, såsom specifikke undergrupper af leukocytter (f.eks. granulocytanalyse) eller den blandede leukocytanalyse, hvor hver analyse har forskellige målværdier.
White blood cell (WBC) cystine concentration may for instance be measured by a number of different techniques such as specific WBC subsets (e.g., granulocyte assay) or the mixed leukocyte assay with each assay having different target values.
PROCYSBI er indiceret til behandling af bevist nefropatisk cystinose.
PROCYSBI is indicated for the treatment of proven nephropathic cystinosis.
I denne undersøgelse var leukocytternes cystinindhold, målt med den blandede leukocytanalyse, altid i gennemsnit under optimal kontrol med < 1 nmol hemicystin/mg protein.
In this study, the WBC cystine measured using the mixed leukocyte assay was always on average under optimal control at < 1 nmol hemicystine/mg protein.
Skift fra cysteaminbitartrat, hårde kapsler, med hurtig udløsning Patienter med cystinose, der er i behandling med cysteaminbitartrat med hurtig udløsning, kan skifte til en samlet daglig dosis af PROCYSBI, der svarer til den tidligere samlede daglige dosis af cysteaminbitartrat med hurtig udløsning.
Transferring patients from immediate-release cysteamine bitartrate hard capsules Patients with cystinosis taking immediate-release cysteamine bitartrate may be transferred to a total daily dose of PROCYSBI equal to their previous total daily dose of immediate-release cysteamine bitartrate.
Den anbefalede maksimale dosis af cysteamin er 1,95 g/m2/dag.
The maximum recommended dose of cysteamine is 1.95 g/m2/day.