natgillin/translations-raw huggingface dataset:
Der blev randomiseret 43 patienter: 27 børn i alderen 6-12 år, 15 unge voksne i alderen 12-21 år og 1 voksen med cystinose og med nativ nyrefunktion baseret på en skønnet glomerulær filtrationshastighed (GFR) (korrigeret for kropsoverfladeareal) > 30 ml/minut/1,73 m2. Af disse 43 patienter udgik 2 søskende i slutningen af den første overkrydsningsperiode, den ene på grund af en planlagt operation; 41 patienter gennemførte protokollen.
Forty-three (43) patients were randomized; twenty-seven (27) children (ages 6 to 12 years old), fifteen (15) adolescents (ages 12 to 21 years old) and one (1) adult with cystinosis and with native kidney function based on an estimated Glomerular Filtration Rate (GFR) (corrected for body surface area) > 30 mL/minute/1.73 m2
Hos nyretransplanterede patienter er afstødningshindrende medicin blevet anvendt sammen med cysteamin.
In cases of patients with kidney transplants, anti-rejection treatments have been used with cysteamine.
Patienten bør instrueres om at fortælle om ethvert af følgende symptomer: hovedpine, tinnitus, svimmelhed, kvalme, diplopi, sløret syn, synstab, smerter bag øjnene og smerter ved øjenbevægelser.
Physicians should instruct patients to report any of the following symptoms: headache, tinnitus, dizziness, nausea, diplopia, blurred vision, loss of vision, pain behind the eye or pain with eye movement.
Blandingen af korn af cysteamin og sur frugt juice eller vand drikkes.
Drink the mixture of cysteamine granules and acidic fruit juice or water.
INFORMATION I BRAILLESKRIFT PROCYSBI 75 mg
INFORMATION IN BRAILLE PROCYSBI 75 mg
Azoospermi er beskrevet hos mandlige patienter med cystinose.
Azoospermia has been reported in male cystinosis patients.
In vitro-data tyder på, at cysteaminbitartrat metaboliseres af en række forskellige CYP-enzymer, herunder CYP1A2, CYP2B6, CYP2C8, CYP2C9, CYP2C19, CYP2D6 og CYP2E1.
In vitro data suggests that cysteamine bitartrate is likely to be metabolized by multiple CYP enzymes, including CYP1A2, CYP2B6, CYP2C8, CYP2C9, CYP2C19, CYP2D6, and CYP2E1.
Måltider og mejeriprodukter bør konsekvent undgås i mindst 1 time før og 1 time efter doseringen med PROCYSBI.
Patients should try to consistently avoid meals and dairy products for at least 1 hour before and 1 hour after PROCYSBI dosing.
Tag kapslen med en sur drik (appelsinjuice eller anden syrlig juice) eller vand.
Take the capsule with an acidic drink (such as orange juice or any acidic juice) or water.
Almindelige bivirkninger: dårlig ånde og kropslugt halsbrand træthed
Common side effects: unpleasant breath and body odour heartburn tiredness
Den normale vedligeholdelsesdosis er 1,3 g/m2/dag.
The targeted maintenance dose is 1.3 g/m2/day.
KVALITATIV OG KVANTITATIV SAMMENSÆTNING PROCYSBI 25 mg hård kapsel
QUALITATIVE AND QUANTITATIVE COMPOSITION PROCYSBI 25 mg hard capsule
Det skal du vide, før du begynder at tage PROCYSBI
What you need to know before you take PROCYSBI
2 patienter blev udelukket fra pr.-protokol analysen, da deres cystinindhold i leukocytterne steg til over 2 nmol hemicystin/mg protein i perioden med behandling med cysteamin med hurtig udløsning.
Two (2) patients were excluded from the per-protocol analysis because their WBC cystine level increased over 2 nmol hemicystine/mg protein during the immediate-release cysteamine treatment period.
Aktivt stof: cysteamin (som mercaptaminbitartrat).
The active substance is cysteamine (as mercaptamine bitartrate).
MARKEDSFØRINGSTILLADELSESNUMMER (-NUMRE) EU/1/13/861/001
MARKETING AUTHORISATION NUMBER(S) EU/1/13/861/001
50 ml hvid HDPE-flaske indeholdende 60 kapsler med én to-i-en cylinder med tørremiddel og én iltabsorberende cylinder; børnesikret lukke af polypropylen.
50 mL white HDPE bottle containing 60 capsules with one 2-in-1 desiccant cylinder and one oxygen absorber cylinder, with a child resistant polypropylene closure.
Målværdierne angivet i produktresuméet er fastlagt med den blandede leukocytanalyse.
The target values provided in the SmPC are obtained from using the mixed leucocyte assay.
Hos sådanne patienter overvåges leukocytternes cystinindhold for at bestemme doseringens egnethed, idet målingen finder sted 30 minutter efter doseringen med PROCYSBI.
WBC cystine is monitored in these patients to determine adequacy of dosing, levels being measured 30 minutes after dosing when treated with PROCYSBI.
MARKEDSFØRINGSTILLADELSESNUMMER (-NUMRE) EU/1/13/861/002
MARKETING AUTHORISATION NUMBER(S) EU/1/13/861/002
Blandingen skal indgives (drikkes) senest 30 minutter efter tilberedning, og opbevares nedkølet fra tilberedningen til indtagelsen.
The mixture must be administered (drunk) within 30 minutes after preparation and must be refrigerated from the time of preparation to the time of administration.
Cysteaminbitartrat hæmmer ikke CYP1A2, CYP2A6, CYP2B6, CYP2C8, CYP2C9, CYP2C19, CYP2D6, CYP2E1 eller CYP3A4 in vitro.
Cysteamine bitartrate is not an inhibitor of CYP1A2, CYP2A6, CYP2B6, CYP2C8, CYP2C9, CYP2C19, CYP2D6, CYP2E1 and CYP3A4 in vitro.
Desuden sås hyppige symptomer fra nervesystemet (hovedpine, somnolens, letargi) og hyppige generaliserede symptomer (asteni).
Common nervous system disorders (headache, somnolence and lethargy) and common general disorders (asthenia) were also seen.
Det er ukendt, om cysteamin udskilles i human mælk.
Cysteamine excretion in human milk is unknown.
Erfaringer efter markedsføring med cysteaminbitartrat med hurtig udløsning I forbindelse med cysteaminbitartrat med hurtig udløsning er indberettet benign intrakraniel hypertension (eller pseudotumor cerebri) med papilødem, hudlæsioner, molluskoide pseudotumorer, striae i huden, skrøbelig hud, hyperekstension af led, smerter i benene, genu valgum, osteopeni, kompressionsfrakturer og skoliose (se pkt.
Post-marketing experience with immediate-release cysteamine bitartrate Benign intracranial hypertension (or pseudotumor cerebri (PTC)) with papilledema; skin lesions, molluscoid pseudotumors, skin striae, skin fragility; joint hyperextension, leg pain, genu valgum, osteopenia, compression fracture and scoliosis have been reported with immediate-release cysteamine bitartrate (see section 4.4).
Indometacin er hos nogle patienter blevet anvendt samtidig med cysteamin.
Indomethacin and cysteamine have been used simultaneously in some patients.
Det er ukendt, om cysteamin fjernes ved hæmodialyse.
It is not known if cysteamine is removed by haemodialysis.
Ved optræden af sådanne bivirkninger efter initiering af behandlingen med cysteamin kan der opnås bedre tolerabilitet ved midlertidig seponering og gradvis genoptagelse af behandlingen.
When these reactions appear at the initiation of cysteamine therapy, temporary suspension and gradual reintroduction of treatment may be effective in improving tolerance.
Ved behandling med cysteaminbitartrat er indberettet benign intrakraniel hypertension (eller pseudotumor cerebri) og/eller papilødem, der svandt ved tilføjelse af diuretikabehandling (erfaringer efter markedsføring med cysteaminbitartrat med hurtig udløsning).
There have been reports of benign intracranial hypertension (or pseudotumor cerebri (PTC)) and/or papilledema associated with cysteamine bitartrate treatment that has resolved with the addition of diuretic therapy (post-marketing experience with the immediate-release cysteamine bitartrate).
Sig det straks til lægen, hvis du får nogen af følgende symptomer, når du tager PROCYSBI: hovedpine, summen eller susen for ørerne, svimmelhed, kvalme, dobbeltsyn, sløret syn, synstab, smerte bag øjnene eller smerte ved øjenbevægelser.
Tell your doctor right away if you develop any of the following symptoms while taking PROCYSBI: headache, buzzing or "whooshing" sound in the ear, dizziness, nausea, double vision, blurry vision, loss of vision, pain behind the eye or pain with eye movement.
Den samlede dosis må normalt ikke være over 1,95 g/m2/dag.
The total usual dose should not exceed 1.95 g/m2/day.
Blandingen skal administreres senest 2 timer efter tilberedningen og kan opbevares nedkølet fra tilberedningen til indtagelsen.
The mixture must be administered within 2 hours after preparation and may be refrigerated from the time of preparation to the time of administration.
Hvis der er mindre end 4 timer til næste dosis, skal patienten dog overspringe den glemte dosis og fortsætte efter den faste doseringsplan.
However, if it is within 4 hours of the next dose, skip the missed dose and go back to the regular dosing schedule.
Hos dialysepatienter har visse former for cysteamin undertiden været dårligere tolereret (medfører flere bivirkninger).
Experience has occasionally shown that some forms of cysteamine are less well tolerated (i.e. leading to more adverse events) when patients are on dialysis.
Øvrige indholdsstoffer: o I kapslerne: mikrokrystallinsk cellulose, methacrylsyre-ethylacrylat copolymer (1:1),
The other ingredients are: o In the capsules: microcrystalline cellulose, methacrylic acid - ethyl acrylate copolymer
hypromellose, talcum, triethylcitrat, natriumlaurilsulfat.
(1:1), hypromellose, talc, triethyl citrate, sodium lauryl sulfate.
Behandlingen med cysteamin skal påbegyndes straks efter bekræftelse af diagnosen nefropatisk cystinose for at patienten skal få størst mulig fordel af behandlingen.
Cysteamine therapy should be initiated promptly after confirmation of the diagnosis of cystinosis to achieve maximum benefit.
Kapslerne til morgen- eller aftendosis åbnes, og indholdet drysses på ca. 100 gram æblemos eller frugtgelé.
Capsules for either the morning or evening dose should be opened and the contents sprinkled onto approximately 100 grams of apple sauce or berry jelly.
Dosis bør øges, hvis der er tilstrækkelig tolerance, og leukocytternes cystinindhold forbliver > 1 nmol hemicystin/mg protein (målt med den blandede leukocytanalyse).
The dose should be raised if there is adequate tolerance and the WBC cystine level remains > 1 nmol hemicystine/mg protein (when measured using the mixed leukocyte assay).
Patienter med nedsat nyrefunktion Normalt er dosisjustering ikke nødvendig, men leukocytternes cystinniveau bør følges.
Patients with renal impairment Dose adjustment is not normally required; however, WBC cystine levels should be monitored.
Hvis udslættet er af svær grad, skal behandlingen med cysteamin standses.
If the rash is severe, your doctor may discontinue cysteamine treatment.
In vivo koadministration af protonpumpehæmmeren omeprazol havde ingen indvirkning på eksponeringen for cysteaminbitartrat.
Co-administration of the proton pump inhibitor omeprazole and PROCYSBI in vivo showed no effects on cysteamine bitartrate exposure.
Kontakt straks lægen eller sundhedspersonalet, hvis du oplever en eller flere af nedenstående bivirkninger - du behøver muligvis øjeblikkelig lægehjælp: Svær allergisk reaktion (ikke almindelig): Søg akut lægehjælp ved nogen af disse tegn på en allergisk reaktion: nældefeber, vejrtrækningsbesvær, hævelse af ansigt, læber, tunge eller hals.
Severe allergic reaction (seen uncommonly): Get emergency medical help if you have any of these signs of an allergic reaction: hives; difficulty breathing; swelling of face, lips, tongue, or throat.
400 ml hvid HDPE-flaske med børnesikret lukke af polypropylen indeholdende 250 kapsler og en 2-i1-cylinder med tørremiddel og to iltabsorberende cylindre.
400 mL white HDPE bottle containing 250 capsules with one 2-in-1 desiccant cylinder and two oxygen absorber cylinders, with a child resistant polypropylene closure.
Behandlingen med cysteamin skal begynde, så snart diagnosen er bekræftet (dvs. forhøjet indhold af cystin i leukocytterne) for at patienten skal opnå størst mulig fordel af behandlingen.
Cysteamine therapy must be initiated promptly once the diagnosis is confirmed (i.e., increased WBC cystine) to achieve maximum benefit.
Kontakt lægen, hvis der er forandringer.
Report any changes to your doctor.
Hver enterokapsel indeholder 25 mg eller 75 mg cysteamin.
Each gastro-resistant hard capsule contains 25 mg or 75 mg of cysteamine.
CNS-symptomer såsom krampeanfald, letargi, somnolens, depression og encefalopati er blevet sat i forbindelse med cysteamin.
CNS symptoms such as seizures, lethargy, somnolence, depression, and encephalopathy have been associated with cysteamine.
Cystinindhold i leukocytter (mindste kvadraters gennemsnit ± standardfejl) i nmol hemicystin/mg protein*
WBC cystine level (LS Mean ± SE) in nmol hemicystine/mg protein*
Dyreforsøg har vist påvirkning af fertiliteten (se pkt.
Effects on fertility have been seen in animal studies (see section 5.3).
Hos personer med cystinose er leukocytternes cystinindhold forhøjet til over 2 nmol hemicystin/mg protein.
Individuals with cystinosis have elevations of WBC cystine above 2 nmol hemicystine/mg protein.
Lægen vil overvåge dig for godartet forhøjet intrakranielt tryk (falsk hjernetumor) og/eller hævelse af synsnerven (papilødem), som forekommer ved behandling med cysteamin.
Your doctor will monitor you for benign intracranial hypertension (or pseudotumor cerebri (PTC)) and/or swelling of the optic nerve (papilledema) associated with cysteamine treatment.
Problemer med mave-tarmsystemet: Patienter i behandling med cysteamin har fået mavesår og blødning.
Stomach and intestinal (gastrointestinal) problems: Patients taking cysteamine have developed ulcers and bleeding.
Indehaveren af markedsføringstilladelsen udleverer før markedsføringen et sæt informationsmateriale til alle læger, der forventes at ordinere PROCYSBI.
The MAH shall provide an educational pack targeting all physicians who are expected to prescribe PROCYSBI prior to the launch.
PROCYSBI indeholder:
What PROCYSBI contains
Ikke almindelig: Gastrointestinalt ulcus Almindelig: Unormal hudlugt, udslæt
Uncommon: Gastrointestinal ulcer Common: Skin odour abnormal, rash
Symptomer fra centralnervesystemet: Nogle patienter har ved behandling med cysteamin fået krampeanfald og depression og tendens til stærk søvnighed.
Central nervous system symptoms: Some patients taking cysteamine have developed seizures, depression, and become too sleepy (excessive sleepiness).
Patienter med ringe tolerabilitet vil stadig have væsentlig fordel af at få sænket leukocytternes cystinindhold til under 2 nmol hemicystin/mg protein (målt med den blandede leukocytanalyse).
Patients with poorer tolerability still receive significant benefit if white blood cell cystine levels are below 2 nmol hemicystine/mg protein (when measured using the mixed leukocyte assay).
Trykfarve shellac povidon K-17 titaniumdioxid (E171)
Printing ink shellac povidone K-17 titanium dioxide (E171)
o I trykfarven: shellac, povidon (K-17), titaniumdioxid (E171).
o In the printing ink: shellac, povidone (K-17), titanium dioxide (E171).
5.2) Til pædiatriske patienter med risiko for aspiration (under seks år) åbnes de hårde kapsler, og indholdet drysses på mad eller drikke som angivet nedenfor.
It is important to dose PROCYSBI in relation to food intake in a consistent and reproducible way over time (see section 5.2) In paediatric patients who are at risk of aspiration, aged approximately 6 years and under, the hard capsules should be opened and the content sprinkled on food or liquid listed below.
Blandingen skal indtages senest to timer efter at den er tilberedt, og skal opbevares nedkølet fra tilberedningen til indtagelsen.
The mixture must be eaten within 2 hours after preparation and must be refrigerated from the time of preparation to the time of administration.
Overhold følgende for at PROCYSBI skal virke korrekt: - Slug hele kapslen med en sur drik (appelsinjuice eller anden sur juice) eller vand.
In order for this medicine to work correctly, you must do the following: - Swallow the whole capsule with an acidic drink (such as orange juice or any acidic juice) or water.
Hud- eller knogleforandringer bør medføre dosisreduktion eller seponering af cysteamin.
If skin or bone abnormalities appear, the dose of cysteamine should be reduced or stopped.
Der er beskrevet tilfælde, hvor dosisreduktion af cysteamin medførte bedring af alvorlige hudlæsioner hos patienter, der var behandlet med høje doser af cysteaminbitartrat med hurtig udløsning eller med andre salte af cysteamin.
There have been reports of serious skin lesions in patients treated with high doses of immediaterelease cysteamine bitartrate or other cysteamine salts that have responded to cysteamine dose reduction.
PROCYSBI 75 mg hård kapsel
PROCYSBI 75 mg hard capsule
Leukocytternes cystinindhold er < 0,2 nmol hemicystin/mg protein hos normale personer, og sædvanligvis under 1 nmol hemicystin/mg protein hos personer, som er heterozygote for cystinose, målt med den blandede leukocytanalyse.
Normal individuals and persons heterozygous for cystinosis have white blood cell cystine levels of < 0.2 and usually below 1 nmol hemicystine/mg protein, respectively, when measured using the mixed leukocyte assay.
Kapselskal gelatine titaniumdioxid (E171) indigocarmin (E132)
Capsule shell gelatin titanium dioxide (E171) indigo carmine (E132)
Skal kasseres 30 dage efter anbrud af folielukningen.
Discard 30 days after opening the foil seal.
Patienter, der ikke kan faste så længe, bør nøjes med et lille måltid (100 gram) (fortrinsvis kulhydrater) i tidsrummet fra en time før til en time efter administration af PROCYSBI.
If fasting during this period is not possible, it is acceptable to eat only a small amount ( 100 grams) of food (preferentially carbohydrates) during the hour before and after PROCYSBI administration.
Dermatologiske risici
Dermatological
Der er ikke udført karcinogenicitetsundersøgelser med cysteaminbitartrat som enterokapsler.
No carcinogenic studies have been conducted with cysteamine bitartrate gastro-resistant hard capsules.
o I kapselskallen: gelatine, titaniumdioxid (E171), indigocarmin (E132).
o In the capsule shell: gelatin, titanium dioxide (E171), indigo carmine (E132).
Der er rapporteret gastrointestinal ulceration og blødning ved behandling med cysteaminbitartrat med hurtig udløsning.
GI ulceration and bleeding have been reported in patients receiving immediate-release cysteamine bitartrate.
PROCYSBI 25 mg hård kapsel
PROCYSBI 25 mg hard capsule
Få nærmere vejledning hos den læge, der behandler barnet.
Consult your child's doctor for complete directions.
Tag ikke PROCYSBI: hvis du er allergisk over for cysteamin (der også kaldes mercaptamin) eller et af de øvrige indholdsstoffer i PROCYSBI (angivet i afsnit 6) hvis du er allergisk over for penicillamin hvis du ammer
If you are allergic to cysteamine (also known as mercaptamine) or any of the other ingredients of this medicine (listed in section 6).
Hudforandringerne på albuerne svandt hos nogle patienter ved dosisreduktion af cysteamin med hurtig udløsning (se pkt.
In some patients, the skin lesions on elbows regressed after immediate-release cysteamine dose reduction (see section 4.4).
Cysteamin kan give abnorm leverfunktion eller nedsat antal hvide blodlegemer (leukopeni).
Abnormal liver function or reduced white blood cell count (leukopenia) can occur with use of cysteamine.
SÆRLIGE OPBEVARINGSBETINGELSER Opbevares i køleskab (2 °C - 8 °C).
SPECIAL STORAGE CONDITIONS Store in a refrigerator.
Sådan skal du tage PROCYSBI
How to take PROCYSBI
Dato for bortskaffelse:
Discard Date:
Kapsler eller kapselindhold må ikke knuses eller tygges.
Do not crush or chew capsules or capsule contents.
Indholdet omrøres forsigtigt, så cysteamin-kornene blandes op i den bløde mad.
Gently stir the contents into the soft food, creating a mixture of cysteamine granules and the soft food.
Behandling med cysteamin kan medføre mavesår, tarmsår og blødning (se afsnit 4).
Stomach and intestinal ulcers and bleeding can occur in patients receiving cysteamine (see section 4).
For at nå dette niveau kan cysteamindosis øges til højst 1,95 g/m2/dag.
The cysteamine dose can be increased to a maximum of 1.95 g/m2/day to achieve this level.
En dosering over 1,95 g/m2/dag frarådes.
The use of doses higher than 1.95 g/m2/day is not recommended.
• Amning.
• Breast-feeding.
Cysteamin (mercaptaminbitartrat)
Cysteamine (mercaptamine bitartrate)
De indberettede knogleforandringer var genu valgum, smerter i benene, hypermobile led, osteopeni, kompressionsbrud og skoliose.
Bone disorders reported were genu valgum, leg pain and hyperextensive joints, osteopenia, compression fractures, and scoliosis.
Hyppighed: bivirkning
Frequency: adverse reaction
Der sås ingen mutagene virkninger i en undersøgelse med bakteriel tilbagemutation (“Ames test”) af det cysteaminbitartrat, der anvendes til PROCYSBI.
Specific studies with cysteamine did not show any mutagenic effects in the Ames test or any clastogenic effect in the mouse micronucleus test. A bacterial reverse mutation assay study (“Ames test”) was performed with the cysteamine bitartrate used for PROCYSBI and cysteamine bitartrate did not show any mutagenic effects in this test.
Ikke almindelige bivirkninger (forekommer hos indtil 1 ud af 100 behandlede): Hudforandringer, knogleforandringer og ledproblemer: Behandling med høje doser cysteamin kan medføre hudforandringer.
Skin lesions, bone lesions, and joint problems: Treatment with high doses of cysteamine can cause skin lesions to develop.
En dosering over 1,95 g/m2/dag frarådes (se pkt.
The use of doses higher than 1.95 g/m2/day is not recommended (see section 4.2).
5.3) er amning imidlertid kontraindiceret hos kvinder i behandling med PROCYSBI (se pkt.
However, due to the results of animal studies in breast-feeding females and neonates (see section 5.3), breast-feeding is contra-indicated in women taking PROCYSBI (see section 4.3).
PROCYSBI, 75 mg, leveres som blå, hårde enterokapsler.
PROCYSBI 75 mg is presented as blue gastro-resistant hard capsules.
fødedygtige kvinder skal informeres om risikoen for teratogenicitet for fødedygtige kvinder skal der foreligge en negativ graviditetstest før påbegyndelse af behandlingen fødedygtige kvinder skal instrueres i at bruge hensigtsmæssig antikonception under behandlingen fødedygtige kvinder skal instrueres i at kontakte den behandlende læge, hvis de bliver gravide under behandlingen
• The risk of teratogenicity and relevant risk minimisation advice: Women of childbearing potential should be informed about the risk of teratogenicity; For women of child-bearing potential a negative pregnancy test should be confirmed before starting treatment;
Lyseblå hårde kapsler, størrelse 0, med påskriften “75 mg” med hvid trykfarve, og mørkeblå overdel med påtrykt ”PRO”-logo med hvid trykfarve.
Light blue size 0 hard capsules imprinted “75 mg” in white ink and a dark blue cap imprinted with “PRO” in white ink.
Hver hård kapsel indeholder 75 mg cysteamin (som mercaptaminbitartrat).
Each hard capsule contains 75 mg of cysteamine (as mercaptamine bitartrate).
Lyseblå hårde kapsler, størrelse 3, med påskriften “25 mg” med hvid trykfarve, og lyseblå overdel med påtrykt ”PRO”-logo med hvid trykfarve.
Light blue size 3 hard capsules imprinted “25 mg” in white ink and a light blue cap imprinted with “PRO” in white ink.
Hver hård kapsel indeholder 25 mg cysteamin (som mercaptaminbitartrat).
Each hard capsule contains 25 mg of cysteamine (as mercaptamine bitartrate).
Alle 3 cylindre skal forblive i flasken under anvendelsen.
Please keep the three cylinders in each bottle during the use of the bottle.
For de patienter, der får en stabil dosis af PROCYSBI og ikke har let adgang til at få målt de hvide blodlegemers (leukocytter) cystinindhold, skal behandlingen sigte mod, at cysteamin i plasma holdes > 0,1 mg/l 30 minutter efter doseringen.
For example the therapeutic goal is to maintain a WBC cysteine level < 1 nmol hemicystine/mg protein (when measured using the mixed leukocyte assay), 30 min after dosing For patients adherent to a stable dose of PROCYSBI, and who do not have easy access to an adequate facility for measuring their WBC cystine, the goal of therapy should be to maintain plasma cysteamine concentration > 0.1 mg/L, 30 min after dosing.
Den mørkeblå kapseloverdel er påtrykt ”PRO”-logoet med hvid trykfarve, og underdelen er påtrykt “75 mg” med hvid trykfarve.
The dark blue cap is imprinted with “PRO” in white ink and the light blue body is imprinted with “75 mg” in white ink.