natgillin/translations-raw huggingface dataset:
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.
Nakon što im se stanje stabilizira i amonijak bude pod kontrolom, bolesnici koji prelaze s natrijevog fenilacetata / natrijevog benzoata na lijek RAVICTI trebaju primati dozu lijeka RAVICTI iz gornjih vrijednosti terapijskog raspona (11,2 ml/m2/dan) uz mjerenje amonijaka u plazmi prema kojem se usmjerava daljnje doziranje.
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.
Bilo je uključeno ukupno 49 djece u dobi od 2 mjeseca do 17 godina s nedostatkom OTC-a, ASS-a, ASL-a i ARG-a, a sva osim 1 prešla su s natrijevog fenilbutirata na glicerolfenilbutirat.
About urea cycle disorders
O poremećajima ciklusa ureje
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 pediatric patients with UCDs aged 2 months to less than 2 years.
U dodatnom dugotrajnom (24 mjeseca), nekontroliranom, otvorenom kliničkom ispitivanju, sigurnost lijeka RAVICTI procijenjena je u 16 bolesnika s poremećajima ciklusa ureje mlađih od 2 mjeseca i
• tests show higher or lower potassium in your blood
• nalazi pretraga pokazuju povišen ili snižen kalij u krvi
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.
Ukupno je 10 pedijatrijskih bolesnika s poremećajima ciklusa ureje u dobi od 2 mjeseca do manje od 2 godine sudjelovalo u dugotrajnom (24 mjeseca), nekontroliranom, otvorenom ispitivanju, od kojih je 6 bolesnika prešlo s natrijevog fenilbutirata na RAVICTI, a jedan je bolesnik prešao s natrijevog fenilbutirata i natrijevog benzoata.
Probenecid Probenecid may inhibit the renal excretion of metabolites of glycerol phenylbutyrate including PAGN.
Probenecid može inhibirati izlučivanje metabolita glicerolfenilbutirata, uključujući PAGN, putem bubrega.
Two patients were treatment naïve and one additional patient was gradually discontinued from intravenous sodium benzoate and sodium phenylacetate while RAVICTI was initiated.
Dva bolesnika prethodno nisu bila liječena, a jedan dodatni bolesnik postupno je prekinuo primati intravenski natrijev benzoat i natrijev fenilacetat dok se uvodio
In the pooled analysis of the short-term studies in children (Study 3 and Study 4), plasma ammonia was significantly lower after switching to glycerol phenylbutyrate; ammonia AUC0-24h was 626.79 and 871.72 (micromol/L)*hour during treatment with glycerol phenylbutyrate and sodium phenylbutyrate, respectively (n = 26, ratio of geometric means 0.79; 95% confidence intervals 0.647, 0.955).
U združenoj analizi kratkoročnih ispitivanja na djeci (Ispitivanje 3 i Ispitivanje 4), razina amonijaka u plazmi bila je značajno niža nakon prelaska na glicerolfenilbutrat; AUC0-24 amonijak bio je 626,79 odnosno 871,72 (mikromol/l)*sat tijekom liječenja glicerolfenilbutiratom odnosno natrijevim fenilbutiratom (n = 26, omjer geometrijskih sredina 0,79; 95% interval pouzdanosti 0,647; 0,955).
The reclosable bottle cap adapter can be used until the bottle is empty.
Nastavak za bocu s čepom za ponovno zatvaranje može se koristiti dok se boca ne isprazni.
Adjustment based on urinary phenylacetylglutamine U-PAGN measurements may be used to help guide glycerol phenylbutyrate dose adjustment and assess compliance.
Mjerenja U-PAGN se mogu koristiti kao pomoć za određivanje prilagodbe doze glicerolfenilbutirata i procjenu suradljivosti.
• 7000 microgram (mcg/ml) for patients >2 years of age with a BSA of ≤1.3
• 7000 mikrograma (μg/ml) za bolesnike >2 godine starosti s površinom tijela ≤1,3
Constipation, Flatulence, Gastrooesophageal reflux disease
Poremećaji probavnog sustava proljev, konstipacija, vjetrovi, gastroezofagealna refluksna bolest
The daily dose should be individually adjusted according to the patient's estimated urea synthetic capacity, if any, amino acid profile, protein tolerance and the daily dietary protein intake needed to promote growth and development.
Dnevnu dozu treba individualno prilagoditi sukladno procijenjenom kapacitetu sinteze ureje bolesnika, ukoliko je ima, profilu aminokiselina, toleranciji proteina i dnevnom unosu proteina prehranom, potrebnom za poticanje rasta i razvoja.
How to store RAVICTI
Kako čuvati RAVICTI
In the pooled analysis of studies where patients switched from sodium phenylbutyrate to glycerol phenylbutyrate, ammonia AUC0-24h was 774.11 and 991.19 [(micromol/L)*hour] during treatment with glycerol phenylbutyrate and sodium phenylbutyrate, respectively (n = 80, ratio of geometric means 0.84; 95% confidence intervals 0.740, 0.949).
U združenoj analizi ispitivanja u kojima su bolesnici prešli s natrijevog fenilbutirata na glicerolfenilbutrat, AUC0-24 amonijaka bio je 774,11 odnosno 991,19 [(mikromol/l)*sat] tijekom liječenja glicerolfenilbutiratom odnosno natrijevim fenilbutiratom (n=80, omjer geometrijskih sredina 0,84; 95%-tni interval pouzdanosti 0,740; 0,949).
The product contains no excipients.
Lijek ne sadrži pomoćne tvari.
Flatulence, diarrhoea, vomiting, nausea, abdominal pain, dyspepsia, abdominal distension, constipation, oral discomfort, retching
Flatulencija, proljev, povraćanje, mučnina, bol u abdomenu, dispepsija, distenzija abdomena, konstipacija, nelagoda u ustima, dizanje želuca
• joint swelling, muscle spasms, pain in the hands and feet, heel pain
• oticanje zglobova, grčevi mišića, bol u šakama i stopalima, bol u peti
Women of childbearing potential/contraception in males and females Effective contraceptive measures must be taken by women of child-bearing potential (see section 4.6).
Žene reproduktivne dobi moraju koristiti učinkovitu kontracepciju (vidjeti dio 4.6).
• stomach bloating or pain, constipation, diarrhoea, heartburn, wind, feeling sick (nausea), pain in the mouth, retching
• nadutost ili bol u želucu, zatvor, proljev, žgaravica, vjetrovi, mučnina, bol u ustima, dizanje želuca
A new reclosable bottle cap adapter should be used for each new bottle that is opened.
Novi nastavak za bocu s čepom za ponovno zatvaranje treba se koristiti za svaku novootvorenu bocu.
Hyperammonemia may be induced by haloperidol and by valproic acid.
Haloperidol i valproatna kiselina mogu potaknuti hiperamonemiju.
• 5000 microgram (mcg/ml) for patients >2 years of age with a BSA of >1.3
• 5000 mikrograma (μg/ml) za bolesnike >2 godine starosti s površinom tijela >1,3
The daily dose should be individually adjusted according to the patient's estimated urea synthetic capacity, if any, protein tolerance and the daily dietary protein intake needed to promote growth and development.
Dnevnu dozu treba individualno prilagoditi prema procijenjenom kapacitetu sinteze ureje u bolesnika, ukoliko je ima, toleranciji proteina i dnevnom unosu proteina prehranom potrebnom za poticanje rasta i razvoja.
Monitor ammonia levels closely when use of valproic acid or haloperidol is necessary in UCD patients.
Pomno pratite razine amonijaka kada je kod bolesnika s poremećajima ciklusa ureje potrebna primjena valproatne kiseline ili haloperidola.
High levels of ammonia leads to feeling sick (nausea), being sick (vomiting) or feeling confused.
Visoke razine amonijaka uzrokuju mučninu, povraćanje ili smetenost.
• lipase in pancreatic replacement therapies
• lipaza kod nadomjestne terapije enzima gušterače
Clear, Type III glass, bottle with a high density polyethylene (HDPE) child-resistant closure.
Boca od prozirnog stakla tipa III sa zatvaračem sigurnim za djecu od polietilena visoke gustoće
Monitor ammonia levels closely when corticosteroids and glycerol phenylbutyrate are used concomitantly.
Pomno pratite razine amonijaka kod istodobne primjene kortikosteroida i glicerolfenilbutirata.
• feeling tired, feeling dizzy, headache or tremor
• osjećaj umora, omaglica, glavobolja ili nevoljno drhtanje
• The active substance is glycerol phenylbutyrate.
• Djelatna tvar je glicerolfenilbutirat.
Recommended starting dosage in phenylbutyrate-naïve patients:
Preporučena početna doza za bolesnike koji prethodno nisu primali fenilbutirat:
Glycerol phenylbutyrate is hydrolysed by pancreatic lipases to yield, PBA, which is converted by beta oxidation to PAA, the active moiety of glycerol phenylbutyrate.
Glicerolfenilbutirat se hidrolizira lipazama gušterače do PBA koja se pretvara beta oksidacijom u PAA, aktivni dio glicerolfenilbutirata.
The plasma PAA to PAGN ratio may be useful in dosage monitoring.
Omjer PAA i PAGN u plazmi može biti koristan u praćenju doze. (vidjeti dio 5.2).
The recommended dosages for patients naïve to phenylbutyric acid and for patients switching from sodium phenylbutyrate or from sodium phenylacetate/sodium benzoate injection to RAVICTI are different.
Preporučene doze za bolesnike koji prethodno nisu liječeni fenilbutiratnom kiselinom i za bolesnike koji prelaze s natrijevog fenilbutirata ili injekcija natrijevog fenilacetata / natrijevog benzoata na
Mean 24-hour AUCs for venous ammonia during steady-state dosing were 866 micromol/L*hour and 977 micromol/L*hour with glycerol phenylbutyrate and sodium phenylbutyrate, respectively (n = 44, ratio of geometric means 0.91; 95% confidence intervals 0.799, 1.034).
Srednji 24-satni AUC-ovi amonijaka u venskoj krvi tijekom doziranja u stanju dinamičke ravnoteže bili su 866 mikromola/l*sat odnosno 977 mikromola/l*sat s glicerolfenilbutiratom, odnosno natrijevim fenilbutiratom (n=44, omjer geometrijskih sredina 0,91; 95% intervali pouzdanosti 0,799; 1,034).
Uncommon Hypothyroidism
Manje često Hipotireoza
Uncommon Dysphonia, epistaxis, nasal congestion, oropharyngeal pain, throat irritation
Manje često Disfonija, epistaksa, začepljenje nosa, orofaringealna bol, nadraženo grlo
PBA derived from glycerol phenylbutyrate is further converted by β-oxidation to PAA.
PBA dobiven iz glicerolfenilbutirata dalje prelazi u PAA
ADRs in patients 2 months to less than 2 years of age
Ispitivanje u djece u dobi od 2 mjeseca do manje od 2 godine
• Step 1: 100% dose sodium phenylacetate/sodium benzoate and 50% dose of RAVICTI for 4-8
100% doze natrijevog fenilacetata / natrijevog benzoata i 50% doze lijeka RAVICTI tijekom 4-8 sati;
All patients successfully transitioned to RAVICTI within 3 days, where successful transition was defined as no signs and symptoms of hyperammonemia and a venous ammonia value less than 100 micromol/L.
Uspješan prelazak definiran je kao izostanak znakova i simptoma hiperamonemije i vrijednost amonijaka u venskoj krvi manja od 100 mikromol/l.
Saturation of conjugation of PAA and glutamine to form PAGN was suggested by increases in the ratio of plasma PAA to PAGN with increasing dose and with increasing severity of hepatic impairment.
Povećanje omjera PAA i PAGN u plazmi s povećanjem doze i s povećanjem težine oštećenja jetre ukazuje na zasićenje konjugacije PAA s glutaminom kako bi se stvorio PAGN.
1 reclosable bottle cap adapter.
1 nastavak za bocu s čepom za ponovno zatvaranje.
RAVICTI 1.1 g/ml oral liquid Glycerol phenylbutyrate
RAVICTI 1,1 g/ml oralna tekućina glicerolfenilbutirat
Upon oral administration, pancreatic lipases hydrolyse glycerol phenylbutyrate and release PBA. PBA undergoes β-oxidation to PAA, which is conjugated with glutamine in the liver and in the kidney through the enzyme phenylacetyl-CoA: Lglutamine- N-acetyltransferase to form PAGN.
PBA prolazi β-oksidaciju do PAA koja se konjugira s glutaminom u jetri i bubrezima putem enzima fenilacetil-CoA: Lglutamin - N-acetiltransferaze, te nastaje PAGN.
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.
Egzokrini enzimi gušterače hidroliziraju glicerolfenilbutirat u tankom crijevu odvajajući aktivni dio molekule, fenilbutirat, od glicerola.
Blood and lymphatic system disorders
Poremećaji krvi i limfnog sustava anemija, trombocitoza
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.
može ukazivati na zasićenje sposobnosti pretvorbe PAA u PAGN i potrebu za smanjenjem doze i/ili povećanjem učestalosti doziranja.
A new reclosable bottle cap adapter should be used when a new bottle is opened.
Novi nastavak za bocu s čepom za ponovno zatvaranje treba koristiti od otvaranja nove boce.
• Each ml of liquid contains 1.1 g of glycerol phenylbutyrate.
• Jedan ml tekućine sadrži 1,1 g glicerolfenilbutirata.
Do not drive or use machines whilst experiencing these side effects.
Kada osjećate te nuspojave nemojte voziti ni upravljati strojevima.
The mean normalized venous ammonia values in pediatric patients aged less than 2 months were within normal limits during long-term treatment with glycerol phenylbutyrate (range: 35 to 94 micromol/L).
Srednje vrijednosti normaliziranih vrijednosti amonijaka u venskoj krvi u pedijatrijskih bolesnika mlađih od 2 mjeseca bile su unutar normalnih granica tijekom dugotrajnog liječenja glicerol fenilbutiratom (raspon: 35-94 mikromol/l).
Intact glycerol phenylbutyrate was not detectable in plasma in UCD patients.
Nepromijenjeni glicerolfenilbutirat nije se mogao otkriti u plazmi bolesnika s poremećajima ciklusa ureje.
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.
In vitro ispitivanja za procjenu postotka povrata ukupne doze primijenjene putem nazogastrične, nazojejunalne sonde ili gastrostome pokazala su postotak povrata doze > 99% za doze > 1 ml, odnosno 70% za dozu od 0,5 ml.
Place the tip of the oral syringe into the reclosable bottle cap adapter.
Stavite vrh oralne štrcaljke u nastavak za bocu s čepom za ponovno zatvaranje.
Do not rinse the reclosable bottle cap or the oral syringe between daily doses, as the presence of water causes glycerol phenylbutyrate to degrade.
Nemojte ispirati nastavak za bocu s čepom za ponovno zatvaranje niti oralnu štrcaljku između dnevnih doza jer prisutnost vode uzrokuje razgradnju glicerolfenilbutirata.
Important note: Do not rinse the reclosable bottle cap adapter or the oral syringe between daily doses as the introduction of water causes glycerol phenylbutyrate to degrade.
Važna napomena: Nemojte ispirati nastavak za bocu s čepom za ponovno zatvaranje ni oralne štrcaljke između dnevnih doza, jer će dodavanje vode uzrokovati razgradnju glicerolfenilbutirata.
Mean fasting venous ammonia values were within normal limits during long-term treatment with glycerol phenylbutyrate (range: 17-25 micromol/L).
Srednje vrijednosti amonijaka u venskoj krvi natašte su bile u granicama normale tijekom dugotrajnog liječenja glicerolfenilbutiratom (raspon: 17-25 mikromol/l).
Respiratory, thoracic and mediastinal disorder
Poremećaji dišnog sustava, prsišta i sredoprsja
Mean venous ammonia values in paediatric patients in Study 5 were within normal limits during long-term (24 months) treatment with glycerol phenylbutyrate (range: 1525 micromol/L).
Srednja vrijednost razina amonijaka u venskoj krvi pedijatrijskih bolesnika u Ispitivanju 5 bila je unutar granica normale tijekom dugoročnog (24 mjeseca) liječenja glicerolfenilbutiratom (raspon: 15-25 mikromol/l).
• gallbladder, bladder or back pain
• bolovi u žučnom mjehuru, mokraćnom mjehuru ili leđima
‘gastrostomy tube'
• brazdanje noktiju
Abnormal skin odour, acne Uncommon Alopecia, hyperhidrosis, pruritic rash
Neuobičajen miris kože, akne Manje često Alopecija, hiperhidroza, osip sa svrbežom
Initial dosage in patients switching from sodium phenylbutyrate to RAVICTI:
Početna doza za bolesnike koji prelaze s injekcija natrijevog fenilacetata / natrijevog benzoata na
Twist the reclosable bottle cap adapter onto the bottle.
Zavrnite nastavak za bocu s čepom za ponovno zatvaranje na bocu.
The ratio of mean values for PAA to PAGN among all patients dosed with 6 ml BID ranged from 0.96 to 1.28 and for patients dosed with 9 ml twice daily ranged from 1.18-3.19.After multiple doses, a PAA concentration >200 mcg/L was associated with a ratio of plasma PAA to PAGN concentrations higher than 2.5.
Omjer srednjih vrijednosti za PAA i PAGN kod svih bolesnika koji su primali dozu od 6 ml dvaput dnevno bio je u rasponu od 0,96 do 1,28 i za bolesnike koji su primali dozu od 9 ml dvaput dnevno bio je u rasponu od 1,18-3,19.
Dizziness, headache, tremor Uncommon Dysgeusia, lethargy, paraesthesia, psychomotor hyperactivity, somnolence, speech disorder
Manje često Disgeuzija, letargija, parestezija, psihomotorna hiperaktivnost, somnolencija, poremećaj govora Manje često Stanje smetenosti, depresivno raspoloženje
• 7 ml/m2/day (8 g/m2/day) in patients with a BSA ≥ 1.3 m2
• 7 ml/m2/dan (8 g/m2/dan) za bolesnike s površinom tijela ≥ 1,3 m2 Početna doza za bolesnike koji prelaze s natrijevog fenilbutirata na RAVICTI:
Mean fasting venous ammonia values in adults in Study 2 were within normal limits during long-term treatment with glycerol phenylbutyrate (range: 630 micromol/L).
Srednje vrijednosti amonijaka u venskoj krvi natašte kod odraslih u Ispitivanju 2 su bile u granicama normale tijekom dugotrajnog liječenja glicerolfenilbutiratom (raspon: 6-30 mikromol/l).
Tell your doctor or pharmacist if you notice any of the following side effects:
Recite liječniku ili ljekarniku ako primijetite bilo koju od sljedećih nuspojava: Česte: mogu se javiti u do 1 na 10 osoba
Do not rinse the reclosable bottle cap adapter or the oral syringe between daily doses as the introduction of water causes glycerol phenylbutyrate to degrade.
Nemojte ispirati nastavak za bocu s čepom za ponovno zatvaranje niti oralnu štrcaljku između dnevnih doza jer unošenje vode uzrokuje razgradnju glicerolfenilbutirata.
RAVICTI must be used with a special low protein diet. - This diet will be designed for you by your doctor and dietician.
RAVICTI se mora uzimati s posebnom prehranom s niskim udjelom proteina.
You must follow a special low protein diet during treatment with glycerol phenylbutyrate.
Tijekom liječenja glicerolfenilbutiratom morate se pridržavati posebne prehrane s niskim udjelom proteina.
The following side effects may happen with this medicine:
Ovaj lijek može imati sljedeće nuspojave: Ozbiljne nuspojave Odmah recite liječniku ako:
No precipitant trigger or missing dose was reported for the other 2 subjects (1 age < 1 month, 1 age 1-2- months).
U preostala 2 ispitanika (jednog u dobi < 1 mjeseca i drugog u dobi od 1-2 mjeseca) nije zabilježen nijedan okidač koji je potaknuo hiperamonemiju ni propuštena doza.
The following side effects have been observed in a clinical study including 16 patients less than 2 months of age:
Dodatne nuspojave u djece mlađe od 2 mjeseca Sljedeće nuspojave opažene su u kliničkom ispitivanju u koje je bilo uključeno 16 bolesnika mlađih od
• You should have the medicine with the reclosable bottle cap adapter along with an oral syringe to administer the correct amount of glycerol phenylbutyrate.
• Za primjenu točne količine glicerolfenilbutirata potrebno je imati lijek s nastavkom za bocu s čepom za ponovno zatvaranje i oralnu štrcaljku.
Tabulated list of adverse reactions The adverse reactions are listed below, by system organ class and by frequency.
U nastavku su navedene nuspojave prema klasifikaciji organskih sustava i učestalosti.
continued according to feeding schedule for 4-8 hours.
prekid primjene natrijevog fenilacetata / natrijevog benzoata i nastavak punom dozom lijeka RAVICTI u skladu s rasporedom hranjenja tijekom 4-8 sati.
In the same study, following 6 ml of glycerol phenylbutyrate for 3 days (TID), mean Cmax and AUC were 100 mcg/ml and 1400 mcg•h/ml for PBA and 65 mcg/ml and 2064 mcg•h/ml for PAA, respectively.
100 μg/ml i 1400 μg•h/ml za PBA, odnosno 65 μg/ml i 2064 μg•h/ml za PAA.
CE marked oral syringes compatible with the reclosable bottle cap adapter can be obtained from a pharmacy.
Oralne štrcaljke s oznakom CE koje su kompatibilne s nastavkom za bocu s čepom za ponovno zatvaranje,
Hypophagia
Dojenje
Constipation Diarrhoea
Oštećenje bubrega
Each ml of liquid contains 1.1g of glycerol phenylbutyrate.
NAVOĐENJE DJELATNE(IH) TVARI Jedan ml sadrži 1,1 g glicerolfenilbutirata.
Nine patients successfully transitioned to RAVICTI within 4 days, followed by 3 days of observation for a total of 7 days, where successful transition was defined as no signs and symptoms of hyperammonemia and a venous ammonia value less than 100 micromol/L.
Devet je bolesnika uspješno prešlo na RAVICTI u roku od 4 dana, nakon čega su promatrani 3 dana, što je ukupno 7 dana.
Impairment of fertility
Smanjenje plodnosti
Uncommon Gallbladder pain
Poremećaji probavnog sustava konstipacija proljev
Tightly close the tethered tab on the reclosable bottle adapter after use.
Nakon primjene čvrsto zatvorite povezani čep na nastavku za bocu za ponovno zatvaranje.
Glycerol
Probenecid
Kuvan is taken with a meal at the same time every day, preferably in the morning.
Kuvan se uzima uz obrok u isto vrijeme svakog dana, po mogućnosti ujutro.
This summary was last updated in 07-2017.
Sažetak je posljednji put ažuriran: 07.2017.
What is Kuvan and what is it used for?
Što je Kuvan i za što se koristi?
What measures are being taken to ensure the safe and effective use of Kuvan?
Koje se mjere poduzimaju kako bi se osigurala sigurna i učinkovita primjena lijeka Kuvan?
If this has been achieved after one month, the patient is classified as a ‘responder' and can continue taking Kuvan.
Ako se to postigne nakon mjesec dana, bolesnik se klasificira kao „bolesnik koji odgovara na lijek” i može nastaviti uzimati lijek Kuvan.
The European Commission granted a marketing authorisation valid throughout the European Union for Kuvan on 2 December 2008.
Europska komisija izdala je odobrenje za stavljanje lijeka Kuvan u promet koje vrijedi na prostoru Europske unije od 2. prosinca 2008.
For some patients with BH4 deficiency, the dose may need to be divided into two or three doses over the course of the day to get the best effect.
Kod nekih bolesnika s nedostatkom BH4 dozu će možda trebati podijeliti na dvije ili tri doze tijekom dana kako bi se postigao najbolji učinak.
Other information about Kuvan
Ostale informacije o lijeku Kuvan
For the treatment of BH4 deficiency, which is a very rare condition, the company presented results of three studies from the published literature in which some patients were treated with sapropterin for an average of 15.5 months.
Za liječenje nedostatka BH4, koji je vrlo rijetko stanje, tvrtka je podastrla rezultate triju ispitivanja iz objavljene literature u kojima su neki pacijenti bili liječeni sapropterinom tijekom prosječnog razdoblja od 15,5 mjeseci.
Why is Kuvan approved?
Zašto je lijek Kuvan odobren?
Kuvan sapropterin dihydrochloride
Kuvan sapropterin dihidroklorid
Studies with Kuvan involved adults and children of all ages.
Ispitivanja s lijekom Kuvan uključivala su odrasle i djecu svih dobi.
What are the risks associated with Kuvan?
Koji su rizici povezani s lijekom Kuvan?
This is a summary of the European public assessment report (EPAR) for Kuvan.
Ovo je sažetak europskoga javnog izvješća o procjeni (EPAR) lijeka Kuvan.
The full EPAR for Kuvan can be found on the Agency's website: ema.europa.eu/Find medicine/Human medicines/European Public Assessment Reports.
Cjeloviti EPAR za lijek Kuvan nalazi se na internetskim stranicama Agencije: ema.europa.eu/Find medicine/Human medicines/European Public Assessment Reports.
It is not intended to provide practical advice on how to use Kuvan.
Svrha sažetka nije davati praktične savjete o primjeni lijeka Kuvan.