FrancophonIA/ELRC3.0 huggingface dataset:
English: Discontinuation of treatment should be done only under the supervision of a physician.
Croatian: Prekid liječenja smije se učiniti samo pod nadzorom liječnika.
English: Their frequency is not known (frequency cannot be estimated from the available data).
Croatian: Njihova učestalost nije poznata (učestalost se ne može procijeniti iz dostupnih podataka).
English: Safety and efficacy of Kuvan in patients above 65 years of age have not been established.
Croatian: Sigurnost i djelotvornost lijeka Kuvan u bolesnika u dobi iznad 65 godina nisu ustanovljene.
English: If these are not strictly controlled before or when you become pregnant, this could be harmful to you and to your baby.
Croatian: Ako one nisu pod strogom kontrolom prije i tijekom trudnoće, to može biti štetno za Vas i Vaše dijete.
English: The bottles are sealed with an aluminium seal.
Croatian: Boce su zatvorene aluminijskim pokrovom.
English: Each bottle contains 30, 120 or 240 tablets.
Croatian: Svaka boca sadrži 30, 120 ili 240 tableta.
English: If you are pregnant your doctor will tell you how to control phenylalanine levels adequately.
Croatian: Ako ste trudni, Vaš liječnik će Vam reći kako prikladno nadzirati koncentracije fenilalanina.
English: Contents of the pack and other information What Kuvan contains
Croatian: Sadržaj pakiranja i druge informacije Što Kuvan sadrži
English: This leaflet was last revised in month/YYYY Other sources of information
Croatian: Ova uputa je zadnji puta revidirana u {MM/GGGG} Ostali izvori informacija
English: For instructions for use, see section 4.2.
Croatian: Za upute za upotrebu vidjeti dio 4.2.
English: Excretion of total biopterin in milk was demonstrated in rats by intravenous route.
Croatian: Izlučivanje ukupnog biopterina u mlijeko pokazano je kod štakora intravenskim putem.
English: Open the sachet(s) only when you are ready to use them.
Croatian: Vrećice otvorite tek kada ste ih spremni upotrijebiti.
English: Few cases of allergic reactions (such as skin rash and serious reactions) have been reported.
Croatian: Zabilježeno je nekoliko slučajeva alergijskih reakcija (kao kožni osip i ozbiljne reakcije).
English: • Throw away any remaining solution.
Croatian: • Bacite svu preostalu otopinu.
English: In vitro, sapropterin did not inhibit CYP1A2, CYP2B6, CYP2C8, CYP2C9, CYP2C19, CYP2D6 or CYP3A4/5, nor induce CYP1A2, 2B6, or 3A4/5.
Croatian: In vitro, sapropterin nije inhibirao CYP1A2, CYP2B6, CYP2C8, CYP2C9, CYP2C19, CYP2D6 ili CYP3A4/5, niti inducirao CYP1A2, 2B6 ili 3A4/5.
English: BH4 deficiency
Croatian: Nedostatak BH4
English: Treatment of overdose should be directed to symptoms.
Croatian: Liječenje predoziranja treba biti simptomatsko.
English: What Kuvan looks like and contents of the pack
Croatian: Kako Kuvan izgleda i sadržaj pakiranja
English: You should not take this medicine if you are breast-feeding.
Croatian: Ne biste trebali uzimati ovaj lijek ako dojite.
English: If you stop taking Kuvan
Croatian: Ako prestanete uzimati Kuvan
English: Animal studies do not indicate direct or indirect harmful effects with respect to pregnancy, embryonal/foetal development, parturition or postnatal development.
Croatian: Ispitivanja na životinjama ne ukazuju na izravan ili neizravan štetan učinak na trudnoću, embrionalni ili fetalni razvoj, porođaj ili postnatalni razvoj.
English: CONTENTS BY WEIGHT, BY VOLUME OR BY UNIT OTHER
Croatian: SADRŽAJ PO TEŽINI, VOLUMENU ILI DOZNOJ JEDINICI LIJEKA DRUGO
English: • A medicine cup with graduation markings at 20, 40, 60 and 80 ml
Croatian: • čašica za lijek s mjernim oznakama za 20, 40, 60 i 80 ml
English: The requirements for submission of periodic safety update reports for this medicinal product are set out in the list of Union reference dates (EURD list) provided for under Article 107c(7) of Directive 2001/83/EC and any subsequent updates published on the European medicines web-portal.
Croatian: Zahtjevi za periodička izvješća o neškodljivosti za ovaj lijek navedeni su u referentnom popisu datuma EU (EURD popis) predviđenim člankom 107.(c) stavkom 7. Direktive 2001/83/EZ i svim sljedećim ažuriranim verzijama objavljenim na europskom internetskom portalu za lijekove.
English: If you have red, itchy, raised areas (hives), runny nose, fast or uneven pulse, swelling of your tongue and throat, sneezing, wheezing, serious difficulty in breathing or dizziness, you may be having a serious allergic reaction to the medicine.
Croatian: Ako imate crvena, svrbljiva, izdignuta područja (koprivnjača) na koži, curenje nosa, brz ili nepravilan puls, oticanje jezika i grla, kihanje, piskanje, imate ozbiljne poteškoće pri disanju ili omaglicu, možda imate ozbiljne alergijske reakcije na lijek.
English: Absolute bioavailability or bioavailability for humans after oral administration is not known.
Croatian: Apsolutna bioraspoloživost ili bioraspoloživost kod ljudi nakon oralne primjene nije poznata.
English: No clear evidence of teratogenic activity was found in rats and in rabbits at doses of approximately 3 and 10 times the maximum recommended human dose, based on body surface area.
Croatian: Nisu nađeni jasni pokazatelji teratogene aktivnosti kod štakora i zečeva pri dozama približno 3 do 10 puta većim od maksimalno preporučenih doza za ljude, prema tjelesnoj površini.
English: If the strict diet does not adequately reduce phenylalanine amount in your blood your doctor will consider whether you must take this medicine.
Croatian: Ako stroga dijeta ne smanji primjereno količinu fenilalanina u Vašoj krvi, Vaš liječnik će razmotriti morate li uzimati ovaj lijek.
English: • If your baby is too small to drink from a glass or a cup you may administer the solution via the oral syringe.
Croatian: • Ako je Vaše dijete premaleno da bi pilo iz čaše ili šalice, možete primijeniti otopinu s pomoću oralne štrcaljke
English: Very common side effects (may affect more than 1 in 10 people) Headache and runny nose.
Croatian: Vrlo česte nuspojave (mogu se javiti kod više od 1 od 10 osoba) glavobolja i curenje nosa.
English: ANNEX II A. MANUFACTURER(S) RESPONSIBLE FOR BATCH RELEASE B. CONDITIONS OR RESTRICTIONS REGARDING SUPPLY AND USE C. OTHER CONDITIONS AND REQUIREMENTS OF THE MARKETING AUTHORISATION D. CONDITIONS OR RESTRICTIONS WITH REGARD TO THE SAFE AND EFFECTIVE USE OF THE MEDICINAL PRODUCT
Croatian: PRILOG II. A. PROIZVOĐAČ(I) ODGOVORAN (ODGOVORNI) ZA PUŠTANJE SERIJE LIJEKA U PROMET B. UVJETI ILI OGRANIČENJA VEZANI UZ OPSKRBU I PRIMJENU C. OSTALI UVJETI I ZAHTJEVI ODOBRENJA ZA STAVLJANJE LIJEKA U PROMET D. UVJETI ILI OGRANIČENJA VEZANI UZ SIGURNU I UČINKOVITU PRIMJENU LIJEKA
English: Population pharmacokinetic analysis of sapropterin including patients from birth to 49 years of age showed that body weight is the only covariate substantially affecting clearance or volume of distribution.
Croatian: Analiza populacijske farmakokinetike sapropterina u bolesnika u dobi od rođenja do 49 godina pokazala je da je tjelesna težina jedina kovarijanca koja znatno utječe na njegov klirens ili volumen distribucije.
English: Your child should drink the solution with a meal.
Croatian: Vaše dijete otopinu mora popiti uz obrok.
English: Dosing for BH4 deficiency
Croatian: Doziranje za nedostatak BH4
English: Single-use sachets.
Croatian: Vrećice za jednokratnu uporabu.
English: Non-clinical data reveal no special hazard for humans based on conventional studies of safety pharmacology (CNS, respiratory, cardiovascular, genitourinary), and toxicity to reproduction.
Croatian: Neklinički podaci ne ukazuju na poseban rizik za ljude na temelju konvencionalnih ispitivanja sigurnosne farmakologije (CNS, respiratorni, kardiovaskularni i genitourinarni sustav) i reproduktivne toksičnosti.
English: Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness.
Croatian: Unutar svake grupe učestalosti, nuspojave su prikazane po redoslijedu opadanja ozbiljnosti.
English: Date of revision of the text: MM/YYYY
Croatian: Datum revizije teksta: {MM/GGGG}
English: What you need to know before you take Kuvan Do not take Kuvan
Croatian: Što morate znati prije nego počnete uzimati Kuvan Nemojte uzimati Kuvan
English: EU/1/08/481/004 100 mg sachet EU/1/08/481/005 500 mg sachet
Croatian: EU/1/08/481/004 vrećica od 100 mg EU/1/08/481/005 vrećica od 500 mg
English: Although concomitant administration of inhibitors of dihydrofolate reductase (e.g. methotrexate, trimethoprim) has not been studied, such medicinal products may interfere with BH4 metabolism.
Croatian: Premda istodobna primjena inhibitora dihidrofolat reduktaze (npr. metotreksata, trimetoprima) nije ispitivana, takvi lijekovi mogu interferirati s metabolizmom BH4.
English: For small infants, an oral syringe can be used.
Croatian: Za malu dojenčad može se primijeniti oralna štrcaljka.
English: Kuvan should not be used during breast-feeding.
Croatian: Kuvan se ne bi trebao primjenjivati tijekom dojenja.
English: Other medicines and Kuvan
Croatian: Drugi lijekovi i Kuvan
English: Paediatric population The posology is the same in adults, children, and adolescents.
Croatian: Pedijatrijska populacija Doziranje u odraslih, djece i adolescenata je isto.
English: Doses may be adjusted up to a total of 20 mg/kg per day.
Croatian: Doze se mogu prilagođavati do ukupno 20 mg/kg na dan.
English: You must continue your diet treatment as recommended by your doctor.
Croatian: Vaše liječenje dijetom morate nastaviti na način koji je propisao Vaš liječnik.
English: BH4 deficiency is a group of disorders arising from mutations or deletions in the genes encoding for one of the five enzymes involved in the biosynthesis or recycling of BH4.
Croatian: Nedostatak BH4 je skupina poremećaja uzrokovanih mutacijama ili delecijama gena koji kodiraju jedan od pet enzima uključenih u biosintezu ili recikliranje BH4.
English: The solution should be drank within 15 to 20 minutes.
Croatian: Otopinu treba popiti unutar 15 do 20 minuta.
English: In preclinical studies, no effects of sapropterin on male and female fertility were observed.
Croatian: U nekliničkim ispitivanjima nisu opaženi učinci sapropterina na mušku i žensku plodnost.
English: Taking the medicine • Drink the solution within 30 minutes.
Croatian: Uzimanje lijeka • Otopinu popijte unutar 30 minuta.
English: What Kuvan is and what it is used for What do you need to know before you take Kuvan How to take Kuvan
Croatian: Što je Kuvan i za što se koristi Što morate znati prije nego počnete uzimati Kuvan Kako uzimati Kuvan
English: Be sure that you know what dose of Kuvan powder your doctor prescribed.
Croatian: Morate znati koju vam je dozu praška Kuvan liječnik propisao.
English: If you forget to take Kuvan
Croatian: Ako ste zaboravili uzeti Kuvan
English: If you take more Kuvan than prescribed, you may experience side effects that could include headache and dizziness.
Croatian: Ako uzmete veću količinu Kuvana od propisane, može doći do nuspojava koje uključuju glavobolju i omaglicu.
English: This medicine is also used to treat an inherited disease called BH4 deficiency in patients of all ages, in which the body cannot produce enough BH4.
Croatian: Ovaj se lijek također primjenjuje za liječenje nasljedne bolesti nedostatka BH4 u bolesnika svih dobi, kod kojih tijelo ne može proizvoditi dovoljno BH4.
English: Caution must be exercised when prescribing to pregnant women.
Croatian: Nužan je oprez kod propisivanja trudnicama.
English: Small particles may be visible in the solution, but they will not affect the effectiveness of the medicine.
Croatian: U otopini mogu ostati vidljive male čestice, ali to neće utjecati na učinkovitost lijeka.
English: A higher intestinal concentration of Kuvan is needed to inhibit BCRP than P-gp, as inhibitory potency in intestine for BCRP (IC50=267 µM) is lower than P-gp (IC50=158 µM).
Croatian: Viša koncentracija lijeka Kuvan u crijevima potrebna je za inhibiciju BCRP-a nego za P-gp jer je potencija inhibicije u crijevima za BCRP (IC50=267 µM) niža nego za P-gp (IC50=158 µM).
English: It is of primary importance to initiate treatment as early as possible to avoid the appearance of non-reversible clinical manifestations of neurological disorders in paediatric patients and cognitive deficits and psychiatric disorders in adults due to sustained elevations of blood phenylalanine.
Croatian: Od primarne je važnosti započeti liječenje što je ranije moguće kako bi se izbjegla pojava nereverzibilnih kliničkih manifestacija neuroloških poremećaja kod pedijatrijskih bolesnika te kognitivni nedostaci i psihijatrijski poremećaji kod odraslih, uzrokovani trajnim povećanjem koncentracije fenilalanina u krvi.
English: Polyethylene terephthalate, aluminum, polyethylene laminate sachet, heat sealed on four sides.
Croatian: Višeslojna vrećica od poli(etilentereftalata), aluminija i polietilena, toplinski zalijepljena sa sve četiri strane.
English: Slowly pull back the plunger to withdraw the amount as instructed by your doctor.
Croatian: Polako povlačite klip kako biste uvukli količinu koju Vam je rekao liječnik.
English: Do not stop taking Kuvan without prior discussion with your doctor, as phenylalanine levels in your blood may increase.
Croatian: Nemojte prekinuti uzimati Kuvan bez prethodnog razgovora sa svojim liječnikom, jer bi Vam se koncentracije fenilalanina u krvi mogle povećati.
English: Do not swallow the tube or the contents.
Croatian: Nemojte progutati spremnik niti njegov sadržaj.
English: Limited studies have been conducted in patients under 4 years of age with BH4 deficiency using another formulation of the same active substance (sapropterin) or an un-registered preparation of BH4.
Croatian: Ograničen broj ispitivanja proveden je kod bolesnika s nedostatkom BH4 mlađih od 4 godine drugačijom formulacijom iste djelatne tvari (sapropterin) ili nekim drugim neregistriranim pripravkom BH4.
English: The Phase III clinical development program for Kuvan included 2, randomised placebo-controlled studies in patients with PKU.
Croatian: Faza III kliničkog razvojnog programa za Kuvan uključuje 2 randomizirana, placebom kontrolirana ispitivanja kod bolesnika s PKU.
English: Draw up the prescribed volume from the solution prepared in the medicine cup and place the tip of the oral syringe into your baby's mouth.
Croatian: . Izvucite propisani volumen pripremljene otopine iz čašice za lijek i postavite vrh oralne štrcaljke u usta djeteta.
English: In both cases, phenylalanine cannot be effectively transformed into the amino acid tyrosine, leading to increased phenylalanine levels in the blood.
Croatian: U oba slučaja fenilalanin se ne može učinkovito pretvoriti u aminokiselinu tirozin, što dovodi do povećanih koncentracija fenilalanina u krvi.
English: A shortening of the QT interval (-8.32 msec) was observed in a study with a single supratherapeutic dose of 100 mg/kg (5 times the maximum recommended dose); this should be taken into consideration in managing patients who have a pre-existing shortened QT interval (e.g. patients with familial short QT syndrome).
Croatian: Skraćivanje QT intervala (-8,32 msek.) opaženo je u studiji s jednom supraterapijskom dozom od 100 mg/kg (5 puta većom od maksimalne preporučene doze); to treba uzeti u obzir pri liječenju bolesnika koji imaju postojeći skraćeni QT interval (npr. bolesnici s prirođenim sindromom kratkog QT intervala).
English: The absorption of sapropterin is higher after a high-fat, high-calorie meal as compared to fasting, resulting, in average, in 40-85% higher maximum blood concentrations achieved 4 to 5 hours after administration.
Croatian: Apsorpcija sapropterina je veća nakon jako masnog, visoko kaloričnog obroka nego nakon gladovanja, što u prosjeku rezultira s 40-85% većom maksimalnom koncentracijom u krvi, postignutom 4 do 5 sati nakon uzimanja lijeka.
English: Important information about some of the ingredients of Kuvan
Croatian: Važne informacije o nekim sastojcima lijeka Kuvan
English: • Open the sachet(s) of Kuvan powder for oral solution by folding and tearing, or cutting at the dotted line in the upper right corner of the sachet.
Croatian: • Vrećicu(e) Kuvan praška za oralnu otopinu otvorite presavijanjem i cijepanjem ili rezanjem točkaste crte u gornjem desnom kutu vrećice.
English: Determination of response
Croatian: Procjena odgovora
English: In non-clinical studies, sapropterin was primarily distributed to the kidneys, adrenal glands, and liver as assessed by levels of total and reduced biopterin concentrations.
Croatian: U nekliničkim ispitivanjima sapropterin se primarno distribuirao u bubrege, nadbubrežne žlijezde i jetru, što je procijenjeno mjerenjem koncentracija ukupnog i reduciranog biopterina.
English: Since sapropterin dihydrochloride is a synthetic version of the naturally occurring 6R-BH4, it can be reasonably anticipated to undergo the same metabolism, including 6R-BH4 regeneration.
Croatian: Budući da je sapropterindiklorid sintetička verzija prirodnog 6R-BH4, razumno je očekivati da će imati isti metabolizam, uključujući i regeneraciju 6R-BH4.
English: Treatment with Kuvan must be initiated and supervised by a physician experienced in the treatment of PKU and BH4 deficiency.
Croatian: Liječenje Kuvanom mora započeti i nadzirati liječnik iskusan u liječenju PKU i nedostatka BH4.
English: The calculated daily dose based on body weight should be rounded to the nearest multiple of 100.
Croatian: Izračun dnevne doze na bazi tjelesne težine treba zaokružiti na najbliži višekratnik broja 100.
English: No tumorigenic activity was observed in an oral carcinogenicity study in mice at doses of up to 250 mg/kg/day (12.5 to 50 times the human therapeutic dose range).
Croatian: Nije primijećena tumorogena aktivnost prilikom istraživanja oralne karcinogeneze kod miševa pri dozama do 250 mg/kg/dnevno (12,5 do 50 puta veće od raspona terapijske doze za ljude).
English: Hypersensitivity reactions (including serious allergic reactions) and rash Metabolism and nutrition disorders
Croatian: Nepoznato: reakcije preosjetljivosti (uključujući ozbiljne alergijske reakcije) i osip Poremećaji metabolizma i prehrane
English: - if you are allergic to sapropterin or any of the other ingredients of this medicine (listed in section 6).
Croatian: - ako ste alergični na sapropterin ili neki drugi sastojak ovog lijeka (naveden u dijelu 6).
English: Sustained or recurrent dysfunction in the phenylalanine-tyrosine-dihydroxy-L-phenylalanine (DOPA) metabolic pathway can result in deficient body protein and neurotransmitter synthesis.
Croatian: Produljeni ili ponavljajući poremećaj u metaboličkom putu fenilalanin-tirozin-dihidroksi-L-fenilalanin (DOPA) može rezultirati nedostatnom sintezom tjelesnih proteina i neurotransmitera.
English: Patients who fail to achieve this level of response within the described one month test period should be considered non-responsive, these patients should not be treated with Kuvan and administration of Kuvan should be discontinued.
Croatian: Smatra se da na lijek ne odgovaraju oni bolesnici koji tijekom opisanog jednomjesečnog pokusnog razdoblja liječenja ne uspiju postići ovu razinu odgovora te ove bolesnike ne treba liječiti Kuvanom i primjenu Kuvana treba prekinuti.
English: In rats, following intravenous radiolabeled sapropterin administration, radioactivity was found to distribute in foetuses.
Croatian: Kod štakora, nakon intravenskog davanja radioaktivno označenog sapropterina, pokazalo se da se radioaktivnost distribuira u fetusima.
English: • the amount of solution you will need to give your child for their prescribed dose
Croatian: • koju količinu otopine trebate dati Vašem djetetu kako bi dobilo propisanu dozu.
English: If you are allergic to sapropterin or any of the other ingredients of this medicine (listed in section 6).
Croatian: Ako ste alergični na sapropterin ili neki drugi sastojak ovog lijeka (naveden u dijelu 6).
English: • If your doctor told you to administer only a portion of the solution, point the tip of the oral syringe into the medicine cup.
Croatian: • Ako Vam je liječnik rekao da primijenite samo dio otopine, uronite vrh oralne štrcaljke u čašicu za lijek.
English: Do not swallow the desiccant capsule contained in the bottle.
Croatian: Nemojte progutati kapsulu sa sredstvom za sušenje koja se nalazi u boci.
English: Kuvan has no or negligible influence on the ability to drive and use machines.
Croatian: Kuvan ne utječe ili zanemarivo utječe na sposobnost upravljanja vozilima i rada sa strojevima.
English: Sapropterin dihydrochloride is primarily metabolised in the liver to dihydrobiopterin and biopterin.
Croatian: Sapropterindiklorid se primarno metabolizira u jetri u dihidrobiopterin i biopterin.
English: Sapropterin is absorbed after oral administration of the dissolved tablet, and the maximum blood concentration (Cmax) is achieved 3 to 4 hours after dosing in the fasted state.
Croatian: Sapropterin se apsorbira nakon oralne primjene otopljene tablete, a maksimalna koncentracija u krvi (Cmax) postiže se 3 do 4 sata nakon uzimanja natašte.
English: Volume of dissolution (ml)
Croatian: Volumen otopine (ml)
English: Based on an in vitro study, there is potential for sapropterin dihydrochloride to inhibit p-glycoprotein (P-gp) and breast cancer resistance protein (BCRP) in the gut at the therapeutic doses.
Croatian: Na temelju in vitro ispitivanja, postoji potencijal da sapropterindiklorid inhibira p-glikoprotein (P-gp) i protein rezistencije raka dojke (BCRP) u crijevima pri terapijskim dozama.
English: In healthy subjects, administration of a single dose of Kuvan at the maximum therapeutic dose of 20 mg/kg had no effect on the pharmacokinetics of a single dose of digoxin (P-gp substrate) administered concomitantly.
Croatian: U zdravih ispitanika primjena jedne doze lijeka Kuvan pri maksimalnoj terapijskoj dozi od 20 mg/kg nije imala nikakvog učinka na farmakokinetiku jedne doze digoksina (P-gp supstrat) istodobno primijenjenog.
English: Because of very low BH4 levels phenylalanine is not used properly and its levels rise, resulting in harmful effects.
Croatian: Zbog vrlo niske koncentracije BH4, tijelo ne može koristiti fenilalanin na ispravan način pa njegova koncentracija raste, što dovodi do štetnih učinaka.
English: It is not known whether sapropterin or its metabolites are excreted in human breast milk.
Croatian: Nije poznato izlučuju li se sapropterin/metaboliti u majčino mlijeko.
English: For instance, a calculated dose of 401 to 450 mg should be rounded down to 400 mg corresponding to 4 tablets.
Croatian: Na primjer, izračunatu dozu od 401 do 450 mg treba zaokružiti na 400 mg, što odgovara količini od 4 tablete.
English: The most commonly reported adverse reactions are headache and rhinorrhoea.
Croatian: Najčešće prijavljene nuspojave bile su glavobolja i rinoreja.
English: If the submission of a PSUR and the update of a RMP coincide, they can be submitted at the same time.
Croatian: Ako se rokovi podnošenja periodičkog izvješća o neškodljivosti (PSUR) podudaraju s nadopunama RMP-a, dokumenti mogu biti podneseni istodobno.
English: Divide the total daily dose into 2 or 3 doses, taken over the day.
Croatian: Podijelite ukupnu dnevnu dozu na 2 ili 3 pojedinačna uzimanja tijekom dana.
English: Not known side effects (frequency cannot be estimated from the available data) Gastritis (inflammation of the lining of the stomach), oesophagitis (inflammation of the lining of the gullet).
Croatian: Nepoznate nuspojave (učestalost se ne može procijeniti iz dostupnih podataka) gastritis (upala sluznice želuca), ezofagitis (upala sluznice jednjaka).
English: Hyperphenylalaninaemia (HPA) is diagnosed as an abnormal elevation in blood phenylalanine levels and is usually caused by autosomal recessive mutations in the genes encoding for phenylalanine hydroxylase enzyme (in the case of phenylketonuria, PKU) or for the enzymes involved in 6R-tetrahydrobiopterin (6R-BH4) biosynthesis or regeneration (in the case of BH4 deficiency).
Croatian: Hiperfenilalaninemija (HPA) se dijagnosticira kao abnormalno povećanje koncentracije fenilalanina u krvi, a obično je uzrokovana autosomno recesivnim mutacijama gena za kodiranje enzima fenilalanin hidroksilaze (u slučaju fenilketonurije PKU) ili za enzime uključene u biosintezu ili regeneraciju 6R-tetrahidrobiopterina (6R-BH4) (u slučaju nedostatka BH4).