FrancophonIA/ELRC3.0 huggingface dataset:
English: It is suggested that depending on its activity, the intestinal bacterial flora may contribute to the initiation of the degradation process, thus leading to a variable extent of metabolism of the molecule.
Croatian: Predloženo je da, ovisno o njenoj aktivnosti, crijevna bakterijska flora može doprinijeti započinjanju procesa razgradnje, dovodeći na taj način do promjenjivog stupnja metabolizma molekule.
English: No adverse effects have been observed on male or female fertility.
Croatian: Nisu zabilježeni štetni učinci na plodnost mužjaka ili ženki.
English: The tablets must be dispersed in a minimum of 5 to 10 ml of water and taken immediately.
Croatian: Tablete treba otopiti u najmanje 5 do 10 ml vode i odmah popiti.
English: Uncommon: bradycardia
Croatian: Manje često: bradikardija
English: The effects of Carbaglu on pregnancy and the unborn child are not known.
Croatian: Nisu poznati učinci lijeka Carbaglu na trudnoću i nerođeno dijete.
English: Safety pharmacology studies have shown that Carbaglu administered orally at doses of 250, 500, 1000 mg/kg had no statistically significant effect on respiration, central nervous system and cardiovascular system.
Croatian: Studije o farmakološkoj sigurnosti pokazale su da peroralna primjena Carbaglu u dozama od 250, 500, 1000 mg/kg nije imala statistički značajan učinak na disanje, središnji živčani sustav i kardiovaskularni sustav.
English: Your doctor will determine the dose suitable to you in order to maintain normal ammonia levels in your blood.
Croatian: Vaš liječnik će odrediti dozu prikladnu za Vas kako bi se održale normalne količine amonijaka u Vašoj krvi.
English: Patients with this rare disorder are not able to eliminate nitrogen waste, which builds up after eating protein.
Croatian: Bolesnici s ovim rijetkim poremećajem ne mogu odstraniti ostatni dušik koji se nagomilava nakon proteinskog obroka.
English: Do not take Carbaglu:
Croatian: Nemojte uzimati Carbaglu:
English: Ammonia is especially toxic for the brain and leads, in severe cases, to reduced levels of consciousness and to coma.
Croatian: Amonijak je posebno toksičan za mozak te dovodi, u teškim slučajevima, do poremećaja svijesti i kome.
English: Carbaglu should ONLY be administered by mouth or via a feeding tube into the stomach (using a syringe, if necessary).
Croatian: Carbaglu se smije primijeniti SAMO kroz usta ili putem cijevi za hranjenje u trbuh (koristeći štrcaljku, ako je potrebno).
English: • Uncommon: bradycardia (decreased frequency of the heart), diarrhoea, fever, increased transaminases, vomiting
Croatian: • Manje često: bradikardija (smanjena frekvencija srca), proljev, vrućica, porast vrijednosti transaminaza, povraćanje
English: Carbaglu must be taken orally before meals or feedings.
Croatian: Carbaglu se mora uzimati na usta prije obroka ili hranjenja.
English: Frequencies are defined as: very common (≥ 1/10), common (≥1/100 to 1/10), uncommon (≥1/1,000 to 1/100), rare (≥ 1/10,000 to < 1/1,000), very rare (< 1/10,000), not known (cannot be estimated from the available data).
Croatian: Učestalosti su definirane kao: vrlo često (≥1/10), često (≥1/100 i 1/10), manje često (≥1/1000 i 1/100), rijetko (≥1/10000 i <1/1000), vrlo rijetko (<1/10000), nepoznato (ne može se procijeniti iz dostupnih podataka).
English: Detailed information on this medicine is available on the European Medicines Agency web site: http://www.ema.europa.eu.
Croatian: Detaljne informacije o ovom lijeku dostupne su na web stranici Europske agencije za lijekove: http://www.ema.europa.eu.
English: If you stop taking Carbaglu Do not stop Carbaglu without informing your doctor.
Croatian: Nemojte prestati uzimati Carbaglu bez informiranja svojeg liječnika.
English: Common: increased sweating Not known: rash
Croatian: Često: pojačano znojenje Nepoznato: osip
English: Carbaglu showed no significant mutagenic activity in a battery of genotoxicity tests performed in vitro (Ames test, human lymphocyte metaphase analysis) and in vivo (micronucleus test in rat).
Croatian: Carbaglu nije pokazao značajnu mutagenu aktivnost u skupini genotoksičnih testova provedenih in vitro (Amesov test, analiza metafaze ljudskih limfocita) te in vivo (mikronukleus test na štakoru).
English: Store in a refrigerator (2C - 8C)
Croatian: Čuvati u hladnjaku (2°C - 8°C)
English: As very few data on the safety of carglumic acid are available, systematic surveillance of liver, renal, cardiac functions and haematological parameters is recommended.
Croatian: Kako je dostupno vrlo malo podataka o sigurnosti karglumatne kiseline, preporučuje se sistematsko praćenje jetrene, bubrežne i srčane funkcije te hematoloških parametara.
English: In one patient treated with carglumic acid, where the dose was increased up to 750 mg/kg/day, symptoms of intoxication occurred which can be characterised as a sympathomimetic reaction: tachycardia, profuse sweating, increased bronchial secretion, increased body temperature and restlessness.
Croatian: Kod jednog bolesnika liječenog karglumatnom kiselinom, kod kojeg je doza povišena do 750 mg/kg/dan, pojavili su se simptomi intoksikacije koji se mogu opisati kao simpatomimetička reakcija: tahikardija, obilno znojenje, povećana bronhalna sekrecija, povišena tjelesna temperatura i nemir.
English: Taking Carbaglu with food and drink
Croatian: Uzimanje Carbaglu s hranom i tekućinom
English: The pharmacokinetics of carglumic acid has been studied in healthy male volunteers using both radiolabelled and unlabelled product.
Croatian: Farmakokinetika karglumatne kiseline proučavana je na zdravim muškim dobrovoljcima pomoću radioaktivno označenog i neoznačenog lijeka.
English: Carbaglu is indicated in treatment of
Croatian: Carbaglu je indiciran za liječenje:
English: At that dose-level, in 12 volunteers given Carbaglu tablets, plasma concentration peaked at 2.6 µg/ml (median; range 1.8-4.8) after 3 hours (median; range 2-4).
Croatian: Pri tim dozama, kod 12 dobrovoljaca koji su dobivali Carbaglu tablete, vršna plazmatska koncentracija bila je 2,6 μg/ml (medijan; raspon 1,8-4,8) nakon 3 sata (medijan; raspon 2
English: In rats and rabbits no evidence has been seen of embryotoxicity, foetotoxicity or teratogenicity up to maternotoxic doses leading to fifty times exposure as compared to humans in rats and seven times in rabbits.
Croatian: Kod štakora i kunića nije bilo dokaza embriotoksičnosti, fetotoksičnosti ili teratogenosti sve do doza toksičnih za majku kod pedeset puta veće izloženosti kod štakora u usporedbi s ljudima te sedam puta kod kunića.
English: Nevertheless, as carglumic acid has been shown to be present in the milk of lactating rats with potential toxic effects for their fed pups, you should not breast feed your baby if you are taking Carbaglu.
Croatian: Međutim, kako se pokazalo da se karglumatna kiselina nalazi u mlijeku ženki štakora tijekom laktaciji te može imati toksične učinke na mladunčad koju doje, ne smijete dojiti svoje dijete ukoliko uzimate Carbaglu.
English: If any of the side effects gets serious, or if you notice any side effects not listed in this leaflet, please tell your doctor or pharmacist.
Croatian: Ako se bilo koja nuspojava pogorša ili ako primijetite bilo koju nuspojavu koja nije navedena u ovoj uputi, molimo obavijestite svog liječnika ili ljekarnika.
English: The end product of carglumic acid metabolism is carbon dioxide, which is eliminated through the lungs.
Croatian: Završni produkt metabolizma karglumatne kiseline je ugljični dioksid koji se odstranjuje plućima.
English: Detailed information on this medicinal product is available on the website of the European Medicines Agency (EMA) http://www.ema.europa.eu/.
Croatian: Detaljne informacije o ovom lijeku dostupne su na webstranici Europske agencije za lijekove (EMA) http://www.ema.europa.eu/.
English: Hypersensitivity to the active substance or to any of the excipients.
Croatian: Preosjetljivost na djelatnu tvar ili bilo koju od pomoćnih tvari.
English: Single doses of carglumic acid up to 2800 mg/kg orally and 239 mg/kg intravenously did not induce any mortality or abnormal clinical signs in adult rats.
Croatian: Pojedinačne doze karglumatne kiseline do 2800 mg/kg oralno te 239 mg/kg intravenski nisu dovele do mortaliteta ili abnormalnih kliničkih znakova kod odraslih štakora.
English: Your doctor will evaluate your individual responsiveness to carglumic acid before initiating any long term treatment.
Croatian: Liječnik će ispitati Vaš individualni odgovor na karglumatnu kiselinu prije započinjanja dugotrajnog liječenja.
English: What Carbaglu contains - The active substance is carglumic acid.
Croatian: Što Carbaglu sadrži - Djelatna tvar je karglumatna kiselina.
English: Carbaglu is presented in a plastic container of 5, 15 and 60 tablets which is closed with a child resistant cap.
Croatian: Carbaglu dolazi u plastičnom spremniku od 5, 15 i 60 tableta koji je zatvoren poklopcem sa zaštitom za djecu.
English: Although it is not known whether carglumic acid is secreted into human milk, it has been shown to be present in the milk of lactating rats (see section 5.3).
Croatian: Iako nije poznato izlučuje li se karglumatna kiselina u majčinom mlijeku ljudi, pokazano je da se nalazi u mlijeku štakorica tijekom laktacije (vidjeti dio 5.3).
English: The excretion of carglumic acid into breast milk has not been studied in women.
Croatian: Izlučivanje karglumatne kiseline u majčino mlijeko nije proučeno kod žena.
English: Carglumic acid responsiveness test It is recommended to test individual responsiveness to carglumic acid before initiating any long term treatment.
Croatian: Test odgovora na karglumatnu kiselinu Preporučuje se testirati individualni odgovor na karglumatnu kiselinu prije započinjanja bilo kojeg dugoročnog liječenja.
English: No carcinogenicity study has been conducted with carglumic acid.
Croatian: Studije karcinogenosti nisu provedene s karglumatnom kiselinom.
English: Uncommon: pyrexia
Croatian: Manje često: pireksija
English: Within each frequency grouping, undesirable effects are presented in order of decreasing seriousness.
Croatian: Unutar svake skupine učestalosti nuspojave su prikazane u padajućem nizu prema ozbiljnosti
English: After a single oral dose of 100 mg/kg body weight, approximately 30% of carglumic acid is estimated to be absorbed.
Croatian: Nakon pojedinačne oralne doze od 100 mg/kg tjelesne težine, procijenjeno je da se apsorbira oko 30% karglumatne kiseline.
English: Plasma levels of carglumic acid were measured in patients of all age categories, from newborn infants to adolescents, treated with various daily doses (7 - 122 mg/kg/day).
Croatian: Razina karglumatne kiseline u plazmi izmjerena je kod bolesnika svih dobnih skupina, od novorođenčadi do adolescenata, liječenih raznim dnevnim dozama (7 - 122 mg/kg/dan).
English: For any information about this medicinal product, please contact the local representative of the Marketing Authorisation Holder.
Croatian: Za sve infromacije o ovom lijeku obratite se lokalnom predstavniku nositelja odobrenja za stavljanje u promet gotovog lijeka:
English: Carbaglu treatment should be initiated under the supervision of a physician experienced in the treatment of metabolic disorders.
Croatian: Liječenje lijekom Carbaglu treba započeti pod nadzorom liječnika s iskustvom u liječenju poremećaja metabolizma.
English: Hyperammonaemia may be due to
Croatian: Hiperamonijemija se može javiti zbog
English: The tablets must be dispersed in a minimum of 5-10 ml of water and ingested immediately or administered by fast push through a syringe via a nasogastric tube.
Croatian: Tablete se moraju otopiti u najmanje 5-10 ml vode i odmah popiti ili primijeniti brzim istiskivanjem štrcaljke kroz nazogastrični tubus.
English: Plasma levels of ammonia and amino acids should be maintained within normal limits.
Croatian: Razine amonijaka i aminokiselina u plazmi se moraju održavati unutar normalnih granica.
English: If you forget to take Carbaglu Do not take a double dose to make up for forgotten individual doses.
Croatian: Nemojte uzeti dvostruku dozu kako biste nadoknadili zaboravljenu pojedinačnu dozu.
English: Uncommon: increased transaminases
Croatian: Manje često: povišene vrijednosti transaminaza
English: - Undesirable effects in organic acidaemia
Croatian: - Nuspojave kod organske acidemije
English: After first opening of the container: do not refrigerate, do not store above 30C.
Croatian: Nakon prvog otvaranja spremnika: ne odlagati u hladnjak, ne čuvati na temperaturi iznad 30°C.
English: Discard 3 months after first opening.
Croatian: Lijek zbrinite 3 mjeseca nakon prvog otvaranja.
English: The following side effects were reported as follows: very common (occurring in at least one in 10 patients), common (occurring in at least one in 100 patients), uncommon (occurring in at least one in1,000 patients), rare (occurring in at least one in 10,000 patients), very rare (occurring in at least one in 100,000 patients) and not known (frequency cannot be estimated from the available data).
Croatian: Sljedeće nuspojave prijavljene su kako slijedi: vrlo često (javljaju se kod barem jednog na 10 bolesnika), često (javljaju se kod barem jednog na 100 bolesnika), manje često (javljaju se kod barem jednog na 1 000 bolesnika), rijetko (javljaju se kod barem jednog na 10 000 bolesnika), vrlo rijetko (javljaju se kod barem jednog na 100 000 bolesnika) i nepoznato (učestalost se ne može procijeniti iz dostupnih podataka).
English: Metabolites are detectable in plasma with a peak at 36-48 hours and a very slow decline (half-life around 100 hours).
Croatian: Metaboliti se mogu mjeriti u plazmi s vršnom koncentracijom nakon 36-48 sati te vrlo sporim padom (poluvijek oko 100 sati).
English: When the patient is in hyperammonaemic coma, Carbaglu is administered by fast push through a syringe via the tube set up and used to feed you.
Croatian: Kada je bolesnik u stanju hiperamonijačne kome, Carbaglu se primjenjuje brzim potiskivanjem sadržaja unutar štrcaljke kroz postavljenu cijev koja se koristi za hranjenje.
English: One metabolite that has been identified in the faeces is glutamic acid.
Croatian: Jedan metabolit koji je identificiran u stolici je glutaminska kiselina.
English: Reported adverse reactions are listed below, by system organ class and by frequency.
Croatian: Zabilježene nuspojave navedene su niže, prema klasifikaciji organskih sustava i učestalosti.
English: Breast-feeding during the use of carglumic acid is contraindicated (see sections 4.6 and 5.3).
Croatian: Kontraindicirano je dojenje tijekom primjene karglumatne kiseline (vidjeti dio 4.6 i 5.3).
English: In newborn rats receiving daily carglumic acid by oral gavage for 18 days as well as in young rats receiving daily carglumic acid for 26 weeks, the No Observed Effect Level (NOEL) was established at 500 mg/kg/day and the No Observed Adverse Effect Level (NOAEL) was established at 1000 mg/kg/day.
Croatian: Kod novorođenih štakora koji su dnevno primali karglumatnu kiselinu putem oralne cjevčice za hranjenje kroz 18 dana, kao i kod mladih štakora koji su dnevno primali karglumatnu kiselinu kroz 26 tjedana, utvrđena je doza bez uočenog učinka (No Observed Effect Level; NOEL) pri 500 mg/kg/dan te doza bez uočenog štetnog učinka (No Observed Adverse Effect Level; NOAEL) pri 1000 mg/kg/dan.
English: The initial daily dose should be 100 mg/kg, up to 250 mg/kg if necessary.
Croatian: Početna dnevna doza treba biti 100 mg/kg do 250 mg/kg ako je potrebno.
English: No studies on the effects on the ability to drive and use machines have been performed.
Croatian: Nisu provedene specifične studije o učincima na sposobnost upravljanja vozilima i rada na strojevima.
English: • For isovaleric acidaemia, methylmalonic acidaemia and propionic acidaemia: The treatment should start upon hyperammonaemia in organic acidaemia patients.
Croatian: • Za izovaleričnu acidemiju, metilmaloničnu acidemiju i propionsku acidemiju: Liječenje je potrebno početi nakon hiperamonijemije u bolesnika s organskom acidemijom.
English: Carglumic acid is secreted in the milk of lactating rats and although developmental parameters were unaffected, there were some effects on body weight / body weight gain of pups breast-fed by dams treated with 500 mg/kg/day and a higher mortality of pups from dams treated with 2000 mg/kg/day, a dose that caused maternotoxicity.
Croatian: Karglumatna kiselina izlučuje se u mlijeku ženki štakora te iako nije utjecala na razvojne parametre, bilo je nekih učinaka na tjelesnu težinu/porast tjelesne težine mladunčadi dojene od majki koje su dobivale 500 mg/kg/dan te povećane smrtnosti mladunčadi majki koje su dobivale 2000/kg/dan, dozu koja je dovodila do toksičnosti u ženki.
English: - This medicine has been prescribed for you personally and you should not pass it on to others.
Croatian: - Ovaj je lijek propisan samo Vama.
English: Carglumic acid is a structural analogue of N-acetylglutamate, which is the naturally occurring activator of carbamoyl phosphate synthetase, the first enzyme of the urea cycle.
Croatian: Karglumatna kiselina je strukturni analog N-acetilglutamata koji je prirodan aktivator karbamoil fosfat sintetaze, prvog enzima u ciklusu ureje.
English: The plasma elimination curve of carglumic acid is biphasic with a rapid phase over the first 12 hours after administration followed by a slow phase (terminal half life up to 28 hours).
Croatian: Krivulja eliminacije karglumatne kiseline iz plazme je bifazična s brzom fazom unutar prvih 12 sati od primjene praćena sporom fazom (poluvijek do 28 sati).
English: In patients with organic acidaemia (neonates and non-neonates), the treatment with carglumic acid induced a quick decrease of ammonia plasma levels, reducing the risk of neurological complications.
Croatian: U bolesnika s organskom acidemijom (novorođenčad i dojenčad) liječenje karglumatnom kiselinom dovelo je do brzog smanjenja razine amonijaka u plazmi, smanjujući rizik pojave neuroloških komplikacija.
English: Do not use after the expiry date stated on the tablet container.
Croatian: Ne smije se upotrijebiti nakon isteka roka valjanosti navedenog na spremniku za tablete.
English: After first opening of the tablet container: do not refrigerate, do not store above 30C.
Croatian: Nakon prvog otvaranja spremnika s tabletama: ne odlagati u hladnjak, ne čuvati na temperaturi iznad 30°C.
English: In patients with N-acetylglutamate synthase deficiency, carglumic acid was shown to induce a rapid normalisation of plasma ammonia levels, usually within 24 hours.
Croatian: Kod bolesnika s deficitom N-acetilglutamat sintaze pokazalo se da karglumatna kiselina dovodi do brze normalizacije razine amonijaka u plazmi, obično unutar 24 sata.
English: Despite a lower affinity of carbamoyl phosphate synthetase for carglumic acid than for N-acetylglutamate, carglumic acid has been shown in vivo to stimulate carbamoyl phosphate synthetase and to be much more effective than N-acetylglutamate in protecting against ammonia intoxication in rats.
Croatian: Iako karbamoil fosfat sintetaza ima manji afinitet za karglumatnu kiselinu nego za N-acetilglutamat, pokazalo se kako karglumatna kiselina stimulira karbamoil fosfat sintetazu in vivo te je puno učinkovitija od N-acetilglutamata u zaštiti od intoksikacije amonijakom kod štakora.
English: BEFORE YOU TAKE CARBAGLU
Croatian: PRIJE NEGO POČNETE UZIMATI CARBAGLU
English: If you have any further questions on the use of this product, ask your doctor or pharmacist.
Croatian: Ako imate dodatna pitanja o primjeni ovog lijeka, upitajte svog liječnika ili ljekarnika.
English: Pregnancy and Breast-feeding
Croatian: Trudnoća i dojenje
English: Your doctor may prescribe supplemental arginine or restrict your protein intake.
Croatian: Liječnik Vam može propisati nadomjestak arginina ili Vam ograničiti unos bjelančevina.
English: EXP {MM/YYYY} Discard 3 months after first opening.
Croatian: Rok valjanosti: {MM/GGGG} Zbrinuti 3 mjeseca dana nakon prvog otvaranja.
English: The suspension has a slightly acidic taste.
Croatian: Otopina ima blago kiselkasti okus.
English: Do not take Carbaglu during breast-feeding
Croatian: Nemojte uzimati Carbaglu tijekom dojenja.
English: After a single oral dose of 100 mg/kg body weight, 9% of the dose is excreted unchanged in the urine and up to 60% in the faeces.
Croatian: Nakon pojedinačne oralne doze od 100 mg/kg tjelesne težine, 9% doze izlučuje se nepromijenjeno mokraćom te do 60% stolicom.
English: Carglumic acid was shown to decrease blood ammonia levels and increase urea levels in blood and urine, whereas the liver content of carbamoyl phosphate synthetase activators was significantly increased.
Croatian: Pokazalo se da karglumatna kiselina smanjuje razinu amonijaka u krvi te povećava razinu ureje u krvi i mokraći, dok je razina aktivatora karbamoil fosfat sintetaze u jetri bila značajno povišena.
English: This medicine is for oral use ONLY (ingestion or via a nasogastric tube using a syringe, if necessary).
Croatian: Ovaj je lijek SAMO za peroralnu primjenu (gutanjem ili putem nazogastrične sonde uporabom štrcaljke, ako je potrebno).
English: Each tablet contains 200 mg of carglumic acid.
Croatian: Svaka tableta sadrži 200 mg karglumatne kiseline.
English: Take special care with Carbaglu:
Croatian: Budite posebno oprezni s lijekom Carbaglu:
English: • For N-acetylglutamate synthase deficiency:
Croatian: • Za deficijenciju N-acetilglutamat sintaze:
English: - In a patient with moderate hyperammonaemia, administer a test dose of 100 to 200 mg/kg/day for 3 days with a constant protein intake and perform repeated determinations of ammonia plasma concentration (before and 1 hour after a meal); adjust the dose in order to maintain normal ammonia plasma levels.
Croatian: - Kod bolesnika s umjerenom hiperamonijemijom, primijenite testnu dozu od 100 do 200 mg/kg/dan kroz 3 dana uz stalan unos proteina te ponavljano mjerite koncentracije amonijaka u plazmi (prije i 1 sat nakon obroka).
English: - If you have further questions, please ask your doctor or your pharmacist.
Croatian: - Ukoliko imate dodatnih pitanja, obratite se svom liječniku ili ljekarniku.
English: The usual dose: the initial daily dose is usually 100 mg per kilogram of body weight, up to a maximum of 250 mg per kilogram of body weight (for example, if you weight 10kg, you should take 1g per day, or 5 tablets), For patients suffering from N-acetylglutamate synthase deficiency, in the long term, the daily dose usually ranges from 10 mg to 100 mg per kilogram of body weight.
Croatian: Uobičajena doza: početna dnevna doza je obično 100 mg po kilogramu tjelesne težine, sve do maksimalno 250 mg po kilogramu tjelesne težine (na primjer, ako imate 10 kg, trebate uzimati 1 g na dan, ili 5 tableta).
English: What Carbaglu looks like and contents of the pack Carbaglu 200mg tablet is a bar-shaped tablet, with 4 punches on one side with 3 break-mark sides.
Croatian: Carbaglu tableta od 200 mg je izdužena tableta, s 4 udubljenja na jednoj strani, s 3 oznake za prelamanje.
English: Therefore, breast-feeding during the use of carglumic acid is contraindicated (see section 4.3).
Croatian: Stoga je kontraindicirano dojenje tijekom primjene karglumatne kiseline (vidjeti dio 4.3).
English: The dose should be individually adjusted in order to maintain normal ammonia plasma levels.
Croatian: Dozu je potrebno individualno prilagoditi da bi se održala normalna razina amonijaka u plazmi.
English: HOW TO TAKE CARBAGLU
Croatian: KAKO UZIMATI CARBAGLU
English: Uncommon: diarrhoea, vomiting
Croatian: Manje često: proljev, povraćanje
English: • hyperammonaemia due to N-acetylglutamate synthase primary deficiency.
Croatian: • hiperamonijemije uzrokovane primarnom deficijencijom N-acetilglutamat sintaze.
English: • isovaleric acidaemia, methylmalonic acidaemia or propionic acidaemia.
Croatian: • izovalerične acidemije, metilmalonične acidemije ili propionske acidemije.
English: It should then be individually adjusted in order to maintain normal ammonia plasma levels (see section 4.4).
Croatian: Zatim ju treba individualno prilagoditi da bi se održala normalna razina amonijaka u plazmi (vidjeti dio 4.4).
English: • the lack of a specific liver enzyme N- acetylglutamate synthase.
Croatian: • manjka specifičnog jetrenog enzima N-acetilglutamat sintaze.