FrancophonIA/ELRC3.0 huggingface dataset:
English: If you stop taking Carbaglu Do not stop Carbaglu without informing your doctor.
Danish: Hvis De holder op med at tage Carbaglu De må ikke holde op med at tage Carbaglu uden at informere lægen.
English: The tablets are white and elongated with three score marks and engraved on one side.
Danish: Tabletterne er hvide og aflange med tre brudriller og præget på den ene side.
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.
Danish: Da der foreligger meget få data om sikkerheden ved cargluminsyre, anbefales systematisk overvågning af lever- og nyreparametre, kardielle funktioner og hæmatologiske parametre.
English: In the long term, it may not be necessary to increase the dose according to body weight as long as adequate metabolic control is achieved; daily doses range from 10 mg/kg to 100 mg/kg.
Danish: I det lange løb behøver det ikke at være nødvendigt at øge dosis i henhold til legemsvægt, så længe tilstrækkelig metabolisk kontrol opnås; daglige dosisområde fra 10 mg/kg til 100 mg/kg.
English: Do not take Carbaglu if you are hypersensitive (allergic) to carglumic acid or any of the other ingredients of Carbaglu.
Danish: Tag ikke Carbaglu : -hvis De er overfølsom (allergisk) over for cargluminsyre eller et af de øvrige indholdsstoffer.
English: This leaflet was last approved on
Danish: Denne indlægsseddel blev senest godkendt {dato}
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.
Danish: Sikkerhedsmæssige farmakologiske undersøgelser har vist, at Carbaglu administreret oralt i doser på 250, 500, 1000 mg/kg ikke havde nogen statistisk signifikant virkning på respiration, centralnervesystem og kardiovaskulære system.
English: After a single oral dose of 100 mg/kg body weight, approximately 30% of carglumic acid is estimated to be absorbed.
Danish: Efter en enkelt oral dosis på 100 mg/kg kropsvægt estimeres det, at omtrent 30% af cargluminsyren absorberes.
English: Uncommon: pyrexia
Danish: Ikke almindelig: pyreksi
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.
Danish: Tabletterne skal dispergeres i et minimum af 5-10 ml vand og straks indtages eller gives ved et hurtigt tryk gennem en sprøjte via en nasogastrisk tube.
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).
Danish: Hyppigheder definers som: meget almindelig (≥ 1/10), almindelig (≥1/100 til 1/10) ikke almindelig (≥1/1.000 til 1/100), sjælden (≥ 1/10.000 til < 1/1.000), meget sjælden (< 1/10.000), ikke kendt (kan ikke estimeres ud fra forhåndenværende data).
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).
Danish: Carbaglu viste ingen signifikant mutagen aktivitet i et batteri af genotoksiske tests udført in vitro (Ames test, human lymfocyt metafase analyse) og in vivo (mikronucleustest på rotter).
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.
Danish: Hos rotter og kaniner var der ingen tegn på embryotoksicitet, føtotoksicitet eller teratogenicitet ved op til modertoksiske doser, der for rotter gav halvtreds ganges eksponering sammenlignet med humane doser og syv gange for kaniner.
English: Your doctor may prescribe supplemental arginine or restrict your protein intake.
Danish: Deres læge kan ordinere tilskud af arginin eller begrænse Deres indtagelse af protein.
English: The initial daily dose should be 100 mg/kg, up to 250 mg/kg if necessary.
Danish: Den initiale daglige dosis skal være 100 mg/kg, op til 250 mg/kg hvis nødvendigt.
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.
Danish: Hos en patient behandlet med cargluminsyre, hvor dosis blev øget op til 750 mg/kg/dag, forekom der forgiftningssymptomer, som kan karakteriseres som en sympatomimetisk reaktion: Tachycardi, kraftig sveden, øget bronchial sekretion, øget legemstemperatur og hvileløshed.
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).
Danish: Plasmaeliminationskurven for cargluminsyre er bifasisk med en hurtig fase over de første 12 timer efter administrering efterfulgt af en langsom fase (terminal halveringstid op til 28 timer).
English: The end product of carglumic acid metabolism is carbon dioxide, which is eliminated through the lungs.
Danish: Slutproduktet af cargluminsyremetabolisme er carbondioxid, som elimineres gennem lungerne.
English: Oral use ONLY Read the package leaflet before use.
Danish: KUN til oral anvendelse Læs indlægssedlen inden brug.
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).
Danish: Plasmaniveauer af cargluminsyre blev målt hos patienter i alle aldersgrupper, fra nyfødte spædbørn til halvvoksne, behandlet med forskellige daglige doser (7 - 122 mg/kg/dag).
English: Always take Carbaglu exactly as your doctor has instructed you.
Danish: Tag altid Carbaglu nøjagtigt efter lægens anvisning.
English: Carbaglu is presented in a plastic container of 5, 15 and 60 tablets which is closed with a child resistant cap.
Danish: Carbaglu leveres i en plastbeholder med 5, 15 og 60 tabletter, som er lukket med et børnesikkert låg.
English: Detailed information on this medicinal product is available on the website of the European Medicines Agency (EMA) http://www.ema.europa.eu/.
Danish: Yderligere information om dette lægemiddel er tilgængelig på Det Europæiske Lægemiddelagenturs (EMAs) hjemmeside http://www.ema.europa.eu/.
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.
Danish: Enkeltdosis af cargluminsyre op til 2800 mg/kg oralt og 239 mg/kg intravenøst inducerede ikke nogen mortalitet eller abnorme kliniske tegn hos voksne rotter.
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).
Danish: Følgende bivirkninger blev rapporteret som følger: meget almindelig (forekommer hos mindst en af 10 patienter), almindelig (forekommer hos mindst en af 100 patienter), ikke almindelig (forekommer hos mindst en af 1.000 patienter), sjælden (forekommer hos mindst en af 10.000 patienter), meget sjælden (forekommer hos mindst en af 100.000 patienter) og ikke kendt (hyppigheden kan ikke estimeres ud fra forhåndenværende data).
English: Carbaglu should ONLY be administered by mouth or via a feeding tube into the stomach (using a syringe, if necessary).
Danish: Carbaglu må KUN indtages gnnem munden eller via en ernæringssonde ned i maven (ved brug af en sprøjte, hvis det er nødvendigt).
English: Patients with this rare disorder are not able to eliminate nitrogen waste, which builds up after eating protein.
Danish: Patienter med denne sjældne lidelse er ikke istand til at udskille nitrogen (kvælstof), der hober sig op efter indtagelse af protein.
English: Your doctor will determine the dose suitable to you in order to maintain normal ammonia levels in your blood.
Danish: Deres læge vil fastlægge den dosis, der er passende for Dem for at kunne opretholde normale ammoniakniveauer i Deres blod.
English: Hyperammonaemia may be due to
Danish: Hyperammonæmi kan skyldes
English: Common: increased sweating Not known: rash
Danish: Almindelig: øget svedtendens Ikke kendt:udslæt
English: This medicine is for oral use ONLY (ingestion or via a nasogastric tube using a syringe, if necessary).
Danish: Dette lægemiddel er KUN til oral anvendelse (indtagelse eller via nasogastrisk sonde vha. en sprøjte, hvis det er nødvendigt).
English: Metabolites are detectable in plasma with a peak at 36-48 hours and a very slow decline (half-life around 100 hours).
Danish: Metabollitter kan måles i plasma i maksimale niveauer efter 36-48 timer og med en meget langsom nedbrydning (halveringstid omkring 100 timer).
English: Carbaglu must be taken orally before meals or feedings.
Danish: Carbaglu skal tages gennem munden inden måltider eller fødeindtagelse.
English: This disorder persists during the entire life of the affected patient and therefore the need for this treatment is lifelong.
Danish: Denne lidelse kan ikke kureres, og behovet for behandling er derfor livslangt.
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.
Danish: Den sædvanlige dosis: - Den indledende daglige dosis er sædvanligvis 100 mg pr. kilo legemsvægt, op til maksimalt 250 mg pr. kilo legemsvægt (f.eks. hvis De vejer 10 kg, skal De tage 1 g om dagen, eller 5 tabletter), - Til patienter, der lider af N-acetylglutamatsyntasemangel, vil den daglige dosis sædvanligvis ligge i intervallet fra 10 mg til 100 mg pr. kilo legemsvægt.
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.
Danish: Hos nyfødte rotter, der fik daglig cargluminsyre ved oral gavage i 18 dage såvel som hos unge rotter, der fik daglig cargluminsyre i 26 uger, blev der fastlagt en No Observed Effect Level (NOEL) ved 500 mg/kg/dag og en No Observed Adverse Effect Level (NOAEL) blev fastlagt ved 1000 mg/kg/dag.
English: This could be explained by the following observations: i) The mitochondrial membrane is more readily permeable for carglumic acid than for Nacetylglutamate ii) Carglumic acid is more resistant than N-acetylglutamate to hydrolysis by aminoacylase present in the cytosol.
Danish: Dette kunne forklares ved følgende iagttagelser: i) Mitochondriemembranen gennemtrænges lettere af cargluminsyre end af N-acetylglutamat ii) Cargluminsyre er mere resistent end N-acetylglutamat overfor hydrolyse med aminoacylase tilstede i cytosolen.
English: The pharmacokinetics of carglumic acid has been studied in healthy male volunteers using both radiolabelled and unlabelled product.
Danish: Cargluminsyres farmakokinetik er blevet undersøgt hos raske, mandlige frivillige ved anvendelse af både radioaktivt mærket og umærket stof.
English: Carbaglu treatment should be initiated under the supervision of a physician experienced in the treatment of metabolic disorders.
Danish: Carbaglubehandling skal indledes under overvågning af en læge, der har erfaring i behandlingen af metaboliske sygdomme.
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.
Danish: Cargluminsyre viste sig at nedsætte ammoniakniveauerne i blodet og øge ureaniveauerne i blod og urin, medens leverens indhold af carbamoylfosfatsyntetaseaktivatorer var signifikant øgede.
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.
Danish: Efter en enkelt oral dosis på 100 mg/kg kropsvægt udskilles 9% af dosen uomdannet i urinen og op til 60% i fæces.
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).
Danish: Ved dette dosisniveau, givet som Carbaglu tabletter til 12 frivillige, blev der opnået maksimale plasmakoncentrationer på 2,6 µg/ml (median; værdier mellem 1,8 og 4,8) efter 3 timer (median; værdier mellem 2 og 4).
English: No carcinogenicity study has been conducted with carglumic acid.
Danish: Der er ikke foretaget nogen carcinogenicitetsundersøgelse med cargluminsyre.
English: Uncommon: diarrhoea, vomiting
Danish: Ikke almindelig: diarré, opkastning
English: Carbaglu can help eliminating excessive ammonia plasma levels (elevated ammonia level in the blood).
Danish: Carbaglu kan medvirke til eliminere usædvanligt høje niveauer af ammoniak i blodet (hyperammonæmi).
English: As examples - In a comatose child, start with a dose of 100 to 250 mg/kg/day and measure ammonia plasma concentration at least before each administration; it should normalise within a few hours after starting Carbaglu.
Danish: Som for eksempel : - Hos et komatøst barn startes med en dosis på 100 til 250 mg/kg/dag og ammoniak plasmakoncentration måles i det mindste før hver indgivelse; den skulle normaliseres indenfor et par timer efter start med Carbaglu.
English: • Common: increased sweating
Danish: • Almindelig: øget svedtendends
English: What Carbaglu is and what it is used for Before you take Carbaglu How to take Carbaglu Possible side effects 5 How to store Carbaglu Further information
Danish: Carbaglus virkning og hvad De skal bruge det til Det skal De vide, før De begynder at tage Carbaglu Sådan skal De tage Carbaglu Bivirkninger
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.
Danish: Når patienten er i hyperammonæmisk coma, gives Carbaglu ved et hurtigt tryk gennem en sprøjte via den sonde, der er lagt for at ernære Dem.
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.
Danish: Cargluminsyre er en strukturel analog til N-acetylglutamat, som er den naturligt forekommende aktivator af carbamoylfosfatsyntetase, det første enzym i ureacyklussen.
English: SPECIAL WARNING THAT THE MEDICINAL PRODUCT MUST BE STORED OUT OF THE REACH AND SIGHT OF CHILDREN
Danish: ADVARSEL OM, AT LÆGEMIDLETY SKAL OPBEVARES UTILGÆNGELIGT FOR BØRN
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.
Danish: Hos patienter med N-acetylglutamatsyntase mangel, viste cargluminsyre sig at inducere en hurtig normalisering af plasma ammoniakniveauer, sædvanligvis indenfor 24 timer.
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.
Danish: Hos patienter med organisk acidæmi (nyfødte og ikke-nyfødte) inducerede behandling med cargluminsyre en hurtig reduktion af plasmaniveauet af ammoniak, hvilket reducerer risikoen for neurologiske komplikationer.
English: WHAT CARBAGLU IS AND WHAT IT IS USED FOR
Danish: CARBAGLUS VIRKNING OG HVAD DE SKAL BRUGE DET TIL
English: 2.2 Qualitative and quantitative composition For a full list of excipients, see section 6.1
Danish: 2.2 Kvalitativ og kvantitativ sammensætning Alle hjælpestoffer er anført under pkt.
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.
Danish: Cargluminsyre udskilles i mælken hos ammende rotter, og selvom udviklingsparametre var upåvirkede, var der somme tider en effekt på kropsvægt/forøgelse af kropsvægt hos unger, der diede ved mødre behandlet med 500 mg/kg/dag og en højere mortalitet hos unger af mødre behandlet med 2000 mg/kg/dag, en modertoksisk dosis.
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.
Danish: På trods af en lavere affinitet for carbamoylfosfatasesyntetase overfor cargluminsyre end overfor N-acetylglutamat, har cargluminsyre in vivo vist sig at stimulere carbamoylfosfatasesyntetase og at være langt mere effektiv end N-acetylglutamat til at beskytte mod ammoniakforgiftning hos rotter.
English: General disorders and Administration site conditions
Danish: Almene symptomer og reaktioner på administrationsstedet
English: If you take more Carbaglu than you should Ask your doctor or pharmacist for advice.
Danish: Hvis De har taget for meget Carbaglu Spørg lægen eller apoteket til råds.
English: - Undesirable effects in organic acidaemia
Danish: - Bivirkninger ved organisk acidæmi
English: • Uncommon: bradycardia (decreased frequency of the heart), diarrhoea, fever, increased transaminases, vomiting
Danish: • Ikke almindelig: langsom hjerteaktion (puls), diarré, feber, forhøjede aminotransferaser,
English: Medicinal product subject to restricted medical prescription (See Annex I: Summary of Product Characteristics, section 4.2)
Danish: Lægemidlet må kun udleveres efter begrænset recept (jf. bilag I: Produktresumé, pkt.
English: A proportion of carglumic acid is metabolised.
Danish: En del af cargluminsyre metaboliseres.
English: After first opening of the tablet container: do not refrigerate, do not store above 30C.
Danish: Efter første åbning af tabletbeholderen: Må ikke nedkøles, må ikke opbevares ved temperaturer over 30 C.
English: Your doctor will evaluate your individual responsiveness to carglumic acid before initiating any long term treatment.
Danish: Deres læge vil vurdere, hvordan De reagerer på behandling med cargluminsyre, inden længerevarende behandling indledes.
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).
Danish: Selvom det ikke vides, om cargluminsyre udskilles i human brystmælk, er stoffet blevet påvist i mælken fra ammende rotter (se pkt.
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.
Danish: - Hos en patient med moderat hyperammonæmi gives en testdosis på 100 til 200 mg/kg/dag i 3 dage med en konstant proteinindtagelse og udførelse af gentagne bestemmelser af ammoniak plasmakoncentration (før og 1 time efter et måltid).
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.
Danish: Carbaglu 200 mg tablet er en aflang tablet med 4 huller på den ene side og med 3 brudriller.
English: Driving and using machines Effects on the ability to drive and use machines are not known.
Danish: Trafik- og arbejdssikkerhed Virkninger på evnen til at køre bil og betjene maskiner er ukendt.
English: Do not use after the expiry date stated on the tablet container.
Danish: Brug ikke Carbaglu efter den udløbsdato, der står på tabletbeholderen.
English: Please tell your doctor or pharmacist if you are taking or have recently taken any other medicines, including medicines obtained without a prescription.
Danish: Fortæl det altid til lægen eller apoteket, hvis De/du bruger anden medicin eller har brugt det for nylig.
English: 5-, 15- or 60- high density polyethylene tablet containers closed by a child resistant polypropylene cap with a desiccant unit.
Danish: 5 stk., 15 stk. eller 60 stk. tabletbeholdere af højdensitetspolyethylen lukket med et børnesikkert låg af polypropylen med en tørrende enhed.
English: Carglumic acid has been shown in vitro to activate liver carbamoyl phosphate synthetase.
Danish: Cargluminsyre har vist sig in vitro at aktivere leverens carbamoylfosfatsyntetase.
English: Uncommon: increased transaminases
Danish: Ikke almindelig: forhøjede aminotransferaser
English: Microcrystalline cellulose sodium laurilsulfate hypromellose croscarmellose sodium silica colloidal anhydrous sodium stearyl fumarate
Danish: Mikrokrystallinsk cellulose natriumlaurilsulfat hypromellose croscarmelosenatrium silica, kolloid vandfri natriumstearylfumarat
English: It should then be individually adjusted in order to maintain normal ammonia plasma levels (see section 4.4).
Danish: Den bør derefter justeres individuelt med henblik på at opretholde normale ammoniak plasmaniveauer (se afsnit 4.4).
English: For carglumic acid no clinical data on exposed pregnancies are available.
Danish: Der foreligger ikke kliniske data om eksponering for cargluminsyre under graviditet.
English: 2.1 General description Each tablet contains 200 mg of carglumic acid.
Danish: 2.1 Generel beskrivelse Hver tablet indeholder 200 mg cargluminsyre.
English: NAME AND ADDRESS OF THE MARKETING AUTHORISATION HOLDER
Danish: NAVN OG ADRESSE PÅ INDEHAVER EN AF MARKEDSFØRINGSTILLADELSEN
English: Detailed information on this medicine is available on the European Medicines Agency web site: http://www.ema.europa.eu.
Danish: De kan finde yderligere information om Carbaglu på Det Europæiske Lægemiddelagenturs hjemmeside http://www.ema.europa.eu/.
English: Take special care with Carbaglu:
Danish: Vær ekstra forsigtig med at tage Carbaglu:
English: Carglumic acid responsiveness test It is recommended to test individual responsiveness to carglumic acid before initiating any long term treatment.
Danish: Det anbefales at teste individuelt respons på cargluminsyre, inden der indledes længevarende behandling.
English: PHARMACEUTICAL FORM AND CONTENTS
Danish: LÆGEMIDDELFORM OG INDHOLDSMÆNGDE
English: The excretion of carglumic acid into breast milk has not been studied in women.
Danish: Det er ikke undersøgt , om cargluminsyre udskilles i brystmælk hos kvinder.
English: Distribution
Danish: Fordeling:
English: 60 dispersible tablets
Danish: 60 dispergibel tabletter
English: Therefore, breast-feeding during the use of carglumic acid is contraindicated (see section 4.3).
Danish: Derfor er det kontraindiceret at amme under brug af cargluminsyre (se pkt.
English: • Not known: rash
Danish: • Ikke kendt: udslæt
English: • the lack of a specific liver enzyme N- acetylglutamate synthase.
Danish: • mangel på et specifikt leverenzym, N-acetylglutamatsyntase.
English: - Undesirable effects in N-acetylglutamate synthase deficiency
Danish: - Bivirkninger ved N-acetylglutamatsyntasemangel
English: Pregnancy and Breast-feeding
Danish: Graviditet og amning
English: HOW TO STORE CARBAGLU
Danish: SÅDAN OPBEVARER DE CARBAGLU
English: • isovaleric acidaemia, methylmalonic acidaemia or propionic acidaemia.
Danish: • isovaleriansyreæmi, methylmalonsyreæmi eller propionacidæmi.
English: Each tablet contains 200 mg of carglumic acid.
Danish: Hver tablet indeholder 200 mg cargluminsyre.
English: • hyperammonaemia due to N-acetylglutamate synthase primary deficiency.
Danish: • Hyperammonæmi på grund af primær N-acetylglutamatsyntasemangel.