natgillin/translations-raw huggingface dataset:
It is indicated in all patients with neonatal-onset presentation (complete enzyme deficiencies, presenting within the first 28 days of life).
Indiciran je u svih bolesnika u kojih se znakovi bolesti pojavljuju u neonatalnoj dobi (potpuni nedostatak enzima, koji se pojavljuje unutar prvih 28 dana života).
The elimination half-life was estimated to be 0.8 hours.
Procjenjuje se da poluvijek eliminacije iznosi 0,8 sati.
Date of first authorisation: 08/12/1999 Date of latest renewal: 08/12/2009
Datum posljednje obnove: 08.12.2009.
What you need to know before you take AMMONAPS Do not take AMMONAPS
Što morate znati prije nego počnete uzimati AMMONAPS
If you take more AMMONAPS than you should
Ako uzmete više lijeka AMMONAPS nego što ste trebali
Cerebral synapse maturation was retarded and the number of functioning nerve terminals in the cerebrum was reduced, which resulted in impaired brain growth.
Usporeno je sazrijevanje sinapsi i smanjen je broj funkcionalnih živčanih završetaka u velikom mozgu, što je rezultiralo oštećenjima u razvoju mozga.
250 tablets 500 tablets
250 tableta 500 tableta
What AMMONAPS looks like and contents of the pack
Kako AMMONAPS izgleda i sadržaj pakiranja
AMMONAPS may be required life-long unless orthotopic liver transplantation is elected.
Liječenje lijekom AMMONAPS možda će biti potrebno tijekom cijelog života, osim ako se ne provede ortotopna transplantacija jetre.
532 g granules
532 g granula
If caloric supplementation is indicated, a protein-free product is recommended.
Ako je nadopuna kalorija indicirana, preporučuje se proizvod koji ne sadrži proteine.
The usual total daily dose of sodium phenylbutyrate in clinical experience is:
Uobičajena dnevna doza natrijeva fenilbutirata prema kliničkom iskustvu iznosi:
AMMONAPS does not completely prevent the occurrence of an acute excess of ammonia in the blood and is not appropriate for treating such a condition, which is a medical emergency.
AMMONAPS ne sprječava u potpunosti pojavu akutnog viška amonijaka u krvi i nije prikladan za liječenje takvih hitnih medicinskih stanja.
This may be due to the lipophilicity of sodium phenylbutyrate and consequent differences in volume of distribution.
Razlog može biti lipofilnost natrijeva fenilbutirata i posljedična razlika u volumenu distribucije.
HDPE bottles, with child resistant caps, containing 250 or 500 tablets.
HDPE bočice, sa sigurnosnim zatvaračem za djecu, koje sadrže 250 ili 500 tableta.
Please note that the reconstituted granules will normally produce a milky white suspension.
Suspendiranjem granula obično nastaje mliječno bijela suspenzija.
• 450 - 600 mg/kg/day in children weighing less than 20 kg
• 450 - 600 mg/kg/dan za djecu težine manje od 20 kg
Nitrogen waste, in the form of ammonia, is especially toxic for the brain and leads, in severe cases, to reduced levels of consciousness and to coma.
Suvišni dušik u obliku amonijaka osobito je toksičan za mozak i u teškim slučajevima izaziva smanjene razine svijesti i komu.
Mix the granules with water until there are no dry granules left (stirring the solution helps to dissolve the granules).
Granule miješajte s vodom dok se sve granule ne otope (miješanje otopine pomaže u otapanju granula).
AMMONAPS is not recommended for the management of acute hyperammonaemia, which is a medical emergency.
AMMONAPS se ne preporučuje za liječenje akutne hiperamonemije, koja zahtijeva hitno liječenje.
When the granules are dissolved in water you get a milky white liquid.
Nakon otapanja granula u vodi nastaje mliječno bijela tekućina.
- when given to small children, since they may not be able to swallow the tablets and may choke.
- ako ga dajete maloj djeci, budući da oni možda neće moći progutati tablete, što može dovesti do gušenja.
If this occurs, please discuss it with your doctor, because the absence of your period may be caused by pregnancy (see Pregnancy and breast-feeding section above).
U slučaju izostanka mjesečnice obratite se svom liječniku, jer uzrok može biti trudnoća (vidjeti dio o trudnoći i dojenju).
In normal volunteers gender differences were found in the pharmacokinetic parameters of phenylbutyrate and phenylacetate (AUC and Cmax about 30 - 50 % greater in females), but not phenylacetylglutamine.
U zdravih dobrovoljaca razlike u spolu utjecale su na farmakokinetičke parametre fenilbutirata i fenilacetata (AUC i Cmax oko 30 - 50% viši u žena), ali ne i na farmakokinetičke parametre fenilacetilglutamina.
AMMONAPS contains sodium
AMMONAPS sadrži natrij
Corticosteroids may cause the breakdown of body protein and thus increase plasma ammonia levels.
Kortikosteroidi mogu uzrokovati razgradnju proteina u organizmu i tako povisiti razine amonijaka u plazmi.
If you require laboratory tests, it is important to remind your doctor that you are taking AMMONAPS, since sodium phenylbutyrate may influence certain laboratory test results.
Ako morate obaviti laboratorijske pretrage, važno je da liječniku napomenete da uzimate AMMONAPS, budući da natrijev fenilbutirat može utjecati na neke rezultate laboratorijskih testova.
Pharmacotherapeutic group: various alimentary tract and metabolism products, ATC code: A16A X03.
Farmakoterapijska skupina: ostali lijekovi za probavni sustav i metabolizam, ATK oznaka: A16A X03.
Each bottle contains 266 g or 532 g of granules.
Jedna bočica sadrži 266 g ili 532 g granula.
AMMONAPS tablets should not be used in patients with dysphagia due to the potential risk of oesophageal ulceration if tablets are not promptly delivered to the stomach.
AMMONAPS ne smiju uzimati bolesnici s disfagijom zbog mogućeg rizika od ulceracije jednjaka ako tablete brzo ne dospiju u želudac.
Very common: Amenorrhoea, irregular menstruation
Vrlo često: amenoreja, neredovita menstruacija
The granules are off-white.
Granule su gotovo bijele.
How to store AMMONAPS Contents of the pack and other information
Kako čuvati AMMONAPS Sadržaj pakiranja i druge informacije
Prenatal exposure of rat pups to phenylacetate (the active metabolite of phenylbutyrate) produced lesions in cortical pyramidal cells; dendritic spines were longer and thinner than normal and reduced in number.
Prenatalno izlaganje mladunčadi štakora fenilacetatu (aktivnom metabolitu fenilbutirata) izazvalo je oštećenje piramidalnih stanica u korteksu; dendritičkih trnova bilo je manje i bili su dulji i tanji od normalnih.
AMMONAPS helps the body to eliminate nitrogen waste, reducing the amount of ammonia in your body.
AMMONAPS pomaže tijelu u uklanjanju suvišnog dušika i snizuje razinu amonijaka u tijelu.
It is especially important to tell your doctor if you are taking medicines containing: - valproic acid (an antiepileptic drug), - haloperidol (used in certain psychotic disorders), - corticosteroids (cortisone-like medicines that are used to provide relief for inflamed areas of the body), - probenecid (for treatment of hyperuricemia associated with gout).
Osobito je važno da obavijestite liječnika ako uzimate lijekove koji sadrže: - valproičnu kiselinu (antiepileptični lijek), - haloperidol (koji se primjenjuje kod određenih duševnih bolesti), - kortikosteroide (lijekovi poput kortizona, koji se koriste za ublažavanje upale određenih dijelova tijela), - probenecid (za liječenje hiperuricemije povezane s gihtom).
If persistent vomiting occurs, you should contact your doctor immediately.
Ako se javi dugotrajno povraćanje, odmah se obratite svom liječniku.
Patients with neonatal-onset disease had a high incidence of mental retardation.
Bolesnici u kojih se bolest pojavila u neonatalnoj dobi imali su visoku incidenciju mentalne retardacije.
The daily dose of AMMONAPS will be calculated from your protein tolerance, diet and body weight or body surface.
Dnevna doza lijeka AMMONAPS izračunava se prema Vašoj toleranciji na proteine, prehrani i tjelesnoj težini ili površini tijela.
266 g granules
266 g granula
• Shake the bottle lightly before opening
• Prije otvaranja lagano protresite bočicu
Increased blood alkaline phosphatase, transaminases, bilirubin, uric acid, chloride, phosphate and sodium.
Povišena razina alkalne fosfataze, transaminaza, bilirubina, mokraćne kiseline, klorida, fosfata i natrija.
She recovered following dose reduction except for recurrent pancreatitis episodes that eventually prompted treatment discontinuation.
Nakon sniženja doze bolesnica se oporavila, ali su epizode recidivirajućeg pankreatitisa naposljetku dovele do prekida liječenja.
Contains sodium, see package leaflet for further information.
Sadrži natrij, vidjeti Uputu o lijeku za dodatne informacije.
Do not use this medicine after the expiry date which is stated on the carton and the bottle label after “EXP”.
Ovaj lijek se ne smije upotrijebiti nakon isteka roka valjanosti navedenog na pakovanju i naljepnici na bočici iza oznake „Rok valjanosti“.
• Pass a flat surface, e.g. the back of a knife blade, over the top of the spoon to remove the excess of granules
• Preko vrha žlice pređite ravnom površinom, npr. oštricom noža, kako biste uklonili višak granula
How to take AMMONAPS
Kako uzimati AMMONAPS
Approximately 80 - 100 % of the medicinal product is excreted by the kidneys within 24 hours as the conjugated product, phenylacetylglutamine.
Oko 80 - 100% lijeka izlučuje se bubrezima unutar 24 sata u konjugiranom obliku, tj. kao fenilacetilglutamin.
If you are sexually active and your period stops altogether, do not assume that this is caused by AMMONAPS.
Ako ste spolno aktivni i Vaša mjesečnica u potpunosti izostane, to ne mora biti zbog lijeka AMMONAPS.
The daily dose should be individually adjusted according to the patient's protein tolerance and the daily dietary protein intake needed to promote growth and development.
Dnevnu dozu treba prilagoditi pojedinom bolesniku, obzirom na toleranciju proteina i dnevni unos proteina hranom, potreban za poticanje rasta i razvoja.
OUTER CARTON AND BOTTLE LABEL FOR TABLETS
VANJSKO KARTONSKO PAKOVANJE I NALJEPNICA NA BOČICI ZA TABLETE
The total daily dose of AMMONAPS should be divided into equal amounts and given with each meal (e.g. three times per day).
Ukupnu dnevnu dozu lijeka AMMONAPS treba podijeliti u jednake količine i uzimati uz svaki obrok (npr. tri puta dnevno).
In patients diagnosed during gestation and treated prior to any episode of hyperammonaemic encephalopathy, survival was 100 %, but even in these patients, many subsequently demonstrated cognitive impairment or other neurologic deficits.
Bolesnici kojima je bolest dijagnosticirana prije rođenja i koji su liječeni prije epizode hiperamonijemijske encefalopatije imali su stopu preživljavanja od 100%, ali i u tih su se bolesnika naknadno javila kognitivna oštećenja ili drugi neurološki deficiti.
If you are uncertain if your medicines contain these substances, you should check with your doctor or pharmacist.
Ako niste sigurni sadržavaju li lijekovi koje uzimate ove tvari, provjerite sa svojim liječnikom ili ljekarnikom.
You will need regular blood tests to determine the correct daily dose.
Radi određivanja ispravne dnevne doze morat ćete redovito obavljati krvne pretrage.
In children unable to swallow tablets, it is recommended to use AMMONAPS granules instead.
Djeci koja ne mogu progutati tablete preporučuje se AMMONAPS u granulama.
Reversal of pre-existing neurologic impairment is not likely to occur with treatment, and neurologic deterioration may continue in some patients.
Neurološka oštećenja unatoč liječenju nisu reverzibilna, a u nekih se bolesnika neurološko stanje može i pogoršati.
When high doses of phenylacetate (190 - 474 mg/kg) were given subcutaneously to rat pups, decreased proliferation and increased loss of neurons were observed, as well as a reduction in CNS myelin.
Nakon supkutane primjene visokih doza fenilacetata (190 - 474 mg/kg) u mladunčadi štakora uočena je smanjena proliferacija i povećani gubitak neurona te redukcija mijelina u SŽS.
In late-onset deficiency patients, including females heterozygous for ornithine transcarbamylase deficiency, who recovered from hyperammonaemic encephalopathy and were then treated chronically with dietary protein restriction and sodium phenylbutyrate, the survival rate was 98 %.
U bolesnika u kojih se bolest pojavila kasnije, uključujući žene heterozigotne za nedostatak ornitin transkarbamilaze, koji su se oporavili od hiperamonijemijske encefalopatije i koji su potom trajno liječeni prehranom sa smanjenim udjelom proteina i natrijevim fenilbutiratom, stopa preživljenja iznosila je 98%.
The use of AMMONAPS tablets is indicated for adults and children who are able to swallow tablets.
Primjena AMMONAPS tableta indicirana je u odraslih i djece koji mogu progutati tablete.
AMMONAPS tablets should not be given to children who are not able to swallow tablets.
AMMONAPS tablete ne smiju se davati djeci koja ih ne mogu progutati.
- if you have decreased kidney or liver function, since AMMONAPS is eliminated from the body through the kidney and liver.
- ako imate smanjenu funkciju bubrega ili jetre, budući da se AMMONAPS iz tijela izlučuje bubrezima i jetrom.
Do not use AMMONAPS if you are breast-feeding, because this medicine can pass into the breastmilk and harm your baby.
Ne uzimajte AMMONAPS ako dojite, jer lijek može dospjeti u majčino mlijeko i naškoditi Vašem djetetu.
When taken orally, the granules are to be mixed with solid foods (such as mashed potatoes or apple sauce) or liquid foods (such as water, apple juice, orange juice or protein-free infant formulas).
Kad se uzimaju oralno, granule treba pomiješati s krutom (pire ili jabučna kaša) ili tekućom hranom (voda, jabučni sok, sok od naranče ili bezproteinske formule za dojenčad).
- if you suffer from heart failure, a decrease in your kidney function or other diseases, where the retention of the sodium salt contained in this medicine, may make your condition worse.
- ako imate zatajenje srca, smanjenu funkciju bubrega ili druge bolesti koje se mogu pogoršati uslijed zadržavanja natrijeve soli koju ovaj lijek sadrži.
Micronucleus test results indicate that sodium phenylbutyrate was considered not to have produced any clastogenic effect in rats treated at toxic or non-toxic dose levels (examined 24 and 48 hours after a single oral administration of 878 to 2800 mg/kg).
Rezultati mikronukleus testa ukazuju da natrijev fenilbutirat nema klastogeni učinak u štakora liječenih toksičnim ili netoksičnim dozama (pregled 24 i 48 sati nakon jednokratne oralne primjene doze od 878 do 2800 mg/kg).
Since the metabolism and excretion of sodium phenylbutyrate involves the liver and kidneys, AMMONAPS should be used with caution in patients with hepatic or renal insufficiency.
Budući da se metabolizam i izlučivanje natrijeva fenilbutirata odvija jetrom i bubrezima, AMMONAPS treba koristiti uz oprez u bolesnika s insuficijencijom jetre ili bubrega.
In clinical trials with AMMONAPS, 56 % of the patients experienced at least one adverse event and 78 % of these adverse events were considered as not related to AMMONAPS.
U kliničkim ispitivanjima lijeka AMMONAPS 56% ispitanika imalo je barem jednu nuspojavu, dok se za 78% tih nuspojava smatra da nisu povezane s uzimanjem lijeka AMMONAPS.
Three dosing spoons are provided which dispense 1.2 g, 3.3 g or 9.7 g of sodium phenylbutyrate.
Priložene su tri mjerne žlice za količinu od 1,2 g, 3,3 g ili 9,7 g natrijeva fenilbutirata.
Manifestations of neurotoxicity were predominantly somnolence, fatigue and light-headedness.
Neurotoksičnost se uglavnom manifestirala u obliku pospanosti, umora i omaglice.
Therapeutic monitoring: Plasma levels of ammonia, arginine, essential amino acids (especially branched chain amino acids), carnitine and serum proteins should be maintained within normal limits.
Terapijski nadzor: Razine amonijaka, arginina, esencijalnih aminokiselina (osobito aminokiselina razgranatih lanaca) i karnitina u plazmi te proteina u serumu treba održavati unutar granica normale.
HDPE bottles, with child resistant caps, containing 266 g or 532 g of granules.
HDPE bočice, sa sigurnosnim zatvaračem za djecu, koje sadrže 266 g ili 532 g granula.
Where a patient requires administration by tube, it is possible to re-constitute AMMONAPS in water prior to use (solubility for sodium phenylbutyrate is up to 5 g in 10 ml water).
Ako bolesnik mora primati lijek putem sonde, AMMONAPS se prije primjene može suspendirati u vodi (topljivost natrijeva fenilbutirata iznosi do 5 g u 10 ml vode).
In patients with impaired hepatic function the conversion of phenylacetate to phenylacetylglutamine may be relatively slower.
U bolesnika s oštećenom funkcijom jetre pretvorba fenilacetata u fenilacetilglutamin može biti nešto sporija.
These symptoms are consistent with the accumulation of phenylacetate, which showed dose-limiting neurotoxicity when administered intravenously at doses up to 400 mg/kg/day.
Ovi simptomi odgovaraju simptomima nakupljanja fenilacetata koja pokazuje neurotoksičnost ovisnu o dozi pri intravenskoj primjeni u dozama do 400 mg/kg/dan.
The disposition of phenylbutyrate and its metabolites has also been studied in cancer patients following intravenous infusion of sodium phenylbutyrate (up to 2 g/m²) or phenylacetate.
Raspoloživost fenilbutirata i njegovih metabolita također je proučavana u bolesnika s karcinomom nakon intravenske infuzije natrijeva fenilbutirata (do 2 g/m²) ili fenilacetata.
Phenylacetate is also hydrolysed by esterases in liver and blood.
Fenilacetat se osim toga uz pomoć esteraza hidrolizira u jetri i krvi.
If you have difficulty swallowing it is recommended to use AMMONAPS granules instead.
Ako imate poteškoća s gutanjem, preporučuje se primjena AMMONAPS granula.
As a consequence, sodium phenylbutyrate reduces elevated plasma ammonia and glutamine levels in patients with urea cycle disorders.
Stoga, natrijev fenilbutirat snižava povišene razine amonijaka i glutamina u plazmi u bolesnika s poremećajem ciklusa ureje.
Even on therapy, acute hyperammonaemic encephalopathy may occur in a number of patients.
Čak i dok primaju terapiju, u nekih se bolesnika može pojaviti akutna hiperamonijemijska encefalopatija.
• The granules left in the spoon are one spoonful
• Granule koje ostanu u žlici odgovaraju mjeri jedne žlice
It has not been determined if phenylacetate is secreted in human milk and therefore the use of AMMONAPS is contra-indicated during lactation (see section 4.3).
Nije poznato izlučuje li se fenilacetat u majčino mlijeko te je stoga primjena lijeka AMMONAPS kontraindicirana tijekom dojenja (vidjeti dio 4.3).
Previously, neonatal-onset presentation of urea cycle disorders was almost universally fatal within the first year of life, even when treated with peritoneal dialysis and essential amino acids or their nitrogenfree analogues.
U prošlosti je poremećaj ciklusa ureje s nastupom simptoma u neonatalnoj dobi gotovo uvijek završavao smrću u prvoj godini života, čak i ako je novorođenče liječeno peritonejskom dijalizom i esencijalnim aminokiselinama ili njihovim analozima bez dušika.
Take the solution immediately after mixing.
Otopinu primijenite neposredno nakon pripreme.
The granules are packaged in plastic bottles with child-resistant caps.
Granule su pakirane u plastične bočice sa sigurnosnim zatvaračem za djecu.
- The active substance is sodium phenylbutyrate.
- Djelatna tvar je natrijev fenilbutirat.
When taken by mouth
Kroz usta.
Very common side effects: irregular menstrual periods and cessation of menstrual periods.
Vrlo česte nuspojave: neredovita mjesečnica i izostanak mjesečnice.
You should take AMMONAPS in equally divided doses by mouth, through a gastrostomy (tube that goes through the abdomen to the stomach) or through a nasogastric tube (tube that goes through the nose to the stomach).
Način primjene AMMONAPS uzimajte kroz usta u ravnomjerno raspoređenim dozama, putem gastrostome (sonda koja se kroz trbuh uvodi u želudac) ili putem nazogastrične sonde (sonda koja se kroz nos uvodi u želudac).
The significance of these data in pregnant women is not known; therefore the use of AMMONAPS is contra-indicated during pregnancy (see section 4.3).
Primjena lijeka AMMONAPS stoga je kontraindicirana tijekom trudnoće (vidjeti dio 4.3).
If you experience any of these symptoms, you should immediately contact your doctor or the nearest hospital emergency department for supportive treatment.
Ako primijetite neke od ovih simptoma, odmah o tome obavijestite svog liječnika ili se javite na prvu pomoć radi liječenja.
AMMONAPS granules should be administered orally (to infants and children unable to swallow tablets and to patients with dysphagia) or via gastrostomy or nasogastric tube.
AMMONAPS granule treba primjenjivati oralno (u novorođenčadi i djece koja ne mogu progutati tablete i u bolesnika s disfagijom) ili putem gastrostome ili nazogastrične sonde.
Based on studies of phenylacetylglutamine excretion in patients with urea cycle disorders it is possible to estimate that, for each gram of sodium phenylbutyrate administered, between 0.12 and 0.15 g of phenylacetylglutamine nitrogen are produced.
Na temelju ispitivanja o izlučivanju fenilacetilglutamina u bolesnika s poremećajem ciklusa ureje procjenjuje se da se po svakom gramu primijenjenog natrijeva fenilbutirata proizvodi između 0,12 i 0,15 g fenilacetilglutamin dušika.
The tablets are off-white, oval and embossed with “UCY 500”.
Tablete su gotovo bijele, ovalne, s otisnutom oznakom „UCY 500“.
Their cognitive performance remained relatively stable during phenylbutyrate therapy.
Njihove kognitivne sposobnosti tijekom liječenja fenilbutiratom bile su relativno stabilne.
In the majority of patients with urea cycle disorders or haemoglobinopathies receiving various doses of phenylbutyrate (300 - 650 mg/kg/day up to 20 g/day) no plasma level of phenylacetate could be detected after overnight fasting.
U većine bolesnika s poremećajem ciklusa ureje ili hemoglobinopatijom koji su primali različite doze fenilbutirata (300 - 650 mg/kg/dan do 20 g/dan) nakon noćnog posta nije otkrivena prisutnost fenilacetata u plazmi.
Three cirrhotic patients (out of 6) who received repeated oral administration of sodium phenylbutyrate (20 g/day in three doses) showed sustained plasma levels of phenylacetate on the third day that were five times higher than those achieved after the first dose.
U tri bolesnika s cirozom (od 6) koji su primili ponovljene oralne doze natrijeva fenilbutirata (20 g/dan u tri doze) trećeg su dana utvrđene razine fenilacetata u plazmi pet puta više od vrijednosti utvrđenih nakon prve doze.
The tablets are packaged in plastic bottles with child-resistant caps.
Tablete su pakirane u plastičnim bočicama sa sigurnosnim zatvaračem za djecu.
Studies with high intravenous doses of phenylacetate showed non linear pharmacokinetics characterised by saturable metabolism to phenylacetylglutamine.
Ispitivanjima s visokim intravenskim dozama fenilacetata utvrđena je nelinearna farmakokinetika obilježena saturabilnim metabolizmom fenilacetilglutamina.
Common: Renal tubular acidosis
Često: bubrežna tubularna acidoza
The total daily dose should be divided into equal amounts and given with each meal or feeding (e.g. 46 times per day in small children).
Ukupnu dnevnu dozu AMMONAPS granula treba podijeliti u jednake količine i uzimati uz svaki obrok ili hranjenje (npr. 4-6 puta dnevno u male djece).
Mix the measured dose with solid foods (such as mashed potatoes or apple sauce) or liquid foods (such as water, apple juice, orange juice or protein-free infant formulas) and take it immediately after mixing.
Izmjerenu dozu pomiješajte s krutom (pire ili jabučna kaša) ili tekućom hranom (voda, jabučni sok, sok od naranče ili bezproteinske formule za dojenčad) i odmah primijenite.