FrancophonIA/ELRC3.0 huggingface dataset:
English: Cerliponase alfa is a recombinant human protein and systemic exposure is limited due to intracerebroventricular administration, therefore interactions between cerliponase alfa and medicinal products metabolised by cytochrome P450 enzymes are unlikely to occur.
Croatian: Cerliponaza alfa je rekombinantni ljudski protein i sistemska je izloženost ograničena zbog intracerebroventrikularne primjene, stoga nije vjerojatno da će doći do interakcije cerliponaze alfa i lijekova koji se metaboliziraju putem enzima citokroma P450.
English: Overall, 23 (96%) subjects who received cerliponase alfa experienced an event that mapped to the Convulsions Standardized MedDRA Query.
Croatian: 23 (96%) ispitanika koja su primila cerliponazu alfa imalo je događaj koji je prema MedDRA-inim standardnim pojmovima svrstan u konvulzije.
English: After the medicine has been given, a shorter infusion of a solution is given to flush Brineura out of the infusion equipment so that the full dose reaches the brain.
Croatian: Kada je lijek primijenjen, daje se kraća infuzija otopine kojom se Brineura ispire iz opreme za infuziju, tako da cjelokupna doza dospije do mozga.
English: Brineura has not been given to patients with advanced disease at the start of treatment or in children younger than 2 years of age.
Croatian: Lijek Brineura nije primjenjivan bolesnicima s uznapredovalom bolešću na početku liječenja ili u djece mlađe od 2 godine.
English: No association was found between serum or CSF ADA titres and incidence or severity of hypersensitivity.
Croatian: Nije nađena povezanost između serumskog ili CSF ADA titra i incidencije ili težine preosjetljivosti.
English: Brineura has not been given to patients younger than 2 years of age or older than 8 years of age (at the start of the clinical trial).
Croatian: Lijek Brineura nije primjenjivan bolesnicima mlađim od 2 godine ili starijim od 8 godina (na početku kliničkog ispitivanja).
English: Table 3: CLN2 Clinical Rating Scale
Croatian: Tablica 3: Klinička ocjenska ljestvica za CLN2
English: Vision and seizure scores when combined with CLN2 score (motor and language domains) remain stable.
Croatian: Rezultati za vid i napadaje kada su kombinirani s rezultatom za CLN2 (motorička i jezična domena)
English: In cardiac normal patients, regular 12-lead ECG evaluations should be performed every 6 months.
Croatian: U bolesnika s normalnom funkcijom srca, redovite procjene 12-kanalnim EKG-om treba provoditi svakih 6 mjeseci.
English: birth to < 6 months: 100 mg
Croatian: od rođenja do < 6 mjeseci: 100 mg
English: Severe (Common Terminology Criteria for Adverse Events (CTCAE) grade 3) hypersensitivity reactions occurred in three patients and no patients discontinued treatment.
Croatian: Common Terminology Criteria for Adverse Events (CTCAE) 3. stupnja] javile su se u tri bolesnika, ali ni jedan bolesnik nije prekinuo liječenje.
English: The complete infusion time, including Brineura and the required flushing solution, is approximately 2 to 4.5 hours, depending on the dose and volume administered.
Croatian: Cjelokupna infuzija, uključujući infuziju lijeka Brineura i potrebne otopine za ispiranje, traje otprilike 2 do 4,5 sati ovisno o dozi i volumenu za primjenu.
English: The intracerebroventricular access device must be implanted prior to the first infusion.
Croatian: Pomagalo za intracerebroventrikularni pristup mora biti implantirano prije prve infuzije.
English: • Extension Set: Fresenius Injectomat® line, Alaris CC Extension set, Vygon Lectro-Cath Extension tube
Croatian: • nastavci infuzijske linije: linija Fresenius Injectomat, produžni komplet Alaris CC, produžna cjevčica Vygon Lectro-Cath
English: In order to evaluate the long-term safety of cerliponase alfa, including the occurrence of serious hypersensitivity reactions and anaphylaxis, the MAH should submit the results of a study based on adequate source of data deriving from a registry of patients with neuronal ceroid lipfuscinosis Type 2 (CLN2).
Croatian: Kako bi se procijenila dugoročna sigurnost primjene cerliponaze alfa, uključujući pojavu ozbiljnih reakcija preosjetljivosti i anafilaksije, nositelj odobrenja mora dostaviti rezultate ispitivanja temeljenog na odgovarajućem izvoru podataka iz registra bolesnika s neuronalnom ceroidnom lipofuscinozom tip 2 (CLN2).
English: There is limited data in patients older than 8 years of age.
Croatian: Za bolesnike starije od 8 godina podaci su ograničeni.
English: • One empty sterile single-use syringe (for collection of CSF to check patency).
Croatian: • Jedna prazna sterilna štrcaljka za jednokratnu uporabu (za uzimanje CSF-a kako bi se provjerila prohodnost sustava).
English: Table 5: Pharmacokinetic properties following first Intracerebroventricular infusion (approximately 4 hours in duration) of 300 mg cerliponase alfa in CSF
Croatian: Tablica 5: Farmakokinetička svojstva nakon prve intracerebroventrikularne infuzije (približnog trajanja od 4 sata) 300 mg cerliponaze alfa u CSF
English: These particles are removed via the 0.2 μm inline filter without having a detectable effect on the purity or strength of Brineura.
Croatian: Te se čestice uklanjaju ugrađenim filtrom veličine pora 0,2 μm, a što nema opažen učinak na čistoću ili jačinu lijeka Brineura.
English: In order to further evaluate the treatment effectiveness as a delay in progression of CLN2 motor-language clinical sale and to further evaluate the safety and tolerability of cerliponase alfa, the MAH will submit the results of study 190-203 including at least 5 patients below the age of 2 years.
Croatian: Kako bi se bolje procijenila djelotvornost liječenja kao odgoda napredovanja bolesti CLN2 na kliničkoj ljestvici motoričkih i jezičnih funkcija te bolje procijenila sigurnost i podnošljivost cerliponaze alfa, nositelj odobrenja će dostaviti rezultate ispitivanja 190-203 uključujući najmanje 5 bolesnika mlađih od 2 godine.
English: Adverse reactions are presented in order of decreasing seriousness.
Croatian: Nuspojave su prikazane redoslijedom prema manjoj ozbiljnosti.
English: Birth to < 6 months 6 months to < 1 year 1 year to < 2 years
Croatian: 6 mjeseci do < 1 godine 1 godina do < 2 godine
English: Talk to your doctor before you or your child is given Brineura.
Croatian: Obratite se svom liječniku prije nego što Vi ili Vaše dijete primite lijek.
English: • Extension Set with 0.2 micron filter: Impromediform GmbH
Croatian: • komplet za produžetak infuzijske linije s filtrom veličine pora 0,2 mikrometra: Impromediform
English: - The active substance is cerliponase alfa.
Croatian: - Djelatna tvar je cerliponaza alfa.
English: Product should only be withdrawn from the unopened vials immediately prior to use.
Croatian: Lijek se smije izvući samo iz neotvorenih bočica neposredno prije uporabe.
English: Common side effects (may affect up to 1 in 10 people): device-related bacterial infections slower heart beat device does not function correctly due to a blockage detected during preparation for infusion
Croatian: Česte nuspojave (mogu se javiti u do 1 na 10 osoba): bakterijske infekcije povezane s pomagalom usporeni srčani otkucaji pomagalo ne funkcionira ispravno zbog začepljenosti zapažene tijekom pripreme infuzije
English: MedDRA System organ class
Croatian: MedDRA klasifikacija organskih sustava
English: Description of selected adverse reactions
Croatian: Opis odabranih nuspojava Konvulzije
English: Cerliponase alfa is a protein and is expected to be metabolically degraded through peptide hydrolysis.
Croatian: Cerliponaza alfa je protein i očekuje se da će zbog hidrolize peptida doći do njenog metaboličkog raspada.
English: Dispose of the infusion components, needles, unused solutions and other waste materials in accordance with local requirements.
Croatian: 7. Infuzijske komponente, igle, neupotrijebljene otopine i drugi otpadni materijal zbrinite sukladno nacionalnim propisima.
English: Drug-specific neutralizing antibodies (NAb) capable of inhibiting receptor-mediated cellular uptake of cerliponase alfa were not detected in the CSF.
Croatian: Neutralizirajuća protutijela specifična za lijek (engl. neutralizing antibodies, NAb) sposobna inhibirati receptorom posredovano stanično preuzimanje cerliponaze alfa nisu bila ustanovljena u CSF-u.
English: The adverse reactions described in this section were evaluated in 24 patients with CLN2 disease who received at least one dose of Brineura in clinical studies of up to 141 weeks.
Croatian: Nuspojave opisane u ovom dijelu procijenjene su u 24 bolesnika s bolešću CLN2 koji su u kliničkim ispitivanjima primili najmanje jednu dozu lijeka Brineura do 141 tjedna.
English: Transport and distribute frozen (-85°C to -15°C).
Croatian: Prevoziti i dostavljati zamrznuto (od -85 °C do -15 °C).
English: All 23 patients completed study 190-201 and continued to the ongoing extension study 190-202 treated with Brineura at 300 mg every other week to a maximum of 124 weeks.
Croatian: Svih 23 bolesnika završilo je ispitivanje 190-201 i nastavilo u produžetku ispitivanja 190-202 koje još traje, a liječeni su lijekom Brineura u dozi od 300 mg svaki drugi tjedan do najviše 124 tjedna.
English: The signs and symptoms of device-related infections may not be apparent, therefore, CSF samples should routinely be sent for testing to detect subclinical device infections.
Croatian: Znakovi i simptomi infekcija povezanih s pomagalom ne moraju biti očigledni, stoga uzorke CSF-a treba svaki put poslati na testiranje kako bi se otkrile supkliničke infekcije povezane s pomagalom.
English: Your doctor may send samples of brain fluid to check for signs of infection.
Croatian: Liječnik može poslati uzorke tekućine iz mozga (cerebrospinalni likvor) na provjeru, kako bi se ustanovilo postoje li znakovi infekcije.
English: Material degradation of the intracerebroventricular access device reservoir occurs after long periods of use according to preliminary results of benchtop testing and as observed in clinical trials with approximately 4 years of use.
Croatian: Degradacija materijala spremnika pomagala za intracerebroventrikularni pristup javlja se nakon dugotrajnog razdoblja primjene prema preliminarnim rezultatima laboratorijskog testiranja i prema opažanjima u kliničkim ispitivanjima prilikom primjene tijekom otprilike 4 godine.
English: Remove the green flip-off caps from both Brineura vials.
Croatian: Uklonite zelenu kapicu s obje bočice lijeka Brineura.
English: Label the infusion line for “Intracerebroventricular infusion only”.
Croatian: 1. Obilježite infuzijsku liniju oznakom „samo za intracerebroventrikularnu infuziju“.
English: Brineura should be administered with infusion components shown to be chemically and physically compatible with administration of Brineura and flushing solution.
Croatian: Brineura se mora primjenjivati s infuzijskim komponentama za koje se pokazalo da su kemijski i fizikalno kompatibilne s primjenom lijeka Brineura i otopine za ispiranje.
English: Prior to the launch of BRINEURA in each Member State (MS), the Marketing Authorisation Holder (MAH) must agree about the content and format of the educational materials, including communication media, distribution modalities, and any other aspects of the programme, with the National Competent Authority (NCA).
Croatian: Prije stavljanja u promet lijeka BRINEURA u državama članicama, nositelj odobrenja za stavljanje lijeka u promet mora se s nadležnim tijelom pojedine države članice usuglasiti o sadržaju i formatu edukacijskih materijala, uključujući oblik komunikacije, načine distribucije i sve druge aspekte programa.
English: Intracerebroventricular administration of cerliponase alfa is not expected to result in therapeutic concentrations in the eye due to the limited access from the CSF to the affected cells of the retina and the presence of the blood-retinal barrier.
Croatian: Ne očekuje se da će intracerebroventrikularna primjena cerliponaze alfa rezultirati terapijskim koncentracijama u oku zbog ograničenog pristupa iz CSF-a u zahvaćene stanice mrežnice i prisutnosti barijere između krvi i mrežnice.
English: Consultation with a neurosurgeon may be needed to confirm the integrity of the device.
Croatian: Može biti potrebno posavjetovati se s neurokirurgom kako bi se potvrdila ispravnost pomagala.
English: Chemical and physical in-use stability has been demonstrated for up to 12 hours at room temperature (19-25°C).
Croatian: Kemijska i fizikalna stabilnost lijeka u primjeni dokazana je u trajanju do 12 sati na sobnoj temperaturi (19-25 °C).
English: The safety and efficacy of Brineura were assessed in an open label, dose escalation clinical study (190-201) and an ongoing long term extension study (190-202) in patients with CLN2 disease compared to untreated patients with CLN2 disease from a natural history database (natural history control group).
Croatian: Sigurnost i djelotvornost lijeka Brineura procijenjene su u otvorenom kliničkom ispitivanju s postupnim povišenjem doze (ispitivanje 190-201) i dugoročnom produžetku ispitivanja koje još traje (ispitivanje 190-202) u bolesnika s bolešću CLN2 u usporedbi s neliječenim bolesnicima s bolešću CLN2 iz baze podataka o prirodnom tijeku bolesti (kontrolna skupina s prirodnim tijekom bolesti).
English: The mean baseline CLN2 score was 3.5 (standard deviation (SD) 1.20) with a range from 1 to 6; no patients with advanced disease progression were studied (inclusion criteria: mild to moderate progression of CLN2 disease).
Croatian: CLN2 bio je 3,5 [standardno odstupanje (SD) 1,20] s rasponom od 1 do 6; nije ispitivan ni jedan bolesnik s napredovanjem već uznapredovale bolesti (kriterij uključivanja: blago do umjereno napredovanje bolesti CLN2).
English: In study 190-201, 20 out of 23 (87%) patients receiving Brineura for 48 weeks did not have an unreversed 2 point decline in the untreated patient population (p=0.0002, binomial test assuming p0= 0.50).
Croatian: U ispitivanju 190-201, 20 od 23 (87%) bolesnika koji su primali lijek Brineura 48 tjedana, nije imalo nepovratno propadanje funkcija za 2 boda predviđeno za populaciju neliječenih bolesnika (p = 0,0002, pretpostavka binomijalnog testa p0 = 0,50).
English: Patients with advanced CLN2 disease and newborns may have decreased integrity of the blood-brain barrier.
Croatian: U bolesnika s uznapredovalom bolešću CLN2 te u novorođenčadi, moguće je da je propusnost krvno-moždane barijere bila smanjena.
English: Do not re-freeze vials or freeze syringes containing Brineura or flushing solution.
Croatian: Nemojte ponovno zamrzavati bočice ili štrcaljke koje sadrže lijek Brineura ili otopinu za ispiranje.
English: This medicine replaces the missing enzyme, TPP1, which minimises the build-up of the lysosomal storage materials.
Croatian: Ovaj lijek nadomješta enzim koji nedostaje, TPP1, što smanjuje taloženje lizosomskih materijala za nakupljanje.
English: Results from the natural history control group demonstrate CLN2 disease is a rapidly progressive neurodegenerative disease with predictable decline in motor and language function with an estimated mean rate of decline in the CLN2 score of 2 points per 48 weeks.
Croatian: Rezultati kontrolne skupine s prirodnim tijekom bolesti pokazuju da je CLN2 neurodegenerativna bolest koja brzo napreduje, s predvidljivim propadanjem motoričke i jezične funkcije i procijenjenom srednjom brzinom gubitka funkcija u rezultatu za CLN2 od 2 boda na 48 tjedana.
English: If immediate use is not possible, unopened vials of Brineura or flushing solution should be stored at 2-8°C and used within 24 hours.
Croatian: Ako ga nije moguće upotrijebiti odmah, neotvorene bočice lijeka Brineura ili otopine za ispiranje potrebno je čuvati na 2-8 °C i upotrijebiti unutar 24 sata.
English: Thus, the non-clinical development is inconclusive with respect to the clinical safety of cerliponase alfa.
Croatian: Stoga se na temelju kretanja nekliničkih podataka ne može zaključiti o kliničkoj sigurnosti cerliponaze alfa.
English: Your doctor may decide to continue treatment once the device infection or problems are resolved.
Croatian: Liječnik može odlučiti nastaviti liječenje kada infekcija ili problemi vezani uz pomagalo prođu.
English: Prepare the scalp for intracerebroventricular infusion using aseptic technique per institution standard of care.
Croatian: 5. Pripremite vlasište za intracerebroventrikularnu infuziju služeći se aseptičnom tehnikom prema standardima skrbi ustanove.
English: Intracerebroventricular access devices shown to be compatible with Brineura and flushing solution and used in Brineura clinical studies include CodmanHOLTER RICKHAM and HOLTER SALMON-RICKHAM Reservoirs, Codman Ventricular Catheter, and Medtronic CSF-Ventricular Reservoir (with catheter).
Croatian: Pomagala za intracerebroventrikularni pristup za koje se pokazalo da su kompatibilni s lijekom Brineura i otopinom za ispiranje i upotrijebljeni su u kliničkim ispitivanjima s lijekom Brineura, uključuju spremnike „Codman HOLTER RICKHAM” i „HOLTER SALMON-RICKHAM”, kateter „Codman Ventricular Catheter” i spremnik (s kateterom) „Medtronic CSF-Ventricular Reservoir”.
English: Withdraw 0.5 ml to 1 ml of CSF to check patency of the intracerebroventricular access device.
Croatian: Uvucite između 0,5 ml i 1 ml CSF-a kako biste provjerili prohodnost pomagalaza intracerebroventrikularni pristup.
English: The infusion should be interrupted and/or the rate slowed in patients who in the judgement of the treating physician have a possible increase in intracranial pressure during the infusion as suggested by symptoms such as headache, nausea, vomiting, or decreased mental state.
Croatian: Infuziju treba prekinuti i/ili brzinu infuzije usporiti u bolesnika u kojih je prema prosudbi nadležnog liječnika možda došlo do povećanja intrakranijalnog tlaka tijekom infuzije, na što upućuju simptomi kao što su glavobolja, mučnina, povraćanje ili stanje smanjene svijesti.
English: There are no data on the use of Brineura in pregnant women.
Croatian: Nema podataka o primjeni lijeka Brineura u trudnica.
English: Brineura should be given to a pregnant woman only if clearly needed.
Croatian: Brineura se smije primijeniti u trudnica samo ako je to izrazito potrebno.
English: Precautions to be taken before handling or administering the medicinal product Aseptic technique must be strictly observed during preparation and administration.
Croatian: Mjere opreza koje je potrebno poduzeti prije rukovanja ili primjene lijeka Tijekom pripreme i primjene lijeka moraju se strogo primjenjivati pravila aseptične tehnike.
English: The recommended dose is 300 mg cerliponase alfa administered once every other week by intracerebroventricular infusion.
Croatian: Preporučena doza je 300 mg cerliponaze alfa primijenjene intracerebroventrikularnom infuzijom jednom svaki drugi tjedan.
English: The safety and efficacy of Brineura in children less than 3 years of age has not yet been established.
Croatian: Pedijatrijska populacija Sigurnost i djelotvornost lijeka Brineura u djece mlađe od 3 godine nisu još ustanovljene.
English: Life-threatening anaphylactic reaction to the active substance or to any of the excipients listed in section 6.1, if re-challenge is unsuccessful (see section 4.4).
Croatian: Životno opasne anafilaktične reakcije na djelatnu tvar ili neku od pomoćnih tvari navedenih u dijelu 6.1, ako ponovno izlaganje lijeku (re-challenge) nije uspješno (vidjeti dio 4.4).
English: People with CLN2 disease do not have any enzyme called TPP1 or they have too little of it and this causes a build-up of substances called lysosomal storage materials.
Croatian: Osobe s bolešću CLN2 nemaju enzim pod nazivom TPP1 ili ga imaju u premaloj količini, a to uzrokuje taloženje tvari poznatih kao lizosomski materijali za nakupljanje.
English: A total of 24 patients, aged 3 to 8 years, were treated with Brineura 300 mg every other week.
Croatian: Ukupno 24 bolesnika, u dobi od 3 do 8 godina, liječena su lijekom Brineura 300 mg svaki drugi tjedan.
English: The pump must be programmable to deliver the medicinal product at a constant rate of 2.5 ml/hr.
Croatian: Pumpa se mora moći programirati tako da isporučuje lijek konstantnom brzinom od 2,5 ml/h.
English: This being a marketing authorisation under exceptional circumstances and pursuant to Article 14(8) of Regulation (EC) No 726/2004, the MAH shall complete, within the stated timeframe, the following measures:
Croatian: Uredbe (EZ) br. 726/2004, nositelj odobrenja dužan je unutar navedenog vremenskog roka provesti sljedeće mjere:
English: Renal elimination of cerliponase alfa is considered a minor pathway for clearance.
Croatian: Izlučivanje cerliponaze alfa putem bubrega smatra se manje značajnim putem uklanjanja.
English: Convulsions resolved with standard anti-convulsive therapies, and did not result in discontinuation of Brineura treatment.
Croatian: Konvulzije su prestale uz primjenu standardne terapije protiv konvulzija i nisu za posljedicu imale prekid terapije lijekom Brineura.
English: However, the results of these studies in dachshund dogs cannot reliably predict human safety, because the regimen of cerliponase alfa infusions was different and highly variable even within the same study due to difficulties with the indwelling catheter system and prominent hypersensitivity reactions.
Croatian: Međutim, rezultati tih ispitivanja u pasmine jazavčar ne mogu pouzdano predviđati sigurnost u ljudi, jer je zbog teškoća s umetnutim sustavom katetera i izrazitih reakcija preosjetljivosti, režim infuzija cerliponaze alfa bio drugačiji i vrlo promjenjiv čak i unutar istog ispitivanja.
English: The infusion set must be equipped with a 0.2 µm inline filter.
Croatian: Komplet za infuziju mora biti opremljen ugrađenim filtrom s porama veličine 0,2 µm.
English: Cerliponase alfa plasma pharmacokinetics were assessed in 13 patients, and a median Tmax of 12.0 hours (since start of infusion), a mean Cmax of 1.39 µg/ml, and mean AUC0-t of 24.1 µg-hour/ml were characterized.
Croatian: Farmakokinetika cerliponaze alfa u plazmi bila je procijenjena u 13 bolesnika i ustanovljen je medijan Tmax od 12,0 sati (od početka infuzije), srednja vrijednost Cmax od 1,39 µg/ml i srednja vrijednost AUC0-t od 24,1 µg-h/ml.
English: • Port needle: Deltec GRIPPER Needles
Croatian: • ulazna igla: igle Deltec GRIPPER.
English: The flushing solution may contain particles that dissolve when the vial is fully thawed.
Croatian: Otopina za ispiranje može sadržavati čestice koje se otope kada je bočica potpuno odmrznuta.
English: In patients less than 3 years of age the recommended dose is in accordance with the posology used in the ongoing clinical study 190-203, see section 5.1.
Croatian: U bolesnika mlađih od 3 godine, preporučena se doza podudara s doziranjem primijenjenim u kliničkom ispitivanju 190-203 koje još traje, vidjeti dio 5.1.
English: Connect a separate empty sterile syringe (no larger than 3 ml) to the port needle.
Croatian: 7. Na ulaznu iglu spojite zasebnu sterilnu praznu štrcaljku (ne veću od 3 ml).
English: The pharmacokinetics of cerliponase alfa were evaluated in patients with CLN2 disease who received intracerebroventricular infusions of 300 mg over approximately 4.5 hours once every other week.
Croatian: Farmakokinetika cerliponaze alfa procijenjena je u bolesnika s bolešću CLN2 koji su primali intracerebroventrikularne infuzije od 300 mg tijekom približno 4,5 sati jednom svaki drugi tjedan.
English: Using aseptic technique, withdraw the volume of Brineura solution per required dose (see Table 1 in section 4.2) into the sterile syringe labelled “Brineura”.
Croatian: Pridržavajući se aseptične tehnike uvucite volumen lijeka Brineura potreban za dozu (vidjeti tablicu 1 u dijelu 4.2) u sterilnu štrcaljku označenu s „Brineura“.
English: In study 190-201, the mean rate of decline in patients treated with Brineura at 300 mg every other week was 0.40 points per 48 weeks.
Croatian: U ispitivanju 190-201, srednja brzina propadanje funkcija u bolesnika liječenih lijekom Brineura u dozi od 300 mg svaki drugi tjedan bila je 0,40 bodova po periodu od 48 tjedana.
English: See the syringe pump manufacturer's operating manual for details.
Croatian: Pojedinosti pogledajte u priručniku za rad proizvođača pumpe za štrcaljke.
English: Brineura has a green flip-off cap and flushing solution has a yellow flip-off cap.
Croatian: Brineura ima zelenu, a otopina za ispiranje žutu kapicu.