natgillin/translations-raw huggingface dataset:
Discard the empty syringe in accordance with local requirements.
Praznu štrcaljku bacite sukladno nacionalnim propisima.
Anaphylaxis has not been reported with Brineura use during clinical trials; however, the risk of anaphylaxis cannot be excluded.
Tijekom kliničkih ispitivanja lijeka Brineura nije zabilježena anafilaksija; međutim, rizik od anafilaksije ne može se isključiti.
Open vials or product in syringes should be used immediately.
Otvorene bočice ili lijek u štrcaljkama treba upotrijebiti odmah.
- The other ingredients of Brineura and the flushing solution are: sodium phosphate dibasic heptahydrate, sodium dihydrogen phosphate monohydrate, sodium chloride, potassium chloride, magnesium chloride hexahydrate, calcium chloride dihydrate, and water for injections (see section 2 “Brineura contains sodium”)
- Drugi sastojci lijeka Brineura i otopine za ispiranje: natrijev hidrogenfosfat heptahidrat, natrijev dihidrogenfosfat hidrat, natrijev klorid, kalijev klorid, magnezijev klorid heksahidrat, kalcijev klorid dihidrat i voda za injekcije (vidjeti dio 2 „Brineura sadrži natrij“).
Common signs of device leakage and device failure include swelling, erythema of the scalp, extravasation of fluid, or bulging of the scalp around or above the intracerebroventricular access device.
Uobičajeni znakovi propuštanja ili kvara pomagala uključuju oticanje, eritem vlasišta, ekstravazacija tekućine ili ispupčenja na vlasištu oko ili iznad pomagala za intracerebroventrikularni pristup.
Treatment of Brineura was initiated in children 2 to 8 years of age in clinical studies.
U kliničkim ispitivanjima, liječenje lijekom Brineura započeto je u djece u dobi od 2 do 8 godina.
Age groups
Dobne skupine
In case of intracerebroventricular access device-related complications, refer to the manufacturer's labelling for further instruction.
U slučaju komplikacija povezanih s pomagalom za intracerebroventrikularni pristup, daljnje informacije potražite u proizvođačevim uputama za uporabu pomagala.
Store upright in a freezer (-25°C to -15°C).
Čuvati u uspravnom položaju u zamrzivaču (od -25 °C do -15 °C).
Periodically inspect the infusion system during the infusion for signs of leakage or delivery failure.
Redovito provjeravajte sustav tijekom infuzije zbog znakova propuštanja ili greške u protoku infuzije.
Device issue:
Česte nuspojave:
Limited data are available for children aged 2 years and no clinical data is available in children below 2 years of age (see section 5.1).
Dostupni su ograničeni podaci za djecu u dobi od 2 godine, a za djecu mlađu od 2 godine nema kliničkih podataka
Attach the syringe containing Brineura to the extension line, if used, otherwise connect the syringe to the infusion set.
2. Štrcaljku koja sadrži lijek Brineura pričvrstite na produžni dio infuzijske linije, ako se koristi, u protivnom štrcaljku spojite na komplet za infuziju.
As a precautionary measure, appropriate medical support should be readily available when Brineura is administered.
Kao mjera opreza, kada se primjenjuje Brineura treba biti odmah dostupna odgovarajuća medicinska potpora.
CSF protein increased ECG abnormalities CSF protein decreased
Pretrage povišeni proteini u CSF-u abnormalnosti EKG-a sniženi proteini u CSF-u vrlo često vrlo često vrlo često
The implanted intracerebroventricular access device should be appropriate for accessing the cerebral ventricles for therapeutic administration.
Implantirano pomagalo za intracerebroventrikularni pristup treba omogućiti pristup do moždanih komora za primjenu terapije.
Pack size: 3 vials (two vials of Brineura and one vial of flushing solution), each containing 5 ml of solution
Veličina pakiranja: 3 bočice (dvije bočice lijeka Brineura i jedna bočica otopine za ispiranje), svaka sadrži 5 ml otopine.
• One single-use infusion set.
• Jedan komplet za infuziju za jednokratnu uporabu.
Tabulated list of adverse reactions Adverse reactions observed are listed below, by system organ class and frequency, following the MedDRA
Tablični popis nuspojava Opažene nuspojave navedene su u nastavku prema klasifikaciji organskih sustava i u skladu s
Each vial contains 44 mg of sodium.
Jedna bočica sadrži 44 mg natrija.
Brineura must only be administered by a trained healthcare professional knowledgeable in intracerebroventricular administration in a healthcare setting.
Lijek Brineura smiju primjenjivati samo osposobljeni zdravstveni radnici educirani za intracerebroventrikularnu primjenu u kliničkim/bolničkim uvjetima.
Cerliponase alfa is taken up by target cells and translocated to the lysosomes through the Cation Independent Mannose-6-Phosphate Receptor (CI-MPR, also known as M6P/IGF2 receptor).
Cation Independent Mannose-6-Phosphate Receptor, CI-MPR, poznatog i kao receptor M6P/IGF2).
Intelligible and grossly age-appropriate.
Razumljiv i potpuno primjeren dobi.
Vial (type I glass) with a stopper (butyl rubber), a flip-off cap (polypropylene) and crimp seal (aluminium).
Bočica (staklo tip I) s čepom (butilna guma), kapicom (polipropilen) i aluminijskim prstenom.
Intracerebroventricular use.
Intracerebroventrikularna primjena.
Your doctor or pharmacist is responsible for storing Brineura.
Za čuvanje lijeka Brineura odgovoran je Vaš liječnik ili ljekarnik.
Skin and subcutaneous tissue disorders
Poremećaji kože i potkožnog tkiva osip urtikarija često često
Post-authorisation efficacy study (PAES): Study 190-203.
(PAES): ispitivanje 190-203.
Do not shake vials.
Ne tresite bočice.
Attach the infusion set to the port needle (see Figure 1).
8. Pričvrstite komplet za infuziju na ulaznu iglu (vidjeti sliku 1).
It is not known if Brineura impacts human fertility.
Nije poznato utječe li Brineura na plodnost.
How Brineura works
Kako Brineura djeluje
Treatment effect in patients receiving Brineura was assessed using the CLN2 clinical rating scale, and results were compared to the 2 points per 48 weeks predicted decline in the natural history control group.
Učinak liječenja u bolesnika koji su primali lijek Brineura procijenjen je kliničkom ljestvicom za ocjenu bolesti CLN2, a rezultati su uspoređeni s predviđenim gubitkom funkcija u kontrolnoj skupini s prirodnim tijekom bolesti tj. smanjenjem za 2 boda po periodu od 48 tjedana.
Brineura vials may occasionally contain thin translucent fibres or opaque particles.
Bočice s lijekom Brineura mogu katkad sadržavati tanka prozirna vlakna ili neprozirne čestice.
Domain Score
Domena Rezultat
Thaw Brineura vials and flushing solution vial at room temperature for approximately 60 minutes.
Odmrznite lijek Brineura i otopinu za ispiranje Bočice lijeka Brineura i otopine za ispiranje odmrzavajte na sobnoj temperature otprilike 60 minuta.
CSF samples should routinely be sent for testing to detect subclinical device infections (see section 4.2).
Uzorke CSF-a treba svaki put poslati na testiranje kako bi se otkrile supkliničke infekcije povezane s pomagalom (vidjeti dio 4.2).
There is limited experience in a few patients aged 2 years old.
Ograničeno je iskustvo s primjenom u malobrojnih bolesnika u dobi od
Healthcare professionals should inspect the scalp for skin integrity to ensure the intracerebroventricular access device is not compromised prior to each infusion.
Prije svake infuzije zdravstveni radnici trebaju pregledati vlasište zbog provjere postojanja oštećenja kože, kako bi se osiguralo da pomagalo za intracerebroventrikularni pristup nije ugroženo.
The solutions are clear to slightly opalescent, colourless to pale yellow; the Brineura solution may occasionally contain thin translucent fibres or opaque particles.
Otopine su bistre do lagano opalescentne i bezbojne do blijedožute; otopina lijeka Brineura može katkad sadržavati tanka prozirna vlakna ili neprozirne čestice.
No studies on the effect of Brineura on the ability to drive or use machines have been performed.
Nisu provedena ispitivanja o učinku lijeka Brineura na sposobnost upravljanja vozilima i rada sa strojevima.
Brineura and flushing solution should be clear to slightly opalescent and colourless to pale yellow.
Brineura i otopina za ispiranje moraju biti bistre do lagano opalescentne te bezbojne do blijedožute boje.
CSF samples should routinely be sent for testing to detect subclinical device infections.
Uzorke CSF-a treba svaki put poslati na testiranje kako bi se otkrile supkliničke infekcije povezane s pomagalom.
Brineura must not be administered as long as there are signs of acute intracerebroventricular access device leakage, device failure, or device-related infection (see section 4.2 and 4.4).
Brineura se ne smije primjenjivati sve dok ima znakova akutnog propuštanja pomagala za intracerebroventrikularni pristup, kvara pomagala ili infekcije povezane s pomagalom (vidjeti dio 4.2 i
Intracerebroventricular Infusion of Brineura
Intracerebroventrikularna infuzija lijeka Brineura
Vertical bars represent standard error of the mean Solid line: 190-201 and 190-202 clinical studies Dash line: 190-901 Natural history control group
Okomiti stupci prikazuju standardnu grešku prosjeka Puna crta: kliničko ispitivanje 190-201 i 190-202
Label one unused sterile syringe “flushing solution” and attach a syringe needle.
Obilježite jednu neupotrijebljenu sterilnu štrcaljku oznakom „otopina za ispiranje“ i na nju pričvrstite iglu za štrcaljku.
Determine the volume of flushing solution needed to ensure complete delivery of Brineura to the cerebral ventricles.
Odredite volumen otopine za ispiranje potreban kako bi se osiguralo da će cijela količina lijeka Brineura dospjeti u moždane komore.
Each vial of Brineura contains 150 mg cerliponase alfa in 5 ml of solution (30 mg/ml)
Jedna bočica lijeka Brineura sadrži 150 mg cerliponaze alfa u 5 ml otopine (30 mg/ml)
If you or your child gets any side effects, talk to your doctor.
Ako Vi ili Vaše dijete primijetite bilo koju nuspojavu, potrebno je obavijestiti liječnika.
Currently no clinical experience of Brineura in children below 2 years of age is available.
Trenutačno nema kliničkog iskustva s lijekom Brineura u djece mlađe od 2 godine.
How to store Brineura
Kako primjenjivati lijek Brineura
Place the syringe containing Brineura into the syringe pump and program the pump to deliver at an infusion rate of 2.5 ml per hour.
Stavite štrcaljku s lijekom Brineura u pumpu za štrcaljku i programirajte pumpu na brzinu infuzije od 2,5 ml na sat.
- If you or your child gets any side effects, talk to your doctor.
- Ako Vi ili Vaše dijete primijetite bilo koju nuspojavu, potrebno je obavijestiti liječnika.
Insert the port needle into the intracerebroventricular access device.
6. Umetnite ulaznu iglu (engl. port needle) u pomagalo za intracerebroventrikularni pristup.
Animal reproduction studies have not been conducted using Brineura.
S lijekom Brineura nisu provedena ispitivanja reprodukcije u životinja.
Do not administer Brineura if there are signs and symptoms of acute intracerebroventricular access device leakage, device failure, or device-related infection (see section 4.3 and 4.4).
Nemojte primjenjivati lijek Brineura u slučaju znakova i simptoma propuštanja pomagala za intracerebroventrikularni pristup, kvara pomagala ili infekcije povezane s pomagalom (vidjeti dio 4.3 i 4.4).
Thawed Brineura and flushing solution should be used immediately.
Otopljeni lijek Brineura i otopina za ispiranje trebaju se odmah upotrijebiti.
Each vial contains 44 mg of sodium in 5 ml of solution.
Jedna bočica sadrži 44 mg natrija u 5 ml otopine.
Label one unused sterile syringe “Brineura” and attach a syringe needle.
Jednu neupotrijebljenu sterilnu štrcaljku označite s „Brineura“ i pričvrstite na nju iglu za štrcaljku.
Discard the needle and empty vials per local requirements.
Iglu i prazne bočice bacite sukladno nacionalnim propisima.
Initiate infusion of Brineura at a rate of 2.5 ml per hour.
Započnite infuziju lijeka Brineura brzinom od 2,5 ml na sat.
Discard the needle and the vial with the remaining solution per local requirements.
Iglu i bočicu s preostalom otopinom bacite sukladno nacionalnim propisima.
Intracerebroventricular use
Intracerebroventrikularna primjena
Clear to slightly opalescent and colourless to pale yellow solution, that may occasionally contain thin translucent fibres or opaque particles.
Otopina je bistra do lagano opalescentna i bezbojna do blijedožuta, koja povremeno može sadržavati tanka prozirna vlakna ili neprozirne čestice.
Device-related complications
Komplikacije povezane s pomagalom
Keep this medicine out of the sight and reach of children.
Kako čuvati Brineura Lijek čuvajte izvan pogleda i dohvata djece.
Solution for infusion Two vials of Brineura One vial of flushing solution
Dvije bočice lijeka Brineura Jedna bočica otopine za ispiranje
Name and address of the manufacturer(s) responsible for batch release
Sjedinjene Američke Države Naziv i adresa proizvođača odgovornog za puštanje serije lijeka u promet
The most frequent (>20%) adverse reactions observed during Brineura clinical trials include pyrexia, low CSF protein, ECG abnormalities, vomiting, upper respiratory tract infections, and hypersensitivity.
Najčešće nuspojave (> 20%) opažene s lijekom Brineura u kliničkim ispitivanjima uključuju pireksiju, nisku razinu proteina u
Anti-drug antibodies (ADAs) were detected in both serum and CSF in 79% and 21%, respectively, of patients treated with cerliponase alfa for up to 107 weeks.
Protutijela protiv lijeka (engl. anti-drug antibodies, ADA) ustanovljeni su i u serumu i u CSF-u u 79%
Inspect the scalp for signs of intracerebroventricular access device leakage or failure and for potential infections.
4. Provjerite vlasište zbog znakova propuštanja ili kvara pomagala za intracerebroventrikularni pristup te moguće infekcije.
All pharmacokinetic parameters were similar following the initial infusion on Day 1 and following infusions at Week 5 and Week 13, indicating no apparent accumulation or time dependent PK of cerliponase alfa in CSF or plasma when administered at of dose of 300 mg once every other week.
13. tjednu, što nije ukazivalo na vidljivu akumulaciju ili vremenski ovisnu farmakokinetiku cerliponaze alfa u CSF-u ili plazmi kada se primjenjivala u dozi od 300 mg jednom svaki drugi tjedan.
Brineura and the flushing solution are solutions for infusion.
Brineura i otopina za ispiranje otopine su za infuziju.
Flushing solution for Brineura Intracerebroventricular use
Otopina za ispiranje za lijek Brineura Intracerebroventrikularna primjena
The MAH shall ensure that in each MS where BRINEURA is marketed, all HCPs who are expected to handle / administer the product are provided with an educational programme (i.e. a dosing and administration guide), aiming at preventing and/or minimising the important identified risk of Device issues (infection/blockage/dislocation), containing information about: - How to store BRINEURA; - The device-related complications (i.e. infections, device's leakage and/or failure; the integrity of the device should be confirmed by a neurosurgeon); - How to prepare BRINEURA and the flushing solution; - A detailed step-by step description of BRINEURA intra-cerebro-ventricular infusion and the administration of the flushing solution (provided after BRINEURA infusion is complete) - How to monitor the patients receiving BRINEURA.
- komplikacije povezane s pomagalom (tj. infekcije, propuštanje pomagala i/ili greška; cjelovitost pomagala treba potvrditi neurokirug); - kako pripremiti lijek BRINEURA i otopinu za ispiranje; - detaljni opis, korak po korak, intracerebroventrikularne infuzije lijeka BRINEURA i primjene otopine za ispiranje (nakon završene infuzije lijeka BRINEURA); - kako nadzirati bolesnika koji prima lijek BRINEURA.
Not known (frequency cannot be estimated based on available data) device is displaced and does not function correctly when preparing for infusion inflammation of the brain due to device-related infection
pomagalo je pomjereno te ne funkcionira na ispravan način kod pripreme za infuziju upala mozga uzrokovana infekcijom povezanom s pomagalom
The large CSF to plasma ratios in Cmax and AUC0-t (approximately 1000 and 400, respectively) suggest that the majority of administered cerliponase alfa remains localized within the CNS.
Veliki omjeri CSF-a i plazme u vrijednostima Cmax i AUC0-t (približno 1000 odnosno 400)
Prime the infusion components with Brineura.
3. Napunite infuzijske linije lijekom Brineura.
Brineura and flushing solution Sodium phosphate dibasic heptahydrate Sodium dihydrogen phosphate monohydrate Sodium chloride
Brineura i otopina za ispiranje natrijev hidrogenfosfat heptahidrat natrijev dihidrogenfosfat hidrat natrijev klorid kalijev klorid magnezijev klorid heksahidrat kalcijev klorid dihidrat voda za injekcije
Talk to your doctor or nurse immediately if you experience any of the following:
Odmah obavijestite liječnika ili medicinsku sestru ako Vam se pojavi nešto od sljedećeg: Vrlo česte nuspojave (mogu se javiti u više od 1 na 10 osoba):
Life-threatening allergic reactions are possible with Brineura.
Uz lijek Brineura moguće su po život opasne alergijske reakcije.
Remove the yellow flip-off cap from the flushing solution vial.
Uklonite žutu kapicu s bočice otopine za ispiranje.
Store upright in a freezer (-20°C ± 5°C).
POSEBNE MJERE ČUVANJA Čuvati u zamrzivaču u uspravnom položaju (-20 °C ± 5 °C).
Very common side effects (may affect more than 1 in 10 people): fever vomiting feeling irritable convulsions (seizures) reactions during or shortly after being given the medicine, such as hives, itching or flushing, swollen lips, tongue and/or throat, shortness of breath, hoarseness, turning blue around finger tips or lips, low muscle tone, fainting or incontinence
vrućica povraćanje osjećaj razdražljivosti konvulzije (napadaji) reakcije za vrijeme ili ubrzo nakon primanja lijeka, kao što su koprivnjača, svrbež ili naleti crvenila, otečene usne, jezik i/ili grlo, nedostatak zraka, promuklost, plava boja na vrhovima prstiju ruku ili oko usana, nizak tonus (napetost) mišića, nesvjestica i nemogućnost zadržavanja mokraće (inkontinencija).
Common Common Not known Very common a Propionibacterium acnes, staphylococcus epidermis b Atonic seizures, clonic convulsion, drop attacks, epilepsy, generalised tonic-clonic seizure, myoclonic epilepsy, partial seizures, petit mal epilepsy, seizure, seizure cluster, and status epilepticus c Pyrexia includes combined preferred terms “Pyrexia” and “Increased body temperature” d Catheter flow obstruction e Device dislocation did not occur in clinical trials f Dislodgement of infusion needle
parcijalni napadaji, petit mal epilepsija, napadaj, klaster napadaja i epileptički status c Pireksija uključuje kombinirane preporučene pojmove „pireksija“ i „povišena tjelesna temperatura“
You or your child will need to have surgery to implant the device for giving Brineura.
Za primjenu lijeka Brineura, Vama ili Vašem djetetu bit će potrebno kirurškim postupkom usaditi pomagalo koji pomaže da lijek dospije do određenog dijela mozga.
The following components (not supplied) are required for proper administration of Brineura and flushing solution (see Figure 1 in section 4.2).
Za pravilnu primjenu lijeka Brineura i otopine za ispiranje potrebne su sljedeće komponente (nisu priložene) (vidjeti sliku 1 u dijelu 4.2).
The following CE marked disposable infusion components were used in Brineura clinical trials:
U kliničkim ispitivanjima lijeka Brineura, upotrijebljene su sljedeće infuzijske komponente za jednokratnu uporabu, oznake CE:
Symptoms resolved over time or with administration of antipyretics, antihistamines and/or glucocorticosteroids.
antihistaminika i/ili glukokortikosteroida.
Package leaflet: Information for the user Brineura 150 mg solution for infusion cerliponase alfa This medicine is subject to additional monitoring.
Uputa o lijeku: Informacije za korisnika Brineura 150 mg otopina za infuziju cerliponaza alfa
Verify that the “Brineura” syringe in the syringe pump is empty after the infusion is complete.
12. Kada je infuzija završila, provjerite je li štrcaljka „Brineura“ u pumpi prazna.
This should be taken into consideration for patients on a controlled sodium diet.
O tome treba voditi računa u bolesnika na prehrani s ograničenjem unosa natrija.
Initiate infusion of the flushing solution at a rate of 2.5 ml per hour.
3. Započnite infuziju otopine za ispiranje brzinom od 2,5 ml na sat.
Table 2: Frequency of adverse reactions with Brineura
Tablica 2: Učestalost nuspojava lijeka Brineura
MedDRA Preferred term
preporučeni pojam
Access device replacement should be considered prior to 4 years of regular administration of Brineura, however it must always be ensured, that the intracerebroventricular access device is used in accordance with the provisions of the respective medical device manufacturer.
Zamjenu pomagala za pristup treba razmotriti prije isteka 4 godine redovite primjene lijeka Brineura,
Signs that you or your child may have an infection include fever, headache, neck stiffness, light sensitivity, nausea, vomiting, and change in mental status.
glavobolju, ukočenost u vratu, osjetljivost na svjetlost, mučninu, povraćanje i promjenu mentalnog stanja.
Verify that the “flushing solution” syringe in the syringe pump is empty after the infusion is complete.
5. Kada infuzija završi, provjerite je li štrcaljka s oznakom „otopina za ispiranje“ u pumpi prazna.
Withdraw flushing solution
Izvucite otopinu za ispiranje
Very common Common
Potpuno normalan hod.
Each vial of Brineura contains 150 mg of cerliponase alfa in 5 ml of solution.
Jedna bočica lijeka Brineura sadrži 150 mg cerliponaze alfa u 5 ml otopine.
Preparation for administration of Brineura and flushing solution
Priprema za primjenu lijeka Brineura i otopine za ispiranje
Remove the port needle.
6. Izvucite ulaznu iglu.
The infusion rate for Brineura and the flushing solution is 2.5 ml/hour.
Brineura i otopina za ispiranje daju se brzinom infuzije od 2,5 ml/h.
Your doctor will monitor you or your child for symptoms of life threatening allergic reactions, such as hives, itching or flushing, swollen lips, tongue, and/or throat, shortness of breath, hoarseness, turning blue around finger tips or lips, low muscle tone, fainting or incontinence.
svrbež ili naleti crvenila, otečene usne, jezik i/ili grlo, nedostatak zraka, promuklost, plava boja na vrhovima prstiju ruku ili oko usana, nizak tonus mišića, nesvjestica ili nemogućnost zadržavanja mokraće (inkontinencija).
CE marked intracerebroventricular access devices, and disposable components listed below or equivalent should be used to deliver Brineura.
Za primjenu lijeka Brineura potrebno je upotrijebiti pomagala za intracerebroventrikularni pristup i u nastavku navedene komponente za jednokratnu primjenu ili njihove ekvivalente, oboje s oznakom CE.
Brineura and flushing solution excipients: Sodium phosphate dibasic heptahydrate; Sodium dihydrogen phosphate monohydrate; Sodium chloride;
Pomoćne tvari lijeka Brineura i otopine za ispiranje: natrijev hidrogenfosfat heptahidrat; natrijev dihidrogenfosfat hidrat; natrijev klorid; kalijev klorid; magnezijev klorid heksahidrat; kalcijev klorid dihidrat; voda za injekcije
Common
Ukupno
Thaw before use.
Odmrznuti prije primjene.
Water for injections;
Otopina za infuziju
Each vial of Brineura contains 150 mg of cerliponase alfa* in 5 ml of solution.
Jedna bočica lijeka Brineura sadrži 150 mg cerliponaze alfa* u 5 ml otopine.
Administer flushing solution after Brineura.
Otopinu za ispiranje primijeniti nakon lijeka Brineura.
Administer the flushing solution provided after the Brineura infusion is complete.
Intracerebroventrikularna infuzija otopine za ispiranje Predviđenu otopinu za ispiranje primijenite nakon završene infuzije lijeka Brineura.
Signs of problems with the device include swelling, redness of the scalp, fluid leaking from device and bulging of the scalp.
Znakovi problema s pomagalom uključuju: oticanje, crvenilo vlasišta,
Cardiac disorders
Srčani poremećaji bradikardija često
Refer to the intracerebroventricular access device manufacturer's recommendation for the port needle.
Za preporuke o ulaznoj igli pogledati preporuke proizvođača pomagala za intracerebroventrikularni pristup.