FrancophonIA/ELRC3.0 huggingface dataset:
English: Each vial of Myozyme is for single use only.
Croatian: Jedna bočica lijeka Myozyme namijenjena je isključivo za jednokratnu uporabu.
English: IARs may occur at any time during the infusion of Myozyme or generally up to 2 hours after, and are more likely with higher infusion rates.
Croatian: Reakcije povezane s infuzijom mogu se javiti u bilo kojem trenutku tijekom infuzije lijeka Myozyme ili obično do 2 sata nakon nje, a njihova je pojava vjerojatnija pri većim brzinama infuzije.
English: Myozyme should not be used during pregnancy unless clearly necessary.
Croatian: Myozyme se ne smije primjenjivati tijekom trudnoće, osim ako je to neophodno.
English: • Nephrotic Syndrome: swelling of lower limbs, generalized swelling and protein loss in urine
Croatian: • gubitak proteina u mokraći • nefrotski sindrom: oticanje donjih udova, generalizirano oticanje i gubitak proteina u mokraći
English: The distance walked in 6 minutes increased by a median of 15.0 meters for Myozyme-treated patients and decreased by a median of 7.5 meters for placebo-treated patients, indicating a statistically significant Myozyme treatment effect compared to placebo (p=0.0283).
Croatian: Udaljenost prehodana u 6 minuta povećala se za medijan od 15,0 metara u bolesnika koji su primali Myozyme, dok se u bolesnika koji su primali placebo smanjila za medijan od 7,5 metara, što ukazuje na statistički značajan učinak liječenja lijekom Myozyme u usporedbi s placebom (p=0,0283).
English: The pulmonary benefits observed in patients included a clinically meaningful improvement in FVC of 35% in one patient, and significant reductions in the number of hours of ventilator support needed in 2 patients.
Croatian: Primijećen povoljan učinak na pluća obuhvatio je klinički značajno poboljšanje FVC-a od 35% u jednog bolesnika te bitno smanjenje broja sati nužne mehaničke ventilacije u 2 bolesnika.
English: Keep out of the sight and reach of children.
Croatian: Ovaj lijek čuvajte izvan pogleda i dohvata djece.
English: Only one patient became wheelchair-free.
Croatian: Samo jedan bolesnik nije više trebao invalidska kolica.
English: AUC∞ and Cmax were similar at week 0, 12 and 52 visits indicating alglucosidase alfa pharmacokinetics were not timedependent (Table 5).
Croatian: 12. i 52. tjednu AUC∞ i Cmax su bili slični, što ukazuje na to da farmakokinetika alglukozidaze alfa ne ovisi o vremenu (Tablica 5).
English: Gently invert or massage the infusion bag to mix the diluted solution.
Croatian: Lagano preokrenite ili nježno protrljajte infuzijsku vrećicu da biste promiješali razrijeđenu otopinu.
English: Paediatric and older people
Croatian: Djeca i starije osobe
English: All 5 patients who were receiving invasive ventilation at baseline continued to require ventilation throughout the study (4 patients survived beyond week 104 and one patient died).
Croatian: U svih 5 bolesnika koji su trebali invazivnu mehaničku ventilaciju na početku liječenja, ona je bila potrebna tijekom cijelog ispitivanja (4 bolesnika živjela su dulje od 104 tjedna, a jedan je bolesnik umro).
English: The co-primary efficacy outcome assessments were distance walked (meters) in 6 minutes (6-Minute Walk Test, 6MWT) and FVC (Forced Vital Capacity) % predicted in the sitting position.
Croatian: Dvije primarne mjere ishoda ispitivanja djelotvornosti bile su procijenjene uz pomoć prehodane udaljenosti (u metrima) tijekom 6 minuta (šestominutni test hoda, engl. six minute walking test, 6MWT)) i postotka predviđenog forsiranog vitalnog kapaciteta (engl.
English: If severe hypersensitivity or anaphylactic reactions occur, immediate discontinuation of Myozyme infusion should be considered and appropriate medical treatment should be initiated.
Croatian: Ako se jave teške reakcije preosjetljivosti ili anafilaktičke reakcije, treba razmotriti trenutačni prekid davanja infuzije lijeka Myozyme i započeti odgovarajuće liječenje.
English: This should then be further diluted as follows: Slowly withdraw the reconstituted solution from each vial until the volume for the patient's dose is obtained.
Croatian: Tu količinu zatim treba dalje razrijediti na sljedeći način: polako povucite rekonstituiranu otopinu iz svake bočice dok ne dobijete volumen potreban za bolesnikovu dozu.
English: Benefits of treatment on motor function including the regaining of lost motor skills were observed in some patients.
Croatian: U nekih je bolesnika primijećeno povoljno djelovanje liječenja na motoričke funkcije, uključujući ponovno stjecanje izgubljenih motoričkih vještina.
English: The recommended dosage of Myozyme is 20 mg per kg of body weight.
Croatian: Preporučena doza lijeka Myozyme je 20 mg po kilogramu tjelesne težine.
English: Marketing Authorisation Holder Genzyme Europe B.V.
Croatian: Nositelj odobrenja za stavljanje lijeka u promet Genzyme Europe B.V.
English: All patients were freely ambulatory and all but one patient did not require any form of ventilator support (1 patient required nocturnal non-invasive ventilation).
Croatian: Svi su bolesnici mogli hodati, a samo je jedan trebao određeni oblik mehaničke ventilacije (jedan je bolesnik noću trebao neinvazivnu ventilaciju).
English: Reconstitute each 50 mg vial of Myozyme with 10.3 ml water for injections.
Croatian: Rekonstituirajte svaku bočicu od 50 mg lijeka Myozyme s 10,3 ml vode za injekcije.
English: Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness.
Croatian: U svakoj skupini učestalosti nuspojave su prikazane u padajućem nizu prema ozbiljnosti.
English: It is supplied as a powder which will be mixed with sterile water before it is given.
Croatian: Isporučuje se u obliku praška koji će se prije primjene pomiješati sa sterilnom vodom.
English: Data from a small number of patients with HSAT, with or without inhibitory activity, showed limited ITI treatment effect.
Croatian: Podaci malog broja bolesnika s postojanim visokim titrom protutijela, s ili bez inhibitorne aktivnosti, ukazali su na ograničen učinak postupka indukcije imunološke tolerancije.
English: In a retrospective natural history study in patients with infantile-onset Pompe disease (n=168), the median age at onset of symptoms was 2.0 months and the median age of death was 9.0 months.
Croatian: U retrospektivnom ispitivanju prirodnog tijeka bolesti u bolesnika s infantilnim oblikom Pompeove bolesti (n=168) medijan dobi u kojoj su se javili prvi simptomi bio je 2,0 mjeseca, a medijan dobi u kojoj je nastupila smrt bio je 9,0 mjeseci.
English: After reconstitution, it is recommended to promptly dilute the vials (see below).
Croatian: Nakon rekonstitucije preporučuje se odmah razrijediti sadržaj bočica (vidjeti u nastavku).
English: Myozyme is used to treat adults, children and adolescents of all ages who have a confirmed diagnosis of Pompe disease.
Croatian: Myozyme se primjenjuje u liječenju odraslih bolesnika, djece i adolescenata koji imaju potvrđenu dijagnozu Pompeove bolesti.
English: Younger patients diagnosed and treated early and who received ITI concomitantly to ERT initiation had a trend towards better survival rate than patients treated with similar regimen at a later age.
Croatian: Mlađi bolesnici kojima je bolest rano dijagnosticirana i liječena i kod kojih je istovremeno započeta indukcija imunološke tolerancije i enzimsko nadomjesno liječenje imali su trend prema boljoj stopi preživljavanja nego bolesnici liječeni sličnim režimom u kasnijoj dobi.
English: Due to the proteinaceous nature of the product, particle formation may occur in the reconstituted solution and final infusion bags.
Croatian: Zbog proteinske prirode lijeka može doći do stvaranja čestica u rekonstituiranoj otopini, a potom i u infuzijskim vrećicama.
English: • Narrowing of the blood vessels causing blood flow to be decreased
Croatian: • suženje krvnih žila koje uzrokuje smanjen protok krvi
English: In this group of patients a variable response has also been seen with respect to motor function.
Croatian: U toj su skupini bolesnika primijećeni i različiti odgovori na liječenje vezani uz motoričke funkcije.
English: Patients received 20 mg/kg Myozyme once every two weeks for 52 weeks except for 8 patients who received 40 mg/kg after at least 26 weeks of treatment.
Croatian: Bolesnici su primali 20 mg/kg lijeka Myozyme jednom svaka dva tjedna tijekom 52 tjedna, osim 8 bolesnika koji su primali 40 mg/kg nakon najmanje 26 tjedana liječenja.
English: • Swelling around the eyes
Croatian: • oticanje područja oko očiju
English: Echocardiographic indices of cardiomyopathy improved as measured by a decrease in left ventricular mass (LVM).
Croatian: Ehokardiografski pokazatelji kardiomiopatije su se poboljšali, mjereno smanjenjem mase lijeve klijetke.
English: In 12 patients with available data the AUC∞ and Cmax were approximately equivalent to those observed for the 20 mg/kg dose group in the pivotal trial.
Croatian: U 12 bolesnika za koje su podaci bili dostupni vrijednosti AUC∞ i Cmax bile su otprilike jednake onima uočenima u skupini koja je u pivotalnom ispitivanju primala dozu od 20 mg/kg.
English: If you experience a reaction like this, you should tell your doctor immediately.
Croatian: Ako razvijete takvu reakciju, o tome odmah morate obavijestiti svog liječnika.
English: Myozyme is indicated for long-term enzyme replacement therapy (ERT) in patients with a confirmed diagnosis of Pompe disease (acid α-glucosidase deficiency).
Croatian: Myozyme je indiciran za dugotrajno enzimsko nadomjesno liječenje u bolesnika s potvrđenom dijagnozom Pompeove bolesti (nedostatka kisele α-glukozidaze).
English: The cause of a poor clinical outcome and of developing high and sustained IgG antibody titres is thought to be multi-factorial.
Croatian: Smatra se da na loš klinički ishod te razvoj visokog i postojanog titra IgG protutijela utječu brojni faktori.
English: Overall, the numbers of patients experiencing adverse reactions and serious adverse reactions were comparable between the two groups.
Croatian: Sveukupno je broj bolesnika u obje skupine u kojih su se javile nuspojave ili teške nuspojave bio usporediv.
English: However, the invasive ventilator-free survival was not recorded in the untreated historical cohort and a comparison of this endpoint is not possible.
Croatian: Međutim, preživljenje bez invazivne mehaničke ventilacije nije zabilježeno u neliječenoj kohorti bolesnika s prirodnim tijekom bolesti, pa usporedba ove mjere ishoda nije moguća.
English: Patients with advanced Pompe disease may have compromised cardiac and respiratory function, which may predispose them to a higher risk of severe complications from infusion associated reactions.
Croatian: U bolesnika s uznapredovalom Pompeovom bolešću može biti ugrožena srčana i respiratorna funkcija, zbog čega može postojati veći rizik od teških komplikacija prouzročenih reakcijama povezanih s infuzijom.
English: Of the 3 patients with significant pulmonary involvement at screening/baseline (percentage predicted forced vital capacity in the sitting position ranging from 5867%), two demonstrated clinically meaningful improvements in FVC (+11.5% and +16.0%) in the sitting position by Week 26.
Croatian: Od 3 bolesnika u kojih su na probiru/početku ispitivanja pluća bila znatno zahvaćena (postotak predviđenog forsiranog vitalnog kapaciteta u sjedećem položaju kretao se u rasponu od 58-67%) u 2 su primijećena klinički značajna poboljšanja FVC-a (+11,5% i +16,0%) u sjedećem položaju do 26. tjedna liječenja.
English: Life threatening hypersensitivity (anaphylactic reaction) to the active substance or to any of the excipients listed in section 6.1, when rechallenge was unsuccessful (see sections 4.4 and 4.8).
Croatian: Po život opasna preosjetljivost (anafilaktička reakcija) na djelatnu tvar ili neku od pomoćnih tvari navedenih u dijelu 6.1, kada ponovno uvođenje lijeka nije bilo uspješno (vidjeti dijelove 4.4 i 4.8).
English: Note: Results are from a Cox proportional hazards regression analysis which includes treatment as a time-varying covariate, and also includes age of diagnosis and age at symptom onset.
Croatian: Napomena: Rezultati su dobiveni Coxovom regresijskom analizom razmjernih rizika, koja obuhvaća liječenje kao kovarijantu koja se mijenja s vremenom te dob u kojoj je postavljena dijagnoza i dob u kojoj su nastupili simptomi.
English: After the first year (58 up to 168 weeks) of treatment LVM further decreased in 9 out of 12 patients with available data.
Croatian: Nakon prve godine liječenja (58 do 168 tjedana) masa lijeve klijetke se nastavila smanjivati u 9 od 12 bolesnika za koje su podaci bili dostupni.
English: You should not be given Myozyme during pregnancy unless clearly necessary.
Croatian: Myozyme se ne smije primjenjivati tijekom trudnoće, osim ako je to nužno.
English: Keep this medicine out of the sight and reach of children Do not use this medicine after the expiry date which is stated on the label after ‘EXP'.
Croatian: Ovaj lijek se ne smije upotrijebiti nakon isteka roka valjanosti navedenog na naljepnici iza oznake „EXP“.
English: This medicine contains less than 1 mmol sodium (23 mg) per vial, i.e. essentially ‘sodium free'.
Croatian: Ovaj lijek sadrži manje od 1 mmola natrija (23 mg) po bočici, tj. zanemarive količine natrija.
English: There is no experience with overdose of Myozyme.
Croatian: Nema iskustva s predoziranjem lijekom Myozyme.
English: • Feeling hot
Croatian: • osjećaj vrućine
English: Subjects in the untreated historical cohort were born in 1993 or later.
Croatian: Ispitanici u neliječenoj kohorti bolesnika s prirodnim tijekom bolesti rođeni su 1993. ili kasnije.
English: Patients who experience hypersensitivity reactions may also be tested for IgE antibodies to alglucosidase alfa and other mediators of anaphylaxis.
Croatian: Bolesnike u kojih se jave reakcije preosjetljivosti može se također testirati na IgE protutijela na alglukozidazu alfa i druge posrednike anafilaksije.
English: Subjects ranged in age from 6 months to 3.5 years at initiation of treatment.
Croatian: Dob ispitanika bila je na početku liječenja između 6 mjeseci i 3,5 godine.
English: After 104 weeks of treatment, all 16 patients who enrolled in the extension study were alive and 10 of these 16 patients were free of invasive ventilatory support.
Croatian: Nakon 104 tjedna liječenja svih 16 bolesnika koji su uključeni u produžetak ispitivanja bilo je živo, a 10 od tih 16 nije trebalo invazivnu mehaničku ventilaciju.
English: Adverse reactions reported in at least 2 patients are listed in Table 1 by System Organ Class.
Croatian: Nuspojave prijavljene u najmanje 2 bolesnika navedene su u Tablici 1 prema organskim sustavima.
English: In a placebo-controlled study lasting 78 weeks, 90 patients with late-onset Pompe disease, aged 10 to 70 years, were treated with Myozyme or placebo randomized in a 2:1 ratio (see section 5.1).
Croatian: U placebom kontroliranom ispitivanju u trajanju od 78 tjedana 90 bolesnika s kasnim oblikom Pompeove bolesti u dobi od 10 do 70 godina randomizirano je u omjeru 2:1 u skupine koje su primale Myozyme ili placebo (vidjeti dio 5.1).
English: After 52 weeks of treatment, all 18 patients treated with Myozyme were alive and 15 of these 18 patients were alive and free of invasive ventilatory support whereas 1 of 42 patients in the untreated historical cohort was alive at 18 months of age.
Croatian: Nakon 52 tjedna liječenja svih 18 bolesnika liječenih lijekom Myozyme bilo je živo, a 15 od tih 18 bolesnika nije trebalo invazivnu mehaničku ventilaciju, dok je 1 od 42 bolesnika u neliječenoj kohorti bolesnika s prirodnim tijekom bolesti bio živ u dobi od 18 mjeseci.
English: Also remove an equal volume of sodium chloride 9 mg/ml (0.9%) solution for injection, that will be replaced with reconstituted Myozyme.
Croatian: Također, uklonite odgovarajući volumen otopine natrijeva klorida za injekciju od 9 mg/ml (0,9%) koji će se zamijeniti rekonstituiranim lijekom Myozyme.
English: There is no evidence for special considerations when Myozyme is administered to paediatric patients of all ages or older people.
Croatian: Nema znakova koji bi upućivali na potrebu za posebnim mjerama opreza kod primjene lijeka Myozyme u pedijatrijskih bolesnika svih dobi ili starijih osoba.
English: IgG antibody titres should be regularly monitored.
Croatian: Potrebno je redovito pratiti titar IgG protutijela.
English: Myozyme contains sodium
Croatian: Myozyme sadrži natrij
English: Myozyme will be given to you under the supervision of a doctor who is experienced in the treatment of Pompe disease.
Croatian: Myozyme ćete primati pod nadzorom liječnika koji ima iskustva u liječenju Pompeove bolesti.
English: At the end of the study (with individual patient treatment durations ranging from 60 to 150 weeks; mean follow-up period of 119 weeks) 14 of 16 patients were alive and 9 of 16 patients were alive and free of invasive ventilatory support.
Croatian: Na kraju ispitivanja (s trajanjem liječenja pojedinih bolesnika u rasponu od 60 do 150 tjedana i prosječnim vremenom praćenja od 119 tjedana) 14 od 16 bolesnika bilo je živo, a 9 od 16 bolesnika bilo je živo i nije trebalo invazivnu mehaničku ventilaciju.
English: Because it is a recombinant human protein, alglucosidase alfa is an unlikely candidate for cytochrome P450 mediated drug-drug interactions.
Croatian: Budući da je riječ o rekombinantnom ljudskom proteinu, nije vjerojatno da bi alglukozidaza alfa mogla sudjelovati u interakcijama lijekova posredovanima citokromom P450.
English: Serious infusion reactions including urticaria, rales, tachycardia, decreased oxygen saturation, bronchospasm, tachypnea, periorbital edema and hypertension have been reported.
Croatian: Prijavljene su teške reakcije povezane s infuzijom, uključujući urtikariju, hriptanje pri disanju, tahikardiju, smanjenu zasićenost kisikom, bronhospazam, tahipneju, periorbitalni edem i hipertenziju.
English: There does not appear to be a correlation between the onset of IARs and the time of IgG antibody formation.
Croatian: Čini se da nema povezanosti između vremena pojave reakcija povezanih s infuzijom i vremena stvaranja IgG protutijela.
English: Deficiency of this enzyme leads to glycogen accumulation in various tissues, particularly cardiac, respiratory and skeletal muscle, leading to the development of hypertrophic cardiomyopathy and progressive muscle weakness, including impairment of respiratory function.
Croatian: Nedostatak tog enzima dovodi do nakupljanja glikogena u različitim tkivima, osobito u srcu te respiratornim i skeletnim mišićima, što dovodi do razvoja hipertrofične kardiomiopatije i progresivne slabosti mišića, uključujući i slabljenje respiratorne funkcije.
English: 2 Reactions reported in 60 late-onset patients in a placebo-controlled clinical trial.
Croatian: 2 Reakcije prijavljene u 60 bolesnika s kasnim oblikom bolesti u placebom kontroliranom kliničkom ispitivanju.
English: Immunomodulation
Croatian: Imunomodulacija
English: The recommended final concentration of alglucosidase in the infusion bags ranges from 0.5 mg/ml to 4 mg/ml.
Croatian: Preporučena konačna koncentracija alglukozidaze u infuzijskim vrećicama kreće se u rasponu od 0,5 mg/ml do 4 mg/ml.
English: The vast majority of patients with infantile-onset Pompe disease treated with Myozyme demonstrate improvement in cardiac function as well as stabilisation or improvements in growth parameters.
Croatian: U velike većine bolesnika s infantilnim oblikom Pompeove bolesti liječene lijekom Myozyme primijećeno je poboljšanje srčane funkcije te stabilizacija ili poboljšanje parametara rasta.
English: The mean area under the plasma concentration-time curve (AUC∞) ranged from 977.5 to 1,872.5 µg•h/ml for the 20 mg/kg and 40 mg/kg dose groups.
Croatian: Srednja vrijednost površine ispod krivulje koncentracija-vrijeme (AUC∞) kretala se u rasponu od 977,5 do 1872,5 µg•h/ml za skupine koje su primale dozu od 20 mg/kg odnosno 40 mg/kg.
English: In the rabbit embryofoetal development study, following administration of Myozyme (10-40 mg/kg/day) with coadministration of diphenhydramine, a treatment-related increase in the incidence of abortions and early delivery was observed.
Croatian: U ispitivanju embriofetalnog razvoja u kunića nakon primjene lijeka Myozeme (10-40 mg/kg/dan) u kombinaciji s difenhidraminom primijećena je veća incidencija pobačaja i prijevremenog okota povezana s liječenjem.
English: Slightly more patients in the Myozyme group than in the placebo group experienced IARs (28% versus 23%).
Croatian: Reakcije povezane s infuzijom nešto su se češće javljale u bolesnika koji su primali Myozyme nego u onih koji su primali placebo (28% naspram 23%).
English: The majority of patients were successfully re-challenged with alglucosidase alfa using lower doses and/or pretreatment with antiinflammatory drugs and/or corticosteroids and have continued to receive treatment under close clinical supervision.
Croatian: Većina bolesnika uspješno je ponovno liječena alglukozidazom alfa u manjim dozama i/ili uz prethodno liječenje protuupalnim lijekovima i/ili kortikosteroidima te su nastavili primati terapiju pod strogim kliničkim nadzorom.
English: After the first and sixth infusion of Myozyme, mean maximum plasma concentrations (Cmax) ranged from 178.2 to 263.7 µg/ml for the 20 mg/kg and 40 mg/kg dose groups respectively.
Croatian: Nakon prve i šeste infuzije lijeka Myozyme srednja maksimalna koncentracija u plazmi (Cmax) kretala se u rasponu od 178,2 µg/ml do 263,7 µg/ml za skupine ispitanika koje su primale doze od 20 mg/kg odnosno 40 mg/kg.
English: If immune-mediated reactions occur, discontinuation of the administration of alglucosidase alfa should be considered and appropriate medical treatment initiated.
Croatian: Ako se pojave imunološki posredovane reakcije, treba razmotriti prekid primjene alglukozidaze alfa te započeti odgovarajuće liječenje.
English: Subjects in the untreated historical cohort were born in 1995 or later.
Croatian: Ispitanici u neliječenoj kohorti bolesnika s prirodnim tijekom bolesti rođeni su 1995. ili kasnije.
English: MANUFACTURERS OF THE BIOLOGICAL ACTIVE SUBSTANCE AND MANUFACTURERS RESPONSIBLE FOR BATCH RELEASE
Croatian: PROIZVOĐAČI BIOLOŠKE DJELATNE TVARI I PROIZVOĐAČI ODGOVORNI ZA PUŠTANJE SERIJE LIJEKA U PROMET
English: Table 2: Results for endpoint survival using the Cox regression model
Croatian: Tablica 2: Rezultati mjere ishoda preživljenja dobiveni primjenom Coxova regresijskog modela
English: Because of the potential for severe infusion associated reactions, appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available when Myozyme is administered.
Croatian: Zbog mogućih teških reakcija povezanih s infuzijom kod primjene lijeka Myozyme treba na raspolaganju imati odgovarajuću opremu za primjenu medicinskih potpornih mjera, uključujući opremu za kardiopulmonalno oživljavanje.
English: The untreated historical cohort was matched to the pivotal study population and was derived from a retrospective natural history study (n=42) in patients with infantile-onset Pompe disease.
Croatian: Neliječena kohorta bolesnika s prirodnim tijekom bolesti uparena je s populacijom iz pivotalnog ispitivanja, a dobivena je retrospektivnim ispitivanjem prirodnog tijeka bolesti (n=42) u bolesnika s infantilnim oblikom Pompeove bolesti.
English: What you need to know before you are given Myozyme Do not use Myozyme:
Croatian: Što morate znati prije nego počnete primjenjivati Myozyme
English: Approximately half of the patients treated with Myozyme in infantile-onset clinical studies and 28% of the patients treated with Myozyme in a late-onset clinical study developed infusion associated reactions (IARs).
Croatian: U otprilike polovice bolesnika liječenih lijekom Myozyme u kliničkim ispitivanjima infantilnog oblika bolesti te u 28% bolesnika liječenih lijekom Myozyme u kliničkom ispitivanju kasnog oblika bolesti došlo je do reakcija povezanih s infuzijom.