qanastek/EMEA-V3 huggingface dataset:
AE terminology is based upon the Medical Dictionary for Regulatory Activities (MedDRA)
Terminoloġija AE hi bażata fuq id- Dizzjunarju Mediku għal Attivitajiet Regolatorji (MedDRA)
Infusion associated reactions consisted most often of fever and chills.
Reazzjonijiet assoċjati ma ’ l- infużjoni kienu jikkonsistu ta ’ spiss minn deni u sirdat.
Additional symptoms included mild or moderate dyspnoea, throat tightness, chest discomfort, flushing, pruritus, urticaria, face oedema, angioneurotic oedema, rhinitis, bronchospasm, tachypnoea, wheezing, hypertension, hypotension, tachycardia, palpitations, abdominal pain, nausea, vomiting, infusion-related pain including pain at the extremities, myalgia, and headache.
Sintomi addizzjonali kienu jinkludu dispnea ħafifa jew moderata, griżmejn issikkati, skonfort fis- sider, fwawar, ħakk, urtikarja, edema tal- wiċċ, edema anġjonewrotika, rinite, bronkospażmu, takipneja, tħarħir, pressjoni għolja, pressjoni baxxa, takikardja, palpitazzjonijiet, uġigħ addominali, nawsja, rimettar, uġigħ relatat ma ’ l- infużjoni li jinkludi uġigħ fl- estremitajiet, uġigħ fl- għadam u uġigħ ta ’ ras.
The infusion-associated reactions were managed by a reduction in the infusion rate together with the administration of non-steroidal anti-inflammatory medicinal products, antihistamines and/ or corticosteroids.
Reazzjonijiet assoċjati ma ’ l- infużjoni kienu mmaniġġati bi tnaqqis fir- rata ta ’ l- infużjoni flimkien ma ’ l- għoti ta ’ prodotti mediċinali anti- infjammatorji mhux sterojdi, antistamini u/ jew kortikosterojdi.
Sixty seven percent (67%) of the patients experienced at least one infusion-associated reaction.
Sebgħa u sittin fil- mija (67%) tal- pazjenti kellhom ta ’ lanqas reazzjoni waħda assoċjata ma ’ l- infużjoni.
The majority of these reactions can be
Il- frekwenza ta ’ dawn ir- reazzjonijiet naqset maż- żmien.
5 attributed to the formation of IgG antibodies and/ or complement activation.
Il- maġġoranza ta ’ dawn ir- reazzjonijiet jistgħu jkunu attribwiti mal- formazzjoni ta ’ l- antikorpi IgG u/ jew attivazzjoni komplimentari.
In a limited number of patients IgE antibodies were demonstrated (see Section 4.4).
F’ għadd limitat ta ’ pazjenti intwera li kien hemm antikorpi IgE (ara Sezzjoni 4. 4).
Paediatric patients Limited information suggests that the safety profile of Fabrazyme treatment in paediatric patients (above the age of 7) is not different with that seen in adults.
Pazjenti pedjatriċi Informazzjoni ristretta tissuġerixxi li l- profil tas- sigurtà tat- trattament b’ Fabrazyme f’ pazjenti pedjatriċi (li għandhom età ta ’ 7 snin) mhux differenti minn dak muri f’ adulti.
In clinical trials doses up to 3mg/ kg body weight were used.
Fi studji kliniċi dożi sa 3 mg/ kg piż tal- ġisem kienu użati.
Alimentary tract and metabolism products – enzymes.
Prodotti ta ’ l- apparat alimentari u tal- metaboliżmu – enżimi.
Fabry disease is an inherited heterogeneous and multisystemic progressive disease, that affects both males and females.
Il- marda Fabry hi marda eteroġena li tintiret u marda multisistematika progressiva, li taffettwa kemm l- irġiel u n- nisa.
It is characterised by the deficiency of α -galactosidase.
Hija karatterizzata minn nuqqas ta ’ l- α- galactosidase.
Reduced or absent α - galactosidase activity results in the accumulation of GL-3 in the lysosomes of many cell types including the endothelial and parenchymal cells, ultimately leading to life-threatening clinical deteriorations as a result of renal, cardiac and cerebrovascular complications.
Terapija ta ’ sostituzzjoni ta ’ l- enżima issir biex jerġa ’ jintlaħaq
The rationale for enzyme replacement therapy is to restore a level of enzymatic activity sufficient to clear the accumulating substrate in the organ tissues; thereby, preventing, stabilizing or reversing the progressive decline in function of these organs before irreversible damage has occurred.
6 il- livell ta ’ attività enżimatika li jkun biżżejjed biex ineħħi s- subtrat akkumulat fit- tessuti ta ’ l- organi; b’ hekk jiġi evitat, stabilizzat jew irriversjat it- tnaqqis progressiv tal- funzjoni ta ’ dawn l- organi qabel ma ssir ħsara rriversibbli.
After intravenous infusion, agalsidase beta is rapidly removed from the circulation and taken up by vascular endothelial and parenchymal cells into lysosomes, likely through the mannose-6 phosphate, mannose and asialoglycoprotein receptors.
Wara l- infużjoni minn ġol- vina, agalsidase beta malajr jitneħħa miċ- ċirkolazzjoni u jiġi assorbit miċ- ċelluli vaskulari endotiljali u ċelluli parenkimali f’ liżożomi, x’ aktarx permezz ta ’ mannose- 6 phosphate, mannose u riċetturi ta ’ asialoglycoprotein
Efficacy and safety of Fabrazyme was evaluated in one study with children, one dose-finding study, two double-blind placebo-controlled studies, and one open-label extension study in both male and female patients.
Fl- effikaċja u s- sigurtà ta ’ Fabrazyme ġew evalwati fi studju wieħed fuq it- tfal, bi studju wieħed fuq id- dożi, żewġ studji double- blind bi plaċebo użati bħala kontrolli, u studju ta ’ estensjoni open label fuq pazjenti kemm irġiel u n- nisa.
In the dose finding study, the effects of 0.3, 1.0 and 3.0 mg/ kg once every 2 weeks and 1.0 and 3.0 mg/ kg once every 2 days were evaluated.
Fl- istudju dwar id- dożi l- effetti ta ’ 0. 3, 1. 0 u 3. 0 mg/ kg darba kull ġimagħtejn u 1. 0 u 3. 0 mg/ kg darba kull jumejn kienu evalwati.
A reduction in GL-3 was observed in kidney, heart, skin and plasma at all doses.
Kien hemm tnaqqis sinifikanti ta ’ GL- 3 fil- kliewi, qalb, ġilda u plażma fid- dożi kollha.
Plasma GL-3 was cleared in a dose dependent manner, but was less consistent at the dose of 0.3 mg/ kg.
Ir- rata ta ’ tneħħija ta ’ GL- 3 fil- plażma kienet tiddependi mid- doża, imma kienet inqas konsistenti meta d- doża kienet 0. 3 mg/ kg.
In addition, infusion-associated reactions were dose dependent.
Barraminhekk, ir- reazzjonijiet relatati ma ’ l- infużjoni kienu dipendenti mid- doża.
In the first placebo-controlled clinical trial, Fabrazyme was effective in clearing GL-3 from the vascular endothelium of the kidney after 20 weeks of treatment.
Fl- ewwel prova klinika bi plaċebo bħala kontrolli, Fabrazyme kien effettiv fit- tneħħijja ta ’ GL- 3 mill- endotilju vaskulari tal- kilwa wara 20 ġimgħa ta ’ kura.
This clearance was achieved in 69% (20/ 29) of the Fabrazyme treated patients, but in none of the placebo patients (p < 0.001).
Din ir- rata ta ’ tneħħijja ntlaħqet f’ 69% (20/ 29) tal- pazjenti trattati b’ Fabrazyme, imma fl- ebda pazjent li ngħata l- plaċebo (p < 0. 001).
This finding was further supported by a statistically significant decrease in GL-3 inclusions in kidney, heart and skin combined and in the individual organs in patients treated with agalsidase beta compared to placebo patients (p < 0.001).
Dan ir- riżultat kien sostnut aktar bi tnaqqis statiskament sinjifikanti fl- ammont ta ’ GL- 3 fil- kliewi, il- qalb u l- ġilda kif ukoll fl- organi individwali ta ’ pazjenti li ngħataw agalsidase beta meta mqabbla ma ’ pazjenti li ngħataw il- plaċebo (p < 0. 001).
Sustained clearance of GL-3 from kidney vascular endothelium upon agalsidase beta treatment was demonstrated further in the open label extension of this trial.
Tneħħija sostnuta ta ’ GL- 3 mill- endotelju vaskulari tal- kliewi bi trattament b’ agalsidase beta kien appoġġjat aktar fl- estensjoni open label ta ’ din il- prova.
This was achieved in 47 of the 49 patients (96%) with available information at month 6, and in 8 of the 8 patients (100%) with available information at the end of the study (up to a total of 5 years of treatment).
Dan intlaħaq f’ 47 mid- 49 pazjent (96%) b’ informazzjoni disponibbli fis- 6 xahar, u fi 8 mit- 8 pazjenti (100%) b’ informazzjoni disponibbli fl- aħħar ta ’ l- istudju (sa total ta ’ 5 snin ta ’ trattament).
Clearance of GL-3 was also achieved in several other cell types from the kidney.
It- tneħħija ta ’ GL- 3 kien milħuq ukoll f’ diversi tipi ta ’ ċelloli oħra mill- kliewi.
Plasma GL-3 levels rapidly normalised with treatment and remained normal through 5 years.
Livelli ta ’ GL- 3 fil- plażma innormalizzaw malajr bi trattament u baqgħu normali matul il - 5 snin.
Renal function, as measured by glomerular filtration rate and serum creatinine, as well as proteinuria, remained stable in the majority of the patients.
Il- funzjoni renali, kif imkejjel bir- rata ta ’ filtrazzjoni glomerulari u kreatinin fis- serum, kif ukoll proteinurja baqgħu stabbli fil- maġġoranza tal- pazjenti.
However, the effect of Fabrazyme treatment on the kidney function was limited in some patients with advanced renal disease.
Madankollu l- effett ta ’ trattament b’ Fabrazyme fuq il- funzjoni tal- kliewi kien ristrett f’ xi pazjenti li kellhom mard tal- kliewi avvanzat.
6 Although no specific study has been conducted to assess the effect on the neurological signs and symptoms, the results also indicate that patients may achieve reduced pain and enhanced quality of life upon enzyme replacement therapy.
Għalkemm ma sar ebda studju speċifiku biex jassessja l- effett fuq is- sinjali u sintomi newroloġiċi, ir- riżultati jindikaw ukoll li pazjenti jista ’ jkollhom uġigħ mnaqqas u kwalità ta ’ ħajja mtejba permezz ta ’ trattament ta ’ sostituzzjoni ta ’ l- enżimi.
Another double-blind, placebo-controlled study of 82 patients was performed to determine whether Fabrazyme would reduce the rate of occurrence of renal, cardiac, or cerebrovascular disease or death.
Sar studju double- blind ieħor, bi plaċebos użati bħala kontrolli, fuq 82 pazjent biex jistabilixxi jekk Farbrazyme jnaqqasx ir- rata ta ’ mard renali, kardijaku jew ċerebovaskulari jew mewt.
The rate of clinical events was substantially lower among Fabrazyme-treated patients compared to placebo-treated patients (risk reduction = 53% intent-to-treat population (p=0.0577); risk reduction = 61% per-protocol population (p=0.0341)).
Ir- rata ta ’ każijiet kliniċi kien sostanzjalment inqas fost pazjenti trattati b ’ Fabrazyme meta mqabbel ma ’ pazjenti trattati bi plaċebo (tnaqqis fir- riskju = 53% maħsuba biex jittrattaw popolazzjoni (p=0. 0577); tnaqqis fir- riskju = 61% skond popolazzjoni ta ’ protokoll (p=0. 0341)).
This result was consistent across renal, cardiac and cerebrovascular events.
Dan ir- riżultat kien konsistenti għal każijiet renali, kardijaċi u ċerebrovaskulari.
The results of these studies indicate that Fabrazyme treatment at 1 mg/ kg every other week provides clinical benefit on key clinical outcomes in patients with early and advanced Fabry disease.
Ir- riżultati ta ’ dawn l- istudji jindikaw li t- trattament b’ Fabrazyme b’ 1mg/ kg ġimgħa iva u ġimgħa le huwa ta ’ benefiċċju kliniku f’ eżiti kliniċi prinċipali f’ pazjenti li għandhom marda bikrija jew avvanzata ta ’ Fabry.
Because this condition is slowly progressive, early detection and treatment is critical to achieve the best outcomes.
Minħabba li din il- kundizzjoni tipprogressa bil- mod, huwa kritiku li jkun hemm intraċċar u trattament bikri għall- aħjar riżultati.
In the open-label paediatric study, sixteen patients with Fabry disease (8-16 years old; 14 males, 2 females) had been treated for one year.
Fl- istudju pedjatriku open label, sittax- il pazjent bil- marda Fabry (8- 16- il sena; 14- il raġel, 2 nisa) kienu ilhom jiġu trattati għal sena.
Clearance of GL-3 in the superficial skin vascular endothelium was achieved in all patients who had accumulated GL-3 at baseline.
Iż- 2 pazjenti nisa ftit
The 2 female patients had little or no GL-3 accumulation in the superficial skin vascular endothelium at baseline, making this conclusion applicable in male patients only.
7 jew xejn kellhom akkumulazzjoni ta ’ GL- 3 fl- endotilju vaskulari tal- ġilda superfiċjali fil- punt ta ’ linja bażi, li jagħmel din il- konklużjoni applikabbli għal pazjenti rġiel biss.
In an additional study, 21 male patients were enrolled to follow GL3 clearance in kidney and skin tissues at an alternative dosing regimen.
Fi studju addizzjonali, 21 pazjent raġel kienu rekrutati biex isegwu t- tneħħija tal- GL3 mill- kliewi u t- tessuti tal- ġilda bi programm ta ’ dożi alternattivi.
Following treatment with 1 mg/ kg every other week for 24 weeks, a dose regimen of 0.3 mg/ kg every 2 weeks for 18 months was able to maintain the clearance of cellular GL-3 in the capillary endothelium of the kidney, other kidney cell types and skin (superficial skin capillary endothelium) in the majority of patients.
Wara t- tratatment b’ 1 mg/ kg ġimgħa iva u ġimgħa le għal 24 ġimgħa, programm b’ doża ta ’ 0. 3 mg/ kg kull ġimagħtejn għal 18- il xahar seta ’ jikkawża tneħħija ta ’ GL- 3 ċellulari fl- endotelju kapillari tal- kliewi, tipi oħra ta ’ ċelloli tal- kliewi u l- ġilda (endotelju kapillari superfiċjali tal- ġilda) fil- maġġoranza tal- pazjenti.
However, at the lower dose, IgG antibodies may play a role with respect to GL-3 clearance in some patients.
Madankollu, fid- doża aktar baxxa, l- antikorpi IgG jista ’ jkollhom rwol fir- rigward ta ’ tneħħija ta ’ GL- 3 f’ xi pazjenti.
Due to the limitations of the study design (small number of patients), no definitive conclusion regarding the dose maintenance regimen can be drawn, but these findings suggest that, after an initial debulking dose of 1.0 mg/ kg every 2 weeks, 0.3 mg/ kg every 2 weeks may be sufficient in some patients to maintain clearance of GL-3.
Minħabba l- limitazzjonijiet ta ’ kif l- istudju kien ippjanat (għadd żgħir ta ’ pazjenti), ma setgħax ikun hemm konklużjoni definittiva dwar il- programm ta ’ żamma ta ’ doża, imma dawn ir- riżultati jissuġerixxu li, wara doża inizjali ta’ 1. 0 mg/ kg kull ġimagħtejn ġiet imwaqqfa, 0. 3 mg/ kg kull ġimagħtejn tista ’ tkun biżżejjed f’ xi pazjenti biex iżommu t- tneħħija ta ’ GL- 3.
Following an intravenous administration of agalsidase beta to adults at doses of 0.3 mg, 1 mg and 3 mg/ kg body weight, the AUC values increased more than dose proportional, due to a decrease in clearance, indicating a saturated clearance.
Wara l- għoti ġol- vina ta ’ agalsidase beta lil adulti f’ dożi ta ’ 0. 3 mg, 1 mg u 3 mg/ kg piż tal- ġisem, il- valuri ta ’ l- AUC żdiedu b’ rata ogħla milli kieku kienet direttament proporzjonali mad- doża mogħtija, minħabba tnaqqis fit- tneħħija, li turi li tneħħija saturata.
The elimination half-life was dose dependent and ranged from 45 to 100 minutes.
Il- half- life ta ’ l- eliminazzjoni kienet dipendenti fuq id- doża u kienet mifruxa fuq medda ta ’ bejn 45 u 100 minuta.
After intravenous administration of agalsidase beta to adults with an infusion time of ca.
Wara li ngħatat doża minn ġol- vina ta ’ agalsidase beta lil adulti f’ ħin ta ’ infużjoni ta ’ ca.
300 minutes and at a dose of 1 mg/ kg body weight, biweekly, mean Cmax plasma concentrations ranged from 2000- 3500 ng/ ml, while the AUCinf ranged from 370-780 μ g·min/ ml.
300 minuta u b’ doża ta ’ 1 mg/ kg piż tal- ġisem, għal darbtejn fil- ġimgħa, il- livelli medji ta ’ Cmax fil- konċentrazzjonijiet tal- plażma varjaw bejn 2000- 3500 ng/ ml, waqt li l- AUCinf kien bejn 370- 780 μg. min/ ml.
Vss ranged from 8.3-40.8 l, plasma clearance from 119-345 ml/ min and the mean elimination half-life from 80-120 minutes.
Il- Vss varja bejn 8. 3- 40. 8 1/ kg, it- tneħħijja mil- plażma kienet bejn 119- 345 ml/ min u l- half- life ta ’ l- eliminazzjoni medja kienet bejn 80- 120 minuta.
Fabrazyme pharmacokinetics was also evaluated in 15 paediatric patients (8.5 to 16 years old weighing 27.1 to 64.9 kg).
Il- farmakokinetiċi ta ’ Fabrazyme kienu evalwati ukoll fi 15- il- pazjent pedjatriku (8. 5 sa 16- il sena li jiżnu 27. 1 sa 69. 4 kg).
Agalsidase clearance was not influenced by weight in this population.
It- tneħħija ta ’ agalsidase ma kinetx influwenzata mil- piż f’ din il - popolazzjoni.
Baseline clearance was 77 ml/ min with a volume of distribution at steady-state (Vss) of 2.6 l; half-life was 55 min.
Ir- rata ta ’ tneħħija fil- linja bażi kienet ta ’ 77 ml/ min b’ volum ta ’ distribuzzjoni fl- istadju fiss (Vss) ta ’ 2. 61; half- life kien ta ’ 55 min.
After IgG seroconversion, clearance decreased to 35 ml/ min, Vss increased to 5.4 l, and half-life increased to 240 min.
Wara serokonverżjoni ta ’ IgG, it- tneħħija tnaqqset għal 35 ml/ min, Vss żdied għal 5. 4 l u l- half- life żdied għal 240 min.
The net effect of these changes after seroconversion was an increase in exposure of 2- to 3-fold based on AUC and Cmax.
L- effett nett ta ’ dawn il- bidliet wara serokonverżjoni kien żieda ta ’ 2 sa 3 darbiet fl- espożizzjoni skond l- AUC u Cmax.
No unexpected safety issues were encountered in patients with an increase in exposure after seroconversion.
Ma kienx hemm kwistjonijiet dwar sigurtà iffaċċjati f’ pazjenti b’ żieda fl- espożizzjoni wara serokonverżjoni.
Agalsidase beta is a protein and is expected to be metabolically degraded through peptide hydrolysis.
Agalsidase beta hi proteina u hi mistennija li tkun degradata metabolikament permezz ta ’ l- idrolisi tal- peptidi.
Consequently, impaired liver function is not expected to affect the pharmacokinetics of agalsidase beta in a clinically significant way.
L- eliminazzjoni ta ’ agalsidase beta mill- kliewi hu meqjus bħala passaġġ minuri ta ’ kif jitneħħa mil- ġisem.
Renal elimination of agalsidase beta is considered to be a minor pathway for clearance.
5. 3 Tagħrif ta ’ qabel l- użu kliniku dwar is- sigurtà tal- mediċina
Non-clinical data reveal no special hazard for humans based on studies of safety pharmacology, single dose toxicity, repeated dose toxicity and embryonal/ foetal toxicity.
Tagħrif mhux kliniku ma juri l- ebda periklu speċjali għall- bnedmin skond studji tas- sigurtà farmakoloġika, effett tossiku ta ’ doża waħda, effett tossiku ta ’ doża doppja u effett tossiku fuq l- embriju u l- fetu.
Studies with regard to other stages of the development have not been carried out.
Studji dwar l- istadji l- oħra ta ’ l- iżvilupp ma sarux.
Genotoxic and carcinogenic potential are not expected.
Mhux mistenni li jkun jista ’ jkollu effett tossiku fuq il- ġeni jew effett kanċeroġenu.
Mannitol Sodium phosphate monobasic, monohydrate Sodium phosphate dibasic, heptahydrate.
Mannitol Sodium phosphate monobasic, monohydrate Sodium phosphate dibasic, heptahydrate.
In the absence of compatibility studies, Fabrazyme must not be mixed with other medicinal products in the same infusion.
Minħabba li ma sarux studji li juru liema prodotti jaqblu, Fabrazyme m’ għandux jitħallat ma ’ prodotti mediċinali oħra fl- istess infużjoni.
Reconstituted and diluted solutions From a microbiological point of view, the product should be used immediately.
Soluzzjonijiet rikostitwiti u dilwiti Mil- lat mikrobijoloġiku, il- prodott għandu jintuża mill- ewwel.
If not used immediately, in-use storage and conditions prior to use are the responsibility of the user.
Jekk ma jintużax mill- ewwel, il- ħażna waqt l- użu u l- kondizzjonijiet qabel l- użu huma r- responsabbiltà ta ’ min jużah.
The reconstituted solution cannot be stored and should be promptly diluted; only the diluted solution can be held for up to 24 hours at 2 °C-8 °C.
Is- soluzzjoni rikostitwita ma tistax tinħażen u għandha tiġi dilwita minnufih; is- soluzzjoni dilwita biss tista ’ tinżamm għal 24 siegħa f’ temperaura ta ’ bejn 2°C- 8°C.
Store in a refrigerator (2 °C – 8 °C).
Aħżen fi friġġ (2°C – 8°C).
Fabrazyme 35 mg is supplied in clear Type I glass 20 ml vials.
Fabrazyme 35 mg jiġi f’ kunjetti tal- ħġieġ ċar ta ’ Tip 1 ta ’ 20 ml.
The closure consists of a siliconised butyl stopper and an aluminium seal with a plastic flip-off cap.
L- għatu jikkonsisti minn tap tal- butyl silikonizzat b’ siġill ta ’ l- aluminju u għatu tal- plastik li tista ’ tiftħu b’ daqqa ta’ saba ’.
Package sizes:
Qisien tal- pakketti:
1, 5 and 10 vials per carton.
1, 5 u 10 kunjetti kull kartuna.
Not all pack sizes may be marketed.
Jista ’ jkun li mhux il- pakketti tad- daqsijiet kollha jkunu għall- bejgħ.
Use Aseptic Technique
It- trab tal- konċentrat għal soluzzjoni għall- infużjoni irid ikun rikostitwit bl- ilma għall- injezzjonijiet, dilwit b’ soluzzjoni għal ġol vina ta ’ 0. 9% sodium chloride u mbagħad tingħata permezz ta ’ infużjoni minn ġol- vina. Uża Teknika Asettika
Determine the number of vials to be reconstituted based on the individual patient’ s weight and remove the required vials from the refrigerator in order to allow them to reach room temperature (in approximately 30 minutes).
Ikkalkula l- għadd ta ’ kunjetti li jridu jiġu rikostitwiti skond il- piż tal- ġisem individwali tal- pazjent u neħħi l- kunjetti meħtieġa minn ġol- friġġ biex tħallihom jilħqu t- temperatura tal- kamra (f’ madwar 30 minuta).
Each vial of Fabrazyme is intended for single use only.
Kull kunjett ta ‘ Fabrazyme hu maħsub biex jintuża darba biss.
Reconstitution
Rikostituzzjoni
Reconstitute each vial of Fabrazyme 35 mg with 7.2 ml water for injections.
Irrikostitwixxi kull fjala ta ’ Fabrazyme 35 mg b’ 7. 2 ml ta ’ ilma għall- injezzjonijiet.
This is done by slow drop-
Evita l- impatt qawwi ta ’ l- ilma għall- injezzjonijiet fuq it- trab u evita r- ragħwa fis- soluzzjoni.
8 wise addition of the water for injection down the inside of the vial and not directly onto the lyophilized cake.
Dan isir b’ addizzjoni qatra qatra ta ’ l- ilma għall- injezzjoni mal- ġenb ġewwieni tal- kunjett u mhux direttament fuq il- kejk lajofilizzat.
Roll and tilt each vial gently.
Irrollja u mejjel il- kunjett bil- mod.
Do not invert, swirl or shake the vial.
Taqlibx, iddawwarx jew ixxengilx il- kunjett.
The reconstituted solution contains 5 mg agalsidase beta per ml, and appears as a clear colourless solution.
Il- soluzzjoni rikostitwita fiha 5 mg agalsidase beta f’ kull ml, u tidher bħala soluzzjoni ċara u bla kulur.
The pH of the reconstituted solution is approximately 7.0.
Il- pH tas- soluzzjoni rikostitwita hi madwar 7. 0.
Before further dilution, visually inspect the reconstituted solution in each vial for particulate matter and discoloration.
Qabel ma ssir aktar dilwazzjoni, iċċekkja s- soluzzjoni rikostitwita f’ kull kunjett għal frak jew tibdil fil- kulur.
Do not use the solution if foreign particles are observed or if the solution is discoloured.
M’ għandekx tuża s- soluzzjoni jekk tara xi frak jew jekk is- soluzzjoni bidlet il- kulur.
After reconstitution it is recommended to promptly dilute the vials, to minimize protein particle formation over time.
Wara r- rikostituzzjoni huwa rakkomandat li wieħed għandu jiddilwa immedjatament il- kunjetti biex jimminimizza l- formazzjoni ta ’ proteini maż- żmien.
Any unused product or waste material should be disposed of in accordance with local requirements.
5 Kull fdal tal- prodott li ma jiġix użat jew skart li jibqa ’ wara l- użu tal- prodott għandu jintrema kif jitolbu l- liġijiet lokali.
Dilution
Dilwazzjoni
Prior to adding the reconstituted volume of Fabrazyme required for the patient dose, it is recommended to remove an equal volume of 0.9% sodium chloride intravenous solution, from the infusion bag.
Qabel ma żżid il- volum rikostitwit ta ’ Fabryzyme għad- doża tal- pazjent, huwa rakkomandat li tneħħi volum ugwali ta ’ soluzzjoni għal ġol vina ta ’ 0. 9% sodium chloride, minn ġol- borża ta ’ l- infużjoni.
Remove the airspace within the infusion bag to minimize the air/ liquid interface.
Neħħi l- ispazju ta ’ l- arja minn ġol- borża ta ’ l- infużjoni biex timminimizza l- wiċċ bejn l- arja u l- likwidu.
Slowly, withdraw 7.0 ml (equal to 35 mg) of the reconstituted solution from each vial up to the total volume required for the patient dose.
Bil- mod, neħħi 7. 0 ml (ugwali għal 35 mg) ta ’ soluzzjoni rikostitwita minn kull kunjett sakemm iġġib il- volum meħtieġ biex tagħmel id- doża tal- pazjent.
Do not use filter needles and avoid foaming.
Tużax labar bil- filters sabiex tevita r- ragħwa.
Then slowly inject the reconstituted solution directly into the 0.9% sodium chloride intravenous solution (not in any remaining airspace) to a final concentration between 0.05 mg/ ml and 0.7 mg/ ml.
Imbagħad injetta bil- mod is- soluzzjoni rikostitwita fis- soluzzjoni intravenuża ta ’ 0. 9% sodium chloride (mhux f’ xi wiċċ li fadal ta ’ arja) għal konċentrazzjoni finali ta ’ bejn 0. 05 mg/ ml u 0. 7 mg/ ml.
Determine the total volume of sodium chloride 0.9% solution for infusion (between 50 and 500 ml) based on the individual dose.
Iddetermina l- volum totali ta ’ 0. 9% soluzzjoni għall- infużjoni ta ’ sodium chloride (bejn 50 u 500 ml) skond id- doża individwali.
For doses lower than 35 mg use a minimum of 50 ml, for doses 35 to 70 mg use a minimum of 100 ml, for doses 70 to 100 mg use a minimum of 250 ml and for doses greater than 100 mg use only 500 ml.
Għal dożi anqas minn 35 mg uża minimu ta ’ 50 ml, għal dożi ta ’ 35 sa 70 mg uża minimu ta ’ 100 ml, għal dożi ta ’ 70 mg sa 100 mg uża minimu ta ’ 250 mg u għal dożi akbar minn 100 mg uża biss 500 ml.
Gently invert or lightly massage the infusion bag to mix the diluted solution.
Bil- mod aqleb jew immassaġġja l- borża ta ’ l- infużjoni biex tħallat is- soluzzjoni dilwita.
Do not shake or excessively agitate the infusion bag.
M’ għandekx ixxengel jew taġita b’ mod qawwi l- borża ta ’ l- infużjoni.
It is recommended to administer the diluted solution through an in-line low protein-binding 0.2 µm filter to remove any protein particles which will not lead to any loss of agalsidase beta activity.
Huwa rakkomandat li s- soluzzjoni dilwita tingħata permezz ta ’ pajp low- protein binding b’ filter ta ’ 0. 2 µm sabiex jitneħħew il- partiċelli tal- proteini li ma jwasslux għal xi telf fl- attività ta ’ agalsidase beta.
The initial infusion rate should be no more than 0.25 mg/ min (15 mg/ hour) to minimise the potential occurrence of infusion-associated reactions.
Ir- rata inizjali ta ’ l- infużjoni m’ għandix tkun iżjed minn 0. 25 mg/ min (15 mg/ siegħa) biex tnaqqas ir- riskju ta ’ xi reazzjonijiet relatati ma ’ l- infużjoni.
After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions.
Wara li tkun stabbilita t- tolleranza tal- pazjent, ir- rata ta ’ l- infużjoni tista ’ tiżdied bil- mod meta jkun hemm infużjonijiet sussegwenti.
Genzyme Europe B. V., Gooimeer 10, NL-1411DD Naarden, The Netherlands
Genzyme Europe B. V., Gooimeer 10, NL- 1411DD Naarden, l- Olanda.
03/ 08/ 2001 Date of last renewal:
03/ 08/ 2001 Data ta ’ l- aħħar reviżjoni:
Fabrazyme 5 mg, powder for concentrate for solution for infusion.
Fabrazyme 5 mg, trab għall- konċentrat għal soluzzjoni għall- infużjoni.
Each vial of Fabrazyme contains a nominal value of 5 mg of agalsidase beta.
Kull kunjett ta ’ Fabrazyme fih valur nominali ta ’ 5 mg ta ’ agalsidase beta.
After reconstitution with 1.1 ml water for injections, each vial of Fabrazyme contains 5 mg/ ml of agalsidase beta.
Wara r- rikostituzzjoni b’ 1. 1 ml ta ’ ilma għall- injezzjonijiet, kull kunjett ta ’ Fabrazyme ikun fih 5 mg/ ml ta ’ agalsidase beta.
Powder for concentrate for solution for infusion White to off-white lyophilized cake or powder
Kejk jew trab abjad jew off- white lajofilizzat
Fabrazyme is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease (α -galactosidase A deficiency).
Fabrazyme jintuża għal terapija ta ’ sostituzzjoni ta ’ enżimi fuq perijodu ta ’ żmien twil f’ pazjenti li għandhom djanjosi konfermata għal marda Fabry (nuqqas ta ’ α- galactosidase A).
11 Paediatric patients Studies in children 0-7 years have not been performed and no dosage regimen can presently be recommended in patients in this paediatric age group as safety and efficacy have not yet been established.
12 Pazjenti pedjatriċi Ma sarux studji fuq tfal ta ’ bejn 0- 7 snin u l- ebda programm ta ’ dożi ma jista ’ attwalment jiġi rakkomandat għal pazjenti f’ dan il- grupp ta ’ età pedjatrika peress li s- sgurtà u l- effikaċja għadhom ma ġewx stabbiliti.
Patients with antibodies to r-hα GAL have a greater potential to experience infusion-associated reactions (IARs), which are defined as any related adverse event occurring on the infusion day.
Pazjenti b’ antikorpi għal r- hαGA għandhom potenzjal ogħla li jkollhom reazzjonijiet assoċjati ma ’ l- infużjoni (IAR – infusion- associated reactions), li huma mfissra bħala kull każ avvers relatat li jseħħ fil- jum ta ’ l- infużjoni.
If severe allergic or anaphylactic-type reactions occur, immediate discontinuation of the administration of Fabrazyme should be considered and appropriate treatment initiated.
Jekk ikun hemm reazzjonijiet qawwija tat- tip allerġiku jew reazzjonijiet qawwija tat- tip anafilattiku, wieħed għandu jara jekk għandux iwwaqqaf immedjatament Fabrazyme u jgħati kura kif jixraq.
4.5 Interaction with other medicinal products and other forms of interaction
13 4. 5 Prodotti mediċinali oħra li ma jaqblux ma ’ dan il- prodott u affarijiet oħra li jistgħu jibdlu l- effett farmaċewtiku tal- prodott
Fabrazyme should not be administered with chloroquine, amiodarone, benoquin or gentamycin due to a theoretical risk of inhibition of intra-cellular α -galactosidase activity.
Fabrazyme m’ għandux jingħata flimkien ma ’ chloroquine, amiodarone, benoquin jew gentamycin għax hemm il- ħsieb li dawn is- sustanzi jistgħu jimpedixxi l- attività intra- ċellulari ta ’ α- galactosidase.
12 4.6 Pregnancy and lactation
4. 6 Tqala u Treddigħ
Adverse drug reactions (ADRs) reported from clinical trials to be related to Fabrazyme administered at a dose of 1mg/ kg in a total of 168 patients (154 males and 14 females) treated with Fabrazyme for a minimum of one infusion up to a maximum of 5 years are listed by System Organ Class and frequency (very common ≥ 1/ 10; common ≥ 1/ 100 to < 1/ 10 and uncommon ≥ 1/ 1000 to < 1/ 100) in the table below.
Reazzjonijiet avversi tal- mediċina (ADRs) rapportati minn provi kliniċi li kienu relatati ma ’ Fabrazyme mogħti f’ doża ta ’ 1 mg/ kg fuq total ta ’ 168 pazjent (154 raġel u 14- il mara) ittrattati b’ Fabryzyne għal minimu ta ’ infużjoni waħda sa massimu ta ’ 5 snin huma elenkati skond is- Sistema tal- Klassifika ta ’ l- Organi u frekwenza (komuni ħafna ≥1/ 10; komuni ≥1/ 100 sa < 1/ 10 u mhux komuni ≥1/ 1000 sa < 1/ 100) fit- tabella t’ hawn taħt.
The occurrence of an ADR in a single patient is defined as uncommon in light of the relatively small number of patients treated.
L- okkorrenza ta ’ l- ADR f’ xi pazjent hi mfissra bħala mhix komuni fid- dawl ta ’ l- għadd relativament żgħir ta ’ pazjenti trattati.
Incidence of Related Adverse Events with Fabrazyme Treatment
14 Inċidenza ta ’ Kazijiet Kazijiet Avversi Relatati * bi Trattament b’ Fabrazyme
Frequency
komuni
common
Frekwenza mhix
nasopharyngitis
tal- Klassifika ta ’ l- Organi
rhinitis
ħafna
unknown
magħrufa
Immune system disorders
Infezzjonijiet u infestazzjonijiet Disturbi fis- sistema
anaphylactoid reaction
nasofarinġite
Nervous system disorders
rinite
Headache, paraesthesia
immuni
hyperaesthesia, tremor
reazzjoni anafilattojdi
Eye disorders
iperestesja, rogħda sturdament, ħedla, Uġigħ ta ’ Disturbi ipoestesja, ras, fis- sistema sensazzjoni ta ’ paraestesija nervuża ħruq, letarġija, sinkope Disturbi fl- għajnejn
auricular swelling, ear pain
nefħa awrikulari, uġigħ fil- widnejn sinus bradikarja
tachycardia, palpitations, bradycardia
takikardja, bradikardja, palpitazzjonijiet
sinus bradycardia
kesħa periferali
System Organ
Disturbi
Common
fwawar, pressjoni
Frequency
vaskulari
Class
għolja, sfurija, pressjoni baxxa, fwawar sħan dispnea,
unknown
Bronkospażmu,
musculoskeletal pain
fl- estremitajiet, uġigħ fl- għadam,
chills, pyrexia,
muskoluskeletrku
administration site conditions
15 tat- tessut konnettiv
illness, infusion site pain, infusion site reaction, injection site thrombosis, malaise, oedema
uġigħ fid- dahar, spażmi muskolari, artralġja, tagħfis fil- muskoli, ebusija muskolu- skeletriku
For the purpose of this table, ≥ 1% is defined as events occurring in 2 or more patients.
Disturbi ġenerali u kondizzjonijiet ta 'mnejn jingħata
Additional symptoms included mild or moderate dyspnoea, throat tightness, chest discomfort, flushing, pruritus, urticaria, face oedema, angioneurotic oedema, rhinitis, bronchospasm, tachypnea, wheezing, hypertension, hypotension, tachycardia, palpitations, abdominal pain, nausea, vomiting, infusion-related pain including pain at the extremities, myalgia, and headache.
Sintomi addizzjonali kienu jinkludu dispnea ħafifa jew moderata, griżmejn issikkati, skonfort fis- sider, fwawar, ħakk, urtikarja, edema tal- wiċċ, edema anġjonewrotika, rinite, bronkospażmu, takipnea, tħarħir, pressjoni għolja, pressjoni baxxa, takikardja, palpitazzjonijiet, uġigħ addominali, nawsja, rimettar, uġigħ relatat ma ’ l- infużjoni li jinkludi uġigħ fl- estremitajiet, uġigħ fl- għadam u uġigħ ta ’ ras.
14 attributed to the formation of IgG antibodies and/ or complement activation.
Il- maġġoranza ta ’ dawn ir- reazzjonijiet jistgħu jkunu attribwiti mal- formazzjoni ta ’ l- antikorpi l- IgG u/ jew attivazzjoni komplimentari.
Reduced or absent α - galactosidase activity results in the accumulation of GL-3 in the lysosomes of many cell types including the endothelial and parenchymal cells, ultimately leading to life-threatening clinical deteriorations as a result of renal, cardiac and cerebrovascular complications.
Tnaqqis jew l- assenza ta ’ l- attività α- galactosidase tirriżulta fl- akkumulazzjoni tal- GL- 3 f’ ħafna tipi ta ’ ċelluli inklużi ċelluli endotiljali u ċelluli parenkimali, li fl- aħħar mill- aħħar twassal għal deterjorazzjonijiet kliniċi li jistgħu ikunu ta ’ periklu għal ħajja bħala riżultat ta ’ komplikazzjonijiet renali, kardijaċi u ċerebrovaskulari.
The rationale for enzyme replacement therapy is to restore a level of enzymatic activity sufficient to clear the accumulating substrate in the organ tissues; thereby, preventing, stabilizing or reversing the progressive decline in function of these organs before irreversible damage has occurred.
Terapija ta ’ sostituzzjoni ta ’ l- enżima issir biex jerġa ’ jintlaħaq il- livell ta ’ attività enżimatika li jkun biżżejjed biex ineħħi s- subtrat akkumulat fit- tessuti ta ’ l- organi; b’ hekk jiġi evitat, stabilizzat jew irriversjat it- tnaqqis progressiv tal - funzjoni ta ’ dawn l- organi qabel ma ssir ħsara rriversibbli.
After intravenous infusion, agalsidase beta is rapidly removed from the circulation and taken up by vascular endothelial and parenchymal cells into lysosomes, likely through the mannose-6 phosphate, mannose and asialoglycoprotein receptors.
Wara l- infużjoni minn ġol- vina, agalsidase beta malajr jitneħħa miċ- ċirkolazzjoni u jiġi assorbit miċ- ċelluli vaskulari endotiljali u ċelluli parenkimali f’ liżożomi, x’ aktarx permezz ta ’ mannose- 6 phosphate, mannose u riċetturi ta ’ asialoglycoprotein receptors.
Efficacy and safety of Fabrazyme was evaluated in one study with children, one dose-finding study, two double-blind placebo-controlled studies, and one open-label extension study in both male and female patients.
16 Fl- effikaċja u s- sigurtà ta ’ Fabrazyme ġew evalwati fi studju wieħed fuq it- tfal, bi studju wieħed fuq id- dożi, żewġ studji double- blind bi plaċebo użati bħala kontrolli, u studju ta ’ estensjoni open label fuq pazjenti kemm irġiel u n- nisa.
In the dose finding study, the effects of 0.3, 1.0 and 3.0 mg/ kg once every 2 weeks and 1.0 and 3.0 mg/ kg once every 2 days were evaluated.
Fl- istudju dwar id- dożi l- effetti ta ’ 0. 3, 1. 0 u 3. 0 mg/ kg darba kull ġimgħatejn u 1. 0 u 3. 0 mg/ kg darba kull jumejn kienu evalwati.
Plasma GL-3 was cleared in a dose dependent manner, but was less consistent at the dose of 0.3 mg/ kg.
Ir- rata ta ’ tneħħijja ta ’ GL- 3 fil- plażma kienet tiddependi mid- doża, imma kienet inqas konsistenti meta d- doża kienet 0. 3 mg/ kg.
This finding was further supported by a statistically significant decrease in GL-3 inclusions in kidney, heart and skin combined and in the individual organs in patients treated with agalsidase beta compared to placebo patients (p < 0.001).
Dan ir- riżultat kien sostnut aktar bi tnaqqis statistikament sinjifikanti fl- ammont ta ’ GL- 3 fil- kliewi, il- qalb u l- ġilda kif ukoll fl- organi individwali ta ’ pazjenti li ngħataw agalsidase beta meta mqabbla ma ’ pazjenti li ngħataw il- plaċebo (p < 0. 001).
This was achieved in 47 of the 49 patients (96%) with available information at month 6, and in 8 of the 8 patients (100%) with available information at the end of the study (up to a total of 5 years of treatment).
Dan intlaħaq f’ 47 mid- 49pazjent (96%) b’ informazzjoni disponibbli fis- 6 xahar, u fi 8 mit- 8 pazjenti (100%) b’ informazzjoni disponibbli fl- aħħar ta ’ l- istudju (sa total ta ’ 5 snin ta ’ trattament).
Plasma GL-3 levels rapidly normalised with treatment and remained normal through 5 years.
Livelli ta ’ GL- 3 fil- plażma innormalizzaw malajr bi trattament u baqgħu normali matul il- 5 snin.
15 Although no specific study has been conducted to assess the effect on the neurological signs and symptoms, the results also indicate that patients may achieve reduced pain and enhanced quality of life upon enzyme replacement therapy.
Għalkemm ma sar ebda studju speċifiku biex jassessja l- effett fuq is- sinjali u sintomi newroloġiċi, ir- riżultati jindikaw ukoll li pazjenti jista ’ jkollhom uġigħ mnaqqas u kwalità ta ’ ħajja mtejba permezz ta ’ trattament ta ’ sostituzzjoni ta ’ l- enżimi.
Clearance of GL-3 in the superficial skin vascular endothelium was achieved in all patients who had accumulated GL-3 at baseline.
It- tneħħija ta ’ GL- 3 fl- endotilju vaskulari tal- ġilda superfiċjali intlaħaq fil- pazjenti kollha li kienu akkumulaw GL- 3 fil- punt ta ’ linja bażi.
The 2 female patients had little or no GL-3 accumulation in the superficial skin vascular endothelium at baseline, making this conclusion applicable in male patients only.
Iż- 2 pazjenti nisa ftit jew xejn kellhom akkumulazzjoni ta ’ GL- 3 fl- endotilju vaskulari tal- ġilda superfiċjali fil- punt ta ’ linja bażi, li jagħmel din il- konklużjoni applikabbli għal pazjenti rġiel biss.
Due to the limitations of the study design (small number of patients), no definitive conclusion regarding the dose maintenance regimen can be drawn, but these findings suggest that, after an initial debulking dose of 1.0 mg/ kg every 2 weeks, 0.3 mg/ kg every 2 weeks may be sufficient in some patients to maintain clearance of GL-3.
17 Minħabba l- limitazzjonijiet ta ’ kif l- istudju kien ippjanat (għadd żgħir ta ’ pazjenti), ma setgħax ikun hemm konklużjoni definittiva dwar il- programm ta ’ żamma ta ’ doża, imma dawn ir- riżultati jissuġerixxu li, wara doża inizjali ta’ 1. 0 mg/ kg kull ġimagħtejn ġiet imwaqqfa, 0. 3 mg/ kg kull ġimagħtejn tista ’ tkun biżżejjed f’ xi pazjenti biex iżommu t- tneħħija ta ’ GL- 3.
Following an intravenous administration of agalsidase beta to adults at doses of 0.3 mg, 1 mg and 3 mg/ kg body weight, the AUC values increased more than dose proportional, due to a decrease in clearance, indicating a saturated clearance.
Wara l- għoti ġol- vina ta ’ agalsidase beta lil adulti f’ dożi ta ’ 0. 3 mg, 1 mg u 3mg/ kg piż tal- ġisem, il- valuri ta ’ l- AUC żdiedu b’ rata ogħla milli kieku kienet direttament proporzjonali mad- doża mogħtija, minħabba tnaqqis fit- tneħħija, li turi li tneħħija saturata.
300 minutes and at a dose of 1 mg/ kg body weight, biweekly, mean Cmax plasma concentrations ranged from 2000 – 3500 ng/ ml, while the AUCinf ranged from 370-780 μ g. min/ ml.
300 minuta u b’ doża ta ’ 1 mg/ kg piż tal- ġisem, għal darbtejn fil- ġimgħa, il- livelli medji ta ’ Cmax fil- konċentrazzjonijiet tal- plażma varjaw bejn 2000- 3500 ng/ ml, waqt li l- AUCinf kienu bejn 370- 780 μg. min/ ml.
Agalsidase clearance was not influenced by weight in this population.
It- tneħħija ta ’ agalsidase ma kinetx influwenzata mil- piż f’ din il- popolazzjoni.
Mannitol Sodium phosphate monobasic, monohydrate Sodium phosphate dibasic, heptahydrate.
Mannitol Sodium phosphate monobasic, monohydrate Sodium phosphate dibasic, heptahydrate
6.3 Shelf life
18 6. 3 Żmien kemm idum tajjeb il- prodott mediċinali
Fabrazyme 5 mg is supplied in clear Type I glass 5 ml vials.
Fabrazyme 5 mg jiġi f’ kunjetti tal- ħġieġ ċar ta ’ Tip 1 ta ’ 5 ml.
The closure consists of a siliconised butyl stopper and an aluminium seal with a plastic flip-off cap.
L- għatu jikkonsisti minn tap tal- butyl silikonizzat b’ siġill ta ’ l- aluminju u għatu tal- plastik li tista ’ tiftħu b’ daqqa ta ’ saba ’.
The powder for concentrate for solution for infusion has to be reconstituted with water for injections, diluted with 0.9% sodium chloride intravenous solution and then administered by intravenous infusion.
It- trab tal- konċentrat għal soluzzjoni għall- infużjoni irid ikun rikostitwit bl- ilma għall- injezzjonijiet, dilwit b’ soluzzjoni għal ġol vina ta ’ 0. 9% sodium chloride u mbagħad tingħata permezz ta ’ infużjoni minn ġol- vina.
Use Aseptic Technique
Uża Teknika Asettika
Reconstitute each vial of Fabrazyme 5 mg with 1.1 ml water for injections.
Irrikostitwixxi kull fjala ta ’ Fabrazyme 5 mg b’ 1. 1 ml ta ’ ilma għall- injezzjonijiet. Evita l- impatt qawwi ta ’ l- ilma għall- injezzjonijiet fuq it- trab u evita r- ragħwa fis- soluzzjoni.
This is done by slow drop-
Dan isir b’ addizzjoni qatra qatra ta ’ l- ilma għall- injezzjoni mal- ġenb ġewwieni tal- kunjett u mhux direttament fuq il- kejk lajofilizzat.
The reconstituted solution contains 5 mg agalsidase beta per ml, and appears as a clear colourless solution.
3 Il- soluzzjoni rikostitwita fiha 5 mg agalsidase beta f’ kull ml, u tidher bħala soluzzjoni ċara u bla kulur.
Any unused product or waste material should be disposed of in accordance with local requirements.
Kull fdal tal- prodott li ma jiġix użat jew skart li jibqa wara l- użu tal- prodott għandu jintrema kif jitolbu l- liġijiet lokali.
Dilution
19 Dilwazzjoni
Slowly, withdraw 1.0 ml (equal to 5 mg) of the reconstituted solution from each vial up to the total volume required for the patient dose.
Bil- mod neħħi 1. 0 ml (ugwali għal 5 mg) ta ’ soluzzjoni rikostitwita minn kull kunjett sakemm iġġib il- volum meħtieġ biex tagħmel id- doża tal- pazjent.
After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions.
Wara li tkun stabbilita t- tolleranza tal- pazjent, ir- rata ta ’ l- infużjoni tista ’ tiżdied bil- mod meta jkun hemm infużjonijiet oħra.
Genzyme Europe B. V., Gooimeer 10, NL-1411DD Naarden, The Netherlands
Genzyme Europe B. V., Gooimeer 10, NL- 1411 DD Naarden, l- Olanda.
Detailed information on this product is available on the website of the European Medicines Agency (EMEA) http: / /www. emea. europa. eu.
Informazzjoni dettaljata dwar dan il- prodott tinsab fuq il- website ta ’ l- Aġenzija Ewropea dwar il- mediċini (EMEA) http: // www. emea. europa. eu /.
B.
1H
20 A.
20 ANNESS II
MANUFACTURER(S) OF THE BIOLOGICAL ACTIVE SUBSTANCE(S) AND MANUFACTURING AUTHORISATION HOLDER(S) RESPONSIBLE FOR BATCH RELEASE
MANIFATTURI TAS- SUSTANZA BIJOLOĠIKA ATTIVA U DETENTURI TA ’ L- AWTORIZZAZZJONI TAL- MANIFATTURA RESPONSABBLI GĦALL- HRUĠ TAL- LOTT
37 Hollands Road Haverhill Suffolk CB9 8PU United Kingdom
37 Hollands Road Haverhill Suffolk CB9 8PU Ir- Renju Unit
Summary of Product Characteristics, 4.2)
Sommarju tal- Karatteristiċi tal- Prodott, 4. 2).
PSURs should be submitted three-yearly.
PSURs għandhom jiġu sottomessi tliet darbiet fis- sena.
22 A.
22 ANNESS III
23 PARTICULARS TO APPEAR ON THE OUTER PACKAGING
TIKKETTA
OUTER CARTON, 1 VIAL, 5 VIALS, 10 VIALS
KARTUNA TA ’ BARRA, KUNJETT, 5 KUNJETTI, 10 KUNJETTI
Fabrazyme 35 mg powder for concentrate for solution for infusion agalsidase beta
Fabrazyme 35 mg, trab għall- konċentrat għal soluzzjoni għall- infużjoni agalsidase beta
One vial of powder contains 35 mg of agalsidase beta.
Kunjett ta ’ trab fih 35 mg ta ’ agalsidase beta.
Excipients: mannitol sodium phosphate monobasic, monohydrate sodium phosphate dibasic, heptahydrate.
Sustanzi mhux attivi: mannitol sodium phosphate monobasic, monohydrate sodium phosphate dibasic, heptahydrate
1 vial of powder for concentrate for solution for infusion.
Kunjett ta ’ trab għal konċentrat għal soluzzjoni għall- infużjoni.
5 vials of powder for concentrate for solution for infusion.
5 kunjetti ta ’ trab għal konċentrat għal soluzzjoni għall- infużjoni.
10 vials of powder for concentrate for solution for infusion.
10 kunjetti ta ’ trab għal konċentrat għal soluzzjoni għall- infużjoni.
EXP {month/ year}
{XX/ SSSS}
Store in a refrigerator (2 °C – 8 °C).
Aħżen fi friġġ (2°C- 8°C).
Any unused solution should be discarded.
Kull soluzzjoni mhux użata għandha tintrema.
Marketing authorisation holder:
Id- Detentur ta ’ l- Awtorizzazzjoni għat- tqegħid fis- Suq:
Gooimeer 10 NL-1411DD Naarden The Netherlands
Gooimeer 10 NL- 1411 DD Naarden l- Olanda
Batch{number}
Lott {numru}
Fabrazyme 35 mg powder for concentrate for solution for infusion agalsidase beta For intravenous use.
Fabrazyme 35 mg, trab għall- konċentrat għal soluzzjoni għall- infużjoni agalsidase beta Għal użu ġol- vina
Store in a refrigerator (2 °C – 8 °C).
Aħżen fi friġġ (2 °C – 8 °C).
26 PARTICULARS TO APPEAR ON THE OUTER PACKAGING
27 TAGĦRIF LI GĦANDU JIDHER FUQ IL- PAKKETT TA ’ BARRA
Fabrazyme 5 mg powder for concentrate for solution for infusion agalsidase beta
Fabrazyme 5 mg, trab għall- konċentrat għal soluzzjoni għall- infużjoni agalsidase beta
One vial of powder contains 5 mg of agalsidase beta.
Kunjett ta ’ trab fih 5 mg ta ’ agalsidase beta.
1 vial of powder for concentrate for solution for infusion.
Kunjett ta ’ trab għall- konċentrat għal soluzzjoni għall- infużjoni.
10 vials of powder for concentrate for solution for infusion.
5 kunjetti ta ’ trab għal konċentrat għal soluzzjoni għall- infużjoni 10 kunjetti ta ’ trab għal konċentrat għal soluzzjoni għall- infużjoni
Fabrazyme 5 mg powder for concentrate for solution for infusion agalsidase beta For intravenous use.
Fabrazyme 5 mg, trab għall- konċentrat għal soluzzjoni għall- infużjoni agalsidase beta Għal użu ġol- vina
Store in a refrigerator (2 °C – 8 °C)
Aħżen fi friġġ 2 °C – 8 °C.
30 PACKAGE LEAFLET:
30 B.
Fabrazyme 35 mg powder for concentrate for solution for infusion Agalsidase beta
Fabrazyme 35 mg trab għal konċentrat għal soluzzjoni għall- infużjoni Agalsidase beta
What Fabrazyme is and what it is used for 2.
X’ inhu Fabrazyme u għalxiex jintuża 2.
Before you use Fabrazyme 3.
Qabel ma tuża Fabrazyme 3.
How to use Fabrazyme 4.
Kif għandek tuża Fabrazyme 4.
How to store Fabrazyme 6.
Kif taħżen Fabrazyme 6.
WHAT FABRAZYME IS AND WHAT IT IS USED FOR
X’ INHU FABRAZYME U GĦALXIEX JINTUŻA
Fabrazyme is used as enzyme replacement therapy in Fabry disease, where the level of α - galactosidase enzyme activity is absent or lower than normal.
Fabrazyme jingħata bħala terapija ta ’ sostituzzjoni ta ’ enżimi fil- marda Fabry, meta l- livell ta ’ l- attività enżimatika ta ’ α- galactosidase hija nieqsa jew inqas min- normal.
If you suffer from Fabry disease a fat substance, called globotriaosylceramide (GL-3), is not removed from the cells of your body and starts to accumulate in the walls of the blood vessels of your organs.
Jekk tbati mill- marda Fabry, sustanza grassa, msejħa globotriaosylceramide (GL- 3), ma tiġix mneħħija minn ġoċ- ċelloli ta ’ ġismek u tibda takkumula fir- riti tal- vażi tad- demm ta ’ l- organi tiegħek.
Fabrazyme is indicated for use as long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease.
Fabrazyme jintuża għal terapija ta ’ sostituzzjoni ta ’ enżimi fuq perijodu ta ’ żmien twil f’ pazjenti li għandhom djanjosi konfermati għal marda Fabry.
BEFORE YOU USE FABRAZYME
QABEL MA TUŻA FABRAZYME
Do not use Fabrazyme If you have experienced an allergic anaphylactic reaction to agalsidase beta or if you are allergic (hypersensitive) to any of the other ingredients of Fabrazyme.
Tużax Fabrazyme Jekk int kellek reazzjoni allerġika anafilattika għall- agalsidase beta jew jekk int tbati minn allerġija (sensittività eċċessiva) għal kwalunkwe sustanzi oħra ta ’ Fabrazyme.
Take special care with Fabrazyme If you are treated with Fabrazyme, you may develop infusion associated reactions.
Oqgħod attent ħafna b’ Fabrazyme Jekk inti qed tiġi trattat b’ Fabrazyme, tista ’ tiżviluppa reazzjonijiet assoċjati ma ’ l- infużjoni.
An infusion- associated reaction is any side effect occurring during the infusion or until the end of the infusion day (See 4 “ Possible Side Effects”).
Reazzjoni assoċjata ma ’ l- infużjoni hija kull effett sekondarju li jista ’ jiżviluppa waqt l- infużjoni jew sa tmiem il- ġurnata ta ’ l- infużjoni (Ara 4 “ Effetti Sekondarji li Jista ’ Jkollu ”).
You may need to be given additional medicines to prevent such reactions from occurring.
Għandu mnejn ikollok bżonn tingħata mediċini addizzjonali sabiex tevita li reazzjonijiet bħal dawn iseħħu.