natgillin/translations-raw huggingface dataset:
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). 
Ta izsledek je bil podprt tudi s statistično občutnim zmanjšanjem skupnega števila inkluzij GL-3 v ledvicah, srcu in koži ter v posameznih organih bolnikov, zdravljenih z agalzidazo beta, v primerjavi s tistimi, ki so prejemali placebo (p<0,001).
In clinical trials doses up to 3 mg/kg body weight were used. 
V kliničnih preskušanjih so uporabljali odmerke do 3 mg/kg telesne mase.
Remove the airspace within the infusion bag to minimize the air/liquid interface. 
Odstranite zrak iz infuzijske vrečke, da čimbolj zmanjšate medsebojno delovanje zraka/tekočine.
In clinical trials, sixty seven percent (67 %) of the patients experienced at least one infusionassociated reaction (see section 4.8). 
V kliničnih preskušanjih je sedeminšestdeset odstotkov (67 %) bolnikov imelo vsaj eno reakcijo, povezano z infuzijo (glejte poglavje 4.8).
Plasma GL-3 was cleared in a dose dependent manner, but was less consistent at the dose of 0.3 mg/kg. 
Iz plazme se je GL-3 odstranjeval v odvisnosti od odmerka, vendar pa je bil ta učinek manj konsistenten pri odmerku 0,3 mg/kg.
In the postmarketing setting, experience was gained in patients who initiated treatment at a dose of 1 mg/kg every 2 weeks and subsequently received a reduced dose for an extended period. 
V obdobju trženja zdravila so bile pridobljene izkušnje z bolniki, ki so začeli zdravljenje z odmerkom 1 mg/kg vsake dva tedna, nato pa so prejemali zmanjšan odmerek daljše obdobje.
This result was consistent across renal, cardiac and cerebrovascular events. 
Ta rezultat se je ujemal pri ledvičnih, srčnih in cerebrovaskularnih primerih.
Before further dilution, visually inspect the reconstituted solution in each vial for particulate matter and discoloration. 
Pred nadaljnjim redčenjem vizualno preglejte rekonstituirano raztopino v vsaki viali, da ugotovite delce in obarvanje.
There is a theoretical risk of decreased agalsidase beta activity. 
Obstaja teoretično tveganje zmanjšane aktivnosti agalzidaze beta.
Patients with antibodies to r-hαGAL have a greater potential to experience infusion-associated reactions (IARs), which are defined as any related adverse event occurring on the infusion day. 
Bolniki s protitelesi proti r-hαGAL imajo večjo možnost, da bodo imeli reakcije, povezane z infuzijo, ki so opredeljene kot kakršenkoli neželeni učinek, ki se pojavi na dan infuzije.
Fabry disease is an inherited heterogeneous and multisystemic progressive disease, that affects both males and females. 
Fabryjeva bolezen Fabryjeva bolezen je dedna heterogena in multisistemska progresivna bolezen, ki prizadene tako moške kot ženske.
In addition, infusion-associated reactions were dose dependent. 
Tudi reakcije, povezane z infuzijo, so bile odvisne od odmerka.
In a limited number of patients IgE antibodies were demonstrated (see section 4.4). 
Pri omejenem številu bolnikov so dokazali protitelesa IgE (glejte poglavje 4.4).
Over time, the majority of seropositive patients in clinical trials demonstrated either a downward trend in titers (based on a ≥ 4-fold reduction in titer from the peak measurement to the last measurement) (40% of the patients), tolerised (no detectable antibodies confirmed by 2 consecutive radioimmunoprecipitation (RIP) assays) (14% of the patients) or demonstrated a plateau (35% of the patients). 
Sčasoma je večina seropozitivnih bolnikov v kliničnih preskušanjih izkazala padajoči (na osnovi ≥ 4 kratnega zmanjšanja titra od največje meritve do zadnje meritve) (40 % bolnikov) tolerirano (s pomočjo 2 zaporednih testov radioimunske percipitacije (RIP) ni bilo zaznati protiteles) (14 % bolnikov) oziroma je bil prikazan plato (35 % bolnikov).
Do not shake or excessively agitate the infusion bag. 
Infuzijske vrečke ne tresite ali pretirano pretresajte.
If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine. 
Če ste noseči ali dojite, menite, da ste noseči ali načrtujete zanositev, se posvetujtez zdravnikom ali farmacevtom, preden vzamete to zdravilo.
As with any intravenous protein medicinal product, allergic-type hypersensitivity reactions are possible. 
Kot pri vsakem intravenskem beljakovinskem zdravilu so tudi pri uporabi zdravila Fabrazyme možne alergijske preobčutljivostne reakcije.
For instructions on reconstitution and dilution of the medicinal product before administration, see section 6.6. 
Za navodila glede rekonstitucije in raztapljanja zdravila pred dajanjem, glejte poglavje 6.6.
• hyperthermia 
• hipertermija
Do not use filter needles and avoid foaming. 
Ne uporabljajte filtrirnih igel in preprečite nastajanje pene.
If you use more Fabrazyme than you should 
Če uporabite večji odmerek zdravila Fabrazyme, kot bi smeli
Pregnancy There are no adequate data from the use of agalsidase beta in pregnant women. 
Nosečnost Ni zadostnih podatkov o uporabi agalzidaze beta pri nosečnicah.
The safety and efficacy of Fabrazyme in children aged 0 to7 years have not yet been established. 
Varnost in učinkovitost zdravila Fabrazyme pri otrocih v starosti od 0 do 7 let še nista dokazani.
Do not drive or use machines if you experience dizziness, sleepiness, vertigo or fainting during or shortly after administration of Fabrazyme (see section 4). 
Ne vozite avtomobila ali upravljajte s stroji, če se pri vas pojavi omotica, zaspanost, vrtoglavica ali omedlevica med dajanjem ali v kratkem času po uporabi zdravila Fabrazyme (glejte poglavje 4 ).
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. 
V prvem kliničnem preskušanju, nadzorovanem s placebom, je bilo zdravilo Fabrazyme učinkovito pri odstranjevanju GL-3 iz ledvičnega žilnega endotelija po 20 tednih zdravljenja.
Reduced or absent αgalactosidase 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. 
Zmanjšana aktivnost ali odsotnost α-galaktozidaze povzroči kopičenje GL-3 v lizosomih številnih vrst celic, vključno z endotelijskimi in parenhimskimi celicami, kar na koncu privede do življenjsko nevarnih kliničnih poslabšanj, ki so posledica ledvičnih, srčnih ali cerebrovaskularnih zapletov.
In some patients initially treated at the recommended dose, and whose dose was later reduced for an extended period, some symptoms of Fabry disease were reported more frequently. 
Pri nekaterih bolnikih, sprva zdravljenih s priporočenim odmerkom, so se po zmanjšanju odmerka za daljše obdobje nekateri simptomi Fabryjeve bolezni pojavljali pogosteje.
If severe allergic or anaphylactic-type reactions occur, immediate discontinuation of the administration of Fabrazyme should be considered and appropriate treatment initiated. 
Če nastopijo hude alergijske ali anafilaktične reakcije, pretehtajte, ali je potrebna takojšnja ustavitev dajanja zdravila Fabrazyme in uvedite ustrezno zdravljenje.
Because there are no data available on effects in neonates exposed to agalsidase beta via breast milk, it is recommended to stop breast-feeding when Fabrazyme is used. 
Ker ni nobenih podatkov o učinkih agalzidaze beta na novorojenčke, ki so bili zdravilu izpostavljeni prek materinega mleka, priporočamo, da mati preneha dojiti med uporabo zdravila Fabrazyme.
With careful rechallenge Fabrazyme has been re-administered to all 6 patients who tested positive for IgE antibodies or had a positive skin test to Fabrazyme in a clinical trial. 
S previdnim ponovnim izzivanjem so Fabrazyme začeli znova dajati vsem 6 bolnikom, katerih testi na protitelesa IgE so bili pozitivni ali so imeli pozitivni kožni test na Fabrazyme v kliničnem preskušanju.
The diluted solution can be held for up to 24 hours at 2°C - 8°C. 
Razredčeno raztopino lahko shranjujete do 24 ur pri temperaturi 2 °C - 8 °C.
Investigations oxygen saturation decreased 
Preiskave zmanjšana nasičenost s kisikom
The reconstituted solution must be further diluted. 
Rekonstituirano raztopino morate dodatno razredčiti.
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. 
Razredčeno raztopino je priporočljivo dajati skozi 0,2 µm linijski filter, ki veže enostavne beljakovine, da odstranite vse beljakovinske delce, kar ne bo povzročilo nikakršne izgube aktivnosti agalzidaze beta.
The majority of patients developed IgG antibodies to r-hαGAL, typically within 3 months of the first infusion with Fabrazyme. 
Večina bolnikov je protitelesa IgG proti r-hαGAL začela tvoriti v 3 mesecih od prve infuzije zdravila Fabrazyme.
What Fabrazyme is and what it is used for What you need to know before you use Fabrazyme How to use Fabrazyme 
Kaj je zdravilo Fabrazyme in za kaj ga uporabljamo Kaj morate vedeti, preden boste uporabili zdravilo Fabrazyme Kako uporabljati zdravilo Fabrazyme
The risks and benefits of Fabrazyme in children aged 5 to 7 years have not yet been established and therefore no dose can be recommended for this age group. 
Tveganja in koristi zdravila Fabrazyme pri otrocih, starih od 5 do 7 let, še niso potrjena, zato za to starostno skupino ni možno priporočiti odmerka.
List of other side effects: 
Seznam ostalih neželenih učinkov:
Adverse reaction terminology is based upon the Medical Dictionary for Regulatory Activities (MedDRA) 
Terminologija neželenih učinkov temelji na medicinskem slovarju za regulatorne aktivnosti
Please contact your doctor if you would like to be treated at home. 
Posvetujte se z zdravnikom, če se želite zdraviti doma.
The educational material for healthcare professionals should include information on the following key elements: - Guidance regarding patient evaluation and selection and organisational requirements for home infusion. 
Izobraževalno gradivo za zdravstveno osebje mora vsebovati informacije o naslednjih ključnih elementih: - Smernice za ocenjevanje in izbiranje bolnikov ter organizacijske zahteve za infundiranje na domu.
Dose and infusion rate should remain constant while at home, and not be changed without supervision of a healthcare professional. 
Odmerek in hitrost infundiranja se doma ne smeta spremeniti brez dovoljenja zdravstvenega osebja.
After reconstitution it is a clear, colourless liquid, free from foreign matter. 
Po rekonstituciji postane čista, brezbarvna tekočina brez tujkov.
An infusionassociated reaction is any side effect occurring during the infusion or until the end of the infusion day (see section 4). 
Reakcija, povezana z infuzijo, je vsak neželeni učinek, do katerega pride med infundiranjem ali do konca dneva, na katerega ste prejeli infuzijo (glejte poglavje 4).
Roll and tilt each vial gently. 
Vsako vialo nežno zazibajte in nagnite.
If you forget to use Fabrazyme 
Če ste pozabili uporabiti zdravilo Fabrazyme
• increased sensitivity to pain 
• povečana občutljivost na bolečino
The safety and efficacy of Fabrazyme in patients older than 65 years have not been established and no dosage regimen can presently be recommended in these patients. 
Starostniki Varnosti in učinkovitosti zdravila Fabrazyme pri bolnikih, starejših od 65 let, še niso ugotovili in zanje trenutno ne moremo priporočiti nobenega režima odmerjanja.
If you experience a reaction like this, you should tell your doctor immediately. 
Če se pri vas pojavi takšna reakcija, nemudoma obvestite zdravnika.
What Fabrazyme is and what it is used for 
Kaj je zdravilo Fabrazyme in za kaj ga uporabljamo
The amino acid sequence of the recombinant form, as well as the nucleotide sequence which encoded it, are identical to the natural form of α-galactosidase A. 
Aminokislinsko zaporedje rekombinantne oblike, kakor tudi nukleotidno zaporedje, ki ga kodira, sta popolnoma enaka naravni obliki encima α-galaktozidaze A.
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. 
V študijah za ugotavljanje odmerka so ovrednotili učinke odmerkov 0,3, 1,0 in 3,0 mg/kg enkrat na vsaka 2 tedna in odmerkov 1,0 in 3,0 mg/kg enkrat na vsaka 2 dni.
Life threatening hypersensitivity (anaphylactic reaction) to the active substance or any of the excipients listed in section 6.1. 
Smrtno nevarna preobčutljivost (anafilaktična reakcija) na zdravilno učinkovino ali katerokoli pomožno snov, navedeno v poglavju 6.1.
Doses up to 3 mg/kg body weight have shown to be safe. 
Odmerki do 3 mg/kg telesne mase so se izkazali za varne.
Tell your doctor if you use any medicines containing chloroquine, amiodarone, benoquin or gentamicin. 
Obvestite zdravnika, če jemljete katero koli zdravilo, ki vsebuje klorokvin, amiodaron, monobenzon ali gentamicin.
Tell your doctor or pharmacist if you are taking, have recently taken or might take any other medicines. 
Obvestite zdravnika ali farmacevta, če jemljete, ste pred kratkim jemali oziroma če razmišljate o jemanju kateregakoli drugega zdravila, tudi če ste ga dobili brez recepta.
In an additional study, 21 male patients were enrolled to follow GL3 clearance in kidney and skin tissues at an alternative dosing regimen. 
V dodatni študiji je sodelovalo 21 moških bolnikov, pri katerih so opazovali očistek GL-3 v ledvicah in kožnem tkivu pri alternativnem režimu odmerjanja.
It is supplied as a powder which will be mixed with sterile water before it is given (see information for Health Care Professionals at the end of this leaflet). 
Zdravilo je v obliki praška, ki ga bodo pred infundiranjem zmešali s sterilno vodo (glejte informacije za zdravstveno osebje na koncu teh navodil).Pri uporabi tega zdravila natančno upoštevajte navodila zdravnika.
Agalsidase beta is a recombinant form of human α-galactosidase A and is produced by recombinant DNA technology using a mammalian Chinese Hamster Ovary (CHO) cell culture. 
Agalzidaza beta je rekombinantna oblika človeškega encima α-galaktozidaze A, ki ga izdelujejo s tehnologijo rekombinantne DNA v sesalskih celičnih kulturah ovarijskih celic kitajskega hrčka
Hepatic impairment Studies in patients with hepatic insufficiency have not been performed. 
Jetrna insuficienca Študije pri bolnikih z jetrno insuficienco še niso bile opravljene.
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. 
Po intravenski aplikaciji agalzidaze beta pri odraslih v odmerkih 0,3 mg, 1 mg in 3 mg/kg telesne mase so se vrednosti AUC povečale več kot sorazmerno odmerku zaradi zmanjšanja očistka, kar kaže na zasičenje očistka.
In some of these patients, an increase of some of the following symptoms was spontaneously reported: pain, paraesthesia and diarrhoea, as well as cardiac, central nervous system and renal manifestations. 
Pri nekaterih od teh bolnikov so spontano poročali o povečanju nekaterih od naslednjih simptomov: bolečine, parestezija in driska ter kardiološke, centralnoživčne in ledvične manisfestacije.
The marketing authorisation holder (MAH) shall agree the details of the educational programme for Fabrazyme home infusion with the National Competent Authorities prior to implementing the programme nationally. 
Imetnik dovoljenja za promet z zdravilom se bo z nacionalnimi pristojnimi organi dogovoril o podrobnem izobraževalnem programu o infundiranju zdravila Fabrazyme doma pred nacionalno uvedbo programa.
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. 
Reakcije, povezane z infuzijo, so zdravili z zmanjšanjem hitrosti infundiranja in sočasno uporabo nesteroidnih protivnetnih zdravil, antihistaminikov in/ali kortikosteroidov.
Reconstituted and diluted solutions 
Rekonstituirana in razredčena raztopina
The decision to have a patient move to home infusion should be made after evaluation and recommendation by the treating physician. 
Odločitev o prehodu na infuzije doma mora temeljiti na presoji in priporočilu lečečega zdravnika.
Cardiac Disorders tachycardia, palpitations, bradycardia sinus bradycardia -- 
Srčne bolezni tahikardija, palpitacije, bradikardija sinusna bradikardija
Avoid forceful impact of the water for injections on the powder and avoid foaming. 
Preprečite močan curek vode za injekcije na prašek in nastajanje pene.
Genzyme Europe B.V.-NL Store in a refrigerator (2°C - 8°C). 
Genzyme Europe B.V.-NL Shranjujte v hladilniku (2 °C - 8 °C).
After reconstitution it is recommended to promptly dilute the vials, to minimise protein particle formation over time. 
Priporočljivo je, da viale razredčite takoj po rekonstituciji, da čimbolj zmanjšate tvorbo beljakovinskih delcev čez čas.
If not used immediately, in-use storage and conditions prior to use are the responsibility of the user. 
Če ni uporabljeno takoj, je za shranjevanje med uporabo in pogoje pred uporabo odgovoren uporabnik.
A small number of patients have experienced reactions suggestive of immediate (Type I) hypersensitivity. 
Manjše število bolnikov je imelo reakcije, ki nakazujejo na takojšnjo preobčutljivost (vrste 1).
Fabrazyme should not be used during pregnancy unless clearly necessary. 
Zdravila Fabrazyme ne smete uporabljati med nosečnostjo, razen če je nujno potrebno.
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). 
Potrebne viale vzemite iz hladilnika, da se lahko segrejejo na sobno temperaturo (po približno 30 minutah).
Ten (10) patients met per protocol dose increase criteria, two (2) had a dose increase to the recommended dose of 1.0 mg/kg every 2 weeks. 
Deset (10) bolnikov je izpolnilo po protokolu določena merila za povečanje odmerka in dvema (2) so odmerek povečali na priporočeni odmerek 1,0 mg/kg vsaka 2 tedna.
After IgG seroconversion, there was no apparent change in CL (+24% and +6%, resp.), while Vss was 1.8 and 2.2 fold higher, with the net effect being a small decrease in Cmax (up to -34% and -11%, resp.) and no change in AUC (-19% and6%, resp.). 
Po serokonverziji IgG se CL ni opazno spremenil (pri prvih +24 %, pri drugih +6 %), medtem ko je bil Vss pri prvih 1,8-krat in pri drugih 2,2-krat večji; končni učinek je bilo majhno zmanjšanje Cmax (do -34 % pri prvih in -11 % pri drugih), AUC pa se ni spremenila (-19 % pri prvih in -6 % pri drugih).
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. 
Zaradi omejitev zasnove študije (majhno število bolnikov), dokončnih sklepov v zvezi z režimom vzdrževalnega odmerjanja ni možno oblikovati, vendar pa izsledki kažejo, da morda po začetnem odstranitvenem odmerku 1,0 mg/kg vsaka 2 tedna, odmerek 0,3 mg/kg vsaka 2 tedna pri nekaterih bolnikih zadošča za vzdrževanje očistka GL-3.
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. 
Po intravenskem infundiranju se agalzidaza beta hitro odstrani iz krvnega obtoka, ker jo absorbirajo celice žilnega endotelija in parenhimske celice v svoje lizosome, verjetno prek manoza-6-fosfatnih, manoznih in asialoglikoproteinskih receptorjev.
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. 
Vss je bil od 8,3-40,8 l, plazemski očistek od 119-345 ml/min in srednji razpolovni čas izločanja od 80
What you need to know before you use Fabrazyme Do not use Fabrazyme 
Kaj morate vedeti, preden boste uporabili zdravilo Fabrazyme Ne uporabljajte zdravila Fabrazyme
Package sizes: 1, 5 and 10 vials per carton. 
Velikost pakiranja: 1, 5 in 10 vial na škatlo.
Fabrazyme is given through a drip into a vein (by intravenous infusion). 
Zdravilo Fabrazyme se daje s kapalno infuzijo v veno (v obliki intravenske infuzije).
Fabrazyme should not be administered with chloroquine, amiodarone, benoquin or gentamycin due to a theoretical risk of inhibition of intra-cellular α-galactosidase A activity. 
Zdravila Fabrazyme ne smete uporabljati sočasno s klorokvinom, amiodaronom, benokvinom ali gentamicinom zaradi teoretičnega tveganja za inhibicijo znotrajcelične aktivnosti α-galaktozidaze A.
Contents of the pack and other information What Fabrazyme contains 
Vsebina pakiranja in dodatne informacije Kaj vsebuje zdravilo Fabrazyme
• decreased mouth sensitivity 
• zmanjšana občutljivost v ustih
Lower dosing regimens have been used in clinical studies. 
V kliničnih študijah so uporabljali režime odmerjanja z nižjim odmerkom.
Fabrazyme is only used under the supervision of a doctor who is knowledgeable in the treatment of Fabry disease. 
Zdravilo Fabrazyme se uporablja le pod nadzorom zdravnika, ki se spozna na zdravljenje Fabryjeve bolezni.
Sustained clearance of GL-3 from kidney vascular endothelium upon agalsidase beta treatment was demonstrated further in the open label extension of this trial. 
Trajen očistek GL-3 iz žilnega endotelija ledvic po zdravljenju z agalzidazo beta so nadalje dokazali v razširitvi te študije odprtega tipa.
• exacerbated difficulty in breathing 
• poslabšanje težav z dihanjem
Name and address of the manufacturer of the biological active substance 
Ime in naslov proizvajalca biološke zdravilne učinkovine
Common (may affect up to 1 in 10 people): • chest pain 
Pogosti (se lahko pojavijo pri največ 1 bolniku od 10 bolnikov): • bolečine v prsih
Agalsidase beta may be excreted in milk. 
Agalzidaza beta se lahko izloča v materino mleko.
Other medicines and Fabrazyme 
Druga zdravila in zdravilo Fabrazyme
Pharmacotherapeutic group: Other alimentary tract and metabolism products, enzymes. 
Farmakoterapevtska skupina: Druga zdravila za bolezni prebavil in presnove, encimi.
Fabrazyme may have a minor influence on the ability to drive or use machines on the day of Fabrazyme administration because dizziness, somnolence, vertigo and syncope may occur (see section 
Zdravilo Fabrazyme lahko ima blag vpliv na sposobnost vožnje in upravljanja s stroji na dan dajanja zdravila Fabrazyme, ker se lahko pojavijo omotica, zaspanost, vrtoglavica in sinkopa (glejte poglavje 4.8).
The recommended dose of Fabrazyme for children and adolescents 8 - 16 years is 1 mg/kg body weight, once every 2 weeks. 
Priporočeni odmerek zdravila Fabrazyme za otroke in mladostnike od 8 do 16 let je 1 mg/kg telesne mase, enkrat na vsaka 2 tedna.
In clinical studies side effects were mainly seen while patients were being given the medicine or shortly after (“infusion related reactions”). 
Neželeni učinki so bili v kliničnih študijah vidni med infundiranjem ali kmalu po končanem infundiranju zdravila („reakcije, povezane z infuzijo“).
In one of these studies, performed in adult male patients, after an initial dose of 1.0 mg/kg every 2 weeks for 6 months, 0.3 mg/kg every 2 weeks may maintain clearance of GL-3 in certain cell types in some patients; however, the long term clinical relevance of these findings has not been established (see section 5.1). 
V eni od študij, ki je bila narejena pri odraslih bolnikih moškega spola, lahko po začetnem odmerku 1,0 mg/kg vsaka 2 tedna v trajanju 6 mesecev, 0,3 mg/kg vsaka 2 tedna vzdržuje očistek GL-3 pri nekaterih vrstah celic in pri nekaterih bolnikih; vendar pa dolgoročne klinične pomembnosti teh izsledkov niso ugotovili (glejte poglavje 5.1).