FrancophonIA/ELRC3.0 huggingface dataset:
English: Due to the nature of the medicinal product, the reconstituted solution may occasionally contain some proteinaceous particles in form of thin white strands or translucent fibers which will be removed by the in-line filter during infusion (see point e).
Slovenian: Zaradi narave zdravila lahko rekonstituirana raztopina občasno vsebuje proteinske delce v obliki tankih belih lis ali prosojnih vlaken, ki bodo odstranjeni z linijskim filtrom med samim infundiranjem (glejte točko e).
English: Acute renal failure was of moderate severity leading to temporary discontinuation of the study treatment and fully resolved within 3 months.
Slovenian: Akutna odpoved ledvic je bila zmerne resnosti in je povzročila začasno prekinitev zdravljenja v študiji, popolnoma pa je izzvenela v 3 mesecih.
English: Patients
Slovenian: Bolniki
English: As expected for a protein of this size, the steady-state volume of distribution was low (0.27 L/kg), indicating distribution confined to plasma.
Slovenian: Kot pričakovano za protein te velikosti, je bil volumen porazdelitve v stanju dinamičnega ravnovesja majhen (0,27 l/kg), kar kaže, da je porazdelitev omejena na plazmo.
English: Most side effects occur during the infusion or shortly after (“infusion-related reaction”, see section 2 Warnings and precautions).
Slovenian: Večinoma se neželeni učinki pojavijo med infundiranjem ali kmalu po njem („reakcija, povezana z infuzijo“, glejte poglavje 2 Opozorila in previdnostni ukrepi).
English: Paediatric population No dose adjustment is necessary for the paediatric population.
Slovenian: Pediatrična populacija Prilagajanje odmerka pri pediatrični populaciji ni potrebno.
English: A pharmacodynamic effect with statistically significant decrease of serum oligosaccharides in comparison to placebo was demonstrated.
Slovenian: Dokazan je bil farmakodinamični učinek s statistično pomembnim zmanjšanjem oligosaharidov v serumu v primerjavi s placebom.
English: Infusion-related reaction
Slovenian: Reakcija, povezana z infuzijo
English: 4.7 Effects on ability to drive and use machines
Slovenian: 4.7 Vpliv na sposobnost upravljanja vozil in strojev
English: Minimum infusion time (min)
Slovenian: Najkrajši čas infundiranja (min)
English: Overall response
Slovenian: Splošni odziv
English: rhLAMAN-10 study n=33
Slovenian: Študija rhLAMAN-10 N = 33
English: All were mild or moderate in severity and none were reported as a serious adverse event.
Slovenian: Vse so bile blage do zmerne resnosti in o nobeni niso poročali kot o resnem neželenem učinku.
English: In such a case, treatment with an antihistamine and corticosteroids should be considered as being appropriate.
Slovenian: V takem primeru je treba razmisliti o primernem zdravljenju z antihistaminiki in kortikosteroidi.
English: Do not use this medicine after the expiry date which is stated on the label and the carton after ‘EXP'.
Slovenian: Tega zdravila ne smete uporabljati po datumu izteka roka uporabnosti, ki je naveden na nalepki in na škatli poleg oznake „Uporabno do/EXP“.
English: IRRs include hypersensitivity in 3 patients and anaphylactoid reaction in 1 patient.
Slovenian: IRR vključujejo preobčutljivost pri 3 bolnikih in anafilaktoidno reakcijo pri 1 bolniku.
English: The maximum infusion rate is 25 mL/hour (see section 4.2).
Slovenian: Največja hitrost infundiranja je 25 ml/uro (glejte poglavje 4.2).
English: An integrated database was created by pooling cumulative databases from all studies with velmanase alfa.
Slovenian: Celovito zbirko podatkov so ustvarili z združevanjem skupnih zbirk podatkov iz vseh študij z velmanazo alfa.
English: *Velmanase alfa is produced in mammalian Chinese Hamster Ovary (CHO) cells using recombinant DNA technology.
Slovenian: *Velmanaza alfa se proizvaja v sesalskih ovarijskih celicah kitajskega hrčka (CHO-Chinese hamster ovary) s pomočjo tehnologije rekombinantne DNA.
English: The management of IRRs should be based on the severity of the reaction and includes slowing the infusion rate, treatment with medicinal products such as antihistamines, antipyretics and/or corticosteroids, and/or stopping and resuming treatment with increased infusion time.
Slovenian: Obvladovanje IRR mora temeljiti na resnosti reakcij in vključuje: zmanjšanje hitrosti infundiranja, zdravljenje z zdravili, kot so antihistaminiki, antipiretiki in/ali kortikosteroidi, in/ali ustavitev in nadaljevanje zdravljenja z daljšim časom infundiranja.
English: Treatment with velmanase alfa for 12 months (n=15)
Slovenian: Zdravljenje z velmanazo alfa 12 mesecev (n = 15)
English: Metabolism and nutrition disorders Increased appetite
Slovenian: Presnovne in prehranske motnje povečan apetit
English: For infusion rate see section “Method of administration”.
Slovenian: Za hitrost infundiranja glejte poglavje „Način uporabe“.
English: The most common adverse reactions observed were weight increase (18%), IRRs (9%), diarrhoea (12%), headache (9%), arthralgia (9%), increased appetite (6%) and pain in extremity (6%).
Slovenian: Najbolj pogosto opaženi neželeni učinki so bili povečanje telesne mase (18 %), IRR (9 %), driska (12 %), glavobol (9 %), artralgija (9 %), povečanje apetita (6 %) in bolečine v okončinah (6 %).
English: A post-hoc multiparametric responders analysis supports the benefit of longer treatment with velmanase alfa in 87.9% of responders in at least 2 domains at last observation (table 4).
Slovenian: Analiza post-hoc multiparametričnih odzivnih oseb podpira korist daljšega zdravljenja z velmanazo alfa pri 87,9 % odzivnih oseb na vsaj 2 področjih pri zadnjem opazovanju (preglednica 4).
English: The patient should be observed for IRRs for at least one hour after the infusion according to clinical conditions and the physician's judgment.
Slovenian: Bolnika je treba opazovati za IRR najmanj eno uro po infundiranju, v skladu s kliničnimi pogoji in po zdravnikovi presoji.
English: The effects of treatment with velmanase alfa should be periodically evaluated and discontinuation of treatment considered in cases where no clear benefits could be observed.
Slovenian: Učinke zdravljenja z velmanazo alfa je treba redno ocenjevati in razmisliti o prenehanju zdravljenja, če ni mogoče opaziti nedvomnih koristi.
English: Data suggest that the beneficial effects of the treatment with velmanase alfa diminish with the increase of disease burden and disease-related respiratory infections.
Slovenian: Podatki kažejo, da koristni učinki zdravljenja z velmanazo alfa izginjajo z večanjem bremena bolezni in okužb dihal, povezanih z boleznijo.
English: The vial should not be inverted, swirled, or shaken.
Slovenian: Viale ni dovoljeno obračati, vrteti ali stresati.
English: Nevertheless, the absorption of any ingested milk-containing velmanase alfa in the breastfed child is considered to be minimal and no untoward effects are therefore anticipated.
Slovenian: Vendar pa prevladuje mnenje, da je absorpcija zaužitega mleka, ki vsebuje velmanazo alfa, pri dojenem otroku minimalna, zato neželeni učinki niso pričakovani.
English: Proteins larger than 50,000 Da, such as velmanase alfa, are not eliminated renally.
Slovenian: Proteini, ki so večji od 50.000 Da, kot je velmanaza alfa, se ne izločajo prek ledvic.
English: The patient later experienced epileptic seizures that were considered not related.
Slovenian: Bolnik je imel pozneje epileptične napade, ki verjetno niso bili povezani.
English: From the total reconstituted volume, only 22 mL (corresponding to 44 mg) should be administered.
Slovenian: Od skupne količine za rekonstitucijo je treba aplicirati samo 22 ml (ustreza 44 mg).
English: Treatment with placebo for 12 months (n=10)
Slovenian: Zdravljenje s placebom 12 mesecev (n = 10)
English: f) When the last syringe is empty, the dosage syringe is replaced with a 20 mL syringe filled with sodium chloride 9 mg/mL (0.9%) solution for injection.
Slovenian: f) Ko je zadnja brizga prazna, se odmerno brizgo zamenja z 20-ml brizgo, napolnjeno z 9 mg/ml (0,9 %) raztopine natrijevega klorida za injiciranje.
English: 3 years.
Slovenian: 50 minut.
English: Patient's weight (44 kg) × dose (1 mg/kg) = Patient dose (44 mg) 44 mg divided by 10 mg/vial = 4.4 vials, therefore, 5 vials should be reconstituted.
Slovenian: Bolnikova telesna masa (44 kg) × odmerek (1 mg/kg) = odmerek za bolnika (44 mg) 44 mg deljeno z 10 mg/vialo = 4,4 viale, torej je treba rekonstituirati 5 vial.
English: Administration of velmanase alfa may result in an IRR, including anaphylactoid reaction (see section 4.8).
Slovenian: Uporaba velmanaze alfa lahko povzroči IRR, vključno z anafilaktoidno reakcijo (glejte poglavje 4.8).
English: - If the infusion-related reactions are severe and/or there is a loss of effect from this medicine, your doctor will perform a blood test to check for antibodies that might affect the outcome of your treatment.
Slovenian: - Če so reakcije, povezane z zdravilom, resne in/ali zdravilo izgubi svoj učinek, vam bo zdravnik opravil krvne preiskave za preverjanje prisotnosti protiteles, ki lahko vplivajo na rezultat zdravljenja.
English: As velmanase alfa aims at normalizing alpha-mannosidase in alpha-mannosidosis patients, Lamzede should be used during pregnancy only when strictly needed.
Slovenian: Ker je cilj velmanaze alfa normalizacija alfa-manozidaze pri bolnikih z alfa manozidozo, je treba zdravilo Lamzede med nosečnostjo uporabljati le, ko je nujno potrebno.
English: The patient received saline infusion in a hospital setting and was then discharged after 6-hour observation.
Slovenian: Bolnik je v bolnišnici prejel infuzijo fiziološke raztopine, nato pa je bil po 6-urnem opazovanju odpuščen.
English: Criterion
Slovenian: Kriterij
English: In the clinical studies, one patient experienced acute renal failure considered possibly related to the study treatment.
Slovenian: V kliničnih študijah je eden od bolnikov doživel akutno odpoved ledvic, ki bi lahko bila povezana z zdravljenjem v študiji.
English: Other medicines and Lamzede
Slovenian: Druga zdravila in zdravilo Lamzede
English: If severe allergic or anaphylactic-type reactions occur, immediate discontinuation of velmanase alfa is recommended and current medical standards for emergency treatment are to be followed.
Slovenian: Če pride do hude alergijske ali anafilaktične reakcije, priporočamo takojšnjo prekinitev zdravljenja z velmanazo alfa, poleg tega pa je treba upoštevati trenutne zdravstvene standarde za nujno zdravljenje.
English: For the management of adverse reactions, see sections 4.4 and 4.8.
Slovenian: Za obvladovanje neželenih učinkov glejte poglavji 4.4 in 4.8.
English: A total of 2 serious adverse reactions (loss of consciousness in 1 patient and acute renal failure in 1 patient) were observed.
Slovenian: Opazili so skupno 2 resna neželena učinka (izguba zavesti pri 1 bolniku in akutna odpoved ledvic pri 1 bolniku).
English: The safety profile of velmanase alfa in clinical studies involving children and adolescents was similar to that observed in adult patients.
Slovenian: Pediatrična populacija Varnostni profil velmanaze alfa v kliničnih študijah, ki vključujejo otroke in mladostnike, je bil podoben kot pri odraslih bolnikih.
English: If not used immediately, in-use storage times and conditions prior to use are the responsibility of the user and would normally not be longer than 24 hours at 2°C to 8°C.
Slovenian: Če ga ne porabite takoj, je za čas shranjevanja med uporabo in pogoje shranjevanja pred uporabo odgovoren uporabnik, vendar shranjevanje običajno ne sme presegati 24 ur pri temperaturi od 2 °C do 8 °C.
English: This medicine is only to be used under the supervision of a doctor experienced in the treatment of alpha-mannosidosis or other similar diseases and should only be given by a healthcare professional.
Slovenian: Zdravilo se lahko uporablja samo pod nadzorom zdravnika, ki je usposobljen za zdravljenje alfa manozidoze ali drugih podobnih bolezni in ga lahko dajejo samo zdravstveni delavci.
English: If you experience an infusion-related reaction, you must tell your doctor immediately.
Slovenian: Če doživite reakcijo, povezano z infuzijo, se morate nemudoma posvetovati z zdravnikom.
English: Use of velmanase alfa in the age group 6 to 17 years is supported by evidence from clinical studies in paediatric (19 out of 33 patients) and adult patients.
Slovenian: Uporaba velmanaze alfa v starostni skupini od 6 do 17 let je podprta z dokazi iz kliničnih študij pri pediatričnih (19 od 33 bolnikov) in odraslih bolnikih.
English: No treatment was administered.
Slovenian: Bolnika niso zdravili.
English: If the volume of the solution exceeds one syringe capacity, the required number of syringes should be prepared in order to replace the syringe quickly during the infusion.
Slovenian: Če količina raztopine presega prostornino brizge, je treba pripraviti potrebno število brizg, da omogočite hitro zamenjavo brizge med infundiranjem.
English: What Lamzede is and what it is used for What you need to know before Lamzede is used How Lamzede is used
Slovenian: Kaj je zdravilo Lamzede in za kaj ga uporabljamo Kaj morate vedeti, preden boste prejeli zdravilo Lamzede Kako uporabljati zdravilo Lamzede
English: This medicine must not be used if the reconstituted solution contains opaque particles or is discoloured.
Slovenian: Tega zdravila ne smete uporabiti, če rekonstituirana raztopina vsebuje motne delce ali če je obarvana.
English: The maximum dose of velmanase alfa in clinical studies was a single administration of 100 units/kg (approximately corresponding to 3.2 mg/kg).
Slovenian: Največji odmerek velmanaze alfa v kliničnih študijah je bil enkratni odmerek 100 enot/kg (ustreza približno 3,2 mg/kg).
English: (1) Preferred terms considered as IRR as described in the section below (2) Selected adverse reaction as described in the section below
Slovenian: (1) Prednostni izrazi za IRR, kot so opisane v poglavju spodaj
English: The maximum infusion rate is 25 mL/hour.
Slovenian: Največja hitrost infundiranja je 25 ml/uro.
English: It is given for the treatment of non-neurological symptoms of the disease.
Slovenian: Zdravilo je namenjeno zdravljenju simptomov bolezni, ki niso nevrološke narave.
English: - If the infusion-related reaction is severe, your doctor will stop the infusion immediately and start giving you appropriate medical treatment.
Slovenian: - Če je reakcija, povezana z infuzijo, resna, bo vaš zdravnik nemudoma prekinil infundiranje in vas začel zdraviti z ustreznim zdravilom.
English: In both cases the patients recovered without sequelae.
Slovenian: V obeh primerih sta bolnika okrevala brez posledic.
English: <18 years(2) ≥18 years(2)
Slovenian: < 18 let(2)
English: Consequently hepatic and renal impairment are not expected to affect the pharmacokinetic of velmanase alfa.
Slovenian: Posledično ni pričakovati, da bi jetrna in ledvična okvara vplivali na farmakokinetiko velmanaze alfa.
English: The infusion time can be calculated from the following table:
Slovenian: Hitrost infundiranja se lahko preračuna s pomočjo naslednje preglednice:
English: What Lamzede is and what it is used for
Slovenian: Kaj je zdravilo Lamzede in za kaj ga uporabljamo
English: The IRRs observed in clinical studies of velmanase alfa were characterised by a rapid onset of symptoms and were of mild to moderate severity.
Slovenian: IRR, ki so jih opazili v kliničnih študijah velmanaze alfa, so imele značilen hitri nastop simptomov in so bile po resnosti blage do zmerne.
English: Animal studies do not show evidence of impaired fertility.
Slovenian: Študije na živalih niso pokazale škodljivih vplivov na plodnost.
English: Filter needles must not be used during preparation.
Slovenian: Med pripravo ni dovoljeno uporabljati igel s filtrom.
English: Lamzede should be used during pregnancy only when medically necessary.
Slovenian: Zdravilo Lamzede je dovoljeno med nosečnostjo uporabljati le, če je to potrebno iz zdravstvenih razlogov.
English: There is no experience with patients younger than 6 years of age.
Slovenian: Pri bolnikih, mlajših od 6 let, ni izkušenj.
English: Animal studies do not indicate direct or indirect harmful effects with respect to pregnancy, embryonal/foetal development, parturition or postnatal development (see section 5.3).
Slovenian: Študije na živalih ne kažejo neposrednih ali posrednih škodljivih učinkov na nosečnost, razvoj zarodka/plodu, poroda ali postnatalnega razvoja
English: Contents of the pack and other information What Lamzede contains
Slovenian: Vsebina pakiranja in dodatne informacije Kaj vsebuje zdravilo Lamzede
English: All of these adverse reactions were non-serious.
Slovenian: Nobeden od neželenih učinkov ni bil resen.
English: What you need to know before Lamzede is used Lamzede must not be used:
Slovenian: Kaj morate vedeti, preden boste prejeli zdravilo Lamzede Zdravila Lamzede ne smete uporabljati:
English: While under treatment with Lamzede, you may experience some of the following reactions:
Slovenian: Med zdravljenjem z zdravilom Lamzede lahko dobite nekatere izmed naslednjih neželenih učinkov:
English: To further evaluate the relationship, in instances of development of severe IRRs or lack or loss of treatment effect, patients should be tested for the presence of anti-velmanase alfa antibodies.
Slovenian: Za natančnejšo oceno povezave je treba v primeru razvoja resnih IRR ali ob pomanjkanju ali izgubi učinka zdravljenja, bolnike testirati za prisotnost protiteles proti velmanazi alfa.
English: The typical manifestations of the disease include distinctive facial features, mental retardation, difficulty in controlling movements, difficulties in hearing and speaking, frequent infections, skeletal problems, muscle pain and weakness.
Slovenian: Tipični znaki bolezni vključujejo značilne obrazne poteze, duševno zaostalost, težave pri nadziranju gibov, težave s sluhom in govorom, pogoste okužbe, skeletne težave, mišične bolečine in šibkost.
English: As with other enzyme replacement therapies, velmanase alfa does not cross the blood-brain-barrier.
Slovenian: Kot pri drugih encimskih nadomestnih zdravljenjih tudi velmanaza alfa ne prehaja skozi krvno-možgansko pregrado.
English: After the end of the infusion, velmanase alfa plasma concentrations fell in a biphasic fashion with a mean terminal elimination half-life of about 30 hours.
Slovenian: Po koncu infundiranja so koncentracije velmanaze alfa v plazmi padle v dveh fazah, s povprečnim končnim razpolovnim časom izločanja približno 30 ur.
English: Overall effects of treatment were evaluated in the domains of pharmacodynamics (reduction of serum oligosaccharides), functional (three-minute stair climbing test (3MSCT), six-minute walking test (6MWT), and forced vital capacity (FVC) % predicted) and quality of life (childhood health assessment questionnaire (CHAQ) disability index (DI) and CHAQ VAS pain (visual analogue scale)).
Slovenian: Skupne učinke zdravljenja so ocenili na področju farmakodinamike (zmanjšanje oligosaharidov v serumu), funkcije (triminutna hoja po stopnicah (3MSCT- three-minute stair climbing test)), šestminutna hoja (6MWT- six-minute walking test)) in predvidena forsirana vitalna kapaciteta v % (FVC-forced vital capacity) ter kakovost življenja (indeks nezmožnosti (DI- disability index) po vprašalniku za oceno zdravstvenega stanja otrok (CHAQ-childhood health assessment questionnaire) in vizualna analogna lestvica za merjenje (VAS- visual analogue scale) bolečine po CHAQ).
English: The recommended dose regimen is 1 mg/kg of body weight administered once every week by intravenous infusion at a controlled speed.
Slovenian: Odmerjanje Priporočeni režim odmerjanja je 1 mg/kg telesne mase enkrat na teden v obliki intravenske infuzije z nadzorovano hitrostjo.
English: These reactions were non-serious and mild to moderate in intensity.
Slovenian: Te reakcije niso bile resne ter so bile po jakosti blage do zmerne.
English: In one patient, loss of consciousness considered related to the study treatment with recovery after a few seconds was reported.
Slovenian: Poročali so o enem bolniku, ki je izgubil zavest v povezavi z zdravljenjem v študiji in je okreval v nekaj sekundah.
English: b) The powder should be reconstituted in the vial by a slow drop-wise addition of the water for injections down the inside of the vial and not directly onto the lyophilised powder.
Slovenian: b) Prašek je treba rekonstituirati v viali s počasnim kapljanjem vode za injekcije vzdolž notranje stene viale in ne neposredno na liofilizirani prašek.
English: It should be considered by the treating physician that the administration of velmanase alfa does not affect the irreversible complications (i.e. skeletal deformities, disostosis multiplex, neurological manifestations and impaired cognitive function).
Slovenian: Zdravnik mora upoštevati, da dajanje velmanaze alfa ne vpliva na nepopravljive zaplete (tj. okvare okostja, gargoilizem, nevrološke manifestacije in okvarjena kognitivna funkcija).
English: Paediatric Study rhLAMAN-08.
Slovenian: Pediatrična študija rhLAMAN-08.
English: Velmanase alfa is intended to supplement or replace natural alpha-mannosidase, an enzyme that catalyses the sequential degradation of hybrid and complex high-mannose oligosaccharides in the lysosome, reducing the amount of accumulated mannose-rich oligosaccharides.
Slovenian: Velmanaza alfa je namenjena dodajanju ali nadomeščanju naravnega encima alfa-manozidaze, ki katalizira zaporedno razgradnjo hibrida in kompleksnih oligosaharidov z veliko manoze v lizosomu, z zmanjšanjem količine nakopičenih oligosaharidov, bogatih z manozo.
English: Pack sizes of 1, 5 or 10 vials per carton.
Slovenian: Velikosti pakiranj 1, 5 ali 10 vial v škatli.
English: Elderly No data are available and no relevant use in elderly patients is described.
Slovenian: Starostniki Podatkov ni na voljo in zdravilo ni namenjeno za uporabo pri starejših bolnikih.
English: Maximum infusion rate (mL/h)
Slovenian: Največja hitrost infundiranja (ml/h)
English: Based on this criteria mild to moderate patients, presenting heterogeneous severity with ability to perform endurance tests, large variability of clinical manifestations and age of onset were enrolled.
Slovenian: Na podlagi tega kriterija so bili vključeni bolniki z blago do zmerno obliko bolezni, s heterogeno resnostjo in sposobnostjo opraviti preskuse vzdržljivosti, veliko variabilnostjo klinične manifestacije in starosti ob pojavu bolezni.
English: There are no clinical data on the effects of velmanase alfa on fertility.
Slovenian: Ni kliničnih podatkov o učinkih velmanaze alfa na plodnost.
English: Instructions for reconstitution and administration
Slovenian: Navodila za rekonstitucijo in uporabo
English: d) The reconstituted solution is to be slowly withdrawn from each vial with caution to avoid foaming in the syringe.
Slovenian: d) Rekonstituirano raztopino je treba iz vsake viale povleči počasi in previdno, da preprečite penjenje v brizgi.
English: Alpha-mannosidosis disease is a rare genetic disorder caused by a lack of an enzyme named alpha-mannosidase, which is needed to break down certain sugar compounds (called ‘mannose-rich oligosaccharides') in the body.
Slovenian: Alfa manozidoza je redka genetska motnja, ki jo povzroča pomanjkanje encima, imenovanega alfa manozidaza, ki ga potrebujemo za razgradnjo nekaterih sestavin sladkorja (imenovanih oligosaharidi, bogati z manozo) v telesu.
English: Store and transport refrigerated (2°C - 8°C).
Slovenian: Zdravilo shranjujte in prevažajte na hladnem (2 °C-8 °C).
English: FVC (% of predicted) Overall
Slovenian: FVC (% predvidene)
English: A total of 33 patients (20 males and 13 females, ranging in age from 6 to 35 years) were exposed to velmanase alfa in five clinical studies.
Slovenian: Skupno 33 bolnikov (20 moških in 13 žensk z razponom starosti od 6 do 35 let) je bilo izpostavljenih velmanazi alfa v petih kliničnih študijah.
English: For the calculation of the infusion rate and the infusion time based on body weight see the table in section 6.6.
Slovenian: Za preračunavanje hitrosti infundiranja in časa infundiranja glede na telesno maso glejte preglednico v poglavju 6.6.
English: It is not known whether velmanase alfa passes into breast milk.
Slovenian: Ni znano, ali velmanaza alfa prehaja v materino mleko.