natgillin/translations-raw huggingface dataset:
3 ml
Viala (steklena) 3 ml
Elaprase 2 mg/ ml concentrate for solution for infusion.
Elaprase 2 mg/ ml, koncentrat za raztopino za infundiranje.
Each vial of 3 ml contains 6 mg of idursulfase.
Vsaka 3- mililitrska viala vsebuje 6 mg idursulfaze.
Each ml contains 2 mg of idursulfase.
Vsak mililiter vsebuje 2 mg idursulfaze.
Idursulfase is produced by recombinant DNA technology in a continuous human cell line.
Idursulfaza se proizvaja v neprekinjeni humani celični liniji s tehnologijo rekombinantne DNA.
A clear to slightly opalescent, colourless solution.
Koncentrat za raztopino za infundiranje Bistra do rahlo opalescentna, brezbarvna raztopina.
Elaprase treatment should be supervised by a physician or other healthcare professional experienced in the management of patients with MPS II disease or other inherited metabolic disorders.
Zdravljenje z zdravilom Elaprase mora nadzorovati zdravnik ali drug zdravstveni delavec z izkušnjami pri obravnavanju bolnikov z MPS II ali drugimi dednimi presnovnimi boleznimi.
Elaprase is administered at a dose of 0.5 mg/ kg body weight every week by intravenous infusion over a 3 hour period, which may be gradually reduced to 1 hour if no infusion-associated reactions are observed (see section 4.4).
Če se ne opazijo reakcije, povezane z infundiranjem, se trajanje infundiranja lahko postopno skrajša na eno uro (glejte poglavje 4. 4).
For preparation and administration instructions see section 6.6.
Navodila za pripravo in uporabo so v poglavju 6. 6.
Patients with renal or hepatic impairment There is no clinical experience in patients with renal or hepatic insufficiency.
Kliničnih izkušenj pri bolnikih z ledvično ali jetrno insuficienco ni.
See section 5.2.
Glejte poglavje 5. 2.
Elderly patients There is no clinical experience in patients over 65 years of age.
Starejši bolniki Kliničnih izkušenj pri bolnikih, starejših od 65 let, ni.
Paediatric patients The dose for children and adolescents is 0.5 mg/ kg body weight weekly.
Pediatrični bolniki Odmerek za otroke in mladostnike je 0, 5 mg/ kg telesne teže na teden.
There is no clinical experience in children under the age of 5.
Kliničnih izkušenj pri otrocih, mlajših od 5 let, ni.
Patients treated with idursulfase may develop infusion-related reactions (see section 4.8).
Pri bolnikih, ki prejemajo idursulfazo, se lahko pojavijo reakcije, povezane z infundiranjem (glejte poglavje 4. 8).
During clinical trials, the most common infusion-related reactions included cutaneous reactions (rash, pruritus, urticaria), pyrexia, headache, hypertension, and flushing.
Med kliničnimi preskušanji so bile najpogostejše reakcije, povezane z infundiranjem, kožne reakcije (izpuščaj, pruritus, urtikarija), pireksija, glavobol, hipertenzija in vročinski obliv.
Infusion-related reactions were treated or ameliorated by slowing the infusion rate, interrupting the infusion, or by administration of medicines, such as antihistamines, antipyretics, low-dose corticosteroids (prednisone and methylprednisolone), or beta-agonist nebulization.
Reakcije, povezane z infundiranjem, so zdravili ali ublažili z zmanjšanjem hitrosti infundiranja, prekinjanjem infundiranja, z uporabo zdravil, kot so antihistaminiki, antipiretiki, majhni odmerki kortikosteroidov (prednizon in metilprednizolon) ali uporabo nebulizatorja z beta- agonisti.
No patient discontinued treatment due to an infusion reaction during clinical studies.
Zaradi reakcij, povezanih z infundiranjem, med kliničnimi preskušanji niso prekinili zdravljenja pri nobenem bolniku.
Consider delaying the infusion in patients who present with an acute febrile respiratory illness.
Pri bolnikih z akutno febrilno boleznijo dihal bo mogoče treba prestaviti infundiranje.
Patients using supplemental oxygen should have this treatment readily available during infusion in the event of an infusion-related reaction.
Pri bolnikih, ki prejemajo nadomestni kisik, mora biti zaradi možnosti razvoja reakcij, povezanih z infundiranjem, tako zdravljenje na voljo ves čas infundiranja.
Patients who develop IgM or IgG antibodies are at a higher risk of infusion reactions and other adverse reactions, however, IgE antibodies have not been observed.
Pri bolnikih, pri katerih se razvijejo protitelesa IgM in IgG, je tveganje za nastanek reakcij, povezanih z infundiranjem, in drugih neželenih reakcij večje, protiteles IgE pa niso opazili.
Anaphylactoid reactions, which have the potential to be life threatening, have been observed in some patients treated with Elaprase, as with any intravenous protein product.
Pri nekaterih bolnikih, ki so prejemali zdravilo Elaprase, so kot pri drugih intravenskih beljakovinskih zdravilih opazili anafilaktoidne reakcije, ki so lahko smrtno nevarne.
Late emergent symptoms and signs of anaphylactoid reactions have been observed as long as 24 hours after an initial reaction.
Pozne simptome in znake anafilaktoidnih reakcij so opazili tudi 24 ur po prvi reakciji.
If an anaphylactoid reaction occurs the infusion should be immediately suspended and appropriate treatment and observation initiated.
Če pride do anafilaktoidne reakcije, je treba infundiranje takoj ustaviti in bolnika začeti ustrezno zdraviti ter opazovati.
Patients experiencing severe or refractory anaphylactoid reactions may require prolonged clinical monitoring.
Bolnike s hudimi ali refraktarnimi anafilaktoidnimi reakcijami bo morda treba opazovati dalj časa.
Patients who have experienced anaphylactoid reactions should be treated with caution when re-administering Elaprase.
Pri bolnikih, ki so že imeli anafilaktoidne reakcije, je pri ponovni uporabi zdravila Elaprase potrebna previdnost.
No formal drug interaction studies have been conducted with Elaprase.
Formalnih študij o medsebojnem delovanju zdravila Elaprase z drugimi zdravili niso opravili.
Based on its metabolism in cellular lysosomes, idursulfase would not be a candidate for cytochrome P450 mediated interactions.
Idursulfaza se presnavlja v celičnih lizosomih, zato ni kandidat za interakcije, ki jih povzroča citokrom P450.
Elaprase is not indicated for use in women of child-bearing potential.
Zdravilo Elaprase ni indicirano za uporabo pri ženskah v rodni dobi.
No reproductive studies in female animals have been performed.
Študij reprodukcijske toksičnosti na samicah živali niso opravili.
No effects on male fertility were seen in reproductive studies in male rats.
Vplivov na plodnost pri reprodukcijskih študijah na samcih podgan niso opazili.
Excretion of idursulfase in milk has not been studied.
Izločanja idursulfaze v materino mleko niso raziskovali.
Adverse drug reactions that were reported for the 32 patients treated with 0.5 mg/ kg Elaprase weekly in the Phase II/ III 52-week placebo-controlled study were almost all mild to moderate in severity.
Skoraj vse neželene reakcije, o katerih so poročali pri 32 bolnikih, ki so jih zdravili z 0, 5 mg/ kg zdravila Elaprase na teden v 52- tedenski, s placebom nadzorovani študiji II. / III. faze, so bile blage do zmerne.
The most common were infusion-related reactions, 202 of which were reported in 22 out of 32 patients
Med njimi so o 202 reakcijah
3 following administration of a total of 1580 infusions.
3 poročali pri 22 od 32 bolnikov, po infundiranju skupno 1580 infuzij.
In the placebo treatment group 128 infusion- related reactions were reported in 21 out of 32 patients following administration of a total of 1612 infusions.
V skupini, ki je prejemala placebo, so pri 21 od 32 bolnikov poročali o 128 reakcijah, povezanih z infundiranjem, po infundiranju skupno 1612 infuzij.
Since more than one infusion-related reaction may have occurred during any single infusion, the above numbers are likely to over estimate the true incidence of infusion reactions.
Med enim infundiranjem se lahko pojavi več kot ena reakcija, povezana z infundiranjem, zato so navedene številke verjetno večje od dejanske incidence reakcij pri infundiranju.
Related reactions in the placebo group were similar in nature and severity to those in the treated group.
V skupini, ki je prejemala placebo, sta bili narava in pogostnost reakcij podobni kot v skupini, ki je prejemala zdravilo.
The most common of these infusion-related reactions included cutaneous reactions (rash, pruritus, urticaria), pyrexia, headache, and hypertension.
Najpogostejše reakcije, povezane z infundiranjem, so bile kožne reakcije (izpuščaj, pruritus, urtikarija), pireksija, glavobol in hipertenzija.
The frequency of infusion-related reactions decreased over time with continued treatment.
Pogostnost reakcij, povezanih z infundiranjem, se je ob nadaljevanju zdravljenja sčasoma manjšala.
Adverse drug reactions are listed in the table below with information presented by system organ class and frequency.
Neželene reakcije na zdravilo so navedene v tabeli spodaj, podatki pa so prikazani po organskih sistemih in pogostnosti.
Frequency is given as very common (> 1/ 10) or common (> 1/ 100, < 1/ 10).
Pogostnost neželenih učinkov je navedena kot „ zelo pogosti “ (> 1/ 10) ali „ pogosti “ (> 1/ 100, < 1/ 10).
The occurrence of an event in a single patient is defined as common in view of the number of patients treated.
Pojav dogodka pri enem bolniku je zaradi števila sodelujočih bolnikov opredeljen kot pogost.
Adverse drug reactions were defined as treatment-emergent events with suspected causality and excluded non-serious events that were reported only once in a single patient; treatment emergent events with an excess incidence of at least 9% compared with placebo were also considered as adverse drug reactions.
Pri tem so bili izključeni neresni dogodki, o katerih so poročali pri enem bolniku le enkrat; dogodke, ki so se pojavili zaradi zdravljenja, pri katerih je bila pogostnost vsaj 9% večja kot pri placebu, so prav tako opredelili kot neželene reakcije na zdravilo.
Adverse Drug Reaction System Organ Class (Preferred Term) Nervous system disorders Eye disorders
Neželena reakcija na zdravilo Organski sistem (priporočeni izraz) Bolezni živčevja Očesne bolezni
headache dizziness, tremor
glavobol vrtoglavica, tremor
lacrimation increased
povečano solzenje
arrhythmia*, cyanosis
aritmija *, cianoza
Respiratory, thoracic mediastinal disorders
Zelo
Common: and
Pogosti:
hypertension hypotension, flushing
hipertenzija hipotenzija, vročinski oblivi
bronchospasm, pulmonary embolism*, cough, wheezing, tachypnoea, dyspnoea
bronhospazem, pljučna embolija *, kašelj, sopenje, tahipneja, dispneja
Gastrointestinal disorders
Zelo
Very
pogosti:
common Common:
Pogosti:
dyspepsia abdominal pain, nausea, diarrhoea, swollen tongue
dispepsija bolečine v trebuhu, navzeja, driska, otečen jezik
urticaria, rash, pruritus face oedema, erythema, eczema
urtikarija, izpuščaj, pruritus edem obraza, eritem, ekcem
Musculoskeletal and connective tissue disorders General disorders and
Bolezni mišično- skeletnega sistema in vezivnega tkiva
chest pain arthralgia
bolečine v prsnem košu artralgija
pyrexia, infusion site swelling oedema peripheral
pireksija, otekanje na mestu infundiranja periferni edem
* see serious adverse reactions below
* Glejte resne neželene reakcije spodaj.
Across studies, serious adverse reactions were reported in a total of 5 patients who received 0.5 mg/ kg weekly or every other week.
V vseh študijah so o resnih neželenih reakcijah poročali pri skupaj petih bolnikih, ki so prejemali 0, 5 mg/ kg na teden ali na dva tedna.
Four patients experienced a hypoxic episode during one or several
Pri štirih bolnikih je med enim ali več infundiranji prišlo do hipoksične epizode, zaradi katerih je bilo zdravljenje s kisikom nujno pri treh bolnikih z obstoječo
4 infusions, which necessitated oxygen therapy in 3 patients with severe underlying obstructive airway disease (2 with a pre-existing tracheostomy).
4 obstruktivno boleznijo dihalnih poti (dva s predhodno traheotomijo).
The most severe episode, which was associated with a short seizure, occurred in a patient who received his infusion while he had a febrile respiratory exacerbation.
Najhujša epizoda, povezana s kratkim epileptičnim napadom, se je pojavila pri bolniku, ki je prejemal infuzijo med poslabšanjem febrilne bolezni dihal.
These events did not recur with subsequent infusions using a slower infusion rate and administration of pre-infusion medicinal products, usually low-dose steroids, antihistamine, and beta-agonist nebulization.
Do teh dogodkov ni prišlo pri poznejših infundiranjih, pri katerih so zmanjšali hitrost infundiranja in uporabili predinfuzijska zdravila, ponavadi majhne odmerke steroidov, antihistaminike in nebulizator z beta- agonisti.
The fifth patient, who had pre- existing cardiopathy, was diagnosed with ventricular premature complexes and pulmonary embolism during the study.
Pri petem bolniku z obstoječo kardiopatijo so med študijo diagnosticirali prezgodnje ventrikularne komplekse in pljučno embolijo.
There have been post-marketing reports of anaphylactoid reactions.
Na voljo so postmarketinška poročila o anafilaktoidnih reakcijah.
Please see section 4.4 for further information.
Za dodatne informacije glejte poglavje 4. 4.
Across all studies, 53/ 108 patients (49%) developed anti-idursulfase IgG antibodies at some point.
V vseh študijah so se pri 53/ 108 bolnikov (49%) razvila protitelesa IgG proti idursulfazi.
Six of the IgG positive patients also tested positive for IgM antibodies, and 1 patient tested positive for IgA antibodies.
Šest bolnikov, pozitivnih na protitelesa IgG, je bilo pozitivnih tudi na protitelesa IgM, en bolnik pa je bil pozitiven tudi na protitelesa IgA.
No patient developed IgE antibodies during any study.
V nobeni izmed študij se pri nobenem bolniku niso razvila protitelesa IgE.
The overall neutralizing antibody rate was 11/ 108 patients (10%).
Nevtralizirajoča protitelesa so se razvila pri skupno 11/ 108 bolnikov (10%).
In the 52-week study, rates of seropositivity peaked by Weeks 18 to 27 and steadily declined thereafter for the remainder of this study.
V 52 - tedenski študiji je bila stopnja seropozitivnosti največja od 18. do 27. tedna, nato pa se je do konca študije enakomerno zniževala.
In general, patients who tested positive for IgG antibodies were more likely to have infusion-related events than those who did not test positive.
Na splošno je bila pri bolnikih, ki so bili pozitivni na protitelesa IgG, verjetnost za pojav reakcij, povezanih z infundiranjem, večja kot pri bolnikih, ki niso bili pozitivni.
However, overall rates of infusion-related adverse events declined over time, regardless of antibody status.
Vendar se je skupno število neželenih učinkov, povezanih z infundiranjem, sčasoma manjšalo, ne glede na prisotnost protiteles.
The reduction of urinary GAG excretion was somewhat less in patients for whom circulating anti-idursulfase antibodies were detected.
Zmanjšanje izločanja glikozaminoglikanov z urinom je bilo pri bolnikih, pri katerih so odkrili protitelesa proti idursulfazi v obtoku, nekoliko manjše.
Alimentary tract and metabolism products – enzymes, ATC code:
Farmakoterapevtska skupina: pripravki za zdravljenje bolezni prebavil in presnove – encimi, oznaka ATC:
Hunter syndrome is an X-linked disease caused by insufficient levels of the lysosomal enzyme iduronate-2-sulfatase.
Hunterjev sindrom je bolezen, povezana s kromosomom X, ki jo povzročajo nezadostne količine lizosomskega encima iduronat- 2- sulfataze.
Iduronate-2-sulfatase functions to catabolize the glycosaminoglycans (GAG) dermatan sulfate and heparan sulfate by cleavage of oligosaccharide-linked sulfate moieties.
Iduronat- 2- sulfataza katabolizira glikozaminoglikanove (GAG) dermatansulfate in heparanove sulfate s cepljenjem oligosaharidno- povezanih sulfatnih delov.
Due to the missing or defective iduronate-2-sulfatase enzyme in patients with Hunter syndrome, glycosaminoglycans progressively accumulate in the cells, leading to cellular engorgement, organomegaly, tissue destruction, and organ system dysfunction.
Zaradi popolnega ali delnega pomanjkanja encima iduronat- 2- sulfataze pri bolnikih s Hunterjevim sindromom se glikozaminoglikani progresivno kopičijo v celicah, kar povzroča povečanje celic, organomegalijo, razkroj tkiv in okvaro delovanja organskih sistemov.
Idursulfase is a purified form of the lysosomal enzyme iduronate-2-sulfatase, produced in a human cell line providing a human glycosylation profile, which is analogous to the naturally occurring enzyme.
Idursulfaza je prečiščena oblika lizosomskega encima iduronat- 2- sulfataze, izdelanega v humani celični liniji, ki omogoča humani profil glikozilacije, ki je analogen naravnemu encimu.
Treatment of Hunter syndrome patients with intravenous Elaprase provides exogenous enzyme for uptake into cellular lysosomes.
Pri zdravljenju bolnikov s Hunterjevim sindromom z zdravilom Elaprase lahko celični lizosomi privzamejo eksogeni encim.
Mannose-6-phosphate (M6P) residues on the oligosaccharide chains allow specific binding of the enzyme to the M6P receptors on the cell surface, leading to cellular internalization of the enzyme, targeting to intracellular lysosomes and subsequent catabolism of accumulated GAG.
Manoza- 6- fosfatne (M6P) molekule na oligosaharidnih verigah omogočajo specifično vezavo encima na receptorje za M6P na celični površini, zaradi česar lahko encim preide v celico, kjer prispe do lizosomov in omogoči katabolizem nakopičenih GAG.
5 A total of 108 male Hunter syndrome patients with a broad spectrum of symptoms were enrolled in two randomized, placebo-controlled clinical studies, 106 continued treatment in two open-label, extension studies.
106 jih je nadaljevalo zdravljenje v dveh dodatnih odprtih študijah.
In a 52-week, randomized, double-blind, placebo-controlled clinical study, 96 patients between the ages of 5 and 31 years received Elaprase 0.5 mg/ kg every week (n=32) or 0.5 mg/ kg every other week (n=32), or placebo (n=32).
V 52- tedenski, randomizirani, dvojno slepi, s placebom nadzorovani klinični študiji je 96 bolnikov, starih od 5 do 31 let, prejemalo zdravilo Elaprase, in sicer 0, 5 mg/ kg vsak teden (n = 32), 0, 5 mg/ kg vsak drugi teden (n = 32) ali placebo (n = 32).
The study included patients with a documented deficiency in iduronate-2- sulfatase enzyme activity, a percent predicted FVC < 80%, and a broad spectrum of disease severity.
V študiji so sodelovali bolniki z dokumentiranim pomanjkanjem dejavnosti encima iduronat- 2- sulfataze, z odstotkom pričakovane vrednosti FVK < 80% in širokim spektrom stopenj bolezni.
The primary efficacy endpoint was a two-component composite score based on the sum of the ranks of the change from baseline to the end of the study in the distance walked during six minutes (6-minute walk test or 6MWT) as a measure of endurance, and% predicted forced vital capacity (FVC) as a measure of pulmonary function.
Primarni cilj študije je bil dvokomponentni rezultat, ki je temeljil na vsoti ranga spremembe razdalje, prehojene v 6 minutah (6- minutni preizkus s hojo ali 6MWT – „ 6 minute walk test “) kot mere za vzdržljivost in odstotka pričakovane forsirane vitalne kapacitete (FVK) kot mere za delovanje pljuč.
This endpoint differed significantly from placebo for patients treated weekly (p=0.0049).
Ta končna točka se je bistveno razlikovala pri bolnikih, ki so prejemali placebo, in bolnikih, ki so enkrat tedensko prejemali zdravilo (p = 0, 0049).
52 Weeks of Treatment 0.5 mg/ kg Weekly Endpoint
52 tednov zdravljenja 0, 5 mg/ kg/ teden
Marginally Weighted (OM) Mean (SE) Idursulfase Placebo
Marginalno opazovana meja (OM) srednja vrednost (SN) Idursulfaza Placebo
Mean Treatment Difference Compared with Placebo (SE)
Vrednost P (v primerjavi s placebom)
P-value (Compared with Placebo)
placebom (SN)
Composite (6MWT and %FVC)
Sestavljeni rezultat (6MWT in% FVK)
6MWT (m)% Predicted FVC FVC Absolute Volume (cc)
6MWT (m)% pričakovana FVK Absolutna prostornina FVK (cm3)
43.3 (9.6) 8.2 (9.6) 4.2 (1.6) -0.04 (1.6) 230.0 (40.0) 50.0 (40.0)
43, 3 (9, 6) 8, 2 (9, 6) 4, 2 (1, 6) – 0, 04 (1, 6) 230, 0 (40, 0) 50, 0 (40, 0)
Urine GAG Levels (μ g GAG/ mg creatinine)
Ravni GAG v urinu (μg GAG/ mg kreatinina)
-223.3 (20.7) 52.23 (20.7)
– 223, 3 (20, 7) 52, 23 (20, 7)
-275.5 (30.1)
– 275, 5 (30, 1)
% Change in Liver Volume
% spremembe prostornine jeter
-25.7 (1.5) -0.5 (1.6)
– 25, 7 (1, 5) – 0, 5 (1, 6)
-25.2 (2.2)
– 25, 2 (2, 2)
% Change in Spleen Volume
% spremembe prostornine vranice
-25.5 (3.3) 7.7 (3.4)
– 25, 5 (3, 3) 7, 7 (3, 4)
-33.2 (4.8)
– 33, 2 (4, 8)
A total of 11 of 31 (36%) patients in the weekly treatment group versus 5 of 31 (16%) patients in the placebo group had an increase in FEV1 of at least 200 cc at or before the end of the study, indicating a dose-related improvement in airway obstruction.
V skupini, ki je zdravljenje prejemala enkrat tedensko, so pri skupno 11 od 31 (36%) bolnikov, v skupini, ki je prejemala placebo, pa pri skupno 5 od 31 (16%) na koncu študije ali pred njim opazili povečanje vrednosti FEV1 za najmanj 200 cm3, kar kaže na od odmerka odvisno izboljšanje obstrukcije dihalnih poti.
The patients in the weekly treatment group experienced a clinically significant 15% mean improvement in FEV1 at the end of the study.
Pri bolnikih, ki so zdravljenje prejemali enkrat tedensko, je bilo klinično pomembno izboljšanje srednje vrednosti FEV1 na koncu študije 15- odstotno.
Urine GAG levels were normalized below the upper limit of normal (defined as 126.6 µg GAG/ mg creatinine) in 50% of the patients receiving weekly treatment.
Ravni GAG v urinu so se pri 50% bolnikov, ki so prejemali tedensko zdravljenje, normalizirale pod zgornjo mejo normalne vrednosti (opredeljene kot 126, 6 µg GAG/ mg kreatinina).
Of the 25 patients with abnormally large livers at baseline in the weekly treatment group, 80% (20 patients) had reductions in liver volume to within the normal range by the end of the study.
6 Med 25 bolniki z nenormalno povečanimi jetri na začetku študije se je v skupini, ki je prejemala tedensko zdravljenje, pri 80% (20 bolnikih) prostornina jeter do konca študije zmanjšala do obsega normalne prostornine.
6 Of the 9 patients in the weekly treatment group with abnormally large spleens at baseline, 3 had spleen volumes that normalized by the end of the study.
Med 9 bolniki z nenormalno povečano vranico na začetku študije v skupini, ki je zdravljenje prejemala tedensko, se je do konca študije velikost vranice povrnila na normalno prostornino pri treh bolnikih.
Approximately half of the patients in the weekly treatment group (15 of 32; 47%) had left ventricular hypertrophy at baseline, defined as LVM index > 103 g/ m2.
V skupini, ki je prejemala tedensko zdravljenje, je imela na začetku študije približno polovica bolnikov (15 od 32; 47%) hipertrofijo levega prekata, opredeljeno kot indeks LVM > 103 g/ m2.
Of these 6 (40%) had normalised LVM by the end of the study.
Pri šestih bolnikih med njimi (40%) se je do konca študije vrednost LVM povrnila na normalno vrednost.
This medicinal product has been authorised under “ Exceptional Circumstances”.
Zdravilo je pridobilo dovoljenje za promet v „ izjemnih okoliščinah “.
The European Medicines Agency (EMEA) will review any new information which may become available every year and this SPC will be updated as necessary.
Evropska agencija za vrednotenje zdravil (EMEA) bo vsako leto ponovno pregledala vse nove podatke, ki bodo na voljo, in posodobila povzetek glavnih značilnosti zdravila, če bo potrebno.
Idursulfase is taken up by selective receptor-mediated mechanisms involving binding to mannose 6- phosphate receptors.
Prevzem idursulfaze poteka prek mehanizmov, ki jih uravnavajo selektivni receptorji, pri katerih se zdravilo veže na receptorje za manozo- 6- fosfat.
Pharmacokinetics was evaluated in 10 patients at Week 1 and Week 27 following administration of 0.5 mg/ kg weekly as a 3-hour infusion.
Farmakokinetiko so ocenili pri 10 bolnikih 1. in 27. teden po uporabi 0, 5 mg/ kg tedensko v obliki triurne infuzije.
There were no differences in pharmacokinetic parameters following 27 weeks of treatment.
Po 27 tednih zdravljenja ni bilo razlik v farmakokinetičnih parametrih.
Parameter Cmax (μ g/ ml)
Parameter Cmax (μg/ ml)
Week 1 (SD) 1.5 (0.6)
1.
Week 27 (SD) 1.1 (0.3)
27. teden (SD) 1, 1 (0, 3)