natgillin/translations-raw huggingface dataset:
Study LA37-1111 was conducted to evaluate the effect of single therapeutic (33 mg/kg) and supratherapeutic (50 mg/kg) oral doses of deferiprone on the cardiac QT interval duration in healthy subjects.
Ispitivanje LA37-1111 provedeno je kako bi se ocijenio učinak jedne terapijske (33 mg/kg) i supraterapijske (50 mg/kg) oralne doze deferiprona na trajanje QT intervala srca kod zdravih ispitanika.
Ferriprox was concluded to produce no significant prolongation of the QT interval.
Zaključeno je da Ferriprox ne uzrokuje značajno produljenje QT intervala.
Dose per kilogram body weight should be calculated to the nearest 2.5 ml.
Dozu po kilogramu tjelesne težine treba izračunati do najbliže doze koja se postiže primjenom 2,5 ml.
The most common adverse reactions reported during therapy with deferiprone in clinical trials were nausea, vomiting, abdominal pain, and chromaturia, which were reported in more than 10% of patients.
Najčešće nuspojave prijavljene tijekom terapije deferipronom u kliničkim ispitivanjima bile su mučnina, povraćanje, bol u abdomenu i kromaturija, a prijavljene su u više od 10% bolesnika.
If treatment is unavoidable, breast-feeding must be stopped (see section 4.3).
Ako se liječenje na može izbjeći, dojenje se mora prekinuti (vidjeti dio 4.3).
No significant effect of renal impairment was seen on systemic exposure to deferiprone.
Nije primijećen značajan učinak oštećenja bubrega na sistemsku izloženost deferipronu.
The use of combination therapy should be considered on a case-by-case basis.
Primjena kombinirane terapije treba se uzeti u obzir nakon procjene svakog pojedinog slučaja.
The usual dose is 25 mg/kg, 3 times per day, for a total daily dose of 75 mg/kg.
Uobičajena doza je 25 mg/kg, 3 puta na dan, do ukupne dnevne doze od 75 mg/kg.
Provide protective isolation and if clinically indicated, admit patient to the hospital.
Osigurajte zaštitnu izolaciju bolesnika i ako je to klinički indicirano,
Therefore, it is not recommended to concomitantly ingest aluminium-based antacids and deferiprone.
Stoga se ne preporučuje uzimati istodobno antacide na bazi aluminija i deferipron.
A fatal outcome was observed in 8.3% of the reported episodes of agranulocytosis from clinical trials and post-marketing experience.
Smrtni ishod dogodio se u 8,3% epizoda agranulocitoze prijavljenih u kliničkim ispitivanjima i nakon stavljanja lijeka u promet.
There are no reports of acute overdose with Ferriprox.
Nema prijava o akutnom predoziranju Ferriproxom.
Based on the reported adverse interaction that can occur between deferoxamine and vitamin C, caution should be used when administering deferiprone and vitamin C concurrently.
Na temelju prijavljenih neželjenih interakcija koje mogu nastati između deferoksamina i vitamina C, potreban je oprez kod istodobne primjene deferiprona i vitamina C.
No effects on fertility or early embryonic development were noted in non-iron-loaded male and female rats that received deferiprone orally at doses of up to 75 mg/kg twice daily for 28 days (males) or 2 weeks (females) prior to mating and until termination (males) or through early gestation (females).
Nisu uočeni učinci na plodnost ili rani embrionalni razvoj u željezom ne opterećenih mužjaka i ženki štakora koji su primali deferipron peroralno u dozama do 75 mg/kg dvaput dnevno kroz 28 dana (mužjaci) odnosno 2 tjedna (ženke) prije parenja, te do kraja parenja (mužjaci) odnosno tijekom rane gestacije (ženke).
Study LA12-9907 compared survival, incidence of cardiac disease, and progression of cardiac disease in 129 patients with thalassaemia major treated for at least 4 years with Ferriprox (N=54) or deferoxamine (N=75).
Ispitivanje LA12-9907 uspoređivalo je preživljavanje, incidenciju bolesti srca i progresiju bolesti srca u 129 bolesnika s talasemijom major koji su liječeni najmanje 4 godine Ferriproxom (n=54) ili deferoksaminom (n=75).
Systemic exposure to deferiprone and to its metabolite deferiprone 3-O-glucuronide was assessed by the PK parameters Cmax and AUC.
Sistemska izloženost deferipronu i njegovu metabolitu deferipron 3-O-glukuronidu ocijenjena je pomoću farmakokinetičkih parametara Cmax i AUC.
Doses of Ferriprox in those studies ranged from 50 to 100 mg/kg/day and doses of deferoxamine from 40 to 60 mg/kg/day.
Doze Ferriproxa u tim ispitivanjima bile su u rasponu od 50 do 100 mg/kg/dan, a doze deferoksamina od 40 do 60 mg/kg/dan.
An increase in MRI T2* on treatment indicates that iron is being removed from the heart.
Povećanje MR T2* vrijednosti tijekom liječenja ukazuju da se željezo uklanja iz srca.
Since deferiprone binds to metallic cations, the potential exists for interactions between deferiprone and trivalent cation-dependent medicinal products such as aluminium-based antacids.
Ipak, budući da se deferipron veže za katione metala, postoji mogućnost interakcija između deferiprona i lijekova koji ovise o prisutnosti trivalentnih kationa, poput antacida na bazi aluminija.
• To increase patient awareness of the significance of any symptoms of infection while taking deferiprone
• podizanje razine osviještenosti bolesnika o značaju bilo kojeg simptoma infekcije tijekom liječenja deferipronomom
The incidences of adverse reactions experienced by 18 children and 97 adults treated with combination therapy were not significantly different between the two age groups except in the incidence of arthropathy (11.1% in children vs. none in adults, p=0.02).
Incidencije nuspojava koje su se pojavile u 18 djece i 97 odraslih osoba liječenih kombiniranom terapijom nisu bile značajno različite između dvije dobne skupine osim incidencije artropatije (11,1% kod djece naspram niti jednog slučaja kod odraslih, p=0,02).
Dose adjustments when used with other iron chelators In patients for whom monotherapy is inadequate, Ferriprox may be used with deferoxamine at the standard dose (75 mg/kg/day) but should not exceed 100 mg/kg/day.
Prilagodbe doza kada se koristi s drugim kelatorima željeza Kod bolesnika kod kojih je monoterapija neodgovarajuća, Ferriprox se može koristiti s deferoksaminom u standardnoj dozi (75 mg/kg/dan) ali doza ne bi smjela prijeći 100 mg/kg/dan.
The safety profile of combination therapy (deferiprone and deferoxamine) observed in clinical trials, post-marketing experience or published literature was consistent with that characterized for monotherapy.
Sigurnosni profil kombinirane terapije (deferipron i deferoksamin) koji je opažen u kliničkim ispitivanjima, nakon stavljanja lijeka u promet ili u objavljenoj literaturi bio je u skladu s onim koji je opisan za monoterapiju.
What you need to know before you take Ferriprox
Što morate znati prije nego uzmete Ferriprox
The effect of Ferriprox in decreasing the body iron is directly influenced by the dose and the degree of iron overload.
Učinak Ferriproxa na snižavanje razine željeza u tijelu izravno je ovisan o dozi i stupnju preopterećenja željezom.
Low plasma zinc levels have been associated with deferiprone in a minority of patients.
Niske razine cinka u plazmi povezane su s deferipronom u manjem broju bolesnika.
The influence of severe hepatic impairment on the pharmacokinetics of deferiprone and deferiprone 3-O-glucuronide has not been evaluated.
Učinak teškog oštećenja funkcije jetre na farmakokinetiku deferiprona i deferipron 3-O-glukuronida nije procijenjen.
Carcinogenicity/mutagenicity
Karcinogenost/mutagenost
Episodes of hypotonia, instability, inability to walk, and hypertonia with inability of limb movement, have been reported in children in the post-marketing setting with standard doses of deferiprone.
Epizode hipotonije, nestabilnosti, nemogućnosti hodanja te hipertonije s nemogućnošću pokretanja udova prijavljene su kod djece u periodu nakon stavljanja lijeka u promet sa standardnim dozama deferiprona.
• Whenever the risk management system is modified, especially as a result of new information being received that may lead to a significant change to the benefit/risk profile or as the result of an important (pharmacovigilance or risk minimisation) milestone being reached.
• prilikom svake izmjene sustava za upravljanje rizikom, a naročito kada je ta izmjena rezultat primitka novih informacija koje mogu voditi ka značajnim izmjenama omjera korist/rizik,
Patients should be informed that their urine may show a reddish/brown discoloration due to the excretion of the iron-deferiprone complex.
Bolesnike treba informirati da njihova mokraća može promijeniti boju u crvenkasto/smeđu zbog izlučivanja kompleksa željezo-deferipron.
Pharmacotherapeutic group: Iron chelating agents, ATC code: V03AC02
Farmakoterapijska skupina: lijekovi za kelaciju željeza, ATK oznaka: V03AC02 Mehanizam djelovanja
Immediately interrupt deferiprone if the patient experience infection.
Odmah prekinite primjenu deferiprona ako je bolesnik dobio infekciju.
Ferriprox and deferoxamine were equivalent in promoting a net stabilization or reduction of body iron load, despite the continuous transfusional iron administration in those patients (no difference in proportion of patients with a negative trend in serum ferritin between the two treatment groups by regression analysis; p >0.05).
Ferriprox i deferoksamin bili su jednaki u poboljšanju stabilizacije ili redukcije opterećenja tijela željezom unatoč kontinuiranoj primjeni željeza u transfuzijama u ovih bolesnika (nije bilo razlike u udjelu bolesnika s negativnim trendom feritina u serumu između dviju liječenih skupina dobivene regresijskom analizom; p >0,05).
There was an improvement in cardiac T2* of more than 3 ms in patients treated with Ferriprox compared with a change of about 1 ms in patients treated with deferoxamine.
Srčana vrijednost T2* poboljšala se za više od 3 ms u bolesnika liječenih Ferriproxom u usporedbi s promjenom od oko 1 ms u bolesnika liječenih deferoksaminom.
Studies LA16-0102, LA-01 and LA08-9701 compared the efficacy of Ferriprox with that of deferoxamine in controlling serum ferritin in transfusion-dependent thalassaemia patients.
Ispitivanja LA16-0102, LA-01 i LA08-9701 uspoređivala su djelotvornost Ferriproxa s deferoksaminom za kontrolu feritina u serumu u bolesnika s talasemijom ovisnom o transfuziji.
If you experience nausea or vomiting, it may help to take your Ferriprox with some food.
Ako osjetite mučninu ili povraćate, od pomoći može biti uzimati Ferriprox s nešto hrane.
In these patients careful monitoring of liver histology is recommended.
ovih se bolesnika preporučuje pažljivo praćenje histoloških promjena u jetri.
The change from weekly ANC monitoring to at the time of transfusion visits after 12 months of Ferriprox therapy, should be considered on an individual patient basis, according to the physician's assessment of the patient's understanding of the risk minimization measures required during therapy (see section 4.4 below).
Promjenu od tjednog praćenja ABN-a na praćenje ABN-a tijekom posjeta radi transfuzije, nakon što je prošlo razdoblje od 12 mjeseci liječenja Ferriproxom, treba razmotriti za svakog bolesnika pojedinačno na temelju liječnikove ocjene bolesnikovog razumijevanja mjera minimizacije rizika koje je potrebno provoditi tijekom liječenja (vidjeti dio 4.4 ispod).
Fertility No effects on fertility or early embryonic development were noted in animals (see section 5.3).
Nisu primijećeni učinci na plodnost ili rani embrionalni razvoj u životinja (vidjeti dio 5.3).
After starting Ferriprox therapy, it is recommended that serum ferritin concentrations, or other indicators of body iron load, be monitored every two to three months to assess the long-term effectiveness of the chelation regimen in controlling the body iron load.
Nakon započinjanja terapije Ferriproxom preporučuje se praćenje koncentracija feritina u serumu, ili drugih pokazatelja opterećenja tijela željezom, svaka dva do tri mjeseca, radi procjene dugotrajne učinkovitosti režima kelacije u svrhu kontrole opterećenja tijela željezom.
BOTTLE OF 50 TABLETS
BOCA OD 50 TABLETA
− increase in liver enzymes
Pretrage povišeni jetreni enzimi
Data from the published literature on iron balance studies in patients with thalassaemia major show that the use of Ferriprox concurrently with deferoxamine (coadministration of both chelators during the same day, either simultaneously or sequentially, e.g., Ferriprox during the day and deferoxamine during the night), promotes greater iron excretion than either drug alone.
Podaci iz objavljene literature o ispitivanjima ravnoteže željeza kod bolesnika s talasemijom major pokazuju da primjena Ferriproxa istodobno s deferoksaminom (istodobna primjena oba kelatora istog dana, bilo simultano ili jedan iza drugog, npr. Ferriprox tijekom dana a deferoksamin tijekom noći) potiče veće izlučivanje željeza nego bilo koji od lijekova primijenjen samostalno.
Should any evidence of infection develop concurrently with the neutropenia, the appropriate cultures and diagnostic procedures should be performed and an appropriate therapeutic regimen instituted.
Ako se istodobno s neutropenijom razviju znakovi postojanja infekcije, potrebno je učiniti odgovarajuće mikrobiološke pretrage i dijagnostičke postupke, te uspostaviti odgovarajući terapijski režim.
The higher rate of “Cardiac disorders" reported during combination therapy than monotherapy was possibly due to the higher incidence of pre-existing cardiac disorders in patients who received combination therapy.
Veća stopa "srčanih poremećaja" prijavljenih za vrijeme kombinirane terapije nego za vrijeme monoterapije je možda uzrokovana većom incidencijom postojećih srčanih poremećaja kod bolesnika koji su primali kombiniranu terapiju.
No prenatal and postnatal reproductive studies have been conducted in animals.
Nisu provedena prenatalna i postnatalna reproduktivna ispitivanja na životinjama.
MONITORING YOUR WHITE BLOOD CELL COUNT WITH FERRIPROX
PRATITE BROJ SVOJIH BIJELIH KRVNIH STANICA DOK UZIMATE FERRIPROX
A serious adverse event of acute liver and renal injury was seen in one subject with moderate hepatic impairment.
Ozbiljan štetan događaj akutnog oštećenja jetre i bubrega primijećen je u jednog ispitanika s umjerenim oštećenjem funkcije jetre.
Peak serum concentrations of the glucuronide occur 2 to 3 hours after administration of deferiprone.
Vršne koncentracije glukuronida u serumu nastupaju 2 do 3 sata nakon primjene deferiprona.
Combination therapy with deferoxamine is not recommended when monotherapy with either chelator is adequate or when serum ferritin falls below 500 µg/l.
Kombinirana terapija s deferoksaminom ne preporučuje se ako je monoterapija s jednim od kelatora odgovarajuća ili ako serumski feritin padne ispod 500 µg/l.
DATE OF FIRST AUTHORISATION/RENEWAL OF THE AUTHORISATION Date of first authorisation: 25 August 1999
Datum prvog odobrenja: 25. kolovoza 1999.
Obtain a complete blood cell (CBC) count, with a white blood cell (WBC) count, corrected for the presence of nucleated red blood cells, a neutrophil count, and a platelet count immediately upon diagnosing the event and then repeat daily.
Učinite kompletnu krvnu sliku (KKS), s brojem leukocita, korigiranim za prisutnost eritrocita s jezgrom, brojem neutrofila i brojem trombocita odmah nakon dijagnosticiranja događaja, a zatim je ponavljajte svakog dana.
In the event of agranulocytosis, rechallenge is contraindicated.
U slučaju agranulocitoze ponovna primjena lijeka je kontraindicirana.
The most common findings in non-iron-loaded animals at doses of 100 mg/kg/day and above were hematologic effects such as bone marrow hypocellularity, and decreased WBC, RBC and/or platelet counts in peripheral blood.
Najčešći nalazi u željezom ne opterećenih životinja pri dozama od 100 mg/kg/dan i više bili su hematološki učinci poput hipocelularnosti koštane srži i pada broja leukocita, eritrocita i/ili trombocita u perifernoj krvi.
Data from the published literature are consistent with the results from the Apotex studies, demonstrating less heart disease and/or increased survival in Ferriprox-treated patients than in those treated with deferoxamine.
Podaci objavljeni u literaturi sukladni su rezultatima iz Apotex ispitivanja, pokazujući manje bolesti srca i/ili povećano preživljenje bolesnika liječenih Ferriproxom nego onih liječenih deferoksaminom.
− if you have a history of repeated episodes of neutropenia (low white blood cell (neutrophil)
− ako ste u povijesti bolesti imali ponavljane epizode neutropenije (sniženi broj bijelih krvnih stanica (neutrofila)).
Common side effects (may affect up to 1 in 10 people): - low white blood cell count (agranulocytosis and neutropenia) - headache - diarrhoea - increase in liver enzymes - fatigue - increase in appetite
Česte nuspojave (mogu se pojaviti kod do 1 na 10 ljudi): - nizak broj leukocita (agranulocitoza i neutropenija) - glavobolja - proljev - porast jetrenih enzima - umor
The MAH should provide a patient/carer reminder card in each pack, the text of which is included in the Package Leaflet.
Nositelj odobrenja u svakom pakiranju mora osigurati karticu s podsjetnikom za bolesnika/njegovatelja, čiji tekst je uključen i u uputu o lijeku.
Of patients with cardiac dysfunction at first assessment, none treated with deferiprone compared with four (33%) treated with deferoxamine had worsening of their cardiac status (p=0.245).
Od bolesnika s disfunkcijom srca na prvoj procjeni kod niti jednog koji je liječen deferipronom, u usporedbi s četiri (33%) liječenih deferoksaminom, nije imalo pogoršanje njegovog srčanog statusa (p=0,245).
It is packaged in bottles of 250 ml or 500 ml.
Pakirana je u bocama od 250 ml ili
Fatalities and life-threatening situations (caused by agranulocytosis) have been reported with deferiprone in combination with deferoxamine.
Prijavljeni su smrtni slučajevi i situacije opasne po život (uzrokovane agranulocitozom) kada se deferipron koristio u kombinaciji s deferoksaminom.
If you forget to take Ferriprox
Ako ste zaboravili uzeti Ferriprox
There is a small chance that you may develop agranulocytosis (very low white blood cell count) while taking Ferriprox, which may lead to a serious infection.
Postoji mala mogućnost da se kod Vas, dok uzimate Ferriprox, razvije agranulocitoza (vrlo nizak broj bijelih krvnih stanica), što može dovesti do ozbiljne infekcije.
Following randomization, 32 patients received deferoxamine (34.9 mg/kg/day for 5 days/week) and Ferriprox (75 mg/kg/day) and 33 patients received deferoxamine monotherapy (43.4 mg/kg/day for 5 days/week).
32 bolesnika primala su deferoksamin (34,9 mg/kg/dan tijekom 5 dana u tjednu) i Ferriprox (75 mg/kg/dan), a 33 bolesnika primala su monoterapiju deferoksaminom (43,4 mg/kg/dan tijekom 5 dana u tjednu).
BOTTLE OF 100 TABLETS
BOCA OD 100 TABLETA
The incidences of “Musculoskeletal and connective tissue disorders” and "Renal and urinary disorders" were lower during combination therapy than monotherapy, whereas the incidence of “Cardiac disorders" was higher during combination therapy than monotherapy.
Incidencije "poremećaja mišićno-koštanog sustava i vezivnog tkiva" te "poremećaja bubrega i mokraćnog sustava" bile su manje za vrijeme kombinirane terapije nego za vrijeme monoterapije, dok je incidencija "srčanih poremećaja" bila veća za vrijeme kombinirane terapije nego za vrijeme monoterapije.
This condition, known as severe neutropenia or agranulocytosis, has occurred in 1 to 2 out of 100 people who have taken Ferriprox in clinical studies.
Ovo stanje, poznato kao teška neutropenija ili agranulocitoza, javilo se u 1 do 2 na 100 ljudi koji su uzimali Ferriprox u kliničkim ispitivanjima.
Ferriprox can be taken with or without food; however, you may find it easier to remember to take Ferriprox if you take it with your meals.
Ferriprox se može uzimati s hranom ili bez nje; međutim, možda će Vam ipak biti lakše zapamtiti da uzmete Ferriprox ako ga uzimate s obrocima.
Each pack contains one bottle of 250 ml or 500 ml oral solution.
Svako pakovanje sadrži jednu bocu od 250 ml ili 500 ml oralne otopine.
Iron overload causes concentration-dependent MRI T2* signal loss, thus, increased myocardial iron reduces myocardial MRI T2* values.
Preopterećenje željezom uzrokuje gubitak signala MR T2* ovisnog o koncentraciji, stoga povišeno željezo u miokardu smanjuje MR T2* vrijednosti miokarda.
Subjects were categorized into 3 groups based on the Child-Pugh classification score: healthy volunteers, mild hepatic impairment (Class A: 5- 6 points), and moderate hepatic impairment (Class B: 7- 9 points).
Ispitanici su, na temelju bodova prema Child-Pugh klasifikaciji, bili podijeljeni u 3 skupine: zdravi dobrovoljci, ispitanici s blagim oštećenjem funkcije jetre (stadij A: 5 - 6 bodova) i ispitanici s umjerenim oštećenjem funkcije jetre (stadij B: 7 - 9 bodova).
Episodes of diarrhoea, mostly mild and transient, have been reported in patients treated with deferiprone.
Epizode proljeva, većinom blagog i prolaznog, prijavljene su u bolesnika liječenih deferipronom.
Deferiprone was teratogenic and embryotoxic in reproductive studies in non-iron-loaded pregnant rats and rabbits at doses at least as low as 25 mg/kg/day.
U reproduktivnim ispitivanjima deferipron je bio teratogen i embriotoksičan u željezom ne opterećenih skotnih ženki štakora i kunića u dozama niskim najmanje od 25 mg/kg/dan.
Data from the pooled safety database from clinical trials (1343 patient-years exposure to Ferriprox monotherapy and 244 patient-years exposure to Ferriprox and deferoxamine) showed statistically significant (p<0.05) differences in the incidence of adverse reactions based on System Organ Class for “Cardiac disorders", "Musculoskeletal and connective tissue disorders” and "Renal and urinary disorders".
Podaci iz objedinjene baze podataka o sigurnosti primjene dobivenih iz kliničkih ispitivanja (1343 bolesnik-godina izloženosti monoterapiji Ferriproxom i 244 bolesnik-godina izloženosti Ferriproxu i deferoksaminu) pokazali su statistički značajne (p<0,05) razlike u incidenciji nuspojava koje se temelje na klasifikaciji organskih sustava za "srčane poremećaje", "poremećaje mišićnokoštanog sustava i vezivnog tkiva" te "poremećaja bubrega i mokraćnog sustava".
Ferriprox is packaged in bottles of 50 tablets.
Ferriprox je pakiran u bocama od 50 tableta.
Deferiprone is usually given as 25 mg/kg body weight, orally, three times a day for a total daily dose of 75 mg/kg body weight.
Doziranje Deferipron se obično daje u dozi od 25 mg/kg tjelesne težine, peroralno, tri puta na dan, do ukupne dnevne doze od 75 mg/kg tjelesne težine.
A randomized, placebo-controlled, double-blind trial evaluated the effect of concurrent therapy with Ferriprox and deferoxamine in patients with thalassaemia major, who previously received the standard chelation monotherapy with subcutaneous deferoxamine and had mild to moderate cardiac iron loading (myocardial T2* from 8 to 20 ms).
Randomizirano, placebom kontrolirano, dvostruko slijepo ispitivanje procijenilo je učinak istodobne terapije s Ferriproxom i deferoksaminom kod bolesnika s talasemijom major, koji su prethodno primali standardnu kelacijsku monoterapiju sa supkutano primijenjenim deferoksaminom i imali su blago do umjereno srčano opterećenje željezom (T2* miokarda od 8 do 20 ms).
− the most serious side effect that may occur while taking Ferriprox is a very low white blood cell (neutrophil) count.
− najozbiljnija nuspojava koje se može dogoditi dok uzimate Ferriprox je vrlo nizak broj bijelih krvnih stanica (neutrofila).
Ferriprox is packaged in bottles of 100 tablets.
Ferriprox je pakiran u bocama od 100 tableta.
Renal or hepatic impairment and liver fibrosis
Oštećenje funkcije bubrega ili jetre i fibroza jetre
PHARMACEUTICAL FORM AND CONTENTS 100 film-coated tablets
OBLIK I SADRŽAJ 100 filmom obloženih tableta
Study LA16-0102 compared the efficacy of Ferriprox with that of deferoxamine in decreasing cardiac iron overload and in improving cardiac function (as measured by LVEF) in transfusion-dependent thalassaemia patients.
Ispitivanje LA16-0102 uspoređivalo je djelotvornost Ferriproxa s onom deferoksamina u smanjivanju opterećenja srca željezom i poboljšanju funkcije srca (mjerenjem LVEF) u bolesnika s talasemijom ovisnom o transfuziji.