{"id":5280,"date":"2024-10-10T16:05:47","date_gmt":"2024-10-10T14:05:47","guid":{"rendered":"https:\/\/www.ehc.eu\/bleeding-disorder\/nedostatak-faktora-ix-hemofilija-b\/"},"modified":"2025-12-23T09:57:56","modified_gmt":"2025-12-23T08:57:56","slug":"nedostatak-faktora-ix-hemofilija-b","status":"publish","type":"bleeding-disorder","link":"https:\/\/www.ehc.eu\/hr\/bleeding-disorder\/nedostatak-faktora-ix-hemofilija-b\/","title":{"rendered":"Nedostatak faktora IX (hemofilija B)"},"content":{"rendered":"<!-- hero -->\t<section   class=\"subpage-hero bg-red text-white\">\n\t\t<div class=\"container\">\n\t\t\t<div class=\"relative z-10\">\n\t\t\t\t<div class=\"pt-16 lg:pt-24 pb-24 lg:pb-32\">\n\t\t\t\t\t<div class=\"row justify-between gap-10 lg:gap-0 mt-10 text-center lg:text-left\">\n\t\t\t\t\t\t<div class=\"w-full lg:w-6\/12 lg:self-center\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"title h2\" data-scroll data-fade-up>\n\t\t\t\t\t\t\t\t\t<p class=\"text-[115%]\">Nedostatak faktora IX (hemofilija B)<\/p>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"content mt-14 lg:mt-20\" data-scroll data-fade-up data-delay-02>\n\t\t\t\t\t\t\t\t\t<p>Rijedak hematolo\u0161ki poreme\u0107aj karakteriziran spontanim ili produljenim krvarenjem zbog nedostatka faktora IX.<\/p>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t\t\t\t\t<figure class=\"absolute size-[82.8rem] bottom-[-30%] right-[-5%] image-contain opacity-20 skip-lazy\" data-parallax>\n\t                <img decoding=\"async\" loading=\"eager\" src=\"https:\/\/etngbd86n6b.exactdn.com\/wp-content\/themes\/cbd\/img\/content-logo-colorfull.svg\" alt=\"content-logo-colorfull\">\n\t            <\/figure>\n\t\t\t\t\t\t<\/section>\n<!-- overview -->\t<section   class=\"s-overlay-img s-padding\">\n\t\t<div class=\"container\">\n\t\t\t<div class=\"row w-full lg:flex-row-reverse justify-between text-center lg:text-left relative z-10\">\n\t\t\t\t\t\t\t\t\t<div class=\"w-full lg:w-6\/12 min-h-full\">\n\t\t\t\t\t\t<div class=\"w-full h-[35rem] lg:h-[25vmax] min-h-full relative overflow-hidden radius-20\">\n\t\t\t\t\t\t\t<figure class=\"absolute top-0 left-0 size-full radius-20\" data-img-animation>\n\t\t\t\t\t\t\t\t<img width=\"410\" height=\"491\" src=\"https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1\" class=\"attachment-large size-large\" alt=\"\" decoding=\"async\" loading=\"lazy\" srcset=\"https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1 410w, https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7-251x300.jpg?strip=all&amp;sharp=1 251w, https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1&amp;w=82 82w, https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1&amp;w=164 164w, https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1&amp;w=328 328w\" sizes=\"auto, (max-width: 410px) 100vw, 410px\" \/>\t\t\t\t\t\t\t<\/figure>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t<div class=\"w-full lg:w-6\/12 lg:py-10\">\n\t\t\t\t\t<div class=\"lg:w-[120%] bg-white py-12 px-0 lg:py-24 lg:px-24 relative radius-20 z-10\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"content mt-8\" data-scroll data-fade-up>\n\t\t\t\t\t\t\t\t<p class=\"fs-5 orpha-blue\"><strong>Epidemiologija<\/strong><br \/>\n Prevalencija se procjenjuje na oko 1 na 30 000 mu\u0161karaca. Hemofilija prvenstveno poga\u0111a mu\u0161karce, ali opisan je i simptomatski oblik hemofilije B kod \u017eena nositeljica s op\u0107enito bla\u017eom klini\u010dkom slikom. <\/p>\n<p><strong>Klini\u010dki opis<\/strong><br \/>\n Op\u0107enito, anomalije krvarenja javljaju se kada oboljela dojen\u010dad po\u010dne u\u010diti hodati. Me\u0111utim, novoro\u0111en\u010dad s hemofilijom izlo\u017eena je riziku od intrakranijalnog ili ekstrakranijalnog krvarenja i drugih komplikacija krvarenja. Ozbiljnost klini\u010dkih manifestacija ovisi o stupnju nedostatka faktora IX, i kod mu\u0161karaca i kod \u017eena. Ako je biolo\u0161ka aktivnost faktora IX ispod 1 IU\/dL, hemofilija je te\u0161ka i manifestira se kao \u010desta spontana krvarenja i abnormalna krvarenja kao posljedica manjih ozljeda ili nakon traume, operacije ili va\u0111enja zuba (te\u0161ka hemofilija B). Ako je biolo\u0161ka aktivnost faktora IX izme\u0111u 1 i 5 IU\/dL, hemofilija je umjereno te\u0161ka s abnormalnim krvarenjem kao posljedicom manjih ozljeda ili nakon traume, operacije ili va\u0111enja zuba, ali spontano krvarenje je rijetko (umjereno te\u0161ka hemofilija B). Ako je biolo\u0161ka aktivnost faktora IX izme\u0111u 5 i 40 IU\/dL, hemofilija je blaga s abnormalnim krvarenjem kao posljedicom manjih ozljeda ili nakon traume, operacije ili va\u0111enja zuba, ali spontano krvarenje se ne javlja (blaga hemofilija B). Krvarenje se naj\u010de\u0161\u0107e javlja u zglobovima (hemartroze) i mi\u0161i\u0107ima (hematomi), ali bilo koje mjesto mo\u017ee biti zahva\u0107eno nakon traume ili ozljede. Spontana hematurija je \u010dest i vrlo karakteristi\u010dan znak poreme\u0107aja.       <\/p>\n<p><strong>Etiologija<\/strong><br \/>\n Hemofiliju B uzrokuju mutacije u genu <i>F9<\/i> (Xq27) koji kodira koagulacijski faktor IX.<\/p>\n<p><strong>Dijagnosti\u010dke metode<\/strong><br \/>\n Dijagnoza se postavlja na temelju koagulacijskih testova koji pokazuju produljeno vrijeme zgru\u0161avanja krvi (aktivirano parcijalno tromboplastinsko vrijeme &#8211; aPTT) i mo\u017ee se potvrditi specifi\u010dnim mjerenjima aktivnosti faktora IX i razine antigena.<\/p>\n<p><strong>Diferencijalna dijagnoza<\/strong><br \/>\n Diferencijalna dijagnoza treba uklju\u010divati \u200b\u200bhemofiliju A, von Willebrandovu bolest i druge anomalije koagulacije koje dovode do produljenog vremena zgru\u0161avanja krvi.<\/p>\n<p><strong>Prenatalna dijagnoza<\/strong><br \/>\n Prenatalna dijagnoza provedena na korionskim resicama ili amniocitima je brza i informativna kada je poznata obiteljska, uzro\u010dna mutacija <i>F9<\/i> . Poznavanje obiteljskog statusa mutacije <i>F9<\/i> u fetusu omogu\u0107uje pripremu za porod i rano medicinsko zbrinjavanje novoro\u0111en\u010deta. <\/p>\n<p><strong>Genetsko savjetovanje<br \/>\n<\/strong> Naslje\u0111ivanje je X-vezano recesivno i preporu\u010duje se genetsko savjetovanje za pogo\u0111ene obitelji. Za \u017eensku nositeljicu postoji 50%-tni rizik da \u0107e mu\u0161ko potomstvo biti pogo\u0111eno i 50%-tni rizik da \u0107e svako \u017eensko potomstvo biti nositelji. \u017denske nositeljice mogu pokazivati \u200b\u200bblage do umjerene simptome. Sveukupno, postoji 25%-tni rizik za svaku trudno\u0107u da \u0107e dijete biti mu\u0161ko potomstvo s hemofilijom i 25%-tni rizik da \u0107e dijete biti heterozigotno \u017eensko potomstvo.   <\/p>\n<p><strong>Lije\u010denje i upravljanje<br \/>\n<\/strong> Lije\u010denje pru\u017eaju multidisciplinarni centri za sveobuhvatnu skrb o hemofiliji. Nadomjesna terapija koja se sastoji od primjene nedostaju\u0107eg faktora IX (koncentrati faktora IX dobiveni iz plazme ili rekombinantni koncentrati faktora IX) uobi\u010dajeni je pristup lije\u010denju. Nedavno su bioin\u017eenjerski proizvodi faktora IX s produljenim polu\u017eivotom odobreni za lije\u010denje hemofilije B i zna\u010dajno pobolj\u0161avaju kvalitetu \u017eivota pacijenata. Lije\u010denje se mo\u017ee primijeniti nakon krvarenja (lije\u010denje po potrebi) ili za sprje\u010davanje krvarenja (profilakti\u010dko lije\u010denje). Najozbiljnije komplikacije su stvaranje inhibitornih antitijela protiv primijenjenog faktora koagulacije i anafilakti\u010dki \u0161ok kao odgovor na terapiju faktorom IX. Kirur\u0161ke intervencije, posebno ortopedske, mogu se provoditi, ali ih treba provoditi u specijaliziranim centrima.     <\/p>\n<p><strong>Prognoza<br \/>\n<\/strong> Ako se ne lije\u010di, tijek bolesti je te\u017eak i kod te\u0161ke hemofilije B op\u0107enito je fatalan. Nedovoljno ili neto\u010dno lije\u010denje rekurentnih hemartroza i hematoma dovodi do tjelesnog o\u0161te\u0107enja s te\u0161kim invaliditetom povezanim s uko\u010deno\u0161\u0107u, deformacijom zglobova i tjelesnim invaliditetom. Me\u0111utim, trenutni pristupi lije\u010denju (rana profilaksa) sprje\u010davaju ove komplikacije i prognoza je povoljna. Krvarenje, HIV i HCV infekcije te bolesti jetre vode\u0107i su uzroci smrti kod pacijenata s hemofilijom.   <\/p>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/section>\n<!-- features --><section   class=\"s-padding-bottom\">\n\t<div class=\"container\">\n\t\t\t\t\t<div class=\"grid sm:grid-cols-2 lg:grid-cols-4 gap-10 text-center lg:text-left\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div data-scroll data-fade-left data-delay-0>\n\t\t\t\t\t\t<div class=\"flex flex-col gap-7 min-h-full px-10 lg:px-16 py-14 lg:py-24 text-white bg-blue radius-20 z-10\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"title h4\">\n\t\t\t\t\t\t\t\t\t<p>Normalan<br> Raspon<\/p>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t\t\t\t\t\t\t<p>Postotak normalne aktivnosti faktora u krvi<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"h5\">\n\t\t\t\t\t\t\t\t\t\t\t<p>50%-150%<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t\t\t\t\t\t\t<p>Broj me\u0111unarodnih jedinica (IU) po mililitru (ml) pune krvi<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"h5\">\n\t\t\t\t\t\t\t\t\t\t\t<p>0,50\u20131,5 IU<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div data-scroll data-fade-left data-delay-02>\n\t\t\t\t\t\t<div class=\"flex flex-col gap-7 min-h-full px-10 lg:px-16 py-14 lg:py-24 text-white bg-blue radius-20 z-10\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"title h4\">\n\t\t\t\t\t\t\t\t\t<p>Blago<br> Hemofilija<\/p>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t\t\t\t\t\t\t<p>Postotak normalne aktivnosti faktora u krvi<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"h5\">\n\t\t\t\t\t\t\t\t\t\t\t<p>5%-40%<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t\t\t\t\t\t\t<p>Broj me\u0111unarodnih jedinica (IU) po mililitru (ml) pune krvi<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"h5\">\n\t\t\t\t\t\t\t\t\t\t\t<p>0,050\u20130,40 IU<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div data-scroll data-fade-left data-delay-04>\n\t\t\t\t\t\t<div class=\"flex flex-col gap-7 min-h-full px-10 lg:px-16 py-14 lg:py-24 text-white bg-blue radius-20 z-10\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"title h4\">\n\t\t\t\t\t\t\t\t\t<p>Umjereno<br> Hemofilija<\/p>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t\t\t\t\t\t\t<p>Postotak normalne aktivnosti faktora u krvi<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"h5\">\n\t\t\t\t\t\t\t\t\t\t\t<p>1%-5%<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t\t\t\t\t\t\t<p>Broj me\u0111unarodnih jedinica (IU) po mililitru (ml) pune krvi<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"h5\">\n\t\t\t\t\t\t\t\t\t\t\t<p>0,01\u20130,05 IU<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div data-scroll data-fade-left data-delay-06>\n\t\t\t\t\t\t<div class=\"flex flex-col gap-7 min-h-full px-10 lg:px-16 py-14 lg:py-24 text-white bg-blue radius-20 z-10\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"title h4\">\n\t\t\t\t\t\t\t\t\t<p>Te\u0161ko<br> Hemofilija<\/p>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t\t\t\t\t\t\t<p>Postotak normalne aktivnosti faktora u krvi<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"h5\">\n\t\t\t\t\t\t\t\t\t\t\t<p>manje od 1%<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t\t\t\t\t\t\t<p>Broj me\u0111unarodnih jedinica (IU) po mililitru (ml) pune krvi<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"h5\">\n\t\t\t\t\t\t\t\t\t\t\t<p>manje od 0,01 IU<\/p>\n\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t<\/div>\n<\/section><!-- overview -->\t<section   class=\"s-overlay-img s-padding-medium\">\n\t\t<div class=\"container\">\n\t\t\t<div class=\"row w-full lg:flex-row justify-between text-center lg:text-left relative z-10\">\n\t\t\t\t\t\t\t\t\t<div class=\"w-full lg:w-4\/12 min-h-full\">\n\t\t\t\t\t\t<div class=\"w-full lg:w-[115%] h-[35rem] lg:h-[25vmax] min-h-full relative overflow-hidden radius-20\">\n\t\t\t\t\t\t\t<figure class=\"absolute top-0 left-0 size-full radius-20\" data-img-animation>\n\t\t\t\t\t\t\t\t<img width=\"410\" height=\"491\" src=\"https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1\" class=\"attachment-large size-large\" alt=\"\" decoding=\"async\" loading=\"lazy\" srcset=\"https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1 410w, https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7-251x300.jpg?strip=all&amp;sharp=1 251w, https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1&amp;w=82 82w, https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1&amp;w=164 164w, https:\/\/etngbd86n6b.exactdn.com\/wp-content\/uploads\/2024\/10\/image-7.jpg?strip=all&amp;sharp=1&amp;w=328 328w\" sizes=\"auto, (max-width: 410px) 100vw, 410px\" \/>\t\t\t\t\t\t\t<\/figure>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t<div class=\"w-full lg:w-8\/12 lg:py-10\">\n\t\t\t\t\t<div class=\"bg-white py-12 px-0 lg:py-24 lg:px-32 relative radius-20 z-10\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"content mt-8\" data-scroll data-fade-up>\n\t\t\t\t\t\t\t\t<p>Hemofilija zna\u010di da tijelo pojedinca ne mo\u017ee prirodno zaustaviti krvarenje koje nastaje nakon o\u0161te\u0107enja krvne \u017eile. Ta krvarenja mogu biti uzrokovana i traumom i stresom. Ako se krvarenje ne zaustavi i ne lije\u010di pravilno, mo\u017ee dovesti do o\u0161te\u0107enja zglobova (poput gle\u017enjeva, kukova i koljena), \u0161to mo\u017ee osakatiti pojedinca (do krajnjih granica, postoji rizik od gubitka udova poput nogu i ruku) i ograni\u010diti opseg pokreta. Nadalje, intrakranijalna krvarenja mogu biti i iscrpljuju\u0107a i fatalna. Posljednjih godina jo\u0161 uvijek bilje\u017eimo izvje\u0161\u0107a o smrtnim slu\u010dajevima u Europi zbog nelije\u010denih intrakranijalnih krvarenja.    <\/p>\n<p>Va\u017eno je razumjeti da su krvarenja, osim \u0161to su iscrpljuju\u0107a i opasna po \u017eivot, tako\u0111er izuzetno bolna. Zato osobe s hemofilijom trebaju imati pristup odgovaraju\u0107em lije\u010denju boli. <\/p>\n<p>Hemofilija ima ve\u0107i utjecaj na osobe pogo\u0111ene njezinim te\u0161kim oblikom. Ova populacija pacijenata treba stalan pristup lije\u010denju i specijaliziranim zdravstvenim uslugama. Osobe s umjerenom i blagom hemofilijom mogu trebati lije\u010denje samo ako se ozlijede ili ako su izlo\u017eene velikom stresu. Tako\u0111er im mogu biti potrebne specijalizirane usluge za stomatolo\u0161ku skrb i druge vrste kirur\u0161kih zahvata, no op\u0107enito nisu toliko pogo\u0111ene poreme\u0107ajem kao osobe s te\u0161kom hemofilijom i mogu \u017eivjeti prili\u010dno normalnim \u017eivotom.   <\/p>\n<p>\u017divot s te\u0161kom hemofilijom zna\u010di da osobe s ovim stanjem trebaju infuzijom davati svoj lijek nekoliko puta tjedno kada su na profilaksi. Nedostaju\u0107i faktor primjenjuje intravenozno ili lije\u010dnik ili sam pacijent. Iako je u ve\u0107ini europskih zemalja ku\u0107no lije\u010denje mogu\u0107e (to zna\u010di da pojedinci mogu infuzijom davati svoj lijek bez nadzora lije\u010dnika), to je i dalje dugotrajna aktivnost koja mo\u017ee utjecati na sposobnost pojedinca da putuje, radi i sudjeluje u dru\u0161tvenim aktivnostima.  <\/p>\n<p>Kona\u010dno, va\u017eno je znati da \u0107e trenutno otprilike 30 posto populacije s hemofilijom razviti inhibitor za lije\u010denje. To zna\u010di da \u0107e tijelo odbaciti lije\u010denje, ostavljaju\u0107i hemofili\u010dara bez ikakvih rje\u0161enja za lije\u010denje. Za daljnje informacije o tome, molimo pogledajte odjeljak o inhibitorima.  <\/p>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/section>\n<!-- overview -->\t<section   class=\"s-overlay-img s-padding bg-red\">\n\t\t<div class=\"container\">\n\t\t\t<div class=\"row w-full lg:flex-row justify-between text-center lg:text-left relative z-10\">\n\t\t\t\t\t\t\t\t<div class=\"w-full\">\n\t\t\t\t\t<div class=\"bg-white py-12 px-10 lg:py-44 lg:px-24 xl:px-44 relative radius-20 z-10\">\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"title h4\" data-scroll data-fade-up>\n\t\t\t\t\t\t\t\t<p>Inhibitori<\/p>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<div class=\"content mt-8\" data-scroll data-fade-up>\n\t\t\t\t\t\t\t\t<p><span style=\"font-weight: 400;\">Osobe s poreme\u0107ajima krvarenja lije\u010de se takozvanom nadomjesnom terapijom. To zna\u010di da se nedostaju\u0107i faktor zgru\u0161avanja redovito ubrizgava u njihovo tijelo kako bi se proces zgru\u0161avanja mogao normalno odvijati. Na\u017ealost, neki ljudi razvijaju antitijela, nazvana inhibitori, koja neutraliziraju nadomjesni faktor zgru\u0161avanja.  <\/span><\/p>\n<p><span style=\"font-weight: 400;\">To zna\u010di da tijelo izaziva imunolo\u0161ki odgovor na lije\u010denje i stoga sprje\u010dava normalno funkcioniranje infuziranog faktora zgru\u0161avanja. U tom slu\u010daju, krvarenja je vrlo te\u0161ko kontrolirati i mogu dovesti do trajnog o\u0161te\u0107enja zglobova ili mi\u0161i\u0107a, \u0161to osobe s inhibitorima \u010dini invalidnima. <\/span><\/p>\n<p><span style=\"font-weight: 400;\">Lije\u010denje inhibitora danas je najve\u0107i izazov u lije\u010denju hemofilije. Mogu\u0107e je ukloniti inhibitore tehnikom koja se naziva indukcija imunolo\u0161ke tolerancije (ITT). Me\u0111utim, ova vrsta lije\u010denja zahtijeva specijaliziranu medicinsku stru\u010dnost, skupa je i dugotrajna. Lijekovi koji se nazivaju bypass agensi mogu se koristiti za zaobila\u017eenje inhibitora i pomo\u0107 pri zgru\u0161avanju krvi.   <\/span><\/p>\n<p><span style=\"font-weight: 400;\">Kod hemofilije, inhibitori se \u010de\u0161\u0107e javljaju kod osoba s te\u0161kim oblikom nego kod onih s umjerenom ili blagom hemofilijom. Procjenjuje se da otprilike 30% pacijenata s hemofilijom A i 1,5% do 3% pacijenata s hemofilijom B razvija inhibitore za koncentrate faktora VIII odnosno faktora IX. Za rijetke poreme\u0107aje krvarenja, brojke su nejasne zbog ograni\u010dene populacije pacijenata i nedostatka lije\u010denja.  <\/span><\/p>\n<p><span style=\"font-weight: 400;\">Inhibitori se obi\u010dno javljaju u prvih 75 izlaganja lije\u010denju, zbog \u010dega je va\u017eno da osobe s poreme\u0107ajima krvarenja prime svoj prvi tretman pod lije\u010dni\u010dkim nadzorom u centru za lije\u010denje gdje su dostupne odgovaraju\u0107e medicinske ustanove i stru\u010dnost u slu\u010daju razvoja inhibitora ili drugih alergijskih reakcija. Zapravo, alergijske reakcije se mogu poja\u010dati kada se lije\u010denje provodi tijekom vremena.   <\/span><\/p>\n<p><span style=\"font-weight: 400;\">Idealno bi bilo da se djeca i odrasli kojima je nedavno dijagnosticirana hemofilija redovito testiraju na inhibitore izme\u0111u prvog (1) i pedesetog (50) dana lije\u010denja. \u010cak i nakon 50. dana lije\u010denja, treba ih provjeravati barem dva puta godi\u0161nje dok ne prime 150-200 doza, a nakon toga barem jednom godi\u0161nje. Testiranje na inhibitore treba obaviti i prije svake ve\u0107e operacije.  <\/span><\/p>\n<p><span style=\"font-weight: 400;\">Neke studije su pokazale da ljudi koji redovito primaju tretmane koncentratima faktora kako bi sprije\u010dili krvarenje (profilaksa ili preventivno lije\u010denje) imaju manju vjerojatnost razvoja inhibitora tijekom \u017eivota. Malo se zna o tome igra li vrsta koncentrata faktora (rekombinantni ili dobiveni iz plazme) ulogu, ali postoje studije u tijeku koje \u0107e rasvijetliti ovo pitanje. <\/span><\/p>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/section>\n<!-- newsletter -->","protected":false},"template":"","meta":{"_acf_changed":false},"bleeding-disorder_cat":[],"class_list":["post-5280","bleeding-disorder","type-bleeding-disorder","status-publish","hentry"],"acf":[],"jetpack_sharing_enabled":true,"_links":{"self":[{"href":"https:\/\/www.ehc.eu\/hr\/wp-json\/wp\/v2\/bleeding-disorder\/5280","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.ehc.eu\/hr\/wp-json\/wp\/v2\/bleeding-disorder"}],"about":[{"href":"https:\/\/www.ehc.eu\/hr\/wp-json\/wp\/v2\/types\/bleeding-disorder"}],"wp:attachment":[{"href":"https:\/\/www.ehc.eu\/hr\/wp-json\/wp\/v2\/media?parent=5280"}],"wp:term":[{"taxonomy":"bleeding-disorder_cat","embeddable":true,"href":"https:\/\/www.ehc.eu\/hr\/wp-json\/wp\/v2\/bleeding-disorder_cat?post=5280"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}