{"id":5306,"date":"2024-10-10T14:51:53","date_gmt":"2024-10-10T12:51:53","guid":{"rendered":"https:\/\/www.ehc.eu\/bleeding-disorder\/nedostatak-faktora-i-fibrinogena\/"},"modified":"2025-12-23T09:58:41","modified_gmt":"2025-12-23T08:58:41","slug":"nedostatak-faktora-i-fibrinogena","status":"publish","type":"bleeding-disorder","link":"https:\/\/www.ehc.eu\/hr\/bleeding-disorder\/nedostatak-faktora-i-fibrinogena\/","title":{"rendered":"Nedostatak faktora I (fibrinogena)"},"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 I (fibrinogena)<\/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>Rijetki nasljedni poreme\u0107aji koagulacije karakterizirani simptomima krvarenja u rasponu od blagih do te\u0161kih, a koji su rezultat smanjene koli\u010dine i\/ili kvalitete fibrinogena u cirkulaciji. Afibrinogenemija (potpuni nedostatak fibrinogena) i hipofibrinogenemija (smanjena koncentracija fibrinogena u plazmi) odgovaraju kvantitativnim anomalijama fibrinogena, dok disfibrinogenemija odgovara funkcionalnoj anomaliji fibrinogena. Hipo- i disfibrinogenemija \u010desto se mogu kombinirati (hipodisfibrinogenemija).  <\/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<br \/>\n<\/strong> Prevalencija afibrinogenemije procjenjuje se na 1\/1.000.000. Hipo- i disfibrinogenemija su \u010de\u0161\u0107e.  Oba spola su podjednako pogo\u0111ena. Nedostatak fibrinogena mo\u017ee se otkriti u bilo kojoj dobi, ali afibrinogenemija se obi\u010dno manifestira rano u djetinjstvu, \u010desto u neonatalnom razdoblju.<\/p>\n<p><strong>Klini\u010dki opis<br \/>\n<\/strong> Uobi\u010dajene manifestacije afibrinogenemije uklju\u010duju krvarenje iz pupkovine, epistaksu, hemartrozu, gastrointestinalno krvarenje, menoragiju, traumatsko i kirur\u0161ko krvarenje te, rijetko, intrakranijalno krvarenje. Kod \u017eena pogo\u0111enih afibrinogenemijom mogu se pojaviti ponavljaju\u0107i spontani poba\u010daji. Hipofibrinogenemiju karakteriziraju rje\u0111e i bla\u017ee epizode krvarenja nakon traume ili operacije. Ve\u0107ina pacijenata s disfibrinogenemijom je asimptomatska (60%). Ostali mogu imati simptome krvarenja (28%) ili \u010dak trombozu (20%).    <\/p>\n<p><strong>Etiologija<br \/>\n<\/strong> Kongenitalni nedostaci fibrinogena uzrokovani su mutacijama u genima <i>FGA<\/i> , <i>FGB<\/i> ili <i>FGG<\/i> . Afibrinogenemija je autosomno recesivna; hipofibrinogenemija i disfibrinogenemija su uglavnom autosomno dominantne. <\/p>\n<p><strong>Dijagnosti\u010dke metode<br \/>\n<\/strong> Dijagnoza se temelji na produljenom aktiviranom parcijalnom tromboplastinskom vremenu (aPTT), protrombinskom vremenu (PT), trombinom i reptilaznom vremenu te na razinama fibrinogena mjerenim funkcionalnim (Claussovim) i imunolo\u0161kim testovima.<\/p>\n<p><strong>Diferencijalna dijagnoza<br \/>\n<\/strong> Diferencijalna dijagnoza uklju\u010duje druge kongenitalne nedostatke faktora zgru\u0161avanja (faktori II, V, VII, X, XI, VIII, IX i XIII; vidi ove pojmove) i ste\u010deni nedostatak fibrinogena (konzumptivna koagulopatija, zatajenje jetre). U slu\u010daju tromboze, diferencijalna dijagnoza tako\u0111er uklju\u010duje kongenitalnu ili ste\u010denu trombofiliju (nedostatak antitrombina, nedostatak proteina C ili S, mutaciju faktora V Leiden, lupusni antikoagulant (vidi ove pojmove) i mutaciju FII Leiden). <\/p>\n<p><strong>Prenatalna dijagnoza<br \/>\n<\/strong> Prenatalna dijagnoza afibrinogenemije mogu\u0107a je ako su uzro\u010dne mutacije ve\u0107 identificirane u obitelji.<\/p>\n<p><em>Informacije su preuzete s web stranice Orpha.net<\/em><\/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>Afibrinogenemija<\/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>Prevalencija<\/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\/1,000,000<\/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>Hipofibrinogenemija<\/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>Prevalencija<\/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>1-9\/1,000,000<\/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>Disfibrinogenemija<\/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>Prevalencija<\/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>1-9\/1,000,000<\/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<div class=\"title h4\" data-scroll data-fade-up>\n\t\t\t\t\t\t\t\t<p>Lije\u010denje i upravljanje<\/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 class=\"mx-4\">Koncentrati fibrinogena obi\u010dno se koriste za lije\u010denje krvarenja. Svje\u017ee smrznuta plazma koristi se kada koncentrati fibrinogena nisu dostupni. Ponavljani spontani poba\u010daji mogu se sprije\u010diti rutinskom profilaksom koja se temelji na primjeni koncentrata fibrinogena u ranoj trudno\u0107i.  <\/p>\n<p><strong>Prognoza<br \/>\n<\/strong> Iako se mo\u017ee pojaviti po \u017eivot opasno intrakranijalno krvarenje, prognoza afibrinogenemije je dobra uz ranu dijagnozu i adekvatno lije\u010denje. Prognoza hipo- ili disfibrinogenemije je obi\u010dno dobra. <\/p>\n<p><em>Informacije su preuzete s web stranice Orpha.net<\/em><\/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-5306","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\/5306","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=5306"}],"wp:term":[{"taxonomy":"bleeding-disorder_cat","embeddable":true,"href":"https:\/\/www.ehc.eu\/hr\/wp-json\/wp\/v2\/bleeding-disorder_cat?post=5306"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}