{"id":5272,"date":"2024-10-24T09:47:45","date_gmt":"2024-10-24T07:47:45","guid":{"rendered":"https:\/\/www.ehc.eu\/bleeding-disorder\/nedostatak-faktora-xiii\/"},"modified":"2025-12-23T09:57:43","modified_gmt":"2025-12-23T08:57:43","slug":"nedostatak-faktora-xiii","status":"publish","type":"bleeding-disorder","link":"https:\/\/www.ehc.eu\/hr\/bleeding-disorder\/nedostatak-faktora-xiii\/","title":{"rendered":"Nedostatak faktora XIII"},"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 XIII<\/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>Nasljedni poreme\u0107aj krvarenja uzrokovan smanjenom razinom i aktivno\u0161\u0107u faktora XIII (FXIII), karakteriziran hemoragi\u010dnom dijatezom, \u010desto povezanom sa spontanim poba\u010dajima i poreme\u0107ajem zacjeljivanja rana. Nedostatak faktora XIII jedan je od najrje\u0111ih nedostataka faktora koagulacije.<\/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 homozigotnih oblika procjenjuje se na oko 1\/2 000 000. Oba spola su podjednako pogo\u0111ena.<\/p>\n<p><strong>Klini\u010dki opis<br \/>\n<\/strong> Kongenitalni nedostatak FXIII mo\u017ee se manifestirati u bilo kojoj dobi, ali dijagnoza se \u010desto postavlja tijekom dojena\u010dke dobi. Krvarenje iz pup\u010danog panja javlja se kod \u010dak 80% pacijenata. Drugi uobi\u010dajeni znakovi uklju\u010duju intrakranijalno krvarenje (25-30%), krvarenje u mekom tkivu, modrice, hemartroze (20%) i ponavljaju\u0107e spontane poba\u010daje. U ve\u0107ini slu\u010dajeva, krvarenja su odgo\u0111ena (12-36 sati) nakon traume ili operacije. Pacijenti mogu imati slabo zacjeljivanje rana. Ste\u010deni oblici bolesti tako\u0111er su zabilje\u017eeni u vezi sa zatajenjem jetre, upalnom bole\u0161\u0107u crijeva (vidi ovaj pojam) i mijeloi\u010dnom leukemijom.<\/p>\n<p><strong>Etiologija<br \/>\n<\/strong> Kongenitalni nedostatak FXIII obi\u010dno je uzrokovan mutacijama u genu <i>F13A1<\/i> (6p24.2-p23) koji kodira kataliti\u010dku A podjedinicu, ali mutacije su prona\u0111ene i u genu <i>F13B<\/i> (1q31-q32.1) koji kodira B podjedinicu. Prijenos je autosomno recesivan. Fenotip je manje ozbiljan kada je gen <i>F13B<\/i> mutiran.<\/p>\n<p><strong>Dijagnosti\u010dke metode<br \/>\n<\/strong> Dijagnoza se temelji na kvantitativnom mjerenju aktivnosti FXIII i testovima antigena. Uobi\u010dajeni testovi zgru\u0161avanja krvi poput aktiviranog parcijalnog tromboplastinskog vremena (aPTT) i protrombinskog vremena (PT) su normalni i ne mogu se koristiti za probir. Mo\u017ee se koristiti i test topljivosti ugru\u0161ka (ugru\u0161ak je stabilan dulje od 24 sata u slu\u010daju nedostatka FXIII). Molekularno testiranje je dostupno, ali nije potrebno za dijagnozu.<\/p>\n<p><strong>Diferencijalna dijagnoza<br \/>\n<\/strong> Diferencijalne dijagnoze uglavnom uklju\u010duju ostale kongenitalne nedostatke faktora koagulacije: fibrinogen, faktore II, V, VII, X, XI, VIII, IX (vidjeti ove pojmove).<\/p>\n<p><strong>Prenatalna dijagnoza<br \/>\n<\/strong> Prenatalna dijagnoza je mogu\u0107a ako su uzro\u010dne mutacije prethodno 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>Kongenitalni nedostatak faktora XIII<\/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>&lt;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<\/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\">Za lije\u010denje krvarenja obi\u010dno se koriste koncentrati faktora XIII ili svje\u017ee smrznuta plazma (kada koncentrati FXIII nisu dostupni). Profilakti\u010dka terapija koncentratom FXIII treba biti indicirana kako bi se sprije\u010dila ponovljena krvarenja poput intrakranijalnog krvarenja.<\/p>\n<p><strong>Prognoza<\/strong><br \/>\n Intrakranijalno krvarenje mo\u017ee biti opasno po \u017eivot, ali prognoza je povoljna ako se pru\u017ei odgovaraju\u0107e lije\u010denje.<\/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-5272","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\/5272","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=5272"}],"wp:term":[{"taxonomy":"bleeding-disorder_cat","embeddable":true,"href":"https:\/\/www.ehc.eu\/hr\/wp-json\/wp\/v2\/bleeding-disorder_cat?post=5272"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}