{"id":5286,"date":"2024-10-10T16:05:55","date_gmt":"2024-10-10T14:05:55","guid":{"rendered":"https:\/\/www.ehc.eu\/bleeding-disorder\/nedostatak-faktora-x\/"},"modified":"2025-12-23T09:57:56","modified_gmt":"2025-12-23T08:57:56","slug":"nedostatak-faktora-x","status":"publish","type":"bleeding-disorder","link":"https:\/\/www.ehc.eu\/hr\/bleeding-disorder\/nedostatak-faktora-x\/","title":{"rendered":"Nedostatak faktora X"},"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 X<\/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 nasljedni poreme\u0107aj krvarenja sa smanjenim antigenom i\/ili aktivno\u0161\u0107u faktora X (FX) i karakteriziran blagim do te\u0161kim simptomima krvarenja.<\/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<div class=\"fs-5 orpha-blue\"><strong>Epidemiologija<br \/>\n<\/strong> Prevalencija homozigotnih oblika procjenjuje se na 1\/1 000 000. Oba spola su podjednako pogo\u0111ena. <\/div>\n<p class=\"fs-5 orpha-blue\"><strong>Klini\u010dki opis<br \/>\n<\/strong> Kongenitalni nedostatak FX-a manifestira se u bilo kojoj dobi, ali op\u0107enito se te\u0161ki oblici bolesti manifestiraju rano u \u017eivotu. Pacijenti mogu iskusiti ozbiljno krvarenje iz pup\u010dane vrpce, ponavljaju\u0107u epistaksu, krvarenja u mekom tkivu, menoragiju, lako stvaranje modrica, intrakranijalna krvarenja, hematuriju, hemartroze i prekomjerno krvarenje tijekom ili nakon operacije, poroda ili traume. <\/p>\n<p><strong>Etiologija<br \/>\n<\/strong> Nasljedni kongenitalni nedostatak FX-a uzrokovan je mutacijama u genu <i>F10<\/i> (13q34) koji kontrolira proizvodnju plazmatskog FX-a. Ozbiljnost manifestacija krvarenja korelira s razinom FX-a. <\/p>\n<p><strong>Dijagnosti\u010dke metode<br \/>\n<\/strong> Dijagnoza se temelji na produljenom protrombinskom vremenu, vremenu aktiviranog parcijalnog tromboplastina i vremenu detekcije otrova Russell vipera (PT, aPTT, RVVT) te na smanjenim razinama FX. Molekularno testiranje je dostupno, ali nije potrebno za dijagnozu. <\/p>\n<p><strong>Diferencijalna dijagnoza<br \/>\n<\/strong> Diferencijalne dijagnoze uklju\u010duju nedostatke faktora II, V, VII, VIII, IX, XI, XIII ili ste\u010dene nedostatke FX (amiloidoza).<\/p>\n<p><strong>Genetsko savjetovanje<br \/>\n<\/strong> Prijenos je autosomno recesivan. Genetsko savjetovanje treba ponuditi parovima s pove\u0107anim rizikom (oboje su nositelji mutacije koja uzrokuje bolest) i informirati ih o 25%-tnom riziku od ra\u0111anja djeteta s bole\u0161\u0107u u svakoj trudno\u0107i. Heterozigotni pacijenti naj\u010de\u0161\u0107e ostaju asimptomatski.  <\/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 X<\/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\">Za lije\u010denje hemoragijskih epizoda obi\u010dno se koriste koncentrati protrombinskog kompleksa (PCC) ili svje\u017ee smrznuta plazma (ako PCC nisu dostupni). Sada je dostupan FX koncentrat dobiven iz plazme. <\/p>\n<p><strong>Prognoza<\/strong><br \/>\n Prognoza je dobra uz ranu dijagnozu i adekvatno 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<!-- content-files -->    <section   class=\"s-padding overflow-visible\">\n        <div class=\"container\">\n            <div class=\"w-full lg:w-8\/12 text-center lg:text-left relative z-10\">\n                                    <div class=\"title h4\" data-scroll data-fade-left>\n                        <p>Dodatne informacije<\/p>\n                    <\/div>\n                                                    <div class=\"content mt-14\" data-scroll data-fade-up>\n                        <p>Pro\u010ditajte o nedostatku nasljednog faktora X na <a href=\"https:\/\/academy.ehc.eu\/advocacy-materials\/\">stranici s materijalima za zagovaranje<\/a> Akademije EHC:<\/p>\n<ul>\n<li>\u0160to je nasljedni deficit FX-a &#8211; <strong><a href=\"https:\/\/academy.ehc.eu\/wp-content\/uploads\/2024\/07\/Hereditary-FX-deficiency.pdf\">ovdje<\/a><\/strong> <\/li>\n<li>Ozbiljnost HFX-a &#8211; <strong><a href=\"https:\/\/academy.ehc.eu\/wp-content\/uploads\/2025\/03\/Hereditary-FX-Deficiency-Severity-.pdf\">ovdje<\/a><\/strong><\/li>\n<li><strong><a href=\"https:\/\/academy.ehc.eu\/wp-content\/uploads\/2025\/03\/Hereditary-FX-Deficiency-Testing.pdf\">Testiranje<\/a><\/strong><\/li>\n<li>\u017dene i djevojke s nedostatkom faktora X &#8211; <strong><a href=\"https:\/\/academy.ehc.eu\/wp-content\/uploads\/2025\/03\/FX-Deficiency-in-Women-and-Girls-1-1.pdf\">ovdje<\/a><\/strong><\/li>\n<\/ul>\n                    <\/div>\n                                            <\/div>\n        <\/div>\n        <figure class=\"absolute size-[72.8rem] top-0 right-[-5%] image-contain opacity-40 lg:opacity-100\">\n            <img decoding=\"async\" loading=\"lazy\" src=\"https:\/\/etngbd86n6b.exactdn.com\/wp-content\/themes\/cbd\/img\/content-logo-colorfull-2.svg\" alt=\"content-logo-colorfull\">\n        <\/figure>\n    <\/section>\n<!-- newsletter -->","protected":false},"template":"","meta":{"_acf_changed":false},"bleeding-disorder_cat":[],"class_list":["post-5286","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\/5286","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=5286"}],"wp:term":[{"taxonomy":"bleeding-disorder_cat","embeddable":true,"href":"https:\/\/www.ehc.eu\/hr\/wp-json\/wp\/v2\/bleeding-disorder_cat?post=5286"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}