{"id":5284,"date":"2024-10-10T16:05:55","date_gmt":"2024-10-10T14:05:55","guid":{"rendered":"https:\/\/www.ehc.eu\/bleeding-disorder\/deficit-en-facteur-x\/"},"modified":"2025-12-23T09:57:56","modified_gmt":"2025-12-23T08:57:56","slug":"deficit-en-facteur-x","status":"publish","type":"bleeding-disorder","link":"https:\/\/www.ehc.eu\/fr\/bleeding-disorder\/deficit-en-facteur-x\/","title":{"rendered":"D\u00e9ficit en facteur 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%]\">D\u00e9ficit en facteur 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>Trouble h\u00e9r\u00e9ditaire rare de la coagulation caract\u00e9ris\u00e9 par une diminution de l&rsquo;antig\u00e8ne et\/ou de l&rsquo;activit\u00e9 du facteur X (FX) et par des sympt\u00f4mes h\u00e9morragiques l\u00e9gers \u00e0 s\u00e9v\u00e8res.<\/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>\u00c9pid\u00e9miologie<br \/>\n<\/strong>La pr\u00e9valence des formes homozygotes est estim\u00e9e \u00e0 1\/1 000 000. Les deux sexes sont \u00e9galement touch\u00e9s. <\/div>\n<p class=\"fs-5 orpha-blue\"><strong>Description clinique<br \/>\n<\/strong>Le d\u00e9ficit cong\u00e9nital en FX peut se manifester \u00e0 tout \u00e2ge, mais en g\u00e9n\u00e9ral, les formes graves de la maladie se manifestent t\u00f4t dans la vie. Les patients peuvent pr\u00e9senter des saignements importants du moignon du cordon ombilical, des \u00e9pistaxis r\u00e9currentes, des h\u00e9morragies des tissus mous, des m\u00e9norragies, des ecchymoses faciles, des h\u00e9morragies intracr\u00e2niennes, des h\u00e9maturies, des h\u00e9marthroses et des saignements excessifs pendant ou apr\u00e8s une intervention chirurgicale, un accouchement ou un traumatisme. <\/p>\n<p><strong>\u00c9tiologie<br \/>\n<\/strong>Le d\u00e9ficit cong\u00e9nital h\u00e9r\u00e9ditaire en FX est d\u00fb \u00e0 des mutations du g\u00e8ne <i>F10<\/i> (13q34) qui contr\u00f4le la production de FX plasmatique. La gravit\u00e9 des manifestations h\u00e9morragiques est en corr\u00e9lation avec le taux de FX. <\/p>\n<p><strong>M\u00e9thodes de diagnostic<br \/>\n<\/strong>Le diagnostic repose sur l&rsquo;allongement des temps de Quick, de thromboplastine partielle activ\u00e9e et de venin de vip\u00e8re du Russell (PT, aPTT, RVVT), ainsi que sur la r\u00e9duction des taux de FX. Des tests mol\u00e9culaires sont disponibles, mais ne sont pas n\u00e9cessaires pour le diagnostic. <\/p>\n<p><strong>Diagnostic diff\u00e9rentiel<br \/>\n<\/strong>Les diagnostics diff\u00e9rentiels incluent les d\u00e9ficiences en facteurs II, V, VII, VIII, IX, XI, XIII ou les d\u00e9ficiences acquises en FX (amylo\u00efdose).<\/p>\n<p><strong>Conseil g\u00e9n\u00e9tique<br \/>\n<\/strong>La transmission est autosomique r\u00e9cessive. Un conseil g\u00e9n\u00e9tique devrait \u00eatre propos\u00e9 aux couples \u00e0 risque (les deux individus sont porteurs d&rsquo;une mutation causant la maladie), les informant du risque de 25 % d&rsquo;avoir un enfant atteint \u00e0 chaque grossesse. Les patients h\u00e9t\u00e9rozygotes restent le plus souvent asymptomatiques.  <\/p>\n<p><em>L&rsquo;information est tir\u00e9e du site web 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>D\u00e9ficit cong\u00e9nital en facteur 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>Pr\u00e9valence<\/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>Prise en charge et traitement<\/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\">Les concentr\u00e9s de complexe prothrombique (CCP) ou le plasma frais congel\u00e9 (si les CCP ne sont pas disponibles) sont g\u00e9n\u00e9ralement utilis\u00e9s pour traiter les \u00e9pisodes h\u00e9morragiques. Un concentr\u00e9 de FX d\u00e9riv\u00e9 du plasma est maintenant disponible. <\/p>\n<p><strong>Pronostic<\/strong><br \/>\nLe pronostic est bon avec un diagnostic pr\u00e9coce et un traitement ad\u00e9quat.<\/p>\n<p><em>L&rsquo;information est tir\u00e9e du site web 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>Plus d&rsquo;informations<\/p>\n                    <\/div>\n                                                    <div class=\"content mt-14\" data-scroll data-fade-up>\n                        <p>Pour en savoir plus sur le d\u00e9ficit h\u00e9r\u00e9ditaire en facteur X, consultez la <a href=\"https:\/\/academy.ehc.eu\/advocacy-materials\/\">page des documents de plaidoyer de l<\/a>&lsquo;EHC Academy :<\/p>\n<ul>\n<li>Qu&rsquo;est-ce que le d\u00e9ficit h\u00e9r\u00e9ditaire en FX ?<strong> <a href=\"https:\/\/academy.ehc.eu\/wp-content\/uploads\/2024\/07\/Hereditary-FX-deficiency.pdf\">ici <\/a><\/strong> <\/li>\n<li>S\u00e9v\u00e9rit\u00e9 HFX &#8211; <strong><a href=\"https:\/\/academy.ehc.eu\/wp-content\/uploads\/2025\/03\/Hereditary-FX-Deficiency-Severity-.pdf\">ici<\/a><\/strong><\/li>\n<li><strong><a href=\"https:\/\/academy.ehc.eu\/wp-content\/uploads\/2025\/03\/Hereditary-FX-Deficiency-Testing.pdf\">Essais<\/a><\/strong><\/li>\n<li>Femmes et jeunes filles atteintes d&rsquo;une d\u00e9ficience en facteur X<strong> <a href=\"https:\/\/academy.ehc.eu\/wp-content\/uploads\/2025\/03\/FX-Deficiency-in-Women-and-Girls-1-1.pdf\">ici<\/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-5284","bleeding-disorder","type-bleeding-disorder","status-publish","hentry"],"acf":[],"jetpack_sharing_enabled":true,"_links":{"self":[{"href":"https:\/\/www.ehc.eu\/fr\/wp-json\/wp\/v2\/bleeding-disorder\/5284","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.ehc.eu\/fr\/wp-json\/wp\/v2\/bleeding-disorder"}],"about":[{"href":"https:\/\/www.ehc.eu\/fr\/wp-json\/wp\/v2\/types\/bleeding-disorder"}],"wp:attachment":[{"href":"https:\/\/www.ehc.eu\/fr\/wp-json\/wp\/v2\/media?parent=5284"}],"wp:term":[{"taxonomy":"bleeding-disorder_cat","embeddable":true,"href":"https:\/\/www.ehc.eu\/fr\/wp-json\/wp\/v2\/bleeding-disorder_cat?post=5284"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}