{"id":2926,"date":"2022-09-06T09:30:35","date_gmt":"2022-09-06T07:30:35","guid":{"rendered":"https:\/\/dev.ircpss.com\/accueil-du-registre-international-des-shunts-portosystemiques-congenitaux\/"},"modified":"2025-11-18T09:53:41","modified_gmt":"2025-11-18T08:53:41","slug":"accueil-du-registre-international-des-shunts-portosystemiques-congenitaux","status":"publish","type":"page","link":"https:\/\/dev.ircpss.com\/fr\/","title":{"rendered":"Accueil du registre international des shunts portosyst\u00e9miques cong\u00e9nitaux"},"content":{"rendered":"

[vc_row full_width=\u00a0\u00bbstretch_row\u00a0\u00bb content_placement=\u00a0\u00bbtop\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1669714031584{background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}\u00a0\u00bb el_class=\u00a0\u00bbheader-bg\u00a0\u00bb][vc_column css=\u00a0\u00bb.vc_custom_1659965425459{background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}\u00a0\u00bb][vc_row_inner][vc_column_inner offset=\u00a0\u00bbvc_hidden-xs\u00a0\u00bb][vc_empty_space height=\u00a0\u00bb120px\u00a0\u00bb][\/vc_column_inner][\/vc_row_inner][vc_row_inner css=\u00a0\u00bb.vc_custom_1734379412026{margin-bottom: 0px !important;}\u00a0\u00bb][vc_column_inner width=\u00a0\u00bb1\/12″][\/vc_column_inner][vc_column_inner width=\u00a0\u00bb10\/12″][vc_column_text css=\u00a0\u00bb.vc_custom_1734379369522{margin-bottom: 0px !important;}\u00a0\u00bb]<\/p>\n

Bienvenue sur le site web du registre international des shunts portosyst\u00e9miques cong\u00e9nitaux<\/span><\/h1>\n

[\/vc_column_text][vc_column_text]L’objectif de ce site web est de promouvoir le Registre International des Shunts Porto-Syst\u00e9miques Cong\u00e9nitaux<\/strong> (IRCPSS) et de fournir des ressources<\/strong> pour un large public, allant des patients aux professionnels de sant\u00e9<\/strong> du monde entier.[\/vc_column_text][\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/12″][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row][vc_row gap=\u00a0\u00bb25″ equal_height=\u00a0\u00bbyes\u00a0\u00bb content_placement=\u00a0\u00bbmiddle\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1734013159949{margin-top: 50px !important;margin-bottom: 25px !important;background-color: #FFFFFF !important;border-radius: 20px !important;}\u00a0\u00bb][vc_column width=\u00a0\u00bb1\/2″ css=\u00a0\u00bb.vc_custom_1734012715756{border-radius: 15px !important;}\u00a0\u00bb][vc_custom_heading text=\u00a0\u00bbLes Shunts Portosyst\u00e9miques Cong\u00e9nitaux (CPSS), une malformation rare qui peut \u00eatre trait\u00e9e<\/span>\u00a0\u00bb font_container=\u00a0\u00bbtag:h2|font_size:24|text_align:left|line_height:1.1″ use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1734451386648{margin-left: 10px !important;}\u00a0\u00bb][vc_column_text css=\u00a0\u00bb.vc_custom_1734379494943{margin-bottom: 0px !important;padding-right: 10px !important;padding-left: 10px !important;}\u00a0\u00bb]Les shunts porto-syst\u00e9miques cong\u00e9nitaux (CPSS) sont des malformations rares qui font que le sang des intestins contourne le foie. Cette malformation rare peut avoir un impact significatif sur la sant\u00e9, mais il existe des solutions pour la traiter et pr\u00e9venir les sympt\u00f4mes graves. Dans cette vid\u00e9o, d\u00e9couvrez plus d’informations sur les CPSS, leurs sympt\u00f4mes et comment les traiter.<\/strong> Vous trouverez \u00e9galement un t\u00e9moignage \u00e9mouvant d’une famille touch\u00e9e par cette maladie rare.[\/vc_column_text][\/vc_column][vc_column width=\u00a0\u00bb1\/2″][vc_video link=\u00a0\u00bbhttps:\/\/youtu.be\/k_-q_KsEZX0?si=PJW69s1jhZK_bo0c\u00a0\u00bb align=\u00a0\u00bbcenter\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1734379535177{margin-bottom: 0px !important;}\u00a0\u00bb][\/vc_column][\/vc_row][vc_row gap=\u00a0\u00bb35″ equal_height=\u00a0\u00bbyes\u00a0\u00bb][vc_column width=\u00a0\u00bb1\/2″ css=\u00a0\u00bb.vc_custom_1669724113081{background-color: #defff1 !important;border-radius: 15px !important;}\u00a0\u00bb el_class=\u00a0\u00bbombre-inner green-card\u00a0\u00bb]

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Qu’est-ce qu’un shunt portosyst\u00e9mique cong\u00e9nital ?<\/h3>\n

Un Shunt Porto-Syst\u00e9mique Cong\u00e9nital (CPSS)<\/a> est une malformation vasculaire rare o\u00f9 le sang passe directement des intestins au c\u0153ur sans passer par le foie pour \u00eatre filtr\u00e9.<\/p>\n

Aujourd’hui, nous savons que si le sang n’est pas filtr\u00e9 par le foie, cela peut causer des complications \u00e0 tout \u00e2ge.<\/p>\n

Il est donc recommand\u00e9 que la plupart des CPSS soient ferm\u00e9s chirurgicalement ou par de petits dispositifs plac\u00e9s dans le vaisseau anormal.<\/p>\n

En savoir plus ><\/strong><\/a>[\/vc_column_text][\/vc_column][vc_column width=\u00a0\u00bb1\/2″ css=\u00a0\u00bb.vc_custom_1669724123037{background-color: #fff0f8 !important;border-radius: 15px !important;}\u00a0\u00bb el_class=\u00a0\u00bbombre-inner pink-card\u00a0\u00bb]

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Histoire naturelle et diagnostic<\/h3>\n

Les CPSS peuvent \u00eatre diagnostiqu\u00e9s fortuitement ou en raison de complications \u00e0 tout \u00e2ge, du f\u0153tus \u00e0 la personne \u00e2g\u00e9e. Pendant la grossesse, les CPSS sont diagnostiqu\u00e9s par \u00e9chographie. Apr\u00e8s la naissance, le type de CPSS le plus fr\u00e9quent – intrah\u00e9patique – tend \u00e0 se fermer spontan\u00e9ment dans les premiers mois de vie. N\u00e9anmoins, certains shunts restent ouverts. Selon la position et la taille du CPSS, le sang provenant des intestins sera d\u00e9vi\u00e9 partiellement ou compl\u00e8tement, contournant ainsi le foie. Cela signifie que le foie ne peut pas remplir sa fonction de d\u00e9toxification. Jusqu’\u00e0 pr\u00e9sent, il n’existe aucun moyen de pr\u00e9dire quelle(s) complication(s) un patient pr\u00e9sentera ou quand elle(s) surviendra(ont). <\/p>\n

En savoir plus ><\/strong><\/a>[\/vc_column_text][\/vc_column][\/vc_row][vc_row gap=\u00a0\u00bb35″ equal_height=\u00a0\u00bbyes\u00a0\u00bb][vc_column width=\u00a0\u00bb1\/2″ css=\u00a0\u00bb.vc_custom_1669724133062{background-color: #fffadd !important;border-radius: 15px !important;}\u00a0\u00bb el_class=\u00a0\u00bbombre-inner yellow-card\u00a0\u00bb]

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Prise en charge des CPSS<\/h3>\n

La prise en charge des CPSS n\u00e9cessite une \u00e9quipe multidisciplinaire pour g\u00e9rer les complications potentielles.<\/p>\n

Une expertise est n\u00e9cessaire pour d\u00e9terminer si le patient b\u00e9n\u00e9ficiera d’une fermeture du shunt par des radiologues interventionnels qui peuvent placer des bouchons dans le shunt, ou si une chirurgie sera n\u00e9cessaire.<\/p>\n

En savoir plus ><\/strong><\/a>[\/vc_column_text][\/vc_column][vc_column width=\u00a0\u00bb1\/2″ css=\u00a0\u00bb.vc_custom_1669724142472{background-color: #e1fdff !important;border-radius: 15px !important;}\u00a0\u00bb el_class=\u00a0\u00bbombre-inner blue-card\u00a0\u00bb]

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Le registre<\/h3>\n

Les shunts porto-syst\u00e9miques cong\u00e9nitaux sont une anomalie des veines abdominales qui conduit le sang \u00e0 contourner le foie. Lorsque le foie est contourn\u00e9, des complications peuvent survenir d\u00e8s le plus jeune \u00e2ge. Bien que la pr\u00e9valence de cette malformation rare ne soit pas connue, les complications suivantes ont \u00e9t\u00e9 d\u00e9crites chez les enfants : tumeurs du foie, insuffisance cardiaque, anomalies vasculaires du poumon, retard de d\u00e9veloppement et plus encore. Par cons\u00e9quent, l’objectif du registre international est d’accro\u00eetre les preuves et les connaissances sur cette malformation rare. <\/p>\n

En savoir plus ><\/strong><\/a>[\/vc_column_text][\/vc_column][\/vc_row][vc_row][vc_column offset=\u00a0\u00bbvc_hidden-xs\u00a0\u00bb][vc_empty_space height=\u00a0\u00bb60px\u00a0\u00bb][\/vc_column][\/vc_row][vc_row][vc_column width=\u00a0\u00bb1\/6″][\/vc_column][vc_column width=\u00a0\u00bb2\/3″ css=\u00a0\u00bb.vc_custom_1669719209851{padding-right: 30px !important;padding-left: 30px !important;background-color: #ffffff !important;border-radius: 15px !important;}\u00a0\u00bb el_class=\u00a0\u00bbombre\u00a0\u00bb][vc_row_inner][vc_column_inner el_class=\u00a0\u00bbpicto-blue\u00a0\u00bb width=\u00a0\u00bb1\/4″]

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Le registre international des shunts portosyst\u00e9miques cong\u00e9nitaux est une initiative mondiale<\/h2>\n

Son objectif est d’aider les professionnels de sant\u00e9 et les patients \u00e0 trouver des ressources, \u00e0 \u00e9viter l’errance diagnostique des patients, \u00e0 am\u00e9liorer le diagnostic et les traitements autour de la condition des Shunts Portosyst\u00e9miques Cong\u00e9nitaux.[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][vc_btn title=\u00a0\u00bbTrouver un expert local\u00a0\u00bb style=\u00a0\u00bbcustom\u00a0\u00bb custom_background=\u00a0\u00bb#4767e7″ custom_text=\u00a0\u00bb#ffffff\u00a0\u00bb size=\u00a0\u00bblg\u00a0\u00bb align=\u00a0\u00bbcenter\u00a0\u00bb link=\u00a0\u00bburl:https%3A%2F%2Fircpss.com%2Fircpss-local-experts%2F|title:Trouver%20un%20expert%20local|\u00a0\u00bb][\/vc_column][vc_column width=\u00a0\u00bb1\/6″][\/vc_column][\/vc_row][vc_row][vc_column offset=\u00a0\u00bbvc_hidden-xs\u00a0\u00bb][vc_empty_space height=\u00a0\u00bb60px\u00a0\u00bb][\/vc_column][\/vc_row]<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"

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