{"id":1639,"date":"2024-05-01T09:20:17","date_gmt":"2024-05-01T07:20:17","guid":{"rendered":"https:\/\/amyloidosisalliance.local\/une-nouvelle-etude-publiee-sur-la-polyneuropathie-amyloide-hereditaire-a-transthyretine\/"},"modified":"2026-09-29T15:32:13","modified_gmt":"2026-09-29T13:32:13","slug":"une-nouvelle-etude-publiee-sur-la-polyneuropathie-amyloide-hereditaire-a-transthyretine","status":"publish","type":"post","link":"https:\/\/www.amyloidosisalliance.org\/fr\/une-nouvelle-etude-publiee-sur-la-polyneuropathie-amyloide-hereditaire-a-transthyretine\/","title":{"rendered":"Une nouvelle \u00e9tude publi\u00e9e sur la polyneuropathie amylo\u00efde h\u00e9r\u00e9ditaire \u00e0 transthyr\u00e9tine"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\">L\u2019\u00e9tude publi\u00e9e dans les Annals of Neurology, intitul\u00e9e \u00ab\u00a0Disease-Modifying Drugs Extend Survival in Hereditary Transthyretin Amyloid Polyneuropathy\u00a0\u00bb, \u00e9claire l\u2019efficacit\u00e9 des traitements de la polyneuropathie amylo\u00efde familiale (PAF). <br\/><br\/>La PAF, due \u00e0 des mutations du g\u00e8ne de la transthyr\u00e9tine, entra\u00eene l\u2019accumulation d\u2019amas de prot\u00e9ines toxiques dans les tissus nerveux, avec des sympt\u00f4mes invalidants. Il n\u2019existe pas de traitement curatif, mais les options de traitement comprennent les m\u00e9dicaments de fond et la greffe de foie. <br\/><br\/>Les chercheurs ont analys\u00e9 les donn\u00e9es de 201 patients atteints de PAF sur trois d\u00e9cennies, et observ\u00e9 des diff\u00e9rences importantes de survie entre les patients trait\u00e9s et ceux qui ne l\u2019\u00e9taient pas.<br\/>Fait notable, presque tous les #patients trait\u00e9s \u00e9taient estim\u00e9s en vie dix ans apr\u00e8s le d\u00e9but de la maladie, contre moins de la moiti\u00e9 des patients non trait\u00e9s. <br\/><br\/>Les m\u00e9dicaments de fond, comme Onpattro, le tafamidis et le diflunisal, ont donn\u00e9 des r\u00e9sultats prometteurs sur la survie, quel que soit l\u2019\u00e2ge de d\u00e9but de la maladie.<br\/>M\u00eame les patients atteints d\u2019une PAF \u00e0 d\u00e9but tardif ont vu leur survie s\u2019am\u00e9liorer avec les m\u00e9dicaments de fond, ce qui remet en question l\u2019id\u00e9e que la greffe de foie \u00e9tait le traitement principal des formes \u00e0 d\u00e9but pr\u00e9coce. <br\/><br\/>Si la greffe de foie a prolong\u00e9 la survie dans les formes \u00e0 d\u00e9but pr\u00e9coce, son effet sur les formes \u00e0 d\u00e9but tardif a \u00e9t\u00e9 moins marqu\u00e9.<br\/>L\u2019\u00e9tude souligne l\u2019importance des m\u00e9dicaments de fond pour am\u00e9liorer la survie \u00e0 long terme des patients atteints de PAF, quel que soit l\u2019\u00e2ge de d\u00e9but ou le type de sympt\u00f4mes. <br\/><br\/>L\u2019analyse porte toutefois sur un seul centre, et d\u2019autres \u00e9tudes sont n\u00e9cessaires pour confirmer ces r\u00e9sultats dans d\u2019autres populations et d\u2019autres contextes m\u00e9dicaux. <br\/><br\/>Retrouvez l\u2019article ci-dessous <br\/><a href=\"https:\/\/urlr.me\/X1tHm\"><mark style=\"background-color:rgba(0, 0, 0, 0)\" class=\"has-inline-color has-light-green-cyan-color\">https:\/\/urlr.me\/X1tHm<\/mark><\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Une \u00e9tude men\u00e9e sur 201 patients pendant trois d\u00e9cennies sugg\u00e8re que les traitements de fond prolongent la survie dans l\u2019amylose ATTRv avec polyneuropathie.<\/p>\n","protected":false},"author":1,"featured_media":1640,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[24],"tags":[],"class_list":["post-1639","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-actualites"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.6 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Une nouvelle \u00e9tude publi\u00e9e sur la polyneuropathie amylo\u00efde h\u00e9r\u00e9ditaire \u00e0 transthyr\u00e9tine<\/title>\n<meta name=\"description\" content=\"Une \u00e9tude men\u00e9e sur 201 patients 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