{"id":5525,"date":"2022-07-18T11:10:51","date_gmt":"2022-07-18T09:10:51","guid":{"rendered":"https:\/\/reseau-amylose.org\/jesuissoignant\/?page_id=5525"},"modified":"2022-07-18T11:38:20","modified_gmt":"2022-07-18T09:38:20","slug":"pour-le-medecin-generaliste","status":"publish","type":"page","link":"https:\/\/reseau-amylose.org\/jesuissoignant\/pour-le-medecin-generaliste\/","title":{"rendered":"Pour le M\u00e9decin G\u00e9n\u00e9raliste"},"content":{"rendered":"\t\t
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Les amyloses cardiaques sont des maladies rares et syst\u00e9miques, entrainant le d\u00e9p\u00f4t de fibrilles amylo\u00efdes dans les organes atteints. Ces organes sont principalement le c\u0153ur, mais \u00e9galement les t\u00e9guments, les reins, le syst\u00e8me nerveux p\u00e9riph\u00e9rique et v\u00e9g\u00e9tatif, tractus gastro-intestinal et le syst\u00e8me ORL. Cette maladie atteint quasi exclusivement les adultes. <\/span><\/h6>
Il existe trois formes d\u2019amyloses cardiaques : AL (\u00e0 chaines l\u00e9g\u00e8res d\u2019immunoglobuline), ATTR sauvage (\u00e0 transthyr\u00e9tine sauvage – ATTRwt, \u00e9galement appel\u00e9e amylose s\u00e9nile), et ATTRv (transthyr\u00e9tine mut\u00e9e – ATTRv). <\/span><\/h6>
Les sympt\u00f4mes peuvent \u00eatre cardiaques et extracardiaques et sont variables d\u2019une forme \u00e0 l\u2019autre et d\u2019un individu \u00e0 l\u2019autre, rendant le diagnostic difficile du fait de l\u2019h\u00e9t\u00e9rog\u00e9n\u00e9it\u00e9 des ph\u00e9notypes. <\/span><\/h6>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t
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Manifestations cardiaques :<\/h5>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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L\u2019infiltration amylo\u00efde cardiaque peut toucher toutes les tuniques cardiaques : myocarde, endocarde (valves), p\u00e9ricarde, et tissu \u00e9lectrique. Ce processus infiltratif est dynamique, et rapidement \u00e9volutif en absence de traitement. <\/span><\/h6>
Le ph\u00e9notype cardiaque d\u00e9pend du degr\u00e9 d\u2019infiltration de ces tuniques cardiaques : \u00e9paississement des parois du c\u0153ur, troubles conductifs ou rythmiques, alt\u00e9ration des fonctions diastolique et systolique. <\/span><\/h6>
L\u2019errance diagnostic est li\u00e9e au fait que les amyloses cardiaques ont les ph\u00e9notypes des syndromes cardiaques suivants : cardiomyopathie hypertrophique (3), insuffisance cardiaque \u00e0 fraction d\u2019\u00e9jection du ventricule gauche pr\u00e9serv\u00e9e, insuffisance cardiaque \u00e0 fraction d\u2019\u00e9jection du ventricule gauche alt\u00e9r\u00e9e avec hypertrophie myocardique, hypertrophie ventriculaire gauche avec BSA3 ou BAV2-3 ou fibrillation atriale ou flutter, r\u00e9tr\u00e9cissement aortique calcifi\u00e9 avec hypertrophie ventriculaire gauche ou bas d\u00e9bit, bas gradient. <\/span><\/h6>
Les sympt\u00f4mes communs aux diff\u00e9rentes formes d\u2019amylose cardiaque, sont ceux de l\u2019insuffisance cardiaque (l\u2019essoufflement, la prise de poids, les \u0153d\u00e8mes et la fatigue (EPOF)) et des troubles du rythme et de la conduction (palpitations, vertige, syncope).<\/span><\/h6>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t
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Manifestations extracardiaques :<\/h5>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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L\u2019infiltration amylo\u00efde peut toucher de nombreux organes. Ces atteintes peuvent pr\u00e9c\u00e9der l\u2019atteinte cardiaque et servir \u00ab red flag \u00bb. <\/span><\/h6>
\u00a0 \u00a0 \u00a0– Atteintes t\u00e9gumentaires : infiltration des canaux carpiens, canal lombaire, canal cervical, maladie de Dupuytren <\/span><\/h6>
\u00a0 \u00a0 \u00a0– Atteintes ORL : surdit\u00e9, dysphagie, macroglossie, dysphonie <\/span><\/h6>
\u00a0 \u00a0 \u00a0– Atteintes dysautonomiques : hypotension art\u00e9rielle, gastropar\u00e9sie, diarrh\u00e9es, constipation, dysfonction urinaire. <\/span><\/h6>
\u00a0 \u00a0 \u00a0– Neuropathie p\u00e9riph\u00e9rique <\/span><\/h6>
\u00a0 \u00a0 \u00a0– Syndrome n\u00e9phrotique <\/span><\/h6>
\u00a0 \u00a0 \u00a0– Alt\u00e9ration de l\u2019\u00e9tat g\u00e9n\u00e9ral, d\u00e9nutrition (perte de poids), fatigue. <\/span><\/h6>
\u00a0 \u00a0 \u00a0– Autres : atteinte h\u00e9patique (h\u00e9patom\u00e9galie, cholestase et cytolyse), atteinte pulmonaire, prostate\u2026<\/span><\/h6>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t
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Conduite \u00e0 tenir par le m\u00e9decin traitant :<\/h5>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t
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La prise en charge des amyloses cardiaques est une urgence car il s\u2019agit d\u2019une pathologie \u00e0 \u00e9volution rapide et encore plus particuli\u00e8rement dans le cas des amyloses AL. <\/span><\/h6>
En cas de suspicion d\u2019amylose cardiaque, le m\u00e9decin traitant ou le cardiologue adressera son patient \u00e0 un des centres de r\u00e9f\u00e9rence ou de comp\u00e9tence de la fili\u00e8re Cardiogen (voir liste en annexe 2 du PNDS ci-dessous).<\/span><\/h6>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t
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Protocole National de Diagnostic et de Soins<\/span><\/strong><\/span><\/h4>
T\u00e9l\u00e9chargez ci-dessous la synth\u00e8se de ce document \u00e0 destination du m\u00e9decin traitant :\u00a0<\/span><\/h6>\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t
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<\/div>\n\t\t\t\t
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\n\t\t\t\t\t\n\t\t\t\t\t\tCliquez ici\t\t\t\t\t<\/span>\n\t\t\t\t\t<\/div>\n\t\t\t\t\t\t\t<\/div>\n\t\t\t\t\t\t<\/a>\n\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t
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\n\t\t\t\t\t\n\t\t\t\t\t\t\n\t\t\t\t\t\t\t\t\tT\u00e9l\u00e9chargez le Protocole National de Diagnostic et de Soins dans son int\u00e9gralit\u00e9 ICI<\/span>\n\t\t\t\t\t<\/span>\n\t\t\t\t\t<\/a>\n\t\t\t\t<\/div>\n\t\t\t\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t<\/div>\n\t\t","protected":false},"excerpt":{"rendered":"

Les amyloses cardiaques sont des maladies rares et syst\u00e9miques, entrainant le d\u00e9p\u00f4t de fibrilles amylo\u00efdes dans les organes atteints. Ces organes sont principalement le c\u0153ur, mais \u00e9galement les t\u00e9guments, les reins, le syst\u00e8me nerveux p\u00e9riph\u00e9rique et v\u00e9g\u00e9tatif, tractus gastro-intestinal et le syst\u00e8me ORL. Cette maladie atteint quasi exclusivement les adultes. Il existe trois formes d\u2019amyloses […]<\/p>\n","protected":false},"author":2,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"footnotes":""},"categories":[],"tags":[],"class_list":["post-5525","page","type-page","status-publish","hentry"],"yoast_head":"\nPour le M\u00e9decin G\u00e9n\u00e9raliste - R\u00e9seau Amylose<\/title>\n<meta name=\"description\" content=\"Amylose : manifestations cardiaques, manifestations extracardiaques, conduite \u00e0 tenir, prise en charge - t\u00e9l\u00e9chargez le PNDS\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/reseau-amylose.org\/jesuissoignant\/pour-le-medecin-generaliste\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Pour le M\u00e9decin G\u00e9n\u00e9raliste - 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