{"id":16715,"date":"2024-09-18T19:46:37","date_gmt":"2024-09-18T17:46:37","guid":{"rendered":"https:\/\/www.cutislaxa.org\/?p=16715"},"modified":"2024-09-18T19:46:37","modified_gmt":"2024-09-18T17:46:37","slug":"cutis-laxa-quoi-de-neuf","status":"publish","type":"post","link":"https:\/\/www.cutislaxa.org\/fr\/cutis-laxa-quoi-de-neuf\/","title":{"rendered":"Cutis Laxa : Quoi de neuf ?"},"content":{"rendered":"

Mise \u00e0 jour de la Cutis Laxa <\/strong><\/p>\n

Lors du Congr\u00e8s Mondial de Paris au mois de Juin, il a \u00e9t\u00e9 pr\u00e9sent\u00e9 une mise \u00e0 jour des connaissances de la Cutis Laxa.<\/p>\n

 <\/p>\n

\"\"<\/p>\n

La Physiopathologie de la CL est repr\u00e9sent\u00e9e par un d\u00e9faut des fibres \u00e9lastiques. Dans le cas des formes h\u00e9rit\u00e9es, il s\u2019agit d\u2019un d\u00e9faut de synth\u00e8se\/d\u2019assemblage des fibres \u00e9lastiques dont le d\u00e9clencheur est g\u00e9n\u00e9tique. Lorsqu\u2019il s\u2019agit de la forme acquise de Cutis Laxa, le processus est diff\u00e9rent puisqu\u2019il s\u2019agit d\u2019une destruction de fibres \u00e9lastiques qui, \u00e0 l\u2019origine, sont normales.<\/p>\n

Aujourd\u2019hui, 18 sous-types ont \u00e9t\u00e9 identifi\u00e9s dans les formes h\u00e9rit\u00e9es en comptant les maladies li\u00e9es telles que le syndrome de Tortuosit\u00e9 Art\u00e9rielle ou le syndrome de MACS. Ces identifications permettent une nouvelle classification.<\/p>\n

 <\/p>\n

\"\"<\/p>\n

On retrouve donc les formes ayant essentiellement des caract\u00e9ristiques dans le tissu conjonctif ( Tortuosit\u00e9, Peau, Os, Emphys\u00e8me) et celles ayant essentiellement des caract\u00e9ristiques neurologiques avec ou sans retard de croissance intraut\u00e9rin. Cette nouvelle classification est encore en cours de validation. Elle sera un meilleur outil de diagnostic en int\u00e9grant les donn\u00e9es cliniques, les d\u00e9couvertes ultrastructurelles et la physiopathologie. Elle servira de base pour de nouvelles guidelines.<\/p>\n

\"\" \"\"<\/p>\n","protected":false},"excerpt":{"rendered":"

Mise \u00e0 jour de la Cutis Laxa Lors du Congr\u00e8s Mondial de Paris au mois de Juin, il a \u00e9t\u00e9 pr\u00e9sent\u00e9 une mise \u00e0 jour des connaissances de la Cutis Laxa.   La Physiopathologie de la CL est repr\u00e9sent\u00e9e par un d\u00e9faut des fibres \u00e9lastiques. Dans le cas des formes h\u00e9rit\u00e9es, il s\u2019agit d\u2019un d\u00e9faut […]<\/p>\n","protected":false},"author":2,"featured_media":5600,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[67,61,60,66],"tags":[],"class_list":["post-16715","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-photos-fr","category-quoi-de-neuf","category-recherche","category-recherche-medecine-genetique"],"yoast_head":"\nCutis Laxa : Quoi de neuf ? - CUTIS LAXA INTERNATIONALE<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.cutislaxa.org\/fr\/cutis-laxa-quoi-de-neuf\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Cutis Laxa : Quoi de neuf ? - CUTIS LAXA INTERNATIONALE\" \/>\n<meta property=\"og:description\" content=\"Mise \u00e0 jour de la Cutis Laxa Lors du Congr\u00e8s Mondial de Paris au mois de Juin, il a \u00e9t\u00e9 pr\u00e9sent\u00e9 une mise \u00e0 jour des connaissances de la Cutis Laxa.   La Physiopathologie de la CL est repr\u00e9sent\u00e9e par un d\u00e9faut des fibres \u00e9lastiques. 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