{"id":2107,"date":"2018-10-20T18:40:32","date_gmt":"2018-10-20T16:40:32","guid":{"rendered":"https:\/\/angh.net\/abstracts\/optimisation-des-criteres-diagnostiques-et-estimation-de-la-frequence-du-syndrome-lpac\/"},"modified":"2018-11-29T00:06:47","modified_gmt":"2018-11-28T23:06:47","slug":"optimisation-des-criteres-diagnostiques-et-estimation-de-la-frequence-du-syndrome-lpac","status":"publish","type":"post","link":"https:\/\/angh.net\/abstracts\/optimisation-des-criteres-diagnostiques-et-estimation-de-la-frequence-du-syndrome-lpac\/","title":{"rendered":"Optimisation des crit\u00e8res diagnostiques et estimation de la fr\u00e9quence du syndrome LPAC"},"content":{"rendered":"

2018<\/strong><\/p>\n

C. DONG\u00b9, B. CONDAT\u00b2, M. PICON-COSTE\u00b3, P. POTIER\u2074, Y. CHRETIEN\u00b9, B. NOBLINSKI\u00b9, MP. HAUUY\u2075, V. BARBU\u00b9, A. MAFTOUH\u2075, F. GAOUAR\u00b9, C. HOUSSET\u00b9, R. POUPON\u00b9, D. ZANDITENAS\u2075, O. CHAZOUILLERES\u00b9, C. CORPECHOT\u00b9
\n\u00b9Centre de r\u00e9f\u00e9rence des Maladies Inflammatoires des Voies Biliaires et des H\u00e9patites Auto-immunes, H\u00f4pital Saint-Antoine, Paris; \u00b2Service d\u2019H\u00e9pato-Gastroent\u00e9rologie, Centre hospitalier de la Polyn\u00e9sie fran\u00e7aise, Pirae; \u00b3Service d\u2019H\u00e9pato-Gastroent\u00e9rologie, Centre hospitalier d\u2019Aix-en-Provence, Aix-en-Provence; \u2074Service d\u2019H\u00e9pato-Gastroent\u00e9rologie, Centre hospitalier d\u2019Orl\u00e9ans, Orl\u00e9ans; \u2075Service d\u2019H\u00e9pato-Gastroent\u00e9rologie, Centre hospitalier de Bry-sur-Marne, Bry-sur-Marne<\/em><\/p>\n

H\u00e9patologie <\/strong>– \u00a02018-05-14 –\u00a0CO –<\/p>\n

________________________________<\/p>\n

INTRODUCTION
\nLe diagnostic de syndrome LPAC (Low-Phospholipid-Associated Cholelithiasis) repose actuellement sur au moins 2 des crit\u00e8res suivants : 1) D\u00e9but des sympt\u00f4mes biliaires avant l\u2019\u00e2ge de 40 ans, 2) R\u00e9cidive des sympt\u00f4mes apr\u00e8s chol\u00e9cystectomie, 3) Pr\u00e9sence de foyers hyper-\u00e9chog\u00e8nes intra-h\u00e9patiques (\u00ab queues de com\u00e8te \u00bb ou micro-spots). Ces crit\u00e8res ont \u00e9t\u00e9 \u00e9tablis sur un petit nombre de patients et de t\u00e9moins et reposent en grande partie sur la r\u00e9cidive des sympt\u00f4mes apr\u00e8s chol\u00e9cystectomie alors que celle-ci pourrait \u00eatre \u00e9vit\u00e9e si le diagnostic \u00e9tait fait plus t\u00f4t. De plus, ces crit\u00e8res ont \u00e9t\u00e9 \u00e9labor\u00e9s pour pr\u00e9dire l\u2019existence d\u2019une mutation du g\u00e8ne ABCB4, qui n\u2019est observ\u00e9e que chez 30 \u00e0 50% des patients. Enfin, la fr\u00e9quence du syndrome LPAC reste inconnue.
\nMETHODES
\nCette \u00e9tude cas-t\u00e9moin r\u00e9trospective multicentrique a inclus l\u2019ensemble des patients ayant eu un diagnostic de syndrome LPAC selon les crit\u00e8res usuels au sein d\u2019un centre de r\u00e9f\u00e9rence et de plusieurs h\u00f4pitaux g\u00e9n\u00e9raux. Ces patients ont \u00e9t\u00e9 r\u00e9partis en une cohorte d\u2019acquisition (2\/3) et une cohorte de validation (1\/3). La cohorte d\u2019acquisition a \u00e9t\u00e9 compar\u00e9e \u00e0 un groupe t\u00e9moin constitu\u00e9 de patients chol\u00e9cystectomis\u00e9s pour lithiase v\u00e9siculaire banale sur une ann\u00e9e dans un h\u00f4pital g\u00e9n\u00e9ral. Les variables associ\u00e9es au ph\u00e9notype de la maladie ont \u00e9t\u00e9 identifi\u00e9es par un mod\u00e8le de r\u00e9gression logistique ajust\u00e9 sur l\u2019\u00e2ge, le sexe, l\u2019indice de masse corporelle (IMC) et l\u2019existence d\u2019un syndrome m\u00e9tabolique. Les patients ABCB4 mut\u00e9s ont \u00e9t\u00e9 compar\u00e9s aux patients ABCB4 non mut\u00e9s. La fr\u00e9quence de la maladie a \u00e9t\u00e9 estim\u00e9e par comparaison au nombre de patients chol\u00e9cystectomis\u00e9s pour lithiase v\u00e9siculaire pendant la m\u00eame p\u00e9riode sur la base du codage CIM-10 et PSMI.
\nR\u00c9SULTATS
\nAu total, 512 individus, 306 patients et 206 t\u00e9moins, ont \u00e9t\u00e9 inclus. Les patients \u00e9taient majoritairement des femmes (77%) sans surpoids. Deux crit\u00e8res diagnostiques suppl\u00e9mentaires ont \u00e9t\u00e9 identifi\u00e9s (Tableau 2): 1) Les signes (biologiques ou radiologiques) de lithiase de la voie biliaire principale, 2) L\u2019absence de chol\u00e9cystite aigue.<\/p>\n

Tableau 2: Score diagnostique \u00e0 5 crit\u00e8res
\nCrit\u00e8res Points
\nD\u00e9but des sympt\u00f4mes biliaires avant 40 ans + 4
\nR\u00e9cidive de la symptomatologique apr\u00e8s chol\u00e9cystectomie + 5
\nPr\u00e9sence de foyers hyper-\u00e9chog\u00e8nes intra-h\u00e9patiques + 7
\nSignes de lithiase de la voie biliaire principale + 4
\nChol\u00e9cystite aigue – 4<\/p>\n

Un score > 9 permet de porter un diagnostic de certitude. Ce score a une performance diagnostique \u00e9lev\u00e9e (statistique C : 0,99; sensibilit\u00e9: 98%; sp\u00e9cificit\u00e9: 96%).
\nCompar\u00e9s aux patients non mut\u00e9s, les patients ABCB4-mut\u00e9s (45%) avaient un risque significativement plus \u00e9lev\u00e9: 1) de lithiase de la voie biliaire principale (80% vs. 68%), 2) de cholestase gravidique (34% vs. 17%), 3) d\u2019\u00e9l\u00e9vation chronique de la GGT (33% vs. 14%) et des transaminases (16% vs. 8%) et 4) d\u2019ant\u00e9c\u00e9dent personnel ou familial de cancer primitif du foie (8% vs. 1%).
\nSoixante-huit t\u00e9moins (un tiers) ont eu une \u00e9chographie experte \u00e0 la recherche de signes de microlithiase intra-h\u00e9patique 2 ans apr\u00e8s leur chol\u00e9cystectomie : un seul d\u2019entre eux (1,5%) pr\u00e9sentait de nombreux spots et queues de com\u00e8te. Le diagnostic de syndrome LPAC a \u00e9t\u00e9 port\u00e9 chez ce patient devant la persistance de sympt\u00f4mes biliaires.
\nLa fr\u00e9quence relative du syndrome LPAC au sein des patients ayant une lithiase biliaire symptomatique \u00e9tait de 0,5% \u00e0 1,9% dans les centres g\u00e9n\u00e9raux et de 7,5% dans le centre de r\u00e9f\u00e9rence.
\nCONCLUSIONS
\nLe syndrome LPAC touche environ 1% des patients ayant une lithiase biliaire symptomatique. Une lithiase de la voie biliaire principale chez un sujet jeune (< 40 ans) doit syst\u00e9matiquement faire rechercher un syndrome LPAC. Une mutation ABCB4 conf\u00e8re un risque accru de cholestase gravidique, de cholestase chronique et de cancer primitif du foie personnel ou familial.<\/p>\n[download id=\u00a0\u00bb2176″]\n","protected":false},"excerpt":{"rendered":"

2018 C. DONG\u00b9, B. CONDAT\u00b2, M. PICON-COSTE\u00b3, P. POTIER\u2074, Y. CHRETIEN\u00b9, B. NOBLINSKI\u00b9, MP. HAUUY\u2075, V. BARBU\u00b9, A. MAFTOUH\u2075, F. GAOUAR\u00b9, C. HOUSSET\u00b9, R. POUPON\u00b9, D. ZANDITENAS\u2075, O. CHAZOUILLERES\u00b9, C. CORPECHOT\u00b9 \u00b9Centre de r\u00e9f\u00e9rence des Maladies Inflammatoires des Voies Biliaires et des H\u00e9patites Auto-immunes, H\u00f4pital Saint-Antoine, Paris; \u00b2Service d\u2019H\u00e9pato-Gastroent\u00e9rologie, Centre hospitalier de la Polyn\u00e9sie fran\u00e7aise, […]<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[3],"tags":[29],"class_list":["post-2107","post","type-post","status-publish","format-standard","hentry","category-hepatologie","tag-29"],"yoast_head":"\nOptimisation des crit\u00e8res diagnostiques et estimation de la fr\u00e9quence du syndrome LPAC - Abstracts des congr\u00e8s de l'ANGH<\/title>\n<meta name=\"description\" content=\"ANGH R\u00e9sum\u00e9s congr\u00e8s H\u00e9patologie Gastroent\u00e9romogie Hepatology Gastroenterology Etudes cliniques Clinical study\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/angh.net\/abstracts\/optimisation-des-criteres-diagnostiques-et-estimation-de-la-frequence-du-syndrome-lpac\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Optimisation des crit\u00e8res diagnostiques et estimation de la fr\u00e9quence du syndrome LPAC - 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