{"id":2097,"date":"2018-10-20T18:40:28","date_gmt":"2018-10-20T16:40:28","guid":{"rendered":"https:\/\/angh.net\/abstracts\/diminution-spectaculaire-des-besoins-de-phlebotomies-apres-introduction-dun-traitement-par-ipp-chez-un-patient-presentant-une-hemochromatose-genetique\/"},"modified":"2018-11-27T00:32:20","modified_gmt":"2018-11-26T23:32:20","slug":"diminution-spectaculaire-des-besoins-de-phlebotomies-apres-introduction-dun-traitement-par-ipp-chez-un-patient-presentant-une-hemochromatose-genetique","status":"publish","type":"post","link":"https:\/\/angh.net\/abstracts\/diminution-spectaculaire-des-besoins-de-phlebotomies-apres-introduction-dun-traitement-par-ipp-chez-un-patient-presentant-une-hemochromatose-genetique\/","title":{"rendered":"Diminution spectaculaire des besoins de phl\u00e9botomies apr\u00e8s introduction d&rsquo;un traitement par IPP chez un patient pr\u00e9sentant une h\u00e9mochromatose g\u00e9n\u00e9tique."},"content":{"rendered":"<p><strong>2018<\/strong><\/p>\n<p><em>J.-F.Cadranel1, C.Mokhtari2, R.Heng1, F.Kazerouni3, M.Medmoun1, T.Delacour2, J.-B.Nousbaum4, A.Garioud1.<\/em><\/p>\n<p>1. Service d&rsquo;H\u00e9pato-gastroent\u00e9rologie, Alcoologie et Nutrition &#8211; GHPSO &#8211; Creil. 2. Service de Biologie m\u00e9dicale &#8211; GHPSO &#8211; Creil. 3. Service d&rsquo;Imagerie m\u00e9dicale &#8211; GHPSO &#8211; Creil. 4. Service d&rsquo;H\u00e9pato-gastroent\u00e9rologie &#8211; CHU La Cavale Blanche &#8211; Brest.<\/p>\n<p><strong>H\u00e9patologie <\/strong>&#8211; \u00a02018-05-09 &#8211;\u00a0CO &#8211;<\/p>\n<p>________________________________<\/p>\n<p>Introduction<br \/>\nL\u2019h\u00e9mochromatose g\u00e9n\u00e9tique est une maladie g\u00e9n\u00e9tique fr\u00e9quente entra\u00eenant une surcharge en fer dans le foie et dans d\u2019autres organes avec une p\u00e9n\u00e9trance variable. Si certains patients n\u2019auront jamais de phl\u00e9botomie, d&rsquo;autres auront un traitement d\u2019attaque astreignant d\u2019une saign\u00e9e par semaine pendant un an voire plus.<br \/>\nNous rapportons ici l\u2019observation d\u2019un patient pr\u00e9sentant une h\u00e9mochromatose g\u00e9n\u00e9tique sans cirrhose avec une surcharge en fer majeure ayant n\u00e9cessit\u00e9 initialement une puis deux saign\u00e9es de grand volume par semaine. L\u2019introduction d\u2019un traitement par inhibiteur de la pompe \u00e0 protons (IPP) a permis d\u2019une part de ramener les saign\u00e9es \u00e0 une par semaine et d\u2019autre part, d\u2019entra\u00eener une diminution spectaculaire de la ferritin\u00e9mie.<br \/>\nObservation :<br \/>\nEn f\u00e9vrier 2017, un homme de 62 ans mesurant 1,75m pour 75kg et ayant pour ant\u00e9c\u00e9dent un accident isch\u00e9mique en 2009 nous est adress\u00e9 en consultation d\u2019H\u00e9patologie pour le bilan d\u2019une h\u00e9mochromatose g\u00e9n\u00e9tique. Il pr\u00e9sente une hypertension art\u00e9rielle trait\u00e9e par hydrochlorothiazide 25\u00b5g\/jour et irb\u00e9sartan 1\/jour. Il prend un traitement par atorvastatine 20 mg\/j et f\u00e9nofibrate 300 mg\/jour. Il boit deux caf\u00e9s par jour et sa consommation d\u2019alcool est nulle.<br \/>\nL\u2019examen clinique est sans particularit\u00e9. Le foie n\u2019est pas augment\u00e9 de volume et n\u2019est pas dur.<br \/>\nBiologiquement, la glyc\u00e9mie \u00e0 jeun est normale, les ASAT \u00e0 1,5N, les ALAT \u00e0 1,2N, la gamma \u00e0 2N, et le bilan lipidique normal. La ferritin\u00e9mie est \u00e0 6.181 ng\/ml avec un coefficient de saturation \u00e0 90 %. Apr\u00e8s recherche g\u00e9n\u00e9tique, la mutation C282Y du g\u00e8ne HFE est pr\u00e9sente \u00e0 l\u2019\u00e9tat homozygote. Le taux de prothrombine et l\u2019\u00e9lectrophor\u00e8se des protides sont normaux. La s\u00e9rologie de l\u2019h\u00e9patite C et l\u2019antig\u00e8n\u00e9mie HBs sont n\u00e9gatifs.<br \/>\nL\u2019IRM h\u00e9patique objective une surcharge en fer majeure \u00e0 331 \u00b5mol\/g de foie ainsi que deux nodules h\u00e9patiques caract\u00e9ris\u00e9s comme angiomes apr\u00e8s relecture par le Professeur VILGRAIN. L\u2019\u00e9lastographie par technique Sheare wave est en faveur d\u2019une fibrose mod\u00e9r\u00e9e \u00e0 8,8 kPa (IQR \u00e0 0,5) superposable au r\u00e9sultat de l\u2019\u00e9lastom\u00e9trie par Fibroscan.<br \/>\nUn traitement par phl\u00e9botomies de 500 ml toutes les semaines est d\u00e9but\u00e9. Entre le 22\/03\/2017 et le fin juin 2017, la ferritin\u00e9mie va passer de 6.000 \u00e0 3.393 ng\/ml. Apr\u00e8s une augmentation \u00e0 deux saign\u00e9es par semaine, on obtient en six mois suppl\u00e9mentaires une diminution fluctuante de la ferritine qui toutefois plafonne \u00e0 1.500 ng\/ml en d\u00e9cembre 2017.<br \/>\nApr\u00e8s lecture d&rsquo;une publication r\u00e9cente*, on d\u00e9cide de repasser \u00e0 une saign\u00e9e hebdomadaire apr\u00e8s introduction d\u2019un traitement par pantoprazole \u00e0 la dose de 40 mg\/j. On assiste \u00e0 partir de ce moment-l\u00e0 \u00e0 une d\u00e9croissance progressive importante et rapide de la ferritin\u00e9mie (figure 1) qui se normalise en quatre mois et est a 103 au 30 06 2018. L\u2019IM h\u00e9patique mets en evidence une st\u00e9tose \u00e015 % et une concentration h\u00e9patique en fer \u00e010\u00b5mol\/g de foie<br \/>\nCette observation souligne l\u2019int\u00e9r\u00eat potentiel des IPP et en particulier du pantoprazole qui diminue l\u2019absorption intestinale du fer par son effet sur l\u2019augmentation du pH gastrique. Actuellement au GHPSO, nous reprenons toutes les observations de patients en phl\u00e9botomies ayant une ferritin\u00e9mie \u00e9lev\u00e9e pour les revoir en consultation et leur proposer l\u2019adjonction d\u2019un traitement par IPP.<br \/>\nPlusieurs centres universitaires se sont joints \u00e0 nous pour une \u00e9tude r\u00e9trospective en cours ( Amiens Brest, Besan\u00e7on, Clermont-Ferrand, Caen, Toulouse h\u00e9pato et Med interne, Limoges) et nous proposons une \u00e9tude r\u00e9trospective au sein de l\u2019ANGH sur ce sujet.<\/p>\n<p>* Proton Pump Inhibitors Decrease Phlebotomy Need in HFE Hemochromatosis: Double-Blind Randomized Placebo-Controlled Trial. A.Vanclooster, C.van Deursen, R.Jaspers, D.Cassiman, and G.Koek. Gastroenterology 2017;153:678\u2013680<\/p>\n<p>Profil d\u2019int\u00e9r\u00eat 0.<\/p>\n[download id=\u00a0\u00bb2171&Prime;]\n","protected":false},"excerpt":{"rendered":"<p>2018 J.-F.Cadranel1, C.Mokhtari2, R.Heng1, F.Kazerouni3, M.Medmoun1, T.Delacour2, J.-B.Nousbaum4, A.Garioud1. 1. Service d&rsquo;H\u00e9pato-gastroent\u00e9rologie, Alcoologie et Nutrition &#8211; GHPSO &#8211; Creil. 2. Service de Biologie m\u00e9dicale &#8211; GHPSO &#8211; Creil. 3. Service d&rsquo;Imagerie m\u00e9dicale &#8211; GHPSO &#8211; Creil. 4. Service d&rsquo;H\u00e9pato-gastroent\u00e9rologie &#8211; CHU La Cavale Blanche &#8211; Brest. H\u00e9patologie &#8211; \u00a02018-05-09 &#8211;\u00a0CO &#8211; ________________________________ Introduction L\u2019h\u00e9mochromatose g\u00e9n\u00e9tique [&hellip;]<\/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-2097","post","type-post","status-publish","format-standard","hentry","category-hepatologie","tag-29"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.2 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Diminution spectaculaire des besoins de phl\u00e9botomies apr\u00e8s introduction d&#039;un traitement par IPP chez un patient pr\u00e9sentant une h\u00e9mochromatose g\u00e9n\u00e9tique. - Abstracts des congr\u00e8s de l&#039;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\/diminution-spectaculaire-des-besoins-de-phlebotomies-apres-introduction-dun-traitement-par-ipp-chez-un-patient-presentant-une-hemochromatose-genetique\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Diminution spectaculaire des besoins de phl\u00e9botomies apr\u00e8s introduction d&#039;un traitement par IPP chez un patient pr\u00e9sentant une h\u00e9mochromatose g\u00e9n\u00e9tique. - 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