{"id":13401,"date":"2019-03-13T17:40:45","date_gmt":"2019-03-13T16:40:45","guid":{"rendered":"http:\/\/cyber.pixilie.fr\/?post_type=publications&p=13401"},"modified":"2025-09-11T16:48:04","modified_gmt":"2025-09-11T14:48:04","slug":"recurrent-attacks-of-acute-hepatic-porphyria-major-role-of-the-chronic-inflammatory-response-in-the-liver","status":"publish","type":"publication","link":"https:\/\/cri1149.fr\/en\/publication\/recurrent-attacks-of-acute-hepatic-porphyria-major-role-of-the-chronic-inflammatory-response-in-the-liver\/","title":{"rendered":"Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver."},"content":{"rendered":"","protected":false},"featured_media":0,"template":"","meta":{"_acf_changed":false,"_EventAllDay":false,"_EventTimezone":"","_EventStartDate":"","_EventEndDate":"","_EventStartDateUTC":"","_EventEndDateUTC":"","_EventShowMap":false,"_EventShowMapLink":false,"_EventURL":"","_EventCost":"","_EventCostDescription":"","_EventCurrencySymbol":"","_EventCurrencyCode":"","_EventCurrencyPosition":"","_EventDateTimeSeparator":"","_EventTimeRangeSeparator":"","_EventOrganizerID":[],"_EventVenueID":[],"_OrganizerEmail":"","_OrganizerPhone":"","_OrganizerWebsite":"","_VenueAddress":"","_VenueCity":"","_VenueCountry":"","_VenueProvince":"","_VenueState":"","_VenueZip":"","_VenuePhone":"","_VenueURL":"","_VenueStateProvince":"","_VenueLat":"","_VenueLng":"","_VenueShowMap":false,"_VenueShowMapLink":false},"category_publication":[],"class_list":["post-13401","publication","type-publication","status-publish","hentry"],"acf":{"numero_de_publication":"J Intern Med.","date_de_publication":"20180701","numero_doi":"","equipe":[124],"auteurs-liste":[{"texte_libre":false,"auteur-lien":13115,"auteur-text":""},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Lenglet H"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Yu A"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Delaby C"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Benecke A"},{"texte_libre":false,"auteur-lien":13104,"auteur-text":""},{"texte_libre":false,"auteur-lien":13106,"auteur-text":""},{"texte_libre":false,"auteur-lien":13135,"auteur-text":""},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Wahlin S"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Sandberg S"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Harper P"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Sardh E"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Sandvik AK"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Hov JR"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Aarsand AK"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Chiche L"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Bazille C"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Scoazec JY"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"To-Figueras J"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Carrascal M"},{"texte_libre":true,"auteur-lien":null,"auteur-text":"Abian J"},{"texte_libre":false,"auteur-lien":13203,"auteur-text":""},{"texte_libre":false,"auteur-lien":13094,"auteur-text":""},{"texte_libre":false,"auteur-lien":13060,"auteur-text":""},{"texte_libre":false,"auteur-lien":13142,"auteur-text":""},{"texte_libre":false,"auteur-lien":13139,"auteur-text":""},{"texte_libre":false,"auteur-lien":13113,"auteur-text":""},{"texte_libre":false,"auteur-lien":13080,"auteur-text":""}],"auteurs-manuel":"","liens_externes":null,"liens":[{"lien":"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/29498764"}],"paragraphe":"

Abstract<\/h3>\r\n
\r\n

BACKGROUND:<\/h4>\r\n

Acute<\/span>\u00a0intermittent\u00a0porphyria<\/span>\u00a0(AIP) is an inherited disorder of haem metabolism characterized by life-threatening\u00a0acute<\/span>\u00a0neurovisceral\u00a0attacks<\/span>\u00a0due to the induction of\u00a0hepatic<\/span>\u00a0\u03b4-aminolevulinic acid synthase 1 (ALAS1) associated with hydroxymethylbilane synthase (HMBS) deficiency. So far, the treatment of choice is hemin which represses ALAS1. The main issue in the medical care of AIP patients is the occurrence of debilitating\u00a0recurrent<\/span>\u00a0attacks<\/span>.<\/p>\r\n

OBJECTIVE:<\/h4>\r\n

The aim of this study was to determine whether chronic hemin administration contributes to the recurrence of\u00a0acute<\/span>\u00a0attacks<\/span>.<\/p>\r\n

METHODS:<\/h4>\r\n

A follow-up study was conducted between 1974 and 2015 and included 602 French AIP patients, of whom 46 had\u00a0recurrent<\/span>\u00a0AIP. Moreover, we studied the\u00a0hepatic<\/span>\u00a0transcriptome, serum proteome, liver macrophage polarization and oxidative and inflammatory profiles of Hmbs-\/-<\/sup>\u00a0mice chronically treated by hemin and extended the investigations to five explanted livers from\u00a0recurrent<\/span>\u00a0AIP patients.<\/p>\r\n

RESULTS:<\/h4>\r\n

The introduction of hemin into the pharmacopeia has coincided with a 4.4-fold increase in the prevalence of chronic patients. Moreover, we showed that both in animal model and in human liver, frequent hemin infusions generate a chronic inflammatory\u00a0hepatic<\/span>\u00a0disease which induces HO1 remotely to hemin treatment and maintains a high ALAS1 level responsible for recurrence.<\/p>\r\n

CONCLUSION:<\/h4>\r\n

Altogether, this study has important impacts on AIP care underlying that hemin needs to be restricted to severe neurovisceral crisis and suggests that alternative treatment targeting the liver such as ALAS1 and HO1 inhibitors, and anti-inflammatory therapies should be considered in patients with\u00a0recurrent<\/span>\u00a0AIP.<\/p>\r\n<\/div>","paragraphe_en":"","documents":null},"yoast_head":"\nRecurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver. - CRI<\/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:\/\/cri1149.fr\/publication\/recurrent-attacks-of-acute-hepatic-porphyria-major-role-of-the-chronic-inflammatory-response-in-the-liver\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver. - 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