Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver

Caroline Schmitt; Hugo Lenglet; Angèle Yu; Constance Delaby; Arndt Benecke; Thibaud Lefebvre; Philippe Letteron; Valérie Paradis; Staffan Wahlin; Sverre Sandberg; Pauline Harper; Eliane Sardh; Arne Kristian Sandvik; Johannes R Hov; Aasne Karine Aarsand; Laurence Chiche; Céline Bazille; Jean-Yves Scoazec; Jordi To Figueras; Montserrat Carrascal; Joaquim Abian; Arienne Mirmiran; Zoubida Karim; Jean-Charles Deybach; Hervé Puy; K. Peoc'H; Hana Manceau; Laurent Gouya

doi:10.1111/joim.12750

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Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver

Journal article

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Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver

Caroline Schmitt, Hugo Lenglet, Angèle Yu, Constance Delaby, Arndt Benecke, Thibaud Lefebvre, Philippe Letteron, Valérie Paradis, Staffan Wahlin, Sverre Sandberg, …

Journal of Internal Medicine, Vol.284(1), pp.78-91

07/2018

DOI: https://doi.org/10.1111/joim.12750

PMID: 29498764

Abstract

acute intermittent porphyria

haem oxygenase 1

hemin

inflammation

iron overload

Acute intermittent porphyria (AIP) is an inherited disorder of haem metabolism characterized by life-threatening acute neurovisceral attacks due to the induction of hepatic δ-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 recurrent attacks.

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Title: Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver
Creators - without role: Caroline Schmitt - Inserm
Hugo Lenglet - Centre de Recherche sur l'Inflammation
Angèle Yu - Centre National de la Recherche Scientifique
Constance Delaby - Université de Montpellier, Cell souches plasticité cellul régénérat tissulaire immuno des maladies inflamm - IRMB
Arndt Benecke - Institut des Hautes Études Scientifiques
Thibaud Lefebvre - Hôpital Louis-Mourier
Philippe Letteron - Centre de Recherche sur l'Inflammation
Valérie Paradis - Centre de Recherche sur l'Inflammation
Staffan Wahlin - Karolinska University Hospital
Sverre Sandberg - Haukeland University Hospital
Pauline Harper - Karolinska University Hospital
Eliane Sardh - Karolinska University Hospital
Arne Kristian Sandvik - St Olav's University Hospital
Johannes R Hov - Oslo University Hospital
Aasne Karine Aarsand - Haukeland University Hospital
Laurence Chiche - Department of Hepatobiliary Surgery
Céline Bazille - Centre Hospitalier Universitaire de Caen
Jean-Yves Scoazec - Département d'anatomopathologie, biopathologie
Jordi To Figueras
Montserrat Carrascal - Institut d'Investigacions Biomèdiques de Barcelona
Joaquim Abian - Instituto de Investigaciones Biomedicas de Barcelona
Arienne Mirmiran - Centre de Recherche sur l'Inflammation
Zoubida Karim - Centre de Recherche sur l'Inflammation
Jean-Charles Deybach
Hervé Puy - Hôpital Louis-Mourier
K. Peoc'H - Hôpital Lariboisière
Hana Manceau - Hôpital Beaujon
Laurent Gouya - Hôpital Louis-Mourier
Publication Details: Journal of Internal Medicine, Vol.284(1), pp.78-91
Identifiers: 9935159009311
Academic Unit: Cell souches plasticité cellul régénérat tissulaire immuno des maladies inflamm - IRMB
Language: English
Resource Type: Journal article
Local Fields: hal-02503870