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

Abstract : 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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Caroline Schmitt, Hugo Lenglet, Angèle Yu, Constance Delaby, Arndt Benecke, et al.. Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver. Journal of Internal Medicine, Wiley, 2018, 284 (1), pp.78-91. ⟨10.1111/joim.12750⟩. ⟨hal-02503870⟩

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