title: [Hepatic porphyria: diagnostic and therapeutic strategies].
abstract: Acute hepatic porphyrias are genetic diseases of heme synthesis with severe prognosis due to strong or acute abdominal pain and neurological complications. Clinical evolution is characterized by acute attacks frequently induced by either forbidden drugs, or infections, alcohol intake or often unknown factors. Modern treatment is perfusion of hematin, which is a stable form of heme. Hematin will induce again delta-aminolévulinic (ALA)-synthase synthesis repression. Its tolerance is perfect whereas clinical and biochemical efficiency is absolute in our experience, if initiated very early. This drug is now considered as the treatment of acute intermittent porphyria crises.
authors.affiliation: Service de médecine interne et centre d'allergie, hôpital Louis-Mourier, Colombes, France.
authors.forename: D
authors.initials: D
authors.lastname: Vincent
authors.forename: J F
authors.initials: JF
authors.lastname: Devars du Mayne
authors.forename: J C
authors.initials: JC
authors.lastname: Deybach
authors.forename: A
authors.initials: A
authors.lastname: Pradalier
authors.forename: Y
authors.initials: Y
authors.lastname: Nordmann
mesh_terms: D000208:Acute Disease; D006801:Humans; D017094:Porphyrias, Hepatic / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D013997:Time Factors
vernacular_title: Porphyries hépatiques: stratégies diagnostiques et thérapeutiques.