title: [Anesthesia and hepatic porphyria].
abstract: Three of the acute hepatic porphyrias, acute intermittent porphyria, variegata porphyria and hereditary coproporphyria, are characterized by an idiosyncratic reaction to many common drugs; the resulting excessive excretion of porphyrin precursors is responsible for episodes of acute neurological dysfunction. This review aimed to focus the attention of the anaesthesiologist on the porphyrinogenic properties of all the drugs used in anaesthesia and intensive care. An outline of the chemistry of porphyrins and the enzymatic pathways were recalled, so as to place the acute porphyrias in their proper perspective. There follows a reminder of the clinical aspect of acute porphyric crises. The part played by drugs is then assessed from clinical and laboratory data concerning their porphyrinogenicity. Those drugs for which there is conflicting evidence regarding their safe use in porphyric patients are discussed: propofol, ketamine, benzodiazepines, etomidate, local anaesthetics. Recommendations supported by clinical and experimental data are given, especially the results obtained with the chick embryo liver model. Treatment of the acute crisis is provided, with particular emphasis on the use of haematin. The anaesthetic management of hepatic porphyric patients is described. Those drugs which are well-known porphyrinogenic compounds in the chick embryo liver must be excluded from use for anaesthesia in the porphyric patients, even if they have been observed to be innocuous in rare cases of asymptomatic patients. Finally, recommendations for anaesthesia in symptomatic cutanea porphyria are given.
authors.affiliation: Département d'Anesthésie-Réanimation, Hôpital Laënnec, CHU, Nantes.
authors.forename: Y
authors.initials: Y
authors.lastname: Blanloeil
authors.forename: J C
authors.initials: JC
authors.lastname: Deybach
authors.forename: D
authors.initials: D
authors.lastname: Portier
authors.forename: M
authors.initials: M
authors.lastname: Joyau
authors.forename: Y
authors.initials: Y
authors.lastname: Nordmann
mesh_terms: D000443:Alcuronium / Q000009:adverse effects; D000768:Anesthesia, General / Q000379:methods*; D000777:Anesthetics / Q000009:adverse effects; D006418:Heme / Q000096:biosynthesis; D006801:Humans; D008107:Liver Diseases; D009022:Morphine Derivatives / Q000009:adverse effects; D011164:Porphyrias / Q000139:chemically induced / Q000628:therapy; D011166:Porphyrins / Q000032:analysis; D012871:Skin Diseases / Q000209:etiology
chemical_list: D000777:Anesthetics; D009022:Morphine Derivatives; D011166:Porphyrins; D006418:Heme; D000443:Alcuronium
vernacular_title: Anesthésie et porphyries hépatiques.