Anesthesia and the porphyrias.
A simplified classification of the porphyrias is given which is thought to be advantageous to the anesthesiologist in determining those patients who are predisposed to acute attacks. These acute attacks may be precipitated by the administration of barbiturates, but may also be spontaneous. The current theory for the precipitation of the acute attack is described, with the probable mechanism being a decrease in uroporphyrinogen synthetase levels and the resultant interference in heme production. Increased formation of cytochrome  P-450 with barbiturates also produces increased levels of delta aminolevulinic acid, which may be a cause of the acute attack. The significance in anesthesia and suggested means of anesthetic management are discussed.
D000624:5-Aminolevulinate Synthetase / Q000378:metabolism; D000208:Acute Disease; D000622:Aminolevulinic Acid; D000642:Ammonia-Lyases / Q000172:deficiency; D000758:Anesthesia / Q000009:adverse effects*; D000777:Anesthetics / Q000009:adverse effects*; D002648:Child; D002707:Chlordiazepoxide / Q000009:adverse effects; D003577:Cytochrome P-450 Enzyme System / Q000378:metabolism; D003711:Demyelinating Diseases / Q000209:etiology; D003975:Diazepam / Q000009:adverse effects; D006418:Heme / Q000096:biosynthesis; D006801:Humans; D009388:Neostigmine / Q000009:adverse effects; D010423:Pentazocine / Q000009:adverse effects; D011162:Porphobilinogen / Q000378:metabolism; D011164:Porphyrias / Q000139:chemically induced* / Q000145:classification / Q000378:metabolism; D013874:Thiopental / Q000009:adverse effects
D000777:Anesthetics; D009388:Neostigmine; D006418:Heme; D002707:Chlordiazepoxide; D011162:Porphobilinogen; D000622:Aminolevulinic Acid; D003577:Cytochrome P-450 Enzyme System; D000624:5-Aminolevulinate Synthetase; D000642:Ammonia-Lyases; D013874:Thiopental; D003975:Diazepam; D010423:Pentazocine