Haematin therapy and leucocyte delta-aminolevulinic-acid-synthase activity in prolonged attack of acute porphyria.
The activity of leucocyte delta-aminolaevulinic-acid (A.L.A.) synthase was monitored throughout a prolonged attack of acute  intermittent porphyria in a 29-year-old women. Clinical severity was associated with a pronounced increase in activity of this enzyme and high urinary excretion of A.L.A. Haematin therapy resulted in clinical improvement associated with a reduction in the activity of A.L.A. synthase and reduction in urinary excretion of A.L.A.
D000624:5-Aminolevulinate Synthetase / Q000097:blood*; D000208:Acute Disease; D000328:Adult; D000622:Aminolevulinic Acid / Q000652:urine; D004334:Drug Administration Schedule; D005260:Female; D006418:Heme / Q000031:analogs & derivatives*; D006427:Hemin / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007263:Infusions, Parenteral; D007962:Leukocytes / Q000201:enzymology*; D010366:Peak Expiratory Flow Rate; D011164:Porphyrias / Q000188:drug therapy / Q000378:metabolism* / Q000503:physiopathology; D012119:Respiration / Q000187:drug effects; D013997:Time Factors
D006418:Heme; D006427:Hemin; D000622:Aminolevulinic Acid; D000624:5-Aminolevulinate Synthetase