title: The measurement of the synthetic rate of bilirubin from hepatic hemes in patients with acute intermittent porphyria.
abstract: A new method for the direct measurement in vivo of the synthetic rate of bilirubin from hepatic hemes is proposed. This method depends on the application of the labeled precursor-product relationship to the hepatic pool of porphobilinogen, which is a common precursor of both urinary porphobilinogen and hepatic-synthesized bilirubin. The hepatic pool of porphobilinogen is labeled by means of an intravenous injection of delta-aminolevulinic acid-4-(14)C. The proportion of total bilirubin production which is derived from hepatic hemes is calculated from the ratio of the mean (14)C specific activities of stercobilin and porphobilinogen estimated in pooled specimens of feces and urine, respectively. The method can be most readily applied to patients with acute intermittent porphyria, as the appreciable quantities of prophobilinogen in the urine of these patients greatly facilitate the measurement of porphobilinogen-(14)C specific activity. In three patients with acute intermittent porphyria, values obtained for the synthetic rate of bilirubin from hepatic hemes were 20.7, 15.8, and 13.3% of total bilirubin production.
mesh_terms: D000208:Acute Disease; D000328:Adult; D000596:Amino Acids / Q000378:metabolism; D001663:Bilirubin / Q000096:biosynthesis* / Q000097:blood; D002247:Carbon Isotopes; D005260:Female; D006418:Heme / Q000378:metabolism*; D006801:Humans; D007982:Levulinic Acids / Q000378:metabolism; D008099:Liver / Q000378:metabolism*; D008297:Male; D011164:Porphyrias / Q000378:metabolism*; D011422:Propionates / Q000378:metabolism / Q000652:urine; D011758:Pyrroles / Q000378:metabolism / Q000652:urine; D013053:Spectrophotometry; D014316:Tritium
chemical_list: D000596:Amino Acids; D002247:Carbon Isotopes; D007982:Levulinic Acids; D011422:Propionates; D011758:Pyrroles; D014316:Tritium; D006418:Heme; D001663:Bilirubin