Repression of the overproduction of porphyrin precursors in acute intermittent porphyria by intravenous infusions of hematin.
In a patient with a severe attack of acute intermittent porphyria, hematin given intravenously caused marked diminution of serum delta-aminolevulinic acid and porphobilinogen. The decline of aminolevulinate was more rapid than that of porphobilinoge. After 2 days of hematin administration, about 5 days were required for delta-aminolevulinic acid, and 11 days for porphobilinogen to return to the concentrations that were detected before treatment. Urinary excretion of both compounds also decreased after hematin administration. Considerable amounts of porphobilinogen were also found in the cerebrospinal fluid of the patient.
D000328:Adult; D000596:Amino Acids / Q000037:antagonists & inhibitors / Q000097:blood / Q000134:cerebrospinal fluid / Q000652:urine; D005260:Female; D006418:Heme / Q000008:administration & dosage*; D006801:Humans; D007275:Injections, Intravenous; D007982:Levulinic Acids / Q000037:antagonists & inhibitors* / Q000097:blood / Q000134:cerebrospinal fluid / Q000652:urine; D011164:Porphyrias / Q000188:drug therapy* / Q000378:metabolism; D011166:Porphyrins / Q000037:antagonists & inhibitors* / Q000097:blood / Q000134:cerebrospinal fluid / Q000652:urine; D011422:Propionates / Q000037:antagonists & inhibitors / Q000097:blood / Q000134:cerebrospinal fluid / Q000652:urine; D011758:Pyrroles / Q000037:antagonists & inhibitors* / Q000097:blood / Q000134:cerebrospinal fluid / Q000652:urine; D013997:Time Factors
D000596:Amino Acids; D007982:Levulinic Acids; D011166:Porphyrins; D011422:Propionates; D011758:Pyrroles; D006418:Heme