[Acute attacks of hepatic porphyria. Treatment with hematin. 5 cases].
The effectiveness of 2 hematins administered by intravenous infusion was compared in acute intermittent porphyria. Judging from subjective symptoms (abdominal pain), clinical improvement was complete and constant. There was a rapid decrease in urinary excretion of porphyrins precursors, with a clearer response of delta-aminolevulinic acid than of porphobilinogen. Urine levels of uroporphyrin often returned to normal after two infusions. The drugs were very well tolerated. Provided it is administered early, before neurological complications develop, treatment with hematin completely relieves abdominal symptoms and suppresses most of the biochemical changes associated with hepatic porphyria.
D000208:Acute Disease; D000328:Adult; D006418:Heme / Q000031:analogs & derivatives*; D006427:Hemin / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007262:Infusions, Intravenous; D008107:Liver Diseases / Q000188:drug therapy*; D011164:Porphyrias / Q000188:drug therapy*
D006418:Heme; D006427:Hemin
Crises aiguës de porphyries hépatiques. Traitement par l'hématine. Cinq observations.