Effects of hematin in hepatic porphyria. Further studies.
The present study was carried out in five cases of hepatic porphyria, including three of acute intermittent porphyria, one of variegate porphyria, and one of porphyria cutanea tarda in clinical remission. In two cases of acute intermittent porphyria (in relapse), a marked lowering effect on serum and urine porphobilinogen and delta-aminolevulinic acic was observed, together with prompt and gratifying clinical improvement. In a third case, in chemical remission but with longstanding psychoneurosis, no significant effects were noted, nor were any observed in the case of porphyria cutanea tarda. Although clinical improvements occurred in the case of variegate porphyria, the results were inconclusive for reasons given. Hematin was generally well tolerated. Preliminary reference is made to a transitory renal injury, without sequelae, where an excess of hematin was given in relation to time. Limits of tolerance are proposed. In the light of these observations the basic mechanism of the acute attack is diccussed.
D000328:Adult; D000622:Aminolevulinic Acid / Q000378:metabolism; D005260:Female; D006418:Heme / Q000031:analogs & derivatives*; D006427:Hemin / Q000009:adverse effects / Q000494:pharmacology* / Q000627:therapeutic use; D006801:Humans; D007263:Infusions, Parenteral; D008099:Liver / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D011162:Porphobilinogen / Q000378:metabolism; D011164:Porphyrias / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism*; D011166:Porphyrins / Q000378:metabolism; D012008:Recurrence
D011166:Porphyrins; D006418:Heme; D006427:Hemin; D011162:Porphobilinogen; D000622:Aminolevulinic Acid