Hematin therapy for acute porphyria.
1. A therapeutic trial of intravenous hematin is presented. Eleven cases of AIP and one of VP who did not improve with conventional treatment (high carbohydrate intake) received this new agent. 2. Urinary ALA, PBG and, when possible, uroporphyrin and coproporphyrin were used to monitor the chemical response to the treatment. Objective clinical parameters of hypertension and tachycardia were followed when present in addition to subjective estimates of acute porphyric symptomatology (abdominal pain, backache, extremity pain and paresthesias, weakness, depression, etc.). 3. At a dosage of approximately 3 mg/kg, diminution of urinary ALA and PBG excretion was achieved in every patients. Hypertension and tachycardia improved in those instances where they were observed in association with the attack. Also, subjective improvements in the clinical status of the patients were observed frequently. 4. Hematin appears to be a promising therapeutic agent for the treatment of acute attack forms of porphyria.
D000208:Acute Disease; D000328:Adult; D000818:Animals; D004341:Drug Evaluation; D005260:Female; D006418:Heme / Q000031:analogs & derivatives*; D006427:Hemin / Q000008:administration & dosage / Q000378:metabolism / Q000627:therapeutic use*; D006801:Humans; D007263:Infusions, Parenteral; D008099:Liver / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000188:drug therapy*
D006418:Heme; D006427:Hemin