TREATMENT WITH HEMATIN IN ACUTE HEPATIC PORPHYRIA

  • 1 January 1981
    • journal article
    • research article
    • Vol. 50  (198) , 161-174
Abstract
The use of i.v. hematin in the treatment of 13 attacks of acute porphyria is reported in 8 patients. Seven patients had acute intermittent porphyria and 1 variegate porphyria. Peripheral neuropathy was a feature of 9 of the attacks and in 2 the neuropathy necessitated assisted ventilation. The hematin lowered the urinary excretion of porphyrins and precursors in all patients by .apprx. 50% of the prehematin values. It repressed the activity of .delta.-aminolevulinic acid synthase, the rate-controlling enzyme of heme biosynthesis, in peripheral leukocytes in 7 of the 9 patients in whom it was monitored. The clinical response was less consistent, with clinical improvement accompanying only 1/2 of the courses. The 2 patients with respiratory paralysis died. There was localized phlebitis at the injection site after 5 of the courses but no other side effects were noted. Previously published reports of hematin therapy for acute porphyria are reviewed. These consist of 45 courses in 32 patients. Biochemical improvement was a consistent finding. Clinical response was less consistent. Of the courses, 24 were associated with sustained improvement, 10 with temporary improvement, relapse occurring within 2-14 days (3 fatal). In 11 there was no improvement and 3 died.