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Early Administration of Heme Arginate for Acute Porphyric Attacks

Pertti Mustajoki, MD; Yves Nordmann, MD
Arch Intern Med. 1993;153(17):2004-2008. doi:10.1001/archinte.1993.00410170078008.
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Background:  We investigated the efficacy of early administration of heme arginate in acute porphyric attacks. Methods: The series consisted of 51 consecutive acute attacks in 22 patients with acute intermittent porphyria and in two patients with variegate porphyria referred to a hospital in France or in Finland. Four attacks were associated with pareses, and 47 attacks were not. Heme in a dose of 250 mg or 3 mg/kg was started within 24 hours after admission in 37 (72.5%) of the attacks and within 4 days in 49 (96%) of the attacks. During all except five attacks, four daily infusions were given.

Results:  The mean (±SD) duration of abdominal or nonabdominal pain was 2.5±0.97 days, and opiates were stopped an average of 2.8±0.72 days after the first heme infusion was started. All patients responded. In 46 (90%) of the attacks, the total hospitalization time was 7 days or less. The mean urinary excretion of porphobilinogen decreased to 16.2%±7.7% and that of 5-aminolevulinic acid to 11.6%±5.6% of pretreatment values. The only side effect was moderate thrombophlebitis in one patient.

Conclusion:  Favorable responses in every attack suggest specific effects of heme. In patients with acute attacks, heme therapy should be started immediately on admission.(Arch Intern Med. 1993;153:2004-2008)


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