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Pertti Mustajoki, MD; Yves Nordmann, MD

Arch Intern Med. 1993;153(17):2004-2008.

Abstract
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)

Author Affiliations
From the Third Department of Medicine, University of Helsinki (Finland) (Dr Mustajoki), and Centre Français des Porphyries, Hôpital Louis Mourier, Colombes, France (Dr Nordmann).

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