In a double-blind study 12 patients were randomized 2 days after admission in attack to receive intravenous haem arginate 3 mg/kg per 24 h for 4 days or placebo 1. 9 patients were readmitted with a further attack and were given the alternative treatment. With haem arginate the median PBG excretion of the 9 patients with two attacks (normal range 0 - 16 mumol per 24 h) fell significantly from 332 mumol per 24 h (range 137 - 772) on admission to a median lowest level of 40 (range 22 - 105). On placebo, median PBG excretion was 382 (range 196 - 542) on admission, falling to 235 (range 128 - 427). Median duration of admission after the start of treatment was 11 days (range 2 - 28) for placebo and 8 days (3 - 26) for haem arginate. Median total analgesic requirement between the start of treatment and discharge was 8 150 mg pethidine equivalents (range 0 - 17 650) with placebo versus 6 425 (range 50 - 20 650) with haem arginate.
In an uncontrolled study 2 51 consecutive acute porphyria attacks in 22 patients were treated with heme in a dose of 250 mg or 3 mg/kg within 24 hours after admission in 37 (72.5%) of the attacks and within 4 days in 49 (96%) of the attacks. The mean 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.
Comment: The quality of evidence is downgraded by sparse data.
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