The Treatment of Acute Intermittent Porphyria with Laevulose

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Abstract
The activities of the enzymes of heme biosynthesis were studied in 23 patients with acute intermittent porphyria. The mitochondrial enzymes .delta.-aminolevulinate synthase, coproporphyrinogen oxidase and ferrochelatase were measured in leukocytes and the cytosolic enzymes .delta.-aminolevulinate dehydratase, porphobilinogen deaminase and uroporphyrinogen decarboxylase in erythrocytes. Leukocyte .delta.-aminolevulinate synthase activity was elevated (P < 0.001), with marked diminution of porphobilinogen deaminase activity (P < 0.001) and reduction in the activities of .delta.-aminolevulinate dehydratase (P < 0.01) and uroporphyrinogen decarboxylase (P < 0.005). A therapeutic regimen based on i.v. levulose infusion was studied. In 4 patients in acute attack and 1 in remission levulose treatment was associated with a fall in .delta.-aminolevulinate synthase activity, a rise in porphobilinogen deaminase and uroporphyrinogen decarboxylase activities, and a fall in urinary porphyrin precursor excretion (P < 0.001). These studies provide a basis for the evaluation of the use of sugars in acute intermittent porphyria.