[Acute intermittent porphyria in pregnancy: glucose or hematin therapy?]

The symptoms and management of acute intermittent porphyria (AIP) during pregnancy and delivery are presented on the basis of three case reports. Two patients became asymptomatic after infusion of a concentrated glucose solution (600 ml 50% glucose = 300 g glucose per day), while the third patient needed treatment with hematin (1 mg/kg body weight, 6 infusions at intervals of 12 hours) because of deterioration of psychic state in spite of glucose infusion. Whenever an attack of AIP occurs, concentrated glucose solution should be administrated as a first measure accompanied by an analgesic and possibly a neuroleptic agent after elimination of precipitating factors. However, if symptoms persist for 48-96 hours or the psychic and/or neurological state of the patient further deteriorates, administration of 1-3 mg hematin per kg body weight can be recommended.