Acute hepatic porphyrias. Detection, prophylaxis and treatment.

Abstract

Between 1962 and 1944, 300 families with acute hepatic porphyrias were seen at the Porphyria Centre in Warsaw. Among members of these families, 443 overt and 707 latent cases were diagnosed, and 211 persons had intermediate values of enzyme activity without clinical symptoms and biochemical changes in urine and stool. Three dual porphyrias were detected. Five children with acute symptoms were also seen. The patients and their relatives were registered and routine prophylactic measures were introduced. The treatment of the attacks was based on glucose and hemearginate infusions, a high carbohydrate diet and maintenance of body homeostasis. As a late consequence of the attack acute intermittent porphyria arterial hypertension was observed.