Porphobilinogen and -Amino Levulinic Acid in Acute Porphyria

Abstract

An acute porphyria patient daily excretes via the urine about 100 mg porphobilinogen and about 40 mg of delta-amino levulinate. The cause of pain does not appear to be due directly to either one of these compounds. On the assumption that acute porphyria is due to a partial block above the porphobilinogen step it would be estimated that the normal liver might produce 100-140 mg heme/day. This heme might then be the source of early labeled stercobilin. One day after intraperitoneal injection of delta-amino levulinate into a rat, a high concentration of protoporphyrin is found in the stomach and duodenum. Suspensions of guinea pig liver cells were found to convert delta-amino levulinate to porphobilinogen and to porphyrins.