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July 1970

Stool Porphyrins in Acute Intermittent and Hereditary Coproporphyria: Adverse Effect of Tranquilizers

Arch Neurol. 1970;23(1):80-84. doi:10.1001/archneur.1970.00480250084012

SYMPTOMS of acute intermittent porphyria may be associated with three forms of hepatic porphyria: acute intermittent, variegate, and hereditary coproporphyria. Acute intermittent porphyria (AIP) occurs more frequently in women, with an incidence of approximately 1 per 100,000 and is associated with intermittent episodes of acute abdominal pain, neurological disorders, and psychiatric disturbances.1 Urinary excretion of aminolevulinic acid (ALA) and porphobilinogen (PBG) is increased during attacks, but may remain normal in remission, and the stool porphyrins are usually normal.2 Variegate porphyria is more common in South Africa, where more than 5,000 cases have been detected among the Boer population. Variegate porphyria is associated with similar acute intermittent symptoms in addition to clinical features of photosensitivity that are similar to porphyria cutanea tarda, with bullae and ulceration on the sun exposed areas of the skin. Urinary ALA and PBG are increased during acute intermittent episodes but are usually normal