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March 25, 1961

Acute Intermittent Porphyria: Urinary Porphobilinogen and Other Ehrlich Reactors in Diagnosis

JAMA. 1961;175(12):1087-1091. doi:10.1001/jama.1961.63040120001010

THE TERM porphobilinogen (PBG) was introduced by Waldenström and Vahlquist1 to designate the Ehrlich aldehyde-reacting chromogen which Sachs2 first distinguished from urobilinogen and which characterizes the urine in cases of acute porphyria. The dominantly genetic character of acute intermittent porphyria, as well as its ease of confusion with many other diseases, the protection that may be offered by knowledge of precipitating factors, such as barbiturates, together with newer knowledge about therapy during relapse, emphasize the need of definitive diagnosis.

Historical Background  PBG was first crystallized by Westall3 and its monopyrrolic structure was soon elucidated by Cookson and Rimington.4 Subsequent studies have shown that it is an intermediary in heme biosynthesis, although not in evidence under normal circumstances and only rarely in diseases other than porphyria.In 1941 Watson and Schwartz5 observed that the Ehrlich aldehyde compound of PBG is not extracted by chloroform, in contradistinction