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June 1946

ACUTE PORPHYRIA: I. Investigations on the Pathology of the Porphyrins and Identification of the Excretion of Uroporphyrin I

Author Affiliations


From the Department of Pathology, St. Thomas's Hospital Medical School.

Arch Intern Med (Chic). 1946;77(6):623-642. doi:10.1001/archinte.1946.00210410029003

IT IS now well recognized that porphyria (so-called hematoporphyrinuria [Garrod,1 1923]) is a condition characterized by two main clinical types, the acute iodiopathic and the congenital, each possessing its characteristic symptomatology. In addition, there is recognized a toxic type due to various drugs, such as sulfonmethane, trinitrotoluene and sulfonamide compounds (Rimington and Hemmings,2 1939) which resembles clinically the acute idiopathic form. The classic case of congenital porphyria, studied in detail from the chemical and histochemical aspects by Fischer and his school and by Borst and Königsdörffer3 (1929), was that of the man Petry. This work established a pathologic excretion of coproporphyrin in urine and feces, together with the appearance of pathologic porphyrin and uroporphyrin in the urine. These porphyrins were in the main type I porphyrin isomers, in contrast to the physiologically occurring type III protoporphyrin of the blood. Rimington4 (1936) carried out a detailed examination

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