Porphyrinurias and occupational disease.
Pathologic porphyrinuria in man is based on a complex etiology and pathogenesis. In hepatic porphyrias, coproporphyrinuria is usually only one of the pathognomostic porphyrin parameters in the urine. Secondary coproporphyrinuria means that an increased excretion of coproporphyrin occurs as the biochemically dominant symptom of a disturbance in porphyrin and heme metabolism during an intoxication, individual condition, or basic disease. Certain foreign and environmental chemicals, such as hexachlorobenzene, polyhalogenated aromatic hydrocarbons, vinyl chloride, and dioxin, alter the heme pathway functionally. Increased porphyrinuria can follow as a toxic response that is differentiated into secondary coproporphyrinuria and chronic hepatic porphyria. This is characterized by a simultaneous increase in hepatic and urinary uroporphyrin and heptacarboxylic porphyrins, owing to inhibition of hepatic uroporphyrinogen decarboxylase. Most of the coproporphyrinurias observed in man are caused by alcohol ingestion. Dioxin, vinyl chloride, and polyhalogenated biphenyls induce an incipient subclinical stage of chronic hepatic porphyria in persons with normal red cell uroporphyrinogen decarboxylase. In contrast, exposure to dioxin on the part of persons with inherited uroporphyrinogen decarboxylase deficiency can cause latent chronic hepatic porphyria to develop into PCT. Coproporphyrinuria and latent chronic hepatic porphyria do not produce clinical symptoms. Secondary porphyrinuria with transition to chronic hepatic porphyria is a metabolic response following various toxic and pathologic conditions; it serves as a sensitive index for chemical exposure and occupational disease.
Doss
M O
MO
Department of Clinical Biochemistry, Faculty of Medicine, University of Marburg, Federal Republic of Germany.
D000435:Alcoholic Intoxication / Q000378:metabolism; D003305:Coproporphyrinogens / Q000378:metabolism; D004147:Dioxins; D006801:Humans; D008107:Liver Diseases / Q000201:enzymology; D009784:Occupational Diseases / Q000201:enzymology; D011164:Porphyrias / Q000201:enzymology; D011166:Porphyrins / Q000652:urine; D014752:Vinyl Chloride
D003305:Coproporphyrinogens; D004147:Dioxins; D011166:Porphyrins; D014752:Vinyl Chloride