Abstract
Based on the assumption that faecal and urinary coproporphyrin excretion is closely dependent on biliary function, coproporphyrin excretion was investigated in severe cholestatic diseases in infants and adults. The following subjects were investigated: biliary atresia (5), neonatal hepatitis (3), normal infants (11), adults with biliary obstruction (5) and normal adults (18). Urinary and faecal coproporphyrin concentrations were determined by solvent partition methods, and the isomers (I and III) were separated by thin-layer chromatography with direct spectrofluorometric scanning. The results showed a significant increase in urinary coproporphyrin in biliary atresia and neonatal hepatitis and in adults with biliary obstruction. All the cholestatic diseases showed the same marked increase in urinary isomer I. In biliary atresia and neonatal hepatitis there was a significant decrease in faecal coproporphyrin and a concomitant increase in isomer III (of bacterial origin) which was related to the extent of the biliary defect. Determination of urinary and faecal coproporphyrin, and particularly of the isomer distribution, may be a sensitive tool for diagnosis.
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