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Faecal and urinary coproporphyrin isomers in biliary atresia and neonatal hepatitis
Insights
Measuring urinary and fecal coproporphyrin levels, especially isomer distribution, can help diagnose severe cholestatic diseases in infants and adults. This method shows significant changes in conditions like biliary atresia and neonatal hepatitis.
Area of Science:
- Biochemistry
- Pediatric Gastroenterology
- Hepatology
Background:
- Biliary function is crucial for coproporphyrin excretion.
- Severe cholestatic diseases significantly impact metabolic pathways.
- Infants and adults with biliary obstruction present unique diagnostic challenges.
Purpose of the Study:
- To investigate coproporphyrin excretion in infants and adults with severe cholestatic diseases.
- To determine if coproporphyrin levels and isomer distribution serve as diagnostic markers.
- To correlate excretion patterns with the extent of biliary dysfunction.
Main Methods:
- Analysis of urinary and fecal coproporphyrin concentrations using solvent partition methods.
- Separation of coproporphyrin isomers (I and III) via thin-layer chromatography.
- Spectrofluorometric scanning for precise isomer quantification.
Main Results:
- Significantly elevated urinary coproporphyrin observed in biliary atresia, neonatal hepatitis, and adults with biliary obstruction.
- A consistent marked increase in urinary isomer I across all studied cholestatic conditions.
- Reduced fecal coproporphyrin and increased bacterial-derived isomer III in biliary atresia and neonatal hepatitis, correlating with disease severity.
Conclusions:
- Urinary and fecal coproporphyrin analysis, particularly isomer profiling, offers a sensitive diagnostic approach for severe cholestatic diseases.
- This method can differentiate between various forms of cholestasis and assess biliary defect extent.
- Coproporphyrin isomer analysis holds potential as a valuable clinical diagnostic tool.
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.