Abstract |
The urinary distribution and relative proportions of the four coproporphyrin isomers I-IV were investigated in 50 patients suffering from hepatic and erythropoietic types of hereditary porphyrias. A highly efficient sample preparation method was applied to isolate urinary coproporphyrins, the isomer ratios of which were quantitated by isocratic ion-pair high-performance liquid chromatography. Results showed a significant decrease (p < 0.001) of the proportion of coproporphyrin I in acute hepatic porphyria ( acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, porphobilinogen synthase deficiency porphyria) as compared with chronic hepatic porphyria ( porphyria cutanea tarda, chronic hepatic porphyria type B and C) (13.2 +/- 5.3%, mean +/- S.D., vs. 31.4 +/- 11.5%). Conversely, the proportion of isomer III was significantly higher (p < 0.001) in acute hepatic porphyria than in chronic hepatic porphyria (80.9 +/- 5.2% vs. 62.2 +/- 10.9%). As expected, the highest level of coproporphyrin I (90.0 +/- 1.9%) was found in congenital erythropoietic porphyria. The atypical coproporphyrins II and IV were detected in all types of porphyria analysed and ranged from 0.2 to 9.0%; no significant differences were seen between acute and chronic hepatic porphyrias. The diagnostic importance of the isomer ratios of coproporphyrins I and III has been confirmed in our study, while the significance of the atypical coproporphyrin isomers II and IV is still unclear at present.
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Authors | K Jacob, M O Doss |
Journal | European journal of clinical chemistry and clinical biochemistry : journal of the Forum of European Clinical Chemistry Societies
(Eur J Clin Chem Clin Biochem)
Vol. 31
Issue 10
Pg. 617-24
(Oct 1993)
ISSN: 0939-4974 [Print] Germany |
PMID | 8292661
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
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Topics |
- Adult
- Chromatography, High Pressure Liquid
- Coproporphyrins
(chemistry, urine)
- Diagnosis, Differential
- Female
- Humans
- Isomerism
- Male
- Porphyrias
(classification, diagnosis, urine)
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