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Porphyrin-untersuchungen eines gallensteines bei der erythropoetischen protoporphyrie

Erythropoietic protoporphyria: Porphyrin content of a gall-stones

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Summary

In the case of a 48 year old woman with characteristic signs of erythropoetic protoporphyria, a solitary gall-stone which had persisted over a period of 10 years was removed.

Porphyrin-concentration of this gall-stone was found to be 22.6. mcg/g, which is almost twenty times the concentration of other cholestrol-stones. Besides protoporphyrin, porphyrins with a higher degree of carboxylation were discovered in four different samples by thin layer chromatography.

It is postulated that the increased bile-concentration of protoporphyrin in the liver causes the cristallisation of cholestrol and the formation and growth of gall-stones. The biochemical finding of porphyrins with a higher degree of carboxylation indicates that the porphyrins of the gall-stone may not only come from hemolysed erythrocytes but possibly also from metabolites of disturbed hepatic porphyrin biosynthesis.

Zusammenfassung

Es wird eine 48jährige Frau mit dem klinischen und biochemischen Vollbild einer erythropoetischen Protoporphyrie (EPP) berichtet. Anamnestisch war seit über zehn Jahren ein Gallenstein bekannt, der operativ entfernt wurde. Im Mittel betrug der Porphyrin-Gehalt dieses Steins 22.6 mcg/g und lag damit fast 20fach über dem Gehalt anderer Cholesterinsteine. Bei der dünnschichtchromatographischen Auftrennung fanden sich in vier verschiedenen Proben außer Protoporphyrin noch 30 bis 70 % höher carboxylierte Porphyrine. Es wird diskutiert, ob es durch das kontinuierliche Überfluten der Leber mit Protoporphyrin zur Auskristallisation des Cholesterins und damit zur Gallensteinbildung kommen könnte. Die höher carboxylierten Porphyrine (mehr COOH-Gruppen als Protoporphyrin) sprechen dafür, daß nicht nur die Porphyrine aus den haemolysierten Erythrozyten im Gallenstein zu finden sind und somit auch eine hepatische Porphyrin-Stoffwechselstörung zu diskutieren wäre.

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Authors and Affiliations

  1. Universitäts-Hautklinik Düsseldorf, Moorenstraße 5, D-4000, Düsseldorf, Germany

    G. Goerz, Th. Krieg & K. Bolsen

  2. Universitäts-Hautklinik Göttingen, von-Siebold-Straße 3, D-3400, Göttingen, Germany

    S. Seubert & H. Ippen

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  1. G. Goerz
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  2. Th. Krieg
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  3. K. Bolsen
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  4. S. Seubert
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  5. H. Ippen
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Goerz, G., Krieg, T., Bolsen, K. et al. Porphyrin-untersuchungen eines gallensteines bei der erythropoetischen protoporphyrie. Arch. Derm. Res. 256, 283–289 (1976). https://doi.org/10.1007/BF00572494

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