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[Erythropoietic protoporphyria: porphyrin content of a gall-stone (author's transl)].

作者信息

Goerz G, Krieg T, Bolsen K, Seubert S, Ippen H

出版信息

Arch Dermatol Res (1975). 1976 Oct 27;256(3):283-9. doi: 10.1007/BF00572494.

DOI:10.1007/BF00572494
PMID:984880
Abstract

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.

摘要

相似文献

1
[Erythropoietic protoporphyria: porphyrin content of a gall-stone (author's transl)].
Arch Dermatol Res (1975). 1976 Oct 27;256(3):283-9. doi: 10.1007/BF00572494.
2
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4
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Clinical, Biochemical, and Genetic Characterization of North American Patients With Erythropoietic Protoporphyria and X-linked Protoporphyria.北美红细胞生成性原卟啉症和X连锁原卟啉症患者的临床、生化及遗传学特征
JAMA Dermatol. 2017 Aug 1;153(8):789-796. doi: 10.1001/jamadermatol.2017.1557.
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Erythropoietic protoporphyria.红细胞生成性原卟啉病
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本文引用的文献

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