[Erythropoietic protoporphyria: porphyrin content of a gall-stone (author's transl)].
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.
D002769:Cholelithiasis / Q000209:etiology* / Q000378:metabolism; D005260:Female; D006801:Humans; D008099:Liver / Q000378:metabolism; D008875:Middle Aged; D011164:Porphyrias / Q000150:complications* / Q000378:metabolism; D011166:Porphyrins / Q000032:analysis*; D011524:Protoporphyrins / Q000032:analysis
D011166:Porphyrins; D011524:Protoporphyrins
Porphyrin-Untersuchungen eines Gallensteines bei der erythropoetischen Protoporphyrie