Literature DB >> 984880

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

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

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.

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Year:  1976        PMID: 984880     DOI: 10.1007/bf00572494

Source DB:  PubMed          Journal:  Arch Dermatol Res            Impact factor:   3.017


  35 in total

1.  ERYTHROPOIETIC PROTOPORPHYRIA. A NEW TYPE OF INBORN ERROR OF METABOLISM.

Authors:  B HAEGER-ARONSEN
Journal:  Am J Med       Date:  1963-10       Impact factor: 4.965

2.  [Light hypersensitivity and porphyrinemia].

Authors:  W KOSENOW; A TREIBS
Journal:  Z Kinderheilkd       Date:  1953

3.  Porphyrin chromogens or precursors in urine, blood, bile, and feces.

Authors:  C J WATSON; R PIMENTA de MELLO; S SCHWARTZ; V E HAWKINSON; I BOSSENMAIER
Journal:  J Lab Clin Med       Date:  1951-06

4.  Cutaneous changes in the porphyrias. A microscopic study.

Authors:  J H Epstein; D L Tuffanelli; W L Epstein
Journal:  Arch Dermatol       Date:  1973-05

5.  -Carotene as a treatment for photohypersensitivity due to erythropoietic protoporphyria.

Authors:  H Baart de la Faille; D Suurmond; L N Went; J van Steveninck; A A Schothorst
Journal:  Dermatologica       Date:  1972

6.  Erythrocyte protoporphyrin and iron uptake in erythropoietic protoporphyria.

Authors:  K G Clark; D C Nicholson
Journal:  Clin Sci       Date:  1971-10       Impact factor: 6.124

7.  Beta-carotene as a photoprotective agent in erythropoietic protoporphyria.

Authors:  M M Mathews-Roth; M A Pathak; T B Fitzpatrick; L C Harber; E H Kass
Journal:  N Engl J Med       Date:  1970-05-28       Impact factor: 91.245

8.  [Normal values for the concentrations of porphyrins and porphyrin precursors].

Authors:  L Goreczky; I Róth; M Breckner
Journal:  Z Klin Chem Klin Biochem       Date:  1968-11

9.  [Analysis of the porphyrins of erythrocytes].

Authors:  M Doss; U Bode
Journal:  Z Klin Chem Klin Biochem       Date:  1968-09

Review 10.  Bile composition, bile salt metabolism and gallstones.

Authors:  R N Redinger; D M Small
Journal:  Arch Intern Med       Date:  1972-10
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  2 in total

1.  Clinical, Biochemical, and Genetic Characterization of North American Patients With Erythropoietic Protoporphyria and X-linked Protoporphyria.

Authors:  Manisha Balwani; Hetanshi Naik; Karl E Anderson; D Montgomery Bissell; Joseph Bloomer; Herbert L Bonkovsky; John D Phillips; Jessica R Overbey; Bruce Wang; Ashwani K Singal; Lawrence U Liu; Robert J Desnick
Journal:  JAMA Dermatol       Date:  2017-08-01       Impact factor: 10.282

Review 2.  Erythropoietic protoporphyria.

Authors:  T M Cox
Journal:  J Inherit Metab Dis       Date:  1997-06       Impact factor: 4.982

  2 in total

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