Advertisement

Springer Nature is making Coronavirus research free. View research | View latest news | Sign up for updates

Renale und enterale Elimination der Koproporphyrin-Isomeren bei Rotor-Syndrom

Renal and enteral elimination of coproporphyrins in Rotor's syndrome

Eine Familienuntersuchung

  • 22 Accesses

  • 2 Citations

Summary

Urinary and fecal total, isomer I, and isomer III coproporphyrin excretion of a Rotor's syndrome patient and his family were determined. The proprositus showed increased urinary total coproporphyrin excretion (248 µg/24 h) and a shift of the coproporphyrin isomer I/III relation (70%/30%). The propositus's father and two siblings also had elevated renal excretion of coproporphyrin I. Total coproporphyrin excretion was enhanced only in the propositus's father and one sibling, while being normal in another sibling. All family members that could be investigated showed considerably decreased fecal porphyrin excretion. In Rotor's syndrome porphyrin excretion is mainly renal. The coproporphyrin isomer I/III relation is shifted towards isomer I. Phenotypically normal relatives with normal bilirubin plasma levels may have alterations in both their renal and enteral coproporphyrin excretion.

This is a preview of subscription content, log in to check access.

Literatur

  1. 1.

    Caesar J, Saldon S, Chianduss L, Gnevardi L, Sherlock S (1961) The use of indocyanine green in the measurement of hepatic blood flow and as a test of hepatic function. Clin Sci 21:43–57

  2. 2.

    Delage Y, Saigot T, Hadchonel P, Touboul JP, Levy VG, Dhumeaux D (1977) Rotor's Syndrome: Evidence for an impairment of hepatic uptake and storage of cholephilic organic anions. Digestion 15:228 (Abstract)

  3. 3.

    Doss M (1987) Porphyrinstoffwechsel. In: Greilling H, Gressner AM (Hrsg) Lehrbuch der Klinischen Chemie und Pathobiochemie. Schattauer, Stuttgart New York, S 337

  4. 4.

    Erlinger S, Dhumeaux D, Desjeux JF, Benhamon JP (1973) Hepatic handling of unconjugated dyes in the Dubin-Johnson-Syndrome. Gastroenterology 64:106–110

  5. 5.

    Evans J, Lefkowitch J, Lim CK, Billing B (1981) Fecal abnormalities in a patient with features of Rotor's Syndrome. Gastroenterology 81:1125–1130

  6. 6.

    Frank M, Baumann H, Doss M (1987) Dubin-Johnson-Syndrom: Relevanz der Koproporphyrine im Urin. Lab Med 11:190–191

  7. 7.

    Friedmann HC, Baldwin ET (1984) Reverse-phase purification and silica gel thin-layer chromatography of porphyrin carboxylic acids. Anal Biochem 137:473–480

  8. 8.

    Jacob K, Sommer W, Meyer HD, Vogt W (1985) Ion-pair high-performance liquid chromatographic separation of porphyrin isomers. J Chromatogr 349:283–293

  9. 9.

    Kaplowitz N, Javitt N, Kappas J (1972) Coproporphyrin I and III excretion in bile and urine. J Clin Invest 51:2895–2899

  10. 10.

    Kondo T, Kuchiba K, Shimizu Y (1976) Coproporphyrin isomers in Dubin-Johnson-Syndrome. Gastroenterology 70:1117–1120

  11. 11.

    Lim CK, Li F, Peters TJ (1986) Clinical application of an advanced automated sample processor. Internat Clin Prod Rev 7/8:34–39

  12. 12.

    Martin JF, Vierling JM, Wolkoff AW, Scharschmidt BF, Vergella J, Waggoner JG, Berk PD (1976) Abnormal hepatic transport of indocyanine green in Gilbert's syndrome. Gastroenterology 70:385–391

  13. 13.

    Namihisa T, Yamaguchi K, Nambu M (1973) The constitutional conjugated hyperbilirubinemia. Gastroenterol Jpn 8:217–222

  14. 14.

    Rapaccini GL, Topi GC, Anti M, D'Allessandro Gandolfol, Griso D, Amantea A, de Vitis J, Fedeli G (1986) Porphyrins in Rotor's Syndrome: A Study on an Italian Family. Hepato-gastroenterol 33:11–13

  15. 15.

    Shimizu Y, Narnto H, Ida S, Kokura M (1981) Urinary coproporphyrin isomers in Rotor's Syndrome: A study in eight families. Hepatol 1:173–178

  16. 16.

    Stiel D, Lunzer M, Poulos V (1982) Urinary coproporphyrin excretion in Rotor's Syndrome: A family study. Aust NZJ Med 12:594–597

  17. 17.

    Wolkoff AW, Wolpert E, Pascasio FN, Arias JM (1976) Rotor's Syndrome: A distinct inheritable pathophysiologic entity. Am J Med 60:173–179

  18. 18.

    Wolpert E, Pascasio FN, Wolkoff AW, Arias JM (1977) Abnormal sulfobromphthalein metabolism in Rotor's syndrome and obligate heterozygotes. NEJM 296:1099–1101

Download references

Author information

Additional information

Herrn Professor Dr. med. N. Zöllner zum 65. Geburtstag gewidmet

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Kellner, H., Zoller, W.G., Jacob, K. et al. Renale und enterale Elimination der Koproporphyrin-Isomeren bei Rotor-Syndrom. Klin Wochenschr 66, 953–956 (1988). https://doi.org/10.1007/BF01738109

Download citation

Key words

  • Hyperbilirubinemia
  • Rotor's syndrome
  • Coproporphyrin isomers