Klinische Wochenschrift

, Volume 66, Issue 19, pp 953–956 | Cite as

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

Eine Familienuntersuchung
  • H. Kellner
  • W. G. Zoller
  • K. Jacob
  • H. S. Füeßl
Originalien
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Renal and enteral elimination of coproporphyrins in Rotor's syndrome

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.

Key words

Hyperbilirubinemia Rotor's syndrome Coproporphyrin isomers 

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Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • H. Kellner
    • 1
  • W. G. Zoller
    • 1
  • K. Jacob
    • 2
  • H. S. Füeßl
    • 1
  1. 1.Medizinische Poliklinik der Universität MünchenGermany
  2. 2.Institut für Klinische Chemie Klinikum GroßhadernUniversität MünchenGermany

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