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Homozygoter Protein C-Mangel: Ein Fallbericht — Klinik, Diagnostik und Therapie mit einem Protein C-Konzentrat

  • T. Beeg
  • D. Mentzer
  • I. Martinez
  • B. Zwinge
  • I. Scharrer
  • W. Kreuz
Conference paper

Zusammenfassung

Der homozygote Protein C-Mangel stellt eine der seltensten und schwersten thrombophilen Koagulopathien dar. Protein C wird physiologischerweise an dem endothelzellständigen Oberflächenrezeptor Thrombomodulin in Anwesenheit von Thrombin aktiviert [5]. Die antithrombophilen Eigenschaften ergeben sich einerseits aus der proteolytischen Spaltung der aktivierten Faktoren V und VIII, andererseits wird durch Inhibition von Plasminogenaktivator-Inhibitor 1 (PAI-I) die Plasminkonzentration und damit das fibrinolytische Potential gesteigert [8].

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Copyright information

© Springer-Verlag Berlin Heidelberg 1994

Authors and Affiliations

  • T. Beeg
  • D. Mentzer
  • I. Martinez
  • B. Zwinge
  • I. Scharrer
  • W. Kreuz

There are no affiliations available

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