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

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24. Hämophilie-Symposion

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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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Literatur

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Authors
  1. T. Beeg
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  2. D. Mentzer
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  3. I. Martinez
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  4. B. Zwinge
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  5. I. Scharrer
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  6. W. Kreuz
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Editors and Affiliations

  1. Abteilung für Angiologie, Zentrum der Inneren Medizin, Klinikum der Johann-Wolfgang-Goethe-Universität, Theodor-Stern-Kai 7, D-60590, Frankfurt am Main, Germany

    Inge Scharrer

  2. Hämostaseologische Abteilung, Medizinische Klinik, Klinikum Innenstadt der Ludwig-Maximilians-Universität, Ziemssenstraße 1a, D-80336, München, Germany

    Wolfgang Schramm

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© 1994 Springer-Verlag Berlin Heidelberg

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Beeg, T., Mentzer, D., Martinez, I., Zwinge, B., Scharrer, I., Kreuz, W. (1994). Homozygoter Protein C-Mangel: Ein Fallbericht — Klinik, Diagnostik und Therapie mit einem Protein C-Konzentrat. In: Scharrer, I., Schramm, W. (eds) 24. Hämophilie-Symposion. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-79009-6_59

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  • DOI: https://doi.org/10.1007/978-3-642-79009-6_59

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-57999-1

  • Online ISBN: 978-3-642-79009-6

  • eBook Packages: Springer Book Archive

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