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Koagulopathien

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Pädiatrische Hämatologie und Onkologie

Part of the book series: Springer Reference Medizin ((SRM))

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Zusammenfassung

Das hämostaseolgische Gleichgewicht ist das Resultat fein abgestimmter prokogulatorischer, inhibitorischer und fibrinolytischer Faktoren. Für die Einordnung der Defekte der plasmatischen Gerinnung ist eine effiziente Diagnostik mit Bestimmung von Thromboplastinzeit, aktivierter partieller Thromboplastinzeit und Thrombinzeit wesentlich. Die Hämophilie ist eine seltene, X-chromosomal vererbte Erkrankung, die auf einem Mangel von Faktor VIII (Hämophilie A) oder IX (Hämophilie B) beruht. Sie wird in drei Schweregrade eingeteilt und kann zu schwerwiegenden Blutungen und bleibenden Einschränkungen führen. Die häufigste hereditäre hämorrhagische Diathese ist die v.-Willebrand-Erkrankung mit einer Prävalenz von bis zu 1:100. Sie beruht auf erblichen quantitativen und qualitativen Defekten des v.-Willebrand-Faktors (vWF) und weist eine ausgeprägte klinische und laborchemische Heterogenität auf. Mit dem Begriff v.-Willebrand-Syndrom werden auch die erworbenen Formen eingeschlossen. Die Kenntnis hereditärer und externer Thrombophiliefaktoren hat zum besseren Verständnis der Pathophysiologie von arteriellen und venösen Gefäßverschlüssen wie z. B. der Sinusvenenthrombose wesentlich beigetragen.

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Author information

Authors and Affiliations

  1. Universitätsklinikum Hamburg-Eppendorf, Klinik für Pädiatrische Hämatologie, Martinistr. 52, 20246, Hamburg, Deutschland

    Prof. Dr. rer. nat. R. Schneppenheim

  2. Universitätsklinikum Freiburg, Zentrum für Kinder-/Jugendmedizin, Mathildenstrasse 1, 79106, Freiburg, Deutschland

    Prof. Dr. med. B. Zieger

  3. Kinderklinik und Kinderpoliklinik, Dr. von Haunersches Kinderspital, Lindwurmstr. 4, 80337, München, Deutschland

    PD Dr. med. C. Bidlingmaier

  4. Kinderklinik im Dr. v Haunerschen Kinderspital, Klinikum der Universität München, Lindwurmstr. 4, 80337, München, Deutschland

    PD Dr. med. K. Kurnik

  5. Laboratoriumsmedizin/Hämostaseologie,, amedes MVZ wagnerstibbe für, Georgstraße 50, 30159, Hannover, Deutschland

    Dr. med. F. Bergmann

Authors
  1. Prof. Dr. rer. nat. R. Schneppenheim
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  2. Prof. Dr. med. B. Zieger
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  3. PD Dr. med. C. Bidlingmaier
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  4. PD Dr. med. K. Kurnik
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  5. Dr. med. F. Bergmann
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Corresponding authors

Correspondence to R. Schneppenheim , B. Zieger , C. Bidlingmaier , K. Kurnik , F. Bergmann , R. Schneppenheim , B. Zieger , C. Bidlingmaier , K. Kurnik , R. Schneppenheim , F. Bergmann or R. Schneppenheim .

Editor information

Editors and Affiliations

  1. Universitätsklinik um Freiburg Zentrum für Kinder- und Jugendmedizin, Freiburg, Deutschland

    Charlotte Niemeyer

  2. Charité-Universitätsmedizin Berlin, Berlin, Deutschland

    Angelika Eggert

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Schneppenheim, R., Zieger, B., Bidlingmaier, C., Kurnik, K., Bergmann, F. (2018). Koagulopathien. In: Niemeyer, C., Eggert, A. (eds) Pädiatrische Hämatologie und Onkologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43686-8_8

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  • DOI: https://doi.org/10.1007/978-3-662-43686-8_8

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-43685-1

  • Online ISBN: 978-3-662-43686-8

  • eBook Packages: Medicine (German Language)

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