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<p>Hemophilia A and B as well as von Willebrand diseases account for 95–97% of all congenital blood coagulation disorders and the rare factor deficiency conditions (factors I, II, V, VII, X, XI, XIII) account for the remaining 3–5%. The clinical presentation varies considerably, whereby the correlation between factor activity and bleeding phenotype is only insufficiently expressed, especially for factor VII deficiency. Pregnancy represents a special challenge as the physiological alterations of hemostasis can compensate for the underlying defect to different extents. These heterogeneous dynamics require an individualized monitoring, which also includes the individual history of bleeding as the central instrument of risk stratification, in addition to factor activities. From an anesthesiological perspective two core aspects are prioritized, the safe performance of neuraxial anesthesia procedures and the management of peripartum hemorrhage.</p>

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Hereditäre Hämostasestörungen in der Geburtshilfe 2/2 – anästhesiologische Aspekte der sekundären Hämostase

  • 期刊论文

  • 发表日期：

    2026年7月22日

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  • F. Fette,
  • F. Roll,
  • P. Kranke,
  • J. Koscielny,
  • K. Zacharowski,
  • Christian F. Weber,
  • M.-L. Lindner

摘要

Hemophilia A and B as well as von Willebrand diseases account for 95–97% of all congenital blood coagulation disorders and the rare factor deficiency conditions (factors I, II, V, VII, X, XI, XIII) account for the remaining 3–5%. The clinical presentation varies considerably, whereby the correlation between factor activity and bleeding phenotype is only insufficiently expressed, especially for factor VII deficiency. Pregnancy represents a special challenge as the physiological alterations of hemostasis can compensate for the underlying defect to different extents. These heterogeneous dynamics require an individualized monitoring, which also includes the individual history of bleeding as the central instrument of risk stratification, in addition to factor activities. From an anesthesiological perspective two core aspects are prioritized, the safe performance of neuraxial anesthesia procedures and the management of peripartum hemorrhage.