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Pädiatrie up2date 2014; 09(03): 231-244
DOI: 10.1055/s-0034-1377341
Hämatologie/Onkologie
© Georg Thieme Verlag KG Stuttgart · New York

Angeborene Gerinnungsstörungen in der Pädiatrie

Johannes Schelling
,
Barbara Zieger
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Publication Date:
19 August 2014 (online)

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Fazit

Angeborene Gerinnungsstörungen fallen nicht immer durch eine Blutungsneigung im Alltag auf. Häufig werden sie erst aufgrund von Nachblutungen nach Eingriffen entdeckt. Mittels Quick und aPTT kann eine Gerinnungsstörung nicht komplett ausgeschlossen werden. Eine Abklärung bei Verdacht auf eine Gerinnungsstörung sollte zumindest die von-Willebrand-Faktor-Parameter (Faktor VIII-Aktivität, vWF-Antigen, vWF-CBA oder -Aktivität) und die Faktor XIII-Aktivität einschließen. Eine Thrombozytenfunktionsdiagnostik wäre ebenfalls wünschenswert, leider steht diese nur an einzelnen Zentren zur Verfügung. Die Behandlung von Patienten mit Gerinnungsstörungen sollte in Absprache mit Hämostaseologen erfolgen.

 

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