Erworbenes von-Willebrand-Syndrom bei Kindern mit hämodynamisch relevantem Vitium

 

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Zusammenfassung

Das erworbene von-Willebrand-Syndrom (VWS) ist eine seltene Gerinnungsstörung im Rahmen anderer (z. B. kardiovaskulärer) Erkrankungen, die bei operativen oder interventionellen Eingriffen zu starken Blutungen führen kann. Zur Verdachtsdiagnose führen die prädisponierende Grunderkrankung, die leere Blutungsanamnese und die pathologisch veränderten von-Willebrand-Faktor-Parameter. Wir berichten über zwei Patienten mit kongenitalen Vitien und negativer Blutungsanamnese, bei denen präoperativ durch erweiterte Gerinnungsdiagnostik die Diagnose eines erworbenen VWS gestellt wurde.

Summary

Acquired von Willebrand syndrome is a rare bleeding disorder associated with other primary diseases such as cardiovascular disorders which can cause severe haemorrhage during surgery or intervenional procedures. It should be suspected if there is no history of bleeding and abnormal von Willebrand factor (VWF) values in patients with predisposing disorders.

We present two children with congenital heart defects and no personal or family history of bleeding. In these patients we preoperative diagnosed acquired von Willebrand syndrome with coagulation tests including analysis of the VWF multimeric pattern.

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