6533b7dcfe1ef96bd127278b

RESEARCH PRODUCT

Von Willebrand-Syndrom - Blutungsrisiko bei HNO-Eingriffen im Kindesalter

Herbert RiechelmannOhler WKeller M

subject

congenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtybiologymedicine.diagnostic_testbusiness.industryPlasma factorStimulationGastroenterologySurgeryOtorhinolaryngologyVon Willebrand factorVon willebrandhemic and lymphatic diseasesInternal medicinebiology.proteinMedicineRisk factorbusinessReduced factor VIII activityComplicationcirculatory and respiratory physiologyPartial thromboplastin time

description

Von Willebrand's disease (vWD) is the most common inherited bleeding disorder. Typical clinical features such as bleeding after surgery or trauma might suggest the disease. We present a series of 24 patients with vWD treated between 1989 and 1992. Diagnosis was confirmed by a reduction in plasma factor VIII antigen concentration, reduction of ristocetin cofactor activity and reduced factor VIII activity. Seventeen of the patients underwent surgery (7 adenoidectomies, 8 tonsillectomies, 2 paranasal sinus operations) and received preoperative stimulation of von Willebrand factor (vWF) using DDAVP. This resulted in a rapid increase in plasma vWF concentration from an average of 56% before stimulation to 190% of the normal value after stimulation. A reduction of partial thromboplastin time from an average of 44.4 seconds to 34.4 seconds was observed following DDAVP. No bleeding complications or other side-effects occurred. Preoperative stimulation of vWF using DDAVP proved to be a safe method to reduce the risk of bleeding in patients with vWD undergoing surgery.

https://doi.org/10.1055/s-2007-997145