title: [Von Willebrand syndrome--risk of hemorrhage in ENT interventions in childhood].
abstract: 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.
authors.affiliation: Hals-, Nasen-, Ohrenklinik, Universität Mainz.
authors.forename: H
authors.initials: H
authors.lastname: Riechelmann
authors.forename: M
authors.initials: M
authors.lastname: Keller
authors.forename: W
authors.initials: W
authors.lastname: Ohler
mesh_terms: D000293:Adolescent; D000328:Adult; D001780:Blood Coagulation Tests; D016063:Blood Loss, Surgical / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008297:Male; D010038:Otorhinolaryngologic Diseases / Q000097:blood / Q000601:surgery*; D011292:Premedication; D014842:von Willebrand Diseases / Q000097:blood / Q000601:surgery*
chemical_list: D003894:Deamino Arginine Vasopressin
vernacular_title: Von Willebrand-Syndrom--Blutungsrisiko bei HNO-Eingriffen im Kindesalter.