title: [Desmopressin testing in children with von Willebrand syndrome in haemostaseologic centers of Saxonia, Saxonia-Anhalt and Thuringia].
abstract: The influence of desmopressin on hemostasis is mediated by the release of von Willebrand factor and of coagulation factor VIII from vascular endothelium. The necessity of testing desmopressin effectiveness on hemostasis is a matter of controversy and the performance of the test is not yet standardized. For this reason the desmopressin tests in 114 children with von Willebrand syndrome (type 1, n=98; type 2A, n=12; type 2M, n=2; type 2N, n=2) carried out in 7 paediatric haemostaseologic centers were retrospectively analyzed. The effectiveness of desmopressin was assessed using defined response criteria. As expected, the test performance showed a wide variation among the centers. In 99 children desmopressin was given intravenously as a short infusion at a dosage ranging from 0.25 to 0.41 microg/kg and in 15 intranasally at an absolute dose of 40 to 300 microg. The points of time for blood taking after desmopressin application ranged from 0.5 to 12 h. The absent desmopressin response in 7 patients (6%) and the partial response in 15 indicate the necessity of testing desmopressin effectiveness before the first therapeutic use. The application of desmopressin was well tolerated by the patients.
authors.affiliation: Klinik und Poliklinik für Kinder- und Jugendmedizin, Bereich Pädiatrische Hämatologie, Onkologie und Hämostaseologie Universitätsklinikum Carl Gustav Carus, Fetscherstr. 74, 01307 Dresden.
authors.forename: B
authors.initials: B
authors.lastname: Huhn
authors.forename: A
authors.initials: A
authors.lastname: Hofmann
authors.forename: K
authors.initials: K
authors.lastname: Hofmann
authors.forename: H
authors.initials: H
authors.lastname: Sirb
authors.forename: V
authors.initials: V
authors.lastname: Aumann
authors.forename: K
authors.initials: K
authors.lastname: Kentouche
authors.forename: A
authors.initials: A
authors.lastname: Sauerbrey
authors.forename: D
authors.initials: D
authors.lastname: Franke
authors.forename: E
authors.initials: E
authors.lastname: Kuhlisch
authors.forename: R
authors.initials: R
authors.lastname: Knöfler
mesh_terms: D000281:Administration, Intranasal; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage* / Q000494:pharmacology / Q000627:therapeutic use; D005260:Female; D005858:Germany; D006487:Hemostasis / Q000187:drug effects; D006490:Hemostatics / Q000008:administration & dosage* / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007260:Infusion Pumps; D008297:Male; D012189:Retrospective Studies; D013997:Time Factors; D014842:von Willebrand Diseases / Q000188:drug therapy*
chemical_list: D006490:Hemostatics; D003894:Deamino Arginine Vasopressin