1/3. DDAVP (desmopressin; 1-deamino-cys-8-D-arginine-vasopressin) treatment in children with haemophilia B.We tested the response to desmopressin (1-deamino-cys-8-D-arginine-vasopressin; DDAVP) in four patients with haemophilia B [factor ix (F IX) at diagnosis 1.4-5%]. The activated partial thromboplastin time (aPTT) was significantly shortened in all patients. Although there was an up to 1.4-fold increase in F IX levels in three patients, maximal F IX activity remained below 10%. Much more prominent were the increases in F VIII (three- to fourfold), in von willebrand factor antigen (VWF:Ag; 2.5-fold) and particularly in VWF collagen-binding activity (VWF:CBA; fivefold). These changes were reflected by the prophylactic efficacy of DDAVP for dental surgery. After pretesting, DDAVP could be a useful drug for reducing the need for plasma products for prevention of minor surgical bleeding in patients with mild to moderate haemophilia B.- - - - - - - - - - ranking = 1keywords = von (Clic here for more details about this article) |
2/3. Uncontrolled bleeding during endodontic treatment as the first symptoms for diagnosing von Willebrand's disease. A case report.Von Willebrand's disease has been established as the most common hereditary hemorrhagic disease. Endodontic treatment, when possible, rather than extraction is the treatment of choice in patients suffering from such a disease. A case is presented in which, during regular endodontic treatment, uncontrollable bleeding occurred, suggestive of a generalized bleeding disorder. A thorough hematologic examination revealed von Willebrand's disease, which the patient was not aware of. The dental surgeon can play an important role in the diagnosis and treatment of such patients.- - - - - - - - - - ranking = 5keywords = von (Clic here for more details about this article) |
3/3. Clinical effectiveness of desmopressin in a case of acquired von Willebrand's syndrome associated with benign monoclonal gammopathy.A case of acquired von Willebrand's syndrome (avWs) secondary to benign monoclonal gammopathy, is described, in which desmopressin (DDAVP) has proven effective repeatedly in preventing bleeding after tooth extraction. The laboratory pattern was similar to that of congenital type IA von Willebrand's disease. After DDAVP, prolonged bleeding time and factor viii/von willebrand factor activities were normalized. The disappearance rate of the elicited activities was similar to that observed in patients with congenital disease. This report adds to the scarce data concerning the haemostatic effectiveness of DDAVP in avWs and suggests that this agent might also be used in controlling or preventing bleeding in patients with the acquired disease, selected on the basis of their biological responsiveness to a test-infusion.- - - - - - - - - - ranking = 7keywords = von (Clic here for more details about this article) |