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.
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University of Freiburg, Department of Paediatrics, Freiburg, Germany.
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D000293:Adolescent; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D003814:Dentistry, Operative; D005164:Factor IX / Q000378:metabolism; D005169:Factor VIII / Q000378:metabolism; D002836:Hemophilia B / Q000150:complications* / Q000188:drug therapy; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D006472:Oral Hemorrhage / Q000209:etiology* / Q000517:prevention & control; D010314:Partial Thromboplastin Time; D019106:Postoperative Hemorrhage / Q000209:etiology / Q000517:prevention & control; D011292:Premedication; D014841:von Willebrand Factor / Q000378:metabolism
D006490:Hemostatics; D014841:von Willebrand Factor; D005169:Factor VIII; D005164:Factor IX; D003894:Deamino Arginine Vasopressin