title: Response to low-dose desmopressin by a subcutaneous route in children with type 1 von Willebrand disease.
abstract: OBJECTIVE
  The primary objective of this study was to determine responses to low-dose desmopressin (DDAVP) by a subcutaneous route in children with type 1 VWD.
  
  
  METHODS
  This study analyzed responses to low doses of DDAVP administered by a subcutaneous route to 14 children between the ages of 3 and 16 with type 1 VWD and a personal and familial history of bleeding. At 0 (baseline) and 1 hour (initial response) after the subcutaneous injection of DDAVP, the vital signs were assessed and blood samples were obtained for VWD panel determinations (VWF:Ag, VWF:RCo, FVIII:C levels, and Col/Epi, Col/ADP). At 4 hours (sustained response), only Col/Epi and Col/ADP were assessed.
  
  
  RESULTS
  The DDAVP mean (min-max range, μg/kg) based on the patient's weight was 0.15 (0.12-0.18). Laboratory values mean (min-max range in U/dl) baseline for VWF:RCo, VWF:Ag, and FVIII:C were 28 (20-36), 34 (25-42), and 40 (29-48), respectively. After a subcutaneous administration, the laboratory values mean (min-max range in U/dl(-1)) achieved for 1 hour for VWF:RCo, VWF:Ag, and FVIII:C were 109 (72-144), 132 (88-166), and 151 (96-198), respectively. PFA 100(®) CT (Col/Epi <134 seconds and Col/ADP <110 seconds) returned to normal values at 1 and 4 hours after a subcutaneous administration.
  
  
  CONCLUSIONS
  Subcutaneous low-dose DDAVP therapy is at least effective as 0.3 µg/kg intravenous therapy for children with type 1 VWD. This study shows that a wider use of DDAVP should be promoted, especially in developing countries.
authors: Department of Pediatric Hematology, Denizli State Hospital, Denizli, Turkey.
authors: Mehmet
authors: M
authors: Akin
mesh_terms: D000293:Adolescent; D001772:Blood Cell Count; D002648:Child; D002675:Child, Preschool; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D005169:Factor VIII / Q000032:analysis; D005260:Female; D006490:Hemostatics / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007279:Injections, Subcutaneous; D008297:Male; D010314:Partial Thromboplastin Time; D011517:Prothrombin Time; D016896:Treatment Outcome; D056725:von Willebrand Disease, Type 1 / Q000097:blood* / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D006490:Hemostatics; D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin