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  • Title: Response to low-dose desmopressin by a subcutaneous route in children with type 1 von Willebrand disease.
    Author: Akin M.
    Journal: Hematology; 2013 Mar; 18(2):115-8. PubMed ID: 23321055.
    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. CONCLUSION: 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.
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