Hermansky‐pudlak syndrome: Correction of bleeding time by 1‐desamino‐8D‐arginine vasopressin

  title={Hermansky‐pudlak syndrome: Correction of bleeding time by 1‐desamino‐8D‐arginine vasopressin},
  author={Pierre W. Wijermans and D B van Dorp},
  journal={American Journal of Hematology},
The effect of the synthetic vasopressin derivative 1‐desamino‐8D‐arginine vasopressin (DDAVP = desmopressin) on bleeding time was studied in three patients with Hermansky Pudlak syndrome. A good response was observed in this type of storage pool disease. DDAVP might be useful in managing the bleeding disorder found in patients with the Hermansky‐Pudlak syndrome. 
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Use of DDAVP in inherited and acquired platelet dysfunction
The bleeding time response did not correlate with changes in the levels of von Willebrand factor (vWf) antigen or ristocetin cofactor activity, nor was it associated withChanges in vWf multimeric analysis or in vitro platelet aggregations following the administration of DDAVP.
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Effect of 1-deamino-8-D-arginine vasopressin on the prolonged bleeding time in chronic renal failure.
The prolonged bleeding times of 12 patients with chronic renal failure were significantly shortened at 1 and 2 h after an infusion of 0.4 microgram/kg 1-deamino-8-D-arginine vasopressin (DDAVP), suggesting that the effect of DDAVP is a temporary one.
Deamino-8-D-arginine vasopressin shortens the bleeding time in uremia.
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Factor VIII and Fibrinolytic Response to Deamino‐8‐d‐Argenine Vasopressin in Normal Subjects and Dissociate Response in Some Patients with Haemophilia and von Willebrand's Disease
Summary Deamino‐8‐d‐argenine vasopressin (DDAVP) was given by intravenous infusion to normal subjects, haemophiliacs and patients with von Willebrand's disease (vWd) and the factor VIII and
Correction of the Bleeding Time in Patients with Storage Pool Deficiency by Infusion of Cryoprecipitate
On four occasions infusion of cryoprecipitate twice daily protected patients with Hermansky‐Pudlak syndrome and storage pool deficiency from bleeding during surgery.
Controlled trial of desmopressin in liver cirrhosis and other conditions associated with a prolonged bleeding time
The findings indicate that DDAVP might be given when biopsies or other surgical procedures must be carried out in patients with prolonged BT, however, the compound is often ineffective in Patients with thrombocytopenia or congenital platelet dysfunction.
Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes
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The Platelet Defect Associated with Albinism
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