Hermansky-Pudlak syndrome: correction of bleeding time by 1-desamino-8D-arginine vasopressin

Am J Hematol. 1989 Mar;30(3):154-7. doi: 10.1002/ajh.2830300307.

Abstract

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.

MeSH terms

  • Adolescent
  • Adult
  • Albinism / blood*
  • Albinism / drug therapy
  • Bleeding Time*
  • Blood Coagulation Factors / analysis
  • Blood Platelet Disorders / blood*
  • Blood Platelets / analysis
  • Child
  • Child, Preschool
  • Deamino Arginine Vasopressin / pharmacology*
  • Female
  • Humans
  • Male
  • Platelet Function Tests*
  • Platelet Storage Pool Deficiency / blood*
  • Platelet Storage Pool Deficiency / drug therapy
  • Serotonin / blood
  • Syndrome

Substances

  • Blood Coagulation Factors
  • Serotonin
  • Deamino Arginine Vasopressin