Hermansky-Pudlak syndrome: correction of bleeding time by 1-desamino-8D-arginine vasopressin.
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.
Wijermans
P W
PW
Department of Hematology, Free University Hospital, Amsterdam, The Netherlands.
van Dorp
D B
DB
D000293:Adolescent; D000328:Adult; D000417:Albinism / Q000097:blood* / Q000188:drug therapy; D001760:Bleeding Time; D001779:Blood Coagulation Factors / Q000032:analysis; D001791:Blood Platelet Disorders / Q000097:blood*; D001792:Blood Platelets / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003894:Deamino Arginine Vasopressin / Q000494:pharmacology*; D005260:Female; D006801:Humans; D008297:Male; D010979:Platelet Function Tests; D010981:Platelet Storage Pool Deficiency / Q000097:blood* / Q000188:drug therapy; D012701:Serotonin / Q000097:blood; D013577:Syndrome
D001779:Blood Coagulation Factors; D012701:Serotonin; D003894:Deamino Arginine Vasopressin