The Hermansky-Pudlak syndrome. Variable reaction to 1-desamino-8D-arginine vasopressin for correction of the bleeding time.
The effect of the synthetic vasopressin derivative 1-desamino-8D-arginine vasopressin (DDAVP = Minrin) on bleeding time was studied in nine patients with Hermansky-Pudlak syndrome; four of them were Dutch, five were Belgian. Shortening of bleeding time was observed in four of the patients with this type of storage pool disease; in one patient the response was equivocal, in two patients the response was not dramatic and in two there was no response at all. DDAVP may be useful in managing the bleeding disorder in some patients with Hermansky-Pudlak syndrome. Therefore, every patient with this syndrome should be tested with DDAVP as a preventive measure.
Van Dorp
D B
DB
Department of Ophthalmology, Free University, Amsterdam, The Netherlands.
Wijermans
P W
PW
Meire
F
F
Vrensen
G
G
D000293:Adolescent; D000328:Adult; D016115:Albinism, Oculocutaneous / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D001760:Bleeding Time; D002648:Child; D002675:Child, Preschool; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D005169:Factor VIII / Q000187:drug effects; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D010974:Platelet Aggregation / Q000187:drug effects; D010976:Platelet Count / Q000187:drug effects; D010981:Platelet Storage Pool Deficiency / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology
D005169:Factor VIII; D003894:Deamino Arginine Vasopressin