Variable response of Hermansky-Pudlak syndrome to prophylactic administration of 1-desamino 8D-arginine in subsequent pregnancies.
Hermansky-Pudlak syndrome is characterised by oculocutaneous albinism and haemorrhagic diathesis. The bleeding tendency that is associated with this autosomal recessive disease is caused by storage-pool deficiency and has been reported to be controllable by prophylactic administration of 1-desamino 8D-arginine (desmopressin, DDAVP). The DDAVP prophylaxis at the first delivery of our patient did not prevent the severe haemorrhagic sequeal requiring transfusion of packed red cells and platelets, but the same preventive measure was successful at her second childbirth. Response to prophylactic DDAVP administration varies between as well as within patients with Hermansky-Pudlak syndrome.
Department of Obstetrics and Gynecology, Medical Faculty, Center for Health and Medical Sciences, University of Debrecen, Debrecen, Hungary. jzatik@yahoo.com
János
J
Zatik
Róbert
R
Póka
Antal
A
Borsos
György
G
Pfliegler
D000293:Adolescent; D002585:Cesarean Section; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D017707:Erythrocyte Transfusion; D005260:Female; D022861:Hermanski-Pudlak Syndrome / Q000150:complications*; D006801:Humans; D017713:Platelet Transfusion; D019106:Postoperative Hemorrhage / Q000517:prevention & control / Q000628:therapy; D006473:Postpartum Hemorrhage / Q000209:etiology* / Q000517:prevention & control*; D011247:Pregnancy; D011248:Pregnancy Complications
D003894:Deamino Arginine Vasopressin