Two successful vaginal births after cesarean section in a patient with Hermansky-Pudlak syndrome who was treated with 1-deamino-8-arginine-vasopression during labor.
We describe the obstetric management for a patient with Hermansky-Pudlak syndrome (HPS) and a previous cesarean delivery. The disease is characterized by oculocutaneous albinism, platelet storage dysfunction, and lipofuscin deposits in the reticuloendothelial system. Patients with the disorder are at high risk for major morbidity secondary to bleeding complications. The patient was a 22-year-old military spouse from Puerto Rico with HPS and a history of severe hemorrhage during cesarean delivery of her first child. In this report, we discuss the pathophysiologic features of HPS and the prophylactic administration of 1-deamino-8-arginine-vasopression during labor to minimize blood loss.
Beesley
Ronald D
RD
Department of Obstetrics and Gynecology, University of Vermont, Burlington, VT 05401, USA.
Robinson
Randal D
RD
Stewart
Theresa L
TL
D000328:Adult; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use*; D022861:Hermanski-Pudlak Syndrome / Q000503:physiopathology*; D006801:Humans; D006473:Postpartum Hemorrhage / Q000188:drug therapy*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000517:prevention & control; D016064:Vaginal Birth after Cesarean
D006490:Hemostatics; D003894:Deamino Arginine Vasopressin