• Military medicine · Oct 2008

    Case Reports

    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.

    • Ronald D Beesley, Randal D Robinson, and Theresa L Stewart.
    • Department of Obstetrics and Gynecology, University of Vermont, Burlington, VT 05401, USA.
    • Mil Med. 2008 Oct 1; 173 (10): 1048-9.

    AbstractWe 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.

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