Authors: Beesley, Ronald D.¹; Robinson, Randal D.²; Stewart, Theresa L.³

Source: Military Medicine, Volume 173, Number 10, October 2008 , pp. 1048-1049(2)

Publisher: AMSUS - Association of Military Surgeons of the U.S.

Abstract:

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.

Document Type: Research article

Affiliations: 1: Department of Obstetrics and Gynecology, University of Vermont, Bington, VT 05401. 2: Department of Obstetrics and Gynecology, Wilford Hall Medical Center, Lackland Air Force Base, TX 78236. 3: Texas Perinatal Group, San Antonio, TX 78229.

Publication date: 2008-10-01

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• Military Medicine is the Association's official monthly journal. The objective of the Journal is to promote awareness of Federal medicine by providing a forum for responsible discussion of common ideas and problems relevant to Federal healthcare. Its mission is: To increase healthcare education by providing scientific and other information to its readers; to facilitate communication; and to offer a prestige publication for members' writings.
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