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.
AuthorsRonald D Beesley, Randal D Robinson, Theresa L Stewart
JournalMilitary medicine (Mil Med) Vol. 173 Issue 10 Pg. 1048-9 (Oct 2008) ISSN: 0026-4075 [Print] United States
PMID19160629 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Hemostatics
  • Deamino Arginine Vasopressin
  • Adult
  • Deamino Arginine Vasopressin (therapeutic use)
  • Female
  • Hemostatics (therapeutic use)
  • Hermanski-Pudlak Syndrome (physiopathology)
  • Humans
  • Postpartum Hemorrhage (drug therapy)
  • Pregnancy
  • Pregnancy Complications (prevention & control)
  • Vaginal Birth after Cesarean

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