title: Medical and Surgical Management of Postpartum Hemorrhage in a Woman with Factor XIII Deficiency.
abstract: Factor XIII deficiency is a rare inherited coagulopathy. Factor XIII is the last clotting factor in the coagulation cascade to insure strength and stability to fibrin clots. Without this enzyme, the fibrous clot is unstable and nonresistant to fibrinolysis. Gravid women with this congenital disease are especially at risk for complications including miscarriages and hemorrhage without appropriate interventions. We present a case of a woman in her 20s with Factor XIII deficiency who was treated with cryoprecipitate and had a successful normal spontaneous vaginal delivery; subsequently, patient suffered from postpartum hemorrhage and consumptive coagulopathy due to consumption of Factor XIII, requiring emergency surgical intervention. Intraoperative management was challenged by an ethical dilemma involving the patient's religious beliefs about not receiving blood. This paper will discuss the mechanism of Factor XIII and the medical and surgical management involved with this patient.
authors.affiliation: Florida Atlantic University Charles E. Schmidt College of Medicine, 777 Glades Road, Boca Raton, FL 33431, USA.
authors.forename: Michael
authors.identifier: 0000-0002-9354-6090
authors.initials: M
authors.lastname: Cheng
authors.affiliation: Florida Atlantic University Charles E. Schmidt College of Medicine, 777 Glades Road, Boca Raton, FL 33431, USA.
authors.forename: Janelle
authors.identifier: 0000-0002-2035-3095
authors.initials: J
authors.lastname: Nassim
authors.affiliation: Saba University School of Medicine, 27 Jackson Road, Devens, MA 01434, USA.
authors.forename: Ario
authors.identifier: 0000-0001-7431-4932
authors.initials: A
authors.lastname: Angha
authors.affiliation: Saba University School of Medicine, 27 Jackson Road, Devens, MA 01434, USA.
authors.forename: Krisna
authors.initials: K
authors.lastname: Srey
authors.affiliation: Division of Acute Care Surgery and Trauma, Memorial Regional Hospital, 3501 Johnson Street, Hollywood, FL 33021, USA.
authors.forename: Alexander
authors.initials: A
authors.lastname: Canales
authors.affiliation: Division of Acute Care Surgery and Trauma, Memorial Regional Hospital, 3501 Johnson Street, Hollywood, FL 33021, USA.
authors.forename: Chauniqua
authors.initials: C
authors.lastname: Kiffin
authors.affiliation: Division of Women's Health Services, Memorial Regional Hospital, 3501 Johnson Street, Hollywood, FL 33021, USA.
authors.forename: Yessin
authors.initials: Y
authors.lastname: Ashmawy
authors.affiliation: Division of Acute Care Surgery and Trauma, Memorial Regional Hospital, 3501 Johnson Street, Hollywood, FL 33021, USA.
authors.forename: Andrew A
authors.identifier: 0000-0001-6069-6458
authors.initials: AA
authors.lastname: Rosenthal