deamino-arginine-vasopressin and Uterine-Hemorrhage

Factor XI deficiency is a rare disease found predominantly in Ashkenazi Jews. There is a poor correlation between factor XI level and bleeding in patients with factor XI deficiency. Individuals with severe factor XI deficiency are usually at risk of excessive bleeding after surgery and injury, particularly when trauma involves tissues rich in fibrinolytic activity. Women with partial or severe deficiency are at risk of excessive uterine bleeding during labor. The unpredictable nature of factor XI deficiency complicates management during pregnancy and delivery. This review gives an overview of the management of pregnant women with factor XI deficiency.

Topics: Adult; Anesthesia, Epidural; Anesthesia, Obstetrical; Anesthesia, Spinal; Blood Loss, Surgical; Cesarean Section; Contraindications; Deamino Arginine Vasopressin; Factor XI; Factor XI Deficiency; Female; Humans; Infant, Newborn; Jews; Mutation; Plasma; Postoperative Hemorrhage; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Uterine Hemorrhage

von Willebrand disease is caused by either a quantitative or qualitative defect in von Willebrand factor (VWF). Patients may have extensive mucosal bleeding (because of platelet dysfunction) and prolonged bleeding after surgery (because of factor VIII deficiency). Up to 6 different subtypes of the disease have been described, and diagnosis is based on clinical suspicion and laboratory confirmation. Accurate diagnosis is of paramount importance because therapy will vary according to the subtype. Bleeding complications during pregnancy are more frequent when levels of the von Willebrand ristocetin cofactor assay and factor VIII levels are <50 IU/dL. In such cases, therapy before any invasive procedure or delivery must be instituted. The mainstays of therapy are desmopressin and plasma concentrates that contain von Willebrand factor. Delayed postpartum hemorrhage may occur, despite adequate prophylaxis. Frequent monitoring and continued prophylaxis and/or treatment are recommended for at least 2 weeks after delivery.

Topics: Blood Component Transfusion; Chromosomes, Human, Pair 12; Continuity of Patient Care; Deamino Arginine Vasopressin; Delivery, Obstetric; Factor VIII; Female; Hemostatics; Humans; Mutation; Postpartum Hemorrhage; Postpartum Period; Pregnancy; Pregnancy Complications, Hematologic; Ristocetin; Uterine Hemorrhage; von Willebrand Diseases; von Willebrand Factor

Willebrand's disease is the most frequent inborn coagulopathy and type 3 is its most severe form. Pregnancy and delivery are critical events in women with Willebrand's disease of type 3. Prophylactic treatment for delivery and early postpartum period is recommended. We report the management of pregnancy and successful delivery of a 32-year-old woman with type 3. Prophylactic treatment with 2000 IU of Willebrand's disease factor (WdF) was given twice a day during the delivery day and the day after, and 1000 IU per day during the next three days. The patient did not show any spontaneous metrorrhagia but anemia. No bleeding was observed in the newborn.

Topics: Adult; Blood Loss, Surgical; Cesarean Section; Consanguinity; Deamino Arginine Vasopressin; Female; Humans; Infant, Newborn; Placenta Previa; Platelet Aggregation; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Uterine Hemorrhage; von Willebrand Diseases; von Willebrand Factor

A patient with the genetic condition neurofibromatosis type I and no known coagulopathy undergoing cesarean delivery, had diffuse uterine and surgical site bleeding that was not correctable by oxytocin, methylergonovine and PGF2 alpha. Despite good uterine tone, hemorrhage continued from the uterus and the surrounding tissues, persisting even after surgical ligation of the uterine arteries. With no change in her condition, which was behaving clinically as a coagulopathy, an infusion of desmopressin acetate (DDAVP) was begun. The patient's bleeding promptly resolved shortly after infusion of this agent. A review of relevant literature suggests that platelet reactivity of patients with neurofibromatosis type 1 is attenuated in some in vitro conditions. Thus, there may be some theoretical basis for using DDAVP in patients with neurofibromatosis type 1 who have bleeding problems with no other known source, such as in the case presented here.

Topics: Adult; Anticoagulants; Blood Coagulation Disorders; Cesarean Section; Deamino Arginine Vasopressin; Female; Hematocrit; Humans; Infant, Newborn; Infusions, Intravenous; Intraoperative Complications; Neurofibromatosis 1; Obstetric Labor Complications; Pregnancy; Uterine Hemorrhage

Topics: Adult; Deamino Arginine Vasopressin; Ehlers-Danlos Syndrome; Female; Humans; Obstetric Labor Complications; Pregnancy; Pregnancy Complications; Uterine Hemorrhage

Six patients having different subtypes of von Willebrand's disease were followed up during eight complete pregnancies. Two additional pregnancies terminated in spontaneous abortions. Five pregnancies ended in cesarean section either because of obstetric problems (three) or electively (two) to avoid infant bleeding. Three deliveries were complicated by vaginal bleeding attributed to von Willebrand's disease, while bleeding during two deliveries had clear obstetric causes. Only two deliveries were associated with no bleeding complications. Five newborn babies were found to have von Willebrand's disease. One of them was born with a head hematoma. Management, which included cryoprecipitate and desmopressin (Stimate), is discussed. It is important to manage each case individually since obstetric parameters and severity of bleeding disorder must be known before treatment is planned.

Topics: Adolescent; Adult; Antigens; Blood Coagulation Tests; Deamino Arginine Vasopressin; Delivery, Obstetric; Factor VIII; Female; Fetal Blood; Fibrinogen; Genetic Counseling; Humans; Infant, Newborn; Perinatology; Pregnancy; Pregnancy Complications, Hematologic; Puerperal Disorders; Uterine Hemorrhage; von Willebrand Diseases; von Willebrand Factor