deamino-arginine-vasopressin and Postpartum-Hemorrhage

Unclassified bleeding disorders account for 2.6% of all new bleeding disorder registrations in the UK. The management of the bleeding phenotype associated with these disorders is poorly described. Systematic review and meta-analysis to determine the bleeding rates associated with tranexamic acid, desmopressin, platelet transfusion, plasma transfusion and recombinant activated factor VII, for patients with unclassified bleeding disorders undergoing surgery, childbirth or with menorrhagia. We searched for randomized controlled trials in MEDLINE, Embase, The Cochrane Central Register of Controlled Trials, PubMed, ISI Web of Science and the Transfusion Evidence Library from inception to 24 February 2020. Wherever appropriate, data were pooled using the metaprop function of STATA. Two studies with 157 participants with unclassified bleeding disorders were identified. The pooled risk of minor bleeding for patients undergoing surgery treated with peri-operative tranexamic acid was 11% (95% confidence interval 3--20%; n = 52; I2 = 0%); the risk for desmopressin and tranexamic acid in combination was 3% (95% confidence interval 0--7%; n = 71; I2 = 0%). There were no instances of major bleeding. In one procedure, 1 of 71 (1.4%), treated with a combination of desmopressin and tranexamic acid, the patient had a line-related deep vein thrombosis. There were too few patients treated to prevent postpartum haemorrhage or for menorrhagia to draw conclusions. The GRADE quality of evidence was very low suggesting considerable uncertainty over the results. However, both tranexamic acid, and the combination of tranexamic and desmopressin have high rates of haemostatic efficacy and have few adverse events.. PROSPERO CRD42020169727.

Topics: Antifibrinolytic Agents; Blood Component Transfusion; Deamino Arginine Vasopressin; Disease Management; Female; Hemorrhage; Hemorrhagic Disorders; Hemostatics; Humans; Menorrhagia; Postoperative Hemorrhage; Postpartum Hemorrhage; Pregnancy; Tranexamic Acid

Inherited bleeding disorders increase the risk of bleeding in the obstetric patient. Randomized controlled trials to compare prophylactic or therapeutic interventions are rare, and guidance documents rely heavily on expert opinion. Here we report the results of a systematic review of the literature for the treatment and prevention of peripartum bleeding in women with an inherited bleeding disorder. The highest-quality evidence is for the use of tranexamic acid in postpartum hemorrhage, which has been shown to decrease bleeding-related mortality in women without bleeding disorders. There is limited evidence for prophylactic use of this agent in women with inherited bleeding disorders. Desmopressin has also been used in observational studies of patients with von Willebrand disease and carriers of hemophilia A with some success, although concerns about the risk of hyponatremia persist. In patients with deficiencies of specific factors, replacement is generally the preferred approach, and concentrates have been studied in deficiencies of VWF and factors VII, VIII, IX, XI, and XIII as well as in patients with fibrinogen deficiency. Because of the small size of these studies, neither safety nor efficacy is well established, although the literature suggests that bleeding history may be more predictive of outcomes than factor levels in many cases. Goal factor levels have not been studied or systematically established in any of these diseases, although observational data suggest that achieving normal levels may be inadequate, particularly for VWF and factor VIII, which are physiologically elevated in pregnancy. For factor deficiencies in which no specific concentrate is available, such as factors II (prothrombin) and V, prothrombin complex concentrate or fresh frozen plasma may be used, and for platelet defects or deficiencies, such as Glanzmann thrombasthenia or Bernard-Soulier syndrome, platelet transfusion is generally first line, although use of recombinant FVIIa has been reported in patients with Glanzmann thrombasthenia to avoid development of, or treat patients with, antibodies to platelet glycoprotein IIbIIIa. Ultimately, data are lacking to definitively support an evidence-based approach to management in any of these disorders, and prospective, controlled studies are desperately needed.

Topics: Blood Coagulation; Deamino Arginine Vasopressin; Female; Fibrinolytic Agents; Hematology; Hemophilia A; Heterozygote; Humans; Hyponatremia; Obstetrics; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Tranexamic Acid; Treatment Outcome; von Willebrand Diseases

The State of the Art in von Willebrand disease (VWD) has been impacted not only by discoveries in the field of haemostasis, but also by changes in practice in other fields. The development of bleeding assessment tools has led to the clarification of bleeding symptoms and phenotype in VWD. New discoveries in the biology and genetics of von Willebrand factor (VWF) are challenging our existing diagnostics and classification(s). An improved understanding of reproductive physiology and the pathology of VWD along with changing obstetric, gynaecologic and haemostatic therapies necessitate an evolving response to the care of women with VWD. The survival of patients with autoimmune disease, malignancies and congenital heart disease along with increasing use of circulatory support devices and extracorporeal membrane oxygenation is increasing the prevalence of acquired von Willebrand syndrome. In each of these challenges, there are opportunities to improve the care of our patients with VWD.

Topics: Antibodies, Neutralizing; Deamino Arginine Vasopressin; Factor VIIa; Female; Humans; Male; Polymorphism, Genetic; Postpartum Hemorrhage; Pregnancy; Recombinant Proteins; von Willebrand Diseases; von Willebrand Factor

von Willebrand disease (VWD) is a common, inherited bleeding disorder. There are three main types of VWD, which result in a quantitative or qualitative deficiency in von Willebrand factor (VWF) and in severe cases, also Factor VIII (FVIII). The severity of bleeding depends on the underlying pathophysiology. Type 1 VWD is usually mild, while types 2 or 3 VWD can be associated with moderate or significant bleeding. Managing pregnant women with VWD requires a multidisciplinary approach. Such patients are at increased risk of postpartum hemorrhage. Whether women with VWD are at increased risk of spontaneous abortion remains unclear. Because of increased risk of bleeding, there are special considerations for delivery and obstetrical analgesia. There is a lack of high-quality evidence supporting monitoring and treatment of VWD in pregnancy. Most experts recommend that FVIII and VWF levels be monitored prior to delivery and treatment initiated when levels remain below 0.50 IU/mL. Some experts consider desmopressin (DDAVP) to be the preferred initial treatment in type 1 and most type 2 VWD. DDAVP is relatively contraindicated in type 2B disease. Plasma-derived FVIII and VWF replacements are the treatment of choice in type 2B and 3 VWD and in type 1 or 2 VWD when patients do not respond to DDAVP.

Topics: Deamino Arginine Vasopressin; Factor VIII; Female; Humans; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Risk Factors; von Willebrand Diseases; von Willebrand Factor

Postpartum hemorrhage is a potentially life-threatening albeit preventable condition that persists as a leading cause of maternal death. Identification of safe and cost-effective hemostatic treatment options remains crucial as a supplement to surgery and uterotonic agents.. This review summarizes the background, current evidence and recommendations with regard to the role of fibrinogen, tranexamic acid, prothrombin complex concentrate, desmopressin, and recombinant factor VIIa in the treatment of patients with postpartum hemorrhage. The benefits and evidence behind traditional standard laboratory tests and viscoelastic hemostatic assays, i.e. thromboelastography TEG(®) and thromboelastometry ROTEM(®) , are discussed. In addition we assess and elaborate on the current paradigm and evidence for transfusion of these patients.. Publications between 1994 and 2014 were identified from PubMed, EMBASE, Cochrane Library databases, and ClinicalTrial.gov.. Viscoelastic hemostatic assays were found to provide a real-time continuum of coagulation and fibrinolysis when introduced as a supplement in transfusion management of postpartum hemorrhage. Fibrinogen should be considered when hypofibrinogenemia is identified. Early administration of 1-2 g tranexamic acid is recommended, followed by an additional dose in cases of ongoing bleeding. Uncontrolled hemorrhage requires early balanced transfusion.. Despite the lack of conclusive evidence for optimal hemostatic resuscitation in postpartum hemorrhage, the use of viscoelastic hemostatic assays, fibrinogen, tranexamic acid and balanced transfusion therapy may prove to be potentially pivotal in the treatment of postpartum hemorrhage.

Topics: Antifibrinolytic Agents; Blood Coagulation Factors; Blood Coagulation Tests; Blood Transfusion; Crystalloid Solutions; Deamino Arginine Vasopressin; Factor VIIa; Female; Fibrinogen; Hemostatics; Humans; Isotonic Solutions; Postpartum Hemorrhage; Pregnancy; Recombinant Proteins; Thrombelastography; Tranexamic Acid

Challenges of modern medicine are peripartum bleeding complications as one of the most frequent cases of emergency in obstetrics with a prevalence of 0.5-5.0 %, meaning the main cause of maternal morbidity and mortality. In this context, inherited diseases such as Hermansky-Pudlak syndrome (HPS) should be recognized. HPS is a rare disease and belongs to a heterogeneous group of autosomal recessive disorders characterized by the triad of partial oculocutaneous albinism, disorder of "ceroid" metabolism and platelet storage pool deficiency with bleeding disorder.. We report on a 30-year-old primipara, to show the peripartum obstetrical and anaesthesiological management. The patient presented with contractions in our outpatient department in the 39th gestational week. In previous operations there were bleeding complications due to HPS.. Therefore, to minimize bleeding complications we aimed a vaginal birth, advised against the peridural anesthesia and optimized the coagulation parameters. The subsequent delivery was performed as vacuum extraction without complications. Patient and newborn could be discharged from the hospital without complications.. In conclusion, decisive factor for patients with bleeding disorders is a close interdisciplinary cooperation between obstetrician and anesthesiologist.

Topics: Deamino Arginine Vasopressin; Female; Hemostatics; Hermanski-Pudlak Syndrome; Humans; Infant, Newborn; Male; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications; Vacuum Extraction, Obstetrical; Young Adult

Desmopressin (DDAVP) is commonly used for treatment and prevention of bleeding complications in patients with bleeding disorders including haemophilia A, von Willebrand's disease (VWD) and other less common disorders. This article reviews the current evidence for the use of DDAVP in pregnancy to clarify its efficacy and safety with regard to maternal and foetal outcome. A search of the literature found 30 studies that reported DDAVP use in pregnancy for prophylaxis or treatment of bleeding complications with 216 pregnancies reported in total. The most common indication was prophylaxis for prevention of bleeding during pregnancy and postpartum haemorrhage. DDAVP was used successfully in the first and early second trimester for bleeding prophylaxis in 50 pregnancies. No postpartum bleeding complications were reported in 167 out of 172 pregnancies when DDAVP was used for peripartum haemostatic cover. Twenty-nine studies reported no significant adverse events as a result of treatment with DDAVP. One case of water intoxication seizure and one case of premature labour following the use of DDAVP was reported in a single study. Other maternal side effects included facial flushing and headache and were reported by one study. These side effects were generally well tolerated by patients. There were no other significant adverse events reported in any of the studies as a result of DDAVP use. Foetal outcome was recorded in ten studies with no adverse foetal outcomes. In conclusion, this review shows that DDAVP in selected cases is effective in reducing bleeding complications associated with pregnancy and childbirth with a good safety record. Further research is needed to confirm these findings as they are based on the currently available evidence from small studies and case series only.

Topics: Blood Coagulation Disorders; Deamino Arginine Vasopressin; Female; Hemostatics; Humans; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic

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

The wide clinical spectrum of von Willebrand disease (VWD), its complex pathophysiology and its classification into distinct quantitative (type 1 or type 3) and qualitative (type 2) types with further subtle distinctions have prevented most clinicians from establishing a straightforward approach to diagnosing and treating this inherited bleeding disorder. The results of studies involving large cohorts of patients with a wide range of bleeding manifestations and variable von Willebrand factor (VWF) reduction have recently become available. These data have allowed the proposal of minimal criteria for a clinically useful diagnosis and for differentiating patients with mild VWD from subjects with borderline or only slightly reduced VWF levels who will not benefit from a specific diagnosis. These criteria are based on measurement of VWF ristocetin cofactor (VWF:RCo), VWF antigen (VWF:Ag), factor VIII and a standardized bleeding score (BS). Demonstration of the inheritance of the disorder could help to classify patients for whom insufficient hemostatic challenges may produce a falsely reassuring BS (like in children). Using this approach, mild VWD appears to be mostly composed of type 1 cases. Complemented by the results of desmopressin trial infusion, these parameters form the basis for a clinically oriented classification of all forms of VWD and may be useful for selecting the best treatment according to the severity of the disease. Although few molecular data have revealed practical utility, there is no doubt that the clarification of the molecular pathophysiology of VWD has allowed the unification of this complex disorder into a simple conceptual framework. This framework underlies the proposed utilization of simple phenotypic markers for optimizing treatments in individual patients.

Topics: Adult; Child; Deamino Arginine Vasopressin; Disease Management; Factor VIII; Female; Hemorrhage; Humans; Male; Mutation; Patient Selection; Phenotype; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Severity of Illness Index; 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

Considerable progress has been made in the past decade in describing the obstetrical and gynaecological aspects of von Willebrand's disease (VWD). In addition, epidemiological studies have established an approximately 11-16% prevalence of the laboratory diagnosis of VWD in women presenting with menorrhagia. However, it is not established presently whether an upfront VWD screening should be a part of the standard evaluation of menorrhagia. This is because it is presently not known whether therapy in the VWD patient tailored specifically for VWD will appreciably alter the natural history of menorrhagia compared with the non-VWD menorrhagia patient. There are also subtleties involved in securing the diagnosis of VWD in women presenting with menorrhagia in terms of fluctuation of von Willebrand factor (VWF) levels vis-à-vis the menstrual cycle and the potential impact of oral contraceptive on VWF levels. Regarding management of VWD-related menorrhagia, pending ongoing comparative trials of intranasal desmopressin (DDAVP), tranexamic acid, oral contraceptive and the levonorgestrel intrauterine device, specific recommendations cannot be made presently regarding the superiority of one intervention compared with the other. The management of VWD-related postpartum haemorrhage is also an area of active debate in terms of 'best practice' in type 1 (? prophylactic DDAVP), type 2 [? expectant management if factor VIII:C (FVIII:C) level normalizes] and type 3 patients (? intensity and duration of infusional therapy with a VWF-containing plasma-derived FVIII concentrate). This review summarizes the present state of knowledge and highlights numerous questions for future study based on our present understanding of VWD in women.

Topics: Deamino Arginine Vasopressin; Female; Hemostatics; Humans; Mass Screening; Menorrhagia; Postpartum Hemorrhage; von Willebrand Diseases

This paper discusses selected reports on the management of acquired haemophilia A, a rare bleeding disorder characterized by the development of an autoantibody directed against plasma coagulation factor VIII (FVIII) (Morrison et al., Blood 81:1513-1520, 1993). The current literature consists of reports of cohorts of patients from referral centres and retrospective surveys of referral centres (Green and Lechner, Thromb Haemost 45:200-203, 1981). This suggests that the current literature may be biased both by referral practice to tertiary centres and reporting bias of these centres and may not be representative of the full spectrum of the disease. The published studies describe various immunosuppressive regimens to eradicate factor VIII inhibitors but usually lack control patients. Only one report describes a randomized, controlled study. Studies that address treatment of bleeding episodes give data on safety and efficacy for individual products but no comparative studies are available.

Topics: Adrenal Cortex Hormones; Adult; Animals; Autoantibodies; Autoimmune Diseases; Bias; Blood Coagulation Factors; Deamino Arginine Vasopressin; Factor VIII; Female; Hemophilia A; Humans; Immunoglobulins, Intravenous; Immunosorbent Techniques; Immunosuppressive Agents; Postpartum Hemorrhage; Pregnancy; Retrospective Studies; Swine

Von Willebrand disease (vWD) is a frequent inherited disorder of hemostasis that affects both sexes. Two abnormalities are characteristic of the disease, which is caused by a deficiency or a defect in the multimeric glycoprotein called von Willebrand factor: low platelet adhesion to injured blood vessels and defective intrinsic coagulation owing to low plasma levels of factor VIII. There are 2 main options available for the treatment of spontaneous bleeding episodes and for bleeding prophylaxis: desmopressin and transfusional therapy with plasma products. Desmopressin is the treatment of choice for most patients with type 1 vWD, who account for approximately 70% to 80% of cases. This nontransfusional hemostatic agent raises endogenous factor VIII and von Willebrand factor 3 to 5 times and thereby corrects both the intrinsic coagulation and the primary hemostasis defects. In patients with the more severe type 3 and in most patients with type 2 disease, desmopressin is ineffective or is contraindicated and it is usually necessary to resort to plasma concentrates containing both factor VIII and von Willebrand factor. Concentrates treated with virucidal methods should be preferred to cryoprecipitate because they are equally effective and are perceived as safer. (Blood. 2001;97:1915-1919)

Topics: Blood Loss, Surgical; Combined Modality Therapy; Contraindications; Deamino Arginine Vasopressin; Endothelium, Vascular; Factor VIII; Female; Hemorrhage; Humans; Isoantibodies; Male; Myocardial Infarction; Postoperative Hemorrhage; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Receptors, Vasopressin; Safety; Stroke; Thrombocytopenia; Transfusion Reaction; Virus Diseases; von Willebrand Diseases; von Willebrand Factor

Bleeding disorders in women are an underestimated problem that deserves increased attention. About 9%-14% of females have menorrhagia and, amongst them, there is a significant over-representation of von Willebrand disease (VWD), with a prevalence of 13% in this group as compared with about 1% in the general population. The bleeding disorder has not been diagnosed in most of these women and they may therefore be withheld from treatment with desmopressin, which is effective in most cases of VWD and also in platelet dysfunctions and mild factor VIII deficiency. This paper is a review of the haemostatic use of desmopressin with special reference to women's bleeding disorders, the mechanisms of action, modes of administration, clinical indications, dosage recommendations, and hospital or home treatment. Desmopressin stimulates endogenous release of FVIII and von Willebrand factor (VWF), it increases platelet adhesiveness and shortens bleeding time. It can be given as intravenous or subcutaneous injection, but the intranasal spray is probably the most practical mode of administration for females with bleeding disorders as it is simple to administer and suitable for home treatment. The spray has been used successfully in connection with menorrhagia and other bleeding symptoms.

Topics: Blood Loss, Surgical; Deamino Arginine Vasopressin; Female; Hemorrhagic Disorders; Hemostatics; Humans; Postpartum Hemorrhage; Pregnancy; Self Administration

Topics: Deamino Arginine Vasopressin; Female; Hemostatics; Humans; Infant, Newborn; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Pregnancy Outcome; Time Factors; Treatment Outcome; von Willebrand Diseases

Patients with a bleeding tendency with normal laboratory tests have been described as having an unclassified bleeding disorder or bleeding disorder of unknown cause (BDUC). There are very little data available on how to manage pregnancy.. To study management and outcomes of these patients at four United Kingdom hemophilia comprehensive care centers.. Retrospective case note review from 2010-2020.. Sixty deliveries in 36 patients were recorded. The median International Society on Thrombosis and Haemostasis bleeding assessment tool score was 9. In 54 cases for which data were available, the odds ratio for post partum hemorrhage (PPH) was 6.3 for no primary hemostatic prophylaxis versus prophylaxis (95% confidence interval 1.2-34.2, p < .05); 7/9 (78%) versus 16/45 (36%) PPH incidence for the groups, respectively. Hemostatic prophylaxis was with tranexamic acid but some patients received desmopressin or platelet infusions. Secondary PPH was seen in 5/60 (8%) of cases. No neonatal bleeding complications or maternal thromboembolic complications were noted. Avoidance of regional anesthesia and fetal delivery precautions were commonly advised, but in the small number of cases in which they occurred no complications were noted.. Despite hemostatic prophylaxis PPH was commonly seen. Further prospective studies of BDUC patients are required to determine optimal management in pregnancy as well as determine the pathophysiological basis of bleeding.

Topics: Deamino Arginine Vasopressin; Female; Hemophilia A; Hemostatics; Humans; Postpartum Hemorrhage; Pregnancy; Prospective Studies; Retrospective Studies; Tranexamic Acid

Oxytocin, a nanopeptide secreted by the posterior pituitary gland, has well-established uterotonic activity. Its role in initiating the vigorous and regular contractions of the first stage of labor is still controversial. We report four cases of panhypopituitarism who had spontaneous onset of labor, undermining the role of maternal oxytocin in the first phase of labor.. Four women with no residual pituitary function conceived through ovulation induction and were treated throughout pregnancy with thyroid replacement therapy, desmopressin and glucocorticoids. In all cases pituitary function was undetectable in repeated blood tests. We report their course of pregnancy and delivery.. All four pregnancies progressed to term with hormonal replacement therapy. All cases went into spontaneous labor. Two women delivered vaginally unassisted by pharmacological intervention and two delivered by cesarean sections during active labor due to obstetrical indications. Three suffered postpartum hemorrhage. Lactation did not ensue in all four cases.. Endogenous pituitary oxytocin is probably not obligatory for initiation of labor in the first phase of parturition.

Topics: Adult; Antidiuretic Agents; Cesarean Section; Deamino Arginine Vasopressin; Delivery, Obstetric; Female; Glucocorticoids; Hormone Replacement Therapy; Humans; Hypopituitarism; Labor, Obstetric; Ovulation Induction; Oxytocin; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications; Thyroid Hormones; Young Adult

The authors report the performance of a labour epidural analgesia in a 26-year-old parturient presenting a moderate factor XI (FXI) deficiency. If haemostasis disorders usually contraindicate an epidural analgesia (with a risk of epidural haematoma), a moderate FXI deficiency is not an absolute contraindication to perform such an epidural analgesia. Desmopressin, sometimes used in surgery to reduce the bleeding, was administered to withdraw the catheter in better haemostasis conditions. No neurological signs were observed.

Topics: Adult; Analgesia, Epidural; Analgesia, Obstetrical; Blood Coagulation Tests; Catheterization; Contraindications; Deamino Arginine Vasopressin; Disease Susceptibility; Factor XI Deficiency; Female; Hematoma, Epidural, Spinal; Humans; Infant, Newborn; Obstetric Labor Complications; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Risk

We describe the peripartum management of a 26-year-old primigravida with a platelet storage pool disorder who underwent spontaneous vaginal delivery of twins with epidural analgesia. Postpartum hemorrhage from uterine atony, and cervical and vaginal lacerations were treated successfully with 1-desamino-8D-arginine vasopressin and blood products. The use of thromboelastography in the assessment and management of bleeding risk in the setting of platelet storage pool disorder is described.

Topics: Adult; Deamino Arginine Vasopressin; Female; Humans; Peripartum Period; Platelet Storage Pool Deficiency; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications; Thrombelastography

Here we report of a patient who developed a Moschcowitz-like syndrome following a desmopressin treatment of severe postpartum hemorrhage. The patient got an anaphylactic reaction after cervical ripening with dinoproston, leading to an emergency cesarean. A postpartum uterine atony with a blood loss more than 1500 ml resulted in a disseminated intravascular coagulation that was treated with mass transfusion of blood products, including platelets and factor VII. Desmopressin is used as rescue medication in situations of severe bleeding. It was given in this life-threatening situation and presumably triggered a Moschcowitz-like syndrome. Desmopressin exerts its haemostatic effect by releasing von Willebrand factor, which is elevated in pregnancy per se. This results in an increased risk of developing microthrombi, leading to a Moschcowitz-like syndrome. In conclusion, desmopressin should not be administered in pregnant patients owing to its potential risk of triggering the development of thrombotic-thrombocytopenic purpura.

Topics: Adult; Blood Transfusion; Cesarean Section; Deamino Arginine Vasopressin; Dinoprostone; Disseminated Intravascular Coagulation; Female; Hemostatics; Humans; Oxytocics; Postpartum Hemorrhage; Pregnancy; Purpura, Thrombotic Thrombocytopenic; Uterine Inertia; von Willebrand Factor

Reproductive tract bleeding in women is a naturally occurring event during menstruation and childbirth. In women with menorrhagia, however, congenital bleeding disorders historically have been underdiagnosed. This consensus is intended to allow physicians to better recognize bleeding disorders as a cause of menorrhagia and consequently offer effective disease-specific therapies.

Topics: Adult; Blood Coagulation Disorders; Deamino Arginine Vasopressin; Decision Trees; Factor VIII; Female; Hemostatics; Humans; Menorrhagia; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Quality of Life; von Willebrand Diseases; von Willebrand Factor

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.

Topics: Adult; Deamino Arginine Vasopressin; Female; Hemostatics; Hermanski-Pudlak Syndrome; Humans; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications; Vaginal Birth after Cesarean

Bernard-Soulier syndrome (BSS) is a rare autosomal recessively inherited bleeding disorder. Pregnancy in patients with BSS is characterized by ante-, intra-, or postpartum haemorrhage, which may be delayed and severe. There is no consensus in the management of BSS in pregnancy and so far only 16 pregnancies in nine patients have been described. We report a further three pregnancies in two women with the syndrome. We also outline our management of pregnant patients with BSS.

Topics: Adolescent; Adult; Bernard-Soulier Syndrome; Blood Platelets; Consanguinity; Deamino Arginine Vasopressin; Erythrocyte Transfusion; Female; Humans; Platelet Aggregation; Platelet Transfusion; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Tranexamic Acid

The Bernard-Soulier syndrome (B-S s.) is rare autosomal recessive bleeding disorder. It is characterized by prolonged bleeding time, fail to agglutinate with ristocetin and a normal to decreased number of unusually large platelets whose membrane lack glycoproteins complex GP Ib/IX/V. The manifestation of the disease may differ in consecutive pregnancies of the same patient. Presently we describe 24 years old white women diagnosed with B-S s. at the age of 22. Two years later she become pregnant. During the pregnancy platelet counts ranged from 26 x 10(9)/1 to 51 x 10(9)/1, without sings of bleeding. Because of the risk of immunization against GP Ib/IX, with might lead to severe isoimmune neonatal thrombocytopenia, 4 times during pregnancy she had done MAIPA-test (monoclonal antibody immobilization of platelet antigens). Specific antibodies were not detected. Subsequent ultrasounds with biophysical profile revealed normal fetus growth. Spontaneous labour in 38th week of gestation. The use of single-donor platelets, intravenous desmopressin and tranexamic acid was found to be useful in controlling postpartum vaginal bleeding in the patient.

Topics: Adult; Antigens, Human Platelet; Bernard-Soulier Syndrome; Deamino Arginine Vasopressin; Female; Humans; Infant, Newborn; Injections, Intravenous; Platelet Transfusion; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Pregnancy Outcome; Tranexamic Acid; Treatment Outcome

Hermansky-Pudlak syndrome is characterised by oculocutaneous albinism and haemorrhagic diathesis. The bleeding tendency that is associated with this autosomal recessive disease is caused by storage-pool deficiency and has been reported to be controllable by prophylactic administration of 1-desamino 8D-arginine (desmopressin, DDAVP). The DDAVP prophylaxis at the first delivery of our patient did not prevent the severe haemorrhagic sequeal requiring transfusion of packed red cells and platelets, but the same preventive measure was successful at her second childbirth. Response to prophylactic DDAVP administration varies between as well as within patients with Hermansky-Pudlak syndrome.

Topics: Adolescent; Cesarean Section; Deamino Arginine Vasopressin; Erythrocyte Transfusion; Female; Hermanski-Pudlak Syndrome; Humans; Platelet Transfusion; Postoperative Hemorrhage; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications

Acquired haemophilia was diagnosed following detailed investigation of a post-partum haemorrhage unresponsive to standard management. Circulating factor VIII inhibitors and low factor VIII levels were detected and intravenous DDAVP treatment lead to a resolution of symptoms. This case highlights the importance of haematological investigations in persisting post-partum haemorrhage.

Topics: Adult; Autoantibodies; Deamino Arginine Vasopressin; Factor VIII; Female; Hemophilia A; Hemostatics; Humans; Partial Thromboplastin Time; Postpartum Hemorrhage; Tranexamic Acid

Topics: Deamino Arginine Vasopressin; Factor VIII; Female; Hemorrhage; Heterozygote; Humans; Mutation, Missense; Phenotype; Point Mutation; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; von Willebrand Diseases; von Willebrand Factor

An international registry was established on the reproductive health of women with types of von Willebrand disease (vWd) unresponsive to DDAVP. Data was collected on 44 women from 16 treatment centers in nine countries. Severe menorrhagia requiring blood product therapy occurred at least once in 80% of the women for whom data was reported. Most of the reported episodes occurred prior to the diagnosis of vWd and/or the use of oral contraceptive (OC) therapy. OC therapy was clinically effective in the treatment of chronic menorrhagia in 22 of 25 (88%) women treated. Two of the women, however, were unable to tolerate chronic OC use and a third became refractory to treatment. Hysterectomy was performed in 10 of the 44 women (23%). The reported indication for six of the procedures was menorrhagia. Seventy percent were performed at two treatment centers, suggesting different thresholds for the performance of the procedure. There were 69 pregnancies reported in 31 of the 44 women. Fifteen of the pregnancies resulted in spontaneous abortions. The incidence of miscarriage was 22% and appeared clustered, with 10 of 15 of the miscarriages occurring in just four of the women.

Topics: Abortion, Spontaneous; Adult; Blood Coagulation Factors; Blood Transfusion; Contraceptives, Oral, Hormonal; Deamino Arginine Vasopressin; Europe; Female; Humans; Hysterectomy; Menorrhagia; Postpartum Hemorrhage; Pregnancy; Pregnancy Outcome; Registries; Treatment Failure; United States; von Willebrand Diseases

Topics: Adult; Aged; Deamino Arginine Vasopressin; Factor VIII; Female; Hemophilia A; Humans; Postpartum Hemorrhage; Pregnancy; Suppressor Factors, Immunologic

Sheehan's syndrome and diabetes insipidus were diagnosed in a 31-year-old woman seven months after postpartum bleeding with a short duration of hypotension. The diagnosis of diabetes insipidus was established by the inability to concentrate urine during water deprivation and the marked increase in urinary osmolality after administration of 1-Desamino-8-D-arginine-vasopressin (DDAVP). Obstetricians should be aware of diabetes insipidus as a postpartum complication.

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Hypopituitarism; Postpartum Hemorrhage; Pregnancy; Puerperal Disorders; Water Deprivation