A standardized treatment regimen for patients with severe haemophilia A undergoing orthopaedic or trauma surgery: a single centre experience.

Recommendations on the administration of clotting factor concentrates in patients with severe haemophilia undergoing surgery are usually determined by monitoring target clotting factor levels. In this retrospective cohort study, we enrolled patients with severe haemophilia A who underwent major orthopaedic or trauma surgery. We wanted to evaluate the feasibility and the safety of a standardized medical treatment procedure.

The influence of antithrombin substitution on heparin sensitivity and activation of hemostasis during coronary artery bypass graft surgery: a dose-finding study.

Background: Cardiopulmonary bypass is associated with a high degree of hemostatic system activation. Supplementation of antithrombin (AT) may attenuate this activation and increase a patient's susceptibility to heparin. However, the appropriate dosage of AT has not been defined.

Genotype and laboratory and clinical phenotypes of protein s deficiency.

The diagnosis of thrombophilia caused by protein S deficiency remains difficult. From 2005 to 2010, we documented 135 patients with suspected hereditary protein S deficiency for whom mutational analysis of the PROS1 gene had been performed by direct double-stranded sequencing of the amplified 15 exons including splice sites. Multiplex ligation-dependent probe amplification was performed on 12 of 15 exons in cases with no mutation found but a large deletion in the PROS1 gene was suspected.

Review article: unexpected bleeding in the operating room: the role of acquired von Willebrand disease.

Acquired von Willebrand disease (AvWD) is a rare bleeding disorder that occurs in association with a variety of underlying disorders and can lead to unforeseen bleeding in surgical patients. Cardiovascular as well as malignant and immunological diseases may be associated with AvWD, and several pathophysiological mechanisms have been proposed. von Willebrand factor (vWF) is a plasma glycoprotein that mediates platelet adhesion to subendothelial collagen and causes platelet aggregation under high shear stress.

Observational study of fibrinogen concentrate in massive hemorrhage: evaluation of a multicenter register.

In acute hemorrhage, a critical decrease in fibrinogen often induces acquired coagulopathy. Fibrinogen concentrate has been used to supplement fibrinogen during massive hemorrhage. However, there are limited data on the utilization of fibrinogen concentrate in this setting.

Hemophilia A in cardiac operations: a model of reduced thrombin generation.

Patients with hemophilia A have a congenital defect in thrombin generation. Only limited data are available on the substitution regimens in hemophilia A patients during and after cardiac operations. There are no data on heparinization of these patients during cardiopulmonary bypass.

Monitoring of direct anticoagulants.

In recent years, new anticoagulants directly targeting individual components of the coagulation cascade have been developed. Although there is no need for routine coagulation monitoring with most of these agents, there are several clinical situations where a specific coagulation test should be available. Global coagulation tests react very sensitive to direct thrombin inhibitors or factor Xa inhibitors.

Postoperative changes in procoagulant factors after major surgery.

Surgical patients are primarily at an increased risk of perioperative bleeding; however, after surgery, these patients develop hypercoagulability that favors thrombotic events. Currently, the time course of postoperative coagulation is not well characterized. Thus, the aim of the present study was to provide a detailed description of the changes in procoagulant factors in patients after major surgery and to evaluate coagulation tests based on their ability to detect hypercoagulability.

Enhanced thrombin generation after cardiopulmonary bypass surgery.

Background: Thrombin generation has a key role in the pathophysiology of hemostasis. Research has focused on the intraoperative course of hemostasis, while little is known about postoperative hemostatic activation. Thrombin generation assays quantify the potential for thrombin generation ex vivo and may be useful for determining hypercoagulability.

Perioperative haemostatic management of Glanzmann thrombasthenia for abdominal surgery.

Glanzmann thrombasthenia is a rare congenital platelet disorder characterized by spontaneous mucocutaneous bleeding and severe bleeding complications during major surgery. This report centres on the perioperative haemostatic management of a patient with Glanzmann thrombasthenia undergoing elective major abdominal surgery. The treatment regimen was based mainly on recombinant activated factor VII, fibrinogen, and factor XIII, reducing platelet transfusion to a minimum.

Fast-track cardiac anesthesia: efficacy and safety of remifentanil versus sufentanil.

Objective: The purpose of this study was to compare the safety and efficacy of fast-track cardiac anesthesia with remifentanil (group R) versus sufentanil (group S).

Design: Prospective, single-blinded, randomized study.

Setting: University hospital.