The hazard ratio for all-cause mortality compared with controls was 2.2, 95% CI: [1.8; 2.7], P < 0.001 for the entire group PFT�� mouse of patients and 1.7, 95% CI: [1.3; 2.2], P < 0.001 when patients with HIV and/or viral hepatitis were excluded. The corresponding figures for the severe haemophilia subgroup were 6.6, 95% CI: [4.5; 10.0], P < 0.001

and 8.2, 95% CI [3.2; 20.8], P < 0.001 respectively. The most common causes of death were related to malignancies and the haemostatic defect. People with haemophilia were 57% less likely to die from ischaemic heart disease than controls. People with haemophilia in Sweden demonstrate higher mortality over time, independent of HIV and viral hepatitis, despite relatively advantageous access to clotting factor concentrates. "
“Summary.  Acquired factor XIII (FXIII) deficiency, arising from an autoantibody against factor XIII, is a rare bleeding disorder. This

autoimmune disorder most commonly occurs in the elderly. Patients who develop such acquired FXIII inhibitors may present with catastrophic bleeding events and are hard to be diagnosed with the normal general coagulation tests. Though the disease is relatively rare, it is known to cause Selleckchem ACP-196 significant mortality. In this article we briefly describe a patient who presented with extensive bleeding and a normal activated partial thromboplastin time and prothrombin time (PT), but had an acquired inhibitor to FXIII; PIK3C2G her primary disease was systemic lupus erythematosus (SLE). Also, we will focus on the clinical features, treatment modalities, fibrin structure and epitope identification for acquired factor XIII inhibitor with a review of the literature. “
“Summary.  Changes in articular cartilage after haemarthrosis

have not been completely elucidated in haemophilic arthropathy. Insights into the pathophysiological mechanisms of blood-induced joint damage mainly derived from histological, inflammatory and biochemical investigations. A structure–function relationship is another reasonable way to determine the joint overall health status. Cartilage, a viscoelastic connective tissue, is at least a biphasic material that should also work under minimal friction. Pendulum friction tester measures the mechanical aspects of joint lubrication and quantifies the biotribological properties of the joint. Indentation test is an in situ method characterizing the biomechanical properties of the cartilage. Gross, biotribological and biomechanical properties were determined in a rabbit model of experimental haemarthrosis. A sample of 1 mL of fresh autologous blood was injected in the left knee of rabbit’s joint twice weekly for four consecutive weeks. The right knee and animals in the control group were left untreated. After 8 days, joint perimeter, biotribological and biomechanical tests were performed. In a consistent manner, all data showed detrimental effects of the blood on the overall cartilage function under loading.