This prospective study investigated inhibitor development after continuous infusion of factor concentrate for surgical procedures in subjects with VWD or a severe form of haemophilia (factor activity <1%). Observations were made on the occurrence of inhibitor formation, adverse events and virus seroconversions. Main inclusion criteria comprised a negative history of inhibitors to replacement factor concentrate, ≥50 exposure days to factor concentrate and anticipated surgery
requiring replacement factor coverage for ≥3 days. Therapy began ABT199 with a bolus dose of 30–50 IU kg−1 body weight of factor concentrate followed by continuous infusion with 3–4 IU kg−1 h−1. Continuous infusion dose of factor concentrate was adjusted based on factor levels measured at least once daily. In 46 subjects included in the study to date, no inhibitors have been identified at Selleckchem Nutlin-3a discharge or follow-up (3–4 weeks after surgery), and no thrombotic events or postoperative wound infections occurred. All subjects underwent surgery without major blood loss, and hemostatic efficacy was generally
rated ‘excellent’. The results of the current study are promising, although the number of subjects is too small to make a definitive statement about the incidence of inhibitor development during continuous infusion of factor concentrate. Therefore, this study will be continued. “
“Clinical problems associated with inhibitors in mild/moderate hemophilia are often considerable, since in the majority of cases adult patients are confronted with a change in phenotype from mild/moderate to severe. Although some
of the risk factors for inhibitor development are similar to those in severe hemophilia, others are specific for mild/moderate hemophilia. The study of the immune response in mild/moderate hemophilia A can help to elucidate some of the mechanisms underlying inhibitor formation and disruption of tolerance. Treatment MCE公司 of bleeding episodes and eradication of inhibitors in mild/moderate hemophilia require specific management and special attention should be paid to the prevention of this complication. “
“There are no evidence-based guidelines for antithrombotic management in people with haemophilia (PWH) presenting with acute coronary syndrome (ACS). The aim of the study was to review the current European Society of Cardiology guidelines, and to consider how best they should be adapted for PWH. Structured communication techniques based on a Delphi-like methodology were used to achieve expert consensus on key aspects of clinical management.