Women with congenital FXIII deficiency suffer significant bleeding complications.

Menorrhagia and ovulation bleeding are common gynaecological problems and more prevalent than reported. Pregnancies in women with FXIII deficiency have a significant risk of miscarriage, placental abruption and PPH if not on prophylaxis treatment. “
“Prophylaxis was introduced for severe hemophilia more than 50 years ago in Sweden. Costs of treatment and the need for frequent infusions have delayed the acceptance of this very effective treatment. With primary prophylaxis, bleeding can be prevented and children can lead a normal life. Dosage and frequency of prophylaxis can be individualized to reduce cost and should make prophylaxis affordable for more

children. In the Western world, prophylaxis should be the standard Adriamycin supplier of care for all children RG-7388 datasheet with severe hemophilia A and B. When to begin prophylaxis in adults with hemophilic arthropathy is still under debate as it is considered less effective. “
“von Willebrand’s disease (VWD) patients undergoing major surgery are prophylactically treated to promote haemostasis. There is variability in perioperative clinical practice; however, most guidelines suggest replacing the deficient factor to a level of 1.0 IU mL−1 (or 100%). A review of the literature reveals a paucity of well constructed descriptive data quantifying the changes in coagulation that occur in response to surgical stress. The aim of this study was to quantify the changes in haemostatic variables occurring in response to elective orthopaedic surgery in normal individuals. Eligible subjects >18 years of age undergoing total hip or knee replacement were recruited. Blood samples were drawn at five time points: baseline, preoperatively, 30 min after surgical incision, 30 min postoperatively, postoperative day (POD) 1. Analyses included t-tests and repeated measures anova. Overall 30 patients, 21 women and 9 men, with a mean age of 65 were included in the final analysis. All von Willebrand factor (VWF) variables were seen to significantly decrease intraoperatively and increase

postoperatively. VWF multimers showed a statistically significant decrease in high molecular weight multimers Fossariinae intraoperatively and an increase postoperatively. On subgroup analysis, age, gender and anaesthesia type were significantly correlated with changes in VWF parameters. Data presented in the current study establish a physiological baseline for VWF parameters in the normal population and demonstrate mean VWF/factor VIII levels greater than 1.0 IU mL−1 intraoperatively. As such, current management in VWD patients does not appear to mimic the normal physiological response to surgery. “
“Our objective was to provide a synthesis of measurement properties for performance-based outcome measures used to evaluate physical function in children with haemophilia.