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The only form of haemophilia treatment that is able to prevent arthropathy and other consequences of bleeding symptoms in patients with severe haemophilia is prophylaxis started at an early age (primary prophylaxis). It is also highly beneficial for the psychological and social wellbeing of patients and their families. Scientific institutions and international organizations such as WHO, the World Federation of Hemophilia (WFH) and the National Haemophilia Foundation (NHF) have recommended that prophylaxis be considered optimum therapy. This paper discusses the barriers to prophylaxis, such as the perceived need, costs and availability, and difficulty of venous access, and describes the authors' experiences with the therapy. [ABSTRACT FROM AUTHOR]

What Is Known and Objective: The dispensing of clotting factor concentrates in hospital pharmacies imposes accessibility constraints on patients and their caregivers, thereby increasing the disease burden. Very few studies have addressed these issues so far in terms of individual perceptions and actual difficulties. The PHAREO study aims to report patient's perception of treatment accessibility and evaluate spatial accessibility. Methods: The PHAREO study is an observational survey based on a questionnaire specifically designed for the study purpose in collaboration with patients' representatives in the second demographic and economic French region. Results and Discussion: We collected 293 responses (participation rate of 64.1%) which show that 89.8% of respondents were either very or rather satisfied with regard to access to treatment. However, respondents reported difficulties in accessing the hospital pharmacy. The data also showed that 79.2% of respondents tended to over‐estimate travel time which was reported above their acceptable threshold for 39.2% of them. The main determinants of dissatisfaction were parental burden (OR 2.5 [1.3; 4.8], p = 0.008) and waiting time at the hospital pharmacy (OR 1.5 [1.1;2.0], p = 0.016, per 10 min increase). What Is New and Conclusion: The PHAREO study provides subjective and objective data regarding satisfaction levels of persons with haemophilia and other coagulation deficiencies, with a high representativeness rate for patients on prophylaxis (87.5%). Both respondents and hospital pharmacists pled for an evolution of the current dispensing circuit to improve access to treatment and reduce the burden for patients. Currently, the community pharmacists are apart from the dispensing circuit. The authors propose improvements in the pathway of care for patients and their caregivers by including the community pharmacists alongside the hospital pharmacists in a centralized coordination scheme. [ABSTRACT FROM AUTHOR]

Purpose: The implementation of a ROTEM®-based algorithm requires reliable thresholds to mirror a prothrombin time (PT) ratio > 1.2 and/or a fibrinogen concentration < 1.5 g l−1. Our goal was to compare the diagnostic performances of two devices (ROTEM® Sigma and Delta, IL Werfen, Munich, Germany) in two level-I trauma centres for the diagnostic of post-traumatic coagulopathy. Methods: We conducted a retrospective analysis of two registries across two periods of time: from September 2014 to December 2015 in Lyon-Sud university trauma centre and from April 2016 to January 2018 in the Grenoble Alps Trauma Centre. Accuracies of EXTEM and FIBTEM assays to detect patients with coagulation disorders were tested for each device using receiver operating characteristic (ROC) analyses. Results: Within the study period, 74 trauma patients in the Grenoble cohort and 75 trauma patients in the Lyon cohort had concomitant ROTEM® and standard coagulation testing on admission. No statistically significant difference was found between the two ROC curves for FIBTEM amplitude at 5 min (A5), FIBTEM maximum clot firmness, EXTEM clotting time (CT) and EXTEM A5 for ROTEM® Sigma and Delta to diagnose post-traumatic coagulation disorders. The best threshold for FIBTEM A5 to predict low fibrinogen concentration was 7 mm for each device. EXTEM CT thresholds to diagnose PT ratio > 1.2 were 78 s and 74 s for ROTEM® Sigma and Delta, respectively. Conclusions: These results suggest that ROTEM®-based algorithms may be transposed from one trauma centre to another independently of the setting and the ROTEM® device in use. [ABSTRACT FROM AUTHOR]

Studies the clinicopathological forms and the prognostic index in Budd-Chiari syndrome by researchers from Clichy, France. Significance of the development of a prognostic index for Budd-Chiari syndrome on the field of gastroentrology; Effects of combining acute and chronic lesions histologically on results of the test using the model; Accuracy of the novel prognostic tool.