Pregnant women with bleeding disorders require specialised peripartum care to prevent postpartum haemorrhage (PPH). If third trimester coagulation factor levels are <0.50 IU mL(-1) , prophylactic treatment is indicated and administered according to international guidelines. However, optimal dose and duration are unknown and bleeding may still occur. The aim of this study was to investigate the outcome in women with von Willebrand disease (VWD) or haemophilia carriership treated according to current practice guidelines. From the period 2002-2011, 185 deliveries in 154 VWD women or haemophilia carriers were retrospectively included. Data on blood loss, bleeding disorder characteristics and obstetric risk factors were obtained. The outcome was primary PPH, defined as blood loss ≥500 mL within 24 h postpartum and severe PPH as blood loss ≥1000 mL. Primary PPH was observed in 62 deliveries (34%), 14 (8%) of which resulted in severe PPH. In 26 deliveries prophylactic treatment was administered due to factor levels below the 0.50 IU mL(-1) cut-off in the third trimester, 14 of which (54%) were complicated by PPH. We found an increased PPH risk in deliveries given prophylactic treatment compared with deliveries without (OR 2.7, 95% CI 1.2-6.3). In conclusion, PPH incidence was highest in deliveries with the lowest factor levels in the third trimester. Currently, delivery outcome in women with bleeding disorders is unsatisfactory, given the high PPH incidence despite specialised care. Future studies are required to optimise management of deliveries in this patient population.

Introduction:Monitoring clinical outcome in persons with haemophilia (PWH) is essential in order to provide optimal treatment for individual patients and compare effectiveness of treatment strategies. Experience with measurement of activities and participation in haemophilia is limited and consensus on preferred tools is lacking. Aim:The aim of this study was to give a comprehensive overview of the measurement properties of a selection of commonly used tools developed to assess activities and participation in PWH.Methods: Electronic databases were searched for articles that reported on reliability, validity or responsiveness of predetermined measurement tools (5 self-reported and 4 performance based measurement tools). Methodological quality of the studies was assessed according to the COSMIN checklist. Best evidence synthesis was used to summarize evidence on the measurement properties. Results:The search resulted in 3453 unique hits. Forty-two articles were included.The self-reported Haemophilia Acitivity List (HAL), Pediatric HAL (PedHAL) and the performance based Functional Independence Score in Haemophilia (FISH) were studied most extensively. Methodological quality of the studies was limited. Measurement error, cross-cultural validity and responsiveness have been insufficiently evaluated. Conclusion:Albeit based on limited evidence, the measurement properties of the PedHAL, HAL and FISH are currently considered most satisfactory. Further research needs to focus on measurement error, responsiveness, interpretability and crosscultural validity of the self-reported tools and validity of performance based tools which are able to assess limitations in sports and leisure activities. K E Y W O R D Sactivities, haemophilia, measurement properties, outcome measure, participationThis is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made. 4 According to the ICF "activity" is defined as "the execution of a task or action by an individual" and participation is defined as "involvement in a life situation". Consistent monitoring outcome at activity and participation level still needs to find its way into haemophilia care and research.Various haemophilia specific and generic tools for assessing activity and participation are available, but consensus on a preferred set of measurement tools is not yet reached. Differences in access to expensive clotting factor concentrates cause significant differences in joint status between PWH in different parts of the world. Differences between age groups, severity and cultural differences also contribute to heterogeneity in the haemophilia population. Moreover, measurement tools might be used for different purposes, eg. monitoring clinical outcome vs comparing treatment groups and evaluation of chronic complaints vs acute bleeds. A core set of measurement tools shou...

Intracranial hemorrhage (ICH) is a severe complication that is relatively common among patients with hemophilia. This systematic review aimed to obtain more precise estimates of ICH incidence and mortality in hemophilia, which may be important for patients, caregivers, researchers, and health policy makers. PubMed and EMBASE were systematically searched using terms related to “hemophilia” and “intracranial hemorrhage” or “mortality.” Studies that allowed calculation of ICH incidence or mortality rates in a hemophilia population ≥50 patients were included. We summarized evidence on ICH incidence and calculated pooled ICH incidence and mortality in 3 age groups: persons of all ages with hemophilia, children and young adults younger than age 25 years with hemophilia, and neonates with hemophilia. Incidence and mortality were pooled with a Poisson-Normal model or a Binomial-Normal model. We included 45 studies that represented 54 470 patients, 809 151 person-years, and 5326 live births of patients with hemophilia. In persons of all ages, the pooled ICH incidence and mortality rates were 2.3 (95% confidence interval [CI], 1.2-4.8) and 0.8 (95% CI 0.5-1.2) per 1000 person-years, respectively. In children and young adults, the pooled ICH incidence and mortality rates were 7.4 (95% CI, 4.9-11.1) and 0.5 (95% CI, 0.3-0.9) per 1000 person-years, respectively. In neonates, the pooled cumulative ICH incidence was 2.1% (95% CI, 1.5-2.8) per 100 live births. ICH was classified as spontaneous in 35% to 58% of cases. Our findings suggest that ICH is an important problem in hemophilia that occurs among all ages, requiring adequate preventive strategies.

Detailed information on the onset, frequency and severity of bleeding in non-severe hemophilia is limited. We aimed to assess the bleeding phenotype of people with non-severe hemophilia, and to analyse the association between baseline factor VIII/IX levels and the joint bleeding rate. In the DYNAMO study, an international multicenter cohort, we included males with non-severe hemophilia (factor VIII/IX (FVIII/IX) 0.02-0.35 IU/mL) aged 12-55 years. Information on age at first treated (joint) bleed and the annual (joint) bleeding rates (A(J)BR) were collected from the medical files. The association between baseline FVIII/IX levels and the joint bleeding rate was assessed using a frailty model for recurrent events. In total, 304 people (70 moderate and 234 mild hemophilia) were included. The median age was 38 years (IQR 25-49) and the median baseline FVIII/IX level was 0.12 IU/mL (IQR 0.05-0.21). In total, 245 (81%) people had experienced at least one bleed and 156 (51%) had experienced at least one joint bleed. The median age at first bleed and first joint bleed was 8 and 10 years, respectively. The median ABR and AJBR was 0.2 (IQR 0.1-0.5) and 0.0 (IQR 0.0-0.2), respectively. From baseline FVIII/IX levels 0.02-0.05 IU/mL to &gt;0.25 IU/mL, the median ABR decreased from 0.6 (IQR 0.2-1.4) to 0.1 (0.0-0.2) and the AJBR from 0.2 (0.0-0.4) to 0.0 (0.0-0.0). Baseline FVIII/IX was inversely associated with the joint bleeding rate (p &lt;0.001). Low bleeding rates were observed in people with non-severe hemophilia. However, half of all adolescents and adults had experienced a joint bleed.