New insights into the outflows from R Aquarii

R Aquarii is a symbiotic binary surrounded by a large and complex nebula with a prominent curved jet. It is one of the closest known symbiotic systems, and therefore offers a unique opportunity to study the central regions of these systems and the formation and evolution of astrophysical jets. We studied the evolution of the central jet and outer nebula of R Aqr taking advantage of a long term monitoring campaign of optical imaging, as well as of high-resolution integral field spectroscopy. Narrow-band images acquired over a period of more than 21 years are compared in order to study the expansion and evolution of all components of the R Aqr nebula. The magnification method is used to derive the kinematic ages of the features that appear to expand radially. Integral field spectroscopy of the OIII 5007A emission is used to study the velocity structure of the central regions of the jet. New extended features, further out than the previously known hourglass nebula, are detected. The kinematic distance to R Aqr is calculated to be 178 pc using the expansion of the large hourglass nebula. This nebula of R Aqr is found to be roughly 650 years old, while the inner regions have ages ranging from 125 to 290 years. The outer nebula is found to be well described by a ballistic expansion, while for most components of the jet strong deviations from such behaviour are found. We find that the Northern jet is mostly red-shifted while its Southern part is blue-shifted, apparently at odds with findings from previous studies but almost certainly a consequence of the complex nature of the jet and variations in ionisation and illumination between observations.Comment: 13 pages, 8 figures, accepted for publication in A&

Laboratory misdiagnosis of von Willebrand disease in post- menarchal females: A multi- center study

Increased awareness of von Willebrand Disease (VWD) has led to more frequent diagnostic laboratory testing, which insurers often dictate be performed at a facility with off- site laboratory processing, instead of a coagulation facility with onsite processing. Off- site processing is more prone to preanalytical variables causing falsely low levels of von Willebrand Factor (VWF) due to the additional transport required. Our aim was to determine the percentage of discordance between off- site and onsite specimen processing for VWD in this multicenter, retrospective study. We enrolled females aged 12 to 50- years who had off- site specimen processing for VWF assays, and repeat testing performed at a consulting institution with onsite coagulation phlebotomy and processing. A total of 263 females from 17 institutions were included in the analysis. There were 251 subjects with both off- site and onsite VWF antigen (VWF:Ag) processing with 96 (38%) being low off- site and 56 (22%) low onsite; 223 subjects had VWF ristocetin co- factor (VWF:RCo), 122 (55%) were low off- site and 71 (32%) were low onsite. Similarly, 229 subjects had a Factor VIII (FVIII) assay, and 67 (29%) were low off- site with less than half, 29 (13%) confirmed low with onsite processing. Higher proportions of patients demonstrated low VWF:Ag, VWF:RCo, and/or FVIII with off- site processing compared to onsite (McNemarʼs test P- value <.0005, for all assays). These results emphasize the need to decrease delays from sample procurement to processing for VWF assays. The VWF assays should ideally be collected and processed at the same site under the guidance of a hematologist.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/156476/2/ajh25869.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/156476/1/ajh25869_am.pd

Building the foundation for a community-generated national research blueprint for inherited bleeding disorders: research priorities for mucocutaneous bleeding disorders

BACKGROUND: Excessive or abnormal mucocutaneous bleeding (MCB) may impact all aspects of the physical and psychosocial wellbeing of those who live with it (PWMCB). The evidence base for the optimal diagnosis and management of disorders such as inherited platelet disorders, hereditary hemorrhagic telangiectasia (HHT), hypermobility spectrum disorders (HSD), Ehlers-Danlos syndromes (EDS), and von Willebrand disease (VWD) remains thin with enormous potential for targeted research. RESEARCH DESIGN AND METHODS: National Hemophilia Foundation and American Thrombosis and Hemostasis Network initiated the development of a National Research Blueprint for Inherited Bleeding Disorders with extensive all-stakeholder consultations to identify the priorities of people with inherited bleeding disorders and those who care for them. They recruited multidisciplinary expert working groups (WG) to distill community-identified priorities into concrete research questions and score their feasibility, impact, and risk. RESULTS: WG2 detailed 38 high priority research questions concerning the biology of MCB, VWD, inherited qualitative platelet function defects, HDS/EDS, HHT, bleeding disorder of unknown cause, novel therapeutics, and aging. CONCLUSIONS: Improving our understanding of the basic biology of MCB, large cohort longitudinal natural history studies, collaboration, and creative approaches to novel therapeutics will be important in maximizing the benefit of future research for the entire MCB community

Von Willebrand disease: gaining a global perspective

Introduction Recent guidelines for von Willebrand Disease (VWD) highlighted the challenges in diagnosis and management. Identifying the number of persons with VWD (PwVWD) internationally will help target support to aid diagnosis of PwVWD. Aim To examine international registration rates of PwVWD, the influence of income status, geographical region and the age and sex profile. Cumulatively, these data will be used to inform future strategy from the World Federation of Haemophilia (WFH) to address unmet clinical and research needs. Methods Data from the 2018/2019 WFH Annual Global Survey (AGS) were analysed, providing a global perspective on VWD registration. Results Registration rates are lowest in South Asia (0.6/million population) and highest in Europe/Central Asia (50.9/million population, 0.005%), but below the expected prevalence rate (0.1%). National economic status impacted VWD registration rates, reflecting variation in access to optimal healthcare infrastructure. Females represented the majority of PwVWD globally, however, in low-income countries (LIC) males predominated. Age profile varied, with markedly higher rates of paediatric registrations in North America, Middle East and North Africa and South Asia. Rates of type 3 VWD registrations were significantly influenced by economic status (81% of VWD diagnoses in LIC), suggesting only the most severe VWD types are diagnosed in resource limited settings. Conclusion Significant variation in registration rates of PwVWD exist internationally and is influenced by income status and the presence of HTC networks. Improved understanding of registration rates will enable targeting of advocacy to improve awareness, diagnosis and support for PwVWD internationally. Key points Registration rates of People with Von Willebrand Disease (PwVWD) vary internationally and are influenced by national income status Although females represent the majority of PwVWD globally, in low income countries (LIC) males predominated, possibly related to stigma surrounding gynaecological bleeding. Rates of type 3 VWD registration were significantly influenced by economic status (81% of VWD diagnoses in LIC), suggesting only the most severe VWD types are diagnosed in resource limited settings

von Willebrand disease: proposing definitions for future research

Thrombosis and Hemostasi

Spawning aggregations of fish in Cabo Delgado, Northern Mozambique: An interview-based survey of artisanal fishers

Seasonal aggregations at specific sites are common among some fish species. Experienced fishermen know where and when these aggregations happen, often targeting these spawning sites to increase their catch. This strategy can further contribute to endangering the survival of these species, especially for those already threatened by other forms of pressure. This study contributes knowledge to the spawning aggregation of fish in the north of Mozambique following survey work conducted around Cabo Delgado Province in six coastal villages in 2016, 2017 and 2018. The objectives of the study were 1) to identify the species and sites of spawning aggregation and 2) to determine the timing of spawning aggregations. Data collection consisted of interviews targeting the most experienced fishermen to obtain information on these subjects. The results indicate that out of 124 fishermen interviewed, 59% had knowledge of spawning aggregations. The information collected from fisher interviews revealed that there are at least six spawning locations and at least eight species aggregate to spawn (Leptoscarus vaigensis, Lethrinus harak, Lethrinus nebulosus, Lethrinus obsoletus, Lethrinus olivaceus, Plectorhinchus gaterinus, Plectorhinchus schotaf, Siganus sutor). Only Siganus sutor was reported to spawn in at least one of the fishing areas used by every village. The timing of spawning aggregations most reported by fishermen was during the Kusi (South) monsoon period