Fanconi's anaemia and haemophilia A are born inherited diseases creating haemostatic defects. The association of these two rare diseases in one patient is described. The patient's haemophilia was studied with a newly developed immunological technique determining the plasma antigen associated with Factor VIII activity, and was found to be a genetic variant of moderately severe haemophilia A. It was

BACKGROUND: The Global Burden of Diseases, Injuries, and Risk Factors Study 2015 provides an up-to-date synthesis of the evidence for risk factor exposure and the attributable burden of disease. By providing national and subnational assessments spanning the past 25 years, this study can inform debates on the importance of addressing risks in context.METHODS: We used the comparative risk assessment

Disaster risk management (DRM) requires the collaboration of a variety of stakeholders working in different sectors. They depend on each other to share risk information, and effective collaboration requires efficient communication. Clearly, the communication of risk descriptions is a key issue for the success of DRM activities. This study investigated the communication of risk-related information

Studies have demonstrated that neoliberal governance dismantles professionals? will to critique, counteracting efforts to improve quality by preventing professionals in all sectors of the labour market from grounding work in their professional convictions. Managing attempts at governance has therefore become an important professional competence. This paper investigates how higher education prepareStudies have demonstrated that neoliberal governance dismantles professionals’ will to critique, counteracting efforts to improve quality by preventing professionals in all sectors of the labour market from grounding work in their professional convictions. Managing attempts at governance has therefore become an important professional competence. This paper investigates how higher education prepare

Floran i den sydvästligaste delen av Västaustralien är idag internationellt utmärkt somen Biodiversity Hotspot, dvs. ett område som måste bevaras för omvärlden på grundav sin unika artrikedom och höga andel endemiska arter.

To reduce the congestion due to the future bandwidth- hungry applications in domains such as Health care, Internet of Things (IoT), etc., we study the benefit of introducing additional Data Centers (DCs) closer to the network edge for the optimal application placement. Our study shows that the edge layer DCs in a Mobile Edge Network (MEN) infrastructure is cost beneficial for the bandwidth-hungry

Den historiedidaktiska processen kring hur en konflikt på en möbelfabrik används ur ett historiebruksperspektiv samt hur historiemedvetande påverkar historiebruket.

Background The familial risks of heart failure (HF) remain largely undetermined. This nationwide follow-up study aimed at determining risk of hospitalization for HF conferred by affected siblings. Methods and results Swedish Multi-generation Register data, with records of at least one full sibling available at start of follow-up, were linked to the Hospital Discharge Register data for 1987–2010. T

This study focuses on the development of unstructured models, including both substrate and product inhibition, that predict the cultivation of Actinobacillus succinogenes and Basfia succiniciproducens on a mixture of C5 and C6 sugars, similar to the sugar composition contained in spent sulphite liquor, the liquid waste stream from the sulphite pulping process. The main sugar monomer contained in t

Brain imaging is essential for the diagnosis and characterization of cerebral small vessel disease. Several magnetic resonance imaging markers have therefore emerged, providing new information on the diagnosis, progression, and mechanisms of small vessel disease. Yet, the reproducibility of these small vessel disease markers has received little attention despite being widely used in cross-sectiona

Survival rates of childhood cancer have improved markedly, and today more than 80 % of those diagnosed with a pediatric malignancy will become 5-year survivors. Nevertheless, survivors exposed to cranial radiotherapy (CRT) are at particularly high risk for long-term morbidity, such as endocrine insufficiencies, metabolic complications, and cardiovascular morbidity. Deficiencies of one or more ante

The localized deformation patterns developed during in-plane compression and folding of paperboard have been studied in this work. X-ray post-mortem images reveal that cellulose fibres have been reoriented along localized bands in both the compression and folding tests. In folding, the paperboard typically fails on the side where the compressive stresses exists and wrinkles are formed. The in-plan

Reformer som fokuserar på ökad privatisering och konkurrens bland utförare och ökad valfrihet för individer(patienter, elever, brukare)har införts i svensk hälso-och sjukvård, skola och omsorg. Individer ska kunnavälja bland offentliga och privata utförare som finansieras med offentliga medel och som förväntas konkurrera om elever, patienter och brukare på en reglerad marknad–en välfärdsmarknad.I

20th century massification of higher education and research in academia is said to have produced structurally stratified higher education systems in many countries. Most manifestly, the research mission of universities appears to be divisive. Authors have claimed that the Swedish system, while formally unified, has developed into a binary state, and statistics seem to support this conclusion. This

Background and study aims: This study aimed to externally validate a recently developed English model for the prediction of 30-day mortality after endoscopic retrograde cholangiopancreatography (ERCP). Real-world mortality data beyond 30 days post-ERCP are scarce; thus, the study also aimed to develop a prediction model for mortality up to 12 months post-ERCP. Patients and methods: All patients w

Small B cell lymphoid neoplasms are the most common lymphoproliferative disorders involving peripheral blood (PB) and bone marrow (BM). The Bone Marrow Workshop (BMW) organized by the European Bone Marrow Working Group (EBMWG) of the European Association for Haematopathology (EAHP) during the XVIIth EAHP Meeting in Istanbul, October 2014, was dedicated to discussion of cases illustrating how the r