The EAS FHSC is an international initiative involving a network of investigators interested in FH from around 70 countries.• Information on FH prevalence is lacking in most countries; where available, data tend to align with contemporary estimates.• FH diagnosis and management varies widely across countries, with overall suboptimal identification and under-treatment.• In most countries diagnosis primarily relies on DLCN criteria, and less frequently on Simon Broom or MEDPED.• Therapy for FH is not universally reimbursed, and criteria vary across countries. Access to PCSK9i and apheresis is limited.

r Endocrinologie m etabolisme et prevention cardiovasculaire, Institut E3M et IHU cardiom etabolique (ICAN), HôpitalPiti eSalpêtri ere,

The EAS FHSC represents an excellent opportunity to integrate individual efforts across the world to tackle the global burden of FH. The information garnered from the registry will help reduce gaps in knowledge, inform best practices, assist in clinical trials design, support clinical guidelines and policies development, and ultimately improve the care of FH patients.

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PurposeWith various challenges in the digital era, stakeholders are expressing growing interests in understanding the impact of emerging and disruptive technologies on freight transportation. This paper provides a systematic literature review of the current state of affairs as well as future trends and aims to support stakeholders' decision-making in logistics management in the era of disruptive technologies.Design/methodology/approachSeveral recent and representative articles from academic, industrial and governmental perspectives were investigated to set the scene for this research and to serve as a baseline for electing nine emerging technologies, which were then used to conduct a systematic literature review covering the literature within the area during the past twelve years.Findings3D printing, artificial intelligence, automated robots, autonomous vehicles, big data analytics, blockchain, drones, electric vehicles and the Internet of Things were identified as the emerging technologies. The current state of existing research and potential future opportunities were analyzed.Research limitations/implicationsSince the potential literature body is almost impossible to fully cover, a tradeoff between the number of emerging technologies and the related literature reviewed has been performed. However, the paper provides a novel approach to select the emerging and disruptive technologies and a systematic literature review to fill the identified research gap in the related literature.Practical implicationsThe research support various stakeholders to better capture the current status of and the future opportunities in freight transportation and gain a clearer understanding of the disruptive technologies as well as to guide them in how to deploy these initiatives in future decision-making.Originality/valueBy providing a systematic literature review on the trends, themes and research opportunities in the era of disruptive technologies, the papers bring about broad and comprehensive review on the impact of disruptive technologies on logistics and transportation as well as opportunities to support management decision support in the logistics industry.

The Virtual Atomic and Molecular Data Centre (VAMDC, http://www.vamdc.eu/) is a European-Union-funded collaboration between groups involved in the generation, evaluation, and use of atomic and molecular data. VAMDC aims to build a reliable, open, flexible and interoperable e-science interface to existing atomic and molecular data. The project will cover establishing the core consortium, the development and deployment of the infrastructure and the development of interfaces to the existing atomic and molecular databases. This paper describes the organisation of the project and the achievements during its first year.

As the main computing paradigm for resourceintensive scientific applications, Grid[1] enables resource sharing and dynamic allocation of computational resources. It promotes access to distributed data, operational flexibility and collaboration, and allows service providers to be distributed both conceptually and physically to meeting different requirements. Largescale grids are normally composed of huge numbers of components from different sites, which increases the requirements of workflows and quality of service (QoS) upon these workflows as many of these components have real-time requirements.In this paper, we describe our web services based QoS-aware workflow management system(WfMS) from GridCC project[7] and show how this WfMS can aid to ensure workflows meet the pre-defined QoS requirements and optimise them accordingly.

This paper describes 'PathGrid'-an analysis and data integration system, developed initially to meet the demands in the analysis of medical microscopy imaging data. An overview of the current system is given, describing the techniques used in developing the data handling infrastructure and the analysis algorithm development. The use of software created in the context of systems designed for the astronomy domain is noted, specifically infrastructure from the astronomy virtual observatory movement for data discovery, access and workflow management, and astronomical image analysis software adapted for the analysis of high-throughput astronomy imaging surveys. This paper notes the applicability of the techniques from the astronomy domain. The testbed infrastructure deployment is described, emphasizing its speed and ease of use and support. The validity of the analysis techniques is confirmed through the pilot study described here-with the application to a large sample of immunohistochemistry microscopy data obtained in part for assessing the oestrogen receptor status of breast cancers. The analysis showed that the specificity and sensitivity values for the automatic scoring using PathGrid were within the errors of those obtained via a 'gold standard' manual pathologist scoring.