Proceedings of the 3rd BEAT-PCD Conference and 4th PCD Training School

Abstract Primary ciliary dyskinesia (PCD) is a chronic suppurative airways disease that is usually recessively inherited and has marked clinical phenotypic heterogeneity. Classic symptoms include neonatal respiratory distress, chronic rhinitis since early childhood, chronic otitis media, recurrent airway infections leading to bronchiectasis, chronic sinusitis, laterality defects with and without congenital heart disease including abnormal situs in approximately 50% of the cases, and male infertility. Lung function deteriorates progressively from childhood throughout life. ‘Better Experimental Approaches to Treat Primary Ciliary Dyskinesia’ (BEAT-PCD) is a network of scientists and clinicians coordinating research from basic science through to clinical care with the intention of developing treatments and diagnostics that lead to improved long-term outcomes for patients. BEAT-PCD activities are supported by EU funded COST Action (BM1407). The third BEAT-PCD conference and fourth PCD training school were held jointly in February 2018 in Lisbon, Portugal. Presentations and workshops focussed on advancing the knowledge and skills relating to PCD in: basic science, epidemiology, diagnostic testing, clinical management and clinical trials. The multidisciplinary conference provided an interactive platform for exchanging ideas through a program of lectures, poster presentations, breakout sessions and workshops. Three working groups met to plan consensus statements. Progress with BEAT-PCD projects was shared and new collaborations were fostered. In this report, we summarize the meeting, highlighting developments made during the meeting

LIS education and cultural heritage information management

It has been more than two decades that LIS Education has been regarding information organizations (libraries, archives and museums) as an integrated field sharing a common basis of acquiring, handling and disseminating information independently of its source or substrate. In this respect LIS curriculum has treated resources at the basis of their descriptive, structural and administrative metadata; their semantic value; and the employment of technology as the means for creating a common platform for its organization, accessing and dissemination purposes. Within this framework, LIS curriculum has expanded in order to meet the informational needs of cultural heritage institutions, such as museums and cultural centers. Furthermore, their requisites to create the fundamentals for their digital information management and digital presence in the web and their obligations to supply information and communicate with each other became also part of the curriculum. The latter introduced almost simultaneously the need to operate in common environment with archives and libraries that not only hold a significant amount of related information but also have common operational attributes. The latter has brought forward issues of interoperability, communication between metadata standards, the use of controlled vocabularies and semantic representation techniques. All of the above, have established the notion in LIS Education that libraries, archives and museums are a global network of information organizations. At the same time, a series of cultural heritage projects, funded through the Horizon2020 call were focusing on employing new technologies for staging cultural heritage content. The need for metadata and semantics had acquired the expertise of information professionals. In this paper the case of the curriculum reform of the Department of Archives, Library and information Studies of the University of West Attica is presented and the specific interrelationship of the Horizon2020 Project “CrossCult” in which the aforementioned Department participated, is viewed as a parameter for shaping a cluster of courses within its curriculum. Horizon 2020 is the biggest EU Research and Innovation programme ever with nearly €80 billion of funding available over 7 years (2014 to 2020) - https://ec.europa.eu/programmes/horizon2020/what-horizon-2020 CrossCult: "Empowering reuse of digital cultural heritage in context-aware crosscuts of European history”, website: https://www.crosscult.eu/en/about/background/ (Horizon 2020)

Association of non-invasive measures of subclinical atherosclerosis and arterial stiffness with mortality and major cardiovascular events in chronic kidney disease: systematic review and meta-analysis of cohort studies

Background Non-invasive cardiovascular disease (CVD) risk prediction, in subclinical stages, aiming to stratify patients and tailor interventions remains an unmet need in chronic kidney disease (CKD). In this meta-analysis, we summarize the association of carotid intima–media thickness (cIMT), coronary artery calcium score (CACS) and pulse wave velocity (PWV) with all-cause mortality, cardiovascular (CV) mortality and CV events in non-dialysis CKD and patients on haemodialysis. Methods Systematic review and meta-analysis of prospective cohort studies. Results Out of 27 984 records, a total of 45 studies were eligible for quantitative synthesis; 11 for cIMT, 18 for CACS and 16 for PWV involving 2235, 4904 and 5717 patients, respectively. Meta-analysis was possible from pooled data of five cIMT studies (708 subjects), eight CACS studies (862 subjects) and nine PWV studies (1508 subjects). In dialysis patients, cIMT was associated with all-cause mortality [relative risk (RR) per unit increase: 1.08, 95% confidence interval (CI) 1.00–1.17, I2: 68%] and CV mortality (RR: 1.29, 95% CI 1.14–1.47, I2: 0%). High versus low CACS was associated with all-cause mortality (RR: 2.51, 95% CI 1.66–3.79, I2: 5.7%) and CV events (RR: 3.77 95% CI 2.16–6.58, I2: 20.2%). High versus low PWV was associated with all-cause (RR: 5.34, 95% CI 3.01–9.47, I2: 0%) and CV mortality (RR: 8.55, 95% CI 4.37–16.73, I2: 0%). The combined estimated for all-cause mortality per 1 m/s increment unit in PWV was 1.25 (95% CI 1.17–1.34, I2: 0%) and for CV mortality was 1.24 (95% CI 1.16–1.34, I2: 15.5%). In non-dialysis patients, CACS was associated with CV events (RR: 4.02, 95% CI 1.57–10.29, I2: 63.4%). High versus low PWV was associated with all-cause mortality (RR: 2.52, 95% CI 1.40–4.55, I2: 62.6%). Conclusions Non-invasive measures of atherosclerosis and arterial stiffening are associated with all-cause and CV mortality as well as CV events among patients with all stages of CKD. These markers could be considered for the evaluation of CV morbidity and mortality risks. Moreover, the results of this meta-analysis support the study of interventions, with effect on these markers of vascular disease, on long-term CVD outcomes