Technology, business model, and market design adaptation toward smart electricity distribution: Insights for policy making

The transformation of the electricity sector towards a sustainable energy supply and use has a disruptive potential for infrastructure and utilities. The spread of digital technologies, renewable energy, and prosumers requires a swift and well-guided adaptation of the electricity distribution industry to smart grid technologies and related business models. This paper, based on the large technical systems (LTS) conceptual framework, investigates the complex evolution and company and market design adaptation needs. Challenges and opportunities are analyzed through nine multi-stakeholder workshops, held in two EU member states (Germany and Portugal) in 2016–2017, engaging distribution system operators, researchers, academics, and integrated utility companies. The results indicate considerable uncertainty for distribution system operators regarding the value of large-scale smart meter rollouts. Also, a corporate culture with resistance to change is observed, challenging the integration of novel technologies and processes. Traditional regulation is seen as a barrier to smart grid investments, and is associated with job losses and knowledge destruction. Policy-makers can benefit from these insights on the dynamics of DSOs, which can contribute to public policy design and market reform which traditionally has often been mainly concerned about operational efficiency in a steady-state, stable economy

Correction to: Solving patients with rare diseases through programmatic reanalysis of genome-phenome data

In the original publication of the article, consortium author lists were missing in the articl

Factors Associated with Revision Surgery after Internal Fixation of Hip Fractures

Background: Femoral neck fractures are associated with high rates of revision surgery after management with internal fixation. Using data from the Fixation using Alternative Implants for the Treatment of Hip fractures (FAITH) trial evaluating methods of internal fixation in patients with femoral neck fractures, we investigated associations between baseline and surgical factors and the need for revision surgery to promote healing, relieve pain, treat infection or improve function over 24 months postsurgery. Additionally, we investigated factors associated with (1) hardware removal and (2) implant exchange from cancellous screws (CS) or sliding hip screw (SHS) to total hip arthroplasty, hemiarthroplasty, or another internal fixation device. Methods: We identified 15 potential factors a priori that may be associated with revision surgery, 7 with hardware removal, and 14 with implant exchange. We used multivariable Cox proportional hazards analyses in our investigation. Results: Factors associated with increased risk of revision surgery included: female sex, [hazard ratio (HR) 1.79, 95% confidence interval (CI) 1.25-2.50; P = 0.001], higher body mass index (fo

Solving patients with rare diseases through programmatic reanalysis of genome-phenome data.

Funder: EC | EC Seventh Framework Programm | FP7 Health (FP7-HEALTH - Specific Programme "Cooperation": Health); doi: https://doi.org/10.13039/100011272; Grant(s): 305444, 305444Funder: Ministerio de Economía y Competitividad (Ministry of Economy and Competitiveness); doi: https://doi.org/10.13039/501100003329Funder: Generalitat de Catalunya (Government of Catalonia); doi: https://doi.org/10.13039/501100002809Funder: EC | European Regional Development Fund (Europski Fond za Regionalni Razvoj); doi: https://doi.org/10.13039/501100008530Funder: Instituto Nacional de Bioinformática ELIXIR Implementation Studies Centro de Excelencia Severo OchoaFunder: EC | EC Seventh Framework Programm | FP7 Health (FP7-HEALTH - Specific Programme "Cooperation": Health)Reanalysis of inconclusive exome/genome sequencing data increases the diagnosis yield of patients with rare diseases. However, the cost and efforts required for reanalysis prevent its routine implementation in research and clinical environments. The Solve-RD project aims to reveal the molecular causes underlying undiagnosed rare diseases. One of the goals is to implement innovative approaches to reanalyse the exomes and genomes from thousands of well-studied undiagnosed cases. The raw genomic data is submitted to Solve-RD through the RD-Connect Genome-Phenome Analysis Platform (GPAP) together with standardised phenotypic and pedigree data. We have developed a programmatic workflow to reanalyse genome-phenome data. It uses the RD-Connect GPAP's Application Programming Interface (API) and relies on the big-data technologies upon which the system is built. We have applied the workflow to prioritise rare known pathogenic variants from 4411 undiagnosed cases. The queries returned an average of 1.45 variants per case, which first were evaluated in bulk by a panel of disease experts and afterwards specifically by the submitter of each case. A total of 120 index cases (21.2% of prioritised cases, 2.7% of all exome/genome-negative samples) have already been solved, with others being under investigation. The implementation of solutions as the one described here provide the technical framework to enable periodic case-level data re-evaluation in clinical settings, as recommended by the American College of Medical Genetics

An Exploratory Economic Analysis of Underground Pumped-Storage Hydro Power Plants in Abandoned Deep Coal Mines

This study researches the concept of underground pumped-storage hydro power plants in closed-down underground hard coal mines in Germany. After a review on how this could be realized technically, an economic feasibility analysis is presented, with a particular focus on the costs for the underground storage reservoir. The analysis is performed for different lower (i.e., underground) reservoir sizes and temporal arbitrage potentials (peak/off-peak electricity price spreads), and cost uncertainty is dealt with by means of a Monte Carlo simulation for two distinct head heights. The findings regarding costs and acceptability are compared with those of a classic (on-surface) pumped-storage hydro power plant in a mountainous area. Based on a techno-economic evaluation we conclude that under favorable conditions the realization of underground pumped-storage hydro power (UPSHP) plants seems both technically feasible and economically reasonable. More specifically, an extension of a tubular system seems the most promising option. A UPSHP plant in a mineshaft is probably slightly more expensive than a conventional one, an outcome that depends strongly on the feasible head height. However, the significant reduction of the adverse impacts on the landscape and on local residents, as well as a potentially large number of feasible sites in flat terrain, could make UPSHPs an interesting option for the future energy transition, not just in Germany but worldwide at sites where underground mining is being abandone