Ehlers-Danlos syndrome type I. Ultrastructural study

Ehlers-Danlos syndrome comprises a very heterogeneous group of collagen diseases characterised in clinical terms by fragility and cutaneous hyperextensability, ligamentous hyperlaxity, ecchymosis, scarring, visceral and neurological manifestations. Having been described in detail by Ehlers in 1899 and Danlos in 1908, it was subsequently classified into various clinical types. At present at least 11 forms are recognised on the basis of their clinical characteristics, methods of transmission and biochemical defect; the first four types of the syndrome account for approximately 95% of cases. Almost all forms are transmitted with a dominant autosomic character. Specific genetic mutations have been ascertained whereas the biochemical defect has been identified in numerous types. Ehlers-Danlos type 1 syndrome is the most frequent and most severe form. The biochemical anomaly underlying the altered deposition of collagen fibre is still unknown and this is responsible for the "storiform" appearance of collagen fibre on ultrastructural examination. The authors have described a typical case of "Ehlers-Danlos type 1 syndrome" in which the diagnosis was confirmed by comparing clinical data and the results of ultrastructural tests which revealed the characteristic "pattern" of collagen fibres.     

Nonlinear fatigue crack propagation in a baffle module of Wendelstein 7‐X under cyclic bending loads

Simulation of the fatigue crack propagation in a Wendelstein 7‐X baffle module is performed in this study using both a finite element method‐based software and the UniGrow nonlinear model for small‐scale yielding (SSY) conditions. Some experimental fatigue tests of several cracked baffle modules have been performed through a servo‐hydraulic machine. One of these experimental tests has been considered to simulate fatigue crack propagation in the baffle module. Before starting the experimental test, a first crack partly contained in the welding seam and partly in the steel pipe is found. Subsequently, owing to the applied load, the crack propagated both into the welding seam and into the steel pipe until the plastic zone in the near field attains SSY conditions. Finally, owing to the increase in the extension of the plastic zone, SSY conditions are not more valid, and the breakage of the steel pipe is produced by plastic collapse.     

Informing additive manufacturing technology adoption: total cost and the impact of capacity utilisation

Informing Additive Manufacturing (AM) technology adoption decisions, this paper investigates the relationship between build volume capacity utilisation and efficient technology operation in an inter-process comparison of the costs of manufacturing a complex component used in the packaging industry. Confronting the reported costs of a conventional machining and welding pathway with an estimator of the costs incurred through an AM route utilising Direct Metal Laser Sintering (DMLS), we weave together four aspects: optimised capacity utilisation, ancillary process steps, the effect of build failure and design adaptation. Recognising that AM users can fill unused machine capacity with other, potentially unrelated, geometries, we posit a characteristic of ‘fungible’ build capacity. This aspect is integrated in the cost estimation framework through computational build volume packing, drawing on a basket of sample geometries. We show that the unit cost in mixed builds at full capacity is lower than in builds limited to a single type of geometry; in our study, this results in a mean unit cost overstatement of 157%. The estimated manufacturing cost savings from AM adoption range from 36 to 46%. Additionally, we indicate that operating cost savings resulting from design adaptation are likely to far outweigh the manufacturing cost advantage.     

Nanodroplet‐Containing Polymers for Efficient Low‐Power Light Upconversion

Sensitized triplet–triplet‐annihilation‐based photon upconversion (TTA‐UC) permits the conversion of light into radiation of higher energy and involves a sequence of photophysical processes between two dyes. In contrast to other upconversion schemes, TTA‐UC allows the frequency shifting of low‐intensity light, which makes it particularly suitable for solar‐energy harvesting technologies. High upconversion yields can be observed for low viscosity solutions of dyes; but, in solid materials, which are better suited for integration in devices, the process is usually less efficient. Here, it is shown that this problem can be solved by using transparent nanodroplet‐containing polymers that consist of a continuous polymer matrix and a dispersed liquid phase containing the upconverting dyes. These materials can be accessed by a simple one‐step procedure that involves the free‐radical polymerization of a microemulsion of hydrophilic monomers, a lipophilic solvent, the upconverting dyes, and a surfactant. Several glassy and rubbery materials are explored and a range of dyes that enable TTA‐UC in different spectral regions are utilized. The materials display upconversion efficiencies of up to ≈15%, approaching the performance of optimized oxygen‐free reference solutions. The data suggest that the matrix not only serves as mechanically coherent carrier for the upconverting liquid phase, but also provides good protection from atmospheric oxygen.     

Application of a semianalytical strain assessment and multiaxial fatigue analysis to compare rolling contact fatigue in twin-disk and full-scale wheel/rail contact conditions

A semianalytical model is introduced to assess rolling contact fatigue problems in railway applications. The constitutive law is based on the nonlinear kinematic and isotropic hardening model of Chaboche–Lemaitre, which allows the cyclic elastoplastic strain under the contact surface to be evaluated. The much higher computational effectiveness in comparison with finite element (FE) analyses is quantified. The Dang Van multiaxial fatigue criterion is implemented to evaluate the rolling contact fatigue in the subsurface elastic region where cracking is relatively rare but more dangerous than surface cracks. The influence of the presence of sulfides in the wheel matrix in decreasing fatigue strength is also assessed by means of Murakami's approach. The model is used to compare conditions under small-scale twin-disk experiments to full-scale wheel/rail contact conditions. It is found that, for the same Hertzian pressure, the small-scale contact is more conservative in that it causes a deeper plasticized layer as compared with the elliptical full-scale contact. In the investigated cases, crack initiation is also not expected according to Dang Van criterion in neither of the studied contact conditions.     

A bibliometric methodology to unveil territorial inequities in the scientific wealth to combat COVID-19

Scientometrics - In this paper we develop a methodology to assess the scientific wealth of territories at field level. Our methodology uses a bibliometric approach based on the observation of...     

Weak quantum discord

Originally introduced as the difference between two possible forms of quantum mutual information, quantum discord has posteriorly been shown to admit a formulation according to which it measures a distance between the state under scrutiny and the closest projectively measured (non-discordant) state. Recently, it has been shown that quantum discord results in higher values when projective measurements are substituted by weak measurements. This sounds paradoxical since weaker measurements should imply weaker disturbance and, thus, a smaller distance. In this work, we solve this puzzle by presenting a quantifier and an underlying interpretation for what we call weak quantum discord. As a by-product, we introduce the notion of symmetrical weak quantum discord.     

The multi-modal interface of Robot-Era multi-robot services tailored for the elderly

Socially assistive robotic platforms are now a realistic option for the long-term care of ageing populations. Elderly users may benefit from many services provided by robots operating in different environments, such as providing assistance inside apartments, serving in shared facilities of buildings or guiding people outdoors. In this paper, we present the experience gained within the EU FP7 ROBOT-ERA project towards the objective of implementing easy-to-use and acceptable service robotic system for the elderly. In particular, we detail the user-centred design and the experimental evaluation in realistic environments of a web-based multi-modal user interface tailored for elderly users of near future multi-robot services. Experimental results demonstrate positive evaluation of usability and willingness to use by elderly users, especially those less experienced with technological devices who could benefit more from the adoption of robotic services. Further analyses showed how multi-modal modes of interaction support more flexible and natural elderly–robot interaction, make clear the benefits for the users and, therefore, increase its acceptability. Finally, we provide insights and lessons learned from the extensive experimentation, which, to the best of our knowledge, is one of the largest experimentation of a multi-robot multi-service system so far.     

Surface Functionalization with Phosphazene Substrates. Part II. Theoretical and Experimental Investigations of the Interactions of Hexachlorocyclophosphazene with Hydroxylated Silicon-based Surfaces

The interaction of hexachlorocyclophosphazene (HCCP) with the hydroxylated silicon-based surfaces is studied by a combination of XPS experimental analysis and theoretical ab initio simulations, including the estimate of the energy barriers via CI-NEB method to determine the minimum energy reaction paths. Several possible, stable chemisorbed configurations are found to exist, whose structural, energetic, and electronic properties have been analyzed in detail. The theoretical results are in good qualitative agreement with available experimental data. In particular, we get indications that water plays a crucial role in the surface functionalization with HCCP, although the presence of a suitable solvent (e.g. tetrahydrofuran, THF) seems to be essential. This paper is dedicated to Prof. Harry R. Allcock and the pioneering discoveries of his research group particularly in polyphospazenes and generally in materials science.     

Multi-Domain Model of Faulty Stator Core for Thermal Effects and Losses Evaluation

In this work, a multi-domain model of the stator core of an electric machine is presented. Its electromagnetic behavior and the thermal dynamics are represented using Bond Graph. This approach makes apparent the topology of the machine and the energy exchange. This feature enables the model for the easy representation of faults and analysis of their consequences in the thermal/magnetic/electric domain. The proposed model is used for reproducing the behavior of the stator core during an inter-laminar insulation test. The model is capable to reproduce the thermal distribution when a fault in the stator core occurs. This contribution helps to estimate the additional iron losses due to the stator core fault based on the thermal distribution. Additionally, this methodology could help to define the proper maintenance intervention to be performed on the machine.