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Objectives: The MetabQoL 1.0 is the first disease-specific health related quality of life (HrQoL) questionnaire for patients with intoxication-type inherited metabolic disorders. Our aim was to assess the validity and reliability of the MetabQoL 1.0, and to investigate neuropsychiatric burden in our patient population. Methods: Data from 29 patients followed at a single center, aged between 8 and 18 years with the diagnosis of methylmalonic acidemia (MMA), propionic acidemia (PA) or isovaleric acidemia (IVA), and their parents were included. The Pediatric Quality of Life Inventory (PedsQoL) was used to evaluate the validity and reliability of MetabQoL 1.0.
Results: The MetabQoL 1.0 was shown to be valid and reliable (Cronbach's alpha: 0.64–0.9). Fourteen out of the 22 patients (63.6%) formally evaluated had neurological findings. Of note, 17 out of 20 patients (85%) had a psychiatric disorder when evaluated formally by a child and adolescent psychiatrist. The median mental scores of the MetabQoL 1.0 proxy report were significantly higher than those of the self report (p = 0.023). Patients with neonatal-onset disease had higher MetabQoL 1.0 proxy physical (p = 0.008), mental (p = 0.042), total scores (p = 0.022); and self report social (p = 0.007) and total scores (p = 0.043) than those with later onset disease.
Conclusions: This study continues to prove that the MetabQoL 1.0 is an effective tool to measure what matters in intoxication-type inherited metabolic disorders. Our results highlight the importance of clinical assessment complemented by patient reported outcomes which further expands the evaluation toolbox of inherited metabolic diseases.
In engineering design, optimization methods are frequently used to improve the initial design of a product. However, the selection of an appropriate method is challenging since many
methods exist, especially for the case of simulation-based optimization. This paper proposes a systematic procedure to support this selection process. Building upon quality function deployment, end-user and design use case requirements can be systematically taken into account via a decision
matrix. The design and construction of the decision matrix are explained in detail. The proposed
procedure is validated by two engineering optimization problems arising within the design of box-type boom cranes. For each problem, the problem statement and the respectively applied optimization methods are explained in detail. The results obtained by optimization validate the use
of optimization approaches within the design process. The application of the decision matrix shows the successful incorporation of customer requirements to the algorithm selection.
A step change is needed in the deployment of renewable energy if the triple challenge of ensuring climate change mitigation, energy security, and energy affordability is to be met. Yet, social acceptance of infrastructure projects and policies remains a key concern. While there has been decades of fruitful research on the social acceptance of wind energy and other renewables, much of the extant research is cross-sectional in nature, failing to capture the important dynamic processes that can make or break renewable energy projects. This paper introduces a Special Issue of Energy Policy which focuses on the neglected topic of the dynamics of social acceptance of renewable energy, drawing on contributions made at an international research conference held in St. Gallen (Switzerland) in June 2022. In addition to introducing these papers and drawing out common themes, we also seek to offer some conceptual clarity on the issue of dynamics in social acceptance, taking into account the influence of time, power, and scale in shaping decision-making processes. We conclude by highlighting a number of avenues of potential future research.
The humidification-dehumidification process (HDH) for desalination is a promising technology to address water scarcity issues in rural regions. However, a low humidifier efficiency is a weakness of the process. Bubble column humidifiers (BCH) are promising for HDH, as they provide enhanced heat and mass transfer and have low maintenance requirements. Previous studies of HDH-systems with BCHs draw different conclusions regarding the impact of superficial air velocity and liquid height on the humidification. Furthermore, the impact of flow characteristics has never been investigated systematically at all. In this study, an optimized BCH test setup that allows for optical analysis of the humidifier is used and evaluated. Our test setup is validated, since the influence of water temperature on the humidification, which is exponential, is reproduced. Measurements with seawater show that the normalised system productivity is increased by about 56 % with an increase in superficial air velocity from 0.5 to 5 cm/s. Furthermore, the system productivity is increased by around 29 % with an increase in liquid height from 60 to 378 mm. While the impact of superficial air velocity can be traced back to temperature changes at the humidifier and dehumidifier outlets, the impact of liquid height is shown to be caused by a smaller heat loss surface in the humidifier with an increase in liquid height. For the impact of sieve plate orifice diameter, a clear influence on the humidification is not apparent, this parameter needs to be investigated further. Finally, our new test setup allows for analysing the humidification of air (1) in a systematic way, (2) in relevant measurement ranges and (3) in comparison with optical analyses of the flow characteristics.
Bubble columns are recently used for the humidification of air in water treatment systems and fuel cells. They are well applicable due to their excellent heat and mass transfer and their low technical complexity. To design and operate such devices with high efficiency, the humidification process and the impact of the operating parameters need to be understood to a sufficient degree. To extend this knowledge, we use a refined and novel method to determine the volumetric air–liquid heat and mass transfer coefficients and the humidifier efficiency for various parametric settings. The volumetric transfer coefficients increase with both of the superficial air velocity and the liquid temperature. It is further shown that the decrease of vapor pressure with an increase of the salinity results in a corresponding decrease in the outlet humidity ratio. In contrast to previous studies, liquid heights smaller than 0.1 m are investigated and significant changes in the humidifier efficiency are seen in this range. We present the expected humidifier efficiency with respect to the superficial air velocity and the liquid height in an efficiency chart, such that optimal operating conditions can be determined. Based on this efficiency chart, recommendations for industrial applications as well as future scientific challenges are drawn.
Vast amounts of oily wastewater are byproducts of the petrochemical and the shipping industry and to this day frequently discharged into water bodies either without or after insufficient treatment. To alleviate the resulting pollution, water treatment processes are in great demand. Bubble column humidifiers (BCHs) as part of humidification–dehumidification systems are predestined for such a task, since they are insensitive to different feed liquids, simple in design and have low maintenance requirements. While humidification in a bubble column has been investigated plentiful for desalination, a systematic investigation of oily wastewater treatment is missing in literature. We filled this gap by analyzing the treatment of an oil–water emulsion experimentally to derive recommendations for future design and operation of BCHs. Our humidity measurements indicate that the air stream is always saturated after humidification for a liquid height of only 10 cm. A residual water mass fraction of 3.5 wt% is measured after a batch run of six hours. Furthermore, continuous measurements show that an increase in oil mass fraction leads to a decrease in system productivity especially for high oil mass fractions. This decrease is caused by the heterogeneity of the liquid temperature profile. A lower liquid height mitigates this heterogeneity, therefore decreasing the heat demand and improving the overall efficiency. The oil content of the produced condensate is below 15 ppm, allowing discharge into various water bodies. The results of our systematic investigation prove suitability and indicate a strong future potential for the use of BCHs in oily wastewater treatment.
Shaolin Wu Fo Dao – die Synthese japanischer und chinesischer Kampfkunst-Lehre auf der Grundlage zen-buddhistischer Theorie und Praxis ist eine neu geschaffene Disziplin. In dieser Fusion verbinden sich wesentliche Elemente aus Budo und Shaolin (-Wudao), deren traditionellen Körper-Geist-Übungen von Kampftechnik (Bu/Wu) und Meditation (Zen/Chan) als originäre Weg- und Heilslehre (Do/Dao) seit jeher der Persönlichkeits- und spirituellen Entwicklung des Ausübenden dienten.
Hier nun, in der Überwindung alter Grenzen zwischen japanischer und chinesischer Kampfkunstsysteme („hart“ und „weich“), wurde hier nun erstmaligen ein zusammengewachsenes Kampfkunstsystem kreiert, um die beidseits positiven Wirkungen auf den Menschen zu bündeln und – und das ist das Besondere – sie in den Dienst buddhistischer (Butsu, jap.: Buddha / chin. Fo) Psychologie und Psychotherapie zu stellen.
Die Verbindung von Budo mit Shaolin als eigenständiges praktisches Lehrkonzept, allein schon eine „revolutionäre Innovation“, erfährt durch ihren Einsatz als spezielle Methode einer Heilkunst zusätzlich Pioniercharakter in der Behandlung von Leid. Gemeint sind sowohl Ansätze aus der Budopädagogik und speziellen Budotherapie zur Heilung, Linderung oder Prävention von physischen, psychosomatischen und psychischen Krisen, Störungen, Krankheiten, Behinderungen und Leiden, als auch im Sinne des philosophischen und wissenschaftlichen Buddhismus, „das Leid in der Welt zu mindern“, zum Wohle des Menschen, für sein Glück und Frieden zu arbeiten.
Transparent laser-structured glasses with superhydrophilic properties for anti-fogging applications
(2019)
Ultrashort pulse laser structuring enables direct modification of glass surfaces to generate superhydrophilic properties for anti-fogging applications. This approach makes coatings dispensable and the generated surfaces remain thermally, mechanically, and chemically resistant. However, the laser-generated structures usually cause scattering, which decreases transmission and may disturb the vision through the modified glass in the dry state. The aim of this study was to find a laser-processing strategy to achieve superhydrophilic, anti-fogging properties on glass surfaces with maximum transmission and minimal visual perception of the generated structure. For this purpose, we used an ultrashort-pulsed laser to generate periodic patterns of rippled circles or rough holes with varying pitch. The water contact angle and transmission of the structured glasses were measured as a function of the structured area. It was found that a periodic pattern of holes, which covers less than 1% of the surface, is already sufficient to reach the superhydrophilic state (contact angle < 5°) and provides nearly the same transmission as pristine glass. Pictures of objects imaged through dry, structured glasses, which were placed close to the lens or object, showed in both cases only a minimal decrease of contrast. If this minor drawback can be accepted, this direct laser structuring approach could be an interesting alternative to coating-based techniques and leaves even room to apply additional coatings for the fabrication of multi-functional special glasses.
In this work, parametric excitation is introduced in a fully balanced flexible rotor mounted on two identical active gas foil bearings. The active gas foil bearings change the top foil shape harmonically with a specific amplitude and frequency. The deformable foil shape is approximated by an analytical function, while the gas pressure distribution is evaluated by the numerical solution of the Reynolds equation for compressible flow. The harmonic variation of the foil shape generates a respective variation in the bearings’ stiffness and damping properties and the system experiences parametric resonances and antiresonances in specific excitation frequencies. The nonlinear gas bearing forces generate bifurcations in the solutions of the system at certain rotating speeds and excitation frequencies; period doubling and Neimark-Sacker bifurcations are noticed in the examined system, and their progress is evaluated as the two bifurcation parameters (rotating speed and parametric excitation frequency) are changed, though a codimension-2 numerical continuation of limit cycles. It is found that at specific range of excitation frequency there are parametric anti-resonances and the bifurcations collide and vanish. Therefore, a bifurcation-free operating range is established and the system can operate stable at a wide speed range.
Compression of ultrashort laser pulses via gated multiphoton intrapulse interference phase scans
(2014)
The paper deals with designing and numerical modelling a 2 x 2 optical switch for photonic integrated circuits based on 2 x 2 MMI elements and phase modulators. The 2 x 2 optical switch was modelled in the RsoftCAD with the simulation tool BeamPROP. The 2 x 2 optical switch is a common element for creating more complex 1 x N or N x N optical switches in all-optical signal processing.
A Telecom optical fibers are still being the best transmission medium of digital data and analogue signals for long distance applications. Progress in integrated photonics enables development of photonic chips with new unique properties, circuits of the future, and overcomes current limits in information and communication technologies. The packaging of photonic chips is necessary for taking them out of research laboratories into real implementation in the information and communication technology applications. One important step of packaging is effective coupling of optical radiation between telecom optical fiber with ten microns core dimension and photonic chip optical waveguide with submicron dimensions. For complex photonic chips, it is necessary to couple not one optical fiber but several optical fibers, which are arranged in fiber arrays. In this case, it is necessary to use a 6D positioning system, which allows to optimally adjust the relative position of the photonic chip and the fiber arrays. After setting the optimal relative position of the photonic chip and the fiber array, the process of their fixation follows. One possibility of fixation is gluing with an adhesive in the optical path between the photonic chip and an array of optical fibers with a refractive index close to the refractive index of the optical fiber core. This paper is focused on the experimental test set-up for the temperature characterization of fiber array to photonics chip butt coupling at 1310 nm and 1550 nm wavelengths fixed themselves by UV adhesive in the optical path. The main aims of this works are selection of better adhesive from two types for gluing of photonic chip and fiber array in packaging process of photonics chips and validation of gluing process developing. The coupling and alignment of fiber arrays to photonics chip were done by automated active alignments system and they were fixed themselves by curable epoxy adhesive. Temperature changes of coupling insertion losses are measured and investigated for two different UV adhesives during three temperature cycles from -40 °C to 80 °C in climatic chamber according to Telcordia. Spectral dependence of insertion losses were measured and compared before and after three temperature cycles for 1530 nm to 1570 nm spectral range at room temperature.
This work was supported by the Slovak Research and Development Agency under the contracts APVV-17-0662 and SK-AT-20-0017 and by the COST Action “European Network for High Performance Integrated Microwave Photonics” (EUIMWP) CA16220.
Effects of hole-transport layer homogeneity in organic solar cells – A multi-length scale study
(2017)
The goal of this paper is to design a low-loss 1 x 32 Y-branch optical splitter for optical transmission systems, using two different design tools employing Beam Propagation Method. As a first step, a conventional 1 x 32 Y-branch splitter was designed and simulated in two-dimensional environment of OptiBPM photonic tool. The simulated optical properties feature high loss, high asymmetric splitting ratio and a large size of the designed structure, too. In the second step of this work we propose an optimization of the conventional splitter design leading to suppression of the asymmetric splitting ratio to one-third of its initial value and to the improvement of the losses by nearly 2 dB. In addition, 50% size reduction of the designed structure was also achieved. This length-optimized low-loss splitter was then modelled in a three-dimensional environment of RSoft photonic tool and the simulated results confirm the strong improvement of the optical properties.