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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 this paper, we consider the question of data aggregation using the practical example of emissions data for economic activities for the sustainability assessment of regional bank clients. Given the current scarcity of company-specific emission data, an approximation relies on using available public data. These data are reported in different standards in different sources. To determine a mapping between the different standards, an adaptation to the Covariance Matrix Self-Adaptation Evolution Strategy is proposed. The obtained results show that high-quality mappings are found. Nevertheless, our approach is transferable to other data compatibility problems. These can be found in the merging of emissions data for other countries, or in bridging the gap between completely different data sets.
Coupling is one of the most frequently mentioned metric in software systems. However, to measure logical coupling between microservices, runtime information is needed or the availability of service-log files to analyze the calls between services is required. This work presents our emerging results, in which we propose a metric to statically calculate logical coupling between microservices based on commits to versioning systems. We performed an initial validation of the proposed metric with a dataset containing 145 open-source microservices projects. The results illustrate how logical coupling affects every system and increases overtime. However, we did not find a correlation between the number of commits or the number of developers and the introduction of logical coupling. In future, we investigate why, how, and when logical coupling is introduced in a system.
This paper presents design, simulation, and optimization of the three-dimensional 1×4 optical multimode interference splitter using IP-Dip polymer as a core and polydimethylsiloxane (PDMS) Sylgard 184 as a cladding. The splitter was simulated by using beam propagation method in BeamPROP simulation engine of RSoft photonic tool and optimized for an operating wavelength of 1.55 µm. According to the minimum insertion loss, the dimensions of the MMI coupler and the length of the whole MMI splitter structure were optimized applying a waveguide with a core size of 4×4 µm2. The objective of the study is to create a design for fabrication by three-dimensional direct laser writing optical lithography.
Purpose – The purpose of this study is to explore the exogenous and endogenous drivers of the high-growth of Unicorn start-ups along their life cycle, with a particular focus on Unicorns in the fintech industry.
Design/methodology/approach – The study employs an explorative longitudinal analysis with a matched pair of two cases of Unicorns start-ups with similar antecedent features to understand holistically drivers over the longer term.
Findings – High-growth patterns over the longer term are the result of a combined industry- and company-life cycle perspective. Drivers and growth patterns vary significantly according to the time of entry in the industry and
its development status. The findings are systematised within a set of propositions to be tested in future research.
Research limitations/implications – The limitations lie in empirical evidence, as the analysis is limited to one matched-pair. The revealed Unicorns’ drivers for long-term growth might encourage future research to further investigate these drivers on a larger scale.
Practical implications – The study offers practical recommendations for start-ups with high-growth ambitions and advice to policy makers regarding the development of tailor-made support programs.
Originality/value – The study significantly extends extant work on growth and high-growth by examining endogenous and exogenous triggers over time and by linking the Unicorn-life cycle to the industry life cycle, an approach which has, to the best of the authors’ knowledge, not yet been applied.
We present design of planar 16-channel, 100-GHz multi-mode polymer-based AWG. This AWG was designed for central wavelength of 1550 nm applying AWG-Parameters tool. The AWG structure was created and simulated in the commercial photonic tool PHASAR from Optiwave. Achieved transmission characteristics were evaluated by AWG-Analyzer tool. For the design, multi-mode waveguides having a cross-section of (4x4) µm2 were used. The simulated results show strong worsening of the transmission characteristics in comparison when using single-mode waveguides. Nevertheless, the transmitting channels are clearly separated. The reason for using thicker multi-mode waveguides in the design is possibility to fabricate the AWG structure on polymer basis using direct laser writing lithography.
Statistische Methodenkompetenz wird im Controlling zunehmend bedeutender, so ist die evidenzbasierte Prognose durch algorithmische Analyse von Datenbeständen ein Schwerpunkt von Controlling Analytics. Die Fallstudie durchleuchtet daher anhand von Datenmaterial des Schweizer Bundesamts für Statistik das Vorgehen bei einer Datenanalyse, insbesondere beim Einsatz der Zeitregression für Prognosezwecke, und geht dabei auf methodische Besonderheiten, Caveats und Einsatzmöglichkeiten in Microsoft Excel ein. Die Fallbeschreibung und Aufgaben sind im WiSt-Heft Nr. 4/23 zu finden.
In Bewegung kommen
(2023)
Organic acidurias (OAs), urea-cycle disorders (UCDs), and maple syrup urine disease (MSUD) belong to the category of intoxication-type inborn errors of metabolism (IT-IEM). Liver transplantation (LTx) is increasingly utilized in IT-IEM. However, its impact has been mainly focused on clinical outcome measures and rarely on health-related quality of life (HRQoL). Aim of the study was to investigate the impact of LTx on HrQoL in IT-IEMs. This single center prospective study involved 32 patients (15 OA, 11 UCD, 6 MSUD; median age at LTx 3.0 years, range 0.8–26.0). HRQoL was assessed pre/post transplantation by PedsQL-General Module 4.0 and by MetabQoL 1.0, a specifically designed tool for IT-IEM. PedsQL highlighted significant post-LTx improvements in total and physical functioning in both patients' and parents' scores. According to age at transplantation (≤3 vs. >3 years), younger patients showed higher post-LTx scores on Physical (p = 0.03), Social (p < 0.001), and Total (p =0.007) functioning. MetabQoL confirmed significant post-LTx changes in Total and Physical functioning in both patients and parents scores (p ≤ 0.009). Differently from PedsQL, MetabQoL Mental (patients p = 0.013, parents p = 0.03) and Social scores (patients p = 0.02, parents p = 0.012) were significantly higher post-LTx. Significant improvements (p = 0.001–0.04) were also detected both in self- and proxy-reports for almost all MetabQoL subscales. This study shows the importance of assessing the impact of transplantation on HrQoL, a meaningful outcome reflecting patients' wellbeing. LTx is associated with significant improvements of HrQol in both self- and parentreports. The comparison between PedsQL-GM and MetabQoL highlighted that MetabQoL demonstrated higher sensitivity in the assessment of diseasespecific domains than the generic PedsQL tool.
Praxisanleiter:innen nehmen eine entscheidende Rolle in der Ausbildung von Pflegepersonen wahr. Die Tätigkeit fordert eine Vielzahl von Kompetenzen. Die Weiterbildung Praxisanleitung bietet eine wichtige Grundlage für die Entwicklung dieser Kompetenzen – deren Rahmenbedingungen, Methoden und Inhalte in Österreich werden in dieser Studie untersucht.
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.
Controlling Analytics: Einsatz für Prognosen im Controlling - Teil 1: Fallbeschreibung und Aufgaben
(2023)
Statistische Methodenkompetenz wird im Controlling zunehmend bedeutender, so ist die evidenzbasierte Prognose durch algorithmische Analyse von Datenbeständen ein Schwerpunkt von Controlling Analytics. Die Fallstudie durchleuchtet daher anhand von Datenmaterial des Schweizer Bundesamts für Statistik das Vorgehen bei einer Datenanalyse, insbesondere beim Einsatz der Zeitregression für Prognosezwecke, und geht dabei auf methodische Besonderheiten, Caveats und Einsatzmöglichkeiten in Microsoft Excel ein.
In the era of digital transformation an evolution takes place. Following this, new perspectives concerning leadership are required, especially in virtual teams. Shared Leadership is a promising leadership form to meet the challenges in a virtual team setting. Particularly, studies show that shared leadership increases performance, team creativity and innovative behavior. Moreover, the responsibility is distributed among several, not one individual. Nevertheless, it is unclear, which skills are needed in shared leadership teams and how they could be trained. Therefore, we develop a conceptual framework to pave the way for an empirical inquiry of the skills for and the role of shared leadership. Moreover, we encourage the discussion, whether the current leadership development is still viable and offer practical implications to develop shared leadership.
In this paper, the design of three-dimensional configuration of Y-branch splitter is compared with Multimode Interference splitter. Both splitters use the IP-Dip polymer as a standard material for 3D laser lithography. The optical properties of the splitters for both approaches are discussed and compared.
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.
In this work, we investigated the influence of different etch depths of the rib waveguides on the performance of SiN-based AWGs. For this purpose, an 8-channel 100 GHz AWG was designed for a center wavelength of 850 nm. The design parameters entered were calculated using the AWG-Parameters tool. The simulations were performed with a commercial photonic tool PHASAR from Optiwave. The simulated performance was evaluated using the AWG-Analyzer tool. For the AWG design, we used three identical rib waveguides with different etch depths to simulate possible etch imperfection. The simulations show the wavelength shift and degradation of the AWG performance.
Highly-sensitive single-step sensing of levodopa by swellable microneedle-mounted nanogap sensors
(2022)
Microneedle (MN) sensing of biomarkers in interstitial fluid (ISF) can overcome the challenges of self-diagnosis of diseases by a patient, such as blood sampling, handling, and measurement analysis. However, the MN sensing technologies still suffer from poor measurement accuracy due to the small amount of target molecules present in ISF, and require multiple steps of ISF extraction, ISF isolation from MN, and measurement with additional equipment. Here, we present a swellable MN-mounted nanogap sensor that can be inserted into the skin tissue, absorb ISF rapidly, and measure biomarkers in situ by amplifying the measurement signals by redox cycling in nanogap electrodes. We demonstrate that the MN-nanogap sensor measures levodopa (LDA), medication for Parkinson disease, down to 100 nM in an aqueous solution, and 1 μM in both the skin-mimicked gelatin phantom and porcine skin.