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Long-Term outcome of infantile onset pompe disease patients treated with enzyme replacement therapy
(2024)
Background: Enzyme replacement therapy (ERT) with recombinant human alglucosidase alfa (rhGAA) was approved in Europe in 2006. Nevertheless, data on the long-term outcome of infantile onset Pompe disease (IOPD) patients at school age is still limited.
Objective: We analyzed in detail cardiac, respiratory, motor, and cognitive function of 15 German-speaking patients aged 7 and older who started ERT at a median age of 5 months.
Results: Starting dose was 20 mg/kg biweekly in 12 patients, 20 mg/kg weekly in 2, and 40 mg/kg weekly in one patient. CRIM-status was positive in 13 patients (86.7%) and negative or unknown in one patient each (6.7%). Three patients (20%) received immunomodulation. Median age at last assessment was 9.1 (7.0–19.5) years. At last follow-up 1 patient (6.7%) had mild cardiac hypertrophy, 6 (42.9%) had cardiac arrhythmias, and 7 (46.7%) required assisted ventilation. Seven patients (46.7%) achieved the ability to walk independently and 5 (33.3%) were still ambulatory at last follow-up. Six patients (40%) were able to sit without support, while the remaining 4 (26.7%) were tetraplegic. Eleven patients underwent cognitive testing (Culture Fair Intelligence Test), while 4 were unable to meet the requirements for cognitive testing. Intelligence quotients (IQs) ranged from normal (IQ 117, 102, 96, 94) in 4 patients (36.4%) to mild developmental delay (IQ 81) in one patient (9.1%) to intellectual disability (IQ 69, 63, 61, 3x < 55) in 6 patients (54.5%). White matter abnormalities were present in 10 out of 12 cerebral MRIs from 7 patients.
The utilization of lasers in dentistry expands greatly in recent years. For instance, fs-lasers are effective for both drilling and caries prevention, while cw-lasers are useful for adhesive hardening. A cutting-edge application of lasers in dentistry is the debonding of veneers. While there are pre-existing tools for this purpose, there is still potential for improvement. Initial efforts to investigate laser assisted debonding mechanisms with measurements of the optical and mechanical properties of teeth and prosthetic ceramics are presented. Preliminary tests conducted with a laser system used for debonding that is commercially available showed differences in the output power set at the systems console to that at specified distances from the handpiece. Furthermore, the optical properties of the samples (human teeth and ceramics) were characterised. The optical properties of the ceramics should closely resemble those of teeth in terms of look and feel, but they also influence the laser assisted debonding technique and thus must be taken into account. In addition first attempts were performed to investigate the mechanical properties of the samples by means of pump-probe-elastography under a microscope. By analyzing the sample surface up to 20 ns after a fs-laser pulse impact, pressure and shock waves could be detected, which can be utilized to determine the elastic constants of specific materials. Together such investigations are needed to shape the basis for a purely optical approach of debonding of veneers utilizing acoustic waves.
This thesis investigates the role of leadership behaviours of C-level executives in the context of post-M&A integration processes. The primary focus is on understanding the impact of specific leadership behaviours on inspiring desirable follower effects and facilitating emotional acceptance during organizational change. Drawing on the frameworks presented in “Six- Dimension Integrative Model of Leadership” and "The Six Domains of Leadership" developed by Sitkin et al., the study conducts expert interviews with managers from middle management who have recently experienced M&A integration. The answers are analysed in depth to identify the most effective leadership behaviours, highlighting those mentioned most frequently and those capable of triggering multiple follower effects simultaneously. The result is a list of behaviours that can serve as a guideline for C-level executives who want to foster desirable follower effects throughout the M&A integration journey.
This study aims to address the research gap surrounding the role of leadership in the formation of high-performance teams within startup companies. While there is existing research on high-performing teams, limited attention has been given to leadership in this environment. To bridge this gap, the study combines a literature review and qualitative analysis through semi-structured interviews with diverse stakeholders in startups, with the goal of providing practical guidance for startup executives based on the research findings. The study uncovers key aspects of leadership in high-performance teams, emphasizing the importance of skills such as motivation and support for team members, fostering psychological safety and trust, and effectively managing uncertainty. In addition to resource constraints and high expectations, the study sheds light on the challenges faced by leaders in startup and high-performance team environments, particularly the blurring of traditional leadership roles as team members seek autonomy and decision-making authority. These findings present opportunities for future research to explore this progressive leadership style. Overall, this study contributes to our understanding of leadership dynamics within high-performance teams operating in the context of startups. It offers valuable insights that can help startup executives navigate the complexities of leadership and foster the development of successful and high-performing teams.
Effective lead management
(2023)
In the last few years the global interest on lead management has increased. This classic topic for marketing and sales departments is aimed at converting potential customers into sales. The following thesis identifies the challenges and solutions for marketing and sales departments in order to process effective lead management. Using data from a literature review and qualitative empirical research, conducted with representatives of marketing and sales departments, the results showed overall and task specific challenges and solutions. The research indicates that overall challenges and solutions regarding the gap between marketing and sales, new processes and data management including data quality, software and silos emerge. In addition task specific challenges and solutions concerning lead generation including purchased leads, lead qualification, lead nurturing and sales specific challenges and solutions conclusively the focus on existing customers, time famine and lead routing were identified. This thesis provides a framework for further studies regarding the challenges and solutions for marketing and sales departments processing lead management.
This thesis focuses on implementing and testing communication over a private 5G standalone network in an industrial environment, with a specific emphasis on communication between two articulated robots. The main objective is to examine machine-to-machine communication behavior in various test scenarios. Initially, the 5G core and radio access network components are described, along with their associated interfaces, to establish foundational knowledge. Subsequently, a use case involving two articulated robots is implemented, and essential metrics are defined for testing, including round-trip time, packet and inter-packet delay, and packet error rate. The tests investigate the impact of 5G quality of service, packet size, and transmission interval on communication between the robots, focusing on the effects of network traffic. The results highlight the significance of prioritizing network resources based on the assigned quality of service identifier (5QI), demonstrate the influence of packet sizes on communication performance, and underscore the importance of transmission intervals for automation purposes. Additionally, the study examines how network disturbances influence the movements of a robot controlled via 5G, establishing a direct relationship between network metrics and the resulting deviations in the robot’s trajectory. The work concludes that while machine-to-machine communication can be successfully implemented with 5G SA, tradeoffs must be carefully considered, especially concerning packet error rate, and emphasizes the importance of understanding the required resources before implementation to ensure feasibility. Future research directions include investigating network slicing, secure remote control of robots, and exploring the use of higher frequency bands. The study highlights the significance of aligning theoretical standards with practical implementation options in the evolving landscape of 5G Networks.
In an oversaturated market, companies are required to use innovative and, above all, creative advertising methods to capture their customers’ attention, and thus differentiate themselves from rival businesses. To this end, companies have been increasingly relying on the use of humor, a phenomenon that remains highly subjective and is perceived differently by each individual. This master’s thesis, which was completed as part of the International Marketing and Sales program at the FH Vorarlberg, focuses on this phenomenon of humor as well as its impact on advertising perception. With the aid of three different theories, the term “humor” is defined. Furthermore, this study explains and researches the so-called vampire effect, wherein various factors (in this case humor) draw attention away from the actual advertising message. In addition, this thesis takes a closer look at involvement, as a person’s involvement or interest in a brand or product can influence brand and product recall and recognition. An online survey was conducted to determine whether the vampire effect caused by humor is able to influence brand and product recall. In other words, this concerns whether the viewer can still remember the brand and product afterward or whether the humor employed triggers the vampire effect. Furthermore, this thesis explored whether the vampire effect caused by humor is able to influence brand and product recognition. Recall is the retrieval of information from memory without direct cues, whereas recognition refers to the recognition of information when it is presented again. Furthermore, within this context, it was discovered that brand and product recall varies with low and high involvement viewers of the advertisement. In other words, this means that the strength of the vampire effect caused by humor changes depending on the strength of the viewer’s involvement. During the course of this research, it was further observed that the humor employed significantly affects the perception of the advertising message, thus confirming the existence of the vampire effect. This effect also influences both brand as well as product recall and recognition. In both cases, participants in the survey were less able to remember the product and brand in the humorous advertising. Furthermore, it was proven that people with low involvement in the advertised product group are more heavily affected by the vampire effect. As such, they are more likely to not remember the product or brand after seeing the advertisement.
Alleviating the curse of dimensionality in minkowski sum approximations of storage flexibility
(2023)
Many real-world applications require the joint optimization of a large number of flexible devices over some time horizon. The flexibility of multiple batteries, thermostatically controlled loads, or electric vehicles, e.g., can be used to support grid operations and to reduce operation costs. Using piecewise constant power values, the flexibility of each device over d time periods can be described as a polytopic subset in power space. The aggregated flexibility is given by the Minkowski sum of these polytopes. As the computation of Minkowski sums is in general demanding, several approximations have been proposed in the literature. Yet, their application potential is often objective-dependent and limited by the curse of dimensionality. In this paper, we show that up to 2d vertices of each polytope can be computed efficiently and that the convex hull of their sums provides a computationally efficient inner approximation of the Minkowski sum. Via an extensive simulation study, we illustrate that our approach outperforms ten state-of-the-art inner approximations in terms of computational complexity and accuracy for different objectives. Moreover, we propose an efficient disaggregation method applicable to any vertex-based approximation. The proposed methods provide an efficient means to aggregate and to disaggregate typical battery storages in quarter-hourly periods over an entire day with reasonable accuracy for aggregated cost and for peak power optimization.
Open tracing tools
(2023)
Background: Coping with the rapid growing complexity in contemporary software architecture, tracing has become an increasingly critical practice and been adopted widely by software engineers. By adopting tracing tools, practitioners are able to monitor, debug, and optimize distributed software architectures easily. However, with excessive number of valid candidates, researchers and practitioners have a hard time finding and selecting the suitable tracing tools by systematically considering their features and advantages. Objective: To such a purpose, this paper aims to provide an overview of popular Open tracing tools via comparison. Methods: Herein, we first identified 30 tools in an objective, systematic, and reproducible manner adopting the Systematic Multivocal Literature Review protocol. Then, we characterized each tool looking at the 1) measured features, 2) popularity both in peer-reviewed literature and online media, and 3) benefits and issues. We used topic modeling and sentiment analysis to extract and summarize the benefits and issues. Specially, we adopted ChatGPT to support the topic interpretation. Results: As a result, this paper presents a systematic comparison amongst the selected tracing tools in terms of their features, popularity, benefits and issues. Conclusion: The result mainly shows that each tracing tool provides a unique combination of features with also different pros and cons. The contribution of this paper is to provide the practitioners better understanding of the tracing tools facilitating their adoption.
Creating a schedule to perform certain actions in a realworld environment typically involves multiple types of uncertainties. To create a plan which is robust towards uncertainties, it must stay flexible while attempting to be reliable and as close to optimal as possible. A plan is reliable if an adjustment to accommodate for a new requirement causes only a few disruptions. The system needs to be able to adapt to the schedule if unforeseen circumstances make planned actions impossible, or if an unlikely event would enable the system to follow a better path. To handle uncertainties, the used methods need to be dynamic and adaptive. The planning algorithms must be able to re-schedule planned actions and need to adapt the previously created plan to accommodate new requirements without causing critical disruptions to other required actions.
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.
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.
Highly-sensitive single-step sensing of levodopa by swellable microneedle-mounted nanogap sensors
(2023)
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.
The production of liquid-gas mixtures with desired properties still places high demands on process technology and is usually realized in bubble columns. The physical calculation models used have individual dimensionless factors which, depending on the application, are only valid for small ranges consisting of flow velocity, nozzle geometry and test setup. An iterative but time-consuming design of such dispersion processes is used in industry for producing a liquid-gas mixture according to desired requirements. In the present investigation, we accelerate the necessary design loops by setting up a physical model, which consists of several subsystems that are enriched by dedicated experiments to realize liquid-gas dispersions with low volume fraction and small air bubble diameters in oil. Our approach allows the extraction of individual dimensionless factors from maps of the introduced subsystems. These maps allow for targeted corrective measures of a production process for keeping the quality. The calculation-based approach avoids the need for performing iterative design loops. Overall, this approach supports the controlled generation of liquid-gas mixtures.
X-ray microtomography is a nondestructive, three-dimensional inspection technique applied across a vast range of fields and disciplines, ranging from research to industrial, encompassing engineering, biology, and medical research. Phasecontrast imaging extends the domain of application of x-ray microtomography to classes of samples that exhibit weak attenuation, thus appearing with poor contrast in standard x-ray imaging. Notable examples are low-atomic-number materials, like carbon-fiber composites, soft matter, and biological soft tissues.We report on a compact and cost-effective system for x-ray phase-contrast microtomography. The system features high sensitivity to phase gradients and high resolution, requires a low-power sealed x-ray tube, a single optical element, and fits in a small footprint. It is compatible with standard x-ray detector technologies: in our experiments, we have observed that single-photon counting offered higher angular sensitivity, whereas flat panels provided a larger field of view. The system is benchmarked against knownmaterial phantoms, and its potential for soft-tissue three-dimensional imaging is demonstrated on small-animal organs: a piglet esophagus and a rat heart.We believe that the simplicity of the setupwe are proposing, combined with its robustness and sensitivity, will facilitate accessing quantitative x-ray phase-contrast microtomography as a research tool across disciplines, including tissue engineering, materials science, and nondestructive testing in general.
Activation of heat pump flexibilities is a viable solution to support balancing the grid via Demand Side Management measures and fulfill the need for flexibility options. Aggregators as interface between prosumers, distribution system operators and balance responsible parties face the challenge due to data privacy and technical restrictions to transform prosumer information into aggregated available flexibility to enable trading thereof. Thereby, literature lacks a generic, applicable and widely accepted flexibility estimation method for heat pumps,which incorporates reduced sensor and system information, system- and demand-dependent behaviour. In this paper, we adapt and extend a method from literature, by incorporating domain knowledge to overcome reduced sensor and system information. We apply data of five real-world heat pump systems, distinguish operation modes, estimate power and energy flexibility of each single heat pump system, proof transferability of the method, and aggregate the flexibilities available to showcase a small HP pool as a proof of concept.