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Why do some countries assign a major role to wind energy in decarbonizing their electricity systems, while others are much less committed to this technology? We argue that processes of (de-)legitimation, driven by discourse coalitions who strategically employ certain storylines in public debates, provide part of the answer. To illustrate our approach, we comparatively investigate public discourses surrounding wind energy in Austria and Switzerland, two countries that differ strongly in wind energy deployment. By combining a qualitative content analysis and a discourse network analysis of 808 newspaper articles published 2010–2020, we identify four distinct sets of storylines used to either delegitimize or legitimize the technology. Our study indicates that low deployment rates in Switzerland can be related to the prominence of delegitimizing storylines in the public discourse, which result in a rather low socio-political acceptance of wind energy. In Austria, by contrast, there is more consistent support for wind energy by discourse coalitions using a broad set of legitimizing storylines. By bridging the related but separate literatures of technology legitimacy and social acceptance, our study contributes to a better understanding of socio-political conflict and divergence in low-carbon technological pathways.
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.
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.
This study presents different approaches to increase the sensing area of NiO based semiconducting metal oxide gas sensors. Micro- and nanopatterned laser induced periodic surface structures (LIPSS) are generated on silicon and Si/SiO2 substrates. The surface morphologies of the fabricated samples are examined by FE SEM. We select the silicon samples with an intermediate Si3N4 layer due to its superior isolation quality over the thermal oxide for evaluating the hydrogen and acetone sensitivity of a NiO based test sensor.
Das Konzept der entscheidungsgetriebenen Organisation basiert auf der Tatsache, dass jede Aktivität entlang der arbeitsteiligen Wertschöpfung in einer Organisation1 im Kern auf Entscheidungen beruht, die aneinander anknüpfen.2 Jede Aktivität kann man vollziehen oder unterlassen, beide Möglichkeiten sind zunächst gleichgewichtig. Jede argumentative Begründung für eine Aktivität ist dabei zugleich eine Begründung gegen alle anderen, ebenso möglichen Aktivitäten. Da dies für alle wertschöpfenden Aktivitäten in einer Organisation gilt, werden Entscheidungen von jeder an der arbeitsteiligen Leistungserstellung beteiligten Person getroffen.
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.