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By a simple femtosecond laser process, we fabricated metal-oxide/gold composite films for electrical and optical gas sensors. We designed a dripple wavelength AWG-spectrometer, matched to the plasma absorption wavelength region of the composite films. H2/CO absorptions fit well with the AWG design for multi gas detection sensor arrays
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.
Zu Beginn wurde im eigenen beruflichen Umfeld beobachtet, dass Informationen und Wissen, die heutzutage im Arbeitsalltag in Fülle ausgetauscht werden, häufig nicht die gewünschte Aufmerksamkeit und Offenheit erhalten. Diese Masterarbeit untersucht, wie bei dieser Art von Beobachtungen, deren komplexe Natur die Identifizierung von Lösungsansätzen erschwert, das eigentliche Problem erkannt werden kann. Frederic Vester zufolge ermöglicht ein vernetztes Denken dabei, Erkenntnisse vor allem aus den miteinander verknüpften Faktoren zu gewinnen. Diese Denkweise lässt sich in einer systemischen wie auch einer transdisziplinären Betrachtungsweise finden, wobei diese der Herausforderung gegenüberstehen vielfältige Perspektiven auf das Problem miteinander in Einklang zu bringen. Dies wirft die Frage auf, inwiefern eine gestalterische Zwischeninstanz dazu beitragen kann, einen gemeinsamen Denkraum zu eröffnen. Das Ziel dieser Arbeit ist, einen Entwurfsvorschlag für die Gestaltung eines Erkenntnisprozesses zu erarbeiten. Der Forschungsansatz dieser Arbeit – (FÜR+ÜBER)DURCH – leitet sich aus Christopher Fraylings Kategorien zur Designforschung ab. Die ‚Recherche FÜR‘ führt dazu, ein Problem als ein System zu betrachten, für das Wolfgang Jonas ein Problem-Design vorschlägt. In der ‚Recherche ÜBER‘ zeigt sich, dass es explizites wie auch implizites Wissen für eine umfassende Problembetrachtung braucht. Dafür wurde die Spuren-sicherung der Kunst auf das Design übertragen, um vorhandenes Wissen visuell zu dokumentieren. Mit Charles S. Peirce visuelles Entwurfs- und Erkenntnisverfahren lässt sich zudem ein anschauliches Denken durch diagrammatisches Schlussfolgern fördern. Die theoretischen Erkenntnisse münden in der ‚Transformation DURCH‘ in einen Vorschlag für ein diagrammatisches Problem-Design. Diesen gilt es in Bezug zu dem praxisbasierten Gestaltungsentwurf aus dem Masterprojekt zu reflektieren. In einer Werkshalle wurden Prototypische Beobachtungen zum ‚Zusammenspiel von Aufmerksamkeit und Offenheit‘ physisch in Beziehung gesetzt und anschließend mit zwei Testgruppen daraufhin untersucht, ob sich daraus neue Erkenntnisse ergeben. Es zeigte sich, das Design auf diese Weise einen gemeinsamen Austausch fördern kann. Gleichzeitig wurde deutlich, dass der Gestaltungsprozess die Vernetztheit eines Problems darstellen kann, jedoch ein vernetztes Denken aktiv initiiert werden muss, wofür im Entwurfsvorschlag dieser Arbeit ein visuelles Verfahren vorgestellt wird.
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.