69 research outputs found
4D Continuous Descent Operations Supported by an Electronic Flight Bag
This paper describes a set of flight simulation
experiments carried out with the DLR’s Generic Cockpit
Simulator (GECO). A new concept named time and energy
managed operations (TEMO), which aims to enable advanced
four dimensional (4D) continuous descent operations (CDO), was
evaluated after three full days of experiments with qualified
pilots. The experiment focused to investigate the possibility of
using a 4D-controller on a modern aircraft with unmodified or
only slightly modified avionic systems. This was achieved by
executing the controller in an Electronic Flight Bag (EFB) and
using the pilot to “close the loop” by entering speed and other
advisories into the autopilot Flight Control Unit (FCU). The
outcome of the experiments include subjective (questionnaires
answered by pilots) and objective (trajectory logs) data. Data
analysis showed a very good acceptance (both in terms of safety
and operability of the procedure) from the participating crews,
only with minor suggestions to be improved in future versions of
the controller and the speed advisories update rates. Good time
accuracy all along the descent trajectory was also observed.Peer ReviewedPostprint (published version
Pair interactions between complex mesoscopic particles from Widom's particle-insertion method
We demonstrate that Widom's particle insertion technique provides a
convenient and efficient method to determine the effective pair interaction
between complex, composite soft-matter particles in the zero-density limit. By
means of three different test systems, i.e. amphiphilic dendrimers,
electrostatic polymers and colloids coated with electrostatic polymers, we
demonstrate the validity and the power of the presented method.Comment: 7 pages, 4 figures, to be published in Soft Matte
Entwicklung eines konfigurierbaren Flugtaxi-Simulators mit Hilfe einer kopfgetragenen Anzeige mit Video-Durchsicht - Ein Vergleich verschiedener Mixed-Reality-Ansätze
Um zukünftige Flugtaxi-Konzepte frühzeitig aus Passagiersicht bewerten zu können, wird ein immersiver und gleichzeitig konfigurierbarer Kabinensimulator benötigt. Dieser Vortrag zeigt wie moderne Mixed-Reality-Technologien bei der Umsetzung eines solchen Simulators helfen können. So kann zum Beispiel mit Hilfe einer Video-Durchsicht-Brille das reale Kabinenmockup flexibel um virtuelle Objekte erweitert werden. Die durchgeführte Probandenstudie zeigt die Vorteile und Grenzen solcher Ansätze
Experiencing Urban Air Mobility: How Passengers evaluate a simulated flight with an Air Taxi
For the successful development and implementation of novel concepts and
technology, the acceptance of potential users is crucial. Therefore, within the
project HorizonUAM, we investigated passengers' acceptance of air taxis. One
challenge is that not many people have real experiences with urban air mobility
(UAM) at the moment and thus requirements formulated by potential users refer
to rather abstract concepts. To allow participants to gain realistic
impressions of UAM concepts, a Mixed Reality Air Taxi Simulator was set up. It
allows participants to experience an inner-city business shuttle flight. A
study with 30 participants assessed the information needs and the influence of
another person on board on wellbeing in nominal situations (experiment 1) as
well as one non-nominal situation (experiment 2). For the latter, participants
experienced a re-routing of the flight due to an unavailability of landing
sites at the vertidrome. During and after the flights, participants answered
questionnaires and extensive interviews were conducted. The study produced
first empirical data on relevant factors regarding interaction, information
needs and comfort within an air taxi. The findings show that passengers want to
be informed about intentions of the vehicle. The presence of a steward on board
is not necessary but can increase wellbeing especially during non-nominal
situations.Comment: 16 pages, 12 figures, 8 table
Etiology and Outcome of Adult and Pediatric Acute Liver Failure in Europe
Acute liver failure (ALF) is rare but life-threatening. Common causes include intoxications, infections, and metabolic disorders. Indeterminate etiology is still frequent. No systematic data on incidence, causes, and outcome of ALF across Europe are available. Via an online survey we reached out to European Reference Network Centers on rare liver diseases. Numbers and etiology of ALF cases during 2020 were retrieved and diagnostic and treatment availabilities assessed. In total, 455 cases (306 adult, 149 pediatric) were reported from 36 centers from 20 countries. Intoxication was the most common cause in adult and pediatric care. The number of cases with indeterminate etiology is low. Diagnostic tools and specific treatment options are broadly available within this network. This is the first approach to report on etiology and outcome of ALF in the pediatric and adult population in Europe. High diagnostic yield and standard of care reflects the expert status of involved centers.</p
Genetic landscape of pediatric acute liver failure of indeterminate origin.
BACKGROUND AIMS
Pediatric acute liver failure (PALF) is a life-threatening condition. In Europe, main causes are viral infections (12-16%) and inherited metabolic diseases (14-28%). Yet, in up to 50% of cases the underlying etiology remains elusive, challenging clinical management, including liver transplantation. We systematically studied indeterminate PALF cases referred for genetic evaluation by whole-exome sequencing (WES), and analyzed phenotypic and biochemical markers, and the diagnostic yield of WES in this condition.
METHODS
With this international, multicenter observational study, patients (0-18 y) with indeterminate PALF were analyzed by WES. Data on the clinical and biochemical phenotype were retrieved and systematically analyzed.
RESULTS
In total, 260 indeterminate PALF patients from 19 countries were recruited between 2011 and 2022, of whom 59 had recurrent PALF (RALF). WES established a genetic diagnosis in 37% of cases (97/260). Diagnostic yield was highest in children with PALF in the first year of life (46%), and in children with RALF (64%). Thirty-six distinct disease genes were identified. Defects in NBAS (n=20), MPV17 (n=8) and DGUOK (n=7) were the most frequent findings. When categorizing, most frequent were mitochondrial diseases (45%), disorders of vesicular trafficking (28%) and cytosolic aminoacyl-tRNA synthetase deficiencies (10%). One-third of patients had a fatal outcome. Fifty-six patients received liver transplants.
CONCLUSION
This study elucidates a large contribution of genetic causes in PALF of indeterminate origin with an increasing spectrum of disease entities. The high proportion of diagnosed cases and potential treatment implications argue for exome or in future rapid genome sequencing in PALF diagnostics
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