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9. An essential role for the Zn2+ transporter ZIP7 in B cell development

Author

Anzilotti, Consuelo, Swan, David J, Boisson, Bertrand, Deobagkar-Lele, Mukta, Oliveira, Catarina, Chabosseau, Pauline, Engelhardt, Karin R, Xu, Xijin, Chen, Rui, Alvarez, Luis, Berlinguer-Palmini, Rolando, Bull, Katherine R, Cawthorne, Eleanor, Cribbs, Adam P, Crockford, Tanya L, Dang, Tarana Singh, Fearn, Amy, Fenech, Emma J, de Jong, Sarah J, Lagerholm, B Christoffer, Ma, Cindy S, Sims, David, van den Berg, Bert, Xu, Yaobo, Cant, Andrew J, Kleiner, Gary, Leahy, T Ronan, de la Morena, M Teresa, Puck, Jennifer M, Shapiro, Ralph S, van der Burg, Mirjam, Chapman, J Ross, Christianson, John C, Davies, Benjamin, McGrath, John A, Przyborski, Stefan, Santibanez Koref, Mauro, Tangye, Stuart G, Werner, Andreas, Rutter, Guy A, Padilla-Parra, Sergi, Casanova, Jean-Laurent, Cornall, Richard J, Conley, Mary Ellen, and Hambleton, Sophie

Subjects
Biochemistry and Cell Biology, Biomedical and Clinical Sciences, Biological Sciences, Pediatric, Genetics, 2.1 Biological and endogenous factors, 1.1 Normal biological development and functioning, Agammaglobulinemia, Animals, B-Lymphocytes, Cation Transport Proteins, Child, Preschool, Cytosol, Disease Models, Animal, Endoplasmic Reticulum, Female, Gene Expression Profiling, Humans, Infant, Male, Mice, Inbred C57BL, Mice, Transgenic, Mutation, Pedigree, Zinc, Immunology, Biochemistry and cell biology
Abstract

Despite the known importance of zinc for human immunity, molecular insights into its roles have remained limited. Here we report a novel autosomal recessive disease characterized by absent B cells, agammaglobulinemia and early onset infections in five unrelated families. The immunodeficiency results from hypomorphic mutations of SLC39A7, which encodes the endoplasmic reticulum-to-cytoplasm zinc transporter ZIP7. Using CRISPR-Cas9 mutagenesis we have precisely modeled ZIP7 deficiency in mice. Homozygosity for a null allele caused embryonic death, but hypomorphic alleles reproduced the block in B cell development seen in patients. B cells from mutant mice exhibited a diminished concentration of cytoplasmic free zinc, increased phosphatase activity and decreased phosphorylation of signaling molecules downstream of the pre-B cell and B cell receptors. Our findings highlight a specific role for cytosolic Zn2+ in modulating B cell receptor signal strength and positive selection.

Published
2019

10. Trends in pediatric ambulatory community acquired infections before and during COVID-19 pandemic: A prospective multicentric surveillance study in France

Author

Cohen, Pr Robert, Rybak, Alexis, Werner, Andreas, Béchet, Stéphane, Desandes, Roxane, Hassid, Fréderic, André, Jean-Marie, Gelbert, Nathalie, Thiebault, Georges, Kochert, Fabienne, Cahn-Sellem, Fabienne, Vié Le Sage, François, Angoulvant, Pr François, Ouldali, Naïm, Frandji, Bruno, and Levy, Corinne

Published
2022
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11. Did the resurgence of childhood lower respiratory infections offset the initial benefit of COVID-19-related non-pharmaceutical interventions in children? A time-series analysis.

Author

Fafi, Inès, Assad, Zein, Lenglart, Léa, Valtuille, Zaba, Kaguelidou, Florentia, Aupiais, Camille, Bourmaud, Aurélie, Rybak, Alexis, Bechet, Stéphane, Levy, Corinne, Cohen, Robert, Frandji, Bruno, Werner, Andreas, Angoulvant, François, Basmaci, Romain, and Ouldali, Naïm

Subjects
RESPIRATORY infections, TIME series analysis, MEDICAL sciences, PLEURAL effusions, COVID-19 pandemic
Abstract

Background: Following non-pharmaceutical interventions (NPI) lifting in 2021, an important surge in childhood lower respiratory tract infections (LRTI) was reported in several countries, raising major concerns about the middle-term consequences of such interventions. Whether this recent upsurge overwhelms the initial benefit of NPI remains unknown. Methods: We conducted an interrupted time-series analysis based on exhaustive national surveillance systems. All hospitalisations from January 2015 to March 2023 and all ambulatory visits for LRTI from a network of 110 paediatricians from June 2017 to March 2023 were included. The main outcome was the monthly incidence of children hospitalised for LRTI per 100,000 over time, assessed by a seasonally adjusted quasi-Poisson regression model. Results: We included 845,047 hospitalisations. The incidence of hospitalisation for LRTI significantly decreased during the NPI period (− 61.7%, 95% CI − 98.4 to − 24.9) and rebounded following NPI lifting, exceeding the pre-NPI baseline trend (+ 12.8%, 95% CI 6.7 to 19.0). We observed similar trends for hospitalisation due to bronchiolitis, pneumonia and pneumonia with pleural effusion, along with ambulatory LRTI. Overall, despite the recent rebound, 31,777 (95% CI, 25,375 to 38,179) hospitalisations for paediatric LRTI were averted since NPI implementation up to 2023. Conclusions: Three years after their implementation, despite an increase in LRTI incidence, the middle-term impact of NPI remains highly beneficial in preventing overall paediatric LRTI. The implementation of some societally acceptable NPI, particularly during epidemics, may be considered in the future to further reduce the burden of paediatric LRTI. [ABSTRACT FROM AUTHOR]

Published
2025
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12. Comparing NIRS and Pulse Oximetry for Cerebral Oxygen Saturation During Hypoxia Testing.

Author

Alevizakos, Vasilios, Werner, Andreas, Schiller, Lisa-Marie, von See, Constantin, and Schiller, Marcus

Subjects
OXYGEN saturation, PULSE oximetry, NEAR infrared spectroscopy, REGRESSION analysis, AERONAUTICAL safety measures, OXIMETRY
Abstract

Objective: This study evaluates the suitability of cerebral oximetry using near-infrared spectroscopy (NIRS) compared to traditional pulse oximetry (SpO2) for measuring cerebral oxygen saturation during hypoxia testing, aiming to enhance safety during flight operations and training. Material and Methods: The study included 106 participants aged 18–60 years at the Aerospace Medicine Training Center in Königsbrück. Cerebral oxygen saturation (rSO2) and peripheral oxygen saturation (SpO2) were measured using the INVOS™ 5100C cerebral oximeter and Masimo™ MS5 pulse oximeter, respectively. Measurements were taken at baseline, during hypoxia at 25,000 feet, and post recovery. Data analysis included regression analysis, Bland–Altman plots, and concordance correlation coefficients (CCC). Ethical approval was obtained from the Hannover Medical School. Data from 100 participants were analyzed. Results: Baseline SpO2 was 97.5 ± 1.5%, and baseline rSO2 was 77.25 ± 6.4%. During hypoxia, SpO2 dropped significantly, while rSO2 showed higher values. SpO2 recovered faster than rSO2. Deviations in rSO2 between the right and left sides during hypoxia were minimal. Lin's CCC indicated moderate to substantial concordance. NIRS measurements were more stable and less prone to disturbances, with 95 disruptions in pulse oximetry, 25 of which were potentially critical. Conclusions: NIRS is a reliable method for detecting cerebral oxygen saturation, offering significant advantages over traditional pulse oximetry in stability and reliability during hypoxia testing. NIRS is less error-prone, supporting its use for continuous monitoring in aviation settings and enhancing flight safety by providing more accurate hypoxia detection. [ABSTRACT FROM AUTHOR]

Published
2024
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16. A big-data approach to producing descriptive anthropometric references: a feasibility and validation study of paediatric growth charts

Author

Heude, Barbara, Scherdel, Pauline, Werner, Andreas, Le Guern, Morgane, Gelbert, Nathalie, Walther, Déborah, Arnould, Michel, Bellaïche, Marc, Chevallier, Bertrand, Cheymol, Jacques, Jobez, Emmanuel, N'Guyen, Sylvie, Pietrement, Christine, Reynaud, Rachel, Salaün, Jean-François, Khoshnood, Babak, Zeitlin, Jennifer, Maccario, Jean, Breart, Gérard, Thalabard, Jean-Christophe, Charles, Marie-Aline, Botton, Jérémie, Frandji, Bruno, and Chalumeau, Martin

Published
2019
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20. The OECD/NEA FIRE Database: an update: The Fire Incidents Records Exchange (FIRE) Database is one of the nuclear power plant (NPP) operational events database projects operated under the auspices of the OECD/NEA covering detailed information on fire events at NPP sites from fourteen NEA member countries-Belgium, Canada, Czech Republic, Finland, France, Germany, Japan, Korea, the Netherlands, Spain, Sweden, Switzerland, the United Kingdom, and the USA. The project was officially launched in 2001 and is currently in its sixth phase (2020-2022)

Author

Rowekamp, Marina, Thompson, Simon, Kuriene, Laimute, Beilmann, Markus, and Werner, Andreas

Subjects
Nuclear energy -- Forecasts and trends, Nuclear power plants -- Forecasts and trends, Risk assessment -- Forecasts and trends, Market trend/market analysis, Business, Business, international, Petroleum, energy and mining industries, Organisation for Economic Co-operation and Development. Nuclear Energy Agency
Abstract

THE DATABASE IS A USEFUL source of operational experience (OPEX) and it is considered mature enough for applications, not only with respect to deterministic safety assessment (DSA) but also in [...]

Published
2021

28. Effect of long range forces on the interfacial profiles in thin binary polymer films

Author

Werner, Andreas, Mueller, Marcus, Schmid, Friederike, and Binder, Kurt

Subjects
Condensed Matter - Statistical Mechanics, Condensed Matter - Soft Condensed Matter
Abstract

We study the effect of surface fields on the interfacial properties of a binary polymer melt confined between two parallel walls. Each wall attracts a different component of the blend by a non-retarded van der Waals potential. An interface which runs parallel to the surfaces is stabilized in the center of the film. Using extensive Monte Carlo simulations we study the interfacial properties as a function of the film thickness, the strength of the surface forces and the lateral size over which the profiles across the film are averaged. We find evidence for capillary wave broadening of the apparent interfacial profiles. However, the apparent interfacial width cannot be described quantitatively by a simple logarithmic dependence on the film thickness. The Monte Carlo simulations reveal that the surface fields give rise to an additional reduction of the intrinsic interfacial width and an increase of the effective interfacial tension upon decreasing the film thickness. These modifications of the intrinsic interfacial properties are confirmed by self-consistent field calculations. Taking account of the thickness dependence of the intrinsic interfacial properties and the capillary wave broadening, we can describe our simulation results quantitatively., Comment: to appear in J.Chem.Phys

Published
1998
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32. Interfaces between highly incompatible polymers of different stiffness: Monte Carlo simulations and self-consistent field calculations

Author

Mueller, Marcus and Werner, Andreas

Subjects
Condensed Matter - Statistical Mechanics, Condensed Matter - Soft Condensed Matter
Abstract

We investigate interfacial properties between two highly incompatible polymers of different stiffness. The extensive Monte Carlo simulations of the binary polymer melt yield detailed interfacial profiles and the interfacial tension via an analysis of capillary fluctuations. We extract an effective Flory-Huggins parameter from the simulations, which is used in self-consistent field calculations. These take due account of the chain architecture via a partial enumeration of the single chain partition function, using chain conformations obtained by Monte Carlo simulations of the pure phases. The agreement between the simulations and self-consistent field calculations is almost quantitative, however we find deviations from the predictions of the Gaussian chain model for high incompatibilities or large stiffness. The interfacial width at very high incompatibilities is smaller than the prediction of the Gaussian chain model, and decreases upon increasing the statistical segment length of the semi-flexible component., Comment: to appear in J.Chem.Phys

Published
1997
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33. Anomalous size-dependence of interfacial profiles between coexisting phases of polymer mixtures in thin film geometry: A Monte-Carlo simulation

Author

Werner, Andreas, Schmid, Friederike, Mueller, Marcus, and Binder, Kurt

Subjects
Condensed Matter - Statistical Mechanics, Condensed Matter - Soft Condensed Matter
Abstract

The interfacial profile between coexisting phases of a binary mixture (A,B) in a thin film of thickness D and lateral linear dimensions L depends sensitively on both linear dimensions and on the nature of boundary conditions and statistical ensembles applied. These phenomena generic for systems in confined geometry are demonstrated by Monte-Carlo simulations of the bond fluctuation model of symmetric polymer mixtures. Both the canonical and semi-grand-canonical ensemble are studied. In the canonical ensemble, the interfacial width w increases (from small values which are of the same order as the intrinsic profile) like sqrt{D}, before a crossover to a saturation value w_max (w_max^2 proportional to ln L) sets in. In the semi-grand-canonical ensemble, however, one finds the same widths (w proportional to sqrt{D}) as in the canonical ensemble for not too large L, while for large L the interfacial profile is smeared out over a finite fraction of the film thickness (w proportional to D for D -> infinity). We discuss the implications of these findings for the interpretation of both simulations and experiments., Comment: 42 pages, including 15 PS figures, to appear in JCP

Published
1997
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37. Status of the new WEST plasma control system

Author

Ravene, Nathalie, Nouailletas, Rémy, Signoret, Jacqueline, Guillerminet, Bernard, Treutterrer, Wolfgang, Spring, Anett, Masand, Harish, Dhongde, Jasraj, Bhandarkar, Manisha, Rapson, Chris, Laqua, Heike, Lewerentz, Marc, Moreau, Philippe, Brémond, Sylvain, Allegretti, Ludovic, Raupp, Gerhard, Werner, Andreas, Laurent, François Saint, and Nardon, Eric

Published
2016
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43. sCO2 test facility at TU Wien: design, operation and results

Author

Illyés, Viktoria, Thanheiser, Stefan, Schwarzmayr, Paul, David, Pierre-Luc, Guerif, Xavier, Werner, Andreas, Haider, Markus, and 5th European sCO2 Conference

Subjects
ddc:620, Externe
Abstract

At TU Wien, a test facility working with supercritical carbon dioxide (sCO2) was commissioned in 2018. Since then, it has been used for various research tasks. This paper gives an overview about the three configurations of the facility with a focus on design, operation, and results. The authors present the design of components in the three configurations of the test facility: proof of concept of the simple cycle in supercritical and transcritical operation mode, heat transfer measurements, and future work. Special emphasis is given to challenges during engineering and operation. Our most relevant lessons learned are: that a commercial CO2 pump is not sufficient for cycle experiments, how to design a measurement section for heat transfer measurements, and that during experimental research, measurement-concepts and data reduction must be prioritized at all times., Conference Proceedings of the European sCO2 Conference5th European sCO2 Conference for Energy Systems: March 14-16, 2023, Prague, Czech Republic, p. 22

Published
2023

45. Der 'Professional Ear User' – Implikationen für die Prävention, Diagnostik und Therapie von Ohrerkrankungen

Author

Bächinger, David, Jecker, Raphael, Hannig, Jean-Christoph, Werner, Andreas, Hildebrandt, Horst, Eidenbenz, Michael, Kompis, Martin, Kleinjung, Tobias, and Veraguth, Dorothe

Subjects
610 Medicine & health
Abstract

BACKGROUND Perfect hearing is crucial to the practice of various professions, such as instrument makers, musicians, sound engineers, and other professions not related to music, such as sonar technicians. For people of these occupational groups, we propose the term "professional ear user" (PEU) in analogy to "professional voice user". PEUs have special requirements for their hearing health, as they have well-known above-average auditory perceptual abilities on which they are professionally dependent. OBJECTIVE The purpose of this narrative review is to summarize selected aspects of the prevention, diagnosis, and treatment of ear disorders in PEUs. RESULTS AND CONCLUSION Prevention of hearing disorders and other ear diseases includes protection from excessive sound levels, avoidance of ototoxins and nicotine, and a safe manner of cleaning the outer auditory canal. Diagnosing hearing disorders in PEUs can be challenging, since subclinical but relevant changes in hearing cannot be reliably objectified by conventional audiometric methods. Moreover, the fact that a PEU is affected by an ear disease may influence treatment decisions. Further, physicians must be vigilant for non-organic ear diseases in PEUs. Lastly, measures to promote comprehensive ear health in PEUs as part of an educational program and to maintain ear health by means of a specialized otolaryngology service are discussed. In contrast to existing concepts, we lay the attention on the entirety of occupational groups that are specifically dependent on their ear health in a professional setting. In this context, we suggest avoiding a sole focus on hearing disorders and their prevention, but rather encourage the maintenance of a comprehensive ear health., Hintergrund Ein vollständig intaktes Hörvermögen ist zentral für die Ausübung verschiedener Berufe wie Instrumentenbaumeister, Musiker, Tonmeister sowie für weitere Berufsgruppen ohne Bezug zu Musik wie beispielsweise Sonar-Techniker. Für Personen all dieser Berufsgruppen schlagen wir in Analogie zum „Professional Voice User“ den Begriff „Professional Ear User“ (PEU) vor. PEU haben spezielle Anforderungen an ihre Ohrgesundheit, da sie über eine überdurchschnittliche auditive Wahrnehmungsfähigkeit verfügen, von der sie beruflich abhängig sind. Fragestellung Die vorliegende narrative Übersichtsarbeit hat zum Ziel, die sich daraus ergebenden speziellen Aspekte der Prävention, Diagnostik und Therapie von Ohrerkrankungen bei PEU zusammenzufassen. Ergebnisse und Schlussfolgerung Die Prävention von Hörstörungen und weiteren Ohrerkrankungen umfasst den Schutz vor zu hohen Schallpegeln, die Vermeidung von Ototoxinen oder Nikotin sowie die korrekte Durchführung einer Gehörgangsreinigung. Die Abklärung von Hörstörungen kann sich bei PEU herausfordernd gestalten, da subklinische, jedoch einschränkende Veränderungen des Hörvermögens mit konventionellen audiometrischen Methoden nicht zuverlässig objektiviert werden können. Schließlich kann das Vorliegen einer Ohrerkrankung bei einem PEU Therapieentscheidungen beeinflussen. Weiter muss bei PEU auch eine hohe Wachsamkeit bezüglich nichtorganischer Ohrerkrankungen bestehen. Abschließend werden Möglichkeiten diskutiert, um bei PEU eine umfassende Ohrgesundheit im Rahmen eines edukativen Programms zu fördern und mittels einer spezialisierten ohrenärztlichen Sprechstunde zu erhalten. Im Gegensatz zu bestehenden Konzepten ist der Fokus dabei auf die Gesamtheit der Berufsgruppen gerichtet, welche in professionellem Rahmen speziell von der Ohrgesundheit abhängig sind. Außerdem soll der Schwerpunkt hierbei nicht nur auf Hörstörungen und deren Prävention, sondern auch auf der Erhaltung einer ganzheitlichen Ohrgesundheit liegen.

Published
2022
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49. Characterization of Ca-Dicarboxylate Salt Hydrates as Thermochemical Energy Storage Materials.

Author

Werner, Jakob, Smith, Jakob, Stöger, Berthold, Artner, Werner, Werner, Andreas, and Weinberger, Peter

Subjects
ENERGY storage, GAS hydrates, HEAT storage, X-ray powder diffraction, APPLICATION stores, WASTE heat, SALT
Abstract

Salt hydrates are highly promising materials for thermochemical energy storage applications to store waste heat below 200 °C. Although highly researched and theoretically promising, in practical applications salt hydrates often cannot fulfill expectations. Based on the promising results of the Ca-oxalate monohydrate/Ca-oxalate system, other Ca-dicarboxylate salt hydrates were investigated to determine whether potential materials for heat storage can be found amongst them. A simultaneous thermal analysis showed that all candidates are applicable in the temperature range of 100–200 °C, and thermally stable up to 220 °C. Calcium malonate dihydrate (637 J/g), calcium terephthalate trihydrate (695 J/g), and tetrafluoro calcium terephthalate tetrahydrate (657 J/g) have shown higher enthalpies of dehydration than Ca-oxalate monohydrate. Due to the investigation of derivatives of Ca-terephthalate, it is possible to report the crystal structure of 2-fluoro calcium terephthalate. In single crystals, it forms a trihydrate and crystallizes in the Pmna space group (Z = 4, Z' = ½) forming infinite chains of Ca atoms. De- and rehydration reactions of the most promising candidates were studied in situ with powder X-ray diffraction showing the structural changes between the hydrate and anhydrate states. [ABSTRACT FROM AUTHOR]

Published
2023
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50. Overview of meningococcal epidemiology and national immunization programs in children and adolescents in 8 Western European countries

Author

Pinto Cardoso, Gaëlle, primary, Lagrée-Chastan, Marion, additional, Caseris, Marion, additional, Gaudelus, Joël, additional, Haas, Hervé, additional, Leroy, Jean-Philippe, additional, Bakhache, Pierre, additional, Pujol, Jean-François, additional, Werner, Andreas, additional, Dommergues, Marie-Aliette, additional, Pauquet, Emilie, additional, and Pinquier, Didier, additional

Published
2022
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