bluuebunny/crossref_metadata_2025_split huggingface dataset:
Can German Health Insurance Claims Data Fill Information Gaps in Rare Chronic Diseases: Use Case of Haemophilia A
Authors: Kratzer Vanessa , Rölz Verena , Bidlingmaier Christoph , Klamroth Robert , Behringer Jochen , Schramm Anja , Mansmann Ulrich , Berger Karin 
Published: July 2024

AbstractClaims data are increasingly discussed to evaluate health care for rare diseases (resource consumption, outcomes and costs). Using haemophilia A (HA) as a use case, this analysis aimed to generate evidence for the aforementioned information using German Statutory Health Insurance (SHI) claims data. Claims data (2017–2019) from the German SHI ‘AOK Bayern - Die Gesundheitskasse’ were used. Patients with ICD-10-GM codes D66 and HA medication were included in descriptive analyses. Severity levels were categorized according to HA medication consumption. In total, 257 patients were identified: mild HA, 104 patients (mean age: 40.0 years; SD: 22.9); moderate HA, 17 patients, (51.2 years; SD: 24.5); severe HA, 128 patients, (34.2 years; SD: 18.5). There were eight patients categorized with inhibitors (37.8 years; SD: 29.6). Psychotherapy was reported among 28.8% (mild) to 32.8% (severe) of patients. Joint disease was documented for 46.2% (mild) to 61.7% (severe) of patients. Mean direct costs per patient per year were 1.34× for mild, 11× for moderate, 81× higher for severe HA patients and 223× higher for inhibitor patients than the mean annual expenditure per AOK Bayern insurant (2019). German SHI data provide comprehensive information. The patient burden in HA is significant with respect to joint disease and psychological stress regardless of the HA severity level. The cost of HA care for patients is high. Large cost ranges suggest that the individual situation of a patient must be considered when interpreting costs. The main limitation of SHI data analysis for HA was the lack of granularity of ICD codes.
Pathophysiologie und Diagnostik der Immunthrombozytopenie
Authors: Giagounidis Aristoteles 
Published: July 2024

ZusammenfassungDie Immunthrombozytopenie (ITP) entsteht durch Autoantikörper-Bildung gegen Oberflächenantigene der Blutplättchen. Die Erkrankung tritt als primäre ITP ohne eindeutigen Auslöser oder als sekundäre ITP (Medikamente, andere Krankheitsbilder) auf. Die Autoantikörper-Bildung führt zu einem Verlust von Thrombozyten in Milz oder Leber und reduziert daneben auch die Bildung von Thrombozyten. Die Synthese von Thrombopoetin ist bei ITP-Patienten – im Gegensatz anderen Bildungsstörungen der Thrombozyten – reduziert. Die ITP ist eine Ausschlussdiagnose, beweisende Laboruntersuchung liegen nicht vor. Nur in 50% der Fälle lassen sich Glykoprotein-spezifische Antikörper nachweisen. Von besonderer Bedeutung ist der Ausschluss der EDTA-induzierten Pseudothrombozytopenie. Andere Ursachen der Thrombozytopenie sollten durch (Medikamenten-)Anamnese, körperliche Untersuchung und gegebenenfalls Knochenmarkpunktion ausgeschlossen werden.
Dynamics of Business Models in Circular Economy: Shifting Challenges in Pilot Projects
Authors: Lauten-Weiss J. , Friege H. , Westphal I. , Brinker J. 
Published: July 2024

AbstractThis study investigates the dynamics of circular business models (CBMs) through a multiple case study of 12 pilot projects funded by the German Federal Ministry of Education and Research between 2019 and 2022. The aim of this study was to draw practical conclusions for CBM pilot projects and public funding tenders, as well as to reveal theoretical insights into the motivation, perceptions of challenges, and potentials regarding CBMs and their shifts over time. Focused on remanufacturing and product-service systems (PSS), the study reveals significant shifts in motivation and perceived challenges before and after the pilot projects. Post-pilot, financial considerations emerged as a primary motivator for CBM adoption, alongside market pull factors and resource availability. However, challenges such as cost-related issues, supply chain complexities, and internal realignment hurdles persisted. Conversely, the perception of market-related challenges had decreased after the pilots, suggesting a growing readiness and adaptability of the market to CBMs. Similarly, experience-related challenges saw a decline, indicating an improvement in knowledge and capabilities within consortia over time. Moreover, the study examines shifts in perceived potential, revealing heterogeneous outcomes across different types of CBMs. While some projects identified new possibilities for value chain cooperation and transparency, others expressed skepticism about further potential, particularly in PSS-focused pilots. This study contributes to the growing body of literature on CBMs by providing empirical insights into the dynamics of circular economy (CE) pilot projects and their impact on organizational learning.
Crowned dens syndrome: Die Krone der
Kristallarthropathien
Authors: Schulz Nils , Lange Uwe , Klemm Philipp 
Published: July 2024

Zusammenfassung
Ziel Dieser Fallbericht soll das Bewusstsein für das seltene Crowned Dens
Syndrom (CDS) als unterdiagnostizierte Entität der Kristallarthropathie schärfen
und zur Verbesserung der Versorgungssituation beitragen in dem er Diagnose- und
Therapiestrategien diskutiert.
Methoden Es wird der Fall einer 68-jährigen Patientin mit CDS vorgestellt,
bei der eine Chondrokalzinose vorlag und rezidivierende Arthralgien bestanden.
Die Patientin verspürte Schmerzen insbesondere bei Rotationsbewegung des Kopfes
und präsentierte sich initial subfebril mit laborchemisch erhöhter
Entzündungsserologie.
Ergebnisse Mittels CT und Röntgenbildgebung konnte ein CDS diagnostiziert
und von anderen Differenzialdiagnosen abgegrenzt werden. Eine Meningitis konnte
mittels Lumbalpunktion ausgeschlossen werden. Unter eingeleiteter Therapie mit
Prednisolon und Ibuprofen besserten sich die Symptome der Patientin rasch. Vier
Wochen nach der Entlassung war die Patientin symptomfrei, mit normalen
Entzündungswerten und erfolgreicher Reduktion der Steroiddosis.
Fazit CDS sollte beim älteren Patienten mit akuten Nacken-/Kopfschmerzen
mit Steifheit und Fieber in Betracht gezogen werden insbesondere nach Ausschluss
einer entzündlich-rheumatischen, vaskulären und infektiösen Genese. Die Diagnose
wird mittels CT gesichert. Die Therapie erfolgt rein symptomatisch und besteht
aus Prednisolon und nichtsteroidalen Antirheumatika und führt in der Regel zu
einer schnellen Symptomlinderung. Eine Anfallsprophylaxe mit niedrigdosiertem
Colchizin kann durchgeführt werden.
Novel Insights into the Aortic Mechanical Properties of Mice Modeling Hereditary Aortic Diseases
Authors: Dubacher Nicolo , Sugiyama Kaori , Smith Jeffrey D. , Nussbaumer Vanessa , Csonka Máté , Ferenczi Szilamér , Kovács Krisztina J. , Caspar Sylvan M. , Lamberti Lisa , Meienberg Janine , Yanagisawa Hiromi , Sheppard Mary B. , Matyas Gabor 
Published: July 2024

Objective Hereditary aortic diseases (hADs) increase the risk of aortic dissections and ruptures. Recently, we have established an objective approach to measure the rupture force of the murine aorta, thereby explaining the outcomes of clinical studies and assessing the added value of approved drugs in vascular Ehlers–Danlos syndrome (vEDS). Here, we applied our approach to six additional mouse hAD models.
Material and Methods We used two mouse models (Fbn1C1041G
and Fbn1mgR
) of Marfan syndrome (MFS) as well as one smooth-muscle-cell-specific knockout (SMKO) of Efemp2 and three CRISPR/Cas9-engineered knock-in models (Ltbp1, Mfap4, and Timp1). One of the two MFS models was subjected to 4-week-long losartan treatment. Per mouse, three rings of the thoracic aorta were prepared, mounted on a tissue puller, and uniaxially stretched until rupture.
Results The aortic rupture force of the SMKO and both MFS models was significantly lower compared with wild-type mice but in both MFS models higher than in mice modeling vEDS. In contrast, the Ltbp1, Mfap4, and Timp1 knock-in models presented no impaired aortic integrity. As expected, losartan treatment reduced aneurysm formation but surprisingly had no impact on the aortic rupture force of our MFS mice.
Conclusion Our read-out system can characterize the aortic biomechanical integrity of mice modeling not only vEDS but also related hADs, allowing the aortic-rupture-force-focused comparison of mouse models. Furthermore, aneurysm progression alone may not be a sufficient read-out for aortic rupture, as antihypertensive drugs reducing aortic dilatation might not strengthen the weakened aortic wall. Our results may enable identification of improved medical therapies of hADs.
Point-of-Care Testing in Patients with Hereditary Disorders of Primary Hemostasis: A Narrative Review
Authors: Bavinck Aernoud P. , Heerde Waander van , Schols Saskia E.M. 
Published: July 2024

AbstractInherited disorders of primary hemostasis, such as von Willebrand disease and congenital platelet disorders, can cause extensive, typically mucocutaneous bleeding. Assays to diagnose and monitor these disorders, such as von Willebrand factor activity assays and light transmission aggregometry, are performed in specialized hemostasis laboratories but are commonly not available in local hospitals. Due to the complexity and relative scarcity of these conventional assays, point-of-care tests (POCT) might be an attractive alternative in patients with hereditary bleeding disorders. POCTs, such as thromboelastography, are increasingly used to assess hemostasis in patients with acquired hemostatic defects, aiding clinical decision-making in critical situations, such as during surgery or childbirth. In comparison, the use of these assays in patients with hereditary hemostasis defects remains relatively unexplored. This review aims to give an overview of point-of-care hemostasis tests in patients with hereditary disorders of primary hemostasis. A summary of the literature reporting on the performance of currently available and experimental POCTs in these disorders is given, and the potential utility of the assays in various use scenarios is discussed. Altogether, the studies included in this review reveal that several POCTs are capable of identifying and monitoring severe defects in the primary hemostasis, while a POCT that can reliably detect milder defects of primary hemostasis is currently lacking. A better understanding of the strengths and limitations of POCTs in assessing hereditary defects of primary hemostasis is needed, after which these tests may become available for clinical practice, potentially targeting a large group of patients with milder defects of primary hemostasis.
The politics of pro-outsider labour market reforms: a configurational study
Authors: Marques Paulo , Branco Rui , Guimarães Rita 
Published: July 2024

AbstractOver the past decades, the level of regulation of fixed-term contracts has been in flux. Many reforms deregulating these contracts were followed by a regulatory countertrend, a puzzling finding considering influential theoretical expectations like the ones developed by the dualization and liberalization literatures. This paper draws on the comparative employment relations and comparative political economy literatures to identify the drivers of pro-outsider reforms. Furthermore, it develops an innovative argument claiming that governments may rely on support from a ‘domestic coalition’ including workers in permanent contracts, or they can leverage the European link to support their intention of conducting a pro-outsider reform. Each of these paths relies on the assemblage of distinct pro-reform coalitions of policy actors. To test our argument, the paper uses fsQCA. We compare 38 reforms enacted in 16 European countries between 1985–2019. After the fsQCA analysis, we conduct a qualitative discussion of the different paths.
Ion association behaviors in the initial stage of calcium carbonate formation: An ab initio study
Authors: Li Yue , Zhang Jiarui , Zeng Hongbo , Zhang Hao 
Published: July 2024

In this work, we performed static density functional theory calculations and ab initio metadynamics simulations to systematically investigate the association mechanisms and dynamic structures of four kinds of ion pairs that could be formed before the nucleation of CaCO3. For Ca2+–HCO3− and Ca2+–CO32− pairs, the arrangement of ligands around Ca2+ evolves between the six-coordinated octahedral structure and the seven-coordinated pentagonal bipyramidal structure. The formation of ion pairs follows an associative ligand substitution mechanism. Compared with HCO3−, CO32− exhibits a stronger affinity to Ca2+, leading to the formation of a more stable precursor phase in the prenucleation stage, which promotes the subsequent CaCO3 nucleation. In alkaline environments, excessive OH− ions decrease the coordination preference of Ca2+. In this case, the formation of Ca(OH)+–CO32− and Ca(OH)2–CO32− pairs favors the dissociative ligand substitution mechanism. The inhibiting effects of OH− ion on the CaCO3 association can be interpreted from two aspects, i.e., (1) OH− neutralizes positive charges on Ca2+, decreases the electrostatic interactions between Ca2+ and CO32−, and thus hinders the formation of the CaCO3 monomer, and (2) OH− decreases the capacity of Ca2+ for accommodating O, making it easier to separate Ca2+ and CO32− ions. Our findings on the ion association behaviors in the initial stage of CaCO3 formation not only help scientists evaluate the impact of ocean acidification on biomineralization but also provide theoretical support for the discovery and development of more effective approaches to manage undesirable scaling issues.
Hyperfine interactions for small systems including transition-metal elements using self-interaction corrected density-functional theory
Authors: Karanovich Anri , Jackson Koblar Alan , Park Kyungwha 
Published: July 2024

The interactions between the electronic magnetic moment and the nuclear spin moment, i.e., magnetic hyperfine (HF) interactions, play an important role in understanding electronic properties of magnetic systems and in realizing platforms for quantum information science applications. We investigate the HF interactions for atomic systems and small molecules, including Ti or Mn, by using Fermi–Löwdin orbital (FLO) based self-interaction corrected (SIC) density-functional theory. We calculate the Fermi contact (FC) and spin-dipole terms for the systems within the local density approximation (LDA) in the FLO-SIC method and compare them with the corresponding values without SIC within the LDA and generalized-gradient approximation (GGA), as well as experimental data. For the moderately heavy atomic systems (atomic number Z ≤ 25), we find that the mean absolute error of the FLO-SIC FC term is about 27 MHz (percentage error is 6.4%), while that of the LDA and GGA results is almost double that. Therefore, in this case, the FLO-SIC results are in better agreement with the experimental data. For the non-transition-metal molecules, the FLO-SIC FC term has the mean absolute error of 68 MHz, which is comparable to both the LDA and GGA results without SIC. For the seven transition-metal-based molecules, the FLO-SIC mean absolute error is 59 MHz, whereas the corresponding LDA and GGA errors are 101 and 82 MHz, respectively. Therefore, for the transition-metal-based molecules, the FLO-SIC FC term agrees better with experiment than the LDA and GGA results. We observe that the FC term from the FLO-SIC calculation is not necessarily larger than that from the LDA or GGA for all the considered systems due to the core spin polarization, in contrast to the expectation that SIC would increase the spin density near atomic nuclei, leading to larger FC terms.
Enhancing the dielectric constant of zwitterionic liquids via dipole moment and anion chemistry
Authors: Mei Wenwen , Colby Ralph H. , Hickey Robert J. 
Published: July 2024

The dielectric constant is a critical parameter in many energy-related applications. Typically, increasing the dielectric constant of soft materials involves adding high dielectric constant polar liquids or inorganic fillers, but there are limitations to this approach due to safety concerns with volatile and flammable solvents and the agglomeration of inorganic fillers. An alternative approach is to add zwitterionic liquids that exhibit exceptionally high dielectric constants with negligible volatility. Here, we report the synthesis of a series of zwitterionic liquids containing an imidazolium cation, exhibiting the highest dielectric constant among all organic molecules (∼350 at 293 K). The cation–anion linkage was tailored in a wide range between three and nine carbons, rendering the zwitterion dipole from 25 to 52 D. Comparing the dielectric constant for zwitterions with different anions (i.e., sulfonylimide, sulfonate, and carboxylate) reveals the beneficial impacts of the delocalized sulfonylimide anion vs the carboxylate anion due to the enlarged molecular dipole and more homogenous liquid morphology. Molecular dipole and liquid morphology are identified as the keys to developing high dielectric constant zwitterionic liquids. The extremely high dielectric constant accessible with the proposed molecular design paves new avenues for developing high dielectric constant zwitterions that act as dielectricizers.
Preparation of carbon/nickel composites by magnetron sputtering and study on electromagnetic shielding effectiveness
Authors: Zhang Yiwen , Fan Jingyi , Ji Peiyu , Li Maoyang , Tan Haiyun , Zhang Xiaoman , Huang Tianyuan , Wu Xuemei 
Published: July 2024

A pure nickel film and a carbon/nickel (C/Ni) composite film were produced using radio frequency magnetron sputtering. The structure of the films was regulated by adjusting the sputtering power and the time allocation of sputtering nickel and graphite targets. Furthermore, a scanning electron microscope, an x-ray diffractometer, and a Raman spectrometer were employed for sample characterization. The results demonstrated that the thickness of the pure nickel film and C/Ni composite film with a total deposition time of 60 s was between 44.96 and 65.31 nm. The nickel film exhibited preferential growth along the crystal plane (111), and the structure of carbon materials was in the second stage of the three-stage model known as “amorphization trajectory of graphite.” The prepared films’ electromagnetic interference shielding effectiveness (EMI SE) in the X-band was investigated using a vector network analyzer. It was observed that the C/Ni composite film with a thickness of 44.96 nm demonstrated a superior EMI SE with a maximum value reaching 21 dB. The EMI SE of the C/Ni composite film can reach the same performance and even exceed that of the pure nickel film obtained at the power of 200 W for 60 s in certain frequency segments. In conclusion, the pure nickel film can be replaced by C/Ni composite film prospectively due to their excellent EMI SE along with advantages such as reduced thickness, lighter weight, and lower cost.
Effect of Rare-Earth Element Substitution in Superconducting R3Ni2O7 under Pressure
Authors: Pan 潘 Zhiming 志明 , Lu 卢 Chen 晨 , Yang 杨 Fan 帆 , Wu 吴 Congjun 从军 
Published: July 2024

Abstract
Recently, high temperature (T
c ≈ 80 K) superconductivity (SC) has been discovered in La3Ni2O7 (LNO) under pressure. This raises the question of whether the superconducting transition temperature T
c could be further enhanced under suitable conditions. One possible route for achieving higher T
c is element substitution. Similar SC could appear in the Fmmm phase of rare-earth (RE) R3Ni2O7 (RNO, R = RE element) material series under suitable pressure. The electronic properties in the RNO materials are dominated by the Ni 3d orbitals in the bilayer NiO2 plane. In the strong coupling limit, the SC could be fully characterized by a bilayer single 3d

x
2–y
2
-orbital t–J
∥–J
⊥ model. With RE element substitution from La to other RE element, the lattice constant of the Fmmm RNO material decreases, and the resultant electronic hopping integral increases, leading to stronger superexchanges between the 3d

x
2–y
2
orbitals. Based on the slave-boson mean-field theory, we explore the pairing nature and the evolution of T
c in RNO materials under pressure. Consequently, it is found that the element substitution does not alter the pairing nature, i.e., the inter-layer s-wave pairing is always favored in the superconducting RNO under pressure. However, the T
c increases from La to Sm, and a nearly doubled T
c could be realized in SmNO under pressure. This work provides evidence for possible higher T
c R3Ni2O7 materials, which may be realized in further experiments.
Application of improved Wolf pack algorithm in planning and operation of multi-microgrid systems with electric vehicles
Authors: Sun Guohao , Zhang Shouming , Yang Sen , Zhao Yuhao 
Published: July 2024

Abstract
With the rapid growth of renewable energy sources and the widespread use of electric vehicles (EVs), the planning and operation problems of multiple microgrids (MMGs) have become more complex and diverse. This paper develop an MMG model with multiple renewable energy sources and small-scale EVs, aiming to maximize the use of renewable energy sources and realize the charging demand of EVs, and highlighting the potential role of EVs in MMGs. In addition, the paper underscores the indispensable role of measurement technology in microgrids and the impetus that microgrid development provides for advancements in measurement technology. To this end, this paper proposes an improved Wolf pack algorithm (IWPA) based on the standard Wolf Pack Algorithm (WPA) with a spiral search approach and chaotic updating of individuals to improve the global search capability of the algorithm and the complexity of solving the scheduling problem. Through simulation experiments on ten standard test functions and examples, it is verified that the IWPA algorithm improves the search accuracy by 2.8%–6.8% and 13.9%–18.3% in the worst and best cases, respectively, in comparison with other algorithms, and it also has a faster convergence speed. Meanwhile, this paper proposes a load interval pricing strategy for the shortcomings of time-of-use pricing strategy and traditional real-time pricing strategy, which is simulated under grid-connected operation, isolated grid operation, and multi-microgrid cooperative operation modes, and the simulation results of the arithmetic example show that this strategy can effectively reduce carbon emissions, and IWPA can effectively coordinate renewable energy, EVs, and other energy resources to achieve efficient energy management of MMGs and supply-demand balance.
Interplay of electromagnetically induced transparency and Doppler broadening in hot atomic vapors
Authors: Zhang (张理达) Lida , Stiesdal Nina , Busche Hannes , Gaard Hansen Mikkel , Pohl Thomas , Hofferberth Sebastian 
Published: July 2024

Abstract
For multi-level systems in hot atomic vapors the interplay between the Doppler shift due to atomic motion and the wavenumber mismatch between driving laser fields strongly influences transmission and absorption properties of the atomic medium. In a three-level atomic ladder-system, Doppler broadening limits the visibility of electromagnetically-induced transparency (EIT) when the probe and control fields are co-propagating, while EIT is recovered under the opposite condition of counter-propagating geometry and

k

p

<

k

c

, with

k

p

and

k

c

being the wavenumbers of the probe and control fields, respectively. This effect has been studied and experimentally demonstrated as an efficient mechanism to realize non-reciprocal probe light transmission, which may enable applications as magnetic-field free optical isolators. Here, we describe the basics of this effect and discuss a simple picture for the underlying mechanism. We illustrate how the non-reciprocity scales with wavelength mismatch and show how to experimentally demonstrate the effect in a simple Rydberg-EIT system using thermal Rubidium atoms.