bluuebunny/crossref_metadata_2025_split huggingface dataset:
Risk factors for impaired health-related quality of life in a cohort of pediatric patients with inborn metabolic diseases
Authors: Siegert Sandy , Roscher Anne , Moeslinger Dorothea , Konstantopoulou Vassiliki , Herle Marion 
Published: October 2021

AbstractIn the last decade, health-related quality of life (HrQoL) has become an increasingly important outcome parameter in children and adolescents with chronic health conditions; among them are pediatric patients with inborn metabolic diseases (IMDs). Hence, knowledge on this topic is increasing, but findings on non-medical influences on the HrQoL of IMD patients are still scarce. In the present study, we retrospectively evaluated the self-reported generic HrQoL of a cohort of pediatric patients (ages 7 to 17 years) with diverse IMDs (n = 204) and explored associations between HrQoL and psychosocial and medical characteristics of the patients. We aimed to identify risk factors for impaired HrQoL to improve and tailor support for the patients and economize resources. Generic HrQoL was assessed with the KINDL-R questionnaire. We compared the HrQoL scores to published German normative data and analyzed the impact of demographic variables and intellectual and psychosocial functioning on the HrQoL. Moreover, we examined the influence of the diagnostic category and the health impairment (as judged by the physicians) on our patients’ HrQoL. Overall, the HrQoL of the adolescent patients was comparable to the HrQoL of the norm group. Disorders of intellectual development, impaired psychosocial functioning, and a severe health impairment were associated with lower HrQoL scores.Conclusion: We recommend evaluating these factors in children and adolescents with IMDs to identify patients at risk for impaired HrQoL.

What is Known:• Studies on HrQoL in pediatric patients with IMDs mainly focused on subgroups with specific diagnoses and found normal HrQoL in some of those subgroups.• In healthy children and adolescents as well as in pediatric patients with various chronic diseases, associations between psychosocial factors and HrQoL are well known.

What is New:• Impaired psychosocial functioning, disorders of intellectual development, and a significant disease and/or treatment burden are risk factors for impaired HrQoL in pediatric patients with IMDs.• Evaluating these factors in children and adolescents with IMDs can help identify patients and families in need of enhanced psychological support.
Numerical Modelling of Microwave Heating Assisted Rock Fracture
Authors: Pressacco Martina , Kangas Jari J. J. , Saksala Timo 
Published: October 2021

AbstractThis paper presents a numerical study on the effects of microwave irradiation on the mechanical properties of hard rock. More specifically, the weakening effect of microwave heating induced damage on the uniaxial compressive and tensile strength of granite-like rock is numerically evaluated. Rock fracture is modelled by means of a damage-viscoplasticity model with separate damage variables for tensile and compressive failure types. We develop a global solution strategy where the electromagnetic problem is solved first separately in COMSOL multiphysics software, and then provided into a staggered implicit solution method for the thermo-mechanical problem. The thermal and mechanical parts of the problem are considered as uncoupled due to the dominance of the microwave-induced heat source. The model performance is tested in 2D finite element simulations of heterogeneous numerical rock specimens subjected first to heating in a microwave oven and then to uniaxial compression and tension tests. According to the results, the compressive and tensile strength of rock can be significantly reduced by microwave heating pretreatment.
Myoclonic status epilepticus and cerebellar hypoplasia associated with a novel variant in the GRIA3 gene
Authors: Rinaldi Berardo , Ge Yu-Han , Freri Elena , Tucci Arianna , Granata Tiziana , Estienne Margherita , Sun Jia-Hui , Gérard Bénédicte , Bayat Allan , Efthymiou Stephanie , Gervasini Cristina , Shi Yun Stone , Houlden Henry , Marchisio Paola , Milani Donatella 
Published: November 2021

AbstractAMPA-type glutamate receptors (AMPARs) are postsynaptic ionotropic receptors which mediate fast excitatory currents. AMPARs have a heterotetrameric structure, variably composed by the four subunits GluA1-4 which are encoded by genes GRIA1-4. Increasing evidence support the role of pathogenic variants in GRIA1-4 genes as causative for syndromic intellectual disability (ID). We report an Italian pedigree where some male individuals share ID, seizures and facial dysmorphisms. The index subject was referred for severe ID, myoclonic seizures, cerebellar signs and short stature. Whole exome sequencing identified a novel variant in GRIA3, c.2360A > G, p.(Glu787Gly). The GRIA3 gene maps to chromosome Xq25 and the c.2360A > G variant was transmitted by his healthy mother. Subsequent analysis in the family showed a segregation pattern compatible with the causative role of this variant, further supported by preliminary functional insights. We provide a detailed description of the clinical evolution of the index subjects and stress the relevance of myoclonic seizures and cerebellar syndrome as cardinal features of his presentation.
Leveraging A Lenient Category in Practicing Responsible Leadership: A Case Study
Authors: Jiang Xueting , Prokopovych Bogdan , DiStefano Garett 
Published: October 2021

AbstractIn this extended case study, we examine how business leaders translate a responsible leadership mindset into practice. By studying the leadership team and stakeholders of a large US college dining provider, we found that organization executives leverage the lenient market category of local food to successfully connect with and satisfy the interests of different stakeholder groups. We show that lenient categories, those with ambiguity and unclear boundaries, could be used by organizations as strategic devices to integrate the diverse needs of their stakeholders and foster positive stakeholder relationships. Based on our findings, we develop a theoretical model to illustrate how responsible leaders take advantage of meaning structures of the market category they have adopted to achieve both financial and social benefits for a broad range of stakeholders.
Transdiagnostic Processes as Mediators of Change in an Internet-Delivered Intervention Based on the Unified Protocol
Authors: Schaeuffele Carmen , Bär Jonathan , Buengener Inken , Grafiadeli Raphaela , Heuthaler Eva , Strieder Judith , Ziehn Patricia , Knaevelsrud Christine , Renneberg Babette , Boettcher Johanna 
Published: October 2021

Abstract
Background
Transdiagnostic treatments target shared mechanisms between disorders to facilitate change across diagnoses. The Unified Protocol (UP) aims at changing dysfunctional reactions towards emotions by increasing mindful emotion awareness and cognitive flexibility, as well as decreasing anxiety sensitivity and emotion avoidance.

Method
We investigated whether these transdiagnostic processes were malleable by treatment and mediated the relationship between treatment and outcome in an internet-delivered adaptation of the UP. N = 129 participants with mixed anxiety, depressive, and somatic symptom disorders were randomized to treatment or waitlist.

Results
The treatment yielded significant changes in all transdiagnostic processes over time in comparison to a waitlist condition. In separate mediator models, significant mediating effects were found for mindfulness, cognitive flexibility, behavioral activation, and experiential avoidance. When all mediators were combined in a multiple mediator model, the indirect effects through mindfulness and cognitive flexibility emerged as significant.

Conclusion
These findings add to the growing body of research on transdiagnostic processes as mediators of change and emphasize mindfulness and cognitive flexibility as a transdiagnostic treatment target. However, these results should be interpreted cautiously, as temporal precedence could not be established.
Predicate Transformer Semantics for Hybrid Systems
Authors: Huerta y Munive Jonathan Julián , Struth Georg 
Published: October 2021

AbstractWe present a semantic framework for the deductive verification of hybrid systems with Isabelle/HOL. It supports reasoning about the temporal evolutions of hybrid programs in the style of differential dynamic logic modelled by flows or invariant sets for vector fields. We introduce the semantic foundations of this framework and summarise their Isabelle formalisation as well as the resulting verification components. A series of simple examples shows our approach at work.
Isavuconazole and Liposomal Amphotericin B as Successful Combination Therapy of Refractory Invasive Candidiasis in a Liver Transplant Recipient: A Case Report and Literature Review
Authors: Odysseos Georgios , Mayr Ulrich , Bozsaki Gabor , Seidensticker Christian , Ehmer Ursula , Schmid Roland M. , Lahmer Tobias , Dill Veronika 
Published: October 2021

AbstractInvasive fungal infections in liver transplant recipients are associated with elevated morbidity and mortality and pose a challenge to the treating physicians. Despite of lacking clinical data, the use of antifungal combination therapy is often considered to improve response rates in an immunocompromised patient population. We herein report a case of refractory invasive candidiasis in a liver transplant recipient treated successfully with a combination of isavuconazole und high-dose liposomal amphotericin B. The antimycotic combination treatment was able to clear a bloodstream infection with C. glabrata and led to regression of bilomas among tolerable side effects. The use of the above-mentioned antifungal combination therapy in a liver transplant recipient has not been reported previously. This case highlights the efficacy and safety of antifungal combination therapy in immunocompromised patients with refractory invasive candidiasis.
Secondary Cities in West Africa: Urbanity, Power, and Aspirations
Authors: Ammann Carole , Sanogo Aïdas , Heer Barbara 
Published: October 2021

AbstractThis article claims space for secondary cities in urban studies. It criticizes that scientists tend to study urban life in metropolises and, hence, do not represent urban life in its full diversity. In reality, the majority of the worlds’ urban dwellers live in secondary cities; therefore, research on urbanity should reflect this fact. The article argues against simple approaches to secondary cities, such as defining them based on a single quantitative variable like population size. It rather proposes that anthropological research has a unique potential to reveal the urban dwellers’ relational and situational perceptions of, and perspectives towards, secondary cities. The paper puts this approach into practice by examining two West African secondary cities: Kankan in Guinea and Bouaké in Côte d’Ivoire.
Modification in gate valve using flexible membrane pipe for flow measurement
Authors: Bhilare S. L. , Hinge G. A. , Kumbhalkar M. A. , Rambhad K. S. 
Published: November 2021

Abstract
A gate valve is used to regulate discharge in pipelines. It is rotated by a wheel that is attached to a circular disc (i.e. gate) at the top of a stem. Each revolution of the wheel creates a particular linear disc movement that changes the flow zone. The paper describes the journey of gate valve from flow control device to flow measuring device and presents an experimentation on conventional gate valve and gate valve with flexible membrane pipe. The findings of experimental study are described and validated with CFD analysis of conventional and modified gate valve. The experimental study has shown that the modified gate valve can be used as flow measuring device. The results obtained represent the excellent improvement in the relationship between disc position (i.e., angle) and discharge. The results of CFD analysis for flow through gate valve with and without flexible membrane are compared. After carrying out the CFD studies without flexible membrane, it is found that, fluctuating separation zones formed on either side of gate are responsible to a great extent to the flow rate descripancies. This paper compares the experimental and CFD results and also suggests the remedy to address the problem.

Article highlights

Introduced simply a flexible membrane pipe inside PVC to convert gate valve into flow measuring device.

Reduction in flow separation and fluctuation in flow rate.

Smooth flow rate even though gate valve opening is small.
How Early in Life do Transgender Adults Begin to Experience Gender Dysphoria? Why This Matters for Patients, Providers, and for Our Healthcare System
Authors: Zaliznyak Michael , Yuan Nance , Bresee Catherine , Freedman Andrew , Garcia Maurice M. 
Published: October 2021

Abstract

Introduction
The age at which transgender women (TW) and men (TM) first experience gender dysphoria (GD) has not been reported in a U.S. population of adults seeking genital gender-affirming surgery (gGAS). Because gender is an innate part of identity, we hypothesized that untreated GD would be a part of individuals’ earliest memories. Understanding GD onset can help guide providers with when and how to focus care to patients not yet identified as “transgender

Aim
(i) Determine the age at which transgender adults seeking gGAS first experience GD
(ii) Determine the number of life-years that transgender adults spend living with untreated GD

Methods
During initial consultation for gGAS, we asked patients the earliest age at which they experienced GD and the age at which they had their earliest episodic memory. We also queried history of anxiety, depression, and suicide attempt.

Main Outcome Measures
Patients self-reported their earliest recollections of experiencing GD, earliest memories in general, and history of anxiety, depression, and suicide attempt.

Results
Data from 155 TW (mean age 41.3; SD 16.3) and 55 TM (mean age 35.4; SD 10.8) were collected. Most patients (TM: 78%; TW: 73%) reported experiencing GD for the first time between ages 3 and 7 years. For TM the mean age of onset was 6.17 years; for TW it was 6.71 years. A total of 81% of TW and 80% of TM described their first recollection of GD as one of their earliest memories. Mean years of persistent GD before the start of gender transition were 22.9 (TM) and 27.1 (TW). Rates of depression, anxiety, and suicide ideation decreased following gender transition.

Conclusion
Our findings suggest that GD typically manifests in early childhood and persists untreated for many years before individuals commence gender transition. Diagnosis and early management during childhood and adolescence can improve quality of life and survival.
PL DENSITY INVARIANT FOR TYPE II DEGENERATING K3 SURFACES, MODULI COMPACTIFICATION AND HYPER-KÄHLER METRIC
Authors: ODAKA YUJI 
Published: November 2021

AbstractA protagonist here is a new-type invariant for type II degenerations of K3 surfaces, which is explicit piecewise linear convex function from the interval with at most
$18$
nonlinear points. Forgetting its actual function behavior, it also classifies the type II degenerations into several combinatorial types, depending on the type of root lattices as appeared in classical examples.From differential geometric viewpoint, the function is obtained as the density function of the limit measure on the collapsing hyper-Kähler metrics to conjectural segments, as in [HSZ19]. On the way, we also reconstruct a moduli compactification of elliptic K3 surfaces by [AB19], [ABE20], [Brun15] in a more elementary manner, and analyze the cusps more explicitly.We also interpret the glued hyper-Kähler fibration of [HSVZ18] as a special case from our viewpoint, and discuss other cases, and possible relations with Landau–Ginzburg models in the mirror symmetry context.
People, animals, and island encounters: A pig’s history of the Pacific
Authors: Sand Jordan 
Published: November 2021

AbstractThis essay traces the diffusion of pigs and the introduction of new practices of pig husbandry in East Asia and the Pacific, with particular attention to the cases of Hawaii, Okinawa, and Japan. Countering the trend in animal history to emphasize environmental and genetic factors, it demonstrates that discourses of property, sovereignty, freedom, and slavery, brought to the region with modern imperialism, played a decisive role in shaping relationships between people and domesticated animals. The essay concludes that global diffusion of capitalist forms of animal husbandry depended on a process of disembedding animals from earlier social roles. This process took different forms in different places. It was in part ecological and in part economic, but must be understood first in the context of the movement of political ideas.
Structure and Stress Field of the Lithosphere Between Pamir and Tarim
Authors: Bloch Wasja , Schurr Bernd , Yuan Xiaohui , Ratschbacher Lothar , Reuter Sanaa , Kufner Sofia‐Katerina , Xu Qiang , Zhao Junmeng 
Published: October 2021

AbstractThe Pamir plateau protrudes ∼300 km between the Tajik‐ and Tarim‐basin lithosphere of Central Asia. Whether its salient location and shape are caused by forceful indentation of a promontory of Indian mantle lithosphere is debated. We present a new local‐seismicity and focal‐mechanism catalog, and a P‐wave velocity model of the eastern part of the collision system. The data suggest a south‐dipping Asian slab that overturns in its easternmost segment. The largest principal stress at depth acts normal on the slab and is orientated parallel to the plate convergence direction. In front (south) of the Asian slab, a volume of mantle with elevated velocities and lined by weak seismicity constitutes the postulated Indian mantle indenter. We propose that the indenter delaminates and overturns the Asian slab, underthrusts the Tarim lithosphere along a compressive transform boundary, and controls the location and shape of the Pamir plateau.
Investigating Predictability of DIC and SST in the Argentine Basin Through Wind Stress Perturbation Experiments
Authors: Swierczek Stan , Mazloff Matthew R. , Russell Joellen L. 
Published: October 2021

AbstractThe confluence of the Malvinas and Brazil currents over the Argentine Basin give the region chaotic dynamics and severely limit potential predictability. To probe the forecast horizon for ocean surface quantities of temperature and carbon, we construct regional models of the Argentine Basin with biogeochemistry at 1/3° and 1/12° resolution and design a series of experiments. We add positive and negative zonal wind stress anomalies over small and large areas during a short period in different model runs. We calculate the response of the surface temperature and DIC. The 1/3° model maintains predictability for up to 45 days, while the 1/12° model has a shorter window of about two weeks. However, the 1/3° model response is only consistent with the 1/12° model for about 8 days calling into question the potential predictive skill of the coarser model at longer lead times.
Strong Seasonal Variations of Seismic Velocity in Eastern Margin of Tibetan Plateau and Sichuan Basin From Ambient Noise Interferometry
Authors: Li Jinwu , Song Xiaodong , Yang Yi , Li Mengkui , Li Jiangtao , Li Yane 
Published: November 2021

AbstractAmbient noise interferometry between seismic stations makes it possible to monitor temporal changes of the Earth's media continuously. Here, we examined seasonal changes of Rayleigh‐wave empirical Greens functions at 2–10‐s period from 8 years of continuous data of 200 seismic station pairs in Sichuan, China. We used a wavelet decomposition to extract the seasonal signals. We found that most station pairs show seasonal variations with velocity perturbations of up to 0.8%. The seasonality varies in strength, amplitude, phase, periods, and location, but the phase is remarkably consistent, with the largest changes mostly in summer or winter months. We believe that the cause of the seasonality is likely the elastic loading due to regional precipitation. The effect is greater in the tectonically active Tibetan Plateau margin than in the stable Sichuan basin and in the deeper crust than in the uppermost crust. The effect is also strongly influenced by local crustal structure. Our results indicate that studies of tectonic crustal changes need to consider non‐tectonic effects.
Assessing the Role of Snow Cover for Post‐Wildfire Revegetation Across the Pacific Northwest
Authors: Wilson Andrew C. , Nolin Anne W. , Bladon Kevin D. 
Published: November 2021

AbstractForested, mountain landscapes in the Pacific Northwest (PNW) are changing at an unprecedented rate, largely due to shifts in the regional climate regime. Documented climate warming trends across the PNW include increasing wildfire frequency and severity and an increasingly ephemeral snowpack, especially at moderate elevations. We analyzed 24 high severity wildfires across four distinct PNW mountainous subregions, examining snow‐vegetation relationships for two years pre‐fire and four years post‐fire. To assess the importance of snow cover for revegetation compared to other climatic, topographic, and burn severity‐related variables, binary regression tree models were constructed for the dominant pre‐fire conifer species within each of the four PNW subregions. Summer precipitation consistently appeared as the most important variable driving post‐fire revegetation across all four subregions. Snow cover variables (snow cover frequency and snow disappearance date), along with elevation, were shown to be secondary but significantly influential explanatory variables for revegetation in the Oregon and Washington Cascades. Revegetation was also analyzed using a time series of linear regressions across 200‐m elevation bands by measuring correlations between winter snow cover and summer vegetation greenness. Results showed strong positive post‐fire correlations at moderate elevations in the western Montana Rockies and at the lowest elevation band in the Idaho Rockies. Considering trends of increasing wildfire activity, lower snowpacks, and earlier snow disappearance dates across the PNW, forests will likely experience more frequent drought conditions that will impact post‐wildfire vegetation regrowth.
The existence of a nonclassical TCA cycle in the nucleus that wires the metabolic-epigenetic circuitry
Authors: Liu Xujun , Si Wenzhe , He Lin , Yang Jianguo , Peng Yani , Ren Jie , Liu Xiaoping , Jin Tong , Yu Huajing , Zhang Zihan , Cheng Xiao , Zhang Wenting , Xia Lu , Huang Yunchao , Wang Yue , Liu Shumeng , Shan Lin , Zhang Yu , Yang Xiaohan , Li Haixia , Liang Jing , Sun Luyang , Shang Yongfeng 
Published: November 2021

AbstractThe scope and variety of the metabolic intermediates from the mitochondrial tricarboxylic acid (TCA) cycle that are engaged in epigenetic regulation of the chromatin function in the nucleus raise an outstanding question about how timely and precise supply/consumption of these metabolites is achieved in the nucleus. We report here the identification of a nonclassical TCA cycle in the nucleus (nTCA cycle). We found that all the TCA cycle-associated enzymes including citrate synthase (CS), aconitase 2 (ACO2), isocitrate dehydrogenase 3 (IDH3), oxoglutarate dehydrogenase (OGDH), succinyl-CoA synthetase (SCS), fumarate hydratase (FH), and malate dehydrogenase 2 (MDH2), except for succinate dehydrogenase (SDH), a component of electron transport chain for generating ATP, exist in the nucleus. We showed that these nuclear enzymes catalyze an incomplete TCA cycle similar to that found in cyanobacteria. We propose that the nTCA cycle is implemented mainly to generate/consume metabolic intermediates, not for energy production. We demonstrated that the nTCA cycle is intrinsically linked to chromatin dynamics and transcription regulation. Together, our study uncovers the existence of a nonclassical TCA cycle in the nucleus that links the metabolic pathway to epigenetic regulation.