bluuebunny/crossref_metadata_2025_split huggingface dataset:
A multidisciplinary approach to inherited retinal dystrophies from diagnosis to initial care: a narrative review with inputs from clinical practice
Authors: Murro Vittoria , Banfi Sandro , Testa Francesco , Iarossi Giancarlo , Falsini Benedetto , Sodi Andrea , Signorini Sabrina , Iolascon Achille , Russo Roberta , Mucciolo Dario Pasquale , Caputo Roberto , Bacci Giacomo Maria , Bargiacchi Sara , Turco Simona , Fortini Stefania , Simonelli Francesca 
Published: July 2023

AbstractBackgroundNon-syndromic inherited retinal dystrophies (IRDs) such as retinitis pigmentosa or Leber congenital amaurosis generally manifest between early childhood and late adolescence, imposing profound long-term impacts as a result of vision impairment or blindness. IRDs are highly heterogeneous, with often overlapping symptoms among different IRDs, and achieving a definite diagnosis is challenging. This narrative review provides a clinical overview of the non-syndromic generalized photoreceptor dystrophies, particularly retinitis pigmentosa and Leber congenital amaurosis. The clinical investigations and genetic testing needed to establish a diagnosis are outlined, and current management approaches are discussed, focusing on the importance of the involvement of an interdisciplinary team from diagnosis and initial care to long-term follow-up and support.ResultsThe effective management of IRDs requires a multidisciplinary, and ideally interdisciplinary, team of experts knowledgeable about IRDs, with experienced professionals from fields as diverse as ophthalmology, neuropsychiatry, psychology, neurology, genetics, orthoptics, developmental therapy, typhlology, occupational therapy, otolaryngology, and orientation and mobility specialties. Accurate clinical diagnosis encompasses a range of objective and subjective assessments as a prerequisite for the genetic testing essential in establishing an accurate diagnosis necessary for the effective management of IRDs, particularly in the era of gene therapies. Improvements in genome sequencing techniques, such as next-generation sequencing, have greatly facilitated the complex process of determining IRD-causing gene variants and establishing a molecular diagnosis. Genetic counseling is essential to help the individual and their family understand the condition, the potential risk for offspring, and the implications of a diagnosis on visual prognosis and treatment options. Psychological support for patients and caregivers is important at all stages of diagnosis, care, and rehabilitation and is an essential part of the multidisciplinary approach to managing IRDs. Effective communication throughout is essential, and the patient and caregivers’ needs and expectations must be acknowledged and discussed.ConclusionAs IRDs can present at an early age, clinicians need to be aware of the clinical signs suggesting visual impairment and follow up with multidisciplinary support for timely diagnoses to facilitate appropriate therapeutic or rehabilitation intervention to minimize vision loss.
Engineering Saccharomyces cerevisiae for improved biofilm formation and ethanol production in continuous fermentation
Authors: Wang Zhenyu , Xu Weikai , Gao Yixuan , Zha Mingwei , Zhang Di , Peng Xiwei , Zhang Huifang , Wang Cheng , Xu Chenchen , Zhou Tingqiu , Liu Dong , Niu Huanqing , Liu Qingguo , Chen Yong , Zhu Chenjie , Guo Ting , Ying Hanjie 
Published: July 2023

Abstract
Background
Biofilm-immobilized continuous fermentation has the potential to enhance cellular environmental tolerance, maintain cell activity and improve production efficiency.

Results
In this study, different biofilm-forming genes (FLO5, FLO8 and FLO10) were integrated into the genome of S. cerevisiae for overexpression, while FLO5 and FLO10 gave the best results. The biofilm formation of the engineered strains 1308-FLO5 and 1308-FLO10 was improved by 31.3% and 58.7% compared to that of the WT strain, respectively. The counts of cells adhering onto the biofilm carrier were increased. Compared to free-cell fermentation, the average ethanol production of 1308, 1308-FLO5 and 1308-FLO10 was increased by 17.4%, 20.8% and 19.1% in the biofilm-immobilized continuous fermentation, respectively. Due to good adhering ability, the fermentation broth turbidity of 1308-FLO5 and 1308-FLO10 was decreased by 22.3% and 59.1% in the biofilm-immobilized fermentation, respectively. Subsequently, for biofilm-immobilized fermentation coupled with membrane separation, the engineered strain significantly reduced the pollution of cells onto the membrane and the membrane separation flux was increased by 36.3%.

Conclusions
In conclusion, enhanced biofilm-forming capability of S. cerevisiae could offer multiple benefits in ethanol fermentation.

Graphical Abstract
Effects of the cascading translocations of larch (Larix decidua Mill.) on canker disease due to Lachnellula willkommii (R. Hartig) Dennis
Authors: Robin Cécile , Wagner Stefanie , Baubet Olivier , Ehrenmann François , Castagneyrol Bastien , Capdevielle Xavier , Fabreguettes Olivier , Petit Rémy J. , Piou Dominique 
Published: July 2023

Abstract
Key message
Appropriate silvicultural practices combined with the use of resistant Central European provenances can reduce the prevalence of larch canker caused by Lachnellula willkommii (R. Hartig) Dennis, a major disease affecting larch plantations in France. However, cascading translocations have resulted in frequent admixture in European larch (Larix decidua Mill.) and subsequent certification errors regarding the origin of plant material. Our study highlights the urgent need to improve the certification process of seed orchards.

Context
The recent history of European larch has been marked by translocations of plant stock within and beyond its native range. In order to increase stand resistance to larch canker disease, it is strongly recommended by French authorities to plant Central European provenances. However, a recent disease outbreak questioned the resistance of Central European provenances and the origin of the forest material used in these plantations.

Aims
Our goal was to assess the effects of cascading translocations and mixing between larch gene pools on genetic composition of plantations and on their vulnerability to the disease.

Methods
In the outbreak area, we checked the genetic origin of the trees and we estimated the percentage of Central European trees, disease prevalence and severity.

Results
Intra-site genetic diversity was high. Genetic composition did not match with geographic origin certificates. A high proportion of trees could not be assigned to either the Alpine or Central European gene pools. These admixed trees were as resistant as Central European trees. Geographic origin turned out to be one of the main drivers of canker prevalence, along with abiotic factors.

Conclusion
We need a precise knowledge of the origin of material used in seed orchards to mitigate canker disease and adapt forests through assisted migration.
Variation in herbivore space use: comparing two savanna ecosystems with different anthrax outbreak patterns in southern Africa
Authors: Huang Yen-Hua , Owen-Smith Norman , Henley Michelle D. , Kilian J. Werner , Kamath Pauline L. , Ochai Sunday O. , van Heerden Henriette , Mfune John K. E. , Getz Wayne M. , Turner Wendy C. 
Published: July 2023

Abstract
Background
The distribution of resources can affect animal range sizes, which in turn may alter infectious disease dynamics in heterogenous environments. The risk of pathogen exposure or the spatial extent of outbreaks may vary with host range size. This study examined the range sizes of herbivorous anthrax host species in two ecosystems and relationships between spatial movement behavior and patterns of disease outbreaks for a multi-host environmentally transmitted pathogen.

Methods
We examined range sizes for seven host species and the spatial extent of anthrax outbreaks in Etosha National Park, Namibia and Kruger National Park, South Africa, where the main host species and outbreak sizes differ. We evaluated host range sizes using the local convex hull method at different temporal scales, within-individual temporal range overlap, and relationships between ranging behavior and species contributions to anthrax cases in each park. We estimated the spatial extent of annual anthrax mortalities and evaluated whether the extent was correlated with case numbers of a given host species.

Results
Range size differences among species were not linearly related to anthrax case numbers. In Kruger the main host species had small range sizes and high range overlap, which may heighten exposure when outbreaks occur within their ranges. However, different patterns were observed in Etosha, where the main host species had large range sizes and relatively little overlap. The spatial extent of anthrax mortalities was similar between parks but less variable in Etosha than Kruger. In Kruger outbreaks varied from small local clusters to large areas and the spatial extent correlated with case numbers and species affected. Secondary host species contributed relatively few cases to outbreaks; however, for these species with large range sizes, case numbers positively correlated with outbreak extent.

Conclusions
Our results provide new information on the spatiotemporal structuring of ranging movements of anthrax host species in two ecosystems. The results linking anthrax dynamics to host space use are correlative, yet suggest that, though partial and proximate, host range size and overlap may be contributing factors in outbreak characteristics for environmentally transmitted pathogens.
Women Veterans’ experiences discussing household firearms with their intimate partners: collaborative, devalued, and deferential relational types
Authors: Polzer Evan R. , Rohs Carly M. , Thomas Suzanne M. , Holliday Ryan , Miller Christin N. , Simonetti Joseph A. , Iverson Katherine M. , Brenner Lisa A. , Monteith Lindsey L. 
Published: July 2023

Abstract
Background
Rates of firearm suicide have increased among women Veterans. Discussing firearm access and reducing access to lethal means of suicide when suicide risk is heightened are central tenets of suicide prevention, as is tailoring suicide prevention strategies to specific populations. While research has begun to explore how to optimize firearm lethal means safety counseling with women Veterans, there is limited knowledge of women Veterans' perspectives on including their intimate partners in such efforts. This gap is notable since many women Veterans have access to firearms owned by other household members. Understanding women Veterans’ experiences and perspectives regarding including their partners in firearm lethal means safety conversations can provide important information for tailoring firearm lethal means safety counseling for women Veterans.

Methods
Qualitative interviews were conducted with 40 women Veterans with current or prior household firearm access. Interview questions focused on the roles of women Veterans’ partners in household firearm access and storage, as well as women Veterans’ perspectives regarding including intimate partners in firearm lethal means safety counseling. Inductive thematic analysis was performed.

Results
Three relational types characterized how household firearms were discussed between women Veterans and their partners: collaborative, devalued, and deferential. These types were distinguished via women Veterans’ agency in decision-making related to household firearms, partners’ receptivity to women Veterans’ mental health or trauma histories, and willingness (or lack thereof) of partners to change household firearm access and storage considering such histories. Intimate partner violence was common in the devalued relational subtype.

Conclusions
Findings extend knowledge regarding the context of women Veterans’ household firearm access, including relational dynamics between women Veterans and their partners. The acceptability, feasibility, challenges, and facilitators of including women Veterans’ partners in firearm lethal means safety efforts likely vary for each relational type. For example, in dyads with a collaborative dynamic, incorporating partners may create opportunities for increased firearm safety, whereas including partners in devalued dynamics may present unique challenges. Research is warranted to determine optimal methods of navigating firearm lethal means safety counseling in the presence of each relational dynamic.
Weaning assessment of veno-arteriovenous (V-AV) extracorporeal membrane oxygenation (ECMO)
Authors: Heymer Johannes , Hein Anna , Ott Matthias , Schilling Tobias , Räpple Daniel 
Published: July 2023

AbstractCurrently, there is a lack of methods for simultaneous assessment of readiness for decannulation of the veno-venous (V-V) and veno-arterial (V-A) components during veno-arteriovenous (V-AV) extracorporal membrane oxygenation (ECMO) support. We describe a novel approach using a simultaneous off-sweep and controlled backflow test to assess readiness for decannulation from V-AV ECMO. This method needs testing in future clinical trials.
Bureaucratic configuration and discretion in asylum case processing: the case of the EUAA in Greece
Authors: Ekstedt Johan 
Published: July 2023

AbstractThis article takes an in-depth look at caseworkers at the European Union Asylum Agency in Greece. The agency’s increased role in asylum case processing and the emergence of the called “integrated European administration” is an unusual but nevertheless critical case to study for scholars of European asylum bureaucracies. Previous research into member state’s national migration authorities has shown that discretionary decision-making is widely deployed by asylum caseworkers. Generally, street-level bureaucrats tend to ‘reinterpret’ policy and creatively make use of the legal framework of the Common European Asylum System in order to make their day-to-day operations run more smoothly and resolve ethical dilemmas. However, this article finds that in the case of the European Union Asylum Agency, the compartmentalized institutional arrangement and short-term contracts removes both the incentive and ability for caseworkers to creatively reinterpret policy and deploy discretionary practices. It is therefore argued that scholars of asylum bureaucracy in Europe must pay increased attention to how the bureaucratic configuration of migration authorities.
An integrated approach for knowledge extraction and analysis in collaborative knowledge construction
Authors: Zhang Ning , Ouyang Fan 
Published: July 2023

AbstractCollaborative knowledge construction (CKC) involved students’ sharing of information, improvement of ideas, and construction of collective knowledge. In this process, knowledge extraction and analysis can provide valuable insights into students’ knowledge capacities, depths, and levels in order to improve the CKC quality. However, existing studies tended to extract and analyze knowledge from a single perspective (e.g., the number of certain knowledge types and knowledge structures), which failed to demonstrate the complexity and dynamics of knowledge construction and advancement. To fill this gap, this research designed a series of computer-supported collaborative concept mapping (CSCCM) activities to facilitate students’ CKC process and then used an integrated approach (i.e., semantic knowledge analysis combined with learning analytics) to extract, analyze, and understand students’ knowledge characteristics and evolutionary trends. Results demonstrated that compared to the low-performing pairs, the high-performing pairs mainly discussed knowledge related to the course content, and their knowledge evolution trend was relatively stable. Based on the results, this research provided analytical implications to extract, analyze, and understand students’ knowledge and pedagogical implications to promote students’ knowledge construction and advancement.
Mesenchymal stem cells modulate IL-17 and IL-9 production induced by Th17-inducing cytokine conditions in autoimmune arthritis: an explorative analysis
Authors: Riekert Maximilian , Almanzar Giovanni , Schmalzing Marc , Schütze Norbert , Jakob Franz , Prelog Martina 
Published: July 2023

Abstract
Background
The importance of proinflammatory T-cells and their cytokine production in patients with autoimmune arthritis has been widely described. Due to their immunomodulatory properties, mesenchymal stem cells (MSCs) have come into focus as a potential therapeutic concept. The aim of this study was to investigate the influence of MSCs on the phenotype, cytokine profile, and functionality of naive and non-naive CD4+ T-cells from healthy donors (HD) and patients with autoimmune arthritis under Th17-cytokine polarizing conditions in an explorative way using a transwell system prohibiting any cell–cell-contact.

Methods
Magnetically isolated naive and non-naive CD4+ T-cells were stimulated under Th17-polarizing proinflammatory cytokine conditions in presence and absence of bone marrow derived mesenchymal stromal cells (MSCs). After an incubation period of 6 days, the proportions of the T-cell subpopulations TEMRA (CD45RA+CD27−), memory (CD45RA−CD27+), effector (CD45RA−CD27−) and naive cells (CD45RA+CD27+) were determined. Quantitative immunofluorescence intensity was used as a measure for IL-9, IL-17 and IFN-γ production in each subpopulation.

Results
In isolated naive CD4+ T-cells from HD and patients, MSCs suppressed the differentiation of naive towards an effector phenotype while memory and naive cells showed higher percentages in culture with MSCs. In patients, MSCs significantly decreased the proportion of IL-9 and IL-17 producing effector T-cells. MSCs also reduced IFN-γ production in the naive and memory phenotype from HD.

Conclusion
The results of the study indicate significant immunomodulatory properties of MSCs, as under Th17-polarizing conditions MSCs are still able to control T-cell differentiation and proinflammatory cytokine production in both HD and patients with autoimmune arthritis.
Analysis of clinical risk factors of failed electrical cardioversion in patients with persistent atrial fibrillation or atrial flutter
Authors: Kim Ki-Hun , Choi Ha-Young , Park Jino , Song Yeo-Jeong , Kim Seunghwan , Kim Dong-Kie , Seol Sang-Hoon , Kim Doo-Il , Yang Pil-Sung , Lim Hong Euy , Park Junbeum , Shim Jae-Min , Ahn Jinhee , Lee Sung Ho , Im Sung Il , Kim Ju Youn 
Published: July 2023

Abstract
Background
Although rhythm control could be the best for symptomatic atrial fibrillation (AF), some patients fail to achieve sinus rhythm (SR). This study aimed to identify clinical risk factors of failed electrical cardioversion (ECV).

Methods
A total of 248 patients who received ECV for persistent AF or atrial flutter (AFL) were retrospectively reviewed. Patients were divided into three groups: Group 1 maintained SR for > 1 year, group 2 maintained SR ≤ 1 year after ECV, and group 3 failed ECV. SR maintenance was assessed using regular electrocardiography or Holter monitoring.

Results
Patients were divided into group 1 (73, 29%), group 2 (146, 59%), and group 3 (29, 12%). The mean age of patients was 60 ± 10 years, and 197 (79%) were male. Age, sex, and baseline characteristics were similar among groups. However, increased cardiac size, digoxin use, heart failure (HF), and decreased left ventricular ejection fraction (LVEF) were more common in group 3. Univariate analysis of clinical risk factors for failed ECV was increased cardiac size [hazard ratio (HR) 2.14 (95% confidence interval [CI], 1.06–4.34, p = 0.030)], digoxin use [HR 2.66 (95% CI, 1.15–6.14), p = 0.027], HF [HR 2.60 (95% CI, 1.32–5.09), p = 0.005], LVEF < 40% [HR 3.45 (95% CI, 1.00–11.85), p = 0.038], and decreased LVEF [HR 2.49 (95% CI, 1.18–5.25), p = 0.012]. Among them, HF showed clinical significance only by multivariate analysis [HR 3.01 (95% CI, 1.13–7.99), p = 0.027].

Conclusions
Increased cardiac size, digoxin use, HF, LVEF < 40%, and decreased LVEF were related to failed ECV for persistent AF or AFL. Among these, HF was the most important risk factor. Further multi-center studies including greater number of participants are planned.
Duo-decapolar catheter entrapment in the Chiari network: successful extraction with the snare catheter
Authors: Lee Kyung-Yeon , Rhee Tae-Min , Lee So-Ryoung , Choi Eue-Keun , Oh Seil 
Published: July 2023

Abstract
Background
The Chiari network (CN) is the net-like embryonic remnants of the right valve of the sinus venosus. Catheter entrapment by CN is a not uncommon complication during catheter manipulation inside the right atrium (RA).

Case presentation
A 49-year-old man with atrial fibrillation was admitted for cryoablation. Transesophageal echocardiography revealed a prominent CN in the RA. Through the sheath placed in the right femoral vein, a diagnostic electrophysiologic duo-decapolar (DD) catheter (Livewire, St Jude Medical, St Paul, MN) was advanced. The catheter tip was knotted after multiple rotations in the RA to properly locate the DD catheter at the RA wall and coronary sinus (CS). Initial attempts to disentangle the folded catheter tip by traction and rotation maneuvers were unsuccessful. We inserted the large-bore sheath (Sentrant; Medtronic, 20 Fr) into the patient’s left femoral vein and then, captured the folded catheter tip with the 20-mm circular snare catheter (Snare Kit, Medtronic, Minneapolis, MN). Then, we cut the proximal portion of the DD catheter externally and removed it retrogradely through the Flexcath sheath. We found that the catheter tip was entrapped by the fibrous tissue of the CN. After a successful retrieval, cryoablation was conducted as planned. Follow-up echocardiography did not reveal any peri-procedural complications.

Conclusions
The electrophysiological procedure that exists at the RA entails a potential risk of catheter entrapment manipulating the catheter at the RA. If catheter entrapment occurs during the procedure, the entrapped catheter may be removed via a large-bore sheath using a snare catheter.
Modeling roadway temperatures for wildfire evacuation and assessment of pavement damage
Authors: Barzegar Mohammadreza , Wen Haifang 
Published: July 2023

AbstractWildfires can cause enormous loss of assets and human lives, and thus, timely evacuation from the wildfire zone is critical to saving lives. Understanding evacuation route conditions, including traffic and temperature data, is imperative for safe evacuation. This study modeled the temperature conditions of an evacuation roadway within a wildfire zone, using the 2018 Camp Fire in Butte County, California as an experiment. The numerical modeling program, FlamMap, was used in this study to analyze the temperature conditions of an evacuation route. The results show that the temperature of the evacuation roadway increased quickly in a matter of a few hours. The change of roadways temperatures ranged from 3°C to 297°C within five hours after ignition, depending on the specific location along the roadway and other conditions. The results from this study can be used to understand temperature conditions for effective evacuation and to estimate roadway damage as well.