bluuebunny/crossref_metadata_2025_split huggingface dataset:
Bernard–Soulier Syndrome: A Review of Epidemiology, Molecular Pathology, Clinical Features, Laboratory Diagnosis, and Therapeutic Management
Authors: Kaya Zühre 
Published: August 2024

AbstractBernard–Soulier syndrome (BSS) is an inherited platelet function disorder caused by mutations in the genes that encode the glycoprotein (GP) Ibα and GPIbβ subunits, as well as the GPIX subunit in the GPIbIX complex, which is located on the platelet surface and has roles in platelet adhesion and activation. Patients with autosomal recessively inherited biallelic BSS have a homozygous or compound heterozygous expression in the GPIbα, GPIbβ, and GPIX subunits of the GPIbIX complex. Patients with autosomal dominantly inherited monoallelic BSS have a heterozygous expression in only the GPIbα and GPIbβ subunits of the GPIbIX complex. To date, no BSS mutations in the GP5 gene have been reported. Patients with biallelic form are usually diagnosed at a young age, typically with mucocutaneous bleeding, whereas monoallelic forms are generally identified later in life and are frequently misdiagnosed with immune thrombocytopenic purpura (ITP). In biallelic BSS, giant platelets in the peripheral blood smear, absence of ristocetin-induced platelet aggregation (RIPA) using light transmission aggregometry (LTA), and complete loss of GPIbIX complex in flow cytometry are observed, whereas in monoallelic forms, genetic diagnosis is recommended due to the presence of large platelets in the peripheral blood smear, decreased or normal RIPA response in LTA, and partial loss or normal GPIbIX complex in flow cytometry. Platelet transfusion is the main therapy but recombinant factor VIIa is advised in alloimmunized patients, and allogeneic stem cell transplantation is suggested in refractory cases. Antifibrinolytics and oral contraceptives are utilized as supplementary treatments. Finally, differentiation from ITP is critical due to differences in management. Thus, BSS should be kept in mind in the presence of individuals with chronic persistent thrombocytopenia, positive family history, unresponsive ITP treatment, macrothrombocytopenia, and absence of RIPA response.
Colorectal Carcinoma: Is There Any Correlation between Her2/neu Expression, Ki-67 Score, and Tumor Budding and Clinicopathological Parameters?—A Prospective Institution-Based Study
Authors: Khan Susnata , Dasgupta Senjuti , Deb Asit Ranjan 
Published: August 2024

Objectives Colorectal cancer is one of the most frequent cancers worldwide and is still a major cause of cancer mortality. Her2/neu, Ki67 score, and tumor budding are independent prognostic factors in colorectal carcinomas. The objectives of the study were to evaluate Her2/neu expression, Ki67 score, and tumor budding index at invasive margin in colorectal carcinoma and find out their possible correlations with different clinicopathological factors.
Materials and Methods An institution-based observational cross-sectional study was conducted for 18 months. Forty-one patients with histologically proven diagnosis of colorectal carcinoma were included. Histopathological and immunohistochemical analyses (Her2/neu and Ki-67) of each case were done.
Statistical Analysis Data analysis was done using the SPSS software.
Results A significant correlation was found between tumor budding status and pathological T stage, Dukes' and American Joint Committee on Cancer stages, and between tumor-infiltrating lymphocytes status and Ki-67 expression status (p < 0.05).
Conclusion The prognostic importance of tumor budding in colorectal carcinoma is very clear. Considering the small sample size of the present study, the prognostic values of Her2/neu and Ki-67 are required to be explored further in larger cohorts in the future.
Treatment Outcomes and Toxicity Profiles with PORTEC-3 Trial Regimen in South Asian Cohort of High-Risk Endometrial Cancer Patients: A Single-Center Ambispective Analysis
Authors: Verma Parth , Rajan Rajasree , Sasidharan Ajay , Kalavagunta Sruthi , Dutta Debnarayan , Kunheri Beena , Kunnath Neethu , Bhati Priya , Rajanbabu Anupama , Nair Indu , Puthenveedu Rakesh Moolayil , Haridas Nikhil , Jose Wesley , Pavithran Keechilat 
Published: August 2024

Objectives Adjuvant chemoradiation followed by chemotherapy is the current standard of care in high-risk endometrial cancer after the PORTEC-3 trial. There is a lack of data on this treatment regimen in the South Asian patient cohort. The present study aims to assess toxicity profiles and outcomes in this cohort of patients.
Materials and Methods High-risk endometrial cancer patients planned for adjuvant chemoradiation followed by chemotherapy were included. Toxicity was graded using the Radiation Therapy Oncology Group and Common Terminology Criteria for Adverse Events criteria. Disease-free survival (DFS) and overall survival (OS) were estimated using the Kaplan–Meier method. Survival curves were compared using the log-rank test. Cox regression analysis was done to find out the predictors of DFS.
Results This study included 58 patients treated from October 2016 to August 2022. Median age was 61 years (interquartile range [IQR] 56–66), with Fédération Internationale de Gynécologie et d'Obstétrique Stages I = 26 (44.8%), II = 5 (8.6%), and III = 27 (46.6%). p53 positivity was seen in 38 (65.5%) patients. Intensity-modulated radiotherapy was used in 44 (79.3%) patients. There was no treatment discontinuation during chemoradiation. Acute Grade 2 and above toxicity during chemoradiation were diarrhea in 10 (17.2%) and hematological in 2 (3.4%). For the planned adjuvant chemotherapy in 55 patients, 51 (92.7%) completed four cycles. Grade 2 or above neuropathy was seen in 11 (20%), with 5 (9%) having persisting neuropathy at 1-year follow-up. At a median follow-up of 31 months, 15 (25.8%) patients recurred; distant = 13 and isolated para-aortic = 2. The median time to recurrence was 16 months (IQR 12–22), with 80% (12 out of 15) of recurrence within the first 2 years of follow-up. The actuarial 5-year DFS and OS were 63.8 and 76.5%, respectively. In univariate analysis, p53 positivity and lymphovascular space invasion were predictors for DFS, with p-values 0.031 and 0.027, respectively. There was no significant predictor identified in multivariate analysis.
Conclusion There is good tolerance and compliance to adjuvant chemoradiation and chemotherapy in this South Asian cohort of patients with high-risk endometrial cancer, with no toxicity-related treatment breaks during radiation. The majority of the recurrences were seen at distant sites and within the first 2 years of follow-up. These findings are in line with the outcomes of the PORTEC-3 trial.
Development and acceptability of a digital tool for promoting syphilis testing in Australian general practice: qualitative study using the Theoretical Framework of Acceptability
Authors: Hunter Barbara , Hocking Jane S. , Manski-Nankervis Jo-Anne , Jung Jun , Wigan Rebecca , Chen Marcus Y. , Boyle Douglas , Chidgey Christine , O’Donnell Heather , Goller Jane L. 
Published: August 2024

Background In Australia, syphilis notifications increased 2.5-fold during 2013–2022 and 83 congenital syphilis cases were reported. Timely diagnosis and management are crucial. We developed a tool to promote syphilis testing into our existing ‘Future Health Today’ (FHT) software and explored its acceptability in general practice. Methods Our tool (FHT-syphilis) scans electronic medical record data to identify and prompt testing for pregnant women, and, people recently tested for sexually transmissible infection (STI) or HIV, but not syphilis. It links to relevant guidelines and patient resources. We implemented FHT-syphilis in 52 general practices using FHT for other conditions and interviewed practice clinicians (n = 9) to explore it’s acceptability. Data were analysed deductively guided by the Theoretical Framework of Acceptability. Results Interviewees considered syphilis an important infection to focus on and broadly viewed FHT-syphilis as acceptable for identifying patients and giving clinicians authority to discuss syphilis testing. Time constraints and unrelated reasons for a patient’s visit were barriers to initiating syphilis testing discussions. Australian STI guidelines were considered appropriate to link to. Some interviewees considered prompts should be based on sexual behaviour, however this is not well captured in the electonic medical record. Two interviewees were alerted to updated Australian STI guidelines via their interaction with FHT-syphilis and expanded their syphilis testing practices. Expertise to initiate discussions about syphilis and risk was deemed important. Conclusions A digital tool for prompting syphilis testing was acceptable to clinicians already using FHT. Linkage to STI guidelines alerted some end-users to updated guidelines, informing STI testing practices.
The impact of an online doctoral writing group
Authors: García Marrugo Alexandra I. , Anson Daniel W. J. 
Published: August 2024

Abstract
Challenges with writing have been identified as a key factor in the timely completion of PhD programs. Doctoral
writing groups, where students provide peer feedback on drafts, are increasingly being implemented as a potential solution to
support and develop student writing. This paper presents an exploration of the impact of an online doctoral writing group program
at a large Australian university. The group supports students in writing a research article for publication in an academic journal
and includes both facilitator-led instruction and peer feedback. We explored the group’s impact on the participants’ writing
ability, feedback literacy, and general well-being. Data were drawn from two doctoral writing groups and derived from several
sources: session transcripts, focus groups, anonymous surveys, student drafts, and published samples of students’ academic journal
articles. We found that instruction was key to demystifying the writing process and developing feedback literacy. Furthermore, the
online delivery mode provided access to support for cohorts traditionally excluded from these opportunities, as well as a safe
space that fostered a sense of well-being. This approach suggests a sustainable and effective model for implementation in graduate
programs.
The emergence of vowel harmony in Armenian dialects
Authors: Hopkins Elizabeth , Vaux Bert 
Published: August 2024

AbstractWe find vowel harmony systems in many non-standard varieties of modern Armenian. It has been speculated that these may have acquired vowel harmony due to contact with Turkic varieties (Scala 2018). On the basis of an exploration of the synchrony and typology of Armenian vowel harmony, consideration of historical changes that could have caused harmony to develop, and evaluation of new data bearing on the origins of backness and rounding harmony in Oghuz, we propose that the vowel harmony systems of the modern Armenian dialects show evidence of having been influenced by Turkish, but the numerous differences between Armenian and Turkish vowel harmony point against a straightforward copying of the Turkish phonological system. We theorize that vowel harmony in Armenian arose due to a combination of language-internal and ‑external factors: vowel shifts in some Armenian dialects, alongside universal analytic and channel biases, provided the necessary preconditions for the development of vowel harmony by the 11th century AD, prior to the arrival of Turkic speakers in the Armenian homeland. Extensive contact with Turkic vowel systems may then have encouraged the phonologization of this assimilation process, but in strikingly different ways than are found in Turkic languages.
Distributions of quasars as an indicator of the homogeneity or inhomogeneity in the Universe
Authors: Hovhannisyan M.A. , Mkhitaryan S.A. , Mahtessian L.A. , Mnatsakanyan A.R. , Vardanyan J.H. , Minasyan A.M. , Mahtessian A.P. 
Published: August 2024

Abstract
Quasars are perhaps the brightest extragalactic sources and thanks to their brightness
they are visible at great distances. By studying the distribution of quasars in the Universe, it
is possible to determine whether there are large-scale inhomogeneities in the Universe or not. If
the distribution of quasars at large distances is not uniform, the same can be said also about the
Universe. At small distances, quasars either do not exist, or their number is very
small. Therefore, the distribution of quasars at small distances is very conditional, and we
cannot draw any conclusions about the homogeneity of the Universe there based on the distribution
of quasars. Therefore, the distribution of quasars allows us to determine the distribution of
matter at large distances, which we use to detect large-scale inhomogeneities in the Universe.
Rooting meta-ecosystems with reciprocal lateral carbon and nitrogen flows in a Yangtze coastal marsh
Authors: Gao Yu , Zhao Bin , Saintilan Neil , Chen Jiquan , Wu Wanben , Wen Li , Zhao Feng , Zhang Tao , Geng Zhi , Yang Gang , Song Chao , Zhuang Ping 
Published: August 2024

Abstract
The dynamics of lateral nutrient fluxes through hydrological movements are crucial for understanding ecological functions related to the flow of energy, materials, and organisms across various spatiotemporal scales. To investigate the connectivity of multiple spatial flow processes, we conducted a one-year field study to measure lateral hydrologic carbon (C) and nitrogen (N) fluxes across the continental shelf in the Yangtze estuary. We observed a significant correlation between the differences in remote sensing-based estimates of gross primary production (GPP) (ΔGPPMODIS) and the differences in eddy covariance (EC) tower-based GPP (ΔGPPEC) at both high-elevation and low-elevation sites. Our findings indicate that the saltmarsh acts as a net source of dissolved total C while serving as a net sink for dissolved total N. Furthermore, there was a significant correlation in the total dissolved stoichiometry of the C/N ratio between imports from and exports to adjacent aquatic systems. These findings highlight the importance of integrating ecological stoichiometric principles to improve our understanding of the complex relationships among physical, chemical, and ecological processes, particularly within the context of the meta-ecosystem framework. Additionally, when reciprocal hydrological lateral C and N flows are considered, a single ecosystem can function as both a source and sink within the meta-ecosystem framework.
Global sustainability scenarios lead to regionally different outcomes for terrestrial biodiversity
Authors: Ambrósio Geanderson , Doelman Jonathan C , Schipper Aafke M , Stehfest Elke , van Vuuren Detlef 
Published: August 2024

Abstract
Mitigating climate change (CC) and reversing biodiversity decline are urgent and interconnected global priorities. Strategies to address both crises must consider the relationships, synergies and trade-offs between key response measures, including sustainable production and consumption patterns, protected areas (PAs) and climate mitigation policy (CP). In this paper, we review a large set of scenarios (n = 96) from the Integrated Model to Assess the Global Environment (IMAGE) describing future development of land use, greenhouse gas emissions and their impact on CC and biodiversity. We calculate the global mean temperature increase (GMTI) and the Mean Species Abundance (MSA) of plants, a metric indicative of local terrestrial biodiversity intactness. The set includes scenarios with and without specific CP to address CC, PA for biodiversity and demand and supply sustainability measures such as increased energy efficiency and reduced meat consumption. Our findings indicate that scenarios with integrated measures can prevent biodiversity loss at the global scale, yet with clear regional differences. By 2050, 15 out of 30 (50%) scenarios with at least 30% of global land as PAs show positive MSA changes in grasslands and tropical non-forests (Grass & TnF), but only 1 (3%) does so in tropical forests (TF). We demonstrate that pasture and food/feed crops are the main drivers of MSA loss in Grass & TnF and that scenarios with high levels of PAs prevent land conversion and increase biodiversity. By 2100, 28 out of 46 (60%) scenarios with mitigation measures to restrict CC to 2 °C or less in 2100 result in positive MSA changes in TF, but only 13 (28%) do so in Grass & TnF, reflecting the larger impacts of land use change in the latter region. These results underscore the importance of time and regionally-tailored approaches to address the biodiversity and CC crises.
Shannon entropy in quasiparticle states of quantum chains
Authors: Ye Wentao , Zhang Jiaju 
Published: August 2024

Abstract
We investigate the Shannon entropy of the total system and its subsystems, as well as the subsystem Shannon mutual information, in quasiparticle excited states of free bosonic and fermionic chains and the ferromagnetic phase of the spin-1/2 XXX chain. For single-particle and double-particle states, we derive various analytical formulas for free bosonic and fermionic chains in the scaling limit. These formulas are also applicable to certain magnon excited states in the XXX chain in the scaling limit. We also calculate numerically the Shannon entropy and mutual information for triple-particle and quadruple-particle states in bosonic, fermionic, and XXX chains. We discover that Shannon entropy, unlike entanglement entropy, typically does not separate for quasiparticles with large momentum differences. Moreover, in the limit of large momentum difference, we obtain universal quantum bosonic and fermionic results that are generally distinct and cannot be explained by a semiclassical picture.
Modes of the Sakai-Sugimoto soliton
Authors: Amano Markus A G , Gudnason Sven Bjarke 
Published: August 2024

Abstract
The instanton in the Sakai-Sugimoto model corresponds to the Skyrmion on the holographic boundary—which is asymptotically flat—and is fundamentally different from the flat Minkowski space Yang–Mills instanton. We use the Atiyah–Patodi–Singer index theorem and a series of transformations to show that there are 6k zeromodes—or moduli—in the limit of infinite ‘t Hooft coupling of the Sakai-Sugimoto model. The implications for the low-energy baryons—the Skyrmions—on the holographic boundary, is a scale separation between 2k ‘heavy’ massive modes and

6
k
−
9

‘light’ massive modes for k > 1; the 9 global transformations that correspond to translations, rotations and isorotations remain as zeromodes. For k = 1 there are 2 ‘heavy’ modes and 6 zeromodes due to degeneracy between rotations and isorotations.
Stability examination of non-linear convection flow with partial slip phenomenon in a Riga plate channel
Authors: Kumar Rakesh , Sharma Tanya 
Published: August 2024

Abstract
The present work examines the linear stability of non-linear convected flow inside a Riga plate channel. The channel is filled with hybrid nanoliquid and is under the novel influence of the partial slip phenomenon in the present scenario. The left domain of the channel is supported by the Riga sheet whereas the right part is bounded by a sheet of slippery nature. The stability model for this partial slip mechanism is developed in the form of an eigenvalue problem which is explored via the Chebyshev pseudospectral method in combination with the QZ-algorithm. It is reported that the convection forces in hybrid nanofluid are amplified with Riga magnetic number (Hr) under slip/no-slip assumptions. It is interestingly noted that the flow is destabilized by 11.47

%

with non-linear convection (Nc) when considering no-slip at the right-hand sheet. However, the stability region is enlarged with Nc by 9.53

%

in the presence of slip at the right-hand sheet. The partial-slip (γ) assumption in the channel decelerates the growth rate of disturbances. The increment in

F

e
3

O
4

-nanoparticles over the fixed volume of

C
o
F

e
2

O
4

-nanoparticles hampers the instability of the hybrid nanofluid mixture.
Green synthesis of ZnO nanoparticles using Lantana camara leaf extract for the enhancement of plant growth
Authors: Singh Pushpendra Pratap , Chaturvedi Sarika , Bhatnagar Tripti , Das Sumistha , Debnath Nitai 
Published: August 2024

Abstract
Zinc is an important micronutrient for plants, involved in numerous physiological processes as well as numerous enzymatic and metabolic events. Zinc deficiency results in slowed plant development, higher chlorosis rates, smaller leaves, and fewer tillers, which lengthen the crop maturity period and lowers crop quality. In the present study, zinc oxide nanoparticles (ZnONPs) were synthesized through co-precipitation approach by using Lantana camara plant leaf extract. The synthesized ZnONPs were hexagonal in shape with mean size of around 60 nm. The bactericidal activity of ZnONPs was assessed against three phytopathogenic bacterial strains namely Ralstonia solanacearum, Xanthomonas campestris and Erwinia carotovora through broth dilution method. The MIC50 of ZnONPs was 248.33 μg ml−1, 320 .27 μg ml−1 and 320.95 μg ml−1 against R. solanacearum, X. campestris and E. carotovora respectively. The fungiciadal activity of ZnONPs against three phytopathogenic fungal strains was studied by poison food technique. It was observed that 500 ppm ZnONPs could inhbit 86%, 85% and 55% growth of Alterneria solani, Fusarium oxysporum and Athelia rolfsii respectively. The efficacy of ZnONPs as nano fertilizer was evaluated in Solanum lycopersicum Linn. by foliar spray under laboratory condition and it was observed that in comparison with micron sized ZnO, ZnONP treatment could significantly boost up fresh and dry weight, root and shoot length, chlorophyll, lipid and carbohydrate content of the plants.
Legal Validity and Other Properties of Legal Norms
Authors: Moreso JJ , Ródenas Ángeles 
Published: August 2024

Abstract
In Kelsen’s very influential account, legal validity is the specific mode of existence of legal norms. For this reason, legal validity entails legal applicability and legal bindingness. The chapter intends to show that these notions should be distinguished. It starts with the notion of membership to a legal system: which are the criteria to identify certain norms as belonging to a legal system? Several issues are relevant here, for instance, is the efficacy a necessary condition of membership of legal norms to legal systems? In which sense the authorization of another legal norm N2, also a member to the legal system LS, is a criterion of membership of a legal norm N1 to a legal system LS? And, is this authorization only a formal authorization or a material authorization too? Secondly, the notion of applicability is introduced: in legal practice there are applicable norms that are not members of a legal system, we can think on foreign legal norms, which are not part of the domestic legal system, but applicable in virtue of conflict of laws rules. Finally, the notion of bindingness of legal norms is presented. This notion refers to the question of justified normativity. In which conditions are legal norms apt to provide us reasons for action? Moreover, is legal normativity linked in some sense to robust normativity?