bluuebunny/crossref_metadata_2025_split huggingface dataset:
AXIN2‐related oligodontia‐colorectal cancer syndrome with cleft palate as a possible new feature
Authors: Roht Laura , Hyldebrandt Hanne K. , Stormorken Astrid T. , Nordgarden Hilde , Sijmons Rolf H. , Bos Dennis K. , Riegert‐Johnson Douglas , Mantia‐Macklin Sarah , Ilves Pilvi , Muru Kai , Wojcik Monica H. , Kahre Tiina , Õunap Katrin 
Published: March 2023

AbstractBackgroundPathogenic variants in AXIN2 have been associated with tooth agenesis, colon polyps, and colon cancer. Given the rare nature of this phenotype, we set out to collect additional genotypic and phenotypic information.MethodsData were collected via a structured questionnaire. Sequencing was performed in these patients mostly due to diagnostic purpose. A little more than half of the AXIN2 variant carriers were identified by NGS; other six were family members.ResultsHere, we report 13 individuals with a heterozygous AXIN2 pathogenic/likely pathogenic variant who have a variable expression of oligodontia‐colorectal cancer syndrome (OMIM 608615) or oligodontia‐cancer predisposition syndrome (ORPHA 300576). Three individuals from one family also had cleft palate, which might represent a new clinical feature of AXIN2 phenotype, also given the fact that AXIN2 polymorphisms have been found in association with oral clefting in population studies. AXIN2 has already been added to multigene cancer panel tests; further research should be conducted to determine whether it should be added to cleft lip/palate multigene panels.ConclusionMore clarity about oligodontia‐colorectal cancer syndrome, about the variable expression, and associated cancer risks is needed to improve clinical management and to establish guidelines for surveillance. We collected information about the surveillance that was advised, which might support clinical management of these patients.
What guides guided self‐help? Recognising the role of formulation in Low Intensity CBT
Authors: Cromarty Paul , Gallagher Dominic 
Published: March 2023

AbstractThe emphasis on formulation, as a lynchpin and driver of cognitive behavior therapy (CBT) has received increasing attention, consensus of value among practitioners and emerging studies of efficacy. The terms, “formulation” and “conceptualisation” are frequently used interchangeably in literature which is exclusively focussed on high intensity CBT. In contrast, little has been included on the value or recognising the existence of formulation, for Low Intensity CBT practitioners providing guided self‐help. This may contribute to misconceptions that Low Intensity CBT is a mechanistic set of techniques. Links to CBT competencies, curricula, role of supervision and roots in UK Behavioral Nurse Therapist training, are made to highlight the implicit presence of Low Intensity formulation. We suggest a definition of formulation, as a key Low Intensity competency. This uses an individualised treatment rationale and problem statement, derived from a structured functional assessment. We propose, explicitly emphasising these, constitutes Low Intensity case formulation, which guides intervention. This is a refocus of existing practice, not introduction of completely new elements. A brief illustration of “how to” deliver formulation‐driven Low Intensity CBT is made. This promotes a concise “within‐session” and “between‐session” thread. This adjustment, on what guides Low Intensity interventions, is relevant to practitioners, supervisors and trainers in promoting Low Intensity best practice. This argues that recognising the value of formulation assists in optimising skills development, client outcomes and satisfaction with Low Intensity CBT. Cautions with the approach and the need for further research are noted.
Facial nerve vulnerability in spinal muscular atrophy and motor unit number index of the orbicularis oculi muscle
Authors: Barp Andrea , Carraro Elena , Salmin Francesca , Lizio Andrea , Cheli Marta , Sansone Valeria 
Published: March 2023

AbstractIntroduction/AimsMeasures for assessing cranial nerve vulnerability in spinal muscular atrophy (SMA) have not yet been determined. Motor unit number index (MUNIX) studies have shown correlations with disease severity but have been used only in limb muscles. In the present study, we explore facial nerve response, MUNIX, and motor unit size index (MUSIX) of the orbicularis oculi muscle in a cohort of patients with SMA.MethodsFacial nerve response (measured as compound muscle action potential, CMAP), MUNIX, and MUSIX of the orbicularis oculi muscle were cross‐sectionally recorded in patients with SMA and compared to healthy control subjects (HCs). Active maximum mouth opening (aMMO) was also measured at baseline in our SMA cohort.ResultsThirty‐seven patients with SMA (21 SMA II; 16 SMA III) and 27 HCs were recruited. CMAP of the facial nerve and MUNIX of orbicularis oculi proved to be feasible and well tolerated techniques. CMAP amplitude and MUNIX scores were significantly lower in patients with SMA compared to HCs (p < .0001). Both MUNIX and CMAP amplitude were significantly higher in patients with SMA III compared to SMA II. No significant difference emerged comparing CMAP amplitude, MUNIX and MUSIX scores between those with different functional status or nusinersen treatment.DiscussionOur results provide neurophysiological evidence of facial nerve and muscle involvement in patients with SMA. CMAP of the facial nerve and MUNIX of orbicularis oculi showed high accuracy in discriminating between the various subtypes of SMA and in quantifying the motor unit loss of the facial nerve.
Health literacy correlates with abbreviated full‐scale IQ in adolescent and young adults with sickle cell disease
Authors: Bhatt Nidhi , Calhoun Cecelia , Longoria Jennifer , Nwosu Chinonyelum , Howell Kristen E. , Varughese Taniya , Kang Guolian , Jacola Lisa M. , Hankins Jane S. , King Allison 
Published: March 2023

AbstractIntroductionSickle cell disease (SCD) is a chronic condition with progressive neurocognitive deficits. Health literacy (HL) is essential during adolescence and young adulthood, as the transition to adult care requires healthcare decisions. HL is known to be low in SCD; however, relation between general cognitive ability and HL has not been investigated.MethodsThis cross‐sectional study included adolescent and yound adults (AYAs) with SCD from two institutions. Logistic regression measured the association between HL, measured by the Newest Vital Sign tool, and general cognitive ability, measured with abbreviated full‐scale intelligence quotient (FSIQ) on the Wechsler Abbreviated Scale of Intelligence.ResultsOur cohort contained 93 participants at two sites: 47 (51%) at Memphis, TN and 46 (49%) at St. Louis, MO, ranging from ages 15–45 years (mean = 21 years) and with a majority (70%) possessing a high school education or greater. Only 40/93 participants (43%) had adequate HL. Lower abbreviated FSIQ (p < .0001) and younger age at assessment (p = .0003) were associated with inadequate HL. For every standard score point increase in abbreviated FSIQ, the odds of having adequate HL compared to limited or possibly limited HL increase by 1.142 (95% confidence interval [CI]: 1.019–1.322) and 1.116 (95% CI: 1.045–1.209), respectively, after adjusting for age, institution, income, and educational attainment.ConclusionsUnderstanding and addressing HL is imperative in improving self‐management and health outcomes. Among AYA with SCD, low HL was prevalent and influenced by abbreviated FSIQ. Routine screening for neurocognitive deficits and HL should be performed to guide development of interventions to adapt to the HL of AYA with SCD.
Long‐term performance and reliability of silicon heterojunction solar modules
Authors: Arriaga Arruti Olatz , Virtuani Alessandro , Ballif Christophe 
Published: March 2023

AbstractThe high‐efficiency silicon heterojunction (SHJ) technology is now perceived mature enough to enter the Giga‐Watt manufacturing scale with several players around the globe. The growth of the SHJ technology requires confidence from manufacturers, investors, and system developers about its reliability and long‐term performance. In this work, we perform a literature survey collecting performance data (and performance loss rates [PLRs]) published for SHJ modules. Publications on this specific subject are still limited; however, enough available data exist to drive some preliminary conclusions. Despite a long list of caveats specific to this type of meta‐analysis, when considering all published datasets, we obtain for SHJ modules median and average PLR values of 0.56%/year and 0.70%/year, respectively. These numbers are in line with PLR generally reported for field‐deployed crystalline silicon (c‐Si modules). We then apply a filtering procedure to distinguish what we perceive to be high‐accuracy datasets from less accurate ones. This methodology is understandably arbitrary, but it helps increasing the robustness of the present analysis. Our refined analysis leads us to slightly higher PLR for SHJ modules: 0.80%/year and 0.83%/year for median and average values, respectively. These values fall between previously reported PLR of c‐Si and thin‐film modules. Additionally, we observe some mild correlations between the PLRs and the climatic conditions of the installation sites, even if we need to stress that for each climate, we find a large variability, including a PLR value as low as 0.29%/year. We complement the survey with information about the main failure modes reported in the literature for this technology and an analysis of the limits and caveats for this type of study. The most significant one is that the reported numbers refer—for the vast majority—to modules from just one manufacturer (i.e., the first company manufacturing and commercializing the SHJ technology). We finally point out that a deep understanding of the potential weaknesses of the technology—collected over the years—has led to several improvements in terms of reliability. A careful material choice and module design may in fact allow the SHJ technology targeting extended service lifetimes of 35+ years.
Does ICT diffusion reduce poverty? Evidence from SAARC countries
Authors: Verma Anushka , Giri Arun K. , Debata Byomakesh 
Published: March 2023

AbstractThe present study aims to explore the relationship between ICT diffusion and poverty reduction in SAARC countries using panel data from 2005 to 2020. This study uses econometric techniques robust to cross‐sectional dependence (CSD) including Pesaran's CSD tests; second‐generation unit root test; Pedroni, Kao, Westerlund cointegration tests; CS‐ARDL, Driscoll‐Kraay (DK) standard error approach; and D&H causality test. The investigation is based on the ICT diffusion index constructed using principal component analysis (PCA). The study's major finding shows that ICT diffusion reduces poverty both in the long and short run, indicating the favorable impact of ICT on the development process in SAARC countries. Further, economic growth, financial development, and remittances all serve to minimize the poverty level. The causality test reveals bidirectional causation between ICT diffusion and poverty reduction. The study highlights the crucial role of ICT diffusion and selected economic variables in reducing poverty. The findings of the present research shall benefit policymakers to formulate appropriate policies and programs to improve the well‐being of people and enhance macroeconomic performance, which impacts both the societal and environmental development of a country.
The effects of spirulina intake on clinical and metabolic parameters in Alzheimer's disease: A randomized, double‐blind, controlled trial
Authors: Tamtaji Omid Reza , Heidari‐soureshjani Reza , Asemi Zatollah , Kouchaki Ebrahim 
Published: March 2023

AbstractThe current study aimed to determine the effects of spirulina intake on cognitive function and metabolic status among patients with Alzheimer's disease (AD). This randomized, double‐blind, controlled clinical trial was done among 60 subjects with AD. Patients were randomly assigned to receive either 500 mg/day spirulina or a placebo (each n = 30) twice a day for 12 weeks. Mini‐mental state examination score (MMSE) was recorded in all patients before and after intervention. Blood samples were obtained at baseline and after 12 weeks’ intervention to determine metabolic markers. Compared with placebo, spirulina intake resulted in a significant improvement in MMSE score (spirulina group: +0.30 ± 0.99 vs. Placebo group: −0.38 ± 1.06, respectively, p = 0.01). In addition, spirulina intake decreased high‐sensitivity C‐reactive protein (hs‐CRP) (spirulina group: −0.17 ± 0.29 vs. Placebo group: +0.05 ± 0.27 mg/L, p = 0.006), fasting glucose (spirulina group: −4.56 ± 7.93 vs. Placebo group: +0.80 ± 2.95 mg/dL, p = 0.002), insulin (spirulina group: −0.37 ± 0.62 vs. Placebo group: +0.12 ± 0.40 μIU/mL, p = 0.001) and insulin resistance (spirulina group: −0.08 ± 0.13 vs. Placebo group: 0.03 ± 0.08, p = 0.001), and increased insulin sensitivity (spirulina group: +0.003 ± 0.005 vs. Placebo group: −0.001 ± 0.003, p = 0.003) compared with the placebo. Overall, our study showed that spirulina intake for 12 weeks in patients with AD improved cognitive function, glucose homeostasis parameters, and hs‐CRP levels.
Biochar, manure and superabsorbent improve the physical quality of saline‐sodic soil under greenhouse conditions
Authors: Fouladidorhani Maliheh , Shayannejad Mohammad , Mosaddeghi Mohammad Reza , Shariatmadari Hossein , Arthur Emmanuel 
Published: March 2023

AbstractThe increasing salinization and sodification of soils in drylands and irrigated systems present a great challenge to agricultural production and threaten soil quality. There is an urgent need to investigate potential strategies to remediate soils that are threatened by these processes. To address this challenge, the current study investigated the effects of organic and inorganic amendments and leaching fraction (LF) on physical and hydraulic properties including the soil water characteristic curve (SWCC), Dexter's index of soil physical quality (SDexter), bulk density (BD), saturated hydraulic conductivity (Ks), and water repellency index (RI) in a saline‐sodic soil. A greenhouse experiment was carried out in a completely randomized design with two factors (amendment and LF). The amendments included superabsorbent (1%) (SA), zeolite modified with CaCl2(2%) (ZC), biochar (2%) (B), biochar + manure (2%) (BM), and a control (no amendment) (CT), and combined with two levels of LF (15% and 30%). Spring wheat (Triticum aestivum, ‘UG99’) was planted in the soil columns. After harvest, undisturbed samples were collected from each column for the determination of soil properties. The results demonstrated the effectiveness of the SA and organic amendments to enhanceKsand decrease BD. There was a significant (p < 0.01) positive effect of the amendments, particularly biochar and superabsorbent on the soil pore size distribution. The SA amendment significantly increased soil water retention, especially at high matric potentials (i.e., 0 to −80 hPa). The effect of organic amendments on the SWCC was in between those of the ZC and SA amendments. The SA and BM treatments were sub‐critically water repellent, while the ZC treatment increased soil water repellency. The SA, B, and BM15 treatments exhibited very good physical quality (SDexter > 0.05). The relative air and moisture capacities were optimal in the B‐amended soil at field capacity (matric potential –330 hPa). We conclude that B, BM, and SA are suitable amendments for reducing the adverse effects of salinity and sodicity via an improvement in soil physical quality.
When do close ties to service intermediaries lead to entrepreneurial improvisation?
Authors: Luo Xiaowei Rose , Mai Yiyuan , Ye Zhuxin 
Published: March 2023

AbstractResearch SummaryPrior research suggests that the founders of new ventures leverage their existing networks, such as close ties to service intermediaries, as a source of entrepreneurial improvisation. We draw attention to the trade‐off between the benefits (trust and access to information and resources) and the costs (dependence and a constrained growth opportunity), and identify two contingency conditions for using close ties to improvise: a shorter time horizon for failure and less turbulent market. Analysis based on an original survey of founding teams in China in the period 2015–2016 provides empirical support for our argument. Our study contributes to research on entrepreneurial improvisation by exposing the tension involved in working with close contacts and enriches the literature on the role of service intermediaries.Managerial SummaryWe consider the trade‐off involved in relying on close ties to service intermediaries and identify conditions under which founders of new ventures are more likely to rely on such intermediaries to improvise. Although close ties can foster trust and facilitate access to a wide range of information and resources, they may also foster a dependency that could constrain future growth. A survey of founding teams in China from 2015 to 2016 reveals that founding teams leveraged such ties when given a shorter time horizon within which to succeed or fail, and when the market was less turbulent. Our findings can help entrepreneurs decide when to resort to close‐tied intermediaries as an aid to improvisation, that is, when the benefits of doing so outweigh the costs.
PEGylated Manganese–Zinc Ferrite Nanocrystals Combined with Intratumoral Implantation of Micromagnets Enabled Synergetic Prostate Cancer Therapy via Ferroptotic and Immunogenic Cell Death
Authors: Wang Hui , Guan Yu , Li Chun , Chen Jia , Yue Shaoyu , Qian Jieying , Dai Bangshun , Jiang Changqin , Wen Chenghao , Wen Longping , Liang Chaozhao , Zhang Yunjiao , Zhang Li 
Published: March 2023

AbstractTherapeutic efficacy for prostate cancer is highly restricted by insufficient drug accumulation and the resistance to apoptosis and immunogenic cell death (ICD). Although enhanced permeability and retention (EPR) effect of magnetic nanomaterials could benefit from external magnetic field, it falls off rapidly with increased distance from magnet surface. Considering the deep location of prostate in pelvis, the improvement of EPR effect by external magnetic field is limited. In addition, apoptosis resistance and cGAS‐STING pathway inhibition‐related immunotherapy resistance are major obstacles to conventional therapy. Herein, the magnetic PEGylated manganese‐zinc ferrite nanocrystals (PMZFNs) are designed. Instead of providing external magnet, micromagnets into tumor tissues are intratumorally implanted to actively attract and retain intravenously‐injected PMZFNs. As a result, PMZFNs accumulate in prostate cancer with high efficacy, depending on the established internal magnetic field, which subsequently elicit potent ferroptosis and the activation of cGAS‐STING pathway. Ferroptosis not only directly suppresses prostate cancer but also triggers burst release of cancer‐associated antigens and consequently initiates ICD against prostate cancer, where activated cGAS‐STING pathway further amplifies the efficacy of ICD by generating interferon‐β. Collectively, the intratumorally implanted micromagnets confer a durable EPR effect of PMZFNs, which eventually achieve the synergetic tumoricidal efficacy with negligible systemic toxicity.
Spark Discharge Doping—Achieving Unprecedented Control over Aggregate Fraction and Backbone Ordering in Poly(3‐hexylthiophene) Solutions
Authors: Eller Fabian , Wenzel Felix A. , Hildner Richard , Havenith Remco W. A. , Herzig Eva M. 
Published: March 2023

AbstractThe properties of semiconducting polymers are strongly influenced by their aggregation behavior, that is, their aggregate fraction and backbone planarity. However, tuning these properties, particularly the backbone planarity, is challenging. This work introduces a novel solution treatment to precisely control the aggregation of semiconducting polymers, namely current‐induced doping (CID). It utilizes spark discharges between two electrodes immersed in a polymer solution to create strong electrical currents resulting in temporary doping of the polymer. Rapid doping‐induced aggregation occurs upon every treatment step for the semiconducting model‐polymer poly(3‐hexylthiophene). Therefore, the aggregate fraction in solution can be precisely tuned up to a maximum value determined by the solubility of the doped state. A qualitative model for the dependences of the achievable aggregate fraction on the CID treatment strength and various solution parameters is presented. Moreover, the CID treatment can yield an extraordinarily high quality of backbone order and planarization, expressed in UV–vis absorption spectroscopy and differential scanning calorimetry measurements. Depending on the selected parameters, an arbitrarily lower backbone order can be chosen using the CID treatment, allowing for maximum control of aggregation. This method may become an elegant pathway to finely tune aggregation and solid‐state morphology for thin‐films of semiconducting polymers.
CLAACS‐IOD: Certificate‐embedded lightweight authentication and access control scheme for Internet of Drones
Authors: Sadhukhan Dipanwita , Ray Sangram , Dasgupta Mou , Rodrigues Joel J. P. C. 
Published: March 2023

AbstractIn recent era, the unmanned aerial vehicles (UAVs) commonly known as drones has emerged as a one of the most significant and promising tools which has demonstrated its wide range of implementations variating from commercial domain to the field of defense due to its distinct capabilities such as inspection, surveillance, precision and so forth. Internet connected drones provides a propitious trend that boosts the flying safety, and service qualities of the UAVs where numerous low‐altitude drones winged in different flying regions for executing a precise task such as gathering the real‐time information from the unuttered environment to be interpretated by users. Nevertheless, the open‐access insecure communications in hostile environment, the issues like safety and confidentiality threats, various security concerns such as the leakage of flying courses, identities, position, and gathered data by the drones are upstretched. To address these security concerns, access control mechanism provides a potential service in terms of authentication and key agreement for securing the communication between the individual drones within their respective flying regions. This article introduces a robust, efficient, lightweight, and privacy preserving ECC integrated access control approach by employing digital certificate with considering the high dynamicity and mobility of the drones. The designing of this proposed approach, that is, CLAASC‐IoD is influenced by the aim of inter‐drone and drone‐to‐ground station communication in the IoD paradigm. The detailed analysis of security using probabilistic random oracle model as well as simulation using well‐accepted security verification tool AVISPA and comparative performance evaluation supports the claim of robustness, effectiveness, and proficiency.
Developing sustainable transnational collaboration in the post-armed conflict areas of the Democratic Republic of the Congo, Rwanda and Uganda
Authors: Deogratias Musoke 
Published: March 2023

AbstractThe Virunga National Park (Parc National des Virunga) is situated in the Albertine Rift Valley in the eastern part of the Democratic Republic of the Congo (DRC), the south-western part of Uganda near Lake George and Lake Edward, and the north-western part of Ruhengeri in Rwanda. It was created in 1925 and is one of the first protected areas in Africa, enlisted as a UNESCO World Heritage property in 1979. The park is host to one of the world’s most famous populations of mountain gorillas but it has been hit by rising instabilities, an influx of refugees, poaching, smuggling activities and violence caused by various rebel groups, such as the Mai-Mai militia and other smugglers, including the recent killing of 12 rangers and the abduction of 2 British tourists in 2018.