bluuebunny/crossref_metadata_2025_split huggingface dataset:
Deliberate paradigm shift in research in rare neurodevelopmental disorders
Authors: Bain Jennifer M. , Ardalan Adel , Goldman Sylvie 
Published: June 2021

AbstractDiagnosis and management of children with rare neurodevelopmental disorders (RNDDs) are complex. The COVID-19 pandemic has forced us to rethink the research activities critical to improve our understanding and treatment of RNDDs, such as creating large international registries and developing natural history studies. In this communication, we reflect on our latest effort in conducting research remotely while providing support, education and feedback to families affected by a specific RNDD. Specifically, we advocate for a deliberate paradigm shift towards virtual family meetings as ecological platforms to enroll and assess individuals with rare disorders. Herein, we demonstrate that such a shift is crucial to substantially increasing geographical and age range coverage, which are essential for capturing the phenotypic variations in RNDDs. Finally, we call on the community to invest in building integrated technological platforms necessary for effective remote research activities, through standardization, collaboration and training.
CD151 drives cancer progression depending on integrin α3β1 through EGFR signaling in non-small cell lung cancer
Authors: Zhu Jianjie , Cai Tingting , Zhou Jieqi , Du Wenwen , Zeng Yuanyuan , Liu Ting , Fu Yulong , Li Yue , Qian Qian , Yang Xiuwei H. , Li Qinglin , Huang Jian-an , Liu Zeyi 
Published: June 2021

Abstract
Background
Tetraspanins CD151, a transmembrane 4 superfamily protein, has been identified participating in the initiation of a variety of cancers. However, the precise function of CD151 in non-small cell lung cancer (NSCLC) remains unclear. Here, we addressed the pro-tumoral role of CD151 in NSCLC by targeting EGFR/ErbB2 which favors tumor proliferation, migration and invasion.

Methods
First, the mRNA expression levels of CD151 in NSCLC tissues and cell lines were measured by RT-PCR. Meanwhile, CD151 and its associated proteins were analyzed by western blotting. The expression levels of CD151 in NSCLC samples and its paired adjacent lung tissues were then verified by Immunohistochemistry. The protein interactions are evaluated by co-immunoprecipitation. Flow cytometry was applied to cell cycle analysis. CCK-8, EdU Incorporation, and clonogenic assays were used to analyze cell viability. Wound healing, transwell migration, and matrigel invasion assays were utilized to assess the motility of tumor cells. To investigate the role of CD151 in vivo, lung carcinoma xenograft mouse model was applied.

Results
High CD151 expression was identified in NSCLC tissues and cell lines, and its high expression was significantly associated with poor prognosis of NSCLC patients. Further, knockdown of CD151 in vitro inhibited tumor proliferation, migration, and invasion. Besides, inoculation of nude mice with CD151-overexpressing tumor cells exhibited substantial tumor proliferation compared to that in control mice which inoculated with vector-transfected tumor cells. Noteworthy, we found that overexpression of CD151 conferred cell migration and invasion by interacting with integrins. We next sought to demonstrate that CD151 regulated downstream signaling pathways via activation of EGFR/ErbB2 in NSCLC cells. Therefore, we infer that CD151 probably affects the sensitivity of NSCLC in response to anti-cancer drugs.

Conclusions
Based on these results, we demonstrated a new mechanism of CD151-mediated tumor progression by targeting EGFR/ErbB2 signaling pathway, by which CD151 promotes NSCLC proliferation, migration, and invasion, which may considered as a potential target of NSCLC treatment.
Site-specific resolution of enthesitis in patients with axial spondyloarthritis treated with tumor necrosis factor inhibitors
Authors: Nissen Michael J. , Möller Burkhard , Ciurea Adrian , Mueller Ruediger B. , Zueger Patrick , Schulz Martin , Ganz Fabiana , Scherer Almut , Papagiannoulis Eleftherios , Hügle Thomas 
Published: June 2021

Abstract
Background
Enthesitis is a hallmark of spondyloarthritis (SpA) with a substantial impact on quality of life. Reports of treatment effectiveness across individual enthesitis sites in real-world patients with axial SpA (axSpA) are limited. We investigated the evolution of enthesitis following tumor necrosis factor inhibitor (TNFi) initiation in axSpA patients, both cumulatively and at specific axial and peripheral sites.

Methods
AxSpA patients in the Swiss Clinical Quality Management Registry were included if they initiated a TNFi, had an available Maastricht Ankylosing Spondylitis Enthesitis Score, modified to include the plantar fascia (mMASES, 0–15), at start of treatment and after 6 and/or 12 months and ≥12 months follow-up. Logistic regression models were utilized to analyze explanatory variables for enthesitis resolution.

Results
Overall, 1668 TNFi treatment courses (TCs) were included, of which 1117 (67%) had active enthesitis at baseline. Reduction in mMASES at the 6- and 12-month timepoints was experienced in 72% and 70% of TCs, respectively. Enthesitis resolution at 6/12 months occurred in 37.9%/43.0% of all TNFi TCs and 40.7%/50.9% of first TNFi TCs. At 6 months, a significant reduction in the frequency of enthesitis was observed at all sites, except for the Achilles tendon and plantar fascia among first TNFi TCs, while at 12 months, reduction was significant at all sites in both TC groups. Enthesitis resolved in 60.3–77% across anatomical sites, while new incident enthesitis occurred in 4.0–13.5% of all TNFi TCs at 12 months. Both baseline and new-incident enthesitis occurred most frequently at the posterior superior iliac spine and the fifth lumbar spinous process. Younger age and lower mMASES at baseline were predictors of complete enthesitis resolution, while female sex and second- or later-line TNFi treatment were associated with persistence of enthesitis at 12 months.

Conclusion
In real-world axSpA patients treated with a TNFi, enthesitis improved in the majority of patients across all anatomical sites. Significant improvement at the Achilles and plantar fascia entheses was observed only at 12 months. Complete and site-specific enthesitis resolution occurred in ≥40% and ≥60% of TCs evaluated at 12 months, with a low incidence of new site-specific enthesitis.

Trial registration
Not applicable.
Evaluation of the WEAP model in simulating subbasin hydrology in the Central Rift Valley basin, Ethiopia
Authors: Abera Abdi Debele , Ayenew Tenalem 
Published: June 2021

Abstract
Background
The subbasin hydrologic behaviors have been altered by many natural and anthropologic factors such as climate change and land development activities. Model-based assessment can be used to simulate both natural hydrological processes, human-induced effects, and management strategies on water resources. For the Ketar subbasin, the WEAP (water evaluation and planning) hydrologic model was developed that aimed at (1) evaluating the application of the WEAP model in the Ketar subbasin, (2) evaluating the demonstration of the WEAP model using model efficiency evaluation criteria, and (3) simulating hydrological processes of the subbasin using the WEAP model.

Methods
WEAP-based soil moisture method (rainfall-runoff) hydrology routine is comprised of a lumped, one-dimensional, two-layer soil water accounting that uses empirical functions to designate evapotranspiration, surface runoff, interflow, and deep percolation for a sub-unit at root zone. A catchment is considered as the smallest hydrologic response unit. The catchment’s surface hydrological balance is typically estimated by discretizing the catchment into multiple land uses for which water balance is estimated at root zone.

Results
The monthly measured and simulated streamflow statistics showed a positive strong relationship with R2 of 0.82, NSE of 0.80, and IA of 0.95; and with R2 of 0.91, NSE of 0.91, and IA of 0.98 for calibration and validation periods respectively. Similarly, the mean monthly measured and simulated streamflow showed an agreement with R2 of 0.99, NSE of 0.97, and IA of 0.99, and R2 of 0.94, NSE of 0.93, and IA of 0.93 for the periods of calibration and validation respectively.

Conclusion
The model has demonstrated the capability to represent the hydrologic dynamics of the subbasin both at monthly and mean monthly periods. In general, the overall model performance evaluation statistics show a very good agreement between measured and simulated streamflow at the outlet of the subbasin.
The impact of pest risk management measures on trade: the case of apples from France and Chile
Authors: Federica DeMaria , Sophie Drogue , Pasquale Lubello 
Published: June 2021

AbstractIn this article, we investigate how pest risk management protocols may affect trade flows of fresh apples. We apply our analysis to two major players in the international trade of fresh apples: France and Chile. These two countries have been chosen because they are among the world’s leading apple exporters and although they have similar market shares, they differ in terms of destination markets, seasonality, local conditions and export strategy. In order to assess the impact of pest risk management protocols on international trade of apples from France and Chile, we introduce in a gravity equation beside the traditional variables, a score able to measure their complexity. The results are interesting in the sense that even if the score for France and Chile by main trading partners are rather close, we found that French apples exporters would be more impacted by pest risk management protocols than their Chilean counterparts.
Imaging evaluation of continuous extrapleural intercostal nerve block for minimally invasive cardiac surgery: a case report
Authors: Terauchi Misa , Okutani Hiroai , Ishimoto Daisuke , Shimode Noriko , Takao Yumiko , Hirose Munetaka 
Published: June 2021

Abstract
Background
Spinal nerve block is difficult with minimally invasive cardiac surgery (MICS), because of the risk of serious bleeding complications due to full heparinization. Continuous extrapleural intercostal nerve block (CEINB) is a postoperative pain treatment for intercostal thoracotomy, with fewer complications. Here, we report a case in which imaging evaluation of CEINB with contrast medium was conducted to anatomically confirm the spread of local anesthetics after MICS.

Case presentation
A 65-year-old woman with severe mitral regurgitation underwent mitral valve plasty under general anesthesia via right-sided mini-thoracotomy. A CEINB catheter was placed before the incision was closed, without creating a conventional extrapleural pocket. We conducted an imaging evaluation with a contrast medium via the inserted catheter and confirmed sufficient spread around the intercostal nerve area. In addition, postoperative pain was well controlled by the nerve block.

Conclusions
Imaging evaluation of CEINB with contrast medium could increase analgesic quality and decrease complications post-MICS.
Successful airway management with combined use of a McGRATHTM MAC videolaryngoscope and fiberoptic bronchoscope in a patient with congenital tracheal stenosis diagnosed in adulthood
Authors: Hasegawa Shoko , Koda Kenichiro , Uzawa Masashi , Kimura Haruka , Kimura Rie , Kitamura Takayuki 
Published: June 2021

Abstract
Background
Most patients with congenital tracheal stenosis (CTS) develop respiratory symptoms early in life. CTS remaining undiagnosed until adulthood is rare.

Case presentation
A 51-year-old female was scheduled for cardiovascular surgery. She had undergone laparoscopic surgery 3 years earlier and was found to have a difficult airway. Postoperatively, she was diagnosed with CTS. For the current cardiovascular surgery, combined use of a McGRATHTM MAC videolaryngoscope and fiberoptic bronchoscope allowed sufficient visualization of the glottis and trachea, resulting in successful intubation.

Conclusions
CTS patients have a high probability of difficult intubation. Our experience suggests the efficacy of combined use of a videolaryngoscope and fiberoptic bronchoscope for airway management in CTS patients.
Vasculitis damage index in Behçet’s disease
Authors: Elgengehy Fatema T. , Gamal Sherif M. , Sobhy Nesreen , Siam Ibrahem , Soliman Ahmed M. , Elhady Ghada W. , Gheita Tamer A. 
Published: June 2021

Abstract
Background
Vasculitis damage index (VDI) is a validated damage index for systemic vasculitis, and as Behçet’s disease is considered one of systemic vascular disease we aimed to study the relationship of the vasculitis damage index to clinical manifestations and comorbidity in patients with Behçet’s disease (BD) to determine if VDI could be used to assess damage in patients with BD.

Methods
A total of 109 patients with BD were recruited from the Rheumatology Department (outpatient and inpatient clinic), Cairo University Hospitals. All patients were subjected to full history taking, clinical examination, and routine laboratory investigations. Disease activity was assessed by the BD current activity form, and the VDI was calculated in all patients. The relationship of the VDI to the disease clinical manifestations was studied. Mann–Whitney and Kruskal Wallis tests were used to estimate differences in quantitative variables. Spearman correlation test was used to test for correlation between quantitative variables.

Results
In the current study, the VDI ranged from 1 to 10, with a mean of 3.5 ± 1.8. It was significantly associated with total thrombosis (P = 0.022); total neurological manifestations (P = 0.000), especially stroke and cranial nerve affection; uveitis (P = 0.005); avascular necrosis (AVN) (P = 0.015); osteoporosis (P = 0.01); impaired vision (P < 0.0001); cataract (P < 0.0001); and diabetes (P = 0.012). Generally, immunosuppressive treatment was significantly associated with VDI (P = 0.039), especially cyclophosphamide (P < 0.0001), biological agent (P = 0.008), chlorambucil (P = 0.003), and anticoagulant (P = 0.02). VDI was also significantly correlated with age (P = 0.033), disease duration (P = 0.029), and duration of eye involvement (P = 0.003).

Conclusion
VDI is significantly associated with most disease parameters of BD, except for parameters such as mucocutaneous manifestations and uncomplicated venous thrombosis; however, further studies may be needed to establish BD-specific damage index.
Study of the role of the transcranial magnetic stimulation on language progress in autism spectrum disorder
Authors: Darwish Mohamed E. , El-Beshlawy Heba W. , Ramadan Ehab S. , Serag Shimaa M. 
Published: June 2021

Abstract
Background
Children with autism spectrum disorder (ASD) are almost universally delayed in the acquisition of spoken language as primary means of communication so they tend to have restricted outcomes in terms of independence and integration. Transcranial magnetic stimulation (TMS) is a promising, emerging tool for the study (study and modulate excitability and plasticity, applied in single pulses to investigate corticospinal excitability, pairs of pulses to study intracortical inhibition and facilitation) and potential treatment of ASD. The purpose of this study is to evaluate the role of repetitive TMS in language progress in children with ASD.

Results
There was a statistically significant clinical improvement in patients receiving active TMS (group I) comparing baseline Childhood Autism Rating Scale (CARS) assessment and after treatment (P ≤ 0.05). There was mild improvement with no significant difference between the patients receiving active TMS (group I) and those of sham TMS (group II), and both groups received language therapy as regard post-treatment CARS. There was significant difference in improvement between the two groups according to eye contact (P ≤ 0.05). There was significant improvement in response to examiner (P ≤ 0.05). There was mild improvement with no statistically significant difference in attention between the two groups. There was significant difference in improvement between the two groups according to active expressive language. There was no statistically significant difference in passive vocabulary between the two groups.

Conclusion
Repetitive transcranial magnetic stimulation (rTMS) over left inferior frontal gyrus may be a safe and effective way of improving language of ASD. The joint application of rTMS and standard language therapy may lead to more rapid improvement in the language progress of children with ASD.
The need for biodiversity champions in psychiatry: the entwined crises of climate change and ecological collapse
Authors: Krzanowski Jacob 
Published: June 2021

SummaryThe past 20 years have seen the disappearance and degradation of biodiversity on earth at unprecedented rates. This phenomenon shares similar drivers to those behind climate change yet requires its own solutions. The twin catastrophes of climate change and biodiversity loss demonstrate how our health is bound up in the vitality of this planet. There has been an increasing effort on the part of healthcare professionals to appreciate this relationship, as evidenced by the growing influence of planetary health as a discipline. However, the health impacts of biodiversity loss have been less focused on than those brought on by climate change. Psychiatry's appreciation of the interface between environments and our health, alongside the evidence for the connection between nature and mental health specifically, prompt psychiatrists to ensure that the institution of healthcare throws its influence equally behind solutions to climate change as well as biodiversity loss.
Korea's First Museum and the Categorization of “Buddhist Statues”
Authors: Lee Seunghye 
Published: June 2021

Abstract
The establishment of a museum in the precinct of Ch'anggyŏnggung Palace in 1909 marked an important moment in the historiography of Korean art. Although recent studies have examined the founding, organization, and financing of the first Korean museum, the formation of its Buddhist art collection and its historical implications remain unexamined. Given that not a single Buddhist temple was allowed to exist within the capital city, the entry of these objects into the palace demonstrates a radical paradigm shift in the royal court's relation to Buddhist icons. The museum's Buddhist art collection reveals what was available in the art market of the time and what was considered worthy of being collected in a royal museum. Through close examination of Korea's first museum and its collection, this study traces the recontextualization of religious icons into art objects and the historical implications behind the category of “Buddhist statues.”
A patient-based medaka alg2 mutant as a model for hypo-N-glycosylation
Authors: Gücüm Sevinç , Sakson Roman , Hoffmann Marcus , Grote Valerian , Becker Clara , Pakari Kaisa , Beedgen Lars , Thiel Christian , Rapp Erdmann , Ruppert Thomas , Thumberger Thomas , Wittbrodt Joachim 
Published: June 2021

ABSTRACT
Defects in the evolutionarily conserved protein-glycosylation machinery during embryonic development are often fatal. Consequently, congenital disorders of glycosylation (CDG) in human are rare. We modelled a putative hypomorphic mutation described in an alpha-1,3/1,6-mannosyltransferase (ALG2) index patient (ALG2-CDG) to address the developmental consequences in the teleost medaka (Oryzias latipes). We observed specific, multisystemic, late-onset phenotypes, closely resembling the patient's syndrome, prominently in the facial skeleton and in neuronal tissue. Molecularly, we detected reduced levels of N-glycans in medaka and in the patient's fibroblasts. This hypo-N-glycosylation prominently affected protein abundance. Proteins of the basic glycosylation and glycoprotein-processing machinery were over-represented in a compensatory response, highlighting the regulatory topology of the network. Proteins of the retinal phototransduction machinery, conversely, were massively under-represented in the alg2 model. These deficiencies relate to a specific failure to maintain rod photoreceptors, resulting in retinitis pigmentosa characterized by the progressive loss of these photoreceptors. Our work has explored only the tip of the iceberg of N-glycosylation-sensitive proteins, the function of which specifically impacts on cells, tissues and organs. Taking advantage of the well-described human mutation has allowed the complex interplay of N-glycosylated proteins and their contribution to development and disease to be addressed.
Body size affects immune cell proportions in birds and non-volant mammals, but not bats
Authors: Cornelius Ruhs Emily , Becker Daniel J. , Oakey Samantha J. , Ogunsina Ololade , Fenton M. Brock , Simmons Nancy B. , Martin Lynn B. , Downs Cynthia J. 
Published: June 2021

ABSTRACTPowered flight has evolved several times in vertebrates and constrains morphology and physiology in ways that likely have shaped how organisms cope with infections. Some of these constraints probably have impacts on aspects of immunology, such that larger fliers might prioritize risk reduction and safety. Addressing how the evolution of flight may have driven relationships between body size and immunity could be particularly informative for understanding the propensity of some taxa to harbor many virulent and sometimes zoonotic pathogens without showing clinical disease. Here, we used a comparative framework to quantify scaling relationships between body mass and the proportions of two types of white blood cells – lymphocytes and granulocytes (neutrophils/heterophils) – across 63 bat species, 400 bird species and 251 non-volant mammal species. By using phylogenetically informed statistical models on field-collected data from wild Neotropical bats and from captive bats, non-volant mammals and birds, we show that lymphocyte and neutrophil proportions do not vary systematically with body mass among bats. In contrast, larger birds and non-volant mammals have disproportionately higher granulocyte proportions than expected for their body size. Our inability to distinguish bat lymphocyte scaling from birds and bat granulocyte scaling from all other taxa suggests there may be other ecological explanations (i.e. not flight related) for the cell proportion scaling patterns. Future comparative studies of wild bats, birds and non-volant mammals of similar body mass should aim to further differentiate evolutionary effects and other aspects of life history on immune defense and its role in the tolerance of (zoonotic) infections.