bluuebunny/crossref_metadata_2025_split huggingface dataset:
Differential diagnosis of progressive intellectual and neurological deterioration in children
Authors: Verity Christopher , Baker Elaine , Maunder Polly , Pal Suvankar , Winstone Anne Marie 
Published: September 2020

AimTo report the differential diagnosis in children with progressive intellectual and neurological deterioration (PIND) in the UK.MethodSince 1997 the PIND Study has searched for variant Creutzfeldt‐Jakob disease (vCJD) in children, using the British Paediatric Surveillance Unit to perform prospective surveillance of those younger than 16 years with PIND.ResultsFrom May 1997 to October 2019, 2255 children meeting PIND criteria had been notified, of whom 2008 (1085 males, 923 females) had underlying diagnoses. There were over 220 different diseases, including six cases of vCJD. The numbers presenting in four age groups were: <1 year, 805 (40%); 1 to 4 years inclusive, 825 (41%); 5 to 9 years inclusive, 264 (13%); and 10 to 15 years inclusive, 114 (6%). The two largest ethnic groups were White and Pakistani (58.2% and 17% of diagnosed cases). The most common diseases in these two ethnic groups are shown for the four age groups. The distribution of diseases varied with age but was quite similar in White and Pakistani children.InterpretationThis paper provides a unique guide to the complex differential diagnosis of childhood PIND, showing considerable differences between four age groups, but similarities between ethnic groups. The PIND Study still provides the only systematic surveillance for vCJD in children in the UK.What this paper adds

The prevalence of diseases causing childhood progressive intellectual and neurological deterioration in the UK is low (approximately 0.1/1000 live births).

There were more than 220 different disorders, mainly genetically determined.

The majority of disorders presented early in childhood: 81% before the age of 5 years.

There were similarities in the disease spectrum in White and Pakistani children.
Decision‐Making: The Process Is the Content in an Experience‐Based Classroom
Authors: Conklin Thomas A. , Boulamatsi Artemis 
Published: September 2020

ABSTRACTThis article describes a multistep exercise focused on decision‐making that can be used in a variety of disciplines. The exercise helps students understand process issues in decision‐making and the complexity of doing so in real world scenarios. The exercise uses a case to illustrate the paradoxical effect of how decision‐makers often focus on the content of decisions in lieu of attending to the decision‐making process. It is best suited for upper‐level undergraduate and graduate students. Insights about process issues are revealed through creating a team contract, a team inventory, and making the decisions required in the case analysis. These revelations occur across three 75‐minute classes but can be adapted to the time available and relevance of the course. The article's structure is organized according to Wiggins and McTighe's curriculum development model.
Effects of visual adaptation on orientation selectivity in cat secondary visual cortex
Authors: Lussiez Rudy , Chanauria Nayan , Ouelhazi Afef , Molotchnikoff Stéphane 
Published: September 2020

AbstractNeuron orientation selectivity, otherwise known as the ability to respond optimally to a preferred orientation, has been extensively described in both primary and secondary visual cortices. This orientation selectivity, conserved through all cortical layers of a given column, is the primary basis for cortical organization and functional network emergence. While this selectivity is programmed and acquired since critical period, it has always been believed that in a mature brain, neurons’ inherent functional features could not be changed. However, a plurality of studies has investigated the mature brain plasticity in V1, by changing the cells’ orientation selectivity with visual adaptation. Using electrophysiological data in both V1 and V2 areas, this study aims to investigate the effects of adaptation on simultaneously recorded cells in both areas. Visual adaptation had an enhanced effect on V2 units, as they exhibited greater tuning curve shifts and a more pronounced decrease of their OSI. Not only did adaptation have a different effect on V2 neurons, it also elicited a different response depending on the neuron's cortical depth. Indeed, in V2, cells in layers II‐III were more affected by visual adaptation, while infragranular layer V units exhibited little to no effect at all.
Is the medicine right?
Authors: Meloni Gabriella 
Published: September 2020

AbstractIs asking the Better Regulation Agenda (BRA) to answer the same preconditions it requires for any regulatory action a proper treatment? Does any assessment of the agenda necessarily imply a thorough definition of the costs and the benefits deriving from its application or is it enough to provide a few key insights to perform it? Is the BRA really so ideological, deriving from “a liberal analytical framework that considers no regulation/state intervention” as the preferred option? Is regulatory quality an issue that “cannot realistically be solved”? Does the principle of subsidiarity as a policy objective need some revision? Several questions come to mind when reading a very thought‐provoking article that is very critical of the BRA but that in the end recognises some of its main qualities.
Cropland connectivity affects genetic divergence of Colorado potato beetle along an invasion front
Authors: Yang Fangyuan , Liu Ning , Crossley Michael S. , Wang Pengcheng , Ma Zhuo , Guo Jianjun , Zhang Runzhi 
Published: September 2020

AbstractThe population genetic structure of invasive species can be strongly affected by environmental and landscape barriers to dispersal. Disentangling the relative contributions of these factors to genetic divergence among invading populations is a fundamental goal of landscape genetics with important implications for invasion management. Here, we relate patterns of genetic divergence in a global invasive agricultural pest, Colorado potato beetle (CPB; Leptinotarsa decemlineata), to environmental and landscape factors along an invasion front in Northwestern China. We first used microsatellite markers and spatial‐temporal samples to assess broad patterns of genetic diversity as well as fine‐scale changes in patterns of genetic divergence. We then distinguished the relative contributions of five factors to genetic divergence among front populations: geographic distance (isolation by distance), climate dissimilarity (isolation by environment), and least‐cost distances (isolation by resistance) modeled with three factors: climate suitability, cropland cover, and road networks. Genetic diversity broadly decreased from West to East, with the exception being Eastern China. Low levels of genetic diversity and varying degrees of divergence were observed in Northwestern China, reflecting the potential effect of landscape heterogeneity. Least‐cost distance across cropland cover was most positively correlated with genetic divergence, suggesting a role of croplands in facilitating gene flow. The contribution of climate to genetic divergence was secondary, whether modeled in terms of local adaptability or connectivity of the climatic landscape, suggesting that constraints to CPB gene flow imposed by a harsh climate may be ameliorated in agricultural landscapes. No evidence was found for an obvious effect of road networks on genetic divergence and population structuring. Our study provides an example of how agricultural landscape connectivity can facilitate the spread of invasive pests, even across a broad climatic gradient. More broadly, our findings can guide decisions about future land management for mitigating further spread.
Prolyl and lysyl hydroxylases in collagen synthesis
Authors: Salo Antti M. , Myllyharju Johanna 
Published: September 2020

AbstractCollagens are the most abundant proteins in the extracellular matrix. They provide a framework to build organs and tissues and give structural support to make them resistant to mechanical load and forces. Several intra‐ and extracellular modifications are needed to make functional collagen molecules, intracellular post‐translational modifications of proline and lysine residues having key roles in this. In this article, we provide a review on the enzymes responsible for the proline and lysine modifications, that is collagen prolyl 4‐hydroxylases, 3‐hydroxylases and lysyl hydroxylases, and discuss their biological functions and involvement in diseases.
Stage‐dependent effects of river flow and temperature regimes on the growth dynamics of an apex predator
Authors: Stoffels Rick J. , Weatherman Kyle E. , Bond Nick R. , Morrongiello John R. , Thiem Jason D. , Butler Gavin , Koster Wayne , Kopf R. Keller , McCasker Nicole , Ye Qifeng , Zampatti Brenton , Broadhurst Ben 
Published: September 2020

AbstractIn the world's rivers, alteration of flow is a major driver of biodiversity decline. Global warming is now affecting the thermal and hydrological regimes of rivers, compounding the threat and complicating conservation planning. To inform management under a non‐stationary climate, we must improve our understanding of how flow and thermal regimes interact to affect the population dynamics of riverine biota. We used long‐term growth biochronologies, spanning 34 years and 400,000 km2, to model the growth dynamics of a long‐lived, apex predator (Murray cod) as a function of factors extrinsic (river discharge; air temperature; sub‐catchment) and intrinsic (age; individual) to the population. Annual growth of Murray cod showed significant, curvilinear, life‐stage‐specific responses to an interaction between annual discharge and temperature. Growth of early juveniles (age 1+ and 2+ years) exhibited a unimodal relationship with annual discharge, peaking near median annual discharge. Growth of late juveniles (3+ to 5+) and adults (>5+) increased with annual discharge, with the rate of increase being particularly high in adults, whose growth peaked during years with flooding. Years with very low annual discharge, as experienced during drought and under high abstraction, suppress growth rates of all Murray cod life‐stages. Unimodal relationships between growth and annual temperature were evident across all life stages. Contrary to expectations of the Temperature Size Rule, the annual air temperature at which maximum growth occurred increased with age. The stage‐specific response of Murray cod to annual discharge indicates that no single magnitude of annual discharge is optimal for cod populations, adding further weight to the case for maintaining and/or restoring flow variability in riverine ecosystems. With respect to climate change impacts, on balance our results indicate that the primary mechanism by which climate change threatens Murray cod growth is through alteration of river flows, not through warming annual mean temperatures per se.
Superchilled, chilled and frozen storage of Atlantic mackerel (Scomber scombrus) – effect on lipids and low molecular weight metabolites
Authors: Mozuraityte Revilija , Standal Inger Beate , Cropotova Janna , Budźko Emilia , Rustad Turid 
Published: September 2020

SummaryQuality changes in Atlantic mackerel fillets during superchilled, chilled and frozen storage focusing on lipid quality, colour and changes in low molecular weight metabolites relevant for quality and safety were studied. Low formation of oxidation products was observed in chilled (up to 7 days), superchilled (up to 14 days) and frozen storage (up to one year). Only slight formation of TBARS was measured in the samples which correlated with the increasing fillet yellowness. The profile of low molecular weight compounds, analysed by high‐resolution NMR, showed clear grouping of the different samples according to both treatment and storage time. The increase in K‐value was highest in the chilled samples, reaching a K‐value of 93 ± 3% on day 5, exceeding the proposed limit of human consumption (80%) while superchilling reached a K‐value of ca 90 % after 14 days. Frozen samples showed acceptable quality in the whole storage period.
Emodin inhibiting neutrophil elastase‐induced epithelial‐mesenchymal transition through Notch1 signalling in alveolar epithelial cells
Authors: Zhou Linshui , Gao Rundi , Hong Huihua , Li Xiaojuan , Yang Jia , Shen Wei , Wang Zhen , Yang Junchao 
Published: September 2020

AbstractThe transition of alveolar type II epithelial cells into fibroblasts has been reported to cause and/or aggravate pulmonary fibrosis (PF), which is characterized by fibroblast proliferation, an enhanced production and accumulation of ECM (extracellular matrix), alveolar wall damage and functional capillary unit loss. Traditional Chinese medicine Emodin has been reported to inhibit TGF‐β‐induced epithelial‐mesenchymal transition (EMT) in alveolar epithelial cells through Notch signalling. In the present study, neutrophil elastase (NE, also known as ELA2) treatment promoted EMT, Notch1 cleavage (NICD/Notch1 ratio increase) and NICD nuclear translocation in RLE‐6TN cells and A549 cells. The promotive roles of NE treatment in these events were significantly reversed by Notch1 knockdown. Traditional Chinese medicine Emodin treatment remarkably inhibited the enzyme activity of NE, suppressed EMT, Notch1 cleavage and NICD nuclear translocation within RLE‐6TN and A549 cells, while NE treatment significantly reversed the effects of Emodin. Moreover, in RLE‐6TN, the effects of NE on EMT, Notch1 cleavage and NICD nuclear translocation were remarkably attenuated by Emodin treatment and more attenuated by the combination of Emodin and neutrophil elastase inhibitor Sivelestat or notch signal pathway inhibitor DAPT. In conclusion, we revealed the involvement of NE‐induced Notch1 cleavage in the functions of Emodin suppressing NE‐caused EMT in RLE‐6TN cells and A549 cells. This novel mechanism of Emodin inhibiting EMT might extend the application of Emodin in PF treatment.
Vitamin B6 prevents excessive inflammation by reducing accumulation of sphingosine‐1‐phosphate in a sphingosine‐1‐phosphate lyase–dependent manner
Authors: Du Xialin , Yang Yalong , Zhan Xiaoxia , Huang Yulan , Fu Yuling , Zhang Zelin , Liu Honglin , Zhang Lijie , Li Yanfen , Wen Qian , Zhou Xinying , Zuo Daming , Zhou Chaoying , Li Laisheng , Hu Shengfeng , Ma Li 
Published: September 2020

AbstractVitamin B6 is necessary to maintain normal metabolism and immune response, especially the anti‐inflammatory immune response. However, the exact mechanism by which vitamin B6 plays the anti‐inflammatory role is still unclear. Here, we report a novel mechanism of preventing excessive inflammation by vitamin B6 via reduction in the accumulation of sphingosine‐1‐phosphate (S1P) in a S1P lyase (SPL)‐dependent manner in macrophages. Vitamin B6 supplementation decreased the expression of pro‐inflammatory cytokines by suppressing nuclear factor‐κB and mitogen‐activated protein kinases signalling pathways. Furthermore, vitamin B6–reduced accumulation of S1P by promoting SPL activity. The anti‐inflammatory effects of vitamin B6 were inhibited by S1P supplementation or SPL deficiency. Importantly, vitamin B6 supplementation protected mice from lethal endotoxic shock and attenuated experimental autoimmune encephalomyelitis progression. Collectively, these findings revealed a novel anti‐inflammatory mechanism of vitamin B6 and provided guidance on its clinical use.
Testicular leiomyoma and spermatogenic failure syndrome in a seabass from broodstock
Authors: Natale Sabrina , Capparucci Fabiano , Abbate Jessica Maria , Panebianco Rosanna , Puleio Roberto , Iaria Carmelo 
Published: September 2020

AbstractTesticular leiomyomas and gonadal dysfunctions are not commonly reported in teleost and particularly in broodstock fish. In the present work, a testicular leiomyoma related to an unusual case of spermatogenic failure syndrome, in a broodstock seabass coming from an Italian aquaculture farm, is reported. At gross pathology, the circumscribed neoplasia showed several white nodular masses, originating from the gonad, deforming the anatomo‐physiological shape. From light microscopy, the presence of neoplastic tissue islands with spindle cells with a whirling pattern and the low presence of connective tissue were evident and originated from smooth muscle cells. Seminiferous tubules showed severe azoospermia with the absence of sperm cells even closer to the neoplastic area. Immunohistochemical analysis was positive for alpha‐SMA, desmin and vimentin but negative for S‐100, confirming a diagnosis of leiomyoma. This is the first report of spermatogenic failure syndrome associated with a testicular leiomyoma in fish from broodstock. More efforts should be made in studying broodstock fish pathologies related to fish maintenance and hormonal treatments that could economically affect aquaculture production.
Modeling of product temperature in a supercritical fluid extrusion process through dimensional analysis
Authors: Pan Tina Ying , Gopirajah Rajamanickam , Krishnamurthy Sugirtha , Rizvi Syed S.H. 
Published: September 2020

AbstractA correlation model for predicting the maximum product temperature reached during a supercritical fluid extrusion (SCFX) process was developed using the Buckingham Pi method. Two dimensionless groups, Dref (ratio of screw diameter to screw length) and Eref (ratio of energy added to energy removed), were developed to model the maximum temperature of an SCFX process using dimensional analysis. The model was validated by comparing predicted and experimental values. Experiments were conducted using two feed formulations: 1. Milk protein concentrate (MPC) with rice flour (30%), and 2. MPC with grape pomace (15%). The average percent error was 2.57% for rice flour extrudate and 1.02% for grape pomace extrudate. Based on the results, the model could be used for prediction of maximum temperature in SCFX systems.Practical ApplicationsMaintaining the extruder temperature below 100°C remains a major challenge during the supercritical fluid extrusion (SCFX) process. The model developed determines the maximum temperature of a given SCFX system based on key extrusion parameters. Hence, the developed model can be used by manufacturers for determining the optimal operating conditions while scaling up an SCFX process.
Modeling of vacuum‐ and ultrasound‐assisted osmodehydration of carrot cubes followed by combined infrared and spouted bed drying using artificial neural network and regression models
Authors: Alizehi Mohammad Hashem , Niakousari Mehrdad , Fazaeli Mahboubeh , Iraji Maryam 
Published: September 2020

AbstractThis research seeks to understand the effects of using various vacuum pretreatments (0 and 200 mBar) and different ultrasound power levels (0 and 130 W) on osmodehydration and spouted bed drying of carrot cubes. Effects of using diverse air‐drying temperatures (55, 65 and 75°C), infrared power (0 and 250 W) and the presence of Teflon beads as inert particles (0 and 30% w/w), were also investigated. The effects of above‐mentioned parameters on drying time and physicochemical characteristics of dried carrot samples including hardness, shrinkage, total carotenoid content, rehydration ratio and total color difference (∆E) were examined. The results explicitly demonstrated that the utilization of osmodehydration in conjunction with ultrasound and vacuum (UVOD) had shorter drying time compared to those just treated with osmotic dehydration. The highest total carotenoid content and the lowest ∆E were belonged to the samples exposed to UVOD. The efficiency of artificial neural network (ANN), genetic algorithm based ANN (GANN) and Regression mathematical models were investigated to determine the nonlinear relationship between the inputs and outputs variables. To find the efficiency of all models, mean square error were compared. The results indicate that the ANN model could provide more reliable prediction over the other models.Practical applicationCombined IR‐convective drying has gained increased attention in the recent years because of the fast and efficient transfer of heat, cheap processing cost, and uniform heat transfer, which resulted in products with better organoleptic properties than common drying processes. The combination of hot air and IR radiation resulted in a synergistic effect, leading to more efficient drying than either individual convective or IR drying. Vegetables with high levels of moisture content can be effectively dried using pretreatments such as ultrasonically vacuum‐assisted osmotic dehydration, which reduced the drying time.
RING finger protein RGLG1 and RGLG2 negatively modulate MAPKKK18 mediated drought stress tolerance in Arabidopsis
Authors: Yu Jiayi , Kang Lu , Li Yuanyuan , Wu Changai , Zheng Chengchao , Liu Pei , Huang Jinguang 
Published: September 2020

AbstractMitogen activated protein kinase kinase kinase 18 (MAPKKK18) mediated signaling cascade plays important roles in Arabidopsis drought stress tolerance. However, the post‐translational modulation patterns of MAPKKK18 are not characterized. In this study, we found that the protein level of MAPKKK18 was tightly controlled by the 26S proteasome. Ubiquitin ligases RGLG1 and RGLG2 ubiquitinated MAPKKK18 at lysine residue K32 and K154, and promoted its degradation. Deletion of RGLG1 and RGLG2 stabilized MAPKKK18 and further enhanced the drought stress tolerance of MAPKKK18‐overexpression plants. Our data demonstrate that RGLG1 and RGLG2 negatively regulate MAPKKK18‐mediated drought stress tolerance in Arabidopsis.
Interleukin 17 receptor A haplotype analysis in chronic spontaneous urticaria: A preliminary study
Authors: Nada Hesham , Hassan Ranya , Ibrahim Rasha Abd El‐Hamed , Abdelsalam Omnia Emad , Fathy Amal , Toraih Eman Ali , Atwa Mona A. 
Published: September 2020

AbstractBackgroundChronic spontaneous urticaria (CSU) is a distressing skin disease. Family clustering and heterogeneity in the onset and progression indicate that susceptibility to CSU is a complex trait. In this study, we performed haplotype analysis for one of the key player gene, IL17RA, for CSU to test the association with disease susceptibility and severity.MethodologyThe study included 70 CSU patients and 30 healthy controls. The severity of the disease was evaluated by autologous serum skin test (ASST) and urticaria activity score (UAS). ASST test was done and quality of life was assessed using a questionnaire. Allelic discrimination analysis for rs4819554 and rs879577 was performed using real‐time polymerase chain reaction technology.ResultsCarriers of rs4819554*G were more prone to develop CSU than its counterpart (P = .039), while rs4819554*A allele displayed more severe phenotype in the form of more prolonged disease duration (P = .040), concurrent angioedema (P < .001), higher level of treatment (P < .001), and higher score of quality of life (P < .001). Additionally, homozygote patients with rs879577*CC were associated with angioedema (P < .001). Haplotype analysis revealed that cohorts with both rs4819554*A and rs879577*T conferred protection against developing CSU (OR = 0.07, 95% CI = 0.01‐0.32, P = .001).ConclusionOur results showed that IL17RA gene polymorphisms might contribute to the increased susceptibility to CSU.
A Dynamic Model of Optimal Creditor Dispersion
Authors: ZHONG HONGDA 
Published: September 2020

ABSTRACTBorrowing from multiple creditors exposes firms to rollover risk due to coordination problems among creditors, but it also improves firms' repayment incentives, thereby increasing pledgeability. Based on this trade‐off, I develop a dynamic debt rollover model to analyze the evolution of creditor dispersion. Consistent with empirical evidence, I find that firms optimally increase creditor dispersion after poor performance. In contrast, cross‐sectionally higher‐growth firms can support more dispersed creditors. Frequent debt renegotiation limits firms' ability to increase pledgeability by having more creditors. Finally, holding a cash balance while borrowing from multiple creditors improves firms' repayment incentives uniformly across all future states.