bluuebunny/crossref_metadata_2025_split huggingface dataset:
Recurrent bacterial infections, but not fungal infections, characterise patients with ELANE‐related neutropenia: a French Severe Chronic Neutropenia Registry study
Authors: Rotulo Gioacchino A. , Plat Geneviève , Beaupain Blandine , Blanche Stéphane , Moshous Despina , Sicre de Fontbrune Flore , Leblanc Thierry , Renard Cécile , Barlogis Vincent , Vigue Marie‐Gabrielle , Freycon Claire , Piguet Christophe , Pasquet Marlène , Fieschi Claire , Abou‐Chahla Wadih , Gandemer Virginie , Rialland Fanny , Millot Frédéric , Marie‐Cardine Aude , Paillard Catherine , Levy Pacifique , Aladjidi Nathalie , Biosse‐Duplan Martin , Bellanné‐Chantelot Christine , Donadieu Jean ,   the French Severe Chronic Neutropenia Registry
Published: August 2021

SummaryAmong 143 patients with elastase, neutrophil‐expressed (ELANE)‐related neutropenia enrolled in the French Severe Chronic Neutropenia Registry, 94 were classified as having severe chronic neutropenia (SCN) and 49 with cyclic neutropenia (CyN). Their infectious episodes were classified as severe, mild or oral, and analysed according to their natural occurrence without granulocyte‐colony stimulating factor (G‐CSF), on G‐CSF, after myelodysplasia/acute leukaemia or after haematopoietic stem‐cell transplantation. During the disease’s natural history period (without G‐CSF; 1913 person‐years), 302, 957 and 754 severe, mild and oral infectious events, respectively, occurred. Among severe infections, cellulitis (48%) and pneumonia (38%) were the most common. Only 38% of episodes were microbiologically documented. The most frequent pathogens were Staphylococcus aureus (37·4%), Escherichia coli (20%) and Pseudomonas aeruginosa (16%), while fungal infections accounted for 1%. Profound neutropenia (<200/mm3), high lymphocyte count (>3000/mm3) and neutropenia subtype were associated with high risk of infection. Only the p.Gly214Arg variant (5% of the patients) was associated with infections but not the overall genotype. The first year of life was associated with the highest infection risk throughout life. G‐CSF therapy achieved lower ratios of serious or oral infectious event numbers per period but was less protective for patients requiring >10 µg/kg/day. Infections had permanent consequences in 33% of patients, most frequently edentulism.
The incidence and type of cancer in patients with ataxia‐telangiectasia via a retrospective single‐centre study
Authors: Bakhtiar Shahrzad , Salzmann‐Manrique Emilia , Donath Helena , Woelke Sandra , Duecker Ruth P. , Fritzemeyer Stefanie , Schubert Ralf , Huenecke Sabine , Kieslich Matthias , Klingebiel Thomas , Bader Peter , Zielen Stefan 
Published: August 2021

SummaryAtaxia‐telangiectasia (A‐T) is a hereditary immune system disorder with neurodegeneration. Its first neurologic symptoms include ataxic gait in early childhood, with slowly progressive cerebellar ataxia, oculomotor apraxia, oculocutaneous telangiectasia, and progressive muscle weakness. Neonatal screening for severe T‐cell deficiency was recently found to diagnose A‐T patients with a significantly reduced naïve T‐cell pool. Our study includes 69 A‐T patients between 8 January 2002 and 1 December 2019. Nineteen cases of cancer were diagnosed in 17 patients (25%), with a median overall survival [OS; 95% cumulative indcidence (CI)] of 26·9 years for the entire cohort. The 15‐year OS of 82·5% (72–95%) was significantly decreased among A‐T patients with malignancies, who had a median OS of 2·11 years, with a two‐year‐estimated OS of 50·7% (31–82%). Haematological malignancies were the major causes of death within the initial years of life with a 15 times increased risk for death [HR (95% CI): 6·9 (3·1–15.2), P < 0·001] upon malignancy diagnosis. Male patients with A‐T are at a higher cancer risk than their female counterparts. This manuscript highlights the need for cancer surveillance and prevention, as well as optimal treatment in this cohort.
Dissimilarities in species assemblages among Amazonian mineral licks
Authors: Griffiths Brian M. , Cooper William Justin , Bowler Mark , Gilmore Michael P. , Luther David 
Published: August 2021

AbstractMineral lick elevation, size, and distance to the closest human community are all associated with mammal and bird species visitations. The most frequently hunted licks have similar species assemblages. Results indicate high variability in species assemblages at different mineral licks suggesting different species‐specific resource needs at different licks.Abstract in Spanish is available with online material.
SHARPIN regulates the development of clear cell renal cell carcinoma by promoting von Hippel‐Lindau protein ubiquitination and degradation
Authors: Yin Rusha , Liu Shuai 
Published: August 2021

AbstractSHANK‐associated RH domain interacting protein (SHARPIN) plays an important role in carcinogenesis, as well as inflammation and immunity. Our study explored the effects and underlying mechanisms of SHARPIN in clear cell renal cell carcinoma (ccRCC). By analyzing The Cancer Genome Atlas database, we found that upregulated SHARPIN in patients with ccRCC led to a poor prognosis. Semiquantitative immunohistochemical analysis of clinical samples was carried out and the results suggested the positive association between SHARPIN and hypoxia‐induced factor‐2α (HIF‐2α). Von Hippel‐Lindau protein (pVHL) is a tumor suppressor that contributes to degrading HIF‐2α. Mechanically, SHARPIN promoted the ubiquitination and proteasomal degradation of pVHL, resulting in the sustained activation of HIF‐2α. The α and β domains of pVHL and ubiquitin‐like domain of SHARPIN are required for the interaction. The knockdown of SHARPIN effectively inhibited acquired sorafenib resistance in ccRCC cell lines and tumor growth in xenograft models. In conclusion, our work reveals a novel posttranslational regulation of SHARPIN on pVHL, indicating that SHARPIN could be a potential target for ccRCC treatment.
Smoke Gets in the Eye: A systematic review of case reports of ocular complications of crack cocaine use
Authors: Gohil Hemant , Miskovic Miroslav , Buxton Jane A. , Holland Simon P. , Strike Carol 
Published: August 2021

AbstractIssuesUse of crack cocaine and associated medical complications persists globally. Some reports in medical literature describe a sight‐threatening condition commonly referred to as ‘crack eye’ or ‘crack eye syndrome’. The purpose of this review is to describe what is known about crack eye from case reports in peer‐reviewed literature.ApproachA structured search was completed in MEDLINE, TOXLINE, EMBASE, PsychInfo, Scopus and Biomed Central, to collect case reports and case series on corneal complications attributed to crack cocaine smoking.Key FindingsOf 111 articles screened, 11 contained case reports or series. Thirty individual cases of ‘crack eye’ were reported. The majority (63%) of cases had bilateral involvement; 83% of all cases with microbial culture results had corneal infections. Aggressive treatment caused an improvement in 95% of all cases and 23% of all cases were lost to follow up. Of those who received treatment for corneal complications associated with crack cocaine, 22% remained with significant visual impairment (hand motions only) in the affected eye.ImplicationsClinicians should consider crack cocaine involvement in patients presenting with corneal disease without known predisposing factors, and elicit comprehensive drug histories to prevent a reduction in visual acuity.ConclusionCorneal complications of crack cocaine smoking are caused by a number of synergistic factors, including direct toxicity of crack cocaine vapours to surface cells, impairment of neurogenic support to corneal epithelial integrity, desiccation of the eye surface due to diminished blinking reflex, low level chemical burns and mechanical denudement of surface cells through eye rubbing.
Piscine predation on juvenile salmon in sub‐arctic Alaskan rivers: Associations with season, habitat, predator size and streamflow
Authors: Schoen Erik R. , Sellmer Kristen W. , Wipfli Mark S. , López Juan A. , Ivanoff Renae , Meyer Benjamin E. 
Published: August 2021

AbstractPredation on anadromous salmon can have important consequences for both predators and prey. Salmon provide large seasonal pulses of energy and nutrients via carcasses, eggs and juveniles to many freshwater consumers, and conversely, predation can represent a significant source of mortality for juvenile salmon. Recent declines of Chinook salmon (Oncorhynchus tshawytscha) populations in Alaska have raised concern that predation might inhibit their recovery. Here, we quantify patterns of predation by freshwater fishes on juvenile salmon across seasons, habitats, predator sizes and streamflow levels in the Arctic‐Yukon‐Kuskokwim region of Alaska. We analysed piscivore stomach contents and identified prey using DNA sequence “barcoding.” In coastal rivers, juvenile pink (O. gorbuscha) and chum (O. keta) salmon contributed heavily to Arctic grayling (Thymallus arcticus) and Dolly Varden char (Salvelinus malma) diets, coho salmon (O. kisutch) prey were rare, and Chinook salmon were not detected. In interior rivers, Arctic grayling, burbot (Lota lota) and northern pike (Esox lucius) consumed small numbers of Chinook salmon. Predation on Chinook salmon was documented disproportionately in sloughs during a summer of exceptionally high streamflow. Dietary and distributional patterns suggested northern pike and burbot may exclude salmon from sloughs in low‐gradient river reaches that would otherwise provide suitable rearing habitat. The data also provided tentative support for the hypothesis that high streamflow induces juvenile Chinook salmon to move from mainstem habitats into sloughs, where they face an increased risk of mortality. Incorporating predation risk into climate adaptation, fisheries management and habitat restoration decisions may help to facilitate Chinook salmon recovery.
Quantifying the energetic cost of food quality constraints on resting metabolism to integrate nutritional and metabolic ecology
Authors: Ruiz Thomas , Koussoroplis Apostolos‐Manuel , Danger Michael , Aguer Jean‐Pierre , Morel‐Desrosiers Nicole , Bec Alexandre 
Published: August 2021

AbstractConsumer metabolism controls the energy uptake from the environment and its allocation to biomass production. In natural ecosystems, available energy in food often fails to predict biomass production which is also (co)limited by the relative availability of various dietary compounds. To date, the link between energy metabolism and the effects of food chemical composition on biomass production remains elusive. Here, we measured the resting metabolic rate (RMR) of Daphnia magna along ontogeny when undergoing various (non‐energetic) nutritional constraints. All types of dietary (co)limitations (Fatty acids, Sterols, Phosphorus) induced an increase in mass‐specific RMR up to 128% between highest and lowest quality diets. We highlight a strong negative correlation between RMR and growth rate indicating RMR as a promising predictor of consumer growth rate. We argue that quantifying the energetic cost imposed by food quality on individual RMR may constitute a common currency enabling the integration of nutritional and metabolic ecology.
Looming extinctions due to invasive species: Irreversible loss of ecological strategy and evolutionary history
Authors: Bellard Céline , Bernery Camille , Leclerc Camille 
Published: August 2021

AbstractBiological invasions are one of the main drivers of biodiversity decline worldwide. However, many associated extinctions are yet to occur, meaning that the ecological debt caused by invasive species could be considerable for biodiversity. We explore extinction scenarios due to invasive species and investigate whether paying off the current extinction debt will shift the global composition of mammals and birds in terms of ecological strategy and evolutionary history. Current studies mostly focus on the number of species potentially at risk due to invasions without taking into account species characteristics in terms of ecological or phylogenetic properties. We found that 11% of phylogenetic diversity worldwide is represented by invasive‐threatened species. Furthermore, 14% of worldwide trait diversity is hosted by invasive‐threatened mammals and 40% by invasive‐threatened birds, with Neotropical and Oceanian realms being primary risk hotspots. Projected extinctions of invasive‐threatened species result in a smaller reduction in ecological strategy space and evolutionary history than expected under randomized extinction scenarios. This can be explained by the strong pattern in the clustering of ecological profiles and families impacted by invasive alien species (IAS). However, our results confirm that IAS are likely to cause the selective loss of species with unique evolutionary and ecological profiles. Our results also suggest a global shift in species composition away from those with large body mass, which mostly feed in the lower foraging strata and have an herbivorous diet (mammals). Our findings demonstrate the potential impact of biological invasions on phylogenetic and trait dimensions of diversity, especially in the Oceanian realm. We therefore call for a more systematic integration of all facets of diversity when investigating the consequences of biological invasions in future studies. This would help to establish spatial prioritizations regarding IAS threats worldwide and anticipate the consequences of losing specific ecological profiles in the invaded community.
Addressing social inequity through improving relational care: A social–ecological model based on the experiences of migrant women and midwives in South Wales
Authors: Goodwin Laura , Jones Aled , Hunter Billie 
Published: August 2021

AbstractBackgroundMigrant and ethnic inequalities in maternal and perinatal mortality persist across high‐income countries. Addressing social adversity and inequities across the childbirth trajectory cannot be left to chance and the good intentions of practitioners. Robust, evidence‐based tools designed to address inequity by enhancing both the quality of provision and the experience of care are needed.MethodsAn inductive modelling approach was used to develop a new evidence‐based conceptual model of woman–midwife relationships, drawing on data from an ethnographic study of relationships between migrant Pakistani women and midwives, conducted between 2013 and 2016 in South Wales, UK. Key analytic themes from early data were translated into social–ecological concepts, and a model was developed to represent how these key themes interacted to influence the woman–midwife relationship.ResultsThree key concepts influencing the woman–midwife relationship were developed from the three major themes of the underpinning research: (1) Healthcare System; (2) Culture and Religion; and (3) Family Relationships. Two additional weaving concepts appeared to act as a link between these three key concepts: (1) Authoritative Knowledge and (2) Communication of Information. Social and political factors were also considered as contextual factors within the model. A visual representation of this model was developed and presented.ConclusionsThe model presented in this paper, along with future work to further test and refine it in other contexts, has the potential to impact on inequalities by facilitating future discussion on cultural issues, encouraging collaborative learning and knowledge production and providing a framework for future global midwifery practice, education and research.Patient or Public ContributionAt the outset of the underpinning research, a project involvement group was created to contribute to study design and conduct. This group consisted of the three authors, an Advocacy Officer at Race Equality First and an NHS Consultant Midwife. This group met regularly throughout the research process, and members were involved in discussions regarding ethical/cultural/social issues, recruitment methods, the creation of participant information materials, interpretation of data and the dissemination strategy. Ideas for the underpinning research were also discussed with members of the Pakistani community during community events and at meetings with staff from minority ethnic and migrant support charities (BAWSO, Race Equality First, The Mentor Ring). Local midwives contributed to study design through conversations during informal observations of antenatal appointments for asylum seekers and refugees.
Massive open online courses and consumer goals
Authors: Howarth Jason , D’Alessandro Steven , Johnson Lester , White Lesley 
Published: August 2021

AbstractMassive open online courses (MOOCS) are increasingly used by higher education providers to promote their fee‐paying courses. But while multiple studies have examined the reasons why individuals enrol into MOOCs, little is understood about the effects of course engagement on their later educational goals. The current work addresses this gap by investigating the impact of MOOC engagement on participants’ future study goals and educational brand choices. This research uses a mixed methods approach to examine the goals and selection preferences of students following their engagement with a MOOC. This is accomplished through two separate longitudinal studies of MOOC participants. The first study consists of two sets of individual interviews (pre and post‐MOOC). The second involves the use of pre‐ and post MOOC surveys of participants enroled into courses on project management and cyber security. The findings show that engagement with a MOOC can positively influence the future academic goals of students, as well as their choice of academic provider. These results highlight the marketing potential of Massive Open Online Courses as a pathway into higher education. The goal framework used in this study is also likely to have broader applicability for understanding goal‐oriented behaviour in other consumer domains.
Physicochemical and bioactive compounds at edible ripeness of eleven varieties of avocado (Persea americana) cultivated in the Andean Region of Peru
Authors: Huaman‐Alvino Cinthya , Chirinos Rosana , Gonzales‐Pariona Fernando , Pedreschi Romina , Campos David 
Published: August 2021

SummaryThis work aimed evaluating some physicochemical compounds, hydrophilic (H‐AC) and lipophilic (L‐AC) antioxidant capacity of eleven avocado genotypes grown in the Andes. The results show great variability in physical and chemical properties, dry matter (DM) and lipid contents with ranges of 18.4–39.3% and 10.1–23.2%, respectively. All genotypes had high oleic acid content (50.4–69.9%) and a high unsaturated fatty acid/saturated fatty acid ratio (UFA/SFA, from 3.05 to 6.05), while the unsaponifiable fraction contained mainly β‐sitosterol (140.83–235.51 mg/100 g DM) and α‐tocopherol (17.44–71.29 µg/g DM). Total phenolic compounds (TPC), H‐AC and L‐AC ranged from 0.48 to 0.88 mg EAG/g DM, 5.37 to 14.00 and 1.87 to 6.71 μmol ET/g DM, respectively. Although the genotype influences avocado characteristics, climatic conditions in the Andean region seem to be important for the biosynthesis of metabolites of interest and should be considered in future studies.
A performance evaluation framework of public health distribution network for essential medicines: lessons from the select Indian states
Authors: Kumar Rahul , Das Debadyuti 
Published: August 2021

AbstractThe present work has been carried out to develop a performance evaluation framework of distribution network design in public health with specific reference to essential medicines based on six different types of public health distribution networks (PHDNs) prevalent across six Indian states. We evaluated the performance of PHDNs on the criteria of cost and service and also on the sub‐criteria of cost and service by following the analytic hierarchy process. We utilized the pairwise comparison method in determining the relative importance of criteria and sub‐criteria by seeking responses from several experts and subsequently evaluated the PHDNs on all sub‐criteria in a 7‐point Likert scale by seeking views from the same experts. Finally, we developed the “cost–service matrix,” in which cost and service have been depicted in the horizontal and vertical axes, respectively. We demonstrated the performance of six PHDNs in this matrix in terms of cost and service by plotting scores obtained by them on cost and service, respectively. The findings of the study provide rich insights to the policy planners of public health.
Three easy fixes for sire use can enhance genetic progress in community‐based breeding programmes
Authors: Mueller Joaquín P. , Getachew Tesfaye , Rekik Mourad , Rischkowsky Barbara , Abate Zelalem , Goshme Shenkute , Wale Yeshiwas , Haile Aynalem 
Published: August 2021

AbstractWe analysed options to optimize the use of sires in sheep and goat community‐based breeding programmes (CBBP) for three scenarios occurring under field conditions: premature sale of selection candidates, fixed service periods of selected sires and incomplete sire pedigrees. The first scenario was studied by looking at the outcome of combinations of selection pressures in successive selection stages. A compromise of early sale of sire candidates and genetic progress can be obtained by selecting in two stages, such that selection pressure in the first stage is chosen in terms of achieving an acceptable selection potential after the second stage. Simulations showed the dependency of this compromise on selection accuracies and correlation between selection criteria. For a typical sheep CBBP, only 20% of the top three months weighting male lambs need to be retained to achieve 80% of the potential selection differential on six months weight. For the second scenario, two alternatives to fixed service periods were analysed. When across‐age BLUP EBVs are not available, individual sires can be programmed to stay in service according to their initial ranking. In typical sheep, CBBPs genetic progress can be increased by about 9% over response to selection with optimum fixed sire service periods. When BLUP EBVs are available, a simulated retrospective analyses of across‐age selection of sires in two current sheep CBBPs increased more than twofold the average breeding values actually observed. Thirdly, we studied the benefit of considering possible sires and their mating probabilities when estimating BLUP breeding values instead of setting such sires as unknown. In a current goat CBBP with up to three possible sires included in the pedigree, the accuracy of breeding values nears the accuracy when sires are known and are higher than accuracies when sires are unknown or when possible sires are ignored.