bluuebunny/crossref_metadata_2025_split huggingface dataset:
Genetic causes of haemophilia in women and girls
Authors: Miller Connie H. , Bean Christopher J. 
Published: December 2020

AbstractWomen and girls reported as “haemophilic females” may have complex genetic causes for their haemophilia phenotype. In addition, women and girls may have excessive bleeding requiring treatment simply because they are heterozygous for haemophilia alleles. While severe and moderate haemophilia are rare in females, 16% of patients with mild haemophilia A and almost one‐quarter of those with mild haemophilia B seen in U.S. haemophilia treatment centres are women and girls. A phenotypic female with a low level of factor VIII or factor IX may be classified into one of the following categories of causality: homozygosity (two identical haemophilia alleles), compound heterozygosity (two different haemophilia alleles), hemizygosity (one haemophilia allele and no normal allele), heterozygosity (one haemophilia allele and one normal allele), genetic causes other than haemophilia and non‐genetic causes. Studies required for classification may include coagulation parameters, F8 or F9 sequencing, F8 inversion testing, multiplex ligation‐dependent probe amplification, karyotyping and X chromosome inactivation studies performed on the patient and parents. Women and girls who are homozygous, compound heterozygous or hemizygous clearly have haemophilia, as they do not have a normal allele. Heterozygous women and girls with factor levels below the haemostatic range also meet the definitions used for haemophilia treatment.
Managing women‐specific bleeding in inherited bleeding disorders: A multidisciplinary approach
Authors: Mauser‐Bunschoten Eveline P. , Kadir Rezan A. , Laan Ellen T. M. , Elfvinge Petra , Haverman Lotte , Teela Lorynn , Degenaar Manon E. L. , Fransen van de Putte Dietje E. , D'Oiron Roseline , van Galen Karin P. M. 
Published: December 2020

AbstractIntroductionMultidisciplinary management of women‐specific bleeding is important to preserve quality of life, healthy reproduction and social participation of women and girls with bleeding disorders (WBD).AimTo support appropriate multidisciplinary care for WBD in haemophilia treatment centres.MethodsTwo case examples are presented and management issues discussed from different health care perspectives, including the nurse, patient, psychologist, gynaecologist, geneticist, psychosexual therapist and haematologist.ResultsWoman with bleeding disorders may experience heavy menstruation from menarche onwards. This has a physical and psychosocial impact requiring a multidisciplinary approach. If a woman with an inherited bleeding disorder desires to become pregnant, preconception counselling is essential, to discuss genetic diagnosis, state of the art treatment options for the bleeding disorder in question and possible choices to prevent having an affected child, as well as maternal bleeding risks during conception, delivery and the post‐partum period.ConclusionAdequate management and good education of WBD requires a patient‐centred multidisciplinary approach with experienced specialists in a haemophilia treatment centre.
The new kid on the block: Perceived contributions and pitfalls of private higher education in Ethiopia
Authors: Tamrat Wondwosen , Fetene Getnet Tizazu 
Published: December 2020

AbstractOver the last few decades, the active promotion of or government acquiescence in a surge of private higher education (PHE) has become a common phenomenon globally. This study examines the specific contributions and pitfalls of private higher education institutions (PHEIs) using questionnaires, interviews and documentary analysis as data gathering tools. The findings reveal that private higher education is perceived as having significant contributions to make in addressing the wider demands of access and gender parity, and local and economic development. However, the sector is equally liable to a wide range of pitfalls related to its poor image, substandard quality, illicit behaviour and prohibitive regulations. It argues that if the current deficiencies of the sector are to be addressed in a meaningful way, more needs to be done in terms of enhancing the capacity of institutions, enforcing the rule of law and engendering public support through improved delivery and image.
Heat transfer from glass melt to cold cap: Computational fluid dynamics study of cavities beneath cold cap
Authors: Abboud Alexander W. , Guillen Donna P. , Hrma Pavel , Kruger Albert A. , Klouzek Jaroslav , Pokorny Richard 
Published: December 2020

AbstractEfficient glass production depends on the continuous supply of heat from the glass melt to the floating layer of batch, or cold cap. Computational fluid dynamics (CFD) are employed to investigate the formation and behavior of gas cavities that form beneath the batch by gases released from the collapsing primary foam bubbles, ascending secondary bubbles, and in the case of forced bubbling, from the rising bubbling gas. The gas phase fraction, temperature, and velocity distributions below the cold cap are used to calculate local and average heat transfer rates as a function of the bubbling rate. It is shown that the thickness of the cavities is nearly independent of the cold cap shape and the amount of foam evolved during batch conversion. It is ~7 mm and up to ~15 mm for the cases without and with forced bubbling used to promote circulation within the melt, respectively. Using computed velocity and temperature profiles, the melting rate of the simulated high‐level nuclear waste glass batch was estimated to increase with the bubbling rate to the power of ~0.3 to 0.9, depending on the flow pattern. The simulation results are in good agreement with experimental data from laboratory‐ and pilot‐scale melter tests.
Are sham‐operated mice a valid comparator in studies using a bladder outlet obstruction model? A pitfall reveals a meaningful insight
Authors: Kitta Takeya , Hattori Tsuyoshi , Chiba Hiroki , Higuchi Madoka , Kanno‐Kakibuchi Yukiko , Ouchi Mifuka , Togo Mio , Abe‐Takahashi Yui , Michishita Mai , Kitano Tatsuya , Kusakabe Naohisa , Shinohara Nobuo 
Published: December 2020

ObjectiveTo evaluate voiding behavior characteristics in intact and sham mice, and to examine whether intact mice show changes in “normal” micturition with aging.MethodsA total of 72 8‐week‐old mice were divided into two groups – intact and sham – and the latter group was subjected to a sham of partial bladder outlet obstruction surgery. Urination frequency was evaluated (through metabolic cages) at 1, 2, 3, 6 and 12 months after the surgery (or at the equivalent time points for the intact mice). To address possible mechanisms for aging and surgical effects on urinary behavior, quantitative real‐time polymerase chain reaction assays were carried out. Primary data were evaluated using scatter plots and descriptive statistics.ResultsIn sham mice, urination frequency showed strong variation at the earlier post‐surgical time points (especially at 1 month), with variation decreasing with time. Quantitative real‐time polymerase chain reaction showed that the serotonin 2C receptor‐encoding mRNA accumulated to >28‐fold higher levels at 24 months compared with 3 months in intact mice. A major limitation of the quantitative real‐time polymerase chain reaction experiments was that we did not separate whole bladder into muscle and mucosa.ConclusionsAlthough a sham operation is typically used in partial bladder outlet obstruction experiments to provide control animals, the sham group might itself show increased variation in micturition frequency at early times after surgery, compared with intact animals.
Serum potassium abnormalities in chronic kidney disease: prevalence, patient characteristics and clinical outcomes
Authors: Brookes Elizabeth M. , Snider Jonathan , Hart Graeme K. , Robbins Raymond , Power David A. 
Published: December 2020

AbstractBackgroundAbnormalities in serum potassium are a well known complication of chronic kidney disease (CKD), but little is known about their impact on inpatient outcomes.AimsTo better understand the role of dyskalaemia in hospital in‐patients, we assessed the epidemiology of potassium disorders among CKD patients, and the association between admission potassium and inpatient mortality or intensive care unit (ICU) requirement.MethodsThis retrospective hospital‐based cohort study (n = 11 156) included patients with an estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2 admitted to Austin Health between 2014 and 2018 and who had an admission potassium value. Dialysis patients or those with a renal transplant were excluded. Multivariate logistic analysis was conducted to identify factors associated with hyperkalaemia (≥5.5 mmol/L) and hypokalaemia (<3.5 mmol/L). Odds ratios for inpatient mortality and ICU admission between potassium categories were obtained by multivariate regression with adjustments for demographics, renal function and comorbidities.ResultsHyperkalaemia and hypokalaemia were present in 6.86% and 2.94% of hospital admissions respectively. In multivariate regression male sex, lower eGFR, diabetes and cardiac failure were associated with higher odds of hyperkalaemia. Thiazide diuretics, loop diuretics, infectious disease and endocrine pathology were associated with higher odds of hypokalaemia. A U‐shaped association was noted between potassium and inpatient mortality. Potassium <4.0 mmol/L and ≥5.0 mmol/L was associated with increased mortality. Only patients with potassium ≥5.5 mmol/L had increased ICU admission risk.ConclusionDerangements in potassium frequently occur in CKD inpatients and are independently associated with higher mortality and ICU requirement. Further studies are required to determine whether interventions to maintain normokalaemia improve outcomes in this population.
Dental visit experience and dental care barriers among Hong Kong preschool children with special education needs
Authors: Zhou Ni , Wong Hai Ming , McGrath Colman 
Published: December 2020

AbstractBackgroundChildren with special education needs (SEN) require additional supports in education settings, as well as extra supports in healthcare services.AimTo investigate dental visit experience and dental care barriers among Hong Kong preschoolers with SEN.DesignThis was designed as a cross‐sectional study. A structured questionnaire was employed to investigate dental attendance and dental care barriers among Hong Kong preschoolers with SEN. Binary logistic regression and multi‐factor ANOVA were performed to identify factors associated with children's dental visit experience.ResultsA total of 383 children were included in the final analysis. The average age of the recruited children was 3.87 ± 0.95 years. 72.5% parents reported that dental visits were a challenging task for their children. Among children with caries, 70.7% had never visited a dentist. Regression models indicated that (a) children over 3 years or children whose parents received tertiary education were more likely to visit a dentist; (b) the existence of dental care barriers was associated with children's primary diagnosis, practical skills, conceptual skills, and parents’ education attainment; and (c) children with low conceptual skills (95%CI:0.71 to 2.40, P < .001), or children from low‐income families (95%CI:1.21 to −0.06, P = .031) had more dental care barriers than their counterparts.ConclusionUnmet dental treatment needs and dental care barriers existed among pre‐schoolers with SEN. Dental care barriers were associated with children's developmental profile, parents’ education attainment, and household income.
Making on the Move: Mobility, Makerspaces, and Art Education
Authors: Jordan Alvaro , Knochel Aaron D. , Meisel Nicholas , Reiger Kelsey , Sinha Swapnil 
Published: December 2020

AbstractCombining the excitement from the maker movement and the novel creation of deployable makerspaces, we review the development of the Mobile Atelier for Kinaesthetic Education (MAKE) 3D. MAKE 3D is a mobile makerspace platform that can be deployed anywhere there is electricity to create a curricular spectacle of digital fabrication in particular additive manufacturing or what is more commonly referred to as 3D printing. Our project combines this notion of curricular spectacle and a mobile makerspace platform, to develop strategies in how to meet the novice user almost anywhere and to entice them into a series of hands‐on activities that would give them a range of knowledge and aptitude for additive techniques in digital fabrication. We review the component parts of our Material to Form curriculum and explore thematic connections between the maker movement and art education including STEAM and interdisciplinarity; design thinking and kinaesthetic learning; and place‐based education and the mobile platform. Informal practices in art education and the mobile makerspace advances forms of place and kinaesthetic learning. Similar curricular setups are therefore encouraged to reinforce and expand prior knowledge, broaden participation and provide an adaptable learning space for STEAM initiatives.
Expression levels of the metalloproteinase ADAM8 critically regulate proliferation, migration and malignant signalling events in hepatoma cells
Authors: Awan Tanzeela , Babendreyer Aaron , Mahmood Alvi Abid , Düsterhöft Stefan , Lambertz Daniela , Bartsch Jörg W. , Liedtke Christian , Ludwig Andreas 
Published: December 2020

AbstractHepatocellular carcinoma (HCC) is one of the most common metastatic tumours. Tumour growth and metastasis depend on the induction of cell proliferation and migration by various mediators. Here, we report that the A Disintegrin and Metalloproteinase (ADAM) 8 is highly expressed in murine HCC tissues as well as in murine and human hepatoma cell lines Hepa1‐6 and HepG2, respectively. To establish a dose‐dependent role of different ADAM8 expression levels for HCC progression, ADAM8 expression was either reduced via shRNA‐ or siRNA‐mediated knockdown or increased by using a retroviral overexpression vector. These two complementary approaches revealed that ADAM8 expression levels correlated positively with proliferation, clonogenicity, migration and matrix invasion and negatively with apoptosis of hepatoma cells. Furthermore, the analysis of pro‐migratory and proliferative signalling pathways revealed that ADAM8 expression level was positively associated with expression of β1 integrin as well as with the activation of focal adhesion kinase (FAK), mitogen‐activated protein kinase (MAPK), Src kinase and Rho A GTPase. Finally, up‐regulation of promigatory signalling and cell migration was also seen with a proteolytically inactive ADAM8 mutant. These findings reveal that ADAM8 is critically up‐regulated in hepatoma cells contributes to cell proliferation and survival and furthermore induces pro‐migratory signalling pathways independently of its proteolytic activity. By this, ADAM8 can promote cell functions most relevant for HCC growth and metastasis.
The cost of travel: How dispersal ability limits local adaptation in host–parasite interactions
Authors: Johnson Pieter , Calhoun Dana M. , Moss Wynne E. , McDevitt‐Galles Travis , Riepe Tawni B. , Hallas Joshua M. , Parchman Thomas L. , Feldman Chris R. , Achatz Tyler J. , Tkach Vasyl V. , Cropanzano Josh , Bowerman Jay , Koprivnikar Janet 
Published: December 2020

AbstractClassical theory suggests that parasites will exhibit higher fitness in sympatric relative to allopatric host populations (local adaptation). However, evidence for local adaptation in natural host–parasite systems is often equivocal, emphasizing the need for infection experiments conducted over realistic geographic scales and comparisons among species with varied life history traits. Here, we used infection experiments to test how two trematode (flatworm) species (Paralechriorchis syntomentera and Ribeiroia ondatrae) with differing dispersal abilities varied in the strength of local adaptation to their amphibian hosts. Both parasites have complex life cycles involving sequential transmission among aquatic snails, larval amphibians and vertebrate definitive hosts that control dispersal across the landscape. By experimentally pairing 26 host‐by‐parasite population infection combinations from across the western USA with analyses of host and parasite spatial genetic structure, we found that increasing geographic distance—and corresponding increases in host population genetic distance—reduced infection success for P. syntomentera, which is dispersed by snake definitive hosts. For the avian‐dispersed R. ondatrae, in contrast, the geographic distance between the parasite and host populations had no influence on infection success. Differences in local adaptation corresponded to parasite genetic structure; although populations of P. syntomentera exhibited ~10% mtDNA sequence divergence, those of R. ondatrae were nearly identical (<0.5%), even across a 900 km range. Taken together, these results offer empirical evidence that high levels of dispersal can limit opportunities for parasites to adapt to local host populations.
The health‐related quality of life and psychological profile in patients with oropharyngeal Pemphigus Vulgaris in complete clinical remission: A case‐control study
Authors: Calabria Elena , Adamo Daniela , Leuci Stefania , Pecoraro Giuseppe , Coppola Noemi , Aria Massimo , Mignogna Michele D. 
Published: December 2020

AbstractBackgroundPemphigus Vulgaris (PV) is a severe autoimmune blistering disease which may affect the patient's health‐related quality of life (HR‐QoL) and mood even during quiescent disease activity. We sought to evaluate HR‐QoL, quality of sleep (QoS), anxiety and depression in oropharyngeal PV patients (OPV) in complete clinical remission on or off therapy (CCR‐on, CCR‐off).MethodsThirty OPV patients and 30 healthy controls were enrolled. The Short Form 36 Health Survey Questionnaire (SF‐36), Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), Hamilton Rating Scale for Depression (HAM‐D) and Hamilton Rating Scale for Anxiety (HAM‐A) were administered. Descriptive statistics, including the Mann‐Whitney U test and hierarchical multiple linear regression analysis, were used.ResultsThe OPV patients had statistically lower scores in the majority of items of the SF‐36 and higher scores in the PSQI, HAM‐A and HAM‐D than the healthy controls (P < .004; P < .001; and P < .001 respectively). Nine (30%) of the OPV patients were poor sleepers (PSQI > 5) with higher scores in the SF‐36, HAM‐A and HAM‐D compared with the good sleepers (PSQI < 5). No statistically significant difference was detected in the OPV group when comparing patients in CCR‐on and CCR‐off, or in consideration of the cumulative time of the disease duration.ConclusionsHR‐QoL of OPV patients can be impaired even over periods of relatively well‐being, therefore, clinicians should monitor periodically their HR‐QoL, QoS and psychological profile in order to guide treatments also toward improving their HR‐QoL.
Cognitive flow in health care settings: A systematic review
Authors: McQueen Sydney , Jiang Stephanie , McParland Aidan , Hammond Mobilio Melanie , Moulton Carol‐anne 
Published: December 2020

AbstractIntroductionThe state of cognitive flow, colloquially known as being ‘in the zone’, has been linked with enhanced performance, happiness, career satisfaction and decreased burnout. However, the concept has not been adopted strongly in health care training, continuing professional development, or daily practice. A systematic review with a narrative synthesis was undertaken to map the evidence for flow in health care.MethodsA search was conducted in MEDLINE, PsycInfo, and EMBASE in July 2019 and updated in October 2020 for manuscripts discussing flow in all health care disciplines. Articles published between 1806 and 13 October 2020 were included. Two authors independently reviewed titles and abstracts (and subsequently full texts where necessary) for inclusion. Disagreements were resolved by consensus. Data were extracted on location, manuscript type, population and context, measures, and key findings.ResultsA total of 4923 unique abstracts were initially retrieved, and 15 articles were included in the final review. We report on the experience, benefits and strategies to support flow in health care. Flow may benefit providers by enhancing career enjoyment, wellness and performance, while mitigating exhaustion, burnout, and stress. Although research from other domains has focused on supporting flow through individualised training, our results highlight the importance of system and environmental factors.ConclusionsSupporting professional and trainee flow in health care requires a holistic approach, including individual training and system‐level interventions.
Orchestration of virulence factor expression and modulation of biofilm dispersal in Erwinia amylovora through activation of the Hfq‐dependent small RNA RprA
Authors: Peng Jingyu , Schachterle Jeffrey K. , Sundin George W. 
Published: December 2020

AbstractErwinia amylovora is the causative agent of the devastating disease fire blight of pome fruit trees. After infection of host plant leaves at apple shoot tips, E. amylovora cells form biofilms in xylem vessels, restrict water flow, and cause wilting symptoms. Although E. amylovora is well known to be able to cause systemic infection, how biofilm cells of E. amylovora transit from the sessile mode of growth in xylem to the planktonic mode of growth in cortical parenchyma remains unknown. Increasing evidence has suggested the important modulatory roles of Hfq‐dependent small RNAs (sRNAs) in the pathogenesis of E. amylovora. Here, we demonstrate that the sRNA RprA acts as a positive regulator of amylovoran exopolysaccharide production, the type III secretion system (T3SS), and flagellar‐dependent motility, and as a negative regulator of levansucrase activity and cellulose production. We also show that RprA affects the promoter activity of multiple virulence factor genes and regulates hrpS, a critical T3SS regulator, at the posttranscriptional level. We determined that rprA expression can be activated by the Rcs phosphorelay, and that expression is active during T3SS‐mediated host infection in an immature pear fruit infection model. We further showed that overexpression of rprA activated the in vitro dispersal of E. amylovora cells from biofilms. Thus, our investigation of the varied role of RprA in affecting E. amylovora virulence provides important insights into the functions of this sRNA in biofilm control and systemic infection.