bluuebunny/crossref_metadata_2025_split huggingface dataset:
Scope of Outcomes in Trials and Observational Studies of Interventions Targeting Medication Adherence in Rheumatic Conditions: A Systematic Review
Authors: Kelly Ayano , Crimston-Smith Luke , Tong Allison , Bartlett Susan J. , Bekker Charlotte L. , Christensen Robin , De Vera Mary A. , de Wit Maarten , Evans Vicki , Gill Michael , March Lyn , Manera Karine , Nieuwlaat Robby , Salmasi Shahrzad , Scholte-Voshaar Marieke , Singh Jasvinder A. , Sumpton Daniel , Toupin-April Karine , Tugwell Peter , van den Bemt Bart , Verstappen Suzanne , Tymms Kathleen 
Published: December 2019

Objective.Nonadherence to medications is common in rheumatic conditions and associated with increased morbidity. Heterogeneous outcome reporting by researchers compromises the synthesis of evidence of interventions targeting adherence. We aimed to assess the scope of outcomes in interventional studies of medication adherence.Methods.We searched electronic databases to February 2019 for published randomized controlled trials and observational studies of interventions with the primary outcome of medication adherence including adults with any rheumatic condition, written in English. We extracted and analyzed all outcome domains and adherence measures with prespecified extraction and analysis protocols.Results.Overall, 53 studies reported 71 outcome domains classified into adherence (1 domain), health outcomes (38 domains), and adherence-related factors (e.g., medication knowledge; 32 domains). We subdivided adherence into 3 phases: initiation (n = 13 studies, 25%), implementation (n = 32, 60%), persistence (n = 27, 51%), and phase unclear (n = 20, 38%). Thirty-seven different instruments reported adherence in 115 unique ways (this includes different adherence definitions and calculations, metric, and method of aggregation). Forty-one studies (77%) reported health outcomes. The most frequently reported were medication adverse events (n = 24, 45%), disease activity (n = 11, 21%), bone turnover markers/physical function/quality of life (each n = 10, 19%). Thirty-three studies (62%) reported adherence-related factors. The most frequently reported were medication beliefs (n = 8, 15%), illness perception/medication satisfaction/satisfaction with medication information (each n = 5, 9%), condition knowledge/medication knowledge/trust in doctor (each n = 3, 6%).Conclusion.The outcome domains and adherence measures in interventional studies targeting adherence are heterogeneous. Consensus on relevant outcomes will improve the comparison of different strategies to support medication adherence in rheumatology.
Association Between Vitamin D Deficiency and Disease Activity, Disability, and Radiographic Progression in Early Rheumatoid Arthritis: The ESPOIR Cohort
Authors: Mouterde Gaël , Gamon Etienne , Rincheval Nathalie , Lukas Cédric , Seror Raphaele , Berenbaum Francis , Dupuy Anne-Marie , Daien Claire , Daurès Jean-Pierre , Combe Bernard 
Published: December 2019

Objective.To evaluate the association of baseline serum level of vitamin D with disease activity, disability, and radiographic damage over the first year in early rheumatoid arthritis (RA).Methods.Among early arthritis patients included in the ESPOIR cohort, patients with early RA were evaluated. Levels of 25-hydroxy vitamin D2 and D3 were measured at baseline. Baseline associations between vitamin D level and 28-joint count Disease Activity Score based on erythrocyte sedimentation rate (DAS28-ESR), Health Assessment Questionnaire–Disability Index (HAQ-DI), and van der Heijde modified total Sharp score (mTSS) were assessed. Bivariate analysis was used to assess the association between vitamin D level and radiographic progression (mTSS increased by ≥ 1 point) or disability (HAQ-DI ≥ 0.5) over 12 months. Forward stepwise multiple logistic regression was used to evaluate the independent association of baseline variables and outcomes.Results.Among 813 patients with early arthritis, data for 645 patients with RA were analyzed. Vitamin D level was < 10 ng/mL (deficiency, group 1), 10–29.9 ng/mL (low level, group 2), and ≥ 30 ng/mL (normal, group 3) for 114 (17.7%), 415 (64.54%), and 114 (17.7%) patients, respectively. At baseline, DAS28-ESR and HAQ-DI were higher with vitamin D deficiency compared with groups 2 and 3 combined (P = 0.007 and P = 0.001, respectively), as was mean mTSS, but not significantly (p = 0.076). On multivariate analysis, baseline vitamin D deficiency was associated with HAQ-DI at 6 months (OR 1.70) and mTSS at 12 months (OR 1.76).Conclusion.Vitamin D deficiency was associated with more active and severe disease at baseline and may predict disability and radiographic progression over 1 year in early RA patients. [ClinicalTrials.gov: NCT03666091]
Large-vessel Vasculitis Affecting the Aorta and its Branches in Relapsing Polychondritis: Case Series and Systematic Review of the Literature
Authors: Tomelleri Alessandro , Campochiaro Corrado , Sartorelli Silvia , Papa Maurizio , De Luca Giacomo , Cavalli Giulio , Baldissera Elena , Dagna Lorenzo 
Published: December 2019

ObjectiveTo describe the features of large-vessel vasculitis (LVV) as it affects the aorta and its branches in patients with relapsing polychondritis (RP).MethodsRetrospective data and systematic literature review.ResultsTwenty-one patients were identified. LVV diagnosis was subsequent to RP and associated with extrachondral involvement in the majority of patients. Supraaortic vessels were more frequently involved (82%). Fourteen patients (67%) were treated with a conventional synthetic disease-modifying antirheumatic drug (csDMARD) and 7 (33%) with a biological DMARD (bDMARD). Vascular interventional procedures were performed in 10 patients (48%). Premature death due to cardiovascular complications was reported in 3 cases (14%).ConclusionExtraaortic LVV is a serious and overlooked RP manifestation. All patients with RP should be investigated for LVV.
Role of Neutrophil Extracellular Traps Regarding Patients at Risk of Increased Disease Activity and Cardiovascular Comorbidity in Systemic Lupus Erythematosus
Authors: Moore Stanley , Juo Hsin-Hsuan , Nielsen Christoffer T. , Tyden Helena , Bengtsson Anders A. , Lood Christian 
Published: December 2019

ObjectiveNeutrophil extracellular traps (NET) are essential in host defense, but are also linked to inflammation and autoimmunity, including in systemic lupus erythematosus (SLE). We recently described that immune complexes (IC) induce NET formation, promoting SLE-like disease in mice. In the current study, we investigated, for the first time to our knowledge, the role of NET in human SLE and their association with disease activity and severity.MethodsLevels of NET (myeloperoxidase-DNA complexes) were analyzed in plasma from 4 cross-sectional SLE cohorts (n = 44–142), 1 longitudinal SLE cohort (n = 47), and healthy individuals (n = 100) using ELISA. Type I interferon activity was determined using a cell reporter system.ResultsPatients with SLE had elevated levels of NET in circulation compared to healthy controls (p < 0.01). NET levels identified patients with a severe disease phenotype characterized by IC-driven nephritis (p < 0.05). Though not associated with current disease activity (p = 0.20), levels of NET were associated with future increase in the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) within 3 months (OR 1.75, p = 0.01), as well as an overall heightened SLEDAI over 1 year (p < 0.01). Finally, levels of NET were associated with arterial events (OR 5.0, p = 0.02) and endothelial cell activation (p < 0.001).ConclusionNET levels are elevated in patients with SLE, associated with IC-driven disease. NET levels provide significant clinical value in identifying patients at risk of active disease and/or severe disease, including nephritis and cardiovascular disease, and may allow for early interventions.
Nostalgia, novelty, and the subversion of authority in "The Testaments of the Twelve Patriarchs"
Authors: De Bruin Tom 
Published: December 2019

The Testaments of the Twelve Patriarchs, by negotiating the authorship and authority of its derivative readings, discusses the place of Israel vis-à-vis Christianity through almost fannish retellings of the lives of the patriarchs of Israel. The text thereby walks a line between nostalgic and novel readings of foundational narratives, in some places perpetuating canonical authority and in others subverting it. The outcome of this interplay is the displacement of the Israelite author and Christianization of Israelite history. Contemporary fan fiction studies discourse provides tools for analyzing this negotiation of textual authority.
Navigating Catholicism and queerness in "Daredevil" fan works
Authors: Griffin Alexandria 
Published: December 2019

In Daredevil (2015–18) fan works, fans confront the character of Daredevil, who is portrayed in the Netflix TV series of the same name as explicitly religious, namely Catholic. This means that fan works seeking to explore sexuality, especially same-sex sexuality, must confront Catholicism's prohibition on extramarital sex, including same-sex sexual relations. I examine how fan works use Daredevil as a site from which to examine and debate Catholicism and queerness.
Queering the Anglo-Saxons through their psalms
Authors: Mussies Martine 
Published: December 2019

For fan fiction based on the TV serial drama The Last Kingdom (2014–), some fan fiction authors use fragments of the translations of the Psalms by King Alfred of Wessex (849–899) to firmly ground their stories in the historical reimagination of the Anglo-Saxons. In the short story "Æthelflaed and Lagertha," fan fiction writer Bandi Crawford uses an Alfredian psalm to connect The Last Kingdom to another major TV series, Vikings (2013–). By developing bisexual and biromantic story lines along the lines of Alfredian psalms, the author constructs a twenty-first-century neomedieval-based culture in which the Alfredian psalms are reinterpreted or critically reexamined through a queer lens, thereby negotiating more diversity within a favorite show's story world.
Increasing the targeting scope and efficiency of base editing with Proxy‐BE strategy
Authors: Liu Yin , Li Guanglei , Yang Guang , Gu Huifeng , Huang Shisheng , Yu Wenxia , Qin Guizhen , Liu Xinyi , Zhou Fuling , Huang Xingxu , Wei Yongchang 
Published: December 2019

Base editors (BEs) are widely used in precise gene editing due to their simplicity and versatility. However, their efficiencies are hindered by various obstacles. Considering the chromatin microenvironment as a possible obstacle, here, we demonstrate a further development of the proxy‐clustered regularly interspaced short palindromic repeats strategy, termed Proxy‐BE, to increase gene editing efficiency. Specifically, a nuclease‐dead Cas9 (dCas9) was bound to the sequence about 20–30 base pair away from the target site, potentially improving access to the DNA and, thus, providing a better editing microenvironment for base editors. Our findings confirm that nuclease‐dead Streptococcus pyogenes Cas9 can assist the base editors SaKKH‐BE3 and dCpf1‐BE to double their canonical base editing efficiency. This work provides a new approach to enhance base editing, extending its scope for biological research and gene therapy.
Radical‐Cation‐Induced Crossed [2+2] Cycloaddition of Electron‐Deficient Anetholes Initiated by Iron(III) Salt
Authors: Horibe Takahiro , Katagiri Kei , Ishihara Kazuaki 
Published: December 2019

AbstractRadical cation‐induced crossed [2+2] cycloaddition of electron‐deficient anetholes was developed with the use of Fe(OTf)3 as an initiator. Various 1,2‐diarylcyclobutanes can be synthesized in high yields with high diastereoselectivity from electron‐deficient anetholes, which have been less explored using conventional methods. This practical reaction system can be used for a decagram‐scale reaction (12 gram), in which the resulting product can be transformed to 1,2‐dicarbonyl compounds.magnified image
An improved subgrid scale model for front‐tracking based simulations of mass transfer from bubbles
Authors: Claassen Claire M. Y. , Islam Shafiul , Peters E. A. J. F. (Frank) , Deen Niels G. , Kuipers J. A. M. (Hans) , Baltussen Maike W. 
Published: December 2019

AbstractGas–liquid bubble column reactors are often used in industry because of their favorable mass transfer characteristics. The bubble mass boundary layer in these systems is generally one order of magnitude thinner than the momentum boundary. To resolve it in simulations, a subgrid scale model will account for the sharp concentration variation in the vicinity of the interface. In this work, the subgrid scale model of Aboulhasanzadeh et al., Chem Eng Sci, 2012, 75:456–467 embedded in our in‐house front tracking framework, has been improved to prevent numerical mass transfer due to remeshing operations. Furthermore, two different approximations of the mass distribution in the boundary layer have been tested. The local and global predicted Sherwood number has been verified for mass transfer from bubbles in the creeping and potential flow regimes. In addition, the correct Sherwood number has been predicted for free rising bubbles at several Eötvös and Morton numbers with industrial relevant Schmidt numbers (103–105).
Hand morphometrics, electrodermal activity, and stone tools haptic perception
Authors: Fedato Annapaola , Silva‐Gago María , Terradillos‐Bernal Marcos , Alonso‐Alcalde Rodrigo , Martín‐Guerra Elena , Bruner Emiliano 
Published: December 2019

AbstractObjectivesTool use requires integration among sensorial, biomechanical, and cognitive factors. Taking into account the importance of tool use in human evolution, changes associated with the genus Homo are to be expected in all these three aspects. Haptics is based on both tactile and proprioceptive feedbacks, and it is associated with emotional reactions. Previous analyses have suggested a difference between males and females, and during haptic exploration of different typologies of stone tools. Here, we analyze the correlation between electrodermal reactions during stone tool handling and hand morphology to provide evidence of possible allometric factors shared by males and females.MethodsElectrodermal analysis was used to investigate some specific parameters involved in these reactions, such as changes in the level of attention and arousal. We analyzed the responses of 46 right‐handed adults to 20 distinct stone tools while blindfolded.ResultsFemales have smaller hands and a wider range of electrodermal reactions. Within males and females, hand diameters and general hand size do not correlate with the degree of electrodermal level and response.ConclusionsSex differences in electrodemal reaction during stone tool handling are apparently not due to the effect of hand size or proportions. Differences between males and females are better interpreted as real sex differences, either due to a biological or cultural influences. Hand size does not influence the degree of arousal or attention during tool exploration, suggesting that other factors trigger individual reactions. These results add to a general cognitive approach on hand‐tool evolution and tool sensing.
Low digit ratio (2D:4D) is associated with early natural menopause
Authors: Kirchengast Sylvia , Dottolo Elisabeth , Praxmarer Elisa , Huber Johannes 
Published: December 2019

AbstractObjectivesIntrauterine environmental conditions may affect the number of primordial follicles and in this way the timing of menopause. The aim of the present study was to investigate association patterns between right hand digit ratio, that is, 2D:4D ‐ as an indicator of prenatal androgen and estrogen exposure, and age at menopause.MethodsOne hundred sixty‐nine women, who had experienced natural menopause, were enrolled in the study. Length of second and fourth finger were measured directly from the palmar side and digit ratios of both hands were calculated. For further analyses the digit ratio of the right hand was used only. Additionally, smoking habits, body weight and body height, body mass index and the number of children were determined. Multiple regression analyses were used to test association patterns between digit ratio and age at menopause, body height, BMI, nicotine consumption as well as number of births and age at menopause.ResultsAge at menopause correlated significantly positively with the digit ratio. A more feminine digit ratio is associated with a higher age at menopause, while a low digit ratio, interpreted as a hint of a higher androgen exposure during prenatal phase was associated with a lower age at menopause.ConclusionsLow digit ratio is associated with an earlier onset of natural menopause.
Cardiac manifestations and gene mutations of patients with RASopathies in Taiwan
Authors: Lee Chung‐Lin , Tan Louis Tan Hock‐Cheong , Lin Hsiang‐Yu , Hwu Wuh‐Liang , Lee Ni‐Chung , Chien Yin‐Hsiu , Chuang Chih‐Kuang , Wu Mei‐Hwan , Wang Jou‐Kou , Chu Shao‐Yin , Lin Ju‐Li , Lo Fu‐Sung , Su Pen‐Hua , Hsu Chia‐Chi , Ko Yu‐Yuan , Chen Ming‐Ren , Chiu Hui‐Ching , Lin Shuan‐Pei 
Published: December 2019

AbstractRASopathies are developmental diseases caused by mutations in rat sarcoma–mitogen‐activated protein kinase pathway genes. These disorders, such as Noonan syndrome (NS) and NS‐related disorders (NSRD), including cardio‐facio‐cutaneous (CFC) syndrome, Costello syndrome (CS), and NS with multiple lentigines (NSML; also known as LEOPARD syndrome), have a similar systemic phenotype. A wide spectrum of congenital heart disease and hypertrophic cardiomyopathy (HCMP) can exhibit major associated characteristics. A retrospective study was conducted at the Mackay Memorial Hospital, National Taiwan University Hospital, Buddhist Tzu‐Chi General Hospital, Chang‐Gung Memorial Hospital, Taichung Veterans General Hospital, and Chung Shan Medical University Hospital from January 2007 to December 2018. We reviewed the clinical records of 76 patients with a confirmed molecular diagnosis of RASopathies, including NS, CS, CFC syndrome, and NSML. We evaluated the demographic data and medical records with clinical phenotypes of cardiac structural anomalies using cross‐sectional and color Doppler echocardiography, electrocardiographic findings, and follow‐up data. A total of 47 (61.8%) patients had cardiac abnormalities. The prevalence of cardiac lesions according to each syndrome was 62.7, 50.0, 60.0, and 66.7% in patients with NS, CFC syndrome, CS, and NSML, respectively. An atrial septal defect was usually combined with other cardiac abnormalities, such as pulmonary stenosis (PS), HCMP, ventricular septal defect, or patent ductus arteriosus. Patients with NS most commonly showed PS. In patients with NSRD and cardiac abnormalities, HCMP (29.4%) was the most commonly observed cardiac lesion. PTPN11 was also the most frequently detected mutation in patients with NS and NSRD. Cardiac abnormalities were the most common symptoms observed in patients with RASopathies at the time of their first hospital visit. Performing precise analyses of genotype–cardiac phenotype correlations in a larger cohort will help us accurately diagnose RASopathy as soon as possible.
Gain‐of‐function pathogenic variants in SMAD4 are associated with neoplasia in Myhre syndrome
Authors: Lin Angela E. , Alali Abdulrazak , Starr Lois J. , Shah Nidhi , Beavis Anna , Pereira Elaine M. , Lindsay Mark E. , Klugman Susan 
Published: December 2019

AbstractMyhre syndrome is an increasingly diagnosed rare syndrome that is caused by one of two specific heterozygous gain‐of‐function pathogenic variants in SMAD4. The phenotype includes short stature, characteristic facial appearance, hearing loss, laryngotracheal stenosis, arthritis, skeletal abnormalities, learning and social challenges, distinctive cardiovascular defects, and a striking fibroproliferative response in the ear canals, airways, and serosal cavities (peritoneum, pleura, pericardium). Confirmation of the clinical diagnosis is usually prompted by the characteristic appearance with developmental delay and autistic‐like behavior using targeted gene sequencing or by whole exome sequencing. We describe six patients (two not previously reported) with molecularly confirmed Myhre syndrome and neoplasia. Loss‐of‐function pathogenic variants in SMAD4 cause juvenile polyposis syndrome and we hypothesize that the gain‐of‐function pathogenic variants observed in Myhre syndrome may contribute to neoplasia in the patients reported herein. The frequency of neoplasia (9.8%, 6/61) in this series (two new, four reported patients) and endometrial cancer (8.8%, 3/34, mean age 40 years) in patients with Myhre syndrome, raises the possibility of cancer susceptibility in these patients. We alert clinicians to the possibility of detecting this syndrome when cancer screening panels are used. We propose that patients with Myhre syndrome are more susceptible to neoplasia, encourage increased awareness, and suggest enhanced clinical monitoring.
Three‐dimensional analysis of sexual dimorphism in the soft tissue morphology of the upper airways in a human population
Authors: Bastir Markus , Megía Irene , Torres‐Tamayo Nicole , García‐Martínez Daniel , Piqueras Francisco M. , Burgos Manuel 
Published: December 2019

AbstractObjectivesSeveral studies have analyzed the sexual dimorphism of the skeletal cranial airways. This study aimed to quantify the three‐dimensional (3D) morphology of the soft tissues of the upper airways in a human population. We addressed hypotheses about morphological features related to respiratory and energetic aspects of nasal sexual dimorphism.MethodsWe reconstructed 3D models of 41 male and female soft tissue nasal airways from computed tomography data. We measured 280 landmarks and semilandmarks for 3D‐geometric morphometric analyses to test for differences in size and 3D morphology of different functional compartments of the soft tissue airways.ResultsWe found statistical evidence for sexual dimorphism: Males were larger than females. 3D features indicated taller and wider inflow tracts, taller outflow tracts and slightly taller internal airways in males. These characteristics are compatible with greater airflow in males.DiscussionThe differences in 3D nasal airway morphology are compatible with the respiratory‐energetics hypothesis according to which males differ from females because of greater energetic demands. Accordingly, structures related to inflow and outflow of air show stronger signals than structures relevant for air‐conditioning.