bluuebunny/crossref_metadata_2025_split huggingface dataset:
Successful diagnosis of attention deficit hyperactivity disorder in later life of an adult with intellectual disability: A case report
Authors: Raji Oyepeju , Javaid Shafaq 
Published: February 2022

AbstractBackgroundDiagnosing and managing attention deficit hyperactivity disorder in adults with intellectual disabilities is currently largely based on findings from assessment and treatment in children or adults without intellectual disabilities. Current evidence suggests that individuals with intellectual disabilities are more likely to have attention deficit hyperactivity disorder compared to those without intellectual disabilities, but more research is needed. There is increasing knowledge of diagnosing and treating attention deficit hyperactivity disorder in people with intellectual disabilities with published guidance on delivering care based on good evidence. More is needed so that clinicians do not have to depend on the evidence from those who do not have intellectual disabilities.MethodTo write this report, we reviewed a female in her 50s with mild intellectual disability who has been known to general and specialist intellectual disability mental health services for at least 20 years over many episodes of care. We assessed her clinically and using the Diagnostic Interview for attention deficit hyperactivity disorder in Adults with intellectual disabilities to make a diagnosis of attention deficit hyperactivity disorder. We call her Mary, which is not her real name.FindingsMary has had many diagnoses however, we found that her neurodevelopmental disorders of attention deficit hyperactivity disorder and we believe, autism were missed which led to a complex presentation of behavioural and mental symptoms; and diagnostic and management challenges. Mary was receiving combinations of medications known as antipsychotics, antidepressants, hypnotics and mood stabilisers to high doses. She was able to stop them all successfully over 12–18 months before being diagnosed with attention deficit hyperactivity disorder. Once diagnosed with attention deficit hyperactivity disorder, Mary rapidly responded to a long acting stimulant as recommended by the National Institute for Health and Care Excellence and this has significantly improved her quality of life.ConclusionAttention deficit hyperactivity disorder in people with intellectual disabilities is often missed and inadequately managed, despite having highly effective and specific treatment strategies. Making the diagnoses of attention deficit hyperactivity disorder and intellectual disabilities is important as individuals with both together have worse outcomes. Therefore, effective treatment of attention deficit hyperactivity disorder in individuals with intellectual disabilities is critical.
A Survey of Tasks and Visualizations in Multiverse Analysis Reports
Authors: Hall Brian D. , Liu Yang , Jansen Yvonne , Dragicevic Pierre , Chevalier Fanny , Kay Matthew 
Published: February 2022

AbstractAnalysing data from experiments is a complex, multi‐step process, often with multiple defensible choices available at each step. While analysts often report a single analysis without documenting how it was chosen, this can cause serious transparency and methodological issues. To make the sensitivity of analysis results to analytical choices transparent, some statisticians and methodologists advocate the use of ‘multiverse analysis’: reporting the full range of outcomes that result from all combinations of defensible analytic choices. Summarizing this combinatorial explosion of statistical results presents unique challenges; several approaches to visualizing the output of multiverse analyses have been proposed across a variety of fields (e.g. psychology, statistics, economics, neuroscience). In this article, we (1) introduce a consistent conceptual framework and terminology for multiverse analyses that can be applied across fields; (2) identify the tasks researchers try to accomplish when visualizing multiverse analyses and (3) classify multiverse visualizations into ‘archetypes’, assessing how well each archetype supports each task. Our work sets a foundation for subsequent research on developing visualization tools and techniques to support multiverse analysis and its reporting.
Skin tests in the work‐up of cutaneous adverse drug reactions: A review and update
Authors: Barbaud Annick , Castagna Julie , Soria Angèle 
Published: February 2022

AbstractSkin tests, including patch tests (PTs), prick tests, and intradermal tests (IDTs), are useful in identifying the culprits of cutaneous adverse drug reactions (CADRs), and determining safer, alternative drugs. PTs have a low sensitivity but are valuable in investigating maculopapular exanthema (MPE), as well as severe CADR, including toxic epidermal necrolysis (TEN), Stevens‐Johnson syndrome (SJS), and in particular, acute generalized exanthematous pustulosis (AGEP), and drug reaction with eosinophilia and systemic symptoms (DRESS). To ensure their specificity, at least 10 control tests should be performed. Prick tests are mainly used in the evaluation of immediate‐type hypersensitivity and can be performed with all drugs, except opiates. IDTs can be used to explore immediate and delayed‐type hypersensitivity, if an injectable form of the drug exists. Except for SJS/TEN, IDTs should be performed by injecting 0.02 mL of the drug. We here provide a practical, up‐to‐date review on the use of these skin tests in the work‐up of CADRs. Numerous negative controls for drug PTs, as well as criteria for the immediate and delayed positivity of prick tests and IDT, are included. It should be emphasized that a negative result never excludes the potential responsibility of a drug in a CADR.
PIPS: A Parallel Planning Model of Sentence Production
Authors: Brehm Laurel , Cho Pyeong Whan , Smolensky Paul , Goldrick Matthew A. 
Published: February 2022

AbstractSubject–verb agreement errors are common in sentence production. Many studies have used experimental paradigms targeting the production of subject–verb agreement from a sentence preamble (The key to the cabinets) and eliciting verb errors (… *were shiny). Through reanalysis of previous data (50 experiments; 102,369 observations), we show that this paradigm also results in many errors in preamble repetition, particularly of local noun number (The key to the *cabinet). We explore the mechanisms of both errors in parallelism in producing syntax (PIPS), a model in the Gradient Symbolic Computation framework. PIPS models sentence production using a continuous‐state stochastic dynamical system that optimizes grammatical constraints (shaped by previous experience) over vector representations of symbolic structures. At intermediate stages in the computation, grammatical constraints allow multiple competing parses to be partially activated, resulting in stable but transient conjunctive blend states. In the context of the preamble completion task, memory constraints reduce the strength of the target structure, allowing for co‐activation of non‐target parses where the local noun controls the verb (notional agreement and locally agreeing relative clauses) and non‐target parses that include structural constituents with contrasting number specifications (e.g., plural instead of singular local noun). Simulations of the preamble completion task reveal that these partially activated non‐target parses, as well the need to balance accurate encoding of lexical and syntactic aspects of the prompt, result in errors. In other words: Because sentence processing is embedded in a processor with finite memory and prior experience with production, interference from non‐target production plans causes errors.
The Role of Stimulus‐Specific Perceptual Fluency in Statistical Learning
Authors: Perfors Andrew , Kidd Evan 
Published: February 2022

AbstractHumans have the ability to learn surprisingly complicated statistical information in a variety of modalities and situations, often based on relatively little input. These statistical learning (SL) skills appear to underlie many kinds of learning, but despite their ubiquity, we still do not fully understand precisely what SL is and what individual differences on SL tasks reflect. Here, we present experimental work suggesting that at least some individual differences arise from stimulus‐specific variation in perceptual fluency: the ability to rapidly or efficiently code and remember the stimuli that SL occurs over. Experiment 1 demonstrates that participants show improved SL when the stimuli are simple and familiar; Experiment 2 shows that this improvement is not evident for simple but unfamiliar stimuli; and Experiment 3 shows that for the same stimuli (Chinese characters), SL is higher for people who are familiar with them (Chinese speakers) than those who are not (English speakers matched on age and education level). Overall, our findings indicate that performance on a standard SL task varies substantially within the same (visual) modality as a function of whether the stimuli involved are familiar or not, independent of stimulus complexity. Moreover, test–retest correlations of performance in an SL task using stimuli of the same level of familiarity (but distinct items) are stronger than correlations across the same task with stimuli of different levels of familiarity. Finally, we demonstrate that SL performance is predicted by an independent measure of stimulus‐specific perceptual fluency that contains no SL component at all. Our results suggest that a key component of SL performance may be related to stimulus‐specific processing and familiarity.
When the “Tabula” is Anything but “Rasa:” What Determines Performance in the Auditory Statistical Learning Task?
Authors: Elazar Amit , Alhama Raquel G. , Bogaerts Louisa , Siegelman Noam , Baus Cristina , Frost Ram 
Published: February 2022

AbstractHow does prior linguistic knowledge modulate learning in verbal auditory statistical learning (SL) tasks? Here, we address this question by assessing to what extent the frequency of syllabic co‐occurrences in the learners’ native language determines SL performance. We computed the frequency of co‐occurrences of syllables in spoken Spanish through a transliterated corpus, and used this measure to construct two artificial familiarization streams. One stream was constructed by embedding pseudowords with high co‐occurrence frequency in Spanish (“Spanish‐like” condition), the other by embedding pseudowords with low co‐occurrence frequency (“Spanish‐unlike” condition). Native Spanish‐speaking participants listened to one of the two streams, and were tested in an old/new identification task to examine their ability to discriminate the embedded pseudowords from foils. Our results show that performance in the verbal auditory SL (ASL) task was significantly influenced by the frequency of syllabic co‐occurrences in Spanish: When the embedded pseudowords were more “Spanish‐like,” participants were better able to identify them as part of the stream. These findings demonstrate that learners’ task performance in verbal ASL tasks changes as a function of the artificial language's similarity to their native language, and highlight how linguistic prior knowledge biases the learning of regularities.
Knowledge in Sight: Toddlers Plan Efficient Epistemic Actions by Anticipating Learning Gains
Authors: Aguirre Marie , Brun Mélanie , Couderc Auriane , Reboul Anne , Senez Philomène , Mascaro Olivier 
Published: February 2022

AbstractAnticipating the learning consequences of actions is crucial to plan efficient information seeking. Such a capacity is needed for learners to determine which actions are most likely to result in learning. Here, we tested the early ontogeny of the human capacity to anticipate the amount of learning gained from seeing. In study 1, we tested infants’ capacity to anticipate the availability of sight. Fourteen‐month‐old infants (N = 72) were invited to search for a toy hidden inside a container. The participants were faster to attempt at opening a shutter when this action allowed them to see inside the container. Moreover, this effect was specifically observed when seeing inside the container was potentially useful to the participants’ goals. Thus, infants anticipated the availability of sight, and they calibrated their information‐seeking behaviors accordingly. In studies 2 and 3, we tested toddlers’ capacity to anticipate whether data would be cognitively useful for their goals. Two‐and‐a‐half‐year‐olds (N = 72) had to locate a target character hidden among distractors. The participants flipped the characters more often, and were comparatively faster to initiate this action when it yielded access to visual data allowing them to locate the target. Thus, toddlers planned their information‐seeking behaviors by anticipating the cognitive utility of sight. In contrast, toddlers did not calibrate their behaviors to the cognitive usefulness of auditory data. These results suggest that cognitive models of learning guide toddlers’ search for information. The early developmental onset of the capacity to anticipate future learning gains is crucial for active learning.
Definition, prevalence, and clinical significance of mitral annular disjunction in different patient cohorts: A systematic review
Authors: Bennett Sadie , Tafuro Jacopo , Duckett Simon , Appaji Anik , Khan Jamal Nasir , Heatlie Grant , Cubukcu Arzu , Kwok Chun Shing 
Published: February 2022

AbstractBackgroundMitral annular disjunction (MAD) is a structural abnormality involving a distinct separation of the left atrium/mitral valve annulus and myocardium continuum. The literature around MAD has increased over recent years, thus we sought to review the current data on the definition, prevalence, and clinical outcomes of MAD.MethodsA search of MEDLINE and EMBASE was conducted to identify studies which evaluated MAD in any patient cohort. The study results were synthesized narratively.ResultsA total of 12 studies were included with 3925 patients (average age 62 years, 63% male). The pooled prevalence of MAD in patients with mitral valve prolapse and/or Barlow's disease was 30.1%. In a general population, MAD prevalence was 8.7%. The definition of MAD was not consistent across all studies. In terms of clinical outcomes, only one study reported MAD to be associated with ventricular arrhythmias.ConclusionsMAD is an increasingly recognized finding amongst patients undergoing cardiac imaging. This review highlights the need for agreed definitions for clinically significant MAD and how identified MAD should be managed. At present, there is insufficient evidence that MAD is associated adverse clinical outcomes.
Incidence and socioeconomic factors in older adults with acute myeloid leukaemia: Real‐world outcomes from a population‐based cohort
Authors: Stubbins Ryan J. , Stamenkovic Maria , Roy Claudie , Rodrigo Judith , Chung Shanee , Kuchenbauer Florian C. , Hay Kevin A. , White Jennifer , Abou Mourad Yasser , Power Maryse M. , Narayanan Sujaatha , Forrest Donna L. , Toze Cynthia L. , Sutherland Heather J. , Nantel Stephen H. , Nevill Thomas J. , Karsan Aly , Song Kevin W. , Sanford David S. 
Published: February 2022

AbstractObjectivesAcute myeloid leukaemia (AML) is a disease of older adults, who are vulnerable to socio‐economic factors. We determined AML incidence in older adults and the impact of socio‐economic factors on outcomes.MethodsWe included 3024 AML patients (1996–2016) identified from a population‐based registry.ResultsAML incidence in patients ≥60 years increased from 11.01 (2001–2005) to 12.76 (2011–2016) per 100 000 population. Among 879 patients ≥60 years in recent eras (2010–2016), rural residents (<100 000 population) were less likely to be assessed by a leukaemia specialist (39% rural, 47% urban, p = .032); no difference was seen for lower (43%, quintile 1–3) vs. higher (47%, quintile 4–5) incomes (p = .235). Similar numbers received induction chemotherapy between residence (16% rural, 18% urban, p = .578) and incomes (17% lower, 17% high, p = 1.0). Differences between incomes were seen for hypomethylating agent treatment (14% low, 20% high, p = .041); this was not seen for residence (13% rural, 18% urban, p = .092). Among non‐adverse karyotype patients ≥70 years, 2‐year overall survival was worse for rural (5% rural, 12% urban, p = .006) and lower income (6% low, 15% high, p = .017) patients.ConclusionsAML incidence in older adults is increasing, and outcomes are worse for older rural and low‐income residents; these patients face treatment barriers.
Interplays between changing biophysical and social dynamics under climate change: Implications for limits to sustainable adaptation in food systems
Authors: Bezner Kerr Rachel , Naess Lars Otto , Allen‐O’Neil Bridget , Totin Edmond , Nyantakyi‐Frimpong Hanson , Risvoll Camilla , Rivera Ferre Marta G. , López‐i‐Gelats Feliu , Eriksen Siri 
Published: February 2022

AbstractClimate change scenarios have significant implications for the livelihoods and food security of particular groups in society and will necessitate a range of adaptation actions. While there is a significant literature on the social as well as biophysical factors and limits to adaptation, less is known about the interactions between these, and what such interactions mean for the prospects of achieving sustainable and resilient food systems. This paper is an attempt at addressing this gap by examining changing biophysical and social factors, with specific consideration of vulnerable groups, across four case studies (Ghana, Malawi, Norway and Spain). In each case, future climate change scenarios and associated biophysical limits are mapped onto four key social factors that drive vulnerability and mediate adaptation, namely, scale, history, power and politics, and social differentiation. We then consider what the interaction between biophysical limits and socio‐political dynamics means for the options for and limits to future adaptation, and how climate may interact with, and reshape, socio‐political elements. We find that biophysical limits and socio‐political factors do not operate in isolation, but interact, with dynamic relationships determining the ‘space’ or set of options for sustainable adaptation. By connecting the perspectives of biophysical and social factors, the study illuminates the risks of unanticipated outcomes that result from the disregard of local contexts in the implementation of adaptation measures. We conclude that a framework focusing on the space for sustainable adaptation conditioned by biophysical and social factors, and their interactions, can help provide evidence on what does and does not constitute sustainable adaptation, and help to counter unhelpful narratives of climate change as a sole or dominant cause of challenges in food systems.
Living with a genetic, undiagnosed or rare disease: A longitudinal journalling study through the COVID‐19 pandemic
Authors: Byun Malia , Feller Hollie , Ferrie Monica , Best Stephanie 
Published: February 2022

AbstractIntroductionCOVID‐19 changed the way we lived with uncertainty from the outset as the pandemic impacted every aspect of our lives from well‐being, socializing to accessing healthcare. For people in vulnerable populations, such as those with genetic, undiagnosed and rare disorders, the experience was heightened.AimThe aim of this study was to identify how the rapidly changing COVID‐19 environment impacted the lives of the Genetic, Undiagnosed and Rare Disease community.MethodsFrom June 2020 to May 2021, we collected monthly open‐ended journals from people living in Australia with genetic, undiagnosed and rare disorders. Data analysis was deductive, using the Resilience Scale for Adults, and inductive using thematic analysis.ResultsWe recruited 29 people (average of n = 9.7 submissions each month). Responses changed over the year, with initial journals focusing on the importance of developing new structures for day‐to‐day lives, while later journals started to focus on mental well‐being. Throughout the project, participants reported challenges in accessing health and social care that was compounded by fear and concern over being exposed to the virus. Later journals highlight inconsistent messaging for vaccinations for this vulnerable community.Discussion/ConclusionIn parallel with the waves of the COVID‐19 pandemic, there need to be waves of targeted support for vulnerable communities. The first support wave needs to focus on facilitating the identification of new frameworks to structure day‐to‐day lives. A later second wave needs to focus on mental well‐being and coping with isolation, while consistent communication relating to health and social care throughout was essential.Patient/Public ContributionThis study was co‐designed, co‐led and analysed with a patient support network.
CDX2 expression in malignant peripheral nerve sheath tumour: a potential diagnostic pitfall associated with PRC2 inactivation
Authors: Odeyemi Olumide O , Ozawa Michael G , Charville Gregory W 
Published: February 2022

AimsMalignant peripheral nerve sheath tumour (MPNST) is a soft tissue sarcoma that exhibits features of Schwann cell differentiation. Heterologous, often mesenchymal‐type differentiation occurs in a subset of MPNST, while glandular morphology also is encountered in rare cases. We observed in MPNST unanticipated expression of CDX2, a transcription factor that regulates intestinal epithelial differentiation, and aimed to further characterize this phenomenon.Methods/ResultsExpression of CDX2 was assessed by immunohistochemistry in a total of 32 high‐grade MPNSTs lacking morphological evidence of epithelial differentiation, including twelve tumours (38%) that developed in the setting of neurofibromatosis and four (13%) in the setting of prior radiation therapy. CDX2 was expressed by 14 of 32 MPNSTs (44%), wherein immunoreactivity, varying from weak to strong, was present in 2–95% of neoplastic spindle cells (median 10%, mean 23%). Notably, CDX2 expression was limited to tumours with PRC2 inactivation (22/32; 69%), as evidenced immunohistochemically by diffuse loss of trimethylated histone H3K27. Analysing publicly available RNA‐sequencing data from twelve MPNST cell lines, two of which are clonally related, we observed CDX2 expression in all six PRC2‐inactivated cell lines, while CDX2 expression was negligible in six cell lines with intact PRC2, amounting to a 58‐fold increase in CDX2 expression on average with PRC2 inactivation.ConclusionsCDX2 is expressed in a subset of MPNSTs, even in the absence of morphological evidence of epithelial differentiation. CDX2 expression in MPNST is strongly associated with underlying PRC2 inactivation.
Substitution of antioxidant Si by andalusite fine powder in Al2O3‐SiC‐C castables
Authors: Fu Yunhe , Niu Zheng , Mu Yuandong , Hao Zhanhong , Wang Qingfeng , Parr Chris , Ye Guotian 
Published: February 2022

AbstractOur previous research showed that the addition of andalusite aggregates can enhance the oxidation resistance of Al2O3‐SiC‐C castables; this is attributed to the transformation of andalusite into a silica‐rich glass phase that blocks pores. Theoretically, andalusite with a smaller particle size would achieve a higher extent of conversion and generate more silica‐rich glass, which would be more conducive to blocking pores. Therefore, this study evaluates the influence of andalusite fine powder on the oxidation resistance of Al2O3‐SiC‐C castables containing a reduced amount of Si antioxidant. The results show that introducing a small amount of andalusite fine powder endows the Al2O3‐SiC‐C castables with significant oxidation resistance so that the traditional addition of Si metal as an antioxidant can be substantially reduced or even completely eliminated. Meanwhile, the thermal shock stability and strength of the castables were also enhanced.