bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mimics of Erdheim–Chester disease
Authors: Abeykoon Jithma P. , Ravindran Aishwarya , Rech Karen L. , Young Jason R. , Oliver Tobin W. , Shah Mithun V. , Nora Bennani N. , Vassallo Robert , Ryu Jay H. , Koster Matthew J. , Davidge‐Pitts Caroline J. , Goyal Gaurav , Go Ronald S. ,   the Mayo Clinic‐University of Alabama at Birmingham Histiocytosis Working Group
Published: November 2021

SummaryErdheim–Chester disease (ECD) is a rare histiocytic neoplasm with frequent multiorgan involvement. An accurate diagnosis of ECD requires the correlation of clinical features, histopathologic and radiologic findings. We describe a case series of patients with a referral diagnosis of ECD, whereby the diagnosis was changed to non‐histiocytic diseases after comprehensive review at a tertiary care center. This accurate revision of the referral diagnosis of ECD enabled initiation of proper disease‐directed therapy in a timely manner for these patients and avoided unnecessary exposure to systemic cytotoxic chemotherapy or targeted agents. Our study highlights the value of a multidisciplinary team of histiocytosis experts in confirming the diagnosis of ECD and also brings attention to other conditions to consider that can mimic ECD, including osteopoikilosis, tenosynovial giant cell tumour, IgG4‐related disease, fibrous dysplasia and chronic recurrent multifocal osteomyelitis.
“@Government There’s a pothole in my street!”: Canadian citizens’ adoption choices of social media use in citizen‐government relations
Authors: Homburg Vincent , Moody Rebecca 
Published: November 2021

AbstractSocial media platforms such as Twitter and Facebook offer new opportunities for co‐production and interaction between citizens and government agencies. Until now, explanations of why citizens use social media to interact with government have been lacking in the literature. This article concludes on the basis of survey data gathered among Canadian citizens that social media use in citizen‐government relations is explained by citizens’ perceived effectiveness and trust in social media organizational infrastructure, with trust in government, social media anxiety and ease of use not having an impact. Implications for research and practice are discussed.
Prevalence of developmental delay among children: A national study based on administrative database of the single‐payer National Health Insurance System in Taiwan
Authors: Ho Li‐Po , Lee Yue‐Chune 
Published: November 2021

AbstractBackgroundDevelopmental delay (DD) indicates a failure to meet the developmental milestones of most children of the same age. Studies based solely on the ICD coding manual may underestimate the prevalence of DD. Real‐world use of rehabilitation data may be useful in the identification of more DD children previously undiagnosed with DD.AimThe aim of this study is to estimate the prevalence of DD among children aged 0–6 by age and sex in Taiwan based on modified ICD codes.MethodsA list of ICD codes specific to DD (including delays and disabilities requiring early intervention) was generated from the existing ICD codebook and modified based on National Health Insurance (NHI) claims data pertaining to developmental rehabilitation of children aged 0–6. The validity of the codes was subsequently assessed by DD experts in various fields using the consensus development technique. The resulting list was used to estimate the prevalence of DD among children in Taiwan from 2000 to 2015 based on analysis of longitudinal NHI data.ResultBetween 2000 and 2015, the prevalence of DD among children aged 0 to 6 years increased from 2.0% to 5.7%, and the sex ratio was 181–197 males per 100 females. The prevalence estimate obtained in this study (5.6%) was 229% higher than existing government statistics (1.6%) published in 2014.ConclusionsThe codes developed using claims data in this study can be used to estimate the prevalence of DD among children and evaluate the effectiveness of intervention programmes. Consistent increases in the prevalence of DD indicate that efforts to promote early intervention have been effective. Nonetheless, the low prevalence rate among 0–2 years children with developmental delay and low prevalence rate of female CWDD means that the policy should notice the lack of access to healthcare services for infants and female children and produce a more equitable or fair distribution of healthcare resources.
Intertriginous mycosis fungoides with T follicular helper cell phenotype progressing to Sézary syndrome
Authors: Amir Ali A. , Shameli A. , Zhang C. , Gniadecki R. , Street L. , Hardin J. 
Published: November 2021

AbstractThis case report highlights the challenges in diagnosis and therapeutic options for an individual who initially presented with intertriginous mycosis fungoides with a T follicular helper cell phenotype, which later evolved to Sézary syndrome.
Prevalence of pouchitis in both ulcerative colitis and familial adenomatous polyposis: A systematic review and meta‐analysis
Authors: Sriranganathan Danujan , Kilic Yakup , Nabil Quraishi Mohammed , Segal Jonathan P. 
Published: November 2021

AbstractAimPouchitis is a clinically significant complication of ileal pouch–anal anastomosis with its prevalence varying in the literature. Pouchitis is thought to occur more commonly in patients with ulcerative colitis (UC) than in patients with familial adenomatous polyposis (FAP). We conducted a systematic review with meta‐analysis of all published literature to report the prevalence of pouchitis in all pouch patients as well as specifically in UC and FAP. We also investigated the prevalence of acute and chronic pouchitis in UC and FAP.MethodsA meta‐analysis was conducted by searching the Embase, Embase Classic and PubMed databases between 1978 and 2021 exploring the prevalence of pouchitis in UC and FAP. We then performed a random effects model in order to find the pooled prevalence of pouchitis and used odds ratios to report differences in the prevalence of pouchitis in UC and FAP.ResultsFifty‐nine full papers and conference abstracts were included in the meta‐analysis comprising 18 117 patients with UC and 860 with FAP. The prevalence of pouchitis in UC was 0.32 (95% CI 0.27–0.37) and in FAP was found to be 0.06 (95% CI 0.03–0.15). The odds ratio of pouchitis in UC patients compared with FAP patients was 4.95 (95% CI 3.17–7.72,P < 0.0001).ConclusionsOur findings support the consensus that the prevalence of pouchitis is higher in UC than in FAP. More significantly our findings suggest that the true prevalence of pouchitis is higher than commonly reported in the literature. This literature may help counsel patients prior to undergoing restorative proctocolectomy.
Kinetics of myelin breakdown products: A labeling study in patients with progressive multiple sclerosis
Authors: Kanhai Kawita M. S. , Goulooze Sebastiaan C. , van der Grond Jeroen , Harms Amy C. , Hankemeier Thomas , Verma Ajay , Dent Gersham , Chavez Juan , Meijering Henri , Groeneveld Geert Jan 
Published: November 2021

AbstractThe majority of disease modifying therapies for multiple sclerosis (MS) reduce inflammation, but do no’t target remyelination. Development of remyelinating therapies will benefit from a method to quantify myelin kinetics in patients with MS. We labeled myelin in vivo with deuterium, and modeled kinetics of myelin breakdown products β‐galactosylceramide (β‐GalC) and N‐Octadecanoyl‐sulfatide (NO‐Sulf). Five patients with MS received 120 ml 70% D2O daily for 70 days and were compared with six healthy subjects who previously received the same procedure. Mass spectrometry and compartmental modeling were used to quantify the turnover rate of β‐GalC and NO‐Sulf in cerebrospinal fluid (CSF). Turnover rate constants of the fractions of β‐GalC and NO‐Sulf with non‐negligible turnover were 0.00186 and 0.00714, respectively, in both healthy subjects and patients with MS. The turnover half‐life of β‐GalC and NO‐Sulf was calculated as 373 days and 96.5 days, respectively. The effect of MS on the NO‐Sulf (49.4% lower fraction with non‐negligible turnover) was more pronounced compared to the effect on β‐GalC turnover (18.3% lower fraction with non‐negligible turnover). Kinetics of myelin breakdown products in the CSF are different in patients with MS compared with healthy subjects. This may be caused by slower myelin production in these patients, by a higher level of degradation of a more stable component of myelin, or, most likely, by a combination of these two processes. Labeling myelin breakdown products is a useful method that can be used to quantify myelin turnover in patients with progressive MS and can therefore be used in proof‐of‐concept studies with remyelination therapies.
Pharmacokinetics of bempedoic acid in patients with renal impairment
Authors: Amore Benny M. , Sasiela William J. , Ries Daniel K. , Tresh Perry , Emery Maurice G. 
Published: November 2021

AbstractBempedoic acid is an ATP citrate lyase inhibitor approved for the treatment of hypercholesterolemia. The objective of this phase I study was to assess the pharmacokinetics (PKs) and safety of bempedoic acid in 24 subjects with normal renal function or mild, moderate, or severe renal impairment. All subjects received a single oral bempedoic acid 180‐mg dose and PK parameters were monitored for up to 23 days. Resulting estimates of area under the concentration‐time curve exposure following bempedoic acid treatment were 1.5‐fold, 2.2‐fold, and 2.2‐fold higher in subjects with mild, moderate, or severe renal impairment, respectively, compared with subjects with normal renal function. With decreases in renal function, plasma free fraction was increased up to 20.1%, whereas total and unbound clearances were decreased by 55.2% and 62.6%, respectively, in subjects with severe renal impairment relative to those with normal renal function. These observed decreases in total and unbound oral clearance in subjects with decreased renal function are not explained by the increases in free fraction and might therefore also be attributable to changes in bioavailability or intrinsic clearance. Bempedoic acid was generally well‐tolerated and the incidence and type of adverse events were not affected by the degree of renal impairment. In conclusion, bempedoic acid exposures in subjects with renal impairment were increased up to approximately two‐fold with no safety signals identified, consistent with findings in phase III patients with mild or moderate renal impairment. No dose adjustments are necessary for patients with mild or moderate renal impairment.
Developmental differences in the prospective organisation of goal‐directed movement between children with autism and typically developing children: A smart tablet serious game study
Authors: Chua Yu Wei , Lu Szu‐Ching , Anzulewicz Anna , Sobota Krzystof , Tachtatzis Christos , Andonovic Ivan , Rowe Philip , Delafield‐Butt Jonathan 
Published: November 2021

AbstractMovement is prospective. It structures self‐generated engagement with objects and social partners and is fundamental to children's learning and development. In autistic children, previous reports of differences in movement kinematics compared to neurotypical peers suggest that its prospective organisation might be disrupted. Here, we employed a smart tablet serious game paradigm to assess differences in the feedforward and feedback mechanisms of prospective action organisation, between autistic and neurotypical preschool children. We analysed 3926 goal‐directed finger movements made during smart‐tablet ecological gameplay, from 28 children with Childhood Autism (ICD‐10; ASD) and 43 neurotypical children (TD), aged 3–6 years old. Using linear and generalised linear mixed‐effect models, we found the ASD group executed movements with longer movement time (MT) and time to peak velocity (TTPV), lower peak velocity (PV), with PV less likely to occur in the first movement unit (MU) and with a greater number of movement units after peak velocity (MU‐APV). Interestingly, compared to the TD group, the ASD group showed smaller increases in PV, TTPV and MT with an increase in age (ASD × age interaction), together with a smaller reduction in MU‐APV and an increase in MU‐APV at shorter target distances (ASD × Dist interaction). Our results are the first to highlight different developmental trends in anticipatory feedforward and compensatory feedback mechanisms of control, contributing to differences in movement kinematics observed between autistic and neurotypical children. These findings point to differences in integration of prospective perceptuomotor information, with implications for embodied cognition and learning from self‐generated action in autism.
Development of gross motor capacity and mobility performance in children with cerebral palsy: a longitudinal study
Authors: Burgess Andrea , Reedman Sarah , Chatfield Mark D , Ware Robert S , Sakzewski Leanne , Boyd Roslyn N 
Published: November 2021

AimTo describe development of gross motor capacity and mobility performance in children with cerebral palsy.MethodThis longitudinal cohort study measured gross motor capacity with the Gross Motor Function Measure and mobility performance with the Pediatric Evaluation of Disability Inventory (PEDI) between 18 months and 5 years, and the PEDI – Computer Adaptive Test (PEDI‐CAT) between 8 years and 12 years. Longitudinal analyses used mixed‐effects regression modelling with interaction between age and Gross Motor Function Classification System (GMFCS). Stability of GMFCS levels over time was measured using agreement.ResultsTwo hundred and twenty‐two children provided 871 observations (median 4 observations, range 1–7). Children classified in GMFCS level I improved in both capacity and performance until 8 to 12 years. Children classified in GMFCS levels II and III continued to develop mobility performance after gross motor capacity had plateaued at 5 years. Children classified in GMFCS level IV plateaued at 5 years in capacity and performance. Children classified in GMFCS level V showed no changes in capacity or performance between 18 months and 8 to 12 years. Stability of GMFCS levels was 73%.InterpretationChange in mobility performance over time somewhat reflected gross motor capacity trajectories. Continued improvement in mobility performance after plateau of gross motor capacity for children classified in GMFCS levels II and III suggests importance of other personal or environmental factors.
Alterations in gut microbiota and elevated serum bilirubin in primary biliary cholangitis patients treated with ursodeoxycholic acid
Authors: Han Weijia , Huang Chunyang , Zhang Qi , Tao Shuhui , Hu Xiaomin , Xu Jianguo , Jiang Ronglong , Xu Bin , Liu Yanmin , Hou Jinlin 
Published: November 2021

AbstractBackgroundThe prognosis of primary bile cholangitis (PBC) is linked to gut microbiota dysbiosis. This study investigated the association between the gut microbiome and elevated total bilirubin (TB) level in PBC patients treated with ursodeoxycholic acid (UCDA).MethodsA total of 47 PBC patients with 12 months of UCDA treatment were enrolled. Patients were divided into the TB (+) (TB>1× upper limit of the normal range [ULN]; n = 20) and TB(−) (TB≤1× ULN; n = 27) groups. Stool and serum specimens were collected, and microbiota composition and functional characteristics in the 2 groups were evaluated by 16S RNA gene sequencing and bioinformatic analysis.ResultsBacterial diversity was lower in the TB(+) group than in the TB(−) group, although there was no significant difference in bacterial community profile. The phylum Saccharibacteria showed differential abundance in the 2 groups. Meanwhile, the TB(−) group had lower abundance of the Gemmiger, Blautia, Anaerostipes and Coprococcus genera than the TB(+) group, whereas Holdemania was absent. The abundance of Gemmiger formicillis and Coprococcus eutactus was positively correlated with that of Faecalibacterium prausnitzii, while Blautia, Anaerostipes and Coprococcus were negatively correlated with total bile acid level.ConclusionTB level in PBC patients treated for 12 months with UCDA is associated with a distinct gut microbiome profile.
Compensating communities for industrial disamenities: The case of shale gas development
Authors: Harleman Max 
Published: November 2021

AbstractGovernments often compensate communities for hosting disruptive industries. Sometimes compensation comes with restrictions that preclude highly‐valued investments. I exploit policy discontinuities at the Pennsylvania‐Ohio border to understand how restrictions affect local investment. Ohio delivers unrestricted revenues to schools and municipalities with shale development. Pennsylvania leaves out schools, and requires that municipalities address the industry's impacts. Municipalities in both states save most of the revenues. Ohio schools leverage them to increase borrowing and finance capital investments. This suggests that affected residents have greater demand for school investments, and that broad use of compensation may benefit communities more than allocating it narrowly.
Aphis spiraecola and Aphis (Toxoptera) citricidus differently manipulate plant metabolism to gain fitness in terms of population abundance or dispersal
Authors: Gao Jing , Xiu Baolin , Sun Ziqiang , Arthurs Steve , Guo Honggang , Gu Shimin , Long Jianzhao , Xia ChuanGuo , Hussain Mubasher , Mao Runqian 
Published: November 2021

AbstractThe response of plant cultivars to infestation by single aphid species has been widely reported, whereas the response of plants to multiple aphid species, and the mechanisms by which various aphids benefit from the induced changes, has been largely ignored. Here we analyzed the symptoms and physiological responses of sweet orange, Citrus sinensis (L.) Osbeck (Rutaceae), as affected by Aphis spiraecola Patch and Aphis (Toxoptera) citricidus (Kirkaldy) (Hemiptera: Aphididae). We further compared the feeding behavior, population abundance, and proportion of winged morph of the two aphid species when separately feeding on sweet orange. Results showed that feeding by A. spiraecola, but not A. citricidus, caused leaf curling, reduced shoot length, shoot fresh weight, and chlorophyll concentration. In addition, A. spiraecola feeding increased the concentration of essential amino acids in leaves when compared with A. citricidus and uninfested control plants. Electrical penetration graph‐studies showed that A. citricidus gained faster access and acceptance of phloem, and spent more time ingesting phloem sap, than A. spiraecola. Furthermore, after 2 weeks of infestation, A. spiraecola produced more winged morphs, whereas A. citricidus achieved higher population abundance. Thus, our results indicate that the two citrus aphids utilize divergent strategies to manipulate host metabolism, and gain fitness benefits in population abundance or winged form production. The results could improve our understanding of varied adaptive strategies of co‐existing insects and extend our knowledge of aphid‐plant interactions.
Testing three hypotheses of rarity among the Buprestidae species of a tropical dry forest
Authors: Reyes‐González Roberto , Toledo‐Hernández Víctor Hugo , Flores‐Palacios Alejandro , Rös Matthias , Bueno‐Villegas Julián , Corona‐López Angélica María 
Published: November 2021

AbstractMany insect communities have a higher proportion of rare species than predicted by community‐structure models. Rarity has been associated with increased extinction risk, therefore, it is essential to understand which factors cause rarity patterns.The importance of three rarity hypotheses (under‐sampling, diffusive rarity, and body size) in a Buprestidae community sampled with active and passive methods are tested in a tropical dry forest in central Mexico.In total, 222 species were recorded. Doubletons and duplicates decreased with the increase of sampled locations, while singletons and uniques increased. At the local level, 67% of the species were no longer rare when all collection methods were considered (n = 60 species), as well as 41% at the regional level (n = 41 species). The diffusive rarity hypothesis was tested in 97 species that appeared in more than two localities; 38% of the species changed their abundance status between localities. The body size hypothesis was tested considering 179 species, and rare species were smaller only in the species of the genusChrysobothris.More than one rarity hypothesis operated simultaneously in a community, but their contribution was different. In the Buprestidae community studied, the under‐sampling hypothesis had the greatest support, affecting a higher proportion of species. Subsequently, the second most important was the diffusive rarity hypothesis, and the least support showed the body size hypothesis, as the size was determined mainly by the taxonomic genus.
Correcting whole‐body motion capture data using rigid body transformation
Authors: Miyakoshi Makoto 
Published: November 2021

AbstractUsing motion capture data as a part of mobile brain–body imaging (MoBI) recording has been increasing. With minimal linear algebra background, this paper explains how the rigid body transformation can be a useful preprocessing step for denoising and missing marker recovery. Such a transformation can provide insight and necessary‐and‐sufficient solutions requiring no assumption other than the minimum number of markers present. First, a simulation test using the empirical datasets from the AudioMaze project published from this journal's same volume demonstrates theoretical accuracy. The simulation results show that the rigid‐body method perfectly recovers missing markers on a rigid body if a minimum of three marker positions is available. Second, the same transformation is applied to the empirical dataset. Before preprocessing, the raw data show that 15–80% of data frames had all markers present for rigid‐body defined body parts. After using the rigid‐body correction, most body parts recovered full markers in 90–95% of the data frames. The result also suggests the necessity for performing across‐trial corrections for within‐participant (42% missing detected in one of the body parts) and across‐participants (11% missing). The discussion section introduces a solution and a performance summary for non‐rigid‐body marker correction using a neural network. Data support that the rigid body transformation is an intuitive and powerful correction method necessary for preprocessing motion capture data for neurocognitive experiments. The supporting information section contains a URL link to Matlab code and example data.