bluuebunny/crossref_metadata_2025_split huggingface dataset:
Frontier and hotspot evolution in Wiskott–Aldrich syndrome: A bibliometric analysis from 2001 to 2021
Authors: Liu Shixu , Yao Xiaoyan , Xia Kun , Zhang Jinzhi , Liu Yanyi , Xia Xiao , Li Guangxi 
Published: December 2022

Background:
Wiskott–Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder. Despite our enormous progress in the strategies used to diagnose, treat, and cure WAS, no bibliometric studies have been performed in this research field. This study explored the trends in WAS research through a bibliometric analysis evaluating relevant literature quantitatively and qualitatively.

Methods:
The literature concerning WAS from 2001 to 2021 was retrieved from the Science Citation Index Expanded (SCI-expanded) of the Web of Science Core Collection database. Acquired data were then visually analyzed using CiteSpace and VOSviewer.

Results:
2036 papers were included in the final analysis. The annual publication outputs reached its peak in 2013 but declined in recent years. The dominant position of the United States in WAS research was quite obvious. Harvard University (USA), University College London (UK), and Inserm (France) were the three most prolific institutions. Adrian J. Thrasher exerted significant publication impact and made the most notable contributions in the field of WAS. Blood was the most influential journal with the highest publication outputs, and nearly all the top 10 journals and co-cited journals belonged to Q1. Immune dysregulation, thrombocytopenia, syndrome protein deficiency, stem cell, mutation, and diagnosis were the keywords with the strongest citation burst.

Conclusion:
From 2001 to 2021, the United States was a global leader in the WAS research. Collaboration between countries and institutions is expected to deepen and strengthen in the future. Research hotspots included pathogenesis, clinical manifestations, diagnosis, and therapy. Our results suggest a greater understanding of the mechanistic underpinnings of immune dysfunction in WAS patients, the application of targeted therapies for individual complications, and the development of curative approaches, which will remain research hotspots in the future.
Efficacy and safety of acetyl-CoA carboxylase (ACC) inhibitors in the treatment of nonalcoholic steatohepatitis (NASH): A protocol for systematic review
Authors: Xu Liubin , Yang Huili , Xu Hongling , Yang Rong , Fen Lian , Jiang Dansheng , Xu Linyi , Xing Yufeng 
Published: December 2022

Background:
The pathological mechanism of nonalcoholic steatohepatitis (NASH) is closely related to abnormal lipid regulation in hepatocytes. Patients with NASH generally have a significant increase in de novo lipogenesis, which acetyl-CoA carboxylase (ACC) catalyzes the first committed step. However, the treatment with ACC inhibitors remains controversial. Thus, our study will systematically evaluate the efficacy and safety of ACC inhibitors for the treatment of NASH.

Methods:
We plan to search PubMed, Cochrane Library, Web of Science, EMBASE, Google Scholar, ClinicalTrials.gov, China Science and Technology Journal Database, Chinese Biomedical Literature Database, Wan-fang Database and China National Knowledge Infrastructure to obtain literatures from January 2015 to January 2030 under the inclusion and exclusion criteria, and include randomized controlled trials containing intervention of ACC inhibitors for NASH. The proportion of patients with reduction in ballooning, inflammation and fibrosis will be accepted as the main outcome. RoB 2 will be used for the risk of bias, as well as Egger’s test and funnel plot for reporting bias. We will adopt Review Manager 5.4.1 for data synthesis, subgroup analysis, meta-regression analysis and sensitivity analysis, and conduct trial sequential analysis and quality of evidence evaluation using trial sequential analysis 0.9.5.10 Beta software and GRADE Profiler 3.6.1 software respectively.

Results:
This systematic review will assess the proportion of patients with reduction of ballooning, inflammation and fibrosis, changes in hepatic steatosis, levels of liver enzymes and liver injury markers, metabolic parameters, safety and tolerability to measure the clinical benefits of ACC inhibitors for NASH.

Conclusion:
The conclusion of this systematic review will achieve convincing evidence to evaluate the efficacy and safety of ACC inhibitors for NASH.
Antibody response durability following three-dose coronavirus disease 2019 vaccination in people with HIV receiving suppressive antiretroviral therapy
Authors: Lapointe Hope R. , Mwimanzi Francis , Cheung Peter K. , Sang Yurou , Yaseen Fatima , Speckmaier Sarah , Barad Evan , Moran-Garcia Nadia , Datwani Sneha , Duncan Maggie C. , Kalikawe Rebecca , Ennis Siobhan , Young Landon , Ganase Bruce , Omondi F. Harrison , Umviligihozo Gisele , Dong Winnie , Toy Junine , Sereda Paul , Burns Laura , Costiniuk Cecilia T. , Cooper Curtis , Anis Aslam H. , Leung Victor , Holmes Daniel , DeMarco Mari L. , Simons Janet , Hedgcock Malcolm , Prystajecky Natalie , Lowe Christopher F. , Romney Marc G. , Barrios Rolando , Guillemi Silvia , Brumme Chanson J. , Montaner Julio S.G. , Hull Mark , Harris Marianne , Niikura Masahiro , Brockman Mark A. , Brumme Zabrina L. 
Published: December 2022

Background:
Limited data exist regarding longer term antibody responses following three-dose coronavirus disease 2019 (COVID-19) vaccination, and the impact of a first SARS-CoV-2 infection during this time, in people with HIV (PWH) receiving suppressive antiretroviral therapy (ART). We quantified wild-type-specific, Omicron BA.1-specific and Omicron BA.5-specific responses up to 6 months post-third dose in 64 PWH and 117 controls who remained COVID-19-naive or experienced their first SARS-CoV-2 infection during this time.

Design:
Longitudinal observational cohort.

Methods:
We quantified wild-type-specific and Omicron-specific anti-Spike receptor-binding domain IgG concentrations, ACE2 displacement activities and live virus neutralization at 1, 3 and 6 months post-third vaccine dose.

Results:
Third doses boosted all antibody measures above two-dose levels, but BA.1-specific responses remained significantly lower than wild-type-specific ones, with BA.5-specific responses lower still. Serum IgG concentrations declined at similar rates in COVID-19-naive PWH and controls post-third dose (median wild-type-specific and BA.1-specific half-lives were between 66 and 74 days for both groups). Antibody function also declined significantly yet comparably between groups: 6 months post-third dose, BA.1-specific neutralization was undetectable in more than 80% of COVID-19 naive PWH and more than 90% of controls. Breakthrough SARS-CoV-2 infection boosted antibody concentrations and function significantly above vaccine-induced levels in both PWH and controls, though BA.5-specific neutralization remained significantly poorer than BA.1 even post-breakthrough.

Conclusion:
Following three-dose COVID-19 vaccination, antibody response durability in PWH receiving ART is comparable with controls. PWH also mounted strong responses to breakthrough infection. Due to temporal response declines, however, COVID-19-naive individuals, regardless of HIV status, would benefit from a fourth dose within 6 months of their third.
Osteoid Osteoma Mimicking Arthritis
Authors: Corrêa Diogo Goulart , Costa Flavia Martins 
Published: December 2022

Abstract
Intra-articular osteoid osteoma may simulate arthritis, due to the intra-articular presence of prostaglandin, which leads to synovitis, joint effusion, pain, and high local temperature. Also, intra-articular osteoid osteoma may present with minimum or no cortical thickening. Therefore, a high suspicion is needed for a correct and early diagnosis. Perfusion weighted imaging, such as dynamic contrast-enhanced imaging, can aid in the localization of the tumor nidus, seen as an early arterial-phase focal enhancement after the gadolinium injection, with fast washout, as a result of its hypervascularity.
COVID-19 Elective Surgery Shutdown and Operative Experience for Graduating Plastic Surgery Independent Residents
Authors: Loughran Atlee , McMackin Katherine , Tolaymat Besher , Jarrett Nicole 
Published: December 2022

IntroductionThe initial wave of the COVID-19 pandemic led to a temporary shutdown of elective procedures. The purpose of this study is to evaluate how the elective surgery shutdown impacted case log numbers for graduating plastic surgery independent residents.MethodsThe Accreditation Council of Graduate Medical Education graduate self-reported case logs were retrospectively compiled for graduating independent track plastic surgery residents for the year before the pandemic (2019), 4 months into the pandemic (2020) and 16 months into the pandemic (2021). Procedures were grouped into resident review committee area and totals for each area were used for analysis. Average number of cases and standard deviations were compiled. Percent change was calculated comparing each year to the prepandemic baseline. Statistical significance was determined with Studentttests comparing average number of cases. Coefficients of variation were calculated to assess for changes in interprogram variation. Average case numbers were then compared with minimum case requirements necessary for graduation.ResultsThe Accreditation Council of Graduate Medical Education graduation case log statistics were available for 238 independent residents. There were no statistically significant differences in average case number for graduating independent residents for total reconstructive breast, trunk, hand and upper extremity, or total reconstructive procedures between graduation years. The average case number of total lower extremity reconstruction increased from 2019 to 2020 (average = 83–97.4,P= 0.02). The average total number of breast aesthetic cases increased between all years (average = 92, 98, 114), with the average increase from 2019 to 2021 being statistically significant (P= 0.02). Differences in head and neck aesthetic deformities and trunk and extremity deformities between years were not statistically significant. The average case number for all graduating plastic surgery independent residents exceeded the minimum case log requirements.ConclusionsThe temporary hold on elective procedures during the pandemic decreased the opportunity for plastic surgery trainees to meet minimum case log requirements. Case log data for graduating plastic surgery independent residents demonstrate that despite the temporary suspension, the pandemic did not greatly impact the average resident case numbers.
MONOCYTIC MYELOID-DERIVED SUPPRESSOR CELL EXPANSION AFTER CARDIAC SURGERY WITH CARDIOPULMONARY BYPASS INDUCES LYMPHOCYTE DYSFUNCTION
Authors: Lesouhaitier Mathieu , Uhel Fabrice , Gregoire Murielle , Gacouin Arnaud , Frerou Aurélien , Gaudriot Baptiste , Bendavid Claude , Boukthir Sarrah , Le Tulzo Yves , Verhoye Jean-Philippe , Flecher Erwan , Roussel Mikael , Tarte Karin , Tadié Jean-Marc 
Published: December 2022

ABSTRACT
Cardiac surgery with cardiopulmonary bypass (CPB) is associated with an immune paresis that predisposes to the development of postoperative infections and sepsis. Among factors responsible for CPB-induced immunosuppression, circulating myeloid-derived suppressor cells (MDSCs) have been found to induce early lymphocyte apoptosis and lymphocyte proliferation inhibition. However, the mechanisms involved are not fully understood. In this study, we found that the main lymphocyte subsets decreased significantly 24 h after cardiac surgery with CBP. As expected, cardiac surgery with CPB induced a monocytic MDSC expansion associated with an increased T-cell apoptosis and decreased proliferation capacity. Noteworthy, granulocytic MDSCs remain stable. Myeloid-derived suppressor cell depletion restored the ability of T-cell to proliferate ex vivo. After CPB, indoleamine 2,3-dioxygenase activity and IL-10 plasma level were increased such as programmed death-ligand 1 monocytic expression, whereas plasma level of arginine significantly decreased. Neither the inhibition of indoleamine 2,3-dioxygenase activity nor the use of anti–programmed death-ligand 1 or anti–IL-10 blocking antibody restored the ability of T-cell to proliferate ex vivo. Only arginine supplementation restored partially the ability of T-cell to proliferate.
Risk of Retention After OnabotulinumtoxinA Injection for Overactive Bladder in a Diverse Urban Population With High BMI and Comorbidity Rates
Authors: Aalami Harandi Arshia , Nauheim Jennifer , Abraham Nitya E. 
Published: December 2022

Importance
OnabotulinumtoxinA (BTX-A) injection is a third-line therapy for overactive bladder (OAB). Patients undergoing treatment have a 6.5% chance of requiring clean intermittent catheterization (CIC) due to postoperative urinary retention (POUR).

Objective
The aim of this study was to evaluate risk factors for retention after BTX-A injections for OAB to decrease treatment hesitancy and enhance shared decision-making.

Study Design
This is a retrospective review of patients who underwent BTX-A injection for OAB at an urban university hospital between November 2015 and January 2021. Patients with neurogenic OAB, incomplete follow-up, or concomitant vaginal surgery were excluded. The primary outcome was POUR requiring CIC or postvoid residual volume (PVR) >200 mL at follow-up visit. Secondary outcomes included number of BTX-A injections, interval to reinjection, and whether patients reported symptom improvement.

Results
Our sample (N = 185) was 84% female with high prevalence of obesity (50%) and diabetes (32%). Twenty-one (11.4%) met the criteria for POUR with 17 (9.2%) requiring CIC. The retention and nonretention groups were similar in age, sex, smoking history, body mass index (BMI), hemogloblin A1c, and birth history, but significantly differed in preoperative PVR. Logistic regression revealed that patients with preoperative PVR 100+ mL had nearly 4-fold higher odds of POUR compared with patients with PVR less than 50 mL (odds ratio, 3.77; 95% confidence interval, 1.08–16.19).

Conclusions
Our findings reassure patients with high BMI, as well as their physicians, that retention after BTX-A injection is not associated with BMI. However, patients presenting with high baseline PVR (100 + mL) should be counseled regarding a potentially higher risk of retention after BTX-A injection.
Efficacy and Safety of Vibegron for the Treatment of Overactive Bladder in Women: A Subgroup Analysis From the Double-Blind, Randomized, Controlled EMPOWUR Trial
Authors: Newman Diane K. , Thomas Elizabeth , Greene Heather , Haag-Molkenteller Cornelia , Varano Susann 
Published: December 2022

Importance
The international phase 3 EMPOWUR trial demonstrated efficacy and safety of vibegron, a newer β3-adrenergic receptor agonist, in adults with overactive bladder (OAB). Women are disproportionately affected by OAB, especially those with bothersome symptoms, such as urge urinary incontinence (UUI).

Objective
This subgroup analysis from EMPOWUR assessed efficacy and safety of vibegron in women.

Study Design
In EMPOWUR, patients with OAB were randomized 5:5:4 to 12 weeks of treatment with once-daily vibegron 75 mg, placebo, or tolterodine 4-mg extended release. Efficacy end points included change from baseline at week 12 in mean daily number of micturitions, UUI episodes, and urgency episodes. Safety was assessed through adverse events (AEs).

Results
Of the patients included in the analysis, 1286 (84.9%) were women (vibegron, n = 463; placebo, n = 459; tolterodine, n = 364). At week 12, women receiving vibegron showed significant reductions (95% confidence intervals of least squares mean differences does not include 0) from baseline versus placebo in mean daily micturitions, UUI episodes, and urgency episodes, with least squares mean differences (95% confidence intervals) of −0.5 (−0.8 to −0.2), −0.7 (−1.0 to −0.4), and −0.8 (−1.3 to −0.4), respectively. Treatment-emergent AE incidence was similar with vibegron (39%) and placebo (35%); the most common AE with incidence higher with vibegron (4.3%) than placebo (2.6%) was headache.

Conclusions
In this subgroup analysis, women receiving vibegron showed significant reductions in key efficacy end points versus placebo and favorable safety profile, consistent with the overall results from EMPOWUR, suggesting that vibegron is efficacious and safe for the treatment of OAB in this patient population.
Evidence-Informed, Interdisciplinary, Multidimensional Action Plan to Advance Overactive Bladder Research and Treatment Initiatives: Directives From State-of-the-Science Conference on Overactive Bladder and Cognitive Impairment
Authors: Sanses Tatiana V.D. , Zillioux Jacqueline , High Rachel A. , Dengler Katherine L. , Ackenbom Mary F. , DuBeau Catherine E. , Alperin Marianna , Wagg Adrian , Birder Lori A. , Dumoulin Chantale , Bavendam Tamara 
Published: December 2022

Abstract
This article outlines an evidence-informed, interdisciplinary, multidimensional, comprehensive action plan for the American Urogynecologic Society to improve care of women with overactive bladder (OAB) while minimizing treatment-related adverse events, including cognitive impairment. It is a “call to action” to advance basic, translational, and clinical research and summarizes initiatives developed at the State-of-the-Science Conference on OAB and Cognitive Impairment to (1) develop framework for a new OAB treatment approach in women, (2) define research gaps and future research priorities, (3) champion health equity and diversity considerations in OAB treatment, (4) foster community and promote education to remove stigma surrounding OAB and urinary incontinence, and (5) elevate visibility and impact of OAB, by creating partnerships through education and engagement with health care professionals, industry, private and public payers, funding agencies, and policymakers.
The facilitators and barriers to improving functional activity and wellbeing in people with dementia: A qualitative study from the Process Evaluation of Promoting Activity, Independence and Stability in Early Dementia
Authors: Di Lorito Claudio , van der Wardt Veronika , Pollock Kristian , Howe Louise , Booth Vicky , Logan Pip , Gladman John , Masud Tahir , das Nair Roshan , Goldberg Sarah , Vedhara Kavita , O’Brien Rebecca , Adams Emma , Cowley Alison , Bosco Alessandro , Hancox Jennie , Burgon Clare , Bajwa Rupinder , Lock Juliette , Long Annabelle , Godfrey Maureen , Dunlop Marianne , Harwood Rowan H. 
Published: December 2022

AbstractBackgroundThe PRomoting Activity, Independence and Stability in Early Dementia (PrAISED) study delivered an exercise and functional activity programme to participants living with dementia. A Randomised Controlled Trial (RCT) showed no measurable benefits in activities of daily living, physical activity or quality of life.ObjectiveTo explore participants’ responses to PrAISED and explain the mechanisms behind a complex intervention that did not lead to expected health gains.MethodsA process evaluation using qualitative methods, comprising interviews and researcher notesSettingData were collected in participants’ homes or remotely by telephone or videoconferencing.SampleEighty-eight interviews were conducted with 44 participants living with dementia (n = 32 intervention group; n = 12 control group) and 39 caregivers. Sixty-nine interviews were conducted with 26 therapists.ResultsParticipants valued the intervention as proactively addressing health issues that were of concern to them, and as sources of social contact, interaction, information, and advice. Facilitators to achieving positive outcomes included perceiving progress toward desired goals, positive expectations, therapists’ skills and rapport with participants, and caregiver support. Barriers included: cognitive impairment, which prevented independent engagement and carryover between sessions; chronic physical health problems and intercurrent acute illness and injury; ‘tapering’ (progressively infrequent supervision intended to help develop habits and independent activity); and the COVID-19 pandemic.ConclusionsInterventions aiming to maintain activity, independence and stability may not be appropriate in the context of dementia even in the mild stages of the condition. Various factors affected outcomes including caregiver support, rapport with therapists, availability of supervision, motivational factors, and the limitations of remote delivery. The effects of cognitive impairment, multimorbidity and frailty overwhelmed any positive impact of the intervention. Maintenance of functional ability is valued, but in the face of inevitable progression of disease, other less tangible outcomes become important, challenging how we frame ‘health gain’ and trial outcomes.
Identification of new drugs to counteract anti-spike IgG-induced hyperinflammation in severe COVID-19
Authors: Geyer Chiara E. , Chen Hung-Jen , Bye Alexander P. , Manz Xue D. , Guerra Denise , Caniels Tom G. , Bijl Tom P.L. , Griffith Guillermo R. , Hoepel Willianne , de Taeye Steven W. , Veth Jennifer , Vlaar Alexander P.J. ,   Amsterdam UMC COVID-19 Biobank, Vidarsson Gestur , Bogaard Harm Jan , Aman Jurjan , Gibbins Jonathan M. , van Gils Marit J. , de Winther Menno P.J. , Dunnen Jeroen den 
Published: December 2022

SummaryPreviously, we and others have shown that SARS-CoV-2 spike-specific IgG antibodies play a major role in disease severity in COVID-19 by triggering macrophage hyperactivation, disrupting endothelial barrier integrity, and inducing thrombus formation. This hyper-inflammation is dependent on high levels of anti-spike IgG with aberrant Fc tail glycosylation, leading to Fcγ receptor hyper-activation. For development of immune-regulatory therapeutics, drug specificity is crucial to counteract excessive inflammation while simultaneously minimizing inhibition of antiviral immunity. We here developed anin vitroactivation assay to screen for small molecule drugs that specifically counteract antibody-induced pathology. We identified that anti-spike induced inflammation is specifically blocked by small molecule inhibitors against SYK and PI3K. We identified SYK inhibitor entospletinib as the most promising candidate drug, which also counteracted anti-spike-induced endothelial dysfunction and thrombus formation. Moreover, entospletinib blocked inflammation by different SARS-CoV-2 variants of concern. Combined, these data identify entospletinib as a promising treatment for severe COVID-19.Graphical abstract