bluuebunny/crossref_metadata_2025 huggingface dataset:
Historia natural de la distonía mioclónica asociada a variantes de SGCE en niños y adolescentes
Authors: De Francesch Valeria , Cazurro‐Gutiérrez Ana , Timmers Elze R. , Español‐Martín Gemma , Ferrero‐Turrión Julia , Gómez‐Andrés David , Marcé‐Grau Anna , Dougherty‐de Miguel Lucía , González Victoria , Moreno‐Galdó Antonio , Tijssen Marina A. J. , Pérez‐Dueñas Belén
Published: January 2025

Objetivo

Investigar la progresión natural de la distonía mioclónica causada por variantes patogénicas de SGCE desde el inicio de los síntomas en la infancia hasta la edad adulta temprana.

Métodos

En dos cohortes de pacientes de España y los Países Bajos, se realizó un seguimiento de las mioclonías y la distonía mediante escalas de valoración. Se calcularon las tasas de cambio anualizadas individuales y se evaluaron las tendencias longitudinales mediante modelos mixtos bayesianos. Las características psiquiátricas se evaluaron transversalmente en la cohorte española.

Resultados

Treinta y ocho pacientes (21 varones; 17 mujeres) fueron evaluados a los 10 ± 4,6 años (rango 2–21) y a los 14,2 ± 4,7 años (rango 4–25). Se observó un empeoramiento significativo de la mioclonía de acción, la distonía global y la distonía durante la escritura (incrementos medios anuales de 1,356, 0,226 y 0,518 en las escalas de valoración Unified Myoclonus Rating Scale, Burke‐Fahn‐Marsden Dystonia Rating Scale y Writer's Cramp Rating Scale, respectivamente). En consecuencia, los pacientes percibieron un empeoramiento significativo de sus capacidades para hablar, escribir y caminar. Veintiséis de los 32 pacientes padecían trastornos de ansiedad (n = 13), obsesivo‐compulsivos (n = 9) y/o déficit de atención con hiperactividad (n = 8).

Interpretación

Este estudio demuestra que, a diferencia de lo que ocurre en la población adulta, la distonía mioclónica en la infancia y la adolescencia sigue un curso progresivo que puede ser debilitante en las primeras etapas de la vida. Estos hallazgos, junto con una alta prevalencia de síntomas psiquiátricos, ponen de relieve la necesidad de intervenciones terapéuticas tempranas para prevenir secuelas motoras y psicológicas a largo plazo.
Evaluating the Performance of Large Language Models (LLMs) in Answering and Analysing the Chinese Dental Licensing Examination
Authors: Xiong Yu‐Tao , Zhan Zheng‐Zhe , Zhong Cheng‐Lan , Zeng Wei , Guo Ji‐Xiang , Tang Wei , Liu Chang
Published: January 2025

Background

This study aimed to simulate diverse scenarios of students employing LLMs for CDLE examination preparation, providing a detailed evaluation of their performance in medical education.

Methods

A stratified random sampling strategy was implemented to select and subsequently revise 200 questions from the CDLE. Seven LLMs, recognised for their exceptional performance in the Chinese domain, were selected as test subjects. Three distinct testing scenarios were constructed: answering questions, explaining questions and adversarial testing. The evaluation metrics included accuracy, agreement rate and teaching effectiveness score. Wald χ2 tests and Kruskal–Wallis tests were employed to determine whether the differences among the LLMs across various scenarios and before and after adversarial testing were statistically significant.

Results

The majority of the tested LLMs met the passing threshold on the CDLE benchmark, with Doubao‐pro 32k and Qwen2‐72b (81%) achieving the highest accuracy rates. Doubao‐pro 32k demonstrated the highest 98% agreement rate with the reference answers when providing explanations. Although statistically significant differences existed among various LLMs in their teaching effectiveness scores based on the Likert scale, all these models demonstrated a commendable ability to deliver comprehensible and effective instructional content. In adversarial testing, GPT‐4 exhibited the smallest decline in accuracy (2%, p = 0.623), while ChatGLM‐4 demonstrated the least reduction in agreement rate (14.6%, p = 0.001).

Conclusions

LLMs trained on Chinese corpora, such as Doubao‐pro 32k, demonstrated superior performance compared to GPT‐4 in answering and explaining questions, with no statistically significant difference. However, during adversarial testing, all models exhibited diminished performance, with GPT‐4 displaying comparatively greater robustness. Future research should further investigate the interpretability of LLM outputs and develop strategies to mitigate hallucinations generated in medical education.
Causes of hospitalization and mortality in persons with epilepsy: The EpiLink Bologna cohort, Italy
Authors: Muccioli Lorenzo , Zenesini Corrado , Licchetta Laura , Belotti Laura Maria Beatrice , Vito Lidia Di , Ferri Lorenzo , Fiorillo Domenico , Mostacci Barbara , Pasini Elena , Riguzzi Patrizia , Soldà Martina , Taruffi Lisa , Volpi Lilia , Mason Federico , Nonino Francesco , Michelucci Roberto , Tinuper Paolo , Vignatelli Luca , Bisulli Francesca
Published: January 2025

Background

Epilepsy significantly impacts on morbidity and mortality. Understanding hospitalization and mortality risks in persons with epilepsy (PWE) is essential for improving healthcare strategies. We aimed to investigate the risk and causes of hospitalization and mortality in PWE compared to a matched general population cohort.

Methods

The EpiLink Bologna historical cohort study analyzed adult PWE in the period 2018–2019. A general population control cohort was used for comparison. Clinical data were linked with health administrative data. PWE were grouped into persons with focal epilepsy, idiopathic generalized epilepsy, and developmental and/or epileptic encephalopathy (PDEE). The primary outcome was the hospitalization rate. Emergency department (ED) visit rate and the risk of death for any cause were also assessed.

Results

The study included 1438 PWE and 14,096 controls. PWE had higher incidence rate ratio (IRR) for ED visit (IRR 1.26, 95% CI 1.20–1.32), hospital admission (IRR 2.05, 95% CI 1.83–2.29), and death (IRR 1.5, 95% CI 1.1–2.2) compared to control cohort. The highest hospitalization risk was in the PDEE group (IRR 4.70; 95% CI 3.28–6.74). The increased hospitalization rate among PWE was due to both their higher ED visit and elective hospital admission rates. PWE on polytherapy were at higher risk of hospitalization for inflammation of jaw, acid–base/electrolyte imbalances, chronic cerebrovascular disease, major traumas and infections.

Conclusions

During a 2‐year‐period, PWE in Bologna had a doubled risk of hospitalization and 50% higher risk of death compared to a matched general population cohort. Hospitalization risks varied significantly by epilepsy type and antiseizure therapy.
PHILOSOPHY OF HISTORY AS A CRITIQUE OF VIOLENCE: HISTORY, THEOLOGY, AND POLITICS IN WALTER BENJAMIN'S EARLY WRITINGS
Authors: NOLLET VINSENT
Published: January 2025

Walter Benjamin's “Critique of Violence” became a classical work on revolutionary politics mainly due to influential political‐theological expositions of its arguments. The main challenge with the political‐theological understanding of the essay, however, is that Benjamin seemingly argued against any reconciliation of politics and theology. For this reason, this article develops a historical‐philosophical rather than a political‐theological interpretation of Benjamin's “Critique of Violence.” It demonstrates how Benjamin's essay on violence can be best understood if it is read as a text containing a speculative philosophy of history. In the essay, Benjamin indeed presented the role of violence in history as a historical “law of oscillation” between lawmaking and law‐preserving violence. The problem of violence transcends the framework of the political, which can witness only “administered” violence, not violence as such, and hence cannot escape the dialectical movement of this “law.” While Benjamin's philosophy of history is usually located in the final period of his philosophical activity and comprises a messianic‐Marxist complex, Benjamin's “Critique of Violence” suggests the existence of a more substantial philosophy of history in Benjamin's early writings than is usually assumed. The period between 1919 and 1922 especially shows a concentration of anarchistic and nihilistic texts devoted to developing this philosophy of history.
Christendom and Hindutva
Authors: Singh David Emmanuel
Published: January 2025

The Council of Nicaea took place at Constantine's initiative to harmonize religious belief and law into a state‐subscribed orthodoxy. From a socio‐political viewpoint, the council appeared to use a standardized version of Christianity as an instrument of the empire. The focus in this paper is not on Constantine's initiative or its impact on the non‐conforming minorities; rather, it uses this impact as a lens for reviewing an emerging Hindu empire in India. For much of its independent history, India was largely successful in keeping Hindutva's 19th‐century undercurrents at bay from polity. The Bharatiya Janata Party (BJP), however, exposed the shallowness of the Indian experiment with secularism. As Hindutva embraces politics, it also exercises social control. In this sense, Hindutva is no different from Christendom, especially when viewed from the perspective of the impact it has on the minorities: a new act on citizenship, dissent‐violence, and reconversions. What this shows is that colonial despotism continues even today.
Worker Runs
Authors: HOFFMANN FLORIAN , VLADIMIROV VLADIMIR
Published: January 2025

The voluntary departure of hard‐to‐replace skilled workers worsens firm prospects, which can lead to additional departures. We develop a model in which firms design compensation to limit the risk of such “worker runs.” To achieve cost‐efficient retention, firms combine fixed wages with dilutable compensation—such as vesting equity or bonus pools—which pays remaining workers more when others leave but gets diluted otherwise. Compensating (identical) workers with differently structured compensation, that is, with a different mix of output‐dependent and output‐independent pay, can further mitigate the risk of worker runs by ensuring a critical retention level in a cost‐efficient way.
Wealth and Insurance Choices: Evidence from U.S. Households
Authors: GROPPER MICHAEL J. , KUHNEN CAMELIA M.
Published: January 2025

Using administrative data for 63,000 individuals across 2,500,000 person‐month observations, we find that wealthier individuals have better life insurance coverage, controlling for the value of the asset insured, namely, the consumption needs of dependents. This positive wealth‐insurance correlation, which is surprising given the prevailing view that wealth substitutes for insurance, persists after allowing for wealth‐related differences in risk or bequest preferences, pricing, background risk, education, employment, or liquidity constraints. Our findings call for further investigation of this wealth‐coverage correlation but support theories emphasizing the consumption‐smoothing role of insurance across not only states of the world, but also time.
GS2 cooperates with IPA1 to control panicle architecture
Authors: Wang Yueying , Lv Yang , Wen Yi , Wang Junge , Hu Peng , Wu Kaixiong , Chai Bingze , Gan Shuxian , Liu Jialong , Wu Yue , Zhu Lixin , Dong Nannan , Tan Yiqing , Wu Hao , Zhang Guangheng , Zhu Li , Ren Deyong , Zhang Qiang , Wang Yuexing , Qian Qian , Hu Jiang
Published: January 2025

Panicle size and grain number are important agronomic traits that determine grain yield in rice. However, the underlying mechanism regulating panicle size and grain number remains largely unknown.

Here, we report that GS2 plays an important role in regulating panicle architecture. The RNAi of GS2™ (target site mutation, TM) produced erect and dense panicle with increased primary and secondary branches and grain number per panicle, whereas the overexpression of GS2™ showed longer panicles and fewer grains than wild‐type.

GS2 directly binds to the GCCA motif and significantly enhances the transcriptional activation ability through the interaction with IPA1. DEP1 is a common target gene of GS2 and IPA1 in regulating branch number and grain number per panicle. The pyramiding of GS2™ and IPA1™1 (Target site mutation1, TM1) on hybrid rice can significantly increase rice yield.

Our findings reveal the novel function of GS2 and the molecular mechanism of GS2/IPA1‐DEP1 module in controlling panicle architecture.
Photoperiodic flowering and AFT/FTL3 gene expression in flowering‐time varieties in chrysanthemum
Authors: Takahashi Shigekazu , Nakano Yoshihiro , Sumitomo Katsuhiko , Hisamatsu Tamotsu , Oda Atsushi , Onoue Noriyuki , Mizubayashi Tatsumi , Yamakawa Hiromoto
Published: January 2025

Chrysanthemum (Chrysanthemum morifolium Ramat.) is a short‐day plant, and flowering is stimulated when the photoperiod is shorter than a variety‐specific threshold (critical day length). In Japan, summer‐to‐autumn‐flowering cultivars (SA‐cvs.) flower from July to September. Little research has been conducted to understand why SA‐cvs. bloom earlier than autumn‐flowering cultivars (A‐cvs.). We conducted a comparative study of the relationship between the photoperiodic response of flowering and the gene expression of florigen FLOWERING LOCUS T‐like 3 (FTL3) and antiflorigen anti‐florigenic FT/TFL1 (AFT). SA‐cvs. had a longer critical day length than A‐cvs. However, in both groups, a decrease in AFT and increase in FTL3 were consistently observed below the critical day length when flowering was promoted. The opposite responses (less flowering, low FTL3, and high AFT) were observed for longer than the critical day lengths. This indicated that flowering in SA‐cvs. was controlled by the regulation of AFT/FTL3 expression, similar to that in A‐cvs. Next, we studied the mechanism that causes a variation in critical day lengths. In SA‐cvs., the photosensitive phase, which occurs at night, occurs earlier than that in A‐cvs. This indicates a variation in the endogenous time‐keeping mechanism. This was supported by the fact that the circadian rhythmicity of leaf movement was weaker in SA‐cvs. than that in A‐cvs. Thus, variation in the endogenous time‐keeping mechanism may cause a longer critical day length and earlier flowering time in SA‐cvs.
Quantum gases in bubble traps
Authors: Dubessy Romain , Perrin Hélène
Published: January 2025

This paper presents a review and perspective on quantum gases in bubble traps. We emphasize how the idea of realizing shell-shaped condensates emerged and was enabled by the invention of the radiofrequency adiabatic potential technique. We review the many subsequent theoretical works that address the new physics emerging for a condensate trapped on a closed surface. We present the current status of the experiments, the challenges ahead, and highlight how a different approach using an immiscible mixture of two condensates enabled the first observation of a shell-shaped degenerate gas. Finally, we list a few open questions that we believe provide interesting research directions.
Identity Conflict Among Politically Engaged Sport Fans: Implications for Fan Loyalty
Authors: Larkin Ben
Published: January 2025

Despite the natural intersection of sport and politics, it remains an understudied topic in academic literature. Further, the majority of scholarship in this area is either rooted in sport sociology, or, aimed at advancing the political science literature. Few studies have looked at the intersection of sport and politics for the purpose of advancing sport marketing scholarship. This is the case in spite of the fact that consumers increasingly expect businesses to take political stands. The current study begins to fill this void by examining the impact of political identity conflict on sport fan loyalty. Across two studies, results indicate that political identity conflict negatively affects attitudinal loyalty, but not behavioral loyalty. Furthermore, differences between liberal and conservative sports fans as it pertains to managing political identity conflict and their perceptions of the intersection of sport and politics are explored. Implications for theory and practice are discussed.
Optogenetic calcium modulation in astrocytes enhances post-stroke recovery in chronic capsular infarct
Authors: Cho Jongwook , Lee Sangkyu , Kook Yeon Hee , Park Jiyoung , Do Heo Won , Lee C. Justin , Kim Hyoung-Ihl
Published: January 2025

Stroke is caused by disruption of cerebral blood flow, leading to neuronal death and dysfunction in the interconnected areas, which results in a wide range of severe symptoms depending on the specific brain regions affected. While previous studies have primarily focused on direct modulation of neuronal activity for post-stroke treatment, accumulating evidence suggests that astrocytes may play a critical role in post-stroke progression and could serve as a potential therapeutic target for recovery. In this study, we investigate the effects of selective modulation of astrocytic calcium signals on chronic stroke using OptoSTIM1, an optogenetic tool that activates endogenous calcium channels. In contrast to channelrhodopsin-2 (ChR2), OptoSTIM1 robustly elevates astrocytic calcium levels, sustaining the increase for over 10 min upon a single activation. The calcium elevation in astrocytes in the ipsilesional sensory-parietal cortex leads to remarkable recovery from post-stroke impairment. Thus, manipulating intracellular calcium levels in astrocytes holds promise as a potential therapeutic strategy for improving recovery following a stroke.
Large-scale transcript variants dictate neoepitopes for cancer immunotherapy
Authors: Ji Shiliang , Wang Feifan , Wu Yongjie , Hu Haoran , Xing Zhen , Zhu Jie , Xu Shi , Han Tiyun , Liu Guilai , Wu Zengding , Fei Caiyi , Kong Lingming , Chen Jiangning , Ding Zhi , Huang Zhen , Zhang Junfeng
Published: January 2025

Precise neoepitope discovery is crucial for effective cancer therapeutic vaccines. Conventional approaches struggle to build a repertoire with sufficient immunogenic epitopes. We developed a workflow leveraging full-length ribosome–nascent chain complex–bound mRNA sequencing (FL-RNC seq) and artificial intelligence–based predictive models to accurately identify the neoepitope landscape, especially large-scale transcript variants (LSTVs) missed by short-read sequencing. In the MC38 mouse model, we identified 22 LSTV-derived neoepitopes encoded by a synthesized mRNA lipid nanoparticle vaccine. As a standalone therapy and combined with anti–PD-1 immunotherapy, the vaccine curbed tumor progression, induced robust T cell–specific immunity, and modulated the tumor microenvironment. This underscores the multifaceted potentials of LSTV-derived vaccines. Our approach expands the neoepitope source repertoire, offering a method for discovering personalized cancer vaccines applicable to a broader tumor range. The results highlight the importance of comprehensive neoepitope identification and the promise of LSTV-based vaccines for cancer immunotherapy.
Investigating gut alterations in Alzheimer’s disease: In-depth analysis with micro- and nano-3D X-ray phase contrast tomography
Authors: Palermo Francesca , Marrocco Nicole , Dacomo Letizia , Grisafi Elena , Moresi Viviana , Sanna Alessia , Massimi Lorenzo , Musella Marianna , Maugeri Laura , Bukreeva Inna , Fiordaliso Fabio , Corbelli Alessandro , Junemann Olga , Eckermann Marina , Cloetens Peter , Weitkamp Timm , Gigli Giuseppe , de Rosbo Nicole Kerlero , Balducci Claudia , Cedola Alessia
Published: January 2025

Alzheimer’s disease (AD), a debilitating neurodegenerative disorder, remains one of the foremost public health challenges affecting more than 30 million people worldwide with the etiology still largely enigmatic. The intricate gut-brain axis, serving as a vital communication network between the gut and the brain, appears to wield influence in the progression of AD. Our study showcases the remarkable precision of x-ray phase-contrast tomography (XPCT) in conducting an advanced three-dimensional examination of gut cellular composition and structure. The exploitation of micro- and nano-XPCT on various AD mouse models unveiled relevant alterations in villi and crypts, cellular transformations in Paneth and goblet cells, along with the detection of telocytes, neurons, erythrocytes, and mucus secretion by goblet cells within the gut cavity. The observed gut structural variations may elucidate the transition from dysbiosis to neurodegeneration and cognitive decline. Leveraging XPCT could prove pivotal in early detection and prognosis of the disease.
Single molecule–driven nanomotors reveal the dynamic-disordered chemomechanical transduction of active enzymes
Authors: Tang Zhuodong , Wu Jingyu , Wu Shaojun , Tang Wenjing , Zhang Jian-Rong , Zhu Wenlei , Zhu Jun-Jie , Chen Zixuan
Published: January 2025

Enzymes facilitate the conversion of chemical energy into mechanical work during biochemical reactions, thereby regulating the dynamic metabolic activity of living systems. However, directly observing the energy release facilitated by fluctuating individual enzymes remains a challenge, leading to a contentious debate regarding the underlying reasons for this phenomenon. Here, we aim to overcome this challenge by developing an oscillating nanomotor powered by a single-molecule enzyme, which allows real-time tracking of energy transduction in enzymatic reactions. Through analysis of the shifts in free energy profiles within the nanomotors, our results unveil not only the heterogeneous energy release patterns of individual enzyme molecules but also the dynamic disorder of a particular enzyme in energy release over extended monitoring periods. By exploring six distinct types of single-molecule enzymatic reactions, we provide the direct evidence supporting the argument that the reaction enthalpy governs the enzymatic energy release. This approach has implications for understanding the mechanism of enzymatic catalysis and developing highly efficient nanomotors.
Selectively fluorinated aromatic lithium salts regulate the solvation structure and interfacial chemistry for all-solid-state batteries
Authors: Yan Shuaishuai , Liu Hao , Lu Yang , Feng Qingqing , Zhou Hangyu , Wu Yuhao , Hou Wenhui , Xia Yingchun , Zhou Haiyu , Zhou Pan , Song Xuan , Ou Yu , Liu Kai
Published: January 2025

Solid polymer electrolytes suffer from the polymer-dominated Li
+
solvation structure, causing unstable electrolyte/electrode interphases and deteriorated battery performance. Here, we design a class of selectively fluorinated aromatic lithium salts (SFALS) as single conducting lithium salts to regulate the solvation structure and interfacial chemistry for all-solid-state lithium metal batteries. By tuning the anionic structure, the Li
+
-polyether coupling is weakened, and the Li
+
-anion coordination is enhanced. The hydrogen bonding between the SFALS and polymer matrix induces a special “triad”-type solvation structure, which improves the electrolyte homogeneity and mechanical strength, and promotes the formation of an ultrathin and robust Li
2
O-rich solid electrolyte interphase. Therefore, the stable cycling of more than 1650 cycles (Coulombic efficiency, 99.8%) for LiFePO
4
/Li half cells and 580 cycles (97.4% capacity retention) for full cells is achieved. This molecular engineering strategy could inspire further advancements of functional lithium salts for practical application of all-solid-state lithium metal batteries.
Stem-like memory and precursors of exhausted T cells share a common progenitor defined by ID3 expression
Authors: Gago da Graça Catarina , Sheikh Amania A. , Newman Dane M. , Wen Lifen , Li Sining , Shen Jian , Zhang Yuqi , Gabriel Sarah S. , Chisanga David , Seow Justine , Poch Annika , Rausch Lisa , Nguyen Minh-Hanh T. , Singh Jayendra , Su Chun-Hsi , Cluse Leonie A. , Tsui Carlson , Burn Thomas N. , Park Simone L. , Von Scheidt Bianca , Mackay Laura K. , Vasanthakumar Ajithkumar , Bending David , Shi Wei , Cui Weiguo , Schröder Jan , Johnstone Ricky W. , Kallies Axel , Utzschneider Daniel T.
Published: January 2025

Stem-like T cells are attractive immunotherapeutic targets in patients with cancer given their ability to proliferate and differentiate into effector progeny. Thus, identifying T cells with enhanced stemness and understanding their developmental requirements are of broad clinical and therapeutic interest. Here, we demonstrate that during acute infection, the transcriptional regulator inhibitor of DNA binding 3 (ID3) identifies stem-like T cells that are uniquely adapted to generate precursors of exhausted T (Tpex) cells in response to chronic infection or cancer. Expression of ID3 itself enables Tpex cells to sustain T cell responses in chronic infection or cancer, whereas loss of ID3 results in impaired maintenance of CD8 T cell immunity. Furthermore, we demonstrate that interleukin-1 (IL-1) family members, including IL-36β and IL-18, promote the generation of ID3
+
T cells that mediate superior tumor control. Overall, we identify ID3 as a common denominator of stem-like T cells in both acute and chronic infections that is specifically required to sustain T cell responses to chronic stimulation.
Treatment options for preventing autoimmune-mediated congenital heart block: a systematic review
Authors: Agarwal Abhishek , Skoutelis Nikolaos , Zhou Yufeng , Kawanami Reina , Charras Amandine , Occhigrossi Felice , Agarwal Umber , Khan Sophia , Donegan Sarah , Hawcutt Daniel
Published: January 2025

Objective

Complete congenital heart block (CHB) due to anti-Ro/La antibodies in pregnancy can lead to adverse fetal outcomes and neonatal death. National and international guidelines do not recommend specific treatment for women identified with anti-Ro antibodies while pregnant.

Design

Systematic review of studies of pregnant women with anti-Ro/La antibodies undertaking treatment(s) to prevent CHB. Studies solely focused on treating developing or established CHB were excluded.

Main outcome measure

CHB incidence.

Results

Of 1049 articles, 20 studies were eligible, of which 13 were retrospective. Sixteen studies contained usable data comparing various treatments (seven hydroxychloroquine (HCQ), six corticosteroids, two intravenous immunoglobulin (IVIG) and one each on combined HCQ and corticosteroids and plasmapheresis), including 2134 women and 2915 pregnancies. Meta-analysis was not possible due to heterogeneity. HCQ as primary prophylaxis showed protective effect on CHB incidence in four of five studies, whereas for preventing recurrence, only one study had a comparator arm, which showed a protective effect. Corticosteroids showed protective effect on CHB incidence as primary prophylaxis in two of three studies, whereas in recurrence, only one study showed protective effect. The IVIG study with a comparator showed no protective effect on CHB recurrence. Two studies (IVIG and plasmapheresis) had no comparator. The long-term toxicity of treatments was poorly investigated.

Conclusion

HCQ showed the most evidence among various treatment options to prevent CHB in babies of women with anti-Ro/La antibodies. However, included studies had high risk of bias and imprecision. Future studies should be prospective, compare existing treatments and capture adverse effects.

PROSPERO registration number CRD42023492934.
Gastric neuroendocrine tumour manifesting as abdominal pain in systemic lupus erythematosus
Authors: Chithrabhanu Anoop , Rajamurugan Arul , Subramanian Ramesh , Lakshmi J Durga
Published: January 2025

Systemic lupus erythematosus (SLE) is an autoimmune disorder with diverse clinical presentations, including gastrointestinal involvement. We report a case of a young woman with a decade-long history of SLE presenting with recurrent abdominal pain, vomiting and weight loss. Initially suspected to be pancreatitis, further investigations revealed gastric nodules and the biopsy was suggestive of neuroendocrine tumour. This case underscores the importance of considering rare causes of abdominal pain in patients with SLE and vigilance for potential malignancies. The association between SLE and gastric neuroendocrine tumours remains poorly understood, emphasising the need for further exploration. This case contributes to the growing literature on rare gastrointestinal manifestations in SLE, prompting clinicians to maintain a wide differential diagnoses for abdominal symptoms in this patient population.
Knee intra-articular loose body causing a compression common peroneal nerve neuropathy with foot drop
Authors: Fakih Mustafa , Alhojailan Khalifa , Volesky Monika
Published: January 2025

We present a unique case of a man in his 60s with a knee intra-articular loose body causing a compression neuropathy on his common peroneal (fibular) nerve (CPN). This neuropathy caused a reduction in function of his leg’s motor function causing a foot drop. Through a strong clinical interview and focused clinical examination, the neuropathy was localised, and the diagnosis was reinforced through nerve conduction studies and imaging. The intra-articular loose body was surgically excised after the careful isolation and decompression of the peroneal nerve. This resulted in a marked improvement in the patient’s motor function and symptoms, allowing him to ambulate and return to normal function. To our knowledge, this is the first case report of an intra-articular loose body in the knee causing a compression CPN neuropathy with symptoms such as foot drop. We hope this case report aids in guiding management should physicians come across this issue in the future.
Health-related quality of life in patients with liver cirrhosis following adjunctive nurse-based care versus standard medical care: a pragmatic, multicentre, randomised controlled study
Authors: Hjorth Maria , Sjöberg Daniel , Svanberg Anncarin , Lo Martire Riccardo , Kaminsky Elenor , Rorsman Fredrik
Published: January 2025

Objectives

Patients have difficulties in understanding how to manage their liver cirrhosis. This highlights a need for support in comprehending health-related information, which remains largely lacking within liver cirrhosis care. Involvement of registered nurses (RNs) in outpatient liver cirrhosis care has potential to improve quality of care and reduce patient mortality. However, the benefits of nursing care on patients’ health-related quality of life (HRQoL) are scarcely studied. This study compared HRQoL in patients receiving either standard medical outpatient care or adjunctive, nurse-led care. The risk of malnutrition, decompensation events and mortality were also compared between the two study groups.

Methods

This was a pragmatic, multicentre, randomised trial, which enrolled 167 patients with liver cirrhosis. The primary outcome measure, HRQoL, was assessed using the RAND-36 questionnaire. The physical component summary (PCS) and the mental component summary (MCS) scores of RAND-36 were compared, using linear mixed-effects models for repeated measures, at 12 and 24 months.

Results

83 patients received standard medical care, and 84 patients received adjunctive, nurse-led care for 24 months. Due to unforeseen circumstances, the final study population of 167 participants was less than the intended 500. Group comparisons were non-significant of the PCS and MCS scores (−1.1, p=0.53 and −0.7, p=0.67, respectively), malnutrition (p=0.62) and decompensation events (p=0.46), after 24 months. However, mortality was three times higher in the control group compared with the intervention group (12 vs 4, p=0.04) after 24 months.

Conclusions

In this study, adjunctive nurse-led care was not superior to standard medical outpatient care regarding HRQoL, risk of developing malnutrition or decompensation. However, RN involvement contributed to early identification of decompensation and reduced mortality.

Trial registration number

NCT02957253.
Decoding mpox: a systematic review and meta-analysis of the transmission and severity parameters of the 2022–2023 global outbreak
Authors: Diaz Brochero Candida , Nocua-Báez Laura Cristina , Cortes Jorge Alberto , Charniga Kelly , Buitrago-Lopez Adriana , Cucunubá Zulma M
Published: January 2025

Introduction

The 2022–2023 mpox outbreak has been the largest in history. We aim to synthesise the key epidemiological parameters related to the dynamics, transmission, and severity of mpox (incubation period, serial interval, generation time, infectious period, basic (R0) and effective (R(t)) reproductive number, and case fatality rate (CFR)).

Methods

Systematic review of observational studies in MEDLINE, EMBASE and other sources up to September 2023 (PROSPERO: CRD42023404503). Quality assessment using the Joanna Briggs Institute Critical Appraisal for case series, cross-sectional and cohort studies, and a designed quality assessment questionnaire for mathematical models. Meta-analysis was performed using a random effects model.

Results

For transmissibility parameters, we estimated a pooled incubation period of 7.60 (95% CI 7.14 to 8.10) days and a pooled serial interval of 8.30 (95% CI 6.74 to 10.23) days. One study reported a generation time of 12.5 days (95% CI 7.5 to 17.3). Three studies reported presymptomatic transmission in 27–50% of paired cases investigated. R(t) varied between 1.16 and 3.74 and R0 varied between 0.006 and 7.84. The epidemic peaked between August and September 2022 in Europe and the Americas whereas transmission has continued in African countries. For severity parameters, we estimated a pooled CFR by continent: 0.19% (95% CI 0.09% to 0.37%) for the Americas and 0.33% (95% CI 0.15% to 0.7%) for Europe. For Africa, we found that the CFRs of countries associated with group I were higher (range 17–64%) than those associated with group IIb (range 0–6%).

Conclusion

Pooled mpox serial interval was slightly larger than pooled incubation period, suggesting transmission occurs mostly postsymptom onset, although presymptomatic transmission can occur in an important proportion of cases. CFR estimates varied by geographical region and were higher in Africa, in countries linked with clade I. Our results contribute to a better understanding of mpox dynamics, and the development of mathematical models to assess the impact of current and future interventions.
Spinal cord demyelination predicts neurological deterioration in patients with mild degenerative cervical myelopathy
Authors: Al-Shawwa Abdul , Craig Michael , Ost Kalum , Anderson David , Casha Steve , Jacobs W Bradley , Evaniew Nathan , Tripathy Saswati , Bouchard Jacques , Lewkonia Peter , Nicholls Fred , Soroceanu Alex , Swamy Ganesh , Thomas Kenneth C , duPlessis Stephan , Yang Michael MH , Cohen-Adad Julien , Dea Nicholas , Wilson Jefferson R , Cadotte David W
Published: January 2025

Background

Degenerative cervical myelopathy (DCM) is the most common form of atraumatic spinal cord injury globally. Clinical guidelines regarding surgery for patients with mild DCM and minimal symptoms remain uncertain. This study aims to identify imaging and clinical predictors of neurological deterioration in mild DCM and explore pathophysiological correlates to guide clinical decision-making.

Methods

Patients with mild DCM underwent advanced MRI scans that included T2-weighted, diffusion tensor imaging and magnetisation transfer (MT) sequences, along with clinical outcome measures at baseline and 6-month intervals after enrolment. Quantitative MRI (qMRI) metrics were derived above and below maximally compressed cervical levels (MCCLs). Various machine learning (ML) models were trained to predict 6 month neurological deterioration, followed by global and local model interpretation to assess feature importance.

Results

A total of 49 patients were followed for a maximum of 2 years, contributing 110 6-month data entries. Neurological deterioration occurred in 38% of cases. The best-performing ML model, combining clinical and qMRI metrics, achieved a balanced accuracy of 83%, and an area under curve-receiver operating characteristic of 0.87. Key predictors included MT ratio (demyelination) above the MCCL in the dorsal and ventral funiculi and moderate tingling in the arm, shoulder or hand. qMRI metrics significantly improved predictive performance compared to models using only clinical (bal. acc=68.1%) or imaging data (bal. acc=57.4%).

Conclusions

Reduced myelin content in the dorsal and ventral funiculi above the site of compression, combined with sensory deficits in the hands and gait/balance disturbances, predicts 6-month neurological deterioration in mild DCM and may warrant early surgical intervention.
Mutational profile dynamics in follicular lymphoma and large cell transformation
Authors: Hesius Eva A M , Stevens Wendy B C , Stewart James P , Kroeze Leonie I , Spek Ellen van der , Issa Djamila , Nooijen Peet , Luijks Jeroen , Gonzalez David , Groenen Patricia J T A , Blijlevens Nicole M A , Spriel Annemiek B van , Brand Michiel van den
Published: January 2025

Aims

Follicular lymphoma (FL) is characterised by significant heterogeneity in both the clinical trajectories and the molecular profiles. This study aimed to investigate clonal dynamics in FL by analysing mutation profiles at various time points during the disease course including at histological transformation (HT), to gain insight into the mutational changes over time.

Methods

We retrospectively analysed 76 biopsies from 25 patients, including 13 cases with three or more FL biopsies and 12 cases with subsequent HT. Hybrid capture-based Next-Generation Sequencing (NGS) with the EuroClonality-NGS DNA capture (EuroClonality-NDC) assay was used to examine clonal rearrangements and mutations.

Results

A total of 204 (potentially) pathogenic mutations were identified. Only 40% of mutations remained stably present during a median follow-up period of 139 months (range 9–198).KMT2DandCREBBPwere the most frequently mutated genes at diagnosis, exhibiting relative stability in follow-up biopsies. Conversely,EZH2displayed a dynamic pattern of mutations gained and lost during the disease course. At HT, pathogenic mutations affectingB2M,MYCandTP53emerged. Changes in mutational burden were observed in both FL-sequential and diagnosis-transformation cohorts, with more pronounced changes in the latter.

Conclusions

This real-world study provides insights into the complex molecular pathogenesis of FL and HT. As targeted therapies emerge as treatment modalities, mutational profiles could influence treatment decisions in the future. Therefore, recognising the significant changes occurring in the mutational landscape of FL throughout the disease course is crucial.
Comparison of flow diverter alone versus flow diverter with coiling for large and giant intracranial aneurysms: systematic review and meta-analysis of observational studies
Authors: Abo Kasem Rahim , Hubbard Zachary , Cunningham Conor , Almorawed Hani , Isidor Julio , Samman Tahhan Imad , Sowlat Mohammad-Mahdi , Babool Sofia , Abodest Layal , Spiotta Alejandro M
Published: January 2025

Background

Large and giant intracranial aneurysms pose treatment challenges. The benefit–risk balance of flow diverters (FDs) alone versus FDs with coiling remains unclear. This study aimed to compare these two strategies.

Methods

This systematic review and meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A comprehensive search of PubMed, Embase, Scopus, Cochrane, and Web of Science was performed up to October 2024. Studies comparing FDs with or without adjunctive coiling in large/giant intracranial aneurysms were included. The primary outcome was complete aneurysm occlusion, defined by the Raymond–Roy Occlusion Classification. Additional outcomes included procedural and postprocedural complications. Data were analyzed using a random effects model.

Results

15 studies with 1130 patients were analyzed, with 557 in the FD alone group and 573 in the FD+coiling group. The meta-analysis revealed that FD+coiling significantly improved complete aneurysm occlusion rates (OR 1.59, 95% CI 1.06 to 2.40, P=0.03). While overall ischemic complications were significantly lower in the FD alone group, a sensitivity analysis showed no significant difference (OR 0.49, 95% CI 0.20 to 1.23, P=0.13). Subgroup analysis of fusiform aneurysms showed no significant difference in complete aneurysm occlusion rates (OR 1.10, 95% CI 0.50 to 2.40, P=0.82). Procedural and hemorrhagic complications did not differ significantly, and no publication bias was detected in the results.

Conclusions

Combining FDs with coiling improved complete aneurysm occlusion rates in large and giant saccular intracranial aneurysms, although the impact on complications remains controversial. Further investigation into the benefit–risk ratio of this combined approach is warranted.
Cognitive changes in patients with relapse-free MS treated with high efficacy therapies: the predictive value of paramagnetic rim lesions
Authors: Boccia Vincenzo Daniele , Leveraro Elisa , Cipriano Emilio , Lapucci Caterina , Sirito Tommaso , Cellerino Maria , Rebella Giacomo , Nasone Lorenza , Boffa Giacomo , Inglese Matilde
Published: January 2025

Background

High-efficacy disease-modifying therapies (HETs) have substantially improved multiple sclerosis (MS) management, yet ongoing cognitive decline remains a concern. This study aims to assess Symbol Digit Modalities Test (SDMT) changes in patients with stable relapsing-remitting MS (RRMS) treated with HETs and to evaluate the role of baseline MRI biomarkers as predictors of SDMT changes.

Methods

Consecutive patients with RRMS treated with HETs underwent clinical, SDMT and MRI assessment at baseline with SDMT and clinical re-evaluation after 24 months. Patients presenting relapses or MRI activity (new T2 and/or gadolinium-enhancing lesions) during follow-up were excluded. Cognitive changes were defined using the 90% CI regression-based reliable change index methodology accounting for sex, age, education and baseline score. Baseline MRI examination included three-dimensional-sagittal Fluid Attenuated Inversion Recovery (FLAIR), T1-Magnetization Prepared - RApid Gradient Echo (T1-MPRAGE) and quantitative susceptibility mapping (QSM) for paramagnetic rim lesions (PRLs) and QSM-isointense lesions (ISO) assessment. Univariate and multivariable regression analyses were performed to predict SDMT changes.

Results

90 patients (mean age: 40.3 years, median Expanded Disability Status Scale: 2.0) were included. PRLs were present in 46 (51.1%) patients. After 24 months, 13 (14.4%) patients showed SDMT decline and 8 (8.9%) showed improvement. At multivariable analyses, PRLs were associated with higher risk of SDMT decline (β: 2.70, p: 0.02, OR: 14.82) while higher ISO lesion volumes were weakly associated with SDMT improvement (β: 0.07, p: 0.01, OR: 1.07).

Conclusions

SDMT decline and improvement are detectable in patients with RRMS without clinical or MRI activity over 2 years. PRLs seem to predict SDMT decline in MS, underscoring the critical role of compartmentalised chronic inflammation in disease progression.
Trends to shorter diagnostic delay in spondyloarthritis patients during the last decades and association with clinical presentation: data from ASAS-COMOSPA study
Authors: Perez-Alamino Rodolfo , Maldonado-Ficco Hernan , Moltó Anna , Waimann Christian , Maldonado-Cocco José , Dougados Maxime , Landewé Robert , van der Heijde Désirée , Van den Bosch Filip
Published: January 2025

Background

Diagnostic delay is one of the greatest challenges in spondyloarthritis (SpA). Better disease knowledge and more accessibility to new image technology could have a positive impact on time to diagnosis.

Objectives

The objectives are (1) to evaluate trends in time to diagnosis in SpA patients during the last decades and (2) to determine the association between clinical presentation and diagnostic delay.

Methods

Cross-sectional, retrospective international study, including 3984 patients with SpA diagnosis.

Statistical analysis

Delay in diagnosis was calculated and patients were stratified according to decade of disease onset and initial clinical presentation. Multivariate logistic model, using an early diagnosis (≤2 years) as dependent variable was used. P value<0.05 was considered statistically significant. A possible interaction between decade of disease onset and initial clinical presentation was performed.

Results

The overall median delay from disease onset to SpA diagnosis was 2.9 (p25−75=0.3–9.8) years. Diagnostic delay showed a progressive decrease during the last decades, patients with disease onset after 2010 showed a shorter delay in diagnosis than those with disease onset during 2000–2010 (m2.00 vs 0.41 years, p<0.01). Age at disease onset (OR 1.02), axial symptom (OR 16.25), peripheral arthritis (OR 6.81), decade at disease onset (OR 3.80) and extra-musculoskeletal manifestation (OR 2.89) were associated with an early diagnosis, while female gender (OR 0.66) was inversely associated.

Conclusions

The proportion of patients with early SpA diagnosis improve from 15% before 1980 to 88% when first symptoms occurred after 2010. Type and number of initial clinical presentation were independent predictors of time to diagnosis.
Real-time classification of Serengeti wildebeest behaviour with edge machine learning and a long-range IoT network
Authors: Kavwele Cyrus M. , Hopcraft J. Grant C. , Morales Juan M. , Nyaffi Gerald , Kimuya Nancy , Torney Colin J.
Published: January 2025

Globally, animal populations are facing increasing levels of environmental disturbance. Human activity, land-use change, and global warming are altering migration routes, space use, activity budgets, and the behaviour of many wildlife species. Understanding impacts on wildlife at a fine scale is essential to identify locations of increased disturbance, mitigate its effects, and predict potential population level outcomes. In this work, we introduce a low-cost animal tracking system that integrates open-source electronics, edge machine learning, and an Internet of Things network, to provide real-time information on the location and behaviour of animals. The system employs an on-board machine learning algorithm to identify distinct behaviours and then transmits classification outputs along with location data over a long-range network. We deployed the system on wildebeest ( Connochaetes taurinus (Burchell, 1823)), in Serengeti National Park, Tanzania, a highly social migratory ungulate population that is ecologically and economically vital to the region. Analysis of the transmitted data showed activity readings were consistent with location data and revealed biologically meaningful fluctuations in daily activity patterns. Our system introduces a new dimension to studying animal behaviour and movement ecology by offering immediate insights into the behaviour and location of collared animals.
Online Stable Streaming Feature Selection via Feature Aggregation
Authors: Zhou Peng , Wang Qi , Zhang Yunyun , Ling Zhaolong , Zhao Shu , Wu Xindong
Published: January 2025

Feature selection is an essential pre-process component in data mining that aims to select the most relevant features from the target dataset. Datasets are always dynamic in real-world applications, and features may exist in stream mode. Then, online streaming feature selection methods are proposed, which deal with streaming features arriving continuously in real-time. However, most existing algorithms prioritize high accuracy and low time consumption but overlook the stability of the selected features. Stable feature selection results are crucial for users in practice. For instance, in the medical field, unstable feature selection results can make it challenging for experts to identify the main causative factors of a disease. Motivated by this, this paper proposes a new Online Stable Streaming Feature Selection method via feature aggregation named OSSFS. Specifically, inspired by the cohesive MeanShift approach, OSSFS applies an incremental aggregation strategy to partition the streaming features into multiple hyperellipsoids. Then, we incrementally update and merge these hyperellipsoids with new streaming features. Finally, we select representative features from each hyperellipsoid as the final selected feature subset. Extensive experiments are conducted on several real-world datasets to compare our new method with state-of-the-art competing algorithms in cases of stability and predictive accuracy. Experimental results indicate that OSSFS achieves optimal stability without losing prediction accuracy.