bluuebunny/crossref_metadata_2025_split huggingface dataset:
Update on B‐cell maturation antigen‐directed therapies in AL amyloidosis
Authors: Jamroziak Krzysztof , Zielonka Klaudia , Khwaja Jahanzaib , Wechalekar Ashutosh D. 
Published: January 2025

SummarySystemic light chain (AL) amyloidosis is a rare clonal plasma cell disorder characterized by the production of amyloidogenic immunoglobulin light chains, which causes the formation and deposition of amyloid fibrils, leading to multi‐organ dysfunction. Current treatment is directed at the underlying plasma cell clone to achieve a profound reduction in the monoclonal free light chain production. The standard‐of‐care first‐line therapy is a combination of daratumumab, cyclophosphamide, bortezomib and dexamethasone (D‐VCd regimen), resulting in high rates of haematological and organ responses. However, AL amyloidosis remains incurable, and all patients inevitably relapse. Hence, novel treatment options are needed for patients with an inadequate response or relapsed/refractory disease. B‐cell maturation antigen (BCMA) is a tumour necrosis factor (TNF receptor superfamily receptor overexpressed on plasma cells in multiple myeloma (MM) and AL amyloidosis. Recently, several novel anti‐BCMA immunotherapies have been approved for the treatment of relapsed/refractory MM, including antibody–drug conjugate belantamab mafodotin, bispecific antibodies teclistamab and elranatamab and chimeric antigen receptor T‐cell therapies idecabtagene vicleucel and ciltacabtagene autoleucel. Despite lower expression than in MM, BCMA is also a promising target in AL amyloidosis. This review aims to provide up‐to‐date information on the efficacy and toxicity of anti‐BCMA therapy in AL amyloidosis.
Transgender Is the Next Big Thing We Have to Really Face Psychoanalytically: What Research Can Tell Us About How Psychoanalytic, Psychodynamic and Jungian Psychotherapists Work Clinically with Transgender Clients
Authors: Full Wayne 
Published: January 2025

AbstractThis paper discusses the challenges and perspectives of psychoanalytic, psychodynamic and Jungian psychotherapists in thinking about and working clinically with transgender clients, based on interviews conducted by the author as part of a PhD study.
Experiences of South African Learners With Severe Intellectual Disabilities From Low Socioeconomic Backgrounds on Their Educational Challenges During the COVID‐19 Pandemic
Authors: Mudali Mandie Cecilia , Munongi Lucia 
Published: January 2025

ABSTRACTBackgroundThe global COVID‐19 pandemic brought various challenges for learners around the world. This paper reports on the perceptions of learners with Severe Intellectual Disabilities coming from low socioeconomic backgrounds in the South African context on the educational challenges that they experienced during the COVID‐19 pandemic.MethodsUsing the qualitative research methodology, data were collected from six learners (three females and three males) using semi‐structured individual interviews.ResultsThematic data analysis yielded four themes, namely, loss of learning time, lack of interaction with others, lack of access to resources and lack of parental support.ConclusionDespite the pandemic affecting every learner negatively, in this study, among those with Severe Intellectual Disabilities who came from low socioeconomic backgrounds, the challenges faced by children with severe intellectual disabilities were unique and likely worsened by having Severe Intellectual Disabilities.
Lymphocytes‐Associated Extracellular Vesicles Activate Natural Killer Cells in HNSCC
Authors: Li Chuwen , Liu Chun , Ma Hailong , Zhang Zhiyuan , Zhang Jianjun 
Published: January 2025

ABSTRACTSmall extracellular vesicles (sEVs) facilitate intercellular communication and play a pivotal role in tumor progression. Accumulated evidence has indicated the diversity of sEVs but with limited results revealing the landscape of heterogeneity of sEVs. The heterogeneity of cargo RNA in sEVs presents the different cell origins and indicates different functions. Here, we analyzed the heterogeneity of sEVs at droplet levels from single‐cell RNA sequencing results of head and neck squamous cell carcinoma (HNSCC) with the previously reported algorithm SEVtras. With the sEVs secretion activity calculated by SEVtras, we also found that the T cells held the major role of sEVs secretion. In addition, we found these sEVs secreted by T cells increased the cytotoxic ability of natural killer cells (NK cells), which illustrated an indirect manner for the anti‐tumor function of T cells. These results revealed the heterogeneity of cargo RNA of sEVs in HNSCC and underlined a sEVs‐dependent manner in which T cells act on NK cells and anti‐tumor immunity.
Prevalence and Predictive Features of CT‐Derived Nonalcoholic Fatty Liver Disease in Metabolically Healthy MACS
Authors: Candemir Burcu , Kisip Kadir , Akın Şafak , Sanal Hatice Tuba , Taşar Mustafa , Candemir Mustafa , Gülçelik Neşe Ersöz 
Published: January 2025

ABSTRACTObjectivePatients with mild autonomous cortisol secretion (MACS) are at increased risk of cardiometabolic outcomes, such as hyperglycemia, metabolic syndrome, and cardiovascular diseases. Nonalcoholic fatty liver disease (NAFLD) is also associated with increased cardiometabolic risk. We aimed to investigate the prevalence and predictors of NAFLD in metabolically healthy subjects with MACS.MethodsForty patients with MACS and 60 patients with nonfunctioning adrenal incidentaloma (NFAI) matched for age, gender, and body mass index were included. We excluded various diseases that may lead to NAFLD, such as diabetes, cardiovascular diseases, and liver disorders. Non‐alcoholic fatty liver disease was evaluated with unenhanced abdominal computed tomography and noninvasive fatty liver indices.ResultsPatients with MACS had lower mean liver attenuation values (Hounsfield units, HU) than those with NFAI (p = 0.001). Visceral adiposity index, hepatic steatosis index, and fatty liver index were higher in the MACS group than in the NFAI group (p = 0.009, p = 0.002, p = 0.023, respectively). However, there was no significant association between the mean liver HU value and these indices. There was a significant association between serum cortisol level after the 1 mg dexamethasone suppression test (DST) and mean liver HU value independent of other traditional risk factors in various models performed in multivariable linear regression analysis.ConclusionsOur findings suggest that MACS is associated with an increased risk of NAFLD, and serum cortisol level after 1 mg DST is an independent predictor of NAFLD in patients with MACS.
A Comparison of the Predictive Value of International Medullary Thyroid Carcinoma Grading System (IMTCGS) With That of Other Risk Factors in a Chinese Medullary Thyroid Carcinoma Cohort
Authors: Ni Jiajia , Zhang Xinyi , Liu Yalan , Ling Yan 
Published: January 2025

ABSTRACTBackgroundThe International Medullary Thyroid Carcinoma Grading System (IMTCGS) was recently introduced in medullary thyroid carcinoma (MTC). This study aimed to assess the predictive value of the IMTCGS for disease response and survival, and compare its predictive ability with that of other traditional risk factors in a Chinese MTC cohort.MethodsThe data of 137 MTC patients undergoing initial surgery between January 2004 and June 2023 were included for analysis. Histologic features were reviewed by two pathologists. Kaplan‐Meier survival analysis and Cox proportional hazard model were performed to analyse the association between risk factors (including IMTCGS high vs low grade) and progression‐free survival (PFS) and disease‐specific survival (DSS). ROC analysis and Delong's test were used to compare the predictive ability of IMTCGS with that of other risk factors.ResultsLocal recurrence, distant metastasis, and disease‐specific death were observed in 14/134 (10.45%), 3/134 (2.24%), and 6/137 (4.38%) MTC patients, respectively. IMTCGS, TNM stage, postoperative calcitonin, postoperative CEA, and vascular invasion were associated with PFS in Kaplan‐Meier survival analysis (all p < 0.05). Postoperative calcitonin was the only independent predictor for PFS in multivariate analysis (HR = 1.002, p = 0.002). ROC analysis and Delong's test showed that postoperative calcitonin had superior predictive value for structural recurrence than IMTCGS (AUC 0.90 vs. 0.64, p = 0.002). IMTCGS, TNM stage, and vascular invasion were associated with DSS in Kaplan−Meier survival analysis (both p < 0.05). In multivariate analysis, IMTCGS was the only independent predictor for DSS (HR = 11.23, p = 0.05). The AUC of IMTCGS was 0.81 (p = 0.01) for disease‐specific death.ConclusionIn this Chinese MTC cohort, IMTCGS was a powerful predictor of disease‐specific death, while postoperative calcitonin was a powerful predictor of structural recurrence.
Novel Phenotypes and Genotype–Phenotype Correlations in a Large Clinical Cohort of Patients With Kleefstra Syndrome
Authors: Frazier Zoë J. , Kilic Seyda , Osika Hailey , Mo Alisa , Quinn Meg , Ballal Sonia , Katz Tamar , Shearer A. Eliot , Horlbeck Max A. , Pais Lynn S. , Dies Kira A. , O'Donnell‐Luria Anne , Kossowsky Joe , Lipton Jonathan O. , Kleefstra Tjitske , Srivastava Siddharth 
Published: January 2025

ABSTRACTKleefstra syndrome (KLEFS) is a genetic neurodevelopmental disorder caused by haploinsufficiency of EHMT1. The full spectrum of clinical features and genotype–phenotype correlations is currently not fully understood. We performed a retrospective chart review of patients with KLEFS evaluated at the Boston Children's Hospital Kleefstra Clinic. There were 65 individuals (40 females, 25 males, mean age 9.3 years). 17% had large 9q34 deletions (≥ 1 Mb), 29% had small 9q34 deletions (< 1 Mb), and 54% had sequence variants. Global developmental delay (GDD) or intellectual disability (ID) was present in 77%. Behavioral disorders, such as autism spectrum disorder (38%), were common. Epilepsy affected 15%. Systemic health issues included structural cardiac defects (40%), hearing loss (32%), and constipation (31%). Novel features including subgroups with significant motor impairment (24%) and refractory epilepsy (9%), as well as small numbers with opsoclonus‐like eye movements (n = 2), thrombocytopenia (n = 2), progressive cerebral atrophy (n = 1), and adrenal carcinoma (n = 1). 9q34 deletion subgroups had higher rates of GDD/ID (p = 0.037), significant motor impairment (p = 0.01), epilepsy (p = 0.004), and cortical visual impairment (p = 0.003) compared to the subgroup with sequence variants. This information may be used to improve clinical care as well as inform research and future therapeutic initiatives.
S‐Nitrosylation of Dexras1 Controls Post‐Stroke Recovery via Regulation of Neuronal Excitability and Dendritic Remodeling
Authors: Han Zhou , Song Yixuan , Qin Cheng , Zhou Haihui , Han Dan , Yan Simin , Ni Huanyu 
Published: January 2025

ABSTRACTAimsStroke is a major public health concern leading to high rates of death and disability worldwide, unfortunately with no effective treatment available for stroke recovery during the repair phase.MethodsPhotothrombotic stroke was induced in mice. Adeno‐associated viruses (AAV) were microinjected into the peri‐infarct cortex immediately after photothrombotic stroke. Grid‐walking task and cylinder task were used to assess motor function. Western blotting, Golgi staining, and electrophysiology recordings were performed to uncover the mechanisms.ResultsThe ternary complex of neuronal nitric oxide synthase (nNOS), carboxy‐terminal PDZ ligand of nNOS (CAPON) and dexamethasone‐induced ras protein 1 (Dexras1) is structurally beneficial for S‐nitrosylation of Dexras1 (SNO‐Dexras1). In our previous study, uncoupling nNOS‐CAPON interaction by Tat‐CAPON‐12C promoted functional recovery after stroke. Here, we show that ischemia elevated the levels of nNOS‐Dexras1 complex and SNO‐Dexras1 in the peri‐infarct cortex in the days 4–10 after stroke induction, and as excepted, Tat‐CAPON‐12C, a peptide disrupting nNOS‐CAPON interaction, significantly reversed these changes. The above information implies that repressed SNO‐Dexras1 may mediate functional‐promoting effects of Tat‐CAPON‐12C and SNO‐Dexras1 could be the vital molecular substrate for post‐stroke functional recovery in the repair phage. Inhibiting the ischemia‐induced SNO‐Dexras1 by AAV vector‐mediated knockdown of Dexras1 or over‐expression of dominant negative Dexras1 (Dexras1‐C11S) produced sustained recovery of motor function from stroke. In contrast, up‐regulation of SNO‐Dexras1 by over‐expressing Dexras1 worsened stroke outcome. Using electrophysiology recordings, we also observed that silence of Dexras1 in the peri‐infarct cortex increased the spike number and the miniature excitatory postsynaptic currents (mEPSCs) frequency, suggesting enhancement of neuronal excitability. In addition, silence of Dexras1 increased dendritic complexity in cultured neuron and more importantly enhanced dendritic spine density in the peri‐infarct cortex, implying dendritic remodeling.ConclusionThus, inhibition of SNO‐Dexras1 positively regulates post‐stroke functional recovery via enhanced neuronal excitability and dendritic remodeling. Our results identify that SNO‐Dexras1 may serve as a novel target for promoting motor functional restoration from stroke in the delayed phase, shedding light on stroke treatment.
Internal Disruption: Examining the Relationship Between Interim CEO Successions and Executive Turnover
Authors: Langan Robert , Deuschel Nicolas 
Published: January 2025

ABSTRACTResearch Question/IssueAlthough research on interim CEO successions has increased, there remains limited knowledge about what internal repercussions interim CEO appointments have on firms, notably on incumbent executives. This study seeks to understand whether and how interim CEO appointments are related to executive turnover, focusing on the role and involvement of the board in the interim succession process.Research Findings/InsightsInterim CEO successions are related to higher executive turnover compared to direct CEO successions. This turnover is further augmented either when the preceding CEO was fired or when the interim CEO is also the board chair. We also find that, when the preceding CEO was fired, the executive turnover related to interim CEO successions is related to poorer subsequent firm performance.Theoretical/Academic ImplicationsThis research fills an important gap in the interim CEO succession literature, focusing on the internal repercussions of interim CEO appointments, particularly how incumbent executives may be affected and how this may influence firm performance. It reconciles the view that interim CEOs may engage in limited decision‐making, whereas the board uses the interim period to plan for the firm's future.Practitioner/Policy ImplicationsThis paper highlights the potential disruptions caused by interim CEO successions and offers some insights into how these may affect executives and firm performance. Moreover, this paper acts as a guide for stakeholders on the nuanced governance decisions around interim CEO appointments.
Thermochromic paint with three‐stage colour transition effect based on thermochromic microcapsules and its crosslinking modification
Authors: Tang Jingli , Wang Yichao , He Mengjuan , Huang Liqian , Wang Xueli , Yu Jianyong 
Published: January 2025

AbstractTo expand the colour change range of thermochromic materials, two types of thermochromic microcapsules (TCMs) with different colour change temperatures and effects were utilised to prepare a TCMs blending system exhibiting a three‐stage colour transition at temperatures ranging from 10 to 60°C. Thermochromic paints were then prepared based on this TCMs blending system using a modified waterborne polyurethane (WPU). The thermochromic properties of the TCMs blending system and the impact of crosslinking modification on the coating properties were investigated. The findings revealed that the ratio of TCMs influences the colour change effect and temperature of the blending system. With temperature variations, the colour of the TCMs blending system can reversibly transition between blue, green and yellow, offering a more vibrant thermochromic effect compared with conventional thermochromic materials. The modified WPU enabled the preparation of paint from the blending system, which was applied to fabric surfaces to create multistage thermochromic fabrics. The mechanical properties, water resistance and adhesion of the modified coating were enhanced, and the colour fastness to washing of the fabrics improved by two grades. This study has practical significance for the application of TCMs and the development of colour‐changing textiles.
Genomic Introgression Between Critically Endangered and Stable Species of Darwin's Tree Finches on the Galapagos Islands
Authors: Dudaniec Rachael Y. , Yadav Sonu , Catchen Julian , Kleindorfer Sonia 
Published: January 2025

ABSTRACTNatural hybridisation among rare or endangered species and stable congenerics is increasingly topical for the conservation of species‐level diversity under anthropogenic impacts. Evidence for beneficial genes being introgressed into or selected for in hybrids raises concurrent questions about its evolutionary significance. In Darwin's tree finches on the island of Floreana (Galapagos Islands, Ecuador), the Critically Endangered medium tree finch (Camarhynchus pauper) undergoes introgression with the stable small tree finch (Camarhynchus parvulus), and hybrids regularly backcross with C. parvulus. Earlier studies in 2005–2013 documented an increase in the frequency of Camarhynchus hybridisation on Floreana using field‐based and microsatellite data. With single nucleotide polymorphism (SNP) data from the same Floreana tree finches sampled in 2005 and 2013 (n = 95), we examine genome‐wide divergence across parental and hybrid birds and evidence for selection in hybrids. We found that just 18% of previously assigned hybrid birds based on microsatellites could be assigned to hybrids using SNPs. Over half of the previously assigned hybrids (63%) were reassigned to C. parvulus, though parental species showed concordance with prior assignments. Of 4869 private alleles found in hybrid birds, 348 were at a high frequency (≥ 0.30) that exceeded their parental species of origin 89%–96% of the time. For private alleles detected in both years (N = 536) between 11%–76% of alleles underwent a frequency increase and 13%–61% a frequency decrease between 2005 and 2013, which was sensitive to sampling effort. We identified 28 private alleles that were candidates under selection via local PCA and outlier tests. Alleles were annotated to genes associated with inflammation, immunity, brain function and development. We provide evidence that introgression among a critically endangered and stable species of Darwin's tree finch across years may aid in the retention of adaptive alleles and genetic diversity in birds threatened with extinction.
Body mass index affects imatinib exposure: Real‐world evidence from TDM with adaptive dosing
Authors: Maroselli Paul , Fanciullino Raphaelle , Colle Julien , Farnault Laure , Roche Pauline , Venton Geoffroy , Costello Régis , Ciccolini Joseph 
Published: January 2025

AbstractBackgroundImatinib is the treatment of elderly or frail patients with chronic myeloid leukemia (CML). Trough levels of around 1000 ng/ml are considered as the target exposure.ObjectivesWe searched for baseline parameters associated with imatinib pharmacokinetics, and studied the clinical impact of subsequent adaptive dosing.MethodsWe present data from 60 adult CML patients upon imatinib with therapeutic drug monitoring (TDM) and adaptive dosing.ResultsMean trough levels after treatment initiation were 994.2 ± 560.6 ng/ml with 56% inter‐patient variability). Only 29% of patients were in the therapeutic range. Body weight, height, body surface area, body mass index (BMI), and age were associated with imatinib plasma levels on univariate analysis. Age and BMI remained the only parameters associated with imatinib trough levels on multivariate analysis. As severe toxicities have been previously reported in patients with low BMI treated with standard imatinib, we evaluated the extent to which low BMI may lead to plasma overexposure. We found a statistically significant difference in trough imatinib levels in patients with BMI < 18.5 kg/m2, with exposure +61.5% higher than in patients with 18.5 < BMI ≤  24.9 and +76.3% higher than in patients with BMI ≥ 25. After TDM with adaptive dosing, a statistically significant difference in dosing between patients was observed, with doses ranging from 200 to 700 mg. No difference in toxicity or efficacy was observed regardless of BMI after adaptive dosing.ConclusionOur data suggest that low BMI has a significant impact on imatinib exposure but that pharmacokinetically‐guided dosing limits its clinical impact in patients.
Pollinator visits increase with bloom amount but decline with building height on extensive green roofs
Authors: Underwood Shannon M. , Sookhan Nicholas , Hung Keng‐Lou James , MacIvor J. Scott 
Published: January 2025

Abstract
Green roofs provide foraging and nesting resources for pollinators that would otherwise be absent. However, green roofs are isolated from ground level, limiting habitat to only species that can reach them. In Eastern North America, green roof design often prioritises water conservation and plant survival, and so species in the genera Sedum and Phedimus (hereafter, stonecrops) that are hardy and drought tolerant are mainly planted.
The purpose of this study was to investigate how building height and bloom amount shape flower‐visiting insect communities on extensive green roofs (EGRs). Bees, wasps, and flies (hereafter, pollinators) were surveyed on five plant species (four non‐native stonecrops: Sedum acre, Sedum album, Phedimus kamtschaticus, Phedimus spurius, and one native herbaceous plant: Rudbeckia hirta) from six EGRs and two replicated ground‐level control sites in 2019.
We identified 26 pollinator species and found that stonecrops and Rudbeckia showed distinct blooming periods, with the stonecrops flowering from June to August and Rudbeckia from August to September. Percent flowering stonecrops during the early bloom was significantly positively correlated with bee abundance and species richness. Pollinator communities determined from distinct stonecrop species were compositionally more alike to one another than R. hirta. The inclusion of R. hirta lengthened the bloom period of stonecrop‐dominated EGRs and attracted five additional bee species. We further determined that pollinator abundance and species richness were negatively correlated with building height.
Despite its limited scope, our data suggest that pollinator habitat design on EGRs should prioritise low‐rise buildings and flowering species with abundant blooms that occur at different times than stonecrops, ensuring complementary flowering periods.