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Background: The resilience of healthcare workers has gained increasing attention, yet comprehensive studies focusing on recent trends and developments are scarce. We conducted an extensive bibliometric analysis from inception to 2023 to address this gap. Methods: Publications on healthcare workers' resilience were extracted from the Web of Science Core Collection database. Bibliometric analysis was conducted with CiteSpace, VOSviewer, and Scimago Graphica, focusing on annual publications, country/region, institution, journal, author, keyword analysis, and reference co-citation analysis related to resilience in healthcare workers. Results: The analysis included 750 documents, revealing a general upward trend in publications across 67 countries/regions, 1,251 institutions, and 3,166 authors. The USA and China emerged as the top contributors, with 192 and 168 publications, respectively. Based on keyword analysis and reference co-citation analysis, the focus areas include the Resilience Scale, the impact of the COVID-19 pandemic on HCWs and their resilience, and nurse resilience. Conclusion: This study highlights the growing interest in healthcare workers' resilience by using bibliometric and visualization techniques for effective analysis. This paper will enhance scholars' understanding of the dynamic evolution of healthcare workers' resilience and identify emerging research topics.
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Introduction: The immunological characteristics that could protect children with coronavirus disease 2019 (COVID-19) from severe or fatal illnesses have not been fully understood yet. Methods: Here, we performed single-cell RNA sequencing (scRNA-seq) analysis on peripheral blood samples of 15 children (8 with COVID-19) and compared them to 18 adults (13 with COVID-19). Results: The child-adult integrated single cell data indicated that children with the disease presented a restrained response to type I interferon in most of the major immune cell types, along with suppression of upstream interferon regulatory factor and toll-like receptor expression in monocytes, which was confirmed by in vitro interferon stimulation assays. Unlike adult patients, children with COVID-19 showed lower frequencies of activated proinflammatory CD14+ monocytes, possibly explaining the rareness of cytokine storm in them. Notably, natural killer (NK) cells in pediatric patients displayed potent cytotoxicity with a rich expression of cytotoxic molecules and upregulated cytotoxic pathways, whereas the cellular senescence, along with the Notch signaling pathway, was significantly downregulated in NK cells, all suggesting more robust cytotoxicity in NK cells of children than adult patients that was further confirmed by CD107a degranulation assays. Lastly, a modest adaptive immune response was evident with more naïve T cells but less activated and proliferated T cells while less naïve B cells but more activated B cells in children over adult patients. Conclusion: Conclusively, this preliminary study revealed distinct cell frequency and activation status of major immune cell types, particularly more robust NK cell cytotoxicity in PBMC that might help protect children from severe COVID-19.
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COVID-19 , Células Matadoras Naturais , SARS-CoV-2 , Análise de Célula Única , Humanos , COVID-19/imunologia , Criança , Adulto , SARS-CoV-2/imunologia , Masculino , Feminino , Células Matadoras Naturais/imunologia , Pré-Escolar , Adolescente , Monócitos/imunologia , Monócitos/metabolismo , Pessoa de Meia-Idade , Imunidade Adaptativa , Citotoxicidade Imunológica , Adulto Jovem , Interferon Tipo I/imunologia , Senescência Celular/imunologiaRESUMO
Congenitally correct transposition of the great arteries (cc-TGA) is an extremely rare congenital cardiac malposition. It can be detected antenatally by echocardiography. This case report describes a 58-year-old female patient who presented with tachycardia. The combination of cc-TGA and isolated levocardia is incidentally diagnosed by transthoracic echocardiography and cardiac magnetic resonance imaging.
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Ecocardiografia , Levocardia , Transposição dos Grandes Vasos , Humanos , Feminino , Transposição dos Grandes Vasos/diagnóstico por imagem , Pessoa de Meia-Idade , Ecocardiografia/métodos , Levocardia/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodosRESUMO
BACKGROUND: Acute myeloid leukemia (AML) with t(8;21) manifests as a diverse hematological malignancy. Although it was categorized into a favorable subtype, 30-40% of patients experience relapse. The objective of this research was to devise a nomogram for the accurate anticipation of both overall survival (OS) and cancer-specific survival (CSS) in t(8;21) AML. METHODS: From the Surveillance, Epidemiology, and End Results (SEER) database, individuals diagnosed with t(8;21) AML from 2000 to 2018 were selected. Prognostic factors for t(8;21) AML were identified using Cox regression analysis and Akaike Information Criterion (AIC), forming the basis for constructing prognostic nomograms. RESULTS: Key variables, including first primary tumor, age group, race, and chemotherapy, were identified and integrated into the nomogram. The C-index values for the nomograms predicting OS and CSS were 0.753 (validation: 0.765) and 0.764 (validation: 0.757), respectively. Ultimately, based on nomogram scores, patients were stratified into high-risk and low-risk groups, revealing significant disparities in both OS and CSS between these groups (P < 0.001). CONCLUSION: This study innovatively crafted nomograms, incorporating clinical and therapeutic variables, to forecast the 1-, 3-, and 5-year survival rates for individuals with t(8;21) AML.
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Cromossomos Humanos Par 21 , Cromossomos Humanos Par 8 , Leucemia Mieloide Aguda , Nomogramas , Programa de SEER , Humanos , Leucemia Mieloide Aguda/genética , Leucemia Mieloide Aguda/mortalidade , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/terapia , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Cromossomos Humanos Par 8/genética , Cromossomos Humanos Par 21/genética , Translocação Genética , Prognóstico , Adolescente , Idoso , Adulto JovemRESUMO
Recently, the utilization of hydrogen-bonded organic frameworks (HOFs) with high crystallinity and inherent well-defined H-bonding networks in the field of proton conduction has received increasing attention, but obtaining HOFs with excellent water stability and prominent proton conductivity (σ) remains challenging. Herein, by employing functionalized terephthalic acids, 2,5-dihydroxyterephthalic acid, 2-hydroxyterephthalic acid, 2-nitro terephthalic acid, and terephthalic acid, respectively, four highly water-stable ionic HOFs (iHOFs), [(C8H5O6)(Me2NH2)]â2H2O (iHOF 1), [(C8H5O5)(Me2NH2)] (iHOF 2), [(C8H4NO6)(Me2NH2)] (iHOF 3) and [(C8H5O4)(Me2NH2)] (iHOF 4) were efficiently prepared by a straightforward synthesis approach in DMF and H2O solutions. The alternating-current (AC) impedance testing in humid conditions revealed that all four iHOFs were temperature- and humidity-dependent σ, with the greatest value reaching 10-2 S·cm-1. As expected, the high density of free carboxylic acid groups, crystallization water, and protonated [Me2NH2]+ units offer adequate protons and hydrophilic environments for effective proton transport. Furthermore, the σ values of these iHOFs with different functional groups were compared. It was discovered that it dropped in the following order under 100 °C and 98 % relative humidity (RH): σ iHOF 1 (1.72 × 10-2 S·cm-1) > σ iHOF 2 (4.03 × 10-3 S·cm-1) > σ iHOF 3 (1.46 × 10-3 S·cm-1) > σ iHOF 4 (4.86 × 10-4 S·cm-1). Finally, we investigated the causes of the above differences and the proton transport mechanism inside the framework using crystal structure data, water contact angle tests, and activation energy values. This study provides new motivation to develop highly proton-conductive materials.
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The tumor microenvironment (TME) has a significant impact on tumor growth and immunotherapy efficacies. However, the precise cellular interactions and spatial organizations within the TME that drive these effects remain elusive. Using advanced multiplex imaging techniques, we have discovered that regulatory T cells (Tregs) accumulate around lymphatic vessels in the peripheral tumor stroma. This localized accumulation is facilitated by mature dendritic cells enriched in immunoregulatory molecules (mregDCs), which promote chemotaxis of Tregs, establishing a peri-lymphatic Treg-mregDC niche. Within this niche, mregDCs facilitate Treg activation, which in turn restrains the trafficking of tumor antigens to the draining mesenteric lymph nodes, thereby impeding the initiation of anti-tumor adaptive immune responses. Disrupting Treg recruitment to mregDCs inhibits tumor progression. Our study provides valuable insights into the organization of TME and how local crosstalk between lymphoid and myeloid cells suppresses anti-tumor immune responses.
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Células Dendríticas , Linfócitos T Reguladores , Microambiente Tumoral , Linfócitos T Reguladores/imunologia , Animais , Microambiente Tumoral/imunologia , Camundongos , Células Dendríticas/imunologia , Células Dendríticas/metabolismo , Humanos , Antígenos de Neoplasias/imunologia , Antígenos de Neoplasias/metabolismo , Vasos Linfáticos/imunologia , Vasos Linfáticos/metabolismo , Camundongos Endogâmicos C57BL , Linfonodos/imunologia , Linhagem Celular Tumoral , Neoplasias/imunologia , Neoplasias/metabolismoRESUMO
Background: Castleman disease (CD) is a rare lymphoproliferative disease. Idiopathic multicentric CD (iMCD), representing a distinct entity in CD, is partly attributed to autoimmune abnormalities and the hyperplastic process in iMCD involving the immune system. Consequently, iMCD presents a range of overlapping manifestations with connective tissue disorder (CTD), resulting in an inability to tell whether they coexist or imitate each other. Reports of CD combined with CTD are rare, more cases are needed to be summarized and analyzed to improve the efficiency of diagnosis and accelerate the development of novel treatments. Case Description: A male pediatric patient was diagnosed with CTD in October 2019 and had been receiving regular treatment with tocilizumab and glucocorticoid or methotrexate since April 2020. He was further diagnosed with iMCD of the hyaline vascular subtype according to biopsy-proven histopathological features and imaging-proven multiple involvement in August 2021. He received 4 doses of rituximab and then a combination of thalidomide and dexamethasone for about 1 year. His clinical symptoms were well controlled throughout the disease for a long period, but inflammatory markers were repeatedly elevated, which eventually turned normal after switching to siltuximab from July 2023, although a significant elevation of interleukin-6 occurred. Conclusions: We reported a pediatric case diagnosed as CTD and iMCD, whose inflammation finally be well controlled by siltuximab. Hopefully, our work will add insight into such rare situations and it is undoubtedly that the pathophysiological mechanism of CD and CTD coexistence and prediction models of treatment response remains to be explored to facilitate the clinical management and optimal treatment.
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Alterations in extracellular matrix (ECM) architecture and stiffness represent hallmarks of cancer. Whether the biomechanical property of ECM impacts the functionality of tumor-reactive CD8+ T cells remains largely unknown. Here, we reveal that the transcription factor (TF) Osr2 integrates biomechanical signaling and facilitates the terminal exhaustion of tumor-reactive CD8+ T cells. Osr2 expression is selectively induced in the terminally exhausted tumor-specific CD8+ T cell subset by coupled T cell receptor (TCR) signaling and biomechanical stress mediated by the Piezo1/calcium/CREB axis. Consistently, depletion of Osr2 alleviates the exhaustion of tumor-specific CD8+ T cells or CAR-T cells, whereas forced Osr2 expression aggravates their exhaustion in solid tumor models. Mechanistically, Osr2 recruits HDAC3 to rewire the epigenetic program for suppressing cytotoxic gene expression and promoting CD8+ T cell exhaustion. Thus, our results unravel Osr2 functions as a biomechanical checkpoint to exacerbate CD8+ T cell exhaustion and could be targeted to potentiate cancer immunotherapy.
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Linfócitos T CD8-Positivos , Fatores de Transcrição , Animais , Feminino , Humanos , Camundongos , Linfócitos T CD8-Positivos/imunologia , Linfócitos T CD8-Positivos/metabolismo , Linhagem Celular Tumoral , Proteína de Ligação ao Elemento de Resposta ao AMP Cíclico/metabolismo , Matriz Extracelular/metabolismo , Histona Desacetilases/metabolismo , Camundongos Endogâmicos C57BL , Neoplasias/imunologia , Neoplasias/metabolismo , Receptores de Antígenos de Linfócitos T/metabolismo , Transdução de Sinais , Exaustão das Células T , Fatores de Transcrição/metabolismo , Microambiente Tumoral , Estresse MecânicoRESUMO
Background: Infantile fibrosarcoma (IFS) is the most prevalent soft tissue sarcoma in children under 1 year old and is known for its rapid growth. The tumor lacks specific immunohistochemical tumor marker and a general view of tumor microenvironment (TME). Its primary therapeutic intervention places patients at a risk of disability or mutilation. This study aimed to elucidate the universal transcriptional characteristics of IFS and explore novel targets for diagnosis and therapy using single-cell RNA sequencing (scRNA-seq). Methods: Fresh tissue samples of IFS for scRNA-seq were collected from four patients before other treatments were administered. We conducted cell clustering, inferring copy number variation from scRNA-seq (InferCNV) analysis, gene differential expression analysis, cell function evaluation, Pearson correlation analysis, and cell-cell and ligand-receptor interaction analysis to investigate the distinct ecosystem of IFS. Results: According to the single-cell resolution data, we depicted the cell atlas of IFS, which comprised 14 cell populations. Through comparison with normal cells, the malignant cells were distinguished, and potential novel markers (POSTN, IGFBP2 and CTHRC1) were identified. We also found four various functional malignant cell subtypes, three of which exhibited cancer stem cells (CSCs) phenotypes, and investigated the interplay between these subtypes and nonmalignant cells in the TME of IFS. Endothelial cells and macrophages were found to dominate the cell-cell communication landscape within the microenvironment, promoting tumorigenesis via multiple receptor-ligand interactions. Conclusions: This study provides a comprehensive characterization of the tumor transcriptome and TME of IFS at the cellular level, offering valuable insights for clinically significant advancements in the immunohistochemical diagnosis and treatment of IFS.
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OBJECTIVES: Epidemiological studies of stroke and its risk factors can help develop strategies to prevent stroke. We aimed to explore the current gender-specific prevalence of stroke and associated risk factors. METHODS: Data were collected using a structured precoded questionnaire designed by the Stroke Screening and Prevention Programme of the National Health and Wellness Commission Stroke Prevention and Control Project Committee, between June 2020 and November 2021. A total of 7394 residents took part in the study, 187 of whom had a stroke. The baseline information of each participant was obtained and included in this study. The chi-square test and Kruskal-Wallis tests were used to examine the relationship between these indicators and stroke, and then multivariate logistic regression was used to construct the prediction scale between different genders. RESULTS: of 7394 participants,4571 (61.82%) were female. The overall prevalence of stroke patients in the study population was 2.53%, Multivariate analysis found that residence status (OR = 0.43, p = 0.002) ãHCY (OR = 0.962, p = 0.000)ãPrevious TIA (OR = 0.200, p = 0.002) ãHypertension (OR = 0.33, p = 0.000) and Dyslipidemia (OR = 0.668, p = 0.028) were significant predictors of stroke. there are gender differences in the traditional risk factors for stroke, and women have more risk factors. ROC analysis confirmed the accuracy of the stroke risk model, and the AUC of the stroke risk model for the general population was 0.79 with p < 0.05. In the gender model, the female AUC was 0.796 (p < 0.05). and the male AUC was 0.786 with p < 0.05. CONCLUSION: The prevalence of stroke in adults aged 40 years and above is high in eastern China were high. management of risk factors can effectively prevent the occurrence of most strokes. more attention should be paid to gender differences associated with stroke.
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AVC Isquêmico , Humanos , Masculino , Feminino , Fatores de Risco , China/epidemiologia , Pessoa de Meia-Idade , AVC Isquêmico/epidemiologia , Idoso , Estudos Longitudinais , Prevalência , Adulto , Fatores Sexuais , Estudos de Coortes , Caracteres Sexuais , Hipertensão/epidemiologiaRESUMO
This retrospective study aimed to analyze the treatment effect and prognostic factors of pediatric acute myeloid leukemia (AML) patients with t(8;21). A total of 268 newly diagnosed pediatric AML (pAML) enrolled from 1 January 2005 to 31 December 2022 were retrospectively reviewed, and 50 (18.7%) patients harbored t(8;21) translocation. CR rate, OS, EFS, and RFS were assessed by multivariate Logistic and Cox regression models in these patients. Of the 50 patients, 2 patients abandoned treatment during the first induction course. Of the remaining 48 patients who received double-induction therapy and were included in the final analyses, CR1 and CR2 were 75.0% (36/48) and 95.8% (46/48), respectively. The overall three-year OS, EFS, and RFS were 68.4% (95% CI, 55.0-85.1), 64.2% (95% CI, 50.7-81.4), and 65.5% (95% CI, 51.9-82.8), respectively. The presence of loss of sex chromosome (LOS) at diagnosis (n = 21) was associated with a better 3-year OS [87.5% (95% CI, 72.7-100) vs. 52.7% (95% CI, 35.1-79.3), p = 0.0089], 3-year EFS [81.6% (95% CI, 64.7-100) vs. 49.7% (95% CI, 32.4-76.4), p = 0.023], and 3-year RFS [81.6% (95% CI, 64.7-100) vs. 51.7% (95% CI, 33.9-78.9), p = 0.036] than those without LOS (n = 27), and it was also an independent good prognostic factor of OS (HR, 0.08 [95% CI, 0.01-0.48], p = 0.005), EFS (HR, 0.22 [95% CI, 0.05-0.85], p = 0.029), and RFS (HR, 0.21 [95% CI, 0.05-0.90], p = 0.035). However, extramedullary leukemia (EML) featured the independent risk factors of inferior OS (HR, 10.99 [95% CI, 2.08-58.12], p = 0.005), EFS (HR, 4.75 [95% CI, 1.10-20.61], p = 0.037), and RFS (HR, 6.55 [95% CI, 1.40-30.63], p = 0.017) in pediatric individuals with t(8;21) AML. Further analysis of combining LOS with EML indicated that the EML+LOS- subgroup had significantly inferior OS (92.9%, [95% CI, 80.3-100]), EFS (86.2%, [95% CI, 70.0-100]), and RFS (86.2%, [95% CI, 80.3-100]) compared to the other three subgroups (all p < 0.001). LOS and EML are independent prognostic factors of OS, EFS, and RFS with t(8;21) pAML patients. LOS combined with EML may help improve risk stratification.
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Although crystalline metal-organic frameworks (MOFs) have gained a great deal of interest in the field of proton conduction in recent years, the low stability and poor proton conductivity (σ) of some MOFs have hindered their future applications. As a result, resolving the issues listed above must be prioritized. Due to their exceptional structural stability, MOFs with ferrocene groups that exhibit particular physical and chemical properties have drawn a lot of attention. This study describes the effective preparation of a set of three-dimensional ferrocene-based MOFs, MIL-53-FcDC-Al/Ga and CAU-43, containing both main group metals and 1,1'-ferrocene dicarboxylic acid (H2FcDC). Multiple measurements, including powder X-ray diffraction (PXRD), infrared (IR), and scanning electron microscopy (SEM), confirmed that the addition of ferrocene groups enhanced the thermal, water, and acid-base stabilities of the three MOFs. Consequently, their proton-conductive behaviors were meticulously measured utilizing the AC impedance approach, and their best proton conductivities are 5.20 × 10-3, 2.31 × 10-3, and 1.72 × 10-4 S/cm at 100 °C/98% relative humidity (RH), respectively. Excitingly, MIL-53-FcDC-Al/Ga demonstrated an extraordinarily ultrahigh σ of above 10-4 S·cm-1 under 30 °C/98% RH. Using data from structural analysis, PXRD, SEM, thermogravimetry (TG), and activation energy, their proton transport mechanisms were thoroughly examined. The fact that these MOFs are notably easy to assemble, inexpensive, toxin-free, and stable will increase the range of practical uses for them.
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INTRODUCTION: High levels of burnout are prevalent among Emergency Department staff due to chronic exposure to job stress. There is a lack of knowledge about anteceding factors and outcomes of burnout in this population. AIMS: To provide a comprehensive overview of burnout and identify its workplace antecedents and outcomes among Emergency Department staff. METHODS: The scoping study will follow the methodology outlined by the Joanna Briggs Institute. PubMed, Scopus, Web of Science, APA PsycInfo, and CINAHL databases will be searched using predefined strategies. Two reviewers will screen the title, abstract and full text separately based on the eligibility criteria. Data will be charted, coded, and narratively synthesized based on the job demands-resources model. CONCLUSION: The results will provide insights into the underlying work-related factors contributing to burnout and its implications for individuals, healthcare organizations, and patient care.
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Esgotamento Profissional , Estresse Ocupacional , Humanos , Esgotamento Profissional/epidemiologia , Estresse Ocupacional/epidemiologia , Serviço Hospitalar de Emergência , Literatura de Revisão como AssuntoRESUMO
Obtaining crystalline materials with high structural stability as well as super proton conductivity is a challenging task in the field of energy and material chemistry. Therefore, two highly stable metal-organic frameworks (MOFs) with macro-ring structures and carboxylate groups, Zr-TCPP (1) and Hf-TCPP (2) assembled from low-toxicity as well as highly coordination-capable Zr(IV)/Hf(IV) cations and the multifunctional linkage, meso-tetra(4-carboxyphenyl)porphine (TCPP) have attracted our strong interest. Note that TCPP as a large-size rigid ligand with high symmetry and multiple coordination sites contributes to the formation of the two stable MOFs. Moreover, the pores with large sizes in the two MOFs favor the entry of more guest water molecules and thus result in high H2O-assisted proton conductivity. First, their distinguished structural stabilities covering water, thermal and chemical stabilities were verified by various determination approaches. Second, the dependence of the proton conductivity of the two MOFs on temperature and relative humidity (RH) is explored in depth. Impressively, MOFs 1 and 2 demonstrated the optimal proton conductivities of 4.5 × 10-4 and 0.78 × 10-3 S·cm-1 at 100 °C/98 % RH, respectively. Logically, based on the structural information, gas adsorption/desorption features, and activation energy values, their proton conduction mechanism was deduced and highlighted.
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In the field of proton conduction, the acquisition of crystalline metal-organic frameworks (MOFs) with high stability and ultrahigh proton conductivity has been of great research value and is worth continuous exploration. Here, we greenly synthesized a three-dimensional porous MOF (MOF-801-Ce) by using [(NH4)2Ce(NO3)6 and fumaric acid as starting materials and solvothermally synthesized Hf-UiO-66-NO2 by using HfCl4 and 2-nitroterephthalic acid as starting materials. A series of measurements have shown that both MOFs exhibit good water stability, acid-base stability, and thermal stability and demonstrate outstanding proton conductivity. At 100 °C and 98% relative humidity (RH), the proton conductivities (σ) could be 2.59 × 10-3 S·cm-1 for MOF-801-Ce and 0.89 × 10-3 S·cm-1 for Hf-UiO-66-NO2. To pursue higher proton conductivity, we further adopted the evaporation approach to encapsulate imidazole molecules in the pores of the two compounds, achieving the imidazole-encapsulated MOFs, Im@MOF-801-Ce and Im@Hf-UiO-66-NO2. As expected, their σ values were significantly boosted by almost an order of magnitude up to 10-2 S·cm-1. Finally, their proton-conductive mechanisms were explored in light of the structural information, gas adsorption/desorption, and other tests. The outstanding structural stability of these MOFs and their durability of the proton conduction capability manifested that they have great promise in electrochemical fields.
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Reactivating silenced γ-globin expression through the disruption of repressive regulatory domains offers a therapeutic strategy for treating ß-hemoglobinopathies. Here, we used transformer base editor (tBE), a recently developed cytosine base editor with no detectable off-target mutations, to disrupt transcription-factor-binding motifs in hematopoietic stem cells. By performing functional screening of six motifs with tBE, we found that directly disrupting the BCL11A-binding motif in HBG1/2 promoters triggered the highest γ-globin expression. Via a side-by-side comparison with other clinical and preclinical strategies using Cas9 nuclease or conventional BEs (ABE8e and hA3A-BE3), we found that tBE-mediated disruption of the BCL11A-binding motif at the HBG1/2 promoters triggered the highest fetal hemoglobin in healthy and ß-thalassemia patient hematopoietic stem/progenitor cells while exhibiting no detectable DNA or RNA off-target mutations. Durable therapeutic editing by tBE persisted in repopulating hematopoietic stem cells, demonstrating that tBE-mediated editing in HBG1/2 promoters is a safe and effective strategy for treating ß-hemoglobinopathies.
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Edição de Genes , Hemoglobinopatias , Humanos , Hemoglobina Fetal/genética , Hemoglobina Fetal/metabolismo , gama-Globinas/genética , gama-Globinas/metabolismo , Sistemas CRISPR-Cas , Mutação/genética , Hemoglobinopatias/genética , Hemoglobinopatias/metabolismo , Células-Tronco Hematopoéticas/metabolismo , Fatores de Transcrição/metabolismoRESUMO
Castleman disease (CD) is a rare lymphoproliferative disorder of undetermined etiology. Unicentric CD (UCD) and multicentric CD (MCD) are two phenotypes of CD diagnosed by the histopathology of lymph nodes. We attempted to describe a pediatric CD cohort to optimize the management of this disease. We reviewed the medical records of pediatric patients diagnosed with CD between April, 2004, and October, 2022, at the Children's Hospital of Fudan University. Prognosis information was collected in January, 2023, by telephone inquiry. Twenty-two patients with UCD and 2 patients with MCD were identified, all with hyaline vascular (HV) type. The median ages at diagnosis were 10.75 years (IQR 8, 12.81) for UCD and 14.42 years (IQR 13.42, 15.42) for MCD. The most common lesion location of UCD was the neck (9/22, 40.91%) and abdomen (9/22, 40.91%). Systematic symptoms occurred on 10/22 (45.45%) patients with UCD and 1/2 (50%) patients with MCD, and abnormal laboratory indexes were detected in both. Resection and biopsy were performed on all patients. One out of two patients with MCD also received rituximab for upfront therapy. After a median of 4 years (IQR 1.5, 6) of follow-up time, the overall survival was 100% and the complete remission rate in UCD was 63%. There was no relapse or progression. CONCLUSIONS: Our series demonstrated that HV-UCD was the most common type in children. Resection and biopsy were used for both deterministic diagnoses and treatments. Despite the high possibility to develop systematic inflammation, children with CD showed promising outcomes. WHAT IS KNOWN: ⢠Castleman disease is a rare lymphoproliferative disorder with limited cohort studies, especially in pediatrics. ⢠The ubiquity of delayed confirmations and misdiagnoses points to a lack of knowledge about etiology and characteristics, which is a prerequisite for novel therapeutics. WHAT IS NEW: ⢠We retrospectively reviewed and analyzed the clinical and pathological symptoms, laboratory and imaging features, and treatment outcomes of a Chinese pediatric cohort with Castleman disease. ⢠Our work may improve the recognition and optimize the management of this rare disease in children.
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Hiperplasia do Linfonodo Gigante , Humanos , Criança , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/terapia , Hiperplasia do Linfonodo Gigante/patologia , Estudos Retrospectivos , Linfonodos/patologia , Resultado do Tratamento , ChinaRESUMO
Recently, researchers have focused on preparing and studying proton exchange membranes. Metal-organic frameworks (MOFs) are candidates for composite membrane fillers due to their high crystallinity and structural characteristics, and Hf-based MOFs have attracted our attention with their high porosity and high stability. Therefore, in this study, Hf-based MOFs were doped into a cost-effective chitosan matrix as fillers to fabricate composite films having excellent proton conductivity (σ). First, the nanoscale MOFs Hf-UiO-66-(OH)2 (1) and Hf-UiO-66-NH2 (2) were chemically modified by a ligand design strategy to obtain SA-1 and CBD-2 bearing free -COOH units. The proton conductivities of SA-1 and CBD-2 under optimal test conditions reached 1.23 × 10-2 and 0.71 × 10-2 S cm-1. After that, we prepared composite membranes CS/SA-1 and CS/CBD-2 by the casting method; tests revealed that the introduction of MOFs improved the stabilities and σ values of the membranes, and their best σ could reach above 10-2 S cm-1 under 100 °C/98% RH. Further structural characterization and activation energy calculation revealed the conductive mechanism of the composite films. This investigation not only proposes a novel chemical modification method for optimizing the σ of MOFs but also promotes the development of MOF-doped composite membranes and provides a basis for future applications of MOFs in fuel cells.
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With the gradual progress of research on proton-conducting metal-organic framework (MOFs), it has become a challenging task to find MOF materials that are easy to prepare and have low toxicity, high stability, and splendid proton conductivity. With the abovementioned objectives in mind, we selected the non-toxic organic ligand 2,5-furandicarboxylic acid and the low toxic quadrivalent metals zirconium(IV) or hafnium(IV) as starting materials and successfully obtained 2 three-dimensional porous MOFs, [M6O4(OH)4(FDC)4(OH)4(H2O)4] [M = ZrIV (1) and HfIV (2)], with ultrahigh water stability using a rapid and green synthesis approach. Their proton conductive ability is remarkable, thanks to the large number of Lewis acidic sites contained in their porous frameworks and the abundant H-bonding network, hydroxyl groups, as well as coordination and crystalline water molecules. The positive correlation of their proton conductivity with relative humidity (RH) and the temperature was observed. Notably, their optimized proton conductivities are 2.80 × 10-3 S·cm-1 of 1 and 3.38 × 10-3 S·cm-1 of 2 under 100 °C/98% RH, which are at the forefront of Zr(IV)/Hf(IV) MOFs with prominent proton conductivity. Logically, their framework features, nitrogen/water adsorption/desorption data, and activation energy values are integrated to deduce their proton conductivity and conducting mechanism differences.
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This work elucidates the potential impact of intramolecular H-bonds within the pore walls of covalent organic frameworks (COFs) on proton conductivity. Employing DaTta and TaTta as representative hosts, it was observed that their innate proton conductivities (σ) are both unsatisfactory and σ(DaTta)<σ(TaTta). Intriguingly, the performance of both imidazole-loaded products, Im@DaTta and Im@TaTta is greatly improved, and the σ of Im@DaTta (0.91×10-2 â S cm-1 ) even surpasses that of Im@TaTta (3.73×10-3 â S cm-1 ) under 100 °C and 98 % relative humidity. The structural analysis, gas adsorption tests, and activation energy calculations forecast the influence of imidazole on the H-bonded system within the framework, leading to observed changes in proton conductivity. It is hypothesized that intramolecular H-bonds within the COF framework impede efficient proton transmission. Nevertheless, the inclusion of an imidazole group disrupts these intramolecular bonds, leading to the formation of an abundance of intermolecular H-bonds within the pore channels, thus contributing to a dramatic increase in proton conductivity. The related calculation of Density Functional Theory (DFT) provides further evidence for this inference.