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1.
Clin Exp Rheumatol ; 2024 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-38607693

RESUMO

OBJECTIVES: Coronary artery calcification (CAC) is frequently observed in Takayasu's arteritis (TAK). Our objective is to calculate the prevalence and severity of CAC in TAK, while evaluating the influence of traditional cardiovascular risk factors, glucocorticoid exposure, and disease activity on CAC. METHODS: This retrospective study involved 155 TAK patients. We measured the Agatston score by coronary computed tomography angiography (CCTA) and categorised all patients into groups with or without CAC (41 vs. 114) to compare clinical characteristics and ancillary findings between the two groups. RESULTS: Among the TAK patients, a total of 41 TAK patients (26.45%) exhibited CAC. Age of onset, disease duration, history of hypertension, history of hyperlipidaemia, Numano V and glucocorticoid use emerged as the independent risk factors for developing CAC in TAK (OR [95% CI] 1.084[1.028-1.142], p=0.003; 1.005 [1.001-1.010], p=0.020; 4.792 [1.713-13.411], p=0.003; 4.199 [1.087-16.219], p=0.037; 3.287 [1.070-10.100], p=0.038; 3.558[1.269-9.977], p=0.016). Nonetheless, CAC was not associated with disease activity. Moreover, the extent of calcification score in TAK showed a positive correlation with the number of traditional cardiovascular risk factors. CONCLUSIONS: We recommend CCTA screening for Numano V classified TAK patients. Glucocorticoid usage significantly escalates the risk of CAC. Therefore, in cases of effectively controlled disease, the inclusion of immunosuppressants aimed at reducing glucocorticoid dosage is advisable.

2.
Clin Neurol Neurosurg ; 240: 108283, 2024 Apr 09.
Artigo em Inglês | MEDLINE | ID: mdl-38608350

RESUMO

OBJECTIVES: The relationship between cognitive function and frailty in moyamoya disease (MMD) remains unclear, and the underlying mechanism is poorly understood. This study aims to investigate whether white matter hyperintensities (WMHs) mediate the association between frailty and cognitive impairment in MMD. METHODS: Patients with MMD were consecutively enrolled in our study from January 2021 to May 2023. Pre-admission frailty and cognition were assessed using the Clinical Frailty Scale (CFS) and cognitive tests, respectively. Regional deep WMH (DWMH) and periventricular WMH (PWMH) volumes were calculated using the Brain Anatomical Analysis using Diffeomorphic deformation toolbox based on SPM 12 software. Multivariate logistic regression analysis was conducted to evaluate the association between frailty and cognitive function in MMD. Mediation analysis was performed to assess whether WMHs explained the association between frailty and cognition. RESULTS: A total of 85 patients with MMD were enrolled in this study. On the basis of the CFS scores, 24 patients were classified as frail, 38 as pre-frail, and 23 as robust. Significant differences were observed in learning, memory, processing speed, executive functions, and semantic memory among the three groups (p < 0.001). Frailty was independently associated with memory and executive functions (p < 0.05); even after controlling for WMH. Mediation analysis indicated that the associations of frailty with memory and executive functions were partially mediated by WMH, DWMH, and PWMH (p < 0.05). CONCLUSION: Frailty is significantly correlated with a higher risk of cognitive impairment in MMD, even after adjusting for other covariates. WMHs partially mediate the association between frailty and cognitive impairment.

3.
Ultrason Sonochem ; 105: 106872, 2024 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-38599128

RESUMO

The present study aimed to investigate the potential of ultrasonic treatment during fermentation for enhancing the quality of fortified wines with varying time and power settings. Chemical analysis and sensory evaluation were conducted to assess the impact of ultrasonic treatment on wine quality. Results showed that ultrasonic treatment could increase total anthocyanin and total phenol content, reduce anthocyanin degradation rate, and improve color stability. Moreover, ethyl carbamate content was lower in the ultrasonic group after aging compared to non-ultrasonic group. A combination of 200 W for 20 min resulted in higher sensory scores and more coordinated taste, while a combination of 400 W for 40 min produced higher levels of volatile compounds (21860.12 µg/L) leading to a richer and more elegant aroma. Therefore, ultrasound can be used as a potential technology to improve the quality of wine.

4.
Methods Mol Biol ; 2795: 95-104, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38594531

RESUMO

Photobodies (PBs) are subnuclear membraneless organelles that self-assemble via the condensation of the plant photoreceptor and thermosensor phytochrome B (phyB). Changes in the light and temperature environment directly modulate PB formation and maintenance by altering the number and size of PBs. In thermomorphogenesis, increases in the ambient temperature incrementally reduce the number of PBs, suggesting that individual PBs possess distinct thermostabilities. Here, we describe a detailed protocol for characterizing cell type-specific PB dynamics induced by warm temperatures in Arabidopsis.


Assuntos
Proteínas de Arabidopsis , Arabidopsis , Proteínas de Arabidopsis/genética , Proteínas de Arabidopsis/metabolismo , Luz , Arabidopsis/metabolismo , Fitocromo B/genética , Fitocromo B/metabolismo , Temperatura , Regulação da Expressão Gênica de Plantas
5.
Front Endocrinol (Lausanne) ; 15: 1364316, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38590823

RESUMO

Background: Leukemia patients undergoing cryopreserved ovarian tissue transplantation (OTT) may carry a high risk of disease induction. Measurable residual disease (MRD) in bone marrow is linked to an elevated risk of relapse. It is controversial whether leukemia patients must be allowed to achieve measurable residual disease negative (MRD-negative) status instead of measurable residual disease positive (MRD-positive) status before ovarian tissue cryopreservation (OTC). Objective: To explore the safety and efficacy of OTT in acute leukemia patients with different MRD status by using xenotransplantation. Method: Cryopreserved ovarian tissue from 19 leukemia patients was thawed and xenotransplanted to ovariectomized BALB/C nude mice (n=36). The mice were divided into 2 groups based on the patient's MRD status before OTC: MRD-negative group (n=18) and MRD-positive group (n=18), additionally, a control group consisted of ovariectomized mice (n=9). Body weight was measured weekly and mortality, emaciation, and other abnormalities were recorded. Twenty-six weeks post-surgery, livers, spleens, uteruses, and ovarian grafts were removed for macroscopic and histological examinations to evaluate the efficacy of xenotransplantation and assess malignant cell contamination in mice. Results: Follicle growth was visible in the ovarian grafts of the MRD-negative and MRD-positive groups. Compared with the ovariectomized group, a significant decrease in body weight (p<0.01) was noted, the uterine volume was notably larger, estradiol (E2) levels were significantly higher (p<0.01), and follicle-stimulating hormone (FSH) levels were significantly lower (p<0.001) in the other two groups. Mice in the MRD-positive group showed a significantly higher incidence of death (p<0.001) and emaciation (p<0.01), compared to the MRD-negative group. Histological observation revealed the presence of malignant cells in the grafts, livers, and spleens of 3 mice in the MRD-positive group. No abnormalities were observed in the mice from the MRD-negative group in both macroscopic and histological observations except one mouse was sacrificed for ascites unrelated to leukemia relapse. Conclusion: For leukemia patients having ovarian tissue preserved in the first and only centralized human ovarian tissue cryobank in China, immunodeficient mice xenotransplantation can be a method to evaluate the safety and efficacy of OTT; the risk of malignant cell reimplantation due to OTT is higher in leukemia patients with MRD-positive status than those with MRD-negative status before OTC.


Assuntos
Medula Óssea , Leucemia , Feminino , Humanos , Animais , Camundongos , Transplante Heterólogo , Camundongos Nus , Emaciação , Camundongos Endogâmicos BALB C , Criopreservação , Recidiva
6.
Vaccine ; 2024 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-38594120

RESUMO

BACKGROUND: The study evaluated the protective effect of 13-valent pneumococcal polysaccharide conjugate vaccine (PCV13) against all-cause hospitalized pneumonia in children in Beijing. METHODS: Based on the vaccination record and inpatient medical record database of Beijing, children born in 2017 in Beijing, matched by age, gender, and district of the children with the ratio of 1:4, were selected as the vaccinated and unvaccinated groups according whether if vaccinated with PCV13. The incidence rate and 95 % confidence interval (95 %CI), vaccine effectiveness (VE) and direct medical costs of all-cause hospitalized pneumonia were calculated and compared within the same period of 12 months, 18 months, 24 months and 30 months after the birth of the child. RESULTS: The decreased incidence rates of all-cause hospitalized pneumonia were observed at the four points in the PCV13 vaccinated group compared to the unvaccinated group, which were significant at the points of 12 months (0.42 % vs. 0.72 %, P = 0.001), 18 months (0.90 % vs. 1.26 %, P = 0.002) and 24 months (1.37 % vs. 1.65 %, P = 0.046). The VE of PCV13 against all-cause hospitalized pneumonia within 12 months was the highest as 41.9 % (95 % CI 19.6 %, 58.0 %), followed by 29.3 % (95 % CI 11.4 %, 43.5 %) within 18 months, 17.1 % (95 % CI 0.3 %, 31.1 %) within 24 months and it almost disappeared within 30 months. The VE of 4-dose vaccination within 18 months and 24 months were 39.9 % (95 % CI 20.3 %, 54.7 %) and 27.2 % (95 % CI 8.6 %, 42.0 %), respectively. The median hospitalization cost of the children in the vaccinated group was higher at the four points but without significance. CONCLUSIONS: PCV13 had a certain protective effect on all-cause hospitalized pneumonia, and the booster immunization strategy had the best protective effect with great public health significance to enter the immunization program.

7.
Reprod Sci ; 2024 Apr 09.
Artigo em Inglês | MEDLINE | ID: mdl-38594585

RESUMO

MicroRNAs (miRNAs) are a class of short single-stranded, noncoding RNAs that affect the translation of mRNAs by imperfectly binding to homologous 3'UTRs. Research on miRNAs in ovarian diseases is constantly expanding because miRNAs are powerful regulators of gene expression and cellular processes and are promising biomarkers. miRNA mimics, miRNA inhibitors and molecules targeting miRNAs (antimiRs) have shown promise as novel therapeutic agents in preclinical development. Granulosa cells (GCs) are supporting cells for developing oocytes in the ovary. GCs regulate female reproductive health by producing sex hormones and LH receptors. Increasing research has reported the relevance of miRNAs in GC pathophysiology. With in-depth studies of disease mechanisms, there are an increasing number of studies on the biomolecular pathways of miRNAs in gynecology and endocrinology. In the present review, we summarize the different functions of GC-related microRNAs in various ovarian disorders, such as polycystic ovary syndrome, premature ovarian insufficiency, premature ovarian failure and ovarian granulosa cell tumors.

8.
J Fish Biol ; 2024 Apr 10.
Artigo em Inglês | MEDLINE | ID: mdl-38596840

RESUMO

The pervasive utilization of plastics and their integration into ecosystems has resulted in significant environmental issues, particularly the pollution of microplastics (MPs). In aquaculture, high-fat feed (HFD) is frequently employed to enhance the energy intake and economic fish production. This study utilized zebrafish as a model organism to investigate the impact of concurrent exposure to HFD and MPs on fish intestinal pathology damage and intestinal microbiome. The experimental design involved the division of zebrafish into two groups: one receiving a normal diet (ND) and the other receiving HFD. The zebrafish were exposed to a control group, as well as polystyrene (PS) MPs of varying sizes (5 and 50 µm). Histopathological examination revealed that the combination of 5 µm MPs and HFD resulted in the most significant damage to the zebrafish intestinal tract. Furthermore, gut microbiome assays indicated that exposure to MPs and HFD altered the composition of the gut microbiome. This study demonstrates that in aquaculture, the issue of HFD must be considered alongside concerns about MPs contamination, as both factors appear to have a combined effect on the intestinal pathology damage and intestinal microbiome. The findings of this research offer valuable insights for the improvement of fish farming practices.

9.
Clin Rheumatol ; 2024 Apr 10.
Artigo em Inglês | MEDLINE | ID: mdl-38598024

RESUMO

OBJECTIVE: The goal of the present study was to investigate the correlation between serum 25-hydroxyvitamin D [25(OH)D] levels and disease remission in Takayasu arteritis (TAK) patients. METHODS: This retrospective study included 59 patients in the study group and 80 patients in the validation cohort with TAK. After 6 months of therapy, patients were re-evaluated, and serum 25(OH)D levels were compared before and after treatment. Correlations between changes in 25(OH)D levels and changes in disease activity scores (NIH, ITAS2010, ITAS.A) were analyzed. Additionally, a predictive cut-off value for disease remission was determined. RESULTS: After 6 months of therapy, serum 25(OH)D levels in TAK patients significantly increased compared to baseline [(18.33 ± 7.25)µg/L vs (11.77 ± 4.14) µg/L] (P < 0.001). Positive correlations were observed between the increasing changes in the 25(OH)D level and the decreasing changes in the reduced NIH, ITAS2010, and ITAS.A scores (r = 0.455, P < 0.001; r = 0.495, P < 0.001; and r = 0.352 P = 0.006, respectively). A change of 8.45 µg/L in 25(OH)D level was identified as the predictive cut-off value for TAK remission (sensitivity 54.1%, specificity 90.9%, area under the curve = 0.741). Similarly for patients with normal baseline ESR, sensitivity is 68.0%, specificity is 92.3%, and area under the curve is 0.831, and for patients with normal baseline CRP, sensitivity is 58.3%, specificity is 90.0%, and area under the curve is 0.748. Validation in an additional 80 patients demonstrated a higher remission rate in those with a 25(OH)D level change > 8.45 µg/L. CONCLUSION: Serum 25(OH)D levels significantly increased after treatment in TAK patients, and an increase of ≥ 8.45 µg/L was predictive of disease remission, especially in individuals with normal baseline ESR and/or CRP levels. Key Points • Following treatment, there was a significant increase in serum 25(OH)D levels among TAK patients. • The elevated changes in 25(OH)D levels before and after treatment demonstrated a positive correlation with the reduction in disease activity scores. • In patients with TAK before and after treatment, an elevation in serum 25(OH)D levels exceeding 8.45 µg/L serves as an indicator for disease remission, particularly prominent in individuals with normal baseline ESR and/or CRP levels.

10.
Artigo em Inglês | MEDLINE | ID: mdl-38616759

RESUMO

BACKGROUND: Hepatocellular carcinoma (HCC) is a globally prevalent malignancy accompanied by high incidence, poor outcomes, and high mortality. Anthocyanins can inhibit tumor proliferation, migration, invasion, and promote apoptosis. Moreover, autophagy-related genes (ARGs) may play vital roles in HCC progression. This study aimed to decipher the mechanisms through which anthocyanins influence HCC via ARGs and to establish a novel prognostic model. METHODS: Based on data from public databases, differential analysis and the Venn algorithm were employed to detect intersecting genes among differentially expressed genes (DEGs), anthocyanin- related targets, and ARGs. Consensus clustering was implemented to delineate molecular subtypes of HCC. The prognostic model was developed by Cox regression analyses. CIBIRSORT was engaged to assess the immune cell infiltration. Kaplan-Meier (KM) analysis and receiver operating characteristic (ROC) curve were utilized to evaluate the predictive efficiency of the prognostic signature. RESULTS: A total of 36 intersecting genes were identified from overlapping 1524 ARGs, 537 anthocyanin- related targets, and 5247 DEGs. Consensus clustering determined three molecular subtypes (cluster 1, cluster 2, and cluster 3). Cluster 1 showed worse outcomes and remarkably higher abundances of plasma cells and T follicular helper cells. Furthermore, four prognostic signatures [KDR (Kinase insert domain receptor), BAK1 (BCL2 antagonist/killer 1), HDAC1 (Histone deacetylase 1), and CDK2 (Cyclin-dependent kinase 2)] were identified and showing substantial predictive efficacy. CONCLUSION: This investigation identified three molecular subtypes of HCC patients and proposed a promising prognostic signature comprising KDR, BAK1, HDAC1, and CDK2, which could supply further robust evidence for additional clinical and functional studies.

11.
BMC Cancer ; 24(1): 458, 2024 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-38609917

RESUMO

BACKGROUND: The identification of survival predictors is crucial for early intervention to improve outcome in acute myeloid leukemia (AML). This study aim to identify chest computed tomography (CT)-derived features to predict prognosis for acute myeloid leukemia (AML). METHODS: 952 patients with pathologically-confirmed AML were retrospectively enrolled between 2010 and 2020. CT-derived features (including body composition and subcutaneous fat features), were obtained from the initial chest CT images and were used to build models to predict the prognosis. A CT-derived MSF nomogram was constructed using multivariate Cox regression incorporating CT-based features. The performance of the prediction models was assessed with discrimination, calibration, decision curves and improvements. RESULTS: Three CT-derived features, including myosarcopenia, spleen_CTV, and SF_CTV (MSF) were identified as the independent predictors for prognosis in AML (P < 0.01). A CT-MSF nomogram showed a performance with AUCs of 0.717, 0.794, 0.796 and 0.792 for predicting the 1-, 2-, 3-, and 5-year overall survival (OS) probabilities in the validation cohort, which were significantly higher than the ELN risk model. Moreover, a new MSN stratification system (MSF nomogram plus ELN risk model) could stratify patients into new high, intermediate and low risk group. Patients with high MSN risk may benefit from intensive treatment (P = 0.0011). CONCLUSIONS: In summary, the chest CT-MSF nomogram, integrating myosarcopenia, spleen_CTV, and SF_CTV features, could be used to predict prognosis of AML.


Assuntos
Leucemia Mieloide Aguda , Nomogramas , Humanos , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Área Sob a Curva , Leucemia Mieloide Aguda/diagnóstico por imagem
12.
Inorg Chem ; 63(15): 7080-7088, 2024 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-38574395

RESUMO

Ceramic capacitors have great potential for application in power systems due to their fantastic energy storage performance (ESP) and wide operating temperature range. In this study, the (1 - x)Bi0.5Na0.47Li0.03Sn0.01Ti0.99O3-xKNbO3 (BNLST-xKN) energy storage ceramics were synthesized through the solid-phase reaction method. The addition of KN disrupts the long-range ferroelectric order of the BNLST ceramic, inducing the emergence of polar nanoregions (PNRs), which enhances the ESP of the ceramics. The BNLST-0.2KN ceramic demonstrates a high recovered energy density (Wrec ∼ 3.66 J/cm3) and efficiency (η ∼ 85.8%) under a low electric field of 210 kV/cm. Meantime, it exhibits a large current density (CD ∼ 831.74 A/cm2), high power density (PD ∼ 78.86 MW/cm3), and fast discharge rate (t0.9 ∼ 0.1 µs), along with good temperature stability and excellent fatigue stability. These properties make the BNLST-0.2KN ceramic a promising candidate for energy storage applications in low electric fields.

13.
BMC Nurs ; 23(1): 221, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38561710

RESUMO

BACKGROUND: The outbreak of Corona Virus Disease (COVID-19) in 2019 has continued until now, posing a huge threat to the public's physical and mental health, resulting in different degrees of mental health problems. As a vulnerable segment of the public, anxiety is one of the most common mental health problems among COVID-19 patients. Excessive anxiety aggravates the physical and psychological symptoms of COVID-19 patients, which is detrimental to their treatment and recovery, increases financial expenditure, affects family relations, and adds to the medical burden. OBJECTIVE: This study aimed to explore the role of psychological capital and self-esteem in the relationship between insomnia and anxiety, thereby shedding light on the mechanism of the effect of insomnia on anxiety in COVID-19 patients. METHODS: A cross-sectional study was conducted from April to May 2022 in Fangcang hospital in Shanghai, China. The self-administered questionnaires were distributed to 718 COVID-19 patients via cell phone using the Internet platform "Questionnaire Star", which included Athens Insomnia Scale, Psychological Capital Questionnaire, Self-esteem Scale, Self-Rating Anxiety Scale, gender, age, marital status, education. Data analysis was performed using descriptive analysis, independent-samples t-test, one-way analysis of variance, Pearson correlation analysis, ordinary least-squares regression, and bootstrap method. RESULTS: Education background had significant impact on anxiety in COVID-19 patients (F = 7.70, P < 0.001). Insomnia, psychological capital, self-esteem and anxiety were significantly correlated, respectively (P < 0.001). And Regression analysis showed that insomnia had a direct negative predictive effect on psychological capital (ß = -0.70, P < 0.001) and self-esteem (ß = -0.13, P < 0.001). Psychological capital had a direct positive predictive effect on self-esteem (ß = 0.12, P < 0.001). Insomnia had a direct positive predictive effect on anxiety (ß = 0.61, P < 0.001). Both psychological capital and self-esteem had significant negative predictive effects on anxiety (ß = -0.06, P < 0.05; ß = -0.72, P < 0.001). The results showed that the mediating effect of psychological capital and self-esteem was significant, and the mediating effect value was 0.21. First, the indirect effect consisting of insomnia - psychological capital - anxiety was 0.04, showing that psychological capital had a significant mediating effect. Second, the indirect effect consisting of insomnia-self-esteem-anxiety had a value of 0.10, indicating that self-esteem had a significant mediating effect. Third, the indirect effect consisting of insomnia-psychological capital-self-esteem-anxiety had a value of 0.06, suggesting that psychological capital and self-esteem had a significant chain mediating effect between insomnia and anxiety. CONCLUSIONS: Insomnia had a significant positive predictive effect on anxiety. Insomnia was first associated with a decrease in psychological capital, followed by a sequential decrease in self-esteem, which in turn was associated with increased anxiety symptoms in COVID-19 patients. Therefore, focusing on improving the psychological capital and self-esteem of patients can help alleviate the anxiety caused by insomnia in COVID-19 patients. It is recommended that patients and health care professionals increase the psychological capital and Self-esteem of COVID-19 patients through various methods to counter the effects of insomnia on anxiety.

14.
J Cardiothorac Surg ; 19(1): 167, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38561765

RESUMO

BACKGROUND: The implantation of left ventricular assist devices (LVADs) as a bridge to transplantation or as destination therapy in end-stage heart failure patients is frequently complicated by the emergence of ventricular arrhythmias (VAs). These arrhythmias have been implicated in precipitating deleterious clinical outcomes, increased mortality rates and augmented healthcare expenditures. CASE PRESENTATION: We present a challenging case of a 49-year-old male with a history of dilated cardiomyopathy who received an LVAD. Post-implantation, the patient suffered from intractable VAs, leading to multiple rehospitalizations and hemodynamic deterioration. Despite exhaustive medical management and electrical cardioversion attempts, the patient's VAs persisted, ultimately necessitating prioritization for cardiac transplantation. DISCUSSION: This case highlights the challenges in managing VAs in LVAD patients and the importance of multidisciplinary collaboration. While pharmacological intervention is the initial strategy, catheter ablation may be considered in selected cases when medication is insufficient. In instances of intractable VAs, expeditious listing for heart transplantation as a high-priority candidate is advisable when feasible.


Assuntos
Insuficiência Cardíaca , Coração Auxiliar , Masculino , Humanos , Pessoa de Meia-Idade , Coração Auxiliar/efeitos adversos , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/cirurgia , Arritmias Cardíacas/etiologia , Hemodinâmica , Cardioversão Elétrica , Resultado do Tratamento
15.
Artigo em Inglês | MEDLINE | ID: mdl-38568462

RESUMO

PURPOSE: This study aimed to identify the genetic causes of male infertility and primary ciliary dyskinesia (PCD)/PCD-like phenotypes in three unrelated Han Chinese families. METHODS: We conducted whole-exome sequencing of three patients with male infertility and PCD/PCD-like phenotypes from three unrelated Chinese families. Ultrastructural and immunostaining analyses of patient spermatozoa and respiratory cilia and in vitro analyses were performed to analyze the effects of SPEF2 variants. Intracytoplasmic sperm injection (ICSI) was administered to three affected patients. RESULTS: We identified four novel SPEF2 variants, including one novel homozygous splicing site variant [NC_000005.10(NM_024867.4): c.4447 + 1G > A] of the SPEF2 gene in family 1, novel compound heterozygous nonsense variants [NC_000005.10(NM_024867.4): c.1339C > T (p.R447*) and NC_000005.10(NM_024867.4): c.1645G > T (p.E549*)] in family 2, and one novel homozygous missense variant [NC_000005.10(NM_024867.4): c.2524G > A (p.D842N)] in family 3. All the patients presented with male infertility and PCD/likely PCD. All variants were present at very low levels in public databases, predicted to be deleterious in silico prediction tools, and were further confirmed deleterious by in vitro analyses. Ultrastructural analyses of the spermatozoa of the patients revealed the absence of the central pair complex in the sperm flagella. Immunostaining of the spermatozoa and respiratory cilia of the patients validated the pathogenicity of the SPEF2 variants. All patients carrying SPEF2 variants underwent one ICSI cycle and delivered healthy infants. CONCLUSION: Our study reported four novel pathogenic variants of SPEF2 in three male patients with infertility and PCD/PCD-like phenotypes, which not only extend the spectrum of SPEF2 mutations but also provide information for genetic counseling and treatment of such conditions.

16.
J Virus Erad ; 10(1): 100366, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38586471

RESUMO

Objectives: To explore epidemiological changes of Japanese encephalitis (JE) in a long-time span and evaluate the impact of mass immunisation. Method: Data on JE cases from hospitals and the county Centers for Disease Control and Prevention in Guizhou Province was collected between 2005 and 2021. Epidemiological changes were analyzed according to a series of policy implementations and the coronavirus disease 2019 (COVID-19) pandemic. Results: A total of 5138 JE cases and 152 deaths were reported in Guizhou Province during 2005-2021. The average incidence and case fatality rates were 0.83/100,000 and 2.96%, respectively. The JE prevalence showed a declining trend over the years with the reduced incidence gap between age groups and narrowing of the high-epidemic regions. During the COVID-19 pandemic, the JE activity reached its nadir in 2020. The inclusion in the Expanded Program on Immunization of the JE vaccine and catch-up immunisations showed a significant impact on the JE declining incidence rate. Conclusions: The implementation of JE immunisation programs has played a crucial role in controlling its spread. Continued efforts should be made to maintain high coverage of the JE vaccine and strengthen disease surveillance systems, ensuring JE effective control and eventual elimination.

17.
Fish Shellfish Immunol ; 149: 109532, 2024 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-38579977

RESUMO

C-type lectins (CTLs) execute critical functions in multiple immune responses of crustaceans as a member of pattern recognition receptors (PRRs) family. In this study, a novel CTL was identified from the exoskeleton of the oriental river prawn Macrobrachium nipponense (MnLec3). The full-length cDNA of MnLec3 was 1150 bp with an open reading frame of 723 bp, encoding 240 amino acids. MnLec3 protein contained a signal peptide and one single carbohydrate-recognition domain (CRD). MnLec3 transcripts were widely distributed at the exoskeleton all over the body. Significant up-regulation of MnLec3 in exoskeleton after Aeromonas hydrophila challenged suggested the involvement of MnLec3 as well as the possible function of the exoskeleton in immune response. In vitro tests with recombinant MnLec3 protein (rMnLec3) manifested that it had polysaccharide binding activity, a wide spectrum of bacterial binding activity and agglutination activity only for tested Gram-negative bacteria (Escherichia coli, Vibrio anguillarum and A. hydrophila). Moreover, rMnLec3 significantly promoted phagocytic ability of hemocytes against A. hydrophila in vivo. What's more, MnLec3 interference remarkably impaired the survivability of the prawns when infected with A. hydrophila. Collectively, these results ascertained that MnLec3 derived from exoskeleton took an essential part in immune defense of the prawns against invading bacteria as a PRR.

18.
Angew Chem Int Ed Engl ; : e202404058, 2024 Mar 25.
Artigo em Inglês | MEDLINE | ID: mdl-38528771

RESUMO

Ultrathin continuous metal-organic framework (MOF) membranes have the potential to achieve high gas permeance and selectivity simultaneously for otherwise difficult gas separations, but with few exceptions for zeolitic-imidazolate frameworks (ZIF) membranes, current methods cannot conveniently realize practical large-area fabrication. Here, we propose a ligand back diffusion-assisted bipolymer-directed metal ion distribution strategy for preparing large-area ultrathin MOF membranes on flexible polymeric support layers. The bipolymer directs metal ions to form a cross-linked two-dimensional (2D) network with a uniform distribution of metal ions on support layers. Ligand back diffusion controls the feed of ligand molecules available for nuclei formation, resulting in the continuous growth of large-area ultrathin MOF membranes. We report the practical fabrication of three representative defect-free MOF membranes with areas larger than 2,400 cm2 and ultrathin selective layers (50-130 nm), including ZIFs and carboxylate-linker MOFs. Among these, the ZIF-8 membrane displays high gas permeance of 3,979 GPU for C3H6, with good mixed gas selectivity (43.88 for C3H6/C3H8). To illustrate its scale-up practicality, MOF membranes were prepared and incorporated into spiral-wound membrane modules with an active area of 4,800 cm2. The ZIF-8 membrane module presents high gas permeance (3,930 GPU for C3H6) with acceptable ideal gas selectivity (37.45 for C3H6/C3H8).

19.
Angew Chem Int Ed Engl ; : e202401118, 2024 Mar 03.
Artigo em Inglês | MEDLINE | ID: mdl-38433100

RESUMO

Inorganic zeolites have excellent molecular sieving properties, but they are difficult to process into macroscopic structures. In this work, we use metal-organic framework (MOF) glass as substrates to engineer the interface with inorganic zeolites, and then assemble the discrete crystalline zeolite powders into monolithic structures. The zeolites are well dispersed and stabilized within the MOF glass matrix, and the monolith has satisfactory mechanical stabilities for membrane applications. We demonstrate the effective separation performance of the membrane for 1,3-butadiene (C4H6) from other C4 hydrocarbons, which is a crucial and challenging separation in the chemical industry. The membrane achieves a high permeance of C4H6 (693.00±21.83 GPU) and a high selectivity over n-butene, n-butane, isobutene, and isobutane (9.72, 9.94, 10.31, and 11.94, respectively). This strategy opens up new possibilities for developing advanced membrane materials for difficult hydrocarbon separations.

20.
Adv Sci (Weinh) ; : e2309306, 2024 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-38483934

RESUMO

Peripheral nerve deficits give rise to motor and sensory impairments within the limb. The clinical restoration of extensive segmental nerve defects through autologous nerve transplantation often encounters challenges such as axonal mismatch and suboptimal functional recovery. These issues may stem from the limited regenerative capacity of proximal axons and the subsequent Wallerian degeneration of distal axons. To achieve the integration of sensory and motor functions, a spatially differential plasmid DNA (pDNA) dual-delivery nanohydrogel conduit scaffold is devised. This innovative scaffold facilitates the localized administration of the transforming growth factor ß (TGF-ß) gene in the proximal region to accelerate nerve regeneration, while simultaneously delivering nicotinamide mononucleotide adenylyltransferase 2 (NMNAT2) to the distal region to mitigate Wallerian degeneration. By promoting autonomous and selective alignment of nerve fiber gap sutures via structure design, the approach aims to achieve a harmonious unification of nerve regeneration, neuromotor function, and sensory recovery. It is anticipated that this groundbreaking technology will establish a robust platform for gene delivery in tissue engineering.

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