VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset, X-linked clonal autoinflammatory disease caused by somatic mutations in the UBA1 gene, characterized by systemic inflammation accompanied by hematologic clonal abnormalities. Somatic UBA1 mutations result in impaired ubiquitination, disruption of protein homeostasis, and sustained activation of inflammatory signaling pathways, including nuclear factor-κB and Janus kinase-signal transducer and activator of transcription (JAK-STAT), which together constitute the core pathogenic mechanism. The disease involves multiple systems and tissues including the hematologic system, skin, cartilage and respiratory system, showing remarkable clinical heterogeneity. Diagnosis depends primarily on the combination of characteristic clinical manifestations and molecular confirmation of UBA1 mutations. Current therapeutic strategies lack a unified standard: glucocorticoids can rapidly control inflammation but are associated with high relapse rates, whereas targeted therapies such as JAK inhibitors, interleukin inhibitors, and hypomethylating agents show variable efficacy, and allogeneic hematopoietic stem cell transplantation offers curative potential in patients with concomitant myelodysplastic syndrome. This review systematically summarizes the genetic background, molecular mechanisms, clinical spectrum, diagnostic criteria, and therapeutic advances of VEXAS syndrome.

ObjectiveTo investigate the correlation between sleep patterns and myopia progression among younger school-age children at a primary school in Changning District of Shanghai, based on the data from the Shanghai Students’ Common Diseases and Health Influencing Factors Monitoring System and a sleep-specific survey, so as to provide data support for myopia prevention and control in this age group. MethodsOne primary school was selected from the common diseases and health influencing factors monitoring system for students in Changning District, Shanghai. A total of 230 first-grade students were included in the study. Myopia and refractive parameters were examined, and sleep patterns were investigated. General demographic characteristics and myopia-related behavior data of the students were also collected. Sleep patterns were evaluated in terms of sleep duration, sleep efficiency, and sleep quality, with the latter assessed using the Chinese version of the Children’s Sleep Habits Questionnaire (CSHQ). Multiple linear regression and binary logistic regression models were used to analyze the association between sleep patterns and myopia progression among these students. ResultsThe results of the regression analyses revealed that the total CSHQ score of the students at baseline survey was (48.85±7.15) points. Their sleep efficiency was (94.49±8.48)%, sleep duration was (9.58±0.93) hours, and the proportion of those with insufficient sleep (<10 hours) was 78.26%. At baseline survey, students’ higher daytime sleepiness scores were associated with lower spherical equivalent (SE) ( β=-0.18, 95%CI: -0.31 to -0.04) and an increased risk of axial length (AL) / corneal radius (CR) ratio >3 (OR=1.52, 95%CI: 1.00 to 2.29), whereas longer sleep duration and higher sleep efficiency were associated with higher SE (β=0.18, 95%CI: 0.05 to 0.32; β=0.17, 95%CI: 0.04 to 0.31, respectively), shorter (AL) (β=-0.15, 95%CI: -0.27 to -0.03; β=-0.13, 95%CI: -0.25 to 0, respectively) and a reduced risk of AL /CR>3 (OR=0.70, 95%CI: 0.51 to 0.96; OR=0.73, 95%CI: 0.53 to 0.99, respectively). At baseline survey, children’s higher propensity for sleep problems (OR=1.70, 95%CI: 1.04 to 2.78), sleep resistance (OR=2.26, 95%CI: 1.36 to 3.75), and sleep anxiety scores (OR=2.15, 95%CI: 1.33 to 3.48) were all associated with an increased risk of AL/CR >3 at follow-up (all P<0.05). Furthermore, higher sleep anxiety scores predicted prolonged AL at follow-up (β=0.03, 95%CI: 0 to 0.05). According to the mixed-effects model, higher daytime sleepiness scores and prolonged sleep duration were independently linked to reduced right-eye SE (β=-0.05, 95%CI: -0.10 to 0, P<0.05) and shorter right-eye AL (β=-0.05, 95%CI: -0.10 to 0, P<0.05). ConclusionIn this school in Shanghai, there are problems of insufficient and poor-quality sleep among young children. Sleep problems such as sleep resistance, delayed sleep onset, sleep anxiety, and daytime sleepiness among children may accelerate the risk of myopia progression, while longer sleep duration and higher sleep efficiency may serve as protective factors against the occurrence and development of myopia.

Objective To investigate the clinical prognostic value of the prognostic nutritional index (PNI) in patients with renal cell carcinoma (RCC) undergoing partial nephrectomy.Methods A retrospective analysis was conducted on the clinical data of RCC patients who underwent partial nephrectomy at our hospital during 2020 and 2024.The optimal cutoff value of PNI was determined based on the maximum Youden index from the receiver operating characteristic (ROC) curve.The clinicopathological characteristics, overall survival (OS), and progression-free survival (PFS) between patients with different PNI levels were compared.Cox regression analysis was used to explore the independent prognostic factors for RCC patients. Results Based on the ROC curve, the optimal cutoff value corresponding to the maximum Youden index was calculated to be 46.675, at which point the sensitivity was 65.3% and the specificity was 62.2%.Of the 815 patients included in the study, 293 (36.0%) patients had a PNI＜46.675.Cox regression analysis confirmed that preoperative PNI, tumor diameter, ISUP classification, and ECOG score were independent risk factors for postoperative OS in patients with RCC who underwent PN (P＜0.05).In addition, preoperative PNI, tumor diameter, ISUP classification and ECOG score were independent risk factors for postoperative PFS (P＜0.05).After covariates in the multivariable Cox regression model were adjusted, patients in the low PNI group demonstrated significantly shorter overall survival (HR=2.392, P＜0.001) and significantly poorer progression-free survival (HR=2.076, P＜0.001) compared to those in the high PNI group.Conclusion Preoperative PNI has significant clinical value in evaluating the prognosis of RCC patients undergoing partial nephrectomy.An elevated preoperative PNI is associated with a better prognosis.

Objective:To develop a prediction model for the dosage of antivenom in patients envenomed by Trimeresurus stejnegeri (bamboo pit viper) using Lasso-Logistic regression.Methods:A retrospective cohort study was conducted including 165 patients with Trimeresurus stejnegeri bites admitted to the Emergency General Ward of Yangjiang Hospital Affiliated to Guangdong Medical University (Yangjiang People's Hospital) between October 31, 2021, and November 1, 2024. Data on demographic characteristics, key clinical signs, laboratory parameters, antivenom treatment regimen, and total antivenom dosage were collected. Predictive variables were selected using LASSO and Logistic regression methods. The prediction model was constructed using R software. Model calibration was assessed using the Hosmer-Lemeshow test, discriminative ability via the receiver operating characteristic (ROC) curve, and clinical utility using decision curve analysis.Results:Among the 165 enrolled patients, 71 received a total antivenom dose of ≤3 vials, and 94 received >3 vials. Six predictors for requiring >3 vials of antivenom were identified: pain severity, swelling degree, serum treatment regimen, 3P-test result, white blood cell count (WBC), and fibrinogen level (FIB). After comparing 22 prediction models incorporating 4 to 6 variables, the model comprising swelling degree, pain severity, WBC, and FIB was selected. The calibration curve indicated good agreement between predicted and actual risks. The area under the ROC curve (AUC) was 0.78 (95% CI: 0.71–0.85), demonstrating satisfactory discriminative ability for predicting whether the total antivenom dose exceeded 3 vials. Conclusions:The risk prediction model shows good performance in identifying whether the total antivenom dose exceeds 3 vials in Trimeresurus stejnegeri bite patients. It incorporates six readily available clinical variables: pain severity, swelling degree, serum regimen, 3P-test, WBC, and FIB. A model based on pain severity, swelling degree, WBC, and FIB can assist clinicians in predicting whether a patient will require more than 3 vials of antivenom after standard treatment.

ObjectiveTo investigate the distribution of traditional Chinese medicine （TCM） syndromes in intrahepatic cholestasis of pregnancy （ICP） and its association with perinatal outcomes， and to provide a basis for precise treatment based on TCM syndrome differentiation. MethodsA cross-sectional study was conducted among 275 patients with ICP who were admitted to The Affiliated Hospital of Chengdu University of Traditional Chinese Medicine from April 2023 to April 2025. A hierarchical cluster analysis was used to summarize TCM syndromes. The Kruskal-Wallis H test was used for comparison of non-normally distributed continuous data between groups， and the chi-square test was used for comparison of categorical data between groups. A multivariate Logistic regression analysis was used to identify the clinical features significantly associated with TCM syndrome. ResultsThe cluster analysis identified three core TCM syndromes among the 275 patients with ICP， i.e.， liver-gallbladder damp-heat syndrome （45.8%）， syndrome of blood deficiency generating wind （30.9%）， and liver depression and spleen deficiency syndrome （23.3%）. There was a significant difference in the distribution of TCM syndromes between different groups stratified by maternal age at delivery， parity， history of ICP recurrence， gestational weeks at disease onset， total bile acid （TBA）， alanine aminotransferase （ALT）， and comorbidity with gestational diabetes mellitus （GDM） （all P<0.05）. The multivariate Logistic regression analysis showed that<34 gestational weeks at disease onset was significantly associated with all three syndromes （damp-heat： odds ratio ［OR］=3.769， P<0.001； blood deficiency： OR=4.031， P<0.001； liver stagnation： OR=3.552， P<0.001）. Liver-gallbladder damp-heat syndrome was associated with maternal age ≥35 years at disease onset （OR=2.048， P=0.014）， parity ≥2 times （OR=1.921， P=0.034）， history of ICP recurrence （OR=2.404， P=0.030）， ALT ≥200 U/L （OR=2.051， P=0.018）， comorbidity with GDM （OR=1.944， P=0.029）， and TBA ≥40 μmol/L （OR=2.542， P=0.024）. The syndrome of blood deficiency generating wind syndrome was associated with maternal age ≥35 years （OR=2.939， P=0.003）， parity ≥2 time （OR=3.222， P=0.003）， history of ICP recurrence （OR=3.809， P=0.010）， ALT ≥200 U/L （OR=2.889， P=0.006）， comorbidity with GDM （OR=3.711， P=0.001）， and comorbidity with hypertensive disorders of pregnancy （OR=4.472， P=0.011）. Liver depression and spleen deficiency syndrome was associated with TBA ≥40 μmol/L （OR=2.995， P=0.044）. The analysis of perinatal outcomes showed that there were significant differences in mode of delivery， gestational weeks at the time of delivery， postpartum blood loss， and neonatal birth weight between the three groups with different TCM syndromes （all P<0.05）. ConclusionLiver-gallbladder damp-heat syndrome， syndrome of blood deficiency generating wind， and liver depression and spleen deficiency syndrome are the main TCM syndrome types in ICP， and the distribution of TCM syndromes is closely associated with clinical factors and perinatal outcomes， which provides a basis for precise TCM syndrome differentiation and individualized treatment.

Necrotizing enterocolitis（NEC）is a gastrointestinal emergency commonly seen in premature infants，and its etiology and high-risk factors have not been fully elucidated.Premature infants who receive blood component transfusions are at significantly increased risk of developing NEC，with a higher incidence and mortality rate.This review focuses on a comprehensive analysis of the association between multiple blood component transfusions and NEC，the pathogenesis，prevention measures，and the threshold of blood component transfusions，aiming to provide a reference for the safe and rational use of blood component transfusions in clinical practice，and to guide fulture research directions.

Objective To evaluate the intravascular volume at different levels of edema and disease course by the fractional excretion of filtered sodium(FeNa)of children with primary nephrotic syndrome(PNS).Methods A total of 172 children with newly diagnosed PNS who were hospitalized in the Affiliated Hospital of Xuzhou Medical University from September 2022 to September 2024 were selected and divided into non-e-dema group(n=51),mild edema group(n=43),moderate edema group(n=46)and severe edema group(n=32)according to the degree of edema at the time of admission.A total of 40 healthy children who underwent physical examination during the same period were selected as the healthy control group.Serum creatinine,ser-um sodium were detected before and after treatment.Urine samples were collected to detect urine creatinine,urine sodium,FeNa was calculated and compared according to the results,and the degree of edema was recor-ded.24 h urine samples were collected on the same day to detect 24 h urine protein quantification and 24 h u-rine volume.Results On day 1 to 2 of the course of the disease,about 12%of the PNS children had FeNa<0.2%,indicating insufficient intravascular volume,which was mainly concentrated in the severe edema group.The moderate,severe edema group had a significantly lower FeNa level than the non-edema group,mild edema group,and healthy control group(P<0.01).The moderate edema group had a significant increase in FeNa on days 6 to 7 of the course of the disease,and the severe edema group had a significant increase in Fena on days 11 to 12 of the course of the disease(P<0.01).Conclusion Intravascular volume of PNS children with mod-erate to severe edema is often reduced,and intravascular volume may be insufficient in severe edema.PNS chil-dren with moderate to severe edema have increased intravascular volume with the extension of the course of disease and the improvement of the condition.

Objective::To systematically study the mechanisms by which Yinzhihuang (YZH), a traditional Chinese medicine, ameliorates intrahepatic cholestasis of pregnancy (ICP), a liver disorder associated with significant maternal and fetal complications.Methods::This experimental study was conducted from January 2024 to August 2024, utilizing data from public databases (Traditional Chinese Medicine Systems Pharmacology, GeneCards, Online Mendelian Inheritance in Man, DisGeNET, Proteome Xchange) alongside in vitro cell culture experiments. Network pharmacology identified active components of YZH and potential therapeutic targets for ICP. Ultra-performance liquid chromatography–mass spectrometry characterized YZH oral liquid, and its effective doses were evaluated in taurocholic acid (TCA)-induced HTR-8/SVneo cells, an in vitro ICP model. ICP-related targets were gathered from multiple databases, and hub genes were selected through bioinformatics and previously identified differentially expressed proteins. Functional annotation and pathway enrichment analyses were conducted, with validation in TCA-induced cells treated with various YZH concentrations (0.1%–5.0%) compared to controls. Molecular docking confirmed predicted interactions.Results::Using network pharmacology, 104 active compounds and 241 potential targets of YZH were identified. Integration of multiple databases yielded 1897 YZH-related therapeutic targets and 3783 ICP-associated genes. Proteomic analysis identified 227 differentially expressed proteins, from which 10 hub genes were selected; among these, APOA2, COL1A1, and ADIPOQ were significantly upregulated in ICP samples. UPLC-ESI-MS/MS detected 2022 compounds, predominantly flavonoids (25.07%, 507/2022) and phenolic acids (14.44%, 292/2022). Molecular docking demonstrated strong binding affinities between several active compounds and the hub genes. In TCA-induced HTR-8/SVneo cells, 0.5% YZH treatment significantly enhanced cell viability and modulated hub gene expression, supporting a potential multi-target mechanism.Conclusion::This study systematically explored the active components and potential targets of YZH in ICP through network pharmacology, proteomics, and in vitro validation. The findings suggest that YZH may act via the PPAR signaling pathway by modulating genes such as PPARA, PPARG, ADIPOQ, and APOA2.

Aim To investigate the effect of triptolide(TP)on corticosterone(CORT)-induced depression-like behaviors in mice and explore the antidepressant mechanism of TP based on the CREB/BDNF/TrkB sig-naling pathway.Methods Sixty 8-week-old male C57BL/6J mice were randomly divided into five groups:control group,CORT group,TP groups of low and high doses(10,30 μg·kg-1),and fluoxetine(FLU)group(10 mg·kg-1).Except for the control group,the other groups received subcutaneous injec-tions of CORT for three consecutive weeks to establish the model of depression.During the last two weeks of modeling,normal saline,TP and FLU were adminis-tered via intraperitoneal injection respectively.After the administration,depression-like behaviors in mice were assessed using forced swimming test,tail suspen-sion test,and sucrose preference test.Biochemical methods were used to measure the levels of SOD and MDA in the hippocampus and prefrontal cortex(PFC).Cell apoptosis was detected by TUNEL meth-od.Immunohistochemistry,immunofluorescence,and Western blotting were employed to detect the expres-sion of apoptosis/autophagy-related proteins,synaptic structure markers,and proteins related to the CREB/BDNF/TrkB signaling pathway.Results TP signifi-cantly ameliorated CORT-induced depression-like be-haviors in mice,mainly manifested by reduced immo-bility time in the tail suspension test and forced swim-ming test,and increased sucrose preference rate.TP alleviated CORT-induced oxidative stress by increasing SOD levels and reducing MDA production in brain tis-sue.Additionally,TP also inhibited apoptosis and ex-cessive autophagy of neurons in the hippocampus and prefrontal cortex,maintained synaptic plasticity,and significantly upregulated the expression of p-CREB,BDNF,and TrkB.Conclusions TP exhibits potential antidepressant effect in mice by upregulating the CREB/BDNF/TrkB signaling pathway,reducing oxida-tive stress,inhibiting excessive neuronal apoptosis and autophagy,and improving synaptic plasticity.

OBJECTIVE: To evaluate the anti-hepatocellular carcinoma (HCC) activity of total alkaloids from Gelsemium elegans Benth. (TAG) in vivo and in vitro and to elucidate their potential mechanisms of action through transcriptomic analysis. METHODS: TAG extraction was conducted, and the primary components were quantified using high-performance liquid chromatography (HPLC). The effects of TAG (100, 150, and 200 µg/mL) on various tumor cells, including SMMC-7721, HepG2, H22, CAL27, MCF7, HT29, and HCT116, were assessed. Effects of TAG on HCC proliferation and apoptosis were detected by colony formation assays and cell stainings. Caspase-3, Bcl-2, and Bax protein levels were detected by Western blotting. In vivo, a tumor xenograft model was developed using H22 cells. Totally 40 Kunming mice were randomly assigned to model, cyclophosphamide (20 mg/kg), TAG low-dose (TAG-L, 0.5 mg/kg), and TAG high-dose (TAG-H, 1 mg/kg) groups, with 10 mice in each group. Tumor volume, body weight, and tumor weight were recorded and compared during 14-day treatment. Immune organ index were calculated. Tissue changes were oberseved by hematoxylin and eosin staining and immunohistochemistry. Additionally, transcriptomic and metabolomic analyses, as well as quatitative real-time polymerase chain reaction (RT-qPCR), were performed to detect mRNA and metabolite expressions. RESULTS: HPLC successfully identified the components of TAG extraction. Live cell imaging and analysis, along with cell viability assays, demonstrated that TAG inhibited the proliferation of SMMC-7721, HepG2, H22, CAL27, MCF7, HT29, and HCT116 cells. Colony formation assays, Hoechst 33258 staining, Rhodamine 123 staining, and Western blotting revealed that TAG not only inhibited HCC proliferation but also promoted apoptosis (P<0.05). In vivo experiments showed that TAG inhibited the growth of solid tumors in HCC in mice (P<0.05). Transcriptomic analysis and RT-qPCR indicated that the inhibition of HCC by TAG was associated with the regulation of the key gene CXCL13. CONCLUSION TAG inhibits HCC both in vivo and in vitro, with its inhibitory effect linked to the regulation of the key gene CXCL13.
Animals ; Apoptosis/drug effects* ; Liver Neoplasms/genetics* ; Carcinoma, Hepatocellular/genetics* ; Humans ; Alkaloids/therapeutic use* ; Gelsemium/chemistry* ; Cell Line, Tumor ; Cell Proliferation/drug effects* ; Mice ; Xenograft Model Antitumor Assays