1.Clinical Characteristics and Prognostic Analysis of Extracranial Malignant Rhabdoid Tumor in Children
Shihan ZHANG ; Wen ZHAO ; Mei JIN ; Hongjun FAN ; Xisi WANG ; Libing FU ; Tong YU ; Yan SU
JOURNAL OF RARE DISEASES 2026;5(1):34-42
To investigate the clinical characteristics and prognosis of extracranial malignant rhabdoid tumor (eMRT) in children, and to provide a reference for the clinical treatment of this disease. A retrospective analysis was performed on the clinical data of children with newly diagnosed eMRT who were admitted and treated in the Department of Pediatric Oncology, Beijing Children's Hospital Affiliated to Capital Medical University, from March 2009 to December 2024. The clinical characteristics were summarized, and survival analysis and prognostic risk factor analysis were conducted. A total of 43 children with eMRT were included in this study, the median age at diagnosis of all patients was 20 months (range: 2-138 months). Among them, 24 cases were malignant renal rhabdoid tumors and 19 cases were extracranial, extrarenal rhabdoid tumors. Of the 43 children, 23 cases (53.5%) were complicated with distant metastasis. Twenty-nine (67.4%) underwent primary tumor resection. Among the children, 24 (55.8%) underwent gross total resection (GTR), 5 (11.6%) partial resection, and 14 (32.6%) biopsy only. Their 3-year overall survival (OS) rates were 40.8%, 35.3%, and 33.3%, respectively ( Children with eMRT have an overall poor prognosis. A diagnostic age < 12 months is an independent risk factor for higher mortality in these children. Further large-scale, long-term follow-up studies are needed to explore the prognostic factors of this disease.
2.Astragali Radix and Its Formulas in Treatment of Diabetic Cardiomyopathy: A Review
Ran CUI ; Linping ZHU ; Jingshun YAN ; Pingting ZHANG ; Chuxuan XIE ; Yansong LIU ; Mei ZHAO ; Yuhong LI
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(18):248-259
Diabetic cardiomyopathy (DCM) is a common chronic complication of diabetes, categorized as consumptive thirst in traditional Chinese medicine (TCM), and it subsequently gives rise to chest impediment. DCM is characterized by a complex pathogenesis and exhibits long-term progression with poor prognosis in clinical practice. Astragali Radix, as a medicinal material with edible value, possesses diverse active components including saponins, polysaccharides, and flavonoids. It has the effects of tonifying Qi, elevating Yang, promoting fluid production, nourishing blood, expressing toxin, and expelling pus. Modern pharmacological studies have confirmed that Astragali Radix and its active components exert therapeutic effects in various aspects such as inhibition of inflammation, alleviation of stress injury, regulation of programmed cell death, modulation of glucose and lipid metabolism, inhibition of myocardial fibrosis, and regulation of gut microbiota and metabolites, treating DCM in a multi-component, multi-target, and multi-pathway manner. The compatibility of Astragali Radix exerts both synergistic and antagonistic effects. The compound preparations and extracts of Astragali Radix are widely used in clinical practice, and the development of novel nano and gel materials and extracellular vesicles helps to improve the oral bioavailability, providing more possibilities for the clinical application of Astragali Radix in DCM, though its potential for clinical translation requires ongoing exploration. This review summarizes the TCM theoretical basis of Astragali Radix in the treatment of DCM, and integrates modern pharmacological research results to delve into the effects and mechanisms of the herb combinations, compound preparations, extracts, and new composite materials of Astragali Radix in the treatment of DCM, aiming to provide a reference for in-depth research and new drug development of Astragali Radix for DCM.
3.Mid-long term follow-up reports on head and neck rhabdomyosarcoma in children
Chao DUAN ; Sidou HE ; Shengcai WANG ; Mei JIN ; Wen ZHAO ; Xisi WANG ; Zhikai LIU ; Tong YU ; Lejian HE ; Xiaoman WANG ; Chunying CUI ; Xin NI ; Yan SU
Chinese Journal of Pediatrics 2025;63(1):62-69
Objective:To analyze the clinical characteristics of children with head and neck rhabdomyosarcoma (RMS) and to summarize the mid-long term efficacy of Beijing Children′s Hospital Rhabdomyosarcoma 2006 (BCH-RMS-2006) regimen and China Children′s Cancer Group Rhabdomyosarcoma 2016 (CCCG-RMS-2016) regimen.Methods:A retrospective cohort study. Clinical data of 137 children with newly diagnosed head and neck RMS at Beijing Children′s Hospital, Capital Medical University from March 2013 to December 2021 were collected. Clinical characteristic of patients at disease onset and the therapeutic effects of patients treated with the BCH-RMS-2006 and CCCG-RMS-2016 regimens were compared. The treatments and outcomes of patients with recurrence were also summarized. Survival analysis was performed by Kaplan-Meier method, and Log-Rank test was used for comparison of survival rates between groups.Results:Among 137 patients, there were 80 males (58.4%) and 57 females (41.6%), the age of disease onset was 59 (34, 97) months. The primary site in the orbital, non-orbital non-parameningeal, and parameningeal area were 10 (7.3%), 47 (34.3%), and 80 (58.4%), respectively. Of all patients, 32 cases (23.4%) were treated with the BCH-RMS-2006 regimen and 105 (76.6%) cases were treated with the CCCG-RMS-2016 regimen. The follow-up time for the whole patients was 46 (20, 72) months, and the 5-year progression free survival (PFS) and overall survival (OS) rates for the whole children were (60.4±4.4)% and (69.3±4.0)%, respectively. The 5-year OS rate was higher in the CCCG-RMS-2016 group than in BCH-RMS-2006 group ((73.0±4.5)% vs. (56.6±4.4)%, χ2=4.57, P=0.029). For the parameningeal group, the 5-year OS rate was higher in the CCCG-RMS-2016 group (61 cases) than in BCH-RMS-2006 group (19 cases) ((57.3±7.6)% vs. (32.7±11.8)%, χ2=4.64, P=0.031). For the group with meningeal invasion risk factors, the 5-year OS rate was higher in the CCCG-RMS-2016 group (54 cases) than in BCH-RMS-2006 group (15 cases) ((57.7±7.7)% vs. (30.0±12.3)%, χ2=4.76, P=0.029). Among the 10 cases of orbital RMS, there was no recurrence. In the non-orbital non-parameningeal RMS group (47 cases), there were 13 (27.6%) recurrences, after re-treatment, 7 cases survived. In the parameningeal RMS group (80 cases), there were 40 (50.0%) recurrences, with only 7 cases surviving after re-treatment. Conclusions:The overall prognosis for patients with orbital and non-orbital non-parameningeal RMS is good. However, children with parameningeal RMS have a high recurrence rate, and the effectiveness of re-treatment after recurrence is poor. Compared with the BCH-RMS-2006 regimen, the CCCG-RMS-2016 regimen can improve the treatment efficacy of RMS in the meningeal region.
4.The Mechanism of Echinococcus Granulosus Sensu Stricto Antigen B to Protect Immune Thrombocytopenia Mouse Model by Influen-cing Autophagy
Hai-Chen SONG ; Xue-Mei WANG ; Dan-Lu LI ; Li ZHAO ; Xue-Hua YANG ; Mei YAN
Journal of Experimental Hematology 2025;33(6):1694-1700
Objective:To investigate the mechanism of natural antigen B(nAgB)to protect Immune thrombocytopenia(ITP)mouse model by influencing autophagy.Methods:Twenty-eight female BALB/c mice aged 8-10 weeks were randomly divided into four groups.7 mice of each group were immunized intraperitoneally,the control group was treated with PBS as the control group;ITP group was treated with anti-CD41 monoclonal antibody(anti-CD41Ab)only;nAgB group was treated with nAgB intraperitoneal injection for 5d;nAgB+ITP group was treated with nAgB intraperitoneal injection for 5d,then treated with anti-CD41 Ab.The peripheral blood platelet count in each group was tested;and the spleen and liver should be isolated and weighed,the organ index was calculated;qRT-PCR was used to detect spleen microtubule-associated protein 1 light chain 3(LC3),p62,Beclin-1 mRNA expression levels.Western blot was used to detect the protein expression level of spleen LC3 Ⅱ/LC3 Ⅰ,p62,Beclin-1.Results:Compared with the control group,mice in the ITP group showed a significant decrease in blood PLT count[(102.1±17.9)× 109/L vs(485.4±185.2)×109/L,P<0.01],a significant increase in spleen index(P<0.01),mice in the nAgB group showed a significant increase in blood PLT count,rising to(1051±127.6)× 109/L on the 3 day after modeling.Compared with the ITP group,mice in the nAgB+ITP group showed a significant increase in PLT count on the 1 day of anti-CD41 Ab administration[(428.6±131.6)× 109/L vs(102.1±17.9)×109/L,P<0.05],however,the spleen index was significantly decreased(P<0.05).qRT-PCR and Western blot results showed that compared with the control group,the mRNA and protein expression levels of spleen LC3,p62 and Beclin-1 were increased in the ITP group of mice(P<0.05,P<0.01).Compared with the ITP group,the nAgB+ITP group could significantly decrease mRNA levels of spleen LC3,p62 and Beclin-1(P<0.05,P<0.01),and also significantly decrease the protein expression levels of LC3 Ⅱ/LC3 Ⅰ,p62 and Beclin-1(P<0.05,P<0.01).Conclusion:nAgB inhibits the transcription and expression levels of autophagy-related genes and regulates immune intolerance,thereby protecting ITP mouse models.
5.Analysis of VWF Gene c.7332G>A Nonsense Mutation Pedigree and Study of Molecular Pathogenesis
Duan-Yang WANG ; Lei WANG ; Dong-Yan FU ; Xiao-Mei LU ; Li-Dong ZHAO ; Jia-Wei ZHENG ; Ya-Lin YU ; Gang WANG ; Lin-Hua YANG
Journal of Experimental Hematology 2025;33(6):1701-1707
Objective:To analyze the genetic characteristics of the VWF gene c.7332G>A nonsense mutation and explore its molecular pathogenesis.Methods:Phenotypic diagnosis of the proband was performed using VWF:Ag,VWF:RCo,FⅧ:C and multimeric analysis.The probands were genotyped by NGS whole-exome sequencing,and the sequencing results were validated by sanger sequencing.The family members were genotyped by Sanger sequencing.The VWF gene c.7332G>A nonsense mutant plasmid was constructed.After transfection,the function of VWF gene c.7332G>A mutant plasmid was verified at cell level in vitro.The mRNA level was detected by qRT-PCR,and the expression level of protein was detected by Western blot,the function of multimerization was verified by the multimeric analysis.Results:VWF:Ag and VWF:RCo were all less than 3%in the proband,and the multimeric analysis showed multimer deficiency.The proband was diagnosed as type 3 VWD.The homozygous nonsense mutation of VWF gene c.7332G>A was detected by gene sequencing.The VWF mRNA level of the mutant plasmid was decreased,and the VWF protein expression in the cell supernatant was decreased,the mutant protein was truncated and the function of VWF multimerization was impaired.Conclusion:A homozygous mutation in exon 43 of VWF gene,c.7332G>A,was responsible for the probands type 3 VWD in the proband.The mutation caused a decrease in the relative level of VWF mRNA and protein,and impaired the function of VWF multimerization.
6.Cost-effectiveness and mortality risk impact on elderly health management of essential public health services:A case study in Henan Province
Zhi-ping GUO ; Rong-mei LIU ; Neng-guang DAI ; Yi LI ; Tong JIN ; Qiu-ping ZHAO ; Hao SHI ; Chun-rong BAO ; Yan-qing MIAO
Chinese Journal of Health Policy 2025;18(11):17-24
Objective:To evaluate the cost-effectiveness and impact on mortality of health management services for the elderly aged 65 years and older in national essential public health service project.Methods:Based on the data of county-level medical institutions in Henan Province from 2019 to 2024,the Random Forest Method was used to construct a counterfactual framework to predict the hospitalization expenses under the unmanaged scenario,and then the cost-benefit ratio(BCR)and net income were calculated.Time-dependent Cox proportional hazards model was used to evaluate the effect of health management on all-cause mortality and cardiovascular and cerebrovascular disease mortality in the elderly.Results:A total of 962 955 elderly patients were included,451 119(46.85%)were included in the management group.The average hospitalization cost of the management group was significantly lower than that of the non-management group(P<0.05).Except for 2020-2021,BCRS in 2019 and 2022-2024 were 6.34,2.05,4.45 and 6.60,respectively.The risk of all-cause death was reduced by 76.96%,and the risk of cardiovascular and cerebrovascular death was reduced by 75.57%in the elderly patients included in the management group compared with those not included in the management group.Suggestions:It is necessary to establish a health outcomes-based evaluation system and promote the transformation and upgrading of the service model from single chronic disease management to"integrated health services with multi-disease management".
7.Analysis of Drug Resistance Risk Factors and Construction and Validation of a Stratified Prediction Model in Elderly Patients with Pulmonary Tuberculosis
Rui KANG ; Gai-yan REN ; Yang-yang YAO ; Yan-mei ZHAO ; Fen-fen WANG
Progress in Modern Biomedicine 2025;25(16):2622-2629
Objective:To analyze the drug resistance status and related risk factors in elderly patients with pulmonary tuberculosis,and to construct and validate a drug resistance risk prediction model for elderly pulmonary tuberculosis patients,providing evidence for early clinical identification of high-risk populations.Methods:A retrospective study was conducted,consecutively enrolling 315 pulmonary tuberculosis patients aged ≥60 years from January 2019 to December 2021.Based on drug susceptibility test results,patients were divided into drug-resistant group(85 cases)and drug-sensitive group(230 cases).Data on demographic characteristics,clinical manifestations,comorbidities,imaging features,and previous treatment history were collected.Univariate and multivariate logistic regression analyses were performed to identify drug resistance-related factors,and a prediction model was constructed accordingly.The model was internally validated using the concordance index(C-index)and Bootstrap method.Results:Among 315 elderly pulmonary tuberculosis patients,the drug resistance rate was 26.98%,predominantly mono-resistance(48.24%)and multidrug-resistance(28.24%).Multivariate logistic regression analysis showed that previous anti-tuberculosis treatment history[OR=4.537,95%CI(1.962-10.498),P=0.005],diabetes mellitus[OR=3.286,95%CI(1.547-6.979),P=0.002],pulmonary cavitation[OR=4.158,95%CI(1.926-8.973),P<0.001],pleural effusion[OR=2.563,95%CI(1.218-5.390),P=0.013],and tracheobronchial tuberculosis[OR=2.819,95%CI(1.352-5.876),P=0.006]were independent risk factors for drug resistance in elderly pulmonary tuberculosis.The prediction model constructed based on these factors had a C-index of 0.815(95%CI:0.762-0.868).Conclusion:The drug resistance rate is high among elderly pulmonary tuberculosis patients.Previous anti-tuberculosis treatment history,diabetes mellitus,pulmonary cavitation,pleural effusion,and tracheobronchial tuberculosis are independent risk factors for drug resistance in elderly pulmonary tuberculosis.The prediction model constructed based on these factors has good predictive value for identifying high-risk populations,which is helpful for early clinical intervention and individualized treatment strategy formulation.
8.Dioscin inhibits IL-17+γδT cells to exert an anti-rheumatoid arthritis effect
Lin-mei PU ; Hao-hong ZHANG ; Chao-yu CHU ; Yuan-yuan NI ; Zhao WU ; Qing-yan MO ; Hong-yun WANG ; Ying XU ; Chun-ping WAN
Chinese Pharmacological Bulletin 2025;41(11):2082-2088
Aim To explore the mechanism by which dioscin regulates IL-17+γδT cells in the treatment of arthritis.Methods A collagen-induced arthritis(CIA)model was established in DBA/1 mice using bovine type Ⅱ collagen.The mice were randomly divid-ed into the CIA model group,methotrexate(MTX)positive control group,and dioscin low-dose(Dioscin-L),medium-dose(Dioscin-M),and high-dose(Dios-cin-H)groups.After intervention,the therapeutic effects were evaluated using scoring methods.Joint pathological damage was analyzed by hematoxylin and eosin(HE)staining.The levels of anti-collagen-spe-cific antibodies and the pro-inflammatory cytokine IL-17 were measured by ELISA.The expressions of γδT cells and their subtypes,as well as the secretion level of IL-17,were detected by flow cytometry.Results Dioscin significantly reduced the arthritis severity score in collagen-induced arthritis(CIA)mice,alleviated joint pathological damage,inhibited the production of IL-17 by splenic lymphocytes and the levels of anti-col-lagen-specific antibodies total IgG and IgG3,and de-creased the proportion of γδT cells in the lymph nodes,splenic γδT cells,and the Vδ4+T-cell subset.The level of IL-17 produced by the Vδ4 subtype in the lymph nodes of the intervention groups was lower than that in the model group,but the difference was not sta-tistically significant.Conclusion Dioscin has signifi-cant therapeutic effect on CIA,and its mechanism may be through the inhibition of γδT cells,but it is unlikely to be related to IL-17 derived from γδT cells.
9.The therapeutic effects of newly formulated Tadalafil tablets on rats with pathological cardiac hypertrophy through regulation of NF-κB signaling pathway
Xue-di ZHANG ; Ye-ding SONG ; Li-mei LI ; Hao-yan CHEN ; Hua-sui CUI ; Zheng-gang ZHAO ; Zi-jian ZHAO ; Yun-ping MU ; Fang-hong LI
Chinese Pharmacological Bulletin 2025;41(8):1485-1492
Aim To investigate the therapeutic effects of a newly developed Tadalafil tablets on pathological myocardial hypertrophy induced by abdominal aortic constriction(AAC)in rats,as well as its influence on the activation of the NF-κB signaling pathway in myo-cardial cells.Methods SD rats were randomly divid-ed into 4 groups:the sham operation group(Sham),the model group(AAC),the tadalafil new tablet treat-ment group(N-Tad,5 mg·kg-1),and the positive control drug treatment group(Cialis,10 mg·kg-1g).The AAC model group and treatment group rats under-went blunt dissection and constrictive ligation of the abdominal aorta at the left renal artery branch point during surgery,while the Sham group rats only had their arteries separated without any constrictive liga-tion.Rats in the treatment groups received either N-Tad or Cialis via gavage three days after modeling,while rats in the sham group and the model group re-ceived physiological saline daily for 8 weeks.Small an-imal ultra-high-resolution echocardiography and hemo-dynamic assessment were applied to evaluate left ven-tricular function in each group of rats,and the calcula-tion of the left ventricular mass index was conducted.By employing Western blot and RT-PCR.we assessed the impact of this treatment on the expression of the hy-pertrophy factor atrial natriuretic peptide(ANP),phosphorylated NF-κB p65 protein(p-NF-κB p65),and phosphorylated IκB-α in the left heart tissue of rats and in H9c2 cardiomyocytes.Results Compared to the Sham group,the AAC rats exhibited a significant decrease in left heart function,an increase in left ven-tricular mass index,and a notable increase in ANP and p-p65 expression in the left heart tissue(P<0.05).Both N-Tad and Cialis treatments could significantly enhance left ventricular function,decrease left ventric-ular mass index,and inhibit the expression of ANP and phosphorylated NF-κB p65 in rats with myocardial hy-pertrophy(P<0.05).Notably,the therapeutic effect of low-dose N-Tad was comparable to that of high-dose Cialis.At the cellular level,Tadalafil significantly in-hibited the activation of the NF-κB signaling pathway and reduced the expression of associated proteins in H9c2 cardiomyocytes.Conclusions N-Tad can sig-nificantly inhibit p65 and IκB-α phosphorylation,and the activation of the NF-κB signaling pathway,reduce ANP expression,and improve pathological myocardial hypertrophy,as well as mitigate left heart function damage caused by abdominal aortic constriction.
10.Analysis on Screening Results of Breast Cancer Among Women Aged 45~74 in Chongqing from 2012 to 2022
Jia DU ; Zhikai YU ; Shenglin ZHAO ; Yan ZHANG ; Qing GUO ; Xiu LIU ; Hong ZHOU ; Mei HE
China Cancer 2025;34(1):43-51
[Purpose]To analyze the results of breast cancer screening among women aged 45~74 in Chongqing from 2012 to 2022 and compare the risk of breast cancer among women with differ-ent characteristics.[Methods]The Urban Cancer Early Diagnosis and Treatment Program was con-ducted in Chongqing from 2012 to 2022,female residents aged 45~74 years old participated in breast cancer screening.The participants who were assessed as at high risk of breast cancer in the preliminary screening were advised to receive ultrasonography and mammography examination in designated hospitals.The study population was followed up annually to obtain information on their health outcomes.The high risk rate of breast cancer,compliance of further examination,the inci-dence density and breast cancer risk were calculated.[Results]A total of 207 891 women com-pleted the questionnaires survey and risk assessment,and 35 947 were assessed as the individuals with high risk of breast cancer with a high risk rate of 17.29%.Among them 14 713 received the imaging screening with a compliance rate of 40.93%.After a mean follow-up of(5.43±3.01)years,847 cases of breast cancer were diagnosed with a cumulative incidence rate of 407.43/105,an in-cidence density of 75.03/105 person-years(95%CI:70.15/105~80.26/105 person-years).Cox pro-portional hazards regression showed that the risk of breast cancer was higher in obese women than that in normal weight women(HR=1.26,95%CI:1.00~1.59);the risk of breast cancer was signifi-cantly higher in those with family history of breast cancer than that in those without family history(HR=1.69,95%CI:1.36~2.09).Compared with non-high-risk groups of breast cancer,the risk of breast cancer was increased in high-risk individuals who were screened or not screened(HR=1.36,95%CI:1.06~1.73 and HR=1.26,95%CI:1.01~1.56).[Conclusion]The breast cancer screening program combining primary risk assessment with ultrasonography and mammography for the high-risk groups can improve the detection rate,and the compliance rate of imaging screening need to be improved in the future and make accurate screening for people with high risk of breast cancer.

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