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.Establishment and evaluation of a lipopolysaccharide-induced acute respiratory distress syndrome model in minipigs
Chuang-Ye WANG ; Ran WANG ; Jian ZHANG ; Ling-Xiao QIU ; Bin QING ; Heng YOU ; Jin-Cheng LIU ; Bin WANG ; Nan-Bo WANG ; Jia-Yu LI ; Xing LIU ; Shuang WANG ; Jin HU ; Jian WEN ; Quan LI ; Xiao-Ou HUANG ; Kun ZHAO ; Shuang-Lin LIU ; Gang LIU ; Mei-Ju WANG ; Qing XIANG ; Hong-Mei WU ; Xiao-Rong SUN ; Tao GU ; Dong ZHANG ; Qi LI ; Zhi XU
Medical Journal of Chinese People's Liberation Army 2025;50(9):1154-1161
Objective To establish a stable,reliable,and clinically relevant porcine model of endotoxin-induced acute respiratory distress syndrome(ARDS).Methods Ten 8-month-old male Bama minipigs were deeply sedated,followed by invasive mechanical ventilation and electrocardiographic monitoring.Lipopolysaccharide(LPS)was intravenously pumped at 600 μg/(kg·h)for 3 hours,then maintained at 15 μg/(kg·h)thereafter.Dynamic monitoring was performed at five time points after LPS injection(LPS 0,1,3,5,and 8 h),including arterial blood gas analysis and chest computed tomography(CT)scans.Pathological examination of lung tissues obtained via bronchoscopic biopsy(HE staining and transmission electron microscopy)was conducted.These indicators were comprehensively used to evaluate the success of the animal model.Results At 5 hours after LPS administration,8 minipigs developed symptoms such as skin cyanosis,elevated body temperature,and respiratory distress.The oxygenation index decreased to<300 mmHg.Chest CT scans showed diffuse pulmonary infiltrates.Histopathology revealed alveolar edema and hyaline membrane formation.Transmission electron microscopy demonstrated disruption of pulmonary blood-air barrier,depletion of lamellar bodies in type Ⅱ pneumocytes,inflammatory cell infiltration,and exudation of plasma proteins and fibrin.Compared with LPS 0 h,at LPS 8 h,the oxygenation index and arterial blood pH were significantly decreased(P<0.001),while blood lactic acid and serum potassium were significantly increased(P<0.05);serum calcium and base excess were significantly decreased(P<0.05),and the lung injury score based on HE-stained lung sections was significantly increased(P<0.01).Conclusion The porcine ARDS model established by continuous LPS injection can dynamically simulate the pathophysiological characteristics and typical pathological manifestations of clinical septic ARDS,making it an effective tool to study the pathogenesis,prevention,and treatment strategies of septic ARDS.
3.AAV vector-mediated Sall2 overexpression slowing disease progression in amyotrophic lateral sclerosis transgenic mice
Xue ZHANG ; Chen-Chen WANG ; Xue-Shuai GAO ; Xue BAI ; Xue-Mei WANG ; Jin-Meng LIU ; Ying-Jun GUAN ; Yan-Chun CHEN
Acta Anatomica Sinica 2025;56(2):127-135
Objective To investigate the effect of sal-like gene 2(Sall2)gene overexpression on the progression of disease in human superoxide dismutase 1(hSOD1)-G93A mutant amyotrophic lateral sclerosis(ALS)transgenic mice,with the aim of identifying potential therapeutic targets for ALS gene therapy.Methods Differential Sall2 gene were screened through bioinformatics analysis.Forty-eight ALS transgenic mice were selected for this study.AAV-PHP.eB-Sall2 adeno-associated virus with a neuron-specific promoter,human synapsin I(hSyn),was constructed and administered via tail vein injection to six-week-old mice.In parallel,the same litter of ALS mice received an injection of AAV-PHP.eB-GFP.The staining of Sall2 and neuron-specific nuclear protein(NeuN)/GFAP in the spinal cord and cerebral cortex of mice were detected through immunofluorescent double-label staining technology.The survival period,weight changes,exercise ability,and electromyographic changes of the gastrocnemius muscle were detected.The morphological changes in the spinal cord anterior horn neurons were detected through Nissl staining.The effect of Sall2 gene overexpression on the expression of the cell cycle protein E1(cyclin E1)was investigated through Western blotting.Results Bioinformatics analysis showed out that Sall2 was differentially expressed in ALS mice.Compared with ALS mice in the control group,the Sall2 protein expression of ALS mice in the overexpressing Sall2 gene group increased in both the spinal cord and cerebral cortex,and the Sall2 integral absorbance values of Sall2+/NeuN+double-positive cells were higher.The survival time of ALS mice in the Sall2 gene overexpressing group was prolonged,the rate of weight loss was slowed down,the performance in the rotarod and inverted grid tests was improved with longer times,and the positive sharp waves and fibrillation potentials in the gastrocnemius electromyography were reduced.The number of Nissl bodies labeled neurons increased in the spinal cord anterior horn of the Sall2 gene overexpressing mice,and the condition of neuronal damage was improved.Overexpression of the Sall2 gene also reduced the expression of cyclin E1 in both the spinal cord and cerebral cortex of ALS transgenic mice.Conclusion Overexpression of the Sall2 gene can delay disease progression and improve motor performance in ALS transgenic mice,affecting the expression of cyclin E1,thus exerting a therapeutic effect on these mice.
4.Role of SLIT-ROBO Rho GTPase-activating protein 2 in motor neuron degeneration in amyotrophic lateral sclerosis
Chen-Chen WANG ; Xue ZHANG ; Xue-Shuai GAO ; Xue BAI ; Qiu-Peng YAN ; Xue-Mei WANG ; Jin-Meng LIU ; Yan-Chun CHEN
Acta Anatomica Sinica 2025;56(4):413-420
Objective To explore the role of SLIT-ROBO Rho GTPase-activating protein 2(srGAP2)in spinal motor neuron degeneration in amyotrophic lateral sclerosis(ALS).Methods Applied bioinformatics analysis to investigate the expression changes of srGAP2 in the spinal cord of human superoxide dismutase 1(hSOD1)mutant ALS transgenic mice.hSOD1 G93A mutant ALS transgenic mice were selected for animal experimental validation,with littermate wild type(WT)mice serving as the control group.A total of 36 pairs were divided into four groups,namely the pre-onset stage,early-onset stage,mid-onset stage,and late-onset stage.The expression changes and cellular localization of srGAP2 in the spinal cord of ALS mice were detected by Real-time PCR,Western blotting and immunofluorescent double-label staining.The hSOD1G93A mutant NSC34 motor neuron-like cell model was established,and in vitro experiments were carried out to detect the changes in srGAP2 expression,and the effects of srGAP2 over-expression on the viability of hSOD1G93A mutant NSC34 cells and the growth of cell protrusions.Results Bioinformatics analysis revealed abnormally low expression of srGAP2 in the spinal cord of hSOD1 mutant ALS mice.Animal experiments verified that compared with the WT mice,the expression of srGAP2 was reduced at both mRNA level and protein level in the spinal cord of hSOD1G93A mutant ALS transgenic mice at early-onset,mid-onset and late-onset stages.Compared with the WT mice,srGAP2 integral absorbance(IA)values in srGAP2+/NeuN+double-positive cells in the anterior horn of the spinal cord of hSOD1G93A mutant ALS transgenic mice were lower,srGAP2 IA values in srGAP2+/GFAP+double-positive cells were higher;Compared with the hSOD1WT NSC34 cells,the expression of srGAP2 was reduced at both mRNA level and protein level in hSOD1G93A mutant NSC34 cells.Over-expression of srGAP2 elevated the viability of hSOD1G93A mutant NSC34 cells,and up-regulated the expression level of synapse-related protein β Ⅲ-tubulin and growth associated protein 43(GAP43).Conclusion Low expression of srGAP2 is closely associated with the progression of ALS,while over-expression of srGAP2 can promote outgrowth of cell protrusions and exert a protective effect on spinal motor neurons in ALS.
5.ESTABLISHMENT AND APPLICATION OF A MULTIPLEX PCR DETECTION METHOD FOR THREE COMMON PATHOGENS CAUSING DIARRHEA IN YAKS
Yao PAN ; Jing ZHANG ; Mei-Jun JIN ; Ling-Xiang XIN ; Hai-Yue XIAO ; Yan LIU ; Wen-Sheng YAO ; Chuan CHENG ; Li-Li HAO ; Lan LAN ; Liang-Quan ZHU
Acta Parasitologica et Medica Entomologica Sinica 2025;32(3):146-152
Objective To establish a rapid,sensitive,and specific multiplex PCR detection method for the simultaneous detection of Cryptosporidium,Eimeria,and bovine parvovirus.Methods Specific primers targeting the SSU rRNA genes of Cryptosporidium and Eimeria,as well as the VP2 gene of bovine parvovirus were designed and the corresponding recombinant plasmid standards were constructed.To establish the multiplex PCR method,the reaction conditions were optimized using temperature gradient PCR and single-variable control methods.The sensitivity,specificity,reproducibility,and clinical application of the protocol were evaluated.Results The optimal annealing temperature was found to be 60.5℃,and the forward and reverse primer concentrations were determined to be 0.2 μmol/L for Eimeria,and 0.4 μmol/L for Cryptosporidium and bovine parvovirus.The assay demonstrated high sensitivity,with detection limits of 243,260,and 3 110 copies for the recombinant plasmid standards of Cryptosporidium,Eimeria,and bovine parvovirus,respectively.Specificity testing showed no cross-reactivity with ten common bovine pathogens,including Salmonella,bovine viral diarrhea virus,and bovine rotavirus.Consistent intra-and inter-batch results confirmed the strong reproducibility of the method.Clinical application to 81 diarrhea samples from various regions in the Ganzi Prefecture,Sichuan,revealed positivity rates of 18.52%(15/81)for Cryptosporidium,34.57%(28/81)for Eimeria,and 18.52%(15/81)forbovineparvovirus,withamixedinfectionrateof3.7%(3/81).Conclusions Themultiplex PCR method established in this study offers a reliable tool for differential diagnosis and epidemiological investigation of the three common diarrheal pathogens in yaks.
6.Effects of ACBT Technique Combined with Resistance Breathing Training on Patients with AECOPD
Mei CEN ; Ming JIN ; Rui ZHANG ; Yi YANG ; Zhixiang ZHANG
Journal of Kunming Medical University 2025;46(3):171-176
Objective To investigate the effects of active respiratory circulation technique(ACBT)combined with resistance breathing training on pulmonary function and exercise capacity in patients with acute exacerbations of chronic obstructive pulmonary disease(AECOPD).Methods A total of 60 hospitalized AECOPD patients from the Geriatrics Department of a tertiary hospital in Kunming were selected as research subjects from January to May 2024.Patients were randomly divided into two groups,with 30 patients in each group.The control group received conventional pulmonary rehabilitation nursing,and the observation group received ACBT combined with resistance breathing training.Pulmonary function indicators,modified British Medical Council(mMRC)dyspnea scores,6-minute walk test(6MWT)distances,and the COPD Assessment Test(CAT)scores were collected 4 weeks after discharge to evaluate the intervention's effectiveness.Results After the intervention,the observation group showed significant improvements in lung function,mMRC scores,6M WT distances,and CAT scores compared to pre-intervention levels,and all were superior to the control group(P<0.05).Conclusion ACBT combined with resistance breathing training can more effectively promote sputum clearance,reduce respiratory muscle fatigue,increase exercise endurance,and improve dyspnea severity and lung function in AECOPD patients.
7.The role and mechanism of gut microbiota in the development of diabetic nephropathy
Mei HAN ; Gang ZHANG ; Jin ZHAO ; Anjing WANG ; Yueqing HUI ; Shiren SUN
Journal of Capital Medical University 2025;46(4):742-748
Diabetic nephropathy(DN)ranks as a frequent and serious complication in diabetes mellitus,and it's a key factor in chronic kidney disease and end-stage renal disease(ESRD),greatly diminishing the life quality of patients.Presently,the conventional treatment approaches for DN mainly involve strict regulation of blood sugar and pressure,in conjunction with the application of renin-angiotensin-aldosterone system inhibitors.While these therapies can slow down the advancement of DN,they are ineffective in stopping its final development into ESRD.Lately,the involvement of the gut-kidney axis in the development and advancement of DN has attracted growing interest.Individuals suffering from DN show changes in the variety of gut microbiota,which are crucial in the development and management of DN due to metabolic interactions with the host.The goal of this analysis is to explore the fundamental processes of gut microbiota's role in DN and explore treatment approaches focusing on gut microbiota,aiming to offer new perspectives for the clinical handling of DN.
8.The anti-heart failure mechanism of N-acetylcysteine in diabetic cardiomyopathy via ERK1/2 path-way
Jian JI ; Ya-hong HUANG ; Ying-min LU ; Dong-mei YUE ; Xiao-hui ZHENG ; Jin-chun ZHANG ; Zhao-xia WANG
Chinese Journal of cardiovascular Rehabilitation Medicine 2025;34(4):543-547
Objective:To investigate the anti-heart failure mechanism of N-acetylcysteine(NAC)in diabetic cardiomyop-athy independent from coronary artery factors.Methods:A total of 40 diabetic mice after heart failure model construction were randomly divided into two groups,NAC group(n=20,NAC 100mg·kg-1·d-1)and control group(n=20,Saline 100 mg·kg-1·d-1).Echocardiography was performed to detect left ventricular end-diastolic volume(LVEDV),left ventricular end-systolic volume(LVESV),left ventricular ejection fraction(LVEF),mitral left ventricular early-dias-tolic peak flow velocity/left ventricular late-diastolic peak flow velocity(E/A),isovolumic relaxation time(IVRT)and cardiac output(CO)after 4 weeks.Terminal uridine nick-end labeling(TUNEL)was performed to detect apoptosis in-dex,and Western Blot was performed to detect the expression of extracellular regulated protein kinases(ERK)1/2 after 6 weeks in two groups.Results:Compared to those in control group,mice in NAC group had significant higher LVEF[(40.5±3.4)%vs.(36.9±3.2)%],E/A[(1.5±0.1)vs.(1.4±0.1)]and CO[(10.3±0.6)ml/min vs.(9.9±0.5)ml/min](P<0.05 or<0.01);and significant lower LVESV[(23.1±1.3)μl vs.(24.7±1.5)μl],apoptosis index[(31.2±0.5)%vs.(45.1±0.9)%]and the expression of ERK1/2[(2.2±0.2)vs.(3.9±0.1)](P<0.001 all).Conclusion:NAC exerts anti-heart failure effect by attenuating apoptosis of cardiomyocytes via regulating ERK1/2 pathway.
9.The role and mechanism of gut microbiota in the development of diabetic nephropathy
Mei HAN ; Gang ZHANG ; Jin ZHAO ; Anjing WANG ; Yueqing HUI ; Shiren SUN
Journal of Capital Medical University 2025;46(4):742-748
Diabetic nephropathy(DN)ranks as a frequent and serious complication in diabetes mellitus,and it's a key factor in chronic kidney disease and end-stage renal disease(ESRD),greatly diminishing the life quality of patients.Presently,the conventional treatment approaches for DN mainly involve strict regulation of blood sugar and pressure,in conjunction with the application of renin-angiotensin-aldosterone system inhibitors.While these therapies can slow down the advancement of DN,they are ineffective in stopping its final development into ESRD.Lately,the involvement of the gut-kidney axis in the development and advancement of DN has attracted growing interest.Individuals suffering from DN show changes in the variety of gut microbiota,which are crucial in the development and management of DN due to metabolic interactions with the host.The goal of this analysis is to explore the fundamental processes of gut microbiota's role in DN and explore treatment approaches focusing on gut microbiota,aiming to offer new perspectives for the clinical handling of DN.
10.CT and MR findings of duodenal stromal tumors
Mei JIN ; Xinyue ZHANG ; Jiyuan LI ; Jinsong GUO ; Ning ZHANG ; Yunlong YUE
Journal of Practical Radiology 2025;41(6):974-978
Objective To explore the CT and MR features of duodenal stromal tumors(DST).Methods The clinical,imaging and pathological data of patients with pancreaticoduodenal groove(PDG)mass detected by CT and/or MR,and pathologically diagnosed as DST were retrospectively analyzed.All imaging features were analyzed.Results Finally,all 11 patients were included,all of whom presented external mass in descending duodenum with clear boundary.There were 5 patients with irregular mass and 6 patients with circular mass.Two patients showed homogeneous mass on plain CT and MR imaging,while 9 patients presented heterogeneous mass(with varying degrees of calcification,hemorrhage,cystic degeneration,and necrosis),showed heterogeneous density on CT findings or high,low and iso signals on T1WI and T2WI without high signal on diffusion weighted imaging(DWI),and high enhancement on arterial phase,portal venous phase and delayed phase.There were no signs of air accumulation in all masses.Conclusion Stromal tumors commonly present external heterogeneous mass in descending duodenum with clear boundary.Familiarity with its predilection location and corresponding CT and MR findings are helpful for the qualitative diagnosis of DST.

Result Analysis
Print
Save
E-mail