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.Accuracy of Magnetic Resonance Spectroscopy–Detected Fumarate Peak for Diagnosing Fumarate Hydratase Deficiency in Uterine Leiomyomas: A Prospective Study
Guiqin LIU ; Wenxin YU ; Shihang PAN ; Yuansheng LUO ; Jingli CHEN ; Mengying ZHU ; Zaoyu WANG ; Yang SONG ; Jin ZHANG ; Jianrong XU ; Yan ZHOU ; Jun MA ; Guangyu WU
Korean Journal of Radiology 2026;27(5):440-451
Objective:
To evaluate the diagnostic performance of magnetic resonance spectroscopy (MRS) in discriminating fumarate hydratase-deficient (FH-d) uterine leiomyomas (ULs) from FH-preserved ULs.
Materials and Methods:
This study consisted of three stages, with independent cohorts recruited for each stage: 1) sample-size estimation was retrospectively performed on UL specimens (diameter ≥3 cm; age, 20–40 years) from our database with immunohistochemistry (IHC) for 2-succinocysteine (2-SC) as the reference, without genetic testing, 2) MRS sequence optimization in confirmed FH germline mutation participants with ultrasound-detected ULs (diameter ≥3 cm), without IHC analysis, and 3) prospective diagnostic test accuracy was evaluated in consecutive participants with ultrasound-detected ULs (diameter ≥3 cm;age, 20–40 years), using IHC for 2-SC for determining the FH status and subsequent genetic testing in those with positive 2-SC results to identify whether FH mutations were germline or somatic in origin. The choline and fumarate peaks in MRS were classified as positive, negative, or technical failure (TF). TFs were analyzed separately and excluded from the primary diagnostic accuracy calculations. T1-, T2-, and diffusion-weighted images were interpreted as hyperintense or hypointense. The enhancement rate and apparent diffusion coefficient were also acquired. Diagnostic performance was compared between MRS and various magnetic resonance imaging (MRI) features.
Results:
The optimal MRS parameters for the fumarate peak were echo time (TE) = 140 ms and an average of 256. Among the 360 prospective participants, 37 were confirmed to have FH-dULs. MRS showed positive fumarate peaks in 35 of 37 FH-dULs.After excluding six TFs, the positive fumarate peak on MRS showed 94.6% (35/37) sensitivity, 99.7% (316/317) specificity, and 99.2% (351/354) accuracy, all of which were significantly superior to those of other MRI features (P ≤ 0.002).
Conclusion
A positive fumarate peak on MRS may be a useful imaging biomarker for diagnosing FH-dULs.
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.Molecular Mechanisms and Research Progress of Hepatic Injury Induced by Hypercholesterolemia
Xing-tong LAN ; Yi-fan WANG ; Meng-yuan LIU ; Zi-yi GUO ; Jin-bo ZHANG ; Qi-hang WANG ; Yu-dian ZHANG
Progress in Modern Biomedicine 2025;25(17):2865-2874
Hypercholesterolemia is pathologically characterized by abnormal accumulation of low-density lipoprotein cholesterol,which is closely associated with metabolic dysfunction-associated fatty liver disease and increased cardiovascular risks.Hepatocytes maintain cholesterol homeostasis through LDL receptor-mediated uptake and esterification storage mechanisms.However,chronic cholesterol overload induces mitochondrial dysfunction,reactive oxygen species accumulation,and endoplasmic reticulum stress,leading to hepatocyte injury.Moreover,systemic hypercholesterolemia disrupts gut microbiota balance and impairs short-chain fatty acid and ketone metabolism,exacerbating metabolic disturbances and aggravating hepatic injury through enhanced metabolic stress.In this article,we review the advance of studies on hypercholesterolemia in recent years and summary its association with hepatic injury,which can provide theoretical support for further research.
5.Construction of CD8+T cell-associated Risk Model in Hepatocellular Carcinoma Based on Bulk and Single-cell RNA-seq Data
Xin-Tong ZHANG ; Jian-Jun ZHU ; Jin WU ; Hao WU ; Fan LU ; Wen-Tao ZHANG ; Jing-Jia CHANG ; Ting TANG ; Zhi-Gao OU ; Feng-Feng JIA ; Li LI ; Peng-Fei YU ; Ming LIU
Chinese Journal of Biochemistry and Molecular Biology 2025;41(10):1511-1528
Hepatocellular carcinoma(HCC),which is essentially primary liver cancer,is closely related to CD8+T cell immune infiltration and immune suppression.We constructed a CD8+T cells related risk score model to pre-dict the prognosis of HCC patients and provided therapeutic guidance based on the risk score.Using integrated bulk RNA sequencing(RNA-seq)and single-cell RNA sequencing(scRNA-seq)datasets,we identified stable CD8+T cell signatures.Based on these signatures,a 3-gene risk score model,comprised of KLRB1,RGS2,and TN-FRSF1B was constructed.The risk score model was well validated through an independent external validation co-hort.We divided patients into high-risk and low-risk groups according to the risk score and compared the differ-ences in immune microenvironment between these two groups.Compared with low-risk patients,high-risk patients have higher M2-type macrophage content(P<0.0001)and lower CD8+T cells infiltration(P<0.0001).High-risk patients predict worse response to immunotherapy treatment than low-risk patients(P<0.01).Drug sensitivity a-nalysis shows that PI3K-β inhibitor AZD6482 and TGFβRII inhibitor SB505124 may be suitable therapies for high-risk patients,while the IGF-1R inhibitor BMS-754807 or the novel pyrimidine-based anti-tumor metabolic drug Gemcitabine could be potential therapeutic choices for low-risk patients.Moreover,expression of these 3-gene mod-el was verified by immunohistochemistry.In summary,the establishment and validation of a CD8+T cell-derived risk model can more accurately predict the prognosis of HCC patients and guide the construction of personalized treatment plans.
6.Design and application of one piece temperature controlled enema device
Yu LI ; Huiyang SUN ; Yanjiang LIU ; Tong JIN ; Ze LI ; Yan BAI
Chinese Journal of Practical Nursing 2025;41(17):1290-1295
Objective:To design a safe and convenient temperature-controlled enema device and to verify its effectiveness in enema operation.Methods:This study was a quasi experimental research. A total of 60 patients who were hospitalized in Jilin Provincial Cancer Hospital from October 2023 to January 2024 were selected as the control group, and 60 patients who underwent enema from February to May 2024 were selected as the experimental group adopting convenience sampling method. The control group used traditional enema operation method, while the experimental group used an integrated temperature controlled enema device for enema operation. The heating time of enema solution, retention time of enema solution in the intestine, patient satisfaction rate, and nursing staff satisfaction rate were compared between the two groups before enema operation.Results:There were 39 males and 21 females in the control group, aged (54.78 ± 10.66) years, and 42 males and18 females in the experimental group, aged (56.15 ± 9.88) years. The warming time of enema fluid in the experimental group was 17.00 (15.25, 18.00) s, which was lower than 55.00 (47.00, 59.75) s in the control group, and the difference was statistically significant ( Z = - 9.47, P<0.01). The intraintestinal retention time of enema fluid in the experimental group was 9.50 (9.00, 10.00) min, which was greater than 7.00 (6.00, 8.75) min in the control group, and the difference was statistically significant ( Z = - 7.93, P<0.01); the satisfaction rate of the experimental group was 96.67% (58/60), which was higher than 73.33% (44/60) in the control group, and the difference was statistically significant ( χ2 = 12.81, P<0.01); the satisfaction rate of the nursing staff in the experimental group was 96.00% (48/50), which was higher than 66.00% (33/50) in the control group, and the difference was statistically significant ( χ2 = 14.62, P<0.01). Conclusions:The integrated temperature-controlled enema device reduces the tediousness of nursing operation. It improves the rate of complete preparation of goods, patient satisfaction and nursing staff satisfaction, thereby ensuring efficacy and patient safety.
7.Perioperative antithrombotic medication use in non-cardiac surgery:a single center survey
Bin-bin DONG ; Yu-tong ZHAO ; Zi-ning WANG ; Huai-jin LI ; Shan ZHU ; Hong ZHANG ; Yan-jun GONG ; Jie JIANG
Chinese Journal of Interventional Cardiology 2025;33(4):181-188
Objective To investigate the perioperative management of antithrombotic drugs in patients undergoing non-cardiac surgery.Methods Patients on long-term antithrombotic drugs who underwent non-cardiac surgery in our hospital were included.Through interviews with patients and physicians,perioperative antithrombotic medication regimens were reviewed and compared with the"Multidisciplinary Expert Consensus on Perioperative Management of Antithrombotic Therapy"to evaluate compliance with consensus and analyze influencing factors.Results A total of 372 patients were included in the analysis.Among them,355 patients were on long-term antiplatelet therapy alone,and 17 were on long-term oral anticoagulantion.364(97.8%)discontinued antithrombotic medications prior to surgery.109 patients(29.3%)received low molecular weight heparin(LMWH)bridging therapy.Among the 355 patients on antiplatelet therapy,108(30.4%)had discontinuation durations consistent with the consensus recommendations,while 186(52.4%)discontinued medications for longer periods.Postoperatively,the average hospital stay for antiplatelet therapy patients was 6.64 days,with only 37(10.4%)resuming therapy during hospitalization.The average hospital stay for patients on anticoagulants was 9.94 days,with only 2(11.8%)resuming therapy during hospitalization.Regarding perioperative risk assessment,only 40(10.8%)of patients underwent additional internal medical evaluation for thromboembolic risk after medication discontinuation,with the remainder assessed soly by surgeons.Coronary heart disease was an independent risk factor associated with internal medical evaluation(OR 2.851,95%CI 1.160-7.011,P=0.022).For bleeding risk assessment,surgeons evaluations aligned with the consensus in 68.0%of cases,but surgeons tended to underestimate risk compared to the consensus.Conclusions In this single-center study,perioperative antithrombotic management showed low compliance with expert consensus,characterized by prolonged preoperative medication discontinuation,high rates of LMWH bridging,and low postoperative in-hospital resumption of therapy.A robust multidisciplinary collaboration system should be established to enhance comprehensive patient assessment.
8.Incidence and risk factors of acute focal bacterial nephritis in children with febrile urinary tract infections
Yu CHEN ; Xiaojian QIU ; Jin LIN ; Feng ZHAO ; Yonghui YANG ; Huajuan TONG ; Zihua YU
Chinese Journal of Pediatrics 2025;63(9):967-971
Objective:To determine the incidence of acute focal bacterial nephritis (AFBN) in children with febrile urinary tract infections (UTI) and to identify associated risk factors.Methods:A retrospective cohort study included 181 children hospitalized with febrile UTI at Fujian Children′s Hospital from April 2021 to September 2023. Demographic data, clinical manifestations, and laboratory findings were collected. Patients were categorized into AFBN group and non-AFBN group based on AFBN diagnosis. The incidence of AFBN within this febrile UTI cohort was determined. The diagnostic value for pre-antibiotic blood white blood cell (WBC) count and C-reactive protein (CRP) level, along with post-treatment fever duration and pyuria duration for AFBN was evaluated using the area under curve (AUC) of the receiver operating characteristic (ROC).Results:The cohort comprised 181 children with febrile UTI (100 boys and 81 girls), age of 0.6 (0.3, 1.0) years. Eleven patients (6.1%) were diagnosed with AFBN. For predicting AFBN, the AUC was 0.71 (95% CI 0.55-0.85, P=0.026) for pre-antibiotic WBC count and 0.80 (95% CI 0.71-0.90, P=0.001) for pre-antibiotic CRP level, with optimal cutoff values of 16.0×10 9/L and 80.1 mg/L, respectively. For post-treatment parameters, the AUC was 0.79 (95% CI 0.69-0.92, P=0.001) for fever duration and 0.84 (95% CI 0.72-0.97, P<0.001) for pyuria duration, with optimal cutoff values of 1.9 d and 5.5 d, respectively. The combination of pre-antibiotic WBC count >16.0×10 9/L and CRP>80.1 mg/L yielded a sensitivity of 0.73 and a specificity of 0.86 for AFBN prediction. Similarly, the combination of post-treatment fever duration ≥2 d and pyuria duration >5 d demonstrated a sensitivity of 0.82 and specificity of 0.88. Conclusions:AFBN incidence was 6.1% in this cohort of children with febrile UTI. AFBN should be highly suspected in febrile UTI children presenting with either a pre-antibiotic WBC count >16.0×10 9/L and CRP >80.1 mg/L, or a post-treatment fever duration ≥2 d and pyuria duration >5 d.
9.Comparative study on the resistance of phage and bacteria indicative of the disinfection effect on object surfaces
Cuicui MA ; Qi ZHANG ; Ying TONG ; Li YU ; Qiongqiong FANG ; Baoying ZHANG ; Jin SHEN
Chinese Journal of Preventive Medicine 2025;59(10):1703-1707
Objective:To investigate the differences in resistance to chemical disinfectants between bacteriophage Phi-X174 and disinfection effect indicator microorganisms, Staphylococcus aureus and Escherichia coli. Methods:Three commonly used disinfectants, including chlorine-containing disinfectants, alcohol-based disinfectants and quaternary ammonium salt disinfectants, were selected to analyze the differences in resistance of phage Phi-X174, Staphylococcus aureus and Escherichia coli by using the quantitative sterilization test of suspension. Results:The 250 mg/L sodium dichloroisocyanurate solution treated for 10 minutes yielded log reduction values of 3.39, 7.25 and 7.37 against phage Phi-X174, Staphylococcus aureus and Escherichia coli respectively. The 40% ethanol for 1 minute yielded log reduction values of 0.03, 2.46 and 7.30 against phage Phi-X174, Staphylococcus aureus and Escherichia coli, respectively. The 100 mg/L benzalkonium chloride for 10 minutes yielded log reduction values of 1.41, 6.84 and 0.93 for phage Phi-X174, Staphylococcus aureus and Escherichia coli, respectively. Conclusion:Phage Phi-X174 has stronger resistance to chlorine-containing disinfectants and alcohol-based disinfectants than Escherichia coli and Staphylococcus aureus. Its resistance to quaternary ammonium salt disinfectants is between that of Staphylococcus aureus and Escherichia coli.
10.Deep learning reconstruction algorithm for improving imaging quality of accelerated T2WI of cervical tumors
Yun WANG ; Tianjiao WANG ; Yu CHEN ; Tong SU ; Feng FENG ; Zhengyu JIN
Chinese Journal of Medical Imaging Technology 2025;41(9):1573-1576
Objective To observe the value of deep learning(DL)reconstruction algorithm for improving imaging quality of accelerated T2WI of cervical tumors.Methods A total of 43 patients with suspected cervical tumors were prospectively enrolled.Cervical conventional T2WI and accelerated T2WI based on DL reconstruction(DL-T2WI)were acquired.The imaging quality was subjectively assessed by 2 physicians using a 4-point system,including overall image quality,artifact,noise,sharpness and lesion detectability scores,and then were compared between conventional T2WI and DL-T2WI.Results The acquisition of conventional T2WI took 116 s,while of DL-T2WI took 101 s.The inter-observer consistency of subjective evaluation results on the overall image quality,artifact,noise,sharpness and lesion detectability scores were all excellent(Kappa=0.851-0.969).No significant difference of subjective evaluation results on overall image quality nor lesion detectability scores was found between conventional T 2WI and DL-T2WI(both P>0.05),while the artifact and sharpness scores of DL-T2WI were significantly higher but the noise score was significantly lower than those of conventional T2WI(all P<0.05).Conclusion DL reconstruction algorithm was helpful for improving imaging quality of accelerated T 2WI of cervical tumors.

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