1.The Clinical and Genetics Characteristics of Oculopharyngodistal Myopathy
Jiaxi YU ; Zhihao QUAN ; Yilei ZHENG ; Jing AN ; Jing LIU ; Qingqing WANG ; Lingchao MENG ; Meng YU ; Zhiying XIE ; Jianwen DENG ; He LYU ; Wei ZHANG ; Yun YUAN ; Zhaoxia WANG
JOURNAL OF RARE DISEASES 2026;5(2):164-174
To analyze the clinical and genetic features of oculopharyngodistal myopathy (OPDM) patients and compare the phenotypic differences among various causative genes. A total of 65 genetically confirmed OPDM patients from 43 unrelated families, who were admitted to the Department of Neurology, Peking University First Hospital between January 2008 and December 2025, were retrospectively included.The general demographic data, clinical manifestations, laboratory/auxiliary examinations, muscle pathology, and genetic test results were systematically collected and analyzed. The clinical and pathological characteristics among different OPDM subtypes were compared. Among the 65 patients(39 male and 26 female), the mean age of onset was (31.20±10.43) years (range: 14 to 63 years). The initial symptom was predominantly distal limb weakness (67.44%), which gradually progressed to involve the extraocular muscles, pharyngeal muscles, facial muscles and proximal limb muscles. Serum creatine kinase levels were mildly to moderately elevated. Muscle pathological examinations revealed rimmed vacuoles and intranuclear inclusions (within muscle fibers). The mean duration from onset to diagnosis was (12.33±7.88) years (range: 1 to 32 years). All probands had negative results on conventional next-generation whole-exome sequencing; pathogenic variants were identified through third-generation long-read sequencing or OPDM-targeted repeat-primed polymerase chain reaction(RP-PCR). Among the 43 families, OPDM2 subtype was the most common genetic subtype ( OPDM2 was the predominant subtype in this study. All subtypes share similar age of onset and muscular pathological changes, yet exhibit distinct disease progression patterns. Future multicenter prospective cohort studies are warranted to further elucidate the clinical characteristics, pathogenetic mechanisms, and prognostic differences among OPDM subtypes.
2.Development and exploration of a closed-loop management model for externally dispensed intravenous prescriptions
Xuhua XIE ; Yun WU ; Songqing HUANG ; Yukun HUANG ; Siyan CHEN ; Zheng ZENG ; Weiyan TANG ; Zuolong HE ; Chunxia ZHOU ; Hongliang ZHANG
China Pharmacy 2026;37(10):1246-1250
OBJECTIVE To construct a closed-loop management model for externally dispensed intravenous prescriptions, and to provide reference for standardized management of externally dispensed intravenous prescriptions. METHODS Based on the Expert Consensus on Closed-loop Management of Externally Dispensed Intravenous Prescriptions in Guangxi Zhuang Autonomous Region previously formulated by our hospital, risk points during the entire process were systematically identified through multidisciplinary team brainstorming and a fishbone diagram. A series of strategies were subsequently formulated and implemented, including qualifying designated external dispensing pharmacies and the drug catalogs, operating and maintaining the hospital information system and the Pharmacy Intravenous Admixture Service (PIVAS) intelligent management platform, and strengthening differentiated training for staff in the whole workflow. A whole-process closed-loop management system was constructed with PIVAS as the co re hub and the daytime chemotherapy center as the safety terminal. RESULTS A total of 3 cooperating pharmacies and an initial drug list comprising 35 product specifications were selected. A closed‑loop management process encompassing hospital outpatient prescribing, patient drug purchase in designated pharmacies, PIVAS drug dispensing, and medication use in daytime chemotherapy center was successfully established. This system enabled the mandatory grouping and association of externally dispensed intravenous prescriptions with in-hospital diluents, full-process verification based on drug traceability codes, intelligent monitoring of infusion parameters, and whole-process data traceability. CONCLUSIONS The constructed model effectively resolves the coordination and safety oversight during the use of externally dispensed intravenous drugs from out-of-hospital circulation to in-hospital use, and has preliminarily enabled procedural standardization, whole-process information traceability, and proactive control of medication risks.
3.Dynamic prediction of JC polyomavirus reactivation after kidney transplantation
Mengyao LI ; Chengfeng ZHANG ; Difei REN ; Xin LIANG ; Shuyu CHEN ; Yushi PENG ; Yanjie WANG ; Meng ZHANG ; Jian XU ; Zheng CHEN ; Yun MIAO ; Yibin WANG
Organ Transplantation 2026;17(4):635-643
Objective To construct a dynamic prediction model for JC polyomavirus (JCV) reactivation after kidney transplantation. Methods A retrospective analysis was conducted on the clinical data of 128 recipients who met the inclusion criteria and received kidney transplantation in Nanfang Hospital, Southern Medical University, from June 2021 to December 2024. Dynamic Cox regression and dynamic restricted mean survival time (RMST) regression based on the landmark method were adopted to establish the dynamic prediction models, and Monte Carlo cross-validation was used to evaluate model performance. Results The dynamic models could predict the incidence and onset time of JCV reactivation within the subsequent 3 months according to covariates at each landmark time point from the 1st to the 6th month after transplantation. Remuzzi score, body mass index and warm ischemia time were independent risk factors for JCV reactivation, while a history of urinary BK polyomavirus reactivation served as a protective factor. The median values of the area under the curve, C-index and Brier score of the dynamic Cox model were 0.873, 0.851 and 0.065 respectively, and the C-index of the dynamic RMST model was 0.829, all of which were superior to those of the static model. Conclusions The landmark-based dynamic models exhibit excellent predictive performance, which may integrate baseline data and post-transplant follow-up information to realize dynamic assessment of the risk and time window of JCV reactivation, thereby providing evidence for optimizing post-operative monitoring and individualized intervention strategies.
4.Research progress on outdoor wet bulb globe temperature prediction models and their application to early warning for occupational heat stress
Xiaojun ZHANG ; Luyang WANG ; Xin SUI ; Xiaoshun WANG ; Tian LAN ; Yun ZHENG ; Dongsheng NIU ; Xiaowen DING
Journal of Environmental and Occupational Medicine 2026;43(8):1032-1038
In the context of global warming, protecting outdoor workers from occupational heat exposure has become an important public health concern. This review summarized the principle, applicability, and limitation of three categories of wet bulb globe temperature (WBGT) prediction models: empirical regression models, heat-balance models, and machine-learning models. Drawing on international experience from the United States, Europe, and Japan, this review further examined the technical challenges of applying WBGT-based prediction models to large-scale occupational heat stress early warning systems. In response to gaps in occupational heat stress management in China, WBGT should be considered a core assessment indicator to support a transition from passive response to proactive intervention. A localized implementation pathway is proposed, incorporating mechanism-based model calibration, regional parameter adaptation, and multi-source data fusion. Strengthening collaboration between public health and meteorological departments and developing platform-based flexible intervention strategies for workers in new forms of employment may improve the targeting of occupational heat stress early warning and health protection. Future research should focus on physics-constrained machine learning, refined microenvironmental prediction, and individualized risk assessment to support the development of a digitally enabled occupational heat-health protection framework.
5.Analysis of factors influencing early recurrence for patients with initially unresectable hepa-tocellular carcinoma who underwent liver resection following downstaging treatment and construction of a predictive model: a multicenter study
Yun YANG ; Peng LU ; Kongying LIN ; Zheng DANG ; Wei GUO ; Zeya PAN ; Weiping ZHOU
Chinese Journal of Digestive Surgery 2025;24(2):223-235
Objective:To investigate the factors influencing early recurrence for patients with initially unresectable hepatocellular carcinoma (HCC) who underwent salvage liver resection (SLR) following transcatheter arterial chemoembolization-based downstaging treatment, and construct a predictive model to evaluate its predicting performance.Methods:The retrospective cohort study was constructed. The clinicopathological data of 305 patients with initially unresectable HCC who were admitted to 4 medical centers in China, including the Third Affiliated Hospital of Naval Medical University (Shanghai Eastern Hepatobiliary Surgery Hospital) et al, from January 2019 to December 2021 were collected. There were 286 males and 19 females, aged (48.7±10.4)years. A total of 133 patients who were admitted from January 2019 to December 2020 were set as the training cohort, and the other 172 patients who were admitted from January to December 2021 were set as the validation cohort. Observation indicators: (1) postoperative recurrence-free survival in HCC patients; (2) analysis of factors influencing postoperative early recurrence in HCC patients; (3) construction and validation of the predictive model. Comparison of measurement data with normal distribution between groups was conducted using the independent sample t test. Comparison of count data between groups was conducted using the chi-square test. Comparison of ordinal data was conducted using the rank sum test. Univariate and multivariate analyses were conducted using the Cox regre-ssion model. The Kaplan-Meier method was used to calculate survival. The Log-rank test was used for survival analysis. The predicting performance of the model was evaluated using the concordance index (C-index) and the area under curve (AUC) of time-dependent receiver operating characteristic (ROC) curve, and the accuracy of the model was validated using the calibration curve. The total net gain of the model was evaluated using the decision curve. Results:(1) Postoperative recurrence-free survival in HCC patients. The recurrence-free survival time of 133 HCC patients in the training cohort was 10.0(range, 1.5-24.0)months, with 1-, 2-year recurrence-free survival rate of 47.3% and 36.8%. The recurrence-free survival time of 172 HCC patients in the validation cohort was 11.0(range, 1.0-24.0)months, with 1-, 2-year recurrence-free survival rate of 51.7% and 37.2%. There was no significant difference in recurrence-free survival between patients in the training cohort and the validation cohort ( χ2=0.075, P>0.05). (2) Analysis of factors influencing postoperative early recur-rence in HCC patients. Results of multivariate analysis showed that tumor burden prior to down-staging treatment, grade of albumin-bilirubin (ALBI) score prior to SLR, alpha-fetoprotein (AFP) half-life prior to SLR, and tumor response prior to SLR were independent factors influencing early recurrence in HCC patients after surgery [ hazard ratio=3.212, 2.526, 2.304, 1.575, 95% confidence interal ( CI) as 1.262-8.175, 1.324-4.818, 1.477-3.595, 1.138-2.180, P<0.05]. (3) Construction and validation of the predictive model. A nomogram predictive model for postoperative early recurrence was constructed base on the results of multivariate analysis. The C-index of predictive model was 0.786 for the training cohort and 0.734 for the validation cohort. The AUC of ROC curve of nomogram predictive model for 12-, 18-, and 24-month recurrence-free survival rate in the training cohort were 0.890 (95% CI as 0.836-0.944), 0.895 (95% CI as 0.842-0.947), and 0.887 (95% CI as 0.831-0.942), respectively. The AUC of ROC curve of nomogram predictive model for 12-, 18-, and 24-month recurrence-free survival rate in the validation cohort were 0.845 (95% CI as 0.781-0.909], 0.888 (95% CI as 0.826-0.950), and 0.919 (95% CI as 0.870-0.968), respectively. Results of calibration curve showed high consistency between the predicted results of nomogram predictive model and actual outcomes. Results of decision curve showed the nomogram predictive model with a good total net gain at a threshold of 0.10-0.50. Conclusions:Tumor burden prior to downstaging treatment, grade of ALBI score prior to SLR, AFP half-life prior to SLR, and tumor response prior to SLR are independent factors influencing early recurrence in initially unresectable HCC patients undergoing SLR following downstaging treatment. The nomogram predictive model based on these factors can effectively evaluate the prognosis of this patient population.
6.The correlation between interleukin-10, albumin levels and short-term prognosis in patients with acute lung injury
Xia TU ; Yun WU ; Jun CHEN ; Jun ZHENG
Chinese Journal of Postgraduates of Medicine 2025;48(5):411-417
Objective:To investigate the correlation between interleukin-10, albumin levels and short-term prognosis in patients with acute lung injury.Methods:A retrospective study was conducted to collect data on 150 patients with acute lung injury admitted to the Fourth People′s Hospital of Longgang District, Shenzhen from January 2021 to December 2023. The short-term prognosis was evaluated based on the mortality rate within 28 d of admission, and the patients were divided into a mortality group 53 cases and a survival group 97 cases. General information, levels of interleukin-10 and albumin within 24 h of admission, and other laboratory indicators were compared between the two groups, with a focus on analyzing the correlation between interleukin-10, albumin levels and the short-term prognosis of patients.Results:Murray lung injury score, acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) score and levels of C-reactive protein, interleukin-10 in the mortality group were higher than those in the survival group: (2.27 ± 0.36) scores vs. (1.98 ± 0.28) scores, (22.72 ± 3.27) scores vs. (19.85 ± 3.12) scores, (106.27 ± 14.22) mg/L vs. (93.22 ± 15.27) mg/L, (51.75 ± 8.12) ng/L vs. (46.27 ± 9.47) ng/L, the levels of platelet and albumin were lower than those in the survival group: (186.67 ± 23.11) ×10 9/L vs. (203.25 ± 25.36) ×10 9/L, (27.86 ± 4.75) g/L vs. (32.21 ± 5.61) g/L, with statistical significant differences ( P<0.05). Cox regression analysis showed that the mortality risk of patients with acute lung injury was related to Murray lung injury score, APACHEⅡ score, platelet, C-reactive protein, interleukin-10 and albumin levels at admission ( P<0.05). Restricted cubic spline (RCS) analysis and interaction testing found that the mortality risk of patients with acute lung injury showed a non-linear dose-response relationship with serum interleukin-10 and albumin levels ( P<0.05), and the two had a negative interaction with the mortality risk of patients. The interleukin-10, albumin-assisted Murray lung injury score, APACHE Ⅱ score, platelet and C-reactive protein were used to construct a nomogram prediction model, and the decision curve and nomogram measurement model were drawn. The model has certain predictive value for the short-term risk of death in patients with acute lung injury. Conclusions:The short-term mortality risk in patients with acute lung injury may be related to abnormal levels of interleukin-10 and albumin, which can assist in the early screening of high-risk mortality patients and serve as clinical intervention targets.
7.Application of a childlike cognition-empowered collaborative nursing model for elderly caregivers and pediatric burn patients aged 3 and younger
Huali ZHENG ; Yinming WANG ; Tao HAN ; Yun MA
Chinese Journal of Practical Nursing 2025;41(16):1222-1229
Objective:To explore the application effect of a childlike cognition-empowered collaborative nursing model in pediatric burn patients aged 3 years and younger and their elderly caregivers, so as to provide reference for improving the cooperative nursing ability of elderly caregivers and improving the treatment and rehabilitation effect of burn children.Methods:This quasi-experimental study enrolled 80 pediatric burn patients aged 3 years and younger admitted to Children′s Hospital of Nanjing Medical University from January 2022 to December 2023 via convenience sampling, each paired with one elderly caregiver. Based on admission time, they were divided into the control group (40 patients and caregivers admitted from January to December in 2022) and the intervention group (40 patients and caregivers admitted from January to December in 2023). The control group received routine nursing, while the intervention group received a childlike cognition-empowered collaborative nursing model. Self-rating Anxiety Scale (SAS) and Chinese version of Positive Aspects of Caregiving (PAC) were used to evaluate the degree of anxiety and positive feelings of the elderly caregivers in the 2 groups. FLACC (F: face; L: legs; A: activity; C: cry; C: consol ability) Pain Assessment Scale, Child Medical Fear Scale (CMFS) and Vancouver Scar Scale (VSS) were used to evaluate dressing change pain, medical fear and scar status of the patients in the 2 groups.Results:A total of 80 pediatric burn patients and 80 elderly caregivers were included. The patients were (2.5 ± 0.2) years old in the control group, with 23 males and 17 females, and were (2.6 ± 0.1) years old in the intervention group, with 24 males and 16 females. The elderly caregivers were (62.0 ± 1.5) years old in the control group, with 9 males and 31 females, and were (61.0 ± 1.2) years old in the intervention group, with 8 males and 32 females. There were no significant differences in SAS and PAC scores between the 2 groups of elderly caregivers before intervention (both P>0.05). There were no significant differences in FLACC Pain Assessment Scale, CMFS before intervention and VSS scores within 1 week after wound healing between the 2 groups of patients (all P>0.05). After the intervention, the SAS score of the elderly caregivers in the intervention group was (45.80 ± 2.81) points, lower than (57.78 ± 4.63) points in the control group, the difference was statistically significant ( t=13.99, P<0.05), and the PAC score in the intervention group was (38.85 ± 3.03) points, higher than (30.15 ± 3.03) points in the control group, the difference was statistically significant ( t=-15.24, P<0.05). The FLACC Pain Assessment Scale, CMFS after intervention, VSS scores at 6 months of rehabilitation treatment of the patients in the intervention group were (3.35 ± 0.45), (22.20 ± 1.07), (6.50 ± 0.68) points, lower than (7.00 ± 0.45), (51.43 ± 2.26), (8.68 ± 0.76) points in the control group, the differences were statistically significant ( t=34.86, 73.87, 13.45, all P<0.05). Conclusions:The childlike cognition-empowered collaborative nursing model can enhance the caregiving enthusiasm of elderly caregivers, alleviate negative emotions among both elderly caregivers and pediatric burn patients aged 3 and younger, improve the collaborative nursing abilities of elderly caregivers, and enhance the treatment and rehabilitation outcomes of pediatric burn patients.
8.Sesquiterpene ZH-13 from Aquilariae Lignum Resinatum Improves Neuroinflammation by Regulating JNK Phosphorylation
Ziyu YIN ; Yun GAO ; Junjiao WANG ; Weigang XUE ; Xueping PANG ; Huiting LIU ; Yunfang ZHAO ; Huixia HUO ; Jun LI ; Jiao ZHENG
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(1):139-145
ObjectiveTo study the pharmacological substances and mechanisms through which sesquiterpene ZH-13 from Aquilariae Lignum Resinatum improves neuroinflammation. MethodsBV-2 microglial cells were stimulated with lipopolysaccharide (LPS) to induce neuroinflammation. The cells were divided into the normal group, the model group, and the ZH-13 low- and high-dose treatment groups (10, 20 μmol·L-1). The model group was treated with 1 μmol·L-1 LPS. Cell viability was assessed using the cell proliferation and activity assay (CCK-8 kit). Nitric oxide (NO) release in the cell supernatant was measured using a nitric oxide kit (Griess method). The mRNA expression levels of interleukin-1β (IL-1β), tumor necrosis factor-α (TNF-α), inducible nitric oxide synthase (iNOS), and interleukin-6 (IL-6) were detected by real-time fluorescence quantitative polymerase chain reaction (Real-time PCR). The phosphorylation of mitogen-activated protein kinase (MAPK) pathway proteins was assessed by Western blot. ResultsCompared with the model group, ZH-13 dose-dependently reduced NO release from BV-2 cells under LPS stimulation (P<0.05, P<0.01). In the 20 μmol·L-1 ZH-13 treatment group, the mRNA expression levels of IL-1β, TNF-α, iNOS, and IL-6 were significantly reduced compared to the model group (P<0.05, P<0.01). In both the low- and high-dose ZH-13 groups, the expression of the inflammatory factor TNF-α and the phosphorylation of c-Jun N-terminal kinase (JNK) in the upstream MAPK pathway were significantly reduced (P<0.05). After stimulation with the JNK agonist anisomycin (Ani), both low- and high-dose ZH-13 treatment groups showed reduced phosphorylation of JNK proteins compared to the Ani-treated group (P<0.01). ConclusionThe sesquiterpene compound ZH-13 from Aquilariae Lignum Resinatum significantly ameliorates LPS-induced neuroinflammatory responses in BV-2 cells by inhibiting excessive JNK phosphorylation and reducing TNF-α expression. These findings elucidate the pharmacological substances and mechanisms underlying the sedative and calming effects of Aquilariae Lignum Resinatum.
9.Reconstruction of proximal tibial defect with infection and soft tissue defect after resection of giant cell tumour of tendon sheath with microsurgical technique: a case report
Lin TIAN ; Liuchao CUI ; Faxiang LI ; Yuzhong TAN ; Liangkun CHENG ; Yun ZHENG
Chinese Journal of Microsurgery 2025;48(4):465-469
On 25th September 2021, a patient was admitted in the Department of Hand Microsurgery, Chongqing Great Wall Orthopaedic Hospital with an infectious proximal tibia defect and soft tissue defect after the resection of a giant cell tumour of tendon sheath (GCTTS). After relevant examinations, it was found that there was a large bone defect at proximal medial tibia, with an extremely thin of medial tibial plateau. Size of bone defect was 8.0 cm×3.5 cm×3.0 cm, and soft tissue defect was 6.0 cm×8.0 cm. A phased surgery was planned: firstly, the internal fixation was removed with thorough debridement and then the cavity was filled by antibiotic bone cement for control of the infection; In the phase-Ⅱ surgery, a series of anterolateral thigh myocutaneous flap (the volume of the muscle flap was 6.0 cm×2.5 cm×2.0 cm, and the size of flap was 4.0 cm×9.0 cm) and an iliac bone flap were used and the cavity of medial proximal tibia was filled with calcium sulphate and anterolateral thigh muscle graft. A piece of iliac bone graft was transferred to support the tibial plateau, and the wound was covered by flap (the volume of the iliac bone fragment was 12.0 cm×5.0 cm×2.0 cm, and the size of flap was 4.0 cm×9.0 cm). At 3 years of postoperative follow-up, the fracture healed well, the cavity at medial proximal tibia was significantly reduced. Appearance of flaps was good with restored protective sensation. Function of the affected knee was close to normal with a score of 94 according to Lysholm Knee Scale.
10.Diagnostic efficacy of spectral CT virtual non-contrast imaging combined with iodine mapping for differenti-ating early postoperative intracerebral hemorrhage from contrast extravasation after endovascular therapy
Yun TAN ; Zhongyi KONG ; Ximing CAO ; Zhenbang WANG ; Junhui ZHENG ; Wei LUO
The Journal of Practical Medicine 2025;41(21):3449-3454
Objective To evaluate the diagnostic value of dual-layer spectral CT(DLCT)virtual non-contrast(VNC)imaging combined with iodine maps in differentiating early post-endovascular therapy(EVT)intracranial hemorrhage from contrast extravasation.Methods Retrospective analysis of 97 patients who underwent DLCT immediately after EVT was conducted.Taking 24-hour follow-up CT/MRI as the gold standard,patients were divided into hemorrhage and non-hemorrhage groups,and their clinical data were compared.VNC CT values and iodine concentration(IC)were measured.Spearman's rank correlation was used to analyze the relationship between VNC CT and IC values,and ROC curve analysis using R software to evaluate the diagnostic performance of VNC,iodine maps,and their combination.Results Among 97 patients,51(52.6%)showed no intracranial hyperdense lesions,while 46(47.4%)with abnormal densities were analyzed.Using 24-hour postoperative CT/MRI as reference stan-dard,among the 46 patients ultimately included in the analysis,38 cases(82.6%)were non-hemorrhagic and 8 cases(17.4%)hemorrhagic.No significant differences existed in age,sex,or treatment methods(all P>0.05).VNC CT values and IC showed significantly negative correlation(r=-0.537,P<0.01).ROC analysis revealed AUCs of 0.917(95%CI:0.786~0.999)for VNC,0.878(95%CI:0.719~0.999)for IC,and 0.919(95%CI:0.812~0.999)for the combination of the two(P<0.05 for combined vs.individual methods).Optimal thresholds were 53.6 HU for VNC and 0.605 mg/ml for IC.Based on the final analysis of 46 enrolled patients,the sensitivity of VNC,iodine map,and their combination in differentiating early cerebral hemorrhage from contrast extravasation was 88.9%,94.3%,and 91.4%,respectively;the specificity 94.3%,77.8%,and 88.9%,respectively;and the accuracy 90.9%,90.9%,and 93.2%,respectively.Conclusion The DLCT VNC-iodine map combination significantly im-proves differentiation between post-EVT hemorrhage and contrast extravasation,and it is recommended for routine clinical application.

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