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.Progress on the hazards and control of ship-borne vector organisms
Yun-chuan HE ; Hui-ling HAO ; Bin SUN ; Hong-yan LYU ; Deng-yong HOU ; Xiao-meng REN
Acta Parasitologica et Medica Entomologica Sinica 2026;33(2):144-151
In the context of burgeoning economic trade, there has been a remarkable increase in the number of ships travelling between domestic and international ports. Inbound and outbound cargo ships, cruise liners, ferries, and other types of vessels have emerged as critical vehicles for the dispersal of vector organisms and the spread of infectious disease agents. Public health concerns associated with these vessels represent a significant threat to human welfare. In this paper, we comprehensively review the hazards posed by vector organisms on ships, the latest advances in prevention and control strategies, and proposes appropriate countermeasures. The aim is to provide effective guidance for the prevention and control of public health security issues caused by ship-borne vector organisms.
3.Chemical constituents of Stelmatocrypton khasianum and their biological activity
Jing YU ; Guo-xu MA ; Cui-yun YIN ; Zhao-you DENG ; Yi-hang LI ; Er LI
Chinese Traditional Patent Medicine 2025;47(11):3645-3650
AIM To study the chemical constituents from Stelmatocrypton khasianum.(Benth.)Baill.and their in vitro anti-inflammatory activity and cytotoxic activity.METHODS Separation and purification were performed using thin layer chromatography,silica gel,semi-preparative HPLC and Sephadex LH-20,then the structures of obtained compounds were identified by physicochemical properties and spectral data.The in vitro anti-inflammatory activity was evaluated by RAW264.7 model,and the cytotoxic activity was determined by CCK-8 method.RESULTS Fifteen compounds were isolated and identified as 2α,3β,19α,23-tetrahydroxy-urs-12-en-28-oic acid-3-O-β-D-glucopyranosyl-28-O-β-D-glucopyranosyl ester(1),dalzienoside(2),benzyl-(6-O-α-L-rhanmopyranosyl)O-β-D-glucopyranoside(3),corchorusoside C(4),cyclo(Ala-Tyr)(5),thymidine(6),(4S,5S)-5-hydroxy-4-hexanolide(7),2-methylpyridin-3-ol(8),butyl isobutyl phthalate(9),bis-(2-ethylhexyl)terephthalate(10),p-hydroxybenzaldehyde(11),vanilic acid(12),salicylic acid(13),isovanilic acid(14),3-hydroxy-p-anisaldehyde(15).The IC50 values of compounds 1,2 and 4 for NO were(27.69±5.51),(25.82±3.58)and(23.35±7.09)μmol/L,respectively.The IC50 value of compound 1 on MCF-7 cells was(18.15±6.45)μmol/L.The IC50 values of compound 4 on MCF-7 and HCCC-9810 cells were(19.43±2.66)and(21.76±5.81)μmol/L,respectively.CONCLUSION Compounds 2-11 are isolated from S.khasianum for the first time.Compounds 1,2 and 4 exhibit good in vitro anti-inflammatory activity,and 1,4 have cytotoxic activity.
4.Guideline for the prevention of intraoperative acquired pressure injury in paraplegic patients with spinal cord injury (version 2025)
Aijun XU ; Shuixia LI ; Bo CHEN ; Mengyuan YE ; Lejiao LANG ; Ning NING ; Lin ZHANG ; Changqing LIU ; Zhonglan CHEN ; Weihu MA ; Weishi LI ; Xiaoning WANG ; Dongmei BIAN ; Jiancheng ZENG ; Xin WANG ; Yuan GAO ; Yaping CHEN ; Jiali CHEN ; Yun HAN ; Xiuting LI ; Yang ZHOU ; Xiaojing SU ; Qiong ZHANG ; Tianwen HUANG ; Ping ZHANG ; Hua LIN ; Xingling XIAO ; Ruifeng XU ; Fanghui DONG ; Bing HAN ; Luo FAN ; Yanling PEI ; Suyun LI ; Xiaoju TAN ; Rongchen GUO ; Yefang ZOU ; Xiaoyun HAN ; Junqin DING ; Yi WANG ; Shuhua DENG ; Jinli GUO ; Yinhua LIANG ; Yuan CEN ; Xiaoqin LIU ; Junru CHEN ; Haiyang YU ; Lunlan LI ; Ying REN ; Yunxia LI ; Jianli LU ; Ying YING ; Lan WEI ; Yin WANG ; Qinhong XU ; Yanqin ZHANG ; Yang LYU ; Shijun ZHANG ; Sui WENJIE ; Sanlian HU ; Shuhong YANG ; Guoqing LI ; Jingjing AN ; Baorong HE ; Leling FENG
Chinese Journal of Trauma 2025;41(6):530-541
Paraplegia caused by spinal cord injury is a serious neurological complication, for which surgery is currently the main treatment method. Due to different surgical approaches, patients are usually expected to maintain a passive prone position for a long time or switch between the supine and prone positions. Affected by multiple factors such as neurogenic sensory disorders, pathological changes in muscle tone and operative duration, the risk of intraoperative acquired pressure injury (IAPI) is significantly increased. Current clinical prevention strategies for IAPI in these patients predominantly focus on localized pressure relief during positioning, lacking systematic, standardized comprehensive prevention protocols or evidence-based guidelines. To address it, Department of Nursing, Orthopedics Branch, China International Exchange and Promotive Association for Medical and Health Care, Spinal Trauma Professional Committee, Orthopedics Branch, Chinese Medical Doctor Association, Nursing Group of Spine and Spinal Cord Professional Committee of Chinese Association of Rehabilitation Medicine organized experts in relevant fields to formulate Guideline for the prevention of intraoperative acquired pressure injury in paraplegic patients with spinal cord injury ( version 2025), based on evidence-based medical evidence and latest research results and clinical practice at home and abroad. Eleven recommendations were put forward from the aspects of preoperative risk assessment, intraoperative prevention strategies, postoperative handover and monitoring, and supportive mechanisms for IAPI prevention, aiming to standardize the prevention measures and management strategies of IAPI in paraplegic patients with spinal cord injury and accelerate the recovery of patients and improve the therapeutic effect.
5.Retrospective clinical analysis of eculizumab treatment for hematopoietic stem cell transplantation-associated thrombotic microangiopathy: a report of 11 cases
Xueyi LUO ; Rui MA ; Huifang WANG ; Lu BAI ; Yun HE ; Yuanyuan ZHANG ; Tingting HAN ; Daoxing DENG ; Yuhong CHEN ; Wei HAN ; Xiaohui ZHANG ; Lanping XU ; Yu WANG ; Xiaojun HUANG ; Yuqian SUN
Chinese Journal of Hematology 2025;46(5):431-436
Objective:To evaluate the efficacy of eculizumab in treating hematopoietic stem cell transplantation-associated thrombotic microangiopathy (TA-TMA) .Methods:This retrospective study included 11 patients who developed TA-TMA after allogeneic hematopoietic stem cell transplantation and subsequently received eculizumab treatment at Peking University People′s Hospital between June 2018 and May 2024. The incidence of TA-TMA, treatment details, and clinical outcomes were analyzed.Results:Among the 11 included patients [4 males, 7 females; median age: 29 years (range: 9-56) ], underlying diseases were severe aplastic anemia (SAA) in 5 patients, acute lymphoblastic leukemia (ALL) in 3 patients, and acute myeloid leukemia (AML) in 3 patients. The median time to TA-TMA diagnosis was 48 days post-transplantation (range: 4-213 days), and all patients met the diagnostic criteria for high-risk TA-TMA. The median interval from TA-TMA diagnosis to the initiation of eculizumab treatment was 12 days (range: 1-56 days). Patients received a median of 3 doses of eculizumab (range: 1-14). Ten of the 11 patients were assessed as having no response (NR) to eculizumab at the end of treatment or at death. One patient achieved a partial response (PR) but subsequently died after TA-TMA relapsed due to infection. At the last follow-up, all patients were either lost to follow-up or had died. The median follow-up duration was 88 days (range: 33-326 days), and the median time from TA-TMA diagnosis to the last follow-up was 31 days (range: 21-113 days) .Conclusion:Eculizumab demonstrated poor efficacy in this TA-TMA cohort. This might be attributable to the critical and complex condition of the patients, delayed initiation of eculizumab treatment, and insufficient dosage.
6.Epidemiological study on traditional Chinese medicine treatment for inflammatory bowel disease in Jiangsu Province from 2019 to 2023
Chujun NI ; Zexing LIN ; Haiyang JIANG ; Jie WU ; Peizhao LIU ; Jiaqi KANG ; Chengliang QIAN ; Haiqing LIU ; Liting DENG ; Huan YANG ; Chenling WU ; Yun ZHAO
Chinese Journal of Inflammatory Bowel Diseases 2025;09(4):318-325
Objective:To explore patterns of traditional Chinese medicine (TCM) use among patients with inflammatory bowel disease (IBD) in Jiangsu Province, China from 2019 to 2023.Methods:Using data from the IBD health data platform of the National Healthcare Big Data (Eastern) Center, a retrospective cohort study was conducted. We performed descriptive analyses on hospitalised patients diagnosed with IBD between 2019 and 2023, who received TCM treatment.Results:The study included 11 095 case records from 4 760 patients, with TCM diagnoses primarily indicating diarrhoea and abdominal pain. Ulcerative colitis (UC) accounted for 4 782 hospitalizations (3 103 patients), while Crohn's disease (CD) accounted for 6 313 hospitalizations (1 657 patients). Patient demographics showed a trend towards younger age and a higher proportion of males. Treatment utilisation was highest in southern Jiangsu compared with the central and northern regions. In terms of disease burden, all treatment costs showed a downward trend. In terms of external TCM therapies, UC patients tend to prefer plasters and enemas, while CD patients are more inclined to use acupuncture. Regarding herbal medicine, licorice, white atractylodes, and white peony root are commonly used single herbs for IBD patients.Conclusions:The number of IBD patients treated with TCM in Jiangsu Province has steadily increased from 2019 to 2023. It is important to identify effective TCM treatment methods to reduce the burden of patients.
7.Expert consensus on sensitive indicators for assessment of the quality of nursing in operating theatre
Yangxi SHEN ; Ping WANG ; Xiaojun CHEN ; Guiyuan LUO ; Fengqiu GONG ; Yun LI ; Chenhui DENG ; Yuqin SUN ; Qin GUO ; Jinyan LI ; Shuyan ZENG
Modern Clinical Nursing 2025;24(5):1-9
Objective To develop the Expert Consensus on Sensitive Indicators for Assessment of the Quality of Nursing in Operating Theatre and provide a scientific and practical guidance for improving the quality of nursing in operating theatre.Methods The writing team established by the Operating Room Nursing Professional Committee of Guangdong Nursing Association conducted systematic literature retrieval and screening,and used the updated clinical Guidelines for Research and Evaluation Ⅱ in UK 2017.AGREE Ⅱ and the evidence evaluation system of the Australian JBI(Joanna Briggs Institute,JBI)Evidence-Based Health Care Center evidence level system(2016 Edition)comprehensively analyzed the evidence related to the sensitive indicators for evaluating the quality of operating room nursing and the suggestions of the writing group members.The first draft was formed based on the three-dimensional quality evaluation theoretical framework of"structure-process-result".Through the Delphi method,after two rounds of expert consultations and members'votes,the first draft was deeply revised and improved.Results Based on the three-dimensional quality evaluation theoretical framework of"structure-process-outcome"proposed by American scholar Donabedian,the expert consensus finally included five primary indicators:basic nursing quality,quality indicators of patient safety,quality indicators of hospital infection control,quality indicators of medication and safety management,and quality indicators of specialised nursing in operating theatre.The secondary indicators consisted of one structural indicator(management of commonly used instrument and equipment in operating theatre)and 17 process indicators(e.g.,infusion and blood transfusion management,body temperature management,etc.).The tertiary indicators included 26 process indicators and 11 outcome indicators(e.g.,incidence of adverse reactions of infusion during surgery,incidence of intra-operative hypothermia,etc.).Conclusion The evidence-and guideline-based Expert Consensus on Sensitive Indicators for Assessment of the Quality of Nursing in Operating Theatre based on eviclence and guidelines was established through rigorous evidence-based methods.It is operational and practical,and offers theoretical support and practical guidance for the managers of operating theatre to improve the quality of nursing.
8.Three-dimensional finite element analysis of different material implants for replacing single missing anterior tooth
Zhaoxin XIA ; Yichen GAO ; Yuyao DENG ; Xia WANG ; Xiaorong LAN ; Yun HE ; Junliang CHEN
Chinese Journal of Tissue Engineering Research 2025;29(22):4687-4693
BACKGROUND:Implant restoration has become an important means to treat anterior tooth loss,and it is particularly important to select appropriate implant restoration materials.However,at present,there are some deficiencies in clinical implant materials,and researchers have been exploring suitable implant materials.OBJECTIVE:To compare the biomechanical characteristics of implants made of different materials in restoring single missing maxillary anterior teeth.METHODS:The cone beam CT data of a patient with single maxillary central incisor loss were imported into 3-matic software to establish a three-dimensional finite element analysis model of single maxillary anterior tooth loss.The model was then imported into Marc Mentat.Eight sets of implant restoration models were designed according to different implant materials(polyetheretherketone,titanium-zirconium alloy,titanium alloy,and zirconia,with the elastic modulus of the four materials increasing in sequence)and cancellous bone density(high density,low density)to simulate the stress conditions of the maxillary anterior teeth in centric occlusion.The total displacements and von Mises stresses of implants,cortical bone stresses and cancellous bone strains were compared and analyzed.RESULTS AND CONCLUSION:(1)The maximum displacement of the implant gradually decreased with the increase of the modulus of elasticity of the material,and the value of the maximum displacement of the implant in the polyetheretherketone group exceeded 10 μm;the implant stress was concentrated in the labial neck in the models.The maximum stress gradually increased with the increase of the modulus of elasticity of the implant material,among which,the zirconia-low density bone model had the highest stress,which was 21.31 MPa;the cortical bone stress was concentrated in the cortical bone at the junction of the implant with the labial side,and the polyetheretherketone-low density bone group had the highest value,which was 29.90 MPa.(2)From the biomechanical point of view,titanium-zirconium alloy,titanium alloy,and zirconia can be used as implant materials for restoration of a single missing anterior tooth,and implant displacement is one of the common problems during implant restorations,but pure polyetheretherketone material may cause excessive implant displacement in implant restorations of anterior areas,causing implant failure.
9.Alisol A 24-acetate ameliorates cerebral ischemia reperfusion injury in brain micro vascular endothelial cells via miR-98-5p/TRPM2
Wei WEI ; Hui-hong LI ; Pei-tao XU ; Da-mei TAO ; Yun-fei DENG ; Zeng-tu ZHAN
Chinese Pharmacological Bulletin 2025;41(4):695-702
Aim To explore the underlying molecular mechanism of Alisol A 24-acetate(24A)in improving oxygen-glucose deprivation/reoxygenation(OGD/R)injury in brain microvascular endothelial cells(BMECs)and its correlation with miR-98-5p/transi-ent receptor potential melastatin-2(TRPM2).Meth-ods The ischemia-reperfusion injury in brain micro-vascular endothelial cells(BMECs)was established u-sing bEnd.3 cells subjected to 8 h of oxygen-glucose deprivation followed by 16 h of re-oxygenation.The cells were intervened by miR-98-5p mimics and/or 18.77 μmol·L-1 24A for 24 h and divided into the control group,OGD/R group,OGD/R+24A group,OGD/R+24A+miR-98-5p mimics group and OGD/R+miR-98-5p mimics group.The mRNA levels of miR-98-5p and TRPM2 were detected by qPCR.IL-1 β and TNF-α levels were detected by ELISA.The expression levels of TRPM2,p-AKT,p-GSK3 β,AKT,GSK3 β,Bcl-2,Bax,ZO-1,Occludin,Claudin-5 were detected by Western blot.Apoptosis and reactive oxygen species(ROS)levels were detected by flow cytometry.The targeting relationship between miR-98-5p and TRPM2 was verified using dual luciferase assay.Results Compared with the control group,the apoptosis of OGD/R group was obvious,Bcl-2/Bax decreased,ZO-1,Occludin,Claudin-5 decreased,IL-1 β,TNF-α and ROS increased,miR-98-5p,p-AKT/AKT,p-GSK3β/GSK3β decreased but TRPM2 increased.But com-pared with the OGD/R group,except the control group,the other three groups showed the opposite trend in the above aspects;compared with the OGD/R+24A group,OGD/R+24A+miR-98-5p mimics group showed decreased apoptosis,decreased degradation of ZO-1,Occludin and Claudin-5,and decreased inflam-mation and ROS.miR-98-5p,p-AKT/AKT,p-GSK3β/GSK3β increased and TRPM2 decreased.However,compared with the OGD/R+24A+miR-98-5p mimics group,the OGD/R+miR-98-5p mimics group reversed this trend.Dual luciferase confirmed that miR-98-5p targeted regulation of TRPM2.Conclusion 24A in-hibits the expression of TRPM2 in BMECs through miR-98-5p,regulates AKT/GSK3β signal pathway,re-duces OGD/R inflammation and oxidative stress-medi-ated apoptosis,prevents the degradation of ZO-1,Oc-cludin and Claudin-5,and improves BBB permeability.
10.Fulminant type 1 diabetic ketoacidosis due to serplulimab
Xu DENG ; Yan DAI ; Tingting LI ; Yun GAO ; Tiantian DAI ; Mingqin GE ; Shilong WANG
Adverse Drug Reactions Journal 2025;27(7):440-442
A 57-year-old female patient with small cell lung cancer was treated with serplulimab (100 mg by intravenous infusion on the first day, 21 days as a cycle). After the 10th cycle of treatment, the patient suddenly presented drowsiness, shortness of breath, dry mouth, nausea, vomiting, fatigue and other symptoms. Laboratory tests showed fasting blood glucose 38.9 mmol/L, glycosylated hemoglobin 7.9%, serum C peptide 0.2 μg/L, pH value 7.05, anion gap 31 mmol/L, anti-islet cell antibody 36 kU/L, anti-glutamic acid decarboxylase antibody 131 kU/L, urine ketone body (+++). The patient was diagnosed with fulminant type 1 diabetic ketoacidosis, which was considered to be related to serplulimab. The drug was stopped, and insulin, rehydration, correction of acid-base imbalance, and other treatments were given. After 3 days, the patient′s consciousness returned to normal. After 12 days, her breathing was stable, dry mouth, nausea, vomiting and other symptoms were relieved. Laboratory tests showed random blood glucose 13.6 mmol/L, pH value 7.44, anion gap 6 mmol/L, and urine ketone body negative. After 26 days, her random blood glucose was controlled at 10.0-12.0 mmol/L.


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