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Porta Hepatis Tuberculous Lymphadenopathy: Clinical and Imaging Features of 10 Cases

Infectious Microbes & diseases 2024年第1期6卷 29-35页

作者： Wenjuan Hu Ming Hu Jiafeng Xia Shengyue Chen Xiaomeng Li Chengmin Sheng Wanru Guo Xuewen Feng Yanwan Shangguan Bin Lu Yunzhen Shi Junke Qiu Kaijin Xu State Key Laboratory for Diagnosis and Treatment of Infectious diseases National Clinical Research Center for Infectious DiseasesCollaborative Innovation Center for Diagnosis and Treatment of Infectious DiseasesThe First Affiliated HospitalZhejiang University School of MedicineHangzhouChina Department of Cardiology the Second Affiliated Hospital of Dalian Medical UniversityDalianChina Department of Infectious diseases Affiliated Dongyang Hospital of Wenzhou Medical UniversityDongyangChina Department of Tuberculosis Intensive Care unit Hangzhou Red Cross HospitalHangzhouChina

Porta hepatis tuberculous lymphadenopathy(TL)is rare,and the clinical and imaging manifestations often mimic *** offer a bet-ter understanding of porta hepatis TL and thus improve its diagnosis and treatment,here,we retrospectively reviewed and analyzed 10 porta hepatis TL cases between May 2017 and November *** this study,porta hepatis TL occurred predominantly in women(70%),with a mean age of 43.1 years(range,16-70 years).Among the patients,60%were initially suspected of malignancy(metastatic lymphadenopathy and lymphoma).The clinical manifestations were nonspecific,such as fever and weight loss,without overt abdominal *** patients showed high concentrations of serum C-reactive protein,erythrocyte sedimentation rate,alkaline phosphatase,γ-glutamyl transpeptidase and carbohydrate antigen *** rim-like enhancement was a characteristic finding on contrast-enhanced CT for all the *** examination confirmed the diagnosis in 90%of patients with caseous granulo-matous inflammation and positive results of Xpert MTB/RIF,quantitative real-time PCR and/or metagenomic next-generation sequenc-ing *** patients underwent antituberculosis therapy with a median treatment duration of 13.5 months(range,1-43 months).They all showed satisfactory therapeutic responses with improved symptoms and resolution on imaging after *** examination by biopsy remains the primary diagnostic method.A combination of the characteristic epidemiologic,clinical,imaging fea-tures and lesion biopsy for histopathology are essential for the diagnosis and treatment of TL.

关键词： porta hepatis tuberculous lymphadenopathy misdiagnosis tumor mimicking

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Heparin-induced thrombocytopenia thrombosis after pancreaticoduodenectomy without definitive prophylactic or therapeutic use of heparin: a case report

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胰腺病学杂志(英文) 2024年第2期07卷 164-166页

作者： Wang Mengyi Huang Nan Liu Qiaofei Liao Quan Department of General Surgery Key Laboratory of Research in Pancreatic Tumor State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Science and Peking Union Medical College# Shuaifuyuan Dongcheng District Beijing China

Heparin-induced thrombocytopenia thrombosis (HITT) is a rare and potentially life-threatening complication after abdominal surgery, and it always occurs after the prophylactic or therapeutic use of heparin. HITT after pancreaticoduodenectomy (PD) has not been reported before. Herein, we reported a case of HITT after PD without prophylactic or therapeutic use of heparin. A 74-year-old female patient who suffered resectable pancreatic head cancer was transferred to our center for surgery. An open PD procedure was performed, and the operation was smooth. No heparin was used after surgery. Nine days after surgery, the platelet sharply declined to 48 × 109/L (100-350), and the D-dimer soared up to 33.56 mg/L (0-0.55). Ultrasound examination showed vein thrombosis in both the lower limb and the right upper limb. HIT-antibody was 6.3 U/mL (0-0.6). The diagnosis of HITT was confirmed. Fondaparinux was used. On postoperative day (POD) 23, the platelet recovered to the normal range. On POD 27, she was discharged without thromboembolism or active bleeding, and oral rivaroxaban was prescribed. One month after discharge, the platelet remained normal, and she did not complain of discomfort.

关键词： Heparin-induced thrombocytopenia Pancreatic cancer Pancreaticoduodenectomy Perioperative anticoagulation

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Risk stratification for radioactive iodine refractoriness using molecular alterations in distant metastatic differentiated thyroid cancer

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Chinese Journal of Cancer Research 2024年第1期36卷 25-35页

作者： Zhuanzhuan Mu Xin Zhang Dongquan Liang Jugao Fang Ge Chen Wenting Guo Di Sun Yuqing Sun Zhentian Kai Lisha Huang Jun Liang Yansong Lin Department of Nuclear Medicine State Key Laboratory of Complex Severe and Rare DiseasesPeking Union Medical College(PUMC)HospitalChinese Academy of Medical Sciences&PUMCBeijing 100730China Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine Beijing 100730China Pepperdine University Graduate School of Education and Psychology Los Angeles 90045USA Department of Head and Neck Surgery Affiliated Beijing Tongren HospitalCapital Medical UniversityBeijing 100730China Department of General Surgery State Key Laboratory of Complex Severe and Rare DiseasesPeking Union Medical College(PUMC)HospitalChinese Academy of Medical Sciences&PUMCBeijing 100730China Central Laboratory State Key Laboratory of Complex Severe and Rare DiseasesPeking Union Medical College(PUMC)HospitalChinese Academy of Medical Sciences&PUMCBeijing 100730China Department of Bioinformatics Zhejiang Shaoxing Topgen Biomedical Technology Co.LtdShanghai 201321China Department of Medicine Zhejiang Shaoxing Topgen Biomedical Technology Co.LtdShanghai 201321China Department of Oncology Peking University International HospitalBeijing 102206China

Objective: Patients with radioactive iodine-refractory differentiated thyroid cancer(RAIR-DTC) are often diagnosed with delay and constrained to limited treatment options. The correlation between RAI refractoriness and the underlying genetic characteristics has not been extensively ***: Adult patients with distant metastatic DTC were enrolled and assigned to undergo next-generation sequencing of a customized 26-gene panel(Thyro Lead). Patients were classified into RAIR-DTC or non-RAIR groups to determine the differences in clinicopathological and molecular characteristics. Molecular risk stratification(MRS) was constructed based on the association between molecular alterations identified and RAI refractoriness, and the results were classified as high, intermediate or low ***: A total of 220 patients with distant metastases were included, 63.2% of whom were identified as RAIRDTC. Genetic alterations were identified in 90% of all the patients, with BRAF(59.7% vs. 17.3%), TERT promoter(43.9% vs. 7.4%), and TP53 mutations(11.5% vs. 3.7%) being more prevalent in the RAIR-DTC group than in the non-RAIR group, except for RET fusions(15.8% vs. 39.5%), which had the opposite pattern. BRAF and TERT promoter are independent predictors of RAIR-DTC, accounting for 67.6% of patients with RAIR-DTC. MRS was strongly associated with RAI refractoriness(P<0.001), with an odds ratio(OR) of high to low MRS of 7.52 [95%confidence interval(95% CI), 3.96-14.28;P<0.001] and an OR of intermediate to low MRS of 3.20(95% CI,1.01-10.14;P=0.041).Conclusions: Molecular alterations were associated with RAI refractoriness, with BRAF and TERT promoter mutations being the predominant contributors, followed by TP53 and DICER1 mutations. MRS might serve as a valuable tool for both prognosticating clinical outcomes and directing precision-based therapeutic interventions.

关键词： Differentiated thyroid cancer distant metastases genetic alterations RAI refractoriness molecular risk stratification

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Potential risk genes for primary Sjogren's syndrome from a meta-analysis by linear regression and random forest classification

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Genes & diseases 2024年第3期11卷 128-131页

作者： Tomas Cerdo Teresa Torres Moral Maimonides Biomedical Research Institute of Cordoba(IMIBIC) Reina Sofia University HospitalUniversity of CordobaCordoba 14004Spain Centre for Rheumatology Research Division of MedicineUniversity College LondonLondon W1T 4JFUK Faculty of Computer Sciences Multimedia and TelecommunicationUniversitat Oberta de CatalunyaBarcelona 08018Spain Dermatology Department Melanoma UnitAugust Pi i Sunyer Biomedical Research Institute(IDIBAPS)and Hospital ClinicBarcelona 08036Spain Center for Networked Biomedical Research on rare diseases(CIBERER) Carlos II Health InstituteMadrid 28029Spain

Primary Sjogren's syndrome(psS)is one of the most widespread autoimmune diseases with unknown origin,characterized by a lymphocytic infiltrate of the exocrine glands and the production of anti-SSA/Ro and anti-SSB/La antibodies that cause dysfunction and destruction mainly of salivary and lachrymal glands,leading to dry eyes and dry *** the absence of a standardized evidence-based screening tool to decide which patients with dry eye must be referred for study of this pathology.

关键词： Sjogren lymphocytic salivary

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Vaccination against coronavirus disease 2019 in patients with pulmonary hypertension:A national prospective cohort study

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Chinese Medical Journal 2024年第6期137卷 669-675页

作者： Xiaohan Wu Jingyi Li Jieling Ma Qianqian Liu Lan Wang Yongjian Zhu Yue Cui Anyi Wang Cenjin Wen Luhong Qiu Yinjian Yang Dan Lu Xiqi Xu Xijie Zhu Chunyan Cheng Duolao Wang Zhicheng Jing Department of Cardiology State Key Laboratory of Complex Severe and Rare DiseasesPeking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China Department of Echocardiography National Center for Cardiovascular DiseasesFuwai HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100037China Department of Pulmonary Circulation Shanghai Pulmonary HospitalTongji University School of MedicineShanghai 200433China Department of Cardiology The First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan 450052China Department of Clinical Sciences Liverpool School of Tropical MedicineLiverpoolUnited Kingdom

Background:Coronavirus disease 2019(COVID-19)has potential risks for both clinically worsening pulmonary hypertension(PH)and increasing ***,the data regarding the protective role of vaccination in this population are still *** study aimed to assess the safety of approved vaccination for patients with ***:In this national prospective cohort study,patients diagnosed with PH(World Health Organization[WHO]groups 1 and 4)were enrolled from October 2021 to April *** primary outcome was the composite of PH-related major adverse *** used an inverse probability weighting(IPW)approach to control for possible confounding factors in the baseline characteristics of ***:In total,706 patients with PH participated in this study(mean age,40.3 years;mean duration after diagnosis of PH,8.2 years).All patients received standardized treatment for PH in accordance with guidelines for the diagnosis and treatment of PH in *** them,278 patients did not receive vaccination,whereas 428 patients completed the vaccination *** of the participants were infected with COVID-19 during our study ***,398 patients received inactivated severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)vaccine,whereas 30 received recombinant protein subunit *** adjusting for baseline covariates using the IPW approach,the odds of any adverse events due to PH in the vaccinated group did not statistically significantly increase(27/428[6.3%]vs.24/278[8.6%],odds ratio=0.72,P=0.302).Approximately half of the vaccinated patients reported at least one post-vaccination side effects,most of which were mild,including pain at the injection site(159/428,37.1%),fever(11/428,2.6%),and fatigue(26/428,6.1%).Conclusions:COVID-19 vaccination did not significantly augment the PH-related major adverse events for patients with WHO groups 1 and 4 PH,although there were some tolerable side effects.A large-scale randomized controlled trial is warr

关键词： Coronavirus disease COVID-19 Pulmonary hypertension Vaccination

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Alpha-gal syndrome

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Chinese Medical Journal 2024年第10期137卷 1234-1236页

作者： Mengyuan Zhan Shaohui Chen Tengda Xu Jia Yin Hao Li Zhouxian Pan Ning Cui Liping Wen Department of Allergy Peking Union Medical College HospitalPeking Union Medical CollegeChinese Academy of Medical SciencesBeijing 100730China State Key Laboratory of Complex Severe and rare diseases Beijing 100730China National Clinical Research Center for Dermatologic and Immunologic diseases(NCRC-DID) Beijing 100730China Department of Health Care Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijing 100730China Department of Anesthesiology Chinese Academy of Medical College and Peking Union Medical College HospitalBeijing 100730China State Key Laboratory of Pathogen and Biosecurity Beijing Institute of Microbiology and EpidemiologyBeijing Key Laboratory of Vector Borne and Natural Focus Infectious DiseasesBeijing 100071China The No.154 Hospital XinyangHenan 464000China

To the Editor:Meat is an important source of protein and could be roughly classied into red and white *** meat,which encompasses poultry,seafood such assh and shrimp,and reptiles,etc.,is characterized by its pale color when *** meat is basically mammalian meat,including domestic animal meats,such as pork,beef,lamb,and wild animal meat in certain regions,such as kangaroos,seals,and *** mammalian meat appears red due to the presence of myoglobin in skeletal *** clinical presentations of mammalian meat allergy comprise two types of allergic reactions,immediatetype and delayed-type,although both are mediated by immunoglobulin E(IgE).The immediate-type hypersensitivity manifests as allergic symptoms occurring shortly after ingestion of red meat and is primarily triggered by serum albumin and immunoglobulin which are the major allergenic *** is the classic pattern of food *** delayed-type hypersensitivity is characterized by delayed onset of allergic symptoms 2-6 h after red meat ingestion and is mediated by oligosaccharideα-gal-specic IgE which is a recently discoveried pattern of food allergy.

关键词： allergic Alpha pattern

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rare dentin defects:Understanding the pathophysiological mechanisms of COLXVA1 mutations

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Genes & diseases 2024年第5期11卷 1-5页

作者： Isaac Maximiliano Bugueno Tristan Rey Alexandra Jimenez-Armijo Marzena Kawczynski Naji Kharouf Marie-Cecile Maniere Yann Herault Agnes Bloch-Zupan Virginie HaushalterLaugel Institute of Genetics and Molecular and Cellular Biology(IGBMC) CNRS-UMR7104INSERM U1258Universitede StrasbourgIllkirch 67400France Faculty of Dentistry Universitede StrasbourgStrasbourg 67000France Institute of Advanced Studies(USIAS) Universitede StrasbourgStrasbourg 67045France Reference Center for rare Oral and Dental diseases(CRMR-O-rares) Oral Medicine and Surgery DepartmentHopitaux Universitaires de Strasbourg(HUS)Strasbourg 67000France rare diseases Health Network“TETE COU”&European Reference Network ERN CRANIO Strasbourg 67000France “Institut de Genetique Medicale d’Alsace” Laboratory of Genetic DiagnosticHopitaux Universitaires de Strasbourg(HUS)Strasbourg 67000France Biomaterials and Bioengineering Laboratory Inserm UMR_S 1121Strasbourg 67000France Orofacial Development and Regeneration Faculty of MedicineInstitute of Oral BiologyCentre of Dental MedicineUniversity of ZurichZurich 8032Switzerland

Dentin is a mineralized tissue with a chemical composition similar to bone but with a higher mineralized density and *** constitutes the central structure of the tooth between the internal pulp and external enamel toward the oral cavity or cementum toward the underlying *** dentin defects occur in a variety of rare genetic *** can manifest as“isolated”occurrences such as in dentinogenesis imperfecta(DI)or dentin dysplasia(DD)or can be associated with other symptoms in diseases such as osteogenesis imperfecta,Goldblatt syndrome,microcephalic osteodysplastic primordial dwarfism type II,among others.

关键词： diseases den rigidity

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Association between ICU quality and in-hospital mortality of V-V ECMO-supported patients—the ECMO quality improvement action(EQIA)study:a national cohort study in China from 2017 to 2019

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Frontiers of Medicine 2024年第2期18卷 315-326页

作者： Wei Cheng Jieqing Chen Xudong Ma Jialu Sun Sifa Gao Ye Wang Longxiang Su Lu Wang Wei Du Huaiwu He Yujie Chen Zunzhu Li Qi Li Jianhua Sun Hongbo Luo Jinbang Liu Guangliang Shan Bing Du Yanhong Guo Dawei Liu Chang Yin Xiang Zhou on behalf of the China National Critical Care Quality Control Center-the ECMO quality improvement action(EQIA)study Department of Critical Care Medicine State Key Laboratory of Complex Severe and Rare DiseasesPeking Union Medical College HospitalChinese Academy of Medical Science and Peking Union Medical CollegeBeijing100730China Information Center Department/Department of Information Management State Key Laboratory of Complex Severe and Rare DiseasesPeking Union Medical College HospitalPeking Union Medical College&Chinese Academy of Medical SciencesBeijing100730China Department of Medical Administration National Health Commission of the People’s Republic of ChinaBeijing100044China School of Population Medicine and Public Health Chinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing100730China Department of Epidemiology and Biostatistics Institute of Basic Medicine SciencesChinese Academy of Medical Sciences(CAMS)&School of Basic MedicinePeking Union Medical CollegeBeijing100730China National Institute of Hospital Administration National Health Commission of the People’s Republic of ChinaBeijing100044China 不详

This cohort study was performed to explore the influence of intensive care unit(ICU)quality on in-hospital mortality of veno-venous(V-V)extracorporeal membrane oxygenation(ECMO)-supported patients in *** study involved all V-V ECMO-supported patients in 318 of 1700 tertiary hospitals from 2017 to 2019,using data from the National Clinical Improvement System and China National Critical Care Quality Control *** quality was assessed by quality control indicators and capacity *** the 2563 V-V ECMO-supported patients in 318 hospitals,a significant correlation was found between ECMO-related complications and *** reintubation rate within 48 hours after extubation and the total ICU mortality rate were independent risk factors for higher in-hospital mortality of V-V ECMO-supported patients(cutoff:1.5%and 7.0%;95%confidence interval:1.05–1.48 and 1.04–1.45;odds ratios:1.25 and 1.23;P=0.012 and P=0.015,respectively).Meanwhile,the V-V ECMO center volume was a protective factor(cutoff of≥50 cases within the 3-year study period;95%confidence interval:0.57–0.83,odds ratio:0.69,P=0.0001).The subgroup analysis of 864 patients in 11 high-volume centers further strengthened these ***,ICU quality may play an important role in improving the prognosis of V-V ECMO-supported patients.

关键词： veno-venous extracorporeal membrane oxygenation in-hospital mortality high-volume centers quality control intensive care unit capacity parameters

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Internal m^(6)A and m^(7)G RNA modifications in hematopoietic system and acute myeloid leukemia

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Chinese Medical Journal 2024年第9期137卷 1033-1043页

作者： Xiaoxu Zhang Yanni Ma Jia Yu Rui Su Xiaoshuang Wang State Key Laboratory of Common Mechanism Research for Major diseases State Key Laboratory for ComplexSevereand Rare DiseasesInstitute of Basic Medical SciencesChinese Academy of Medical Sciences/Peking Union Medical CollegeBeijing 100005China Department of Systems Biology Beckman Research Institute of City of HopeMonroviaCA 91016USA The Institute of Blood Transfusion Chinese Academy of Medical Sciences/Peking Union Medical CollegeChengduSichuan 610052China Key Laboratory of RNA and Hematopoietic Regulation Chinese Academy of Medical SciencesBeijing 100005China Department of Biochemistry and Molecular Biology Chinese Academy of Medical SciencesSchool of Basic Medicine Peking Union Medical CollegeBeijing 100005China

Epitranscriptomics focuses on the RNA-modification-mediated post-transcriptional regulation of gene *** past decade has witnessed tremendous progress in our understanding of the landscapes and biological functions of RNA modifications,as prompted by the emergence of potent analytical *** hematopoietic system provides a lifelong supply of blood cells,and gene expression is tightly controlled during the differentiation of hematopoietic stem cells(HSCs).The dysregulation of gene expression during hematopoiesis may lead to severe disorders,including acute myeloid leukemia(AML).Emerging evidence supports the involvement of the mRNA modification system in normal hematopoiesis and AML pathogenesis,which has led to the development of small-molecule inhibitors that target N6-methyladenosine(m^(6)A)modification machinery as ***,we summarize the latest findings and our most up-to-date information on the roles of m^(6)A and N7-methylguanine in both physiological and pathological conditions in the hematopoietic ***,we will discuss the therapeutic potential and limitations of cancer treatments targeting m^(6)A.

关键词： N6-methyladenosine N7-methylguanine Hematopoiesis Acute myeloid leukemia Therapeutic target RNA modifications

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Elevated peripheral levels of receptor-interacting protein kinase 1(RIPK1)and IL-8 as biomarkers of human amyotrophic lateral sclerosis

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Signal Transduction and Targeted Therapy 2024年第1期9卷 271-278页

作者： Jun Wei Min Li Zhi Ye Xinqian Hu Xiaoyan He Jia Wang Gaofeng Chen Chengyu Zou Daichao Xu Hongbing Zhang Junying Yuan Yunhong Zha Institute of Neural Regeneration and Repair and Department of Neurology The First College of Clinical Medical ScienceYichang Central HospitalCollege of Basic Medical ScienceChina Three Gorges UniversityHubei Province Clinical Medical Research Center for Rare Diseases of Nervous SystemYichang 443000China Interdisciplinary Research Center on Biology and Chemistry Shanghai Institute of Organic ChemistryChinese Academy of SciencesShanghai Key Laboratory of Aging StudiesShanghai 201210China State Key Laboratory of Common Mechanism Research for Major diseases Haihe Laboratory of Cell EcosystemDepartment of PhysiologyInstitute of Basic Medical Sciences and School of Basic MedicineChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina

Amyotrophic lateral sclerosis(ALS)is a devastating fatal neurodegenerative disease with no ***-interacting protein kinase 1(RIPK1)has been proposed to mediate pathogenesis of *** has been identified as an old drug that can also inhibit RIPK1 *** conducted a drug-repurposing biomarker study of primidone as a RIPK1 inhibitor using SOD1G93A mice and ALS ***1G93A mice treated with primidone showed significant delay of symptomatic onset and improved motor ***-hundred-sixty-two ALS participants dosed daily with primidone(62.5 mg)completed 24-week follow-up.A significant reduction was showed in serum levels of RIPK1 and IL-8,which were significantly higher in ALS patients than that of healthy controls(Pdiseases may also be considered,since the activation of RIPK1 has been implicated in mediating a variety of inflammatory diseases including COVID-19-associated cytokine release syndrome(CRS).(ChiCTR2200060149).

关键词： RIPK1 diseases Serum

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