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Clinical manifestations,diagnosis and long-term prognosis of adult autoimmune enteropathy:Experience from peking union medical college hospital

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World Journal of Gastroenterology 2024年第19期30卷 2523-2537页

作者： Mu-Han Li Ge-Chong Ruan Wei-Xun Zhou Xiao-Qing Li Sheng-Yu Zhang Yang Chen Xiao-Yin Bai Hong Yang Yu-Jie Zhang Peng-Yu Zhao Ji Li Jing-Nan Li Department of Gastroenterology Chinese Academy of Medical Sciences&Peking Union Medical College HospitalBeijing 100730China Department of Pathology Chinese Academy of Medical Scinences&Peking Union Medical College HospitalBeijing 100730China Department of Pathology Tianjin First Central HospitalNankai UniversityTianjin 300192China Affairs Office Chinese Academy of Medical Sciences&Peking Union Medical College Hospital(West campus)Beijing 100032China

BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE *** To improve overall understanding of this disease’s diagnosis and *** We retrospectively analyzed the clinical,endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023,whose diagnosis was based on the 2007 diagnostic *** Diarrhea in AIE patients was characterized by secretory *** common endoscopic manifestations were edema,villous blunting and mucosal hyperemia in the duodenum and *** blunting(100%),deep crypt lymphocytic infiltration(67%),apoptotic bodies(50%),and mild intraepithelial lymphocytosis(69%)were observed in the duodenal ***,there were other remarkable abnormalities,including reduced or absent goblet cells(duodenum 94%,ileum 62%),reduced or absent Paneth cells(duodenum 94%,ileum 69%)and neutrophil infiltration(duodenum 100%,ileum 69%).Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte *** patients received glucocorticoid therapy as the initial medication,of which 14/16 patients achieved a clinical response in 5(IQR:3-20)*** were administered to 9 patients with indications of steroid dependence(6/9),steroid refractory status(2/9),or intensified maintenance medication(1/9).During the median of 20.5 months of followup,2 patients died from multiple organ failure,and 1 was diagnosed with non-Hodgkin’s *** cumulative relapse-free survival rates were 62.5%,55.6%and 37.0%at 6 months,12 months and 48 months,*** Certain histopathological findings,including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies,might be potential diagnostic criteria for adult *** long-term prognosis is still unsatisfac

关键词： Autoimmune enteropathy Clinical manifestations Diagnostic criteria Pathological features Long-term prognosis

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

Transcriptomic and machine learning analyses identify hub genes of metabolism and host immune response that are associated with the progression of breast capsular contracture

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Genes & Diseases 2024年第3期11卷 427-437页

作者： Yukun Mao Xueying Hou Su Fu Jie Luan Breast Plastic and Reconstructive surgery Center Plastic Surgery HospitalChinese Academy of Medical SciencesPeking Union Medical CollegeBeijing 100144China

Capsular contracture is a prevalent and severe complication that affects the post-operative outcomes of patients who receive silicone breast *** present,prosthesis replacement is the major treatment for capsular contracture after both breast augmentation procedures and breast reconstruction following breast cancer ***,the mecha-nism(s)underlying breast capsular contracture remains *** study aimed to identify the biological features of breast capsular contracture and reveal the potential underlying mechanism using RNA *** tissues from 12 female patients(15 breast capsules)were divided into low capsular contracture(LCC)and high capsular contracture(HCC)groups based on the Baker ***,41 lipid metabolism-related genes were identified through enrichment analysis,and three of these genes were identified as candidate genes by SVM-RFE and LASSO *** then compared the proportions of the 22 types of im-mune cells between the LCC and HCC groups using a CIBERSORT analysis and explored the Cor-relation between the candidate hub features and immune ***,PRKAR2B was positively correlated with the differentially clustered immune cells,which were M1 macro-phages and follicular helper T cells(area under the ROC=0.786).In addition,the expression of PRKAR2B at the mRNA or protein level was lower in the HCC group than in the LCC *** molecular mechanisms were identified based on the expression levels in the high and low PRKAR2B *** findings indicate that PRKAR2B is a novel diagnostic biomarker for breast capsular contracture and might also influence the grade and progression of capsularcontracture.

关键词： Biomarker Breastcapsular contracture Immune infiltration Machinelearning algorithms PRKAR2B

Type I interferon pathway in pediatric systemic lupus erythematosus

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World Journal of Pediatrics 2024年第7期20卷 653-668页

作者： Yu Zhou Hong‑Mei Song Department of Pediatrics Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijingChina State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College HospitalBeijingChina

Background The role of type I interferon(IFN-I)signaling in systemic lupus erythematosus(SLE)has been well ***,unanswered questions remain regarding the applicability of these findings to pediatric-onset *** aim of this review is to provide an overview of the novel discoveries on IFN-I signaling in pediatric-onset *** sources A literature search was conducted in the PubMed database using the following keywords:“pediatric systemic lupus erythematosus”and“type I interferon”.Results IFN-I signaling is increased in pediatric SLE,largely due to the presence of plasmacytoid dendritic cells and pathways such as cyclic GMP-AMP synthase–stimulator of interferon genes–TANK-binding kinase 1 and Toll-like receptor(TLR)4/*** extracellular traps and oxidative DNA damage further stimulate IFN-I *** variants in IFN-I-related genes,such as IFN-regulatory factor 5 and tyrosine kinase 2,are linked to SLE susceptibility in pediatric *** addition,type I interferonopathies,characterized by sustained IFN-I activation,can mimic SLE symptoms and are thus important to *** on interferonopathies also contribute to exploring the pathogenesis of *** IFN-I activation is crucial for SLE diagnosis and *** IFNstimulated gene expression and serum IFN-α2 levels are common *** cytometry markers such as CD169 and galectin-9 are promising ***-IFN therapies,such as sifalimumab and anifrolumab,show promise in adult patients with SLE,but their efficacy in pediatric patients requires further *** kinase inhibitors are another treatment option for severe pediatric SLE *** This review presents an overview of the IFN-I pathway in pediatric *** the intricate relationship between IFN-I and pediatric SLE may help to identify potential diagnostic markers and targeted therapies,paving the way for improved patient care and outcomes.

关键词： Interferonopathies Pediatric systemic lupus erythematosus Type I interferon

Pancreatic ductal adenocarcinoma chemoresistance:From metabolism reprogramming to novel treatment

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Chinese medical Journal 2024年第4期137卷 408-420页

作者： Jingcheng Zhang Yutong Wang Lejunzi Wang Lei You Taiping Zhang Department of general surgery Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China peking union medical college Chinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China Department of Anaesthesia Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China Clinical Immunology Centre Chinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China

As pancreatic cancer(PC)is highly malignant,its patients tend to develop metastasis at an early stage and show a poor response to conventional ***-line chemotherapies for PC,according to current guidelines,include fluoropyrimidine-and gemcitabine-based *** research on drug resistance has shown that biochemical metabolic aberrations in PC,especially those involving glycolysis and glutamine metabolism,are highly associated with ***,lipid metabolism is a major factor in ***,emerging compounds that target these key metabolic pathways have the potential to overcome *** review summarizes how PC develops chemoresistance through aberrations in biochemical metabolism and discusses novel critical targets and pathways within cancer metabolism for new drug research.

关键词： Pancreatic cancer Drug resistance Drug development Signal transduction

Efficacy of Raw Corn Starch in Insulinoma-Related Hypoglycemia:A Promising Supportive Therapy

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Chinese medical Sciences Journal 2024年第2期39卷 102-110页

作者： Rong-Rong Li Wei Chen Xin-Hua Xiao Miao Yu Fan Ping Lian Duan Department of Clinical Nutrition Beijing Key Laboratory of the Innovative Development of Functional Staple and the Nutritional Intervention for Chronic DiseaseChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100730China Department of Endocrinology Peking Union Medical College HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100730China

Objective To investigate the efficacy of raw corn starch(RCS)in clinical management of insulinoma-induced *** We retrospectively collected clinical data of insulinoma patients who received RCS-supplemented diet preoperatively,and analyzed the therapeutic effects of the RCS intervention on blood glucose control,weight change,and its adverse *** The study population consisted of 24 cases of insulinoma patients,7 males and 17 females,aged 46.08±14.15 *** RCS-supplemented diet,all patients had frequent hypoglycemic episodes(2.51±3.88 times/week),concurrent with neuroglycopenia(in 83.3% of patients)and autonomic manifestations(in 75.0% of patients),with the median fasting blood glucose(FBG)of 2.70(interquartile range[IQR]:2.50-2.90)mmol/*** patients'weight increased by 0.38(IQR:0.05-0.65)kg per month,with 8(33.3%)cases developing overweight and 7(29.2%)cases developing *** patients maintained the RCS-supplemented diet until they underwent tumor resection(23 cases)and transarterial chemoembolization for liver metastases(1 case).For 19 patients receiving RCS throughout the day,the median FBG within one week of nutritional management was 4.30(IQR:3.30-5.70)mmol/L,which was a significant increase compared to pre-nutritional level[2.25(IQR:1.60-2.90)mmol/L;P<0.001].Of them,10 patients receiving RCS throughout the day for over four weeks had sustained improvement in FBG compared to pre-treatment[3.20(IQR:2.60-3.95)mmol/L vs.2.15(IQR:1.83-2.33)mmol/L;P<0.001].Five patients who received RCS only at night also had a significant increase in FBG within one week of nutritional management[3.50(IQR:2.50-3.65)mmol/L vs.2.20(IQR:1.80-2.60)mmol/L;P<0.001],but only one patient who continued to receive RCS for over four weeks did not have a significant improvement in *** improvement in weight gain was observed upon RCS *** diarrhea(2 cases)and flatulence(1 case)occurred,and were relieved by reduction of RCS ***

关键词： corn starch hypoglycemia insulinoma nutrition therapy

Impacts of angiotensin II on retinal artery changes in apolipoprotein E deficient mice

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International Journal of Ophthalmology(English edition) 2024年第1期17卷 16-24页

作者： Li-Hui Meng Shi-Yu Cheng He Chen Yue-Lin Wang Wen-Fei Zhang Huan Chen Xin-Yu Zhao You-Xin Chen Department of Ophthalmology Peking Union Medical College HospitalChinese Academy of Medical SciencesBeijing 100730China Key Lab of Ocular Fundus Diseases Chinese Academy of Medical SciencesBeijing 100730China

AIM:To investigate the impacts of angiotensin II(Ang II)on retinal artery changes in apolipoprotein E deficient(apoE^(-/-))***:ApoE^(-/-)male mice were infused by minipumps with Ang II at 1000 ng/kg·min(Ang II group)or saline(control group)for *** were underwent ophthalmic fundus examination on day 0,14,and 28 of *** examination,ribonucleic acid(RNA)sequencing and local Ang II measurement of retinas were ***:Ophthalmic fundus examination showed Ang II infusion promoted the formation of retinal arterial aneurysm-like lesions on day *** coherence tomography revealed the ganglion cell and inner plexiform layer(GCIPL)thickness in the control group was significantly thinner than that in Ang II group(P<0.001).Hematoxylin-eosin staining demonstrated diffused swelling of GCIPL layer and its disordered structure in Ang II *** electron microscopy showed Ang II infusion caused aggravation of atherosclerotic lesions,including increased swelling,roughness,disorganization of the retinal vasculature,and vacuoles ***-sequencing and gene ontology enrichment analysis demonstrated that the structure and function of cellular membrane might be disturbed and visual function might be compromised by Ang *** local level of Ang II was higher in Ang II infusion group but did not show significant differences compared to the control group(P=0.086).CONCLUSION:Ang II infusion promotes the formation of retinal arterial aneurysm-like lesions in apoE^(-/-)mice,causing aggravation of atherosclerotic lesions,more severe disorganization of the retinal vasculature and disturbance of the cellular membrane.

关键词： angiotensin II retinal artery aneurysm apoE^(-/-)mice

Dermal thickness,rather than drug concentration and injection speed,influences the effective area of botulinum toxin type A in the dermis

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Chinese Journal of Plastic and Reconstructive surgery 2024年第3期6卷 130-134页

作者： Yixin Sun Wangfei Mo Yunzhu Li Ruijia Dong Cheng Chen Jing Gao Nanze Yu Xiao Long Department of Plastic surgery Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing100730China Department of Plastic and Burns surgery West China HospitalSichuan UniversityChengdu610041China Department of Plastic and Reconstructive surgery Beijing Tsinghua Changgung HospitalSchool of Clinical MedicineTsinghua UniversityBeijing102218China Department of Ultrasound Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing100730China

Background:Recently,microbotulinum,a new technique that involves injecting botulinum toxin type A(BoNTA)microdroplets into superficial cutaneous tissue,has gained *** precise distribution of BoNTA in the targeted area profoundly affects *** factors may influence the effective area of BoNTA in the *** study aimed to determine the dermal distribution properties of BoNTA to guide microbotulinum ***:Ten healthy males aged 18–65 years without BoNTA treatment in the previous year were recruited to receive intradermal injections in the chest and *** was used to ensure the intradermal delivery of injections and measure the dermal *** minor iodine starch test was performed at baseline and 3 days,7 days,21 days,1 month,and 2 months after ***:All participants received intradermal *** dermis was thinner on the chest(thickness,0.20±0.03 cm)than on the back(thickness,0.39±0.07 cm)(P<0.05).An injection in the thicker dermis had a significantly smaller effective area at every follow-up *** drug concentration did not affect the effective area except at 3 days after *** speed did not influence the effective area at any follow-up ***:An injection in a thicker dermis leads to a smaller effective area for intradermal *** the BoNTA dose is the same,the drug concentration and injection speed do not matter.

关键词： Botulinum toxin type A Effective area Microbotulinum

Triceps skinfold thickness trajectories and the risk of all-cause mortality:A prospective cohort study

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World Journal of Clinical Cases 2024年第15期12卷 2568-2577页

作者： Na Yang Li-Yun He Zi-Yi Li Yu-Cheng Yang Fan Ping Ling-Ling Xu Wei Li Hua-Bing Zhang Yu-Xiu Li Department of Endocrinology Key Laboratory of Endocrinology of National Health CommissionPeking Union Medical College HospitalChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100730China

BACKGROUND The measurement of triceps skinfold(TSF)thickness serves as a noninvasive metric for evaluating subcutaneous fat *** its clinical utility,the TSF thickness trajectories and their correlation with overall mortality have not been thoroughly *** To explore TSF thickness trajectories of Chinese adults and to examine their associations with all-cause *** This study encompassed a cohort of 14747 adults sourced from the China Health and Nutrition *** class trajectory modeling was employed to identify distinct trajectories of TSF *** were classified into subgroups reflective of their respective TSF thickness *** utilized multivariate Cox regression analyses and mediation examinations to explore the link between TSF thickness trajectory and overall mortality,including contributory *** Upon adjustment for multiple confounding factors,we discerned that males in the‘Class 2:Thin-stable’and‘Class 3:Thin-moderate’TSF thickness trajectories exhibited a markedly reduced risk of mortality from all causes in comparison to the‘Class 1:Extremely thin’*** the mediation analyses,the Geriatric Nutritional Risk Index was found to be a partial intermediary in the relationship between TSF thickness trajectories and *** females,a lower TSF thickness pattern was significantly predictive of elevated all-cause mortality risk exclusively within the non-elderly *** In males and non-elderly females,lower TSF thickness trajectories are significantly predictive of heightened mortality risk,independent of single-point TSF thickness,body mass index,and waist circumference.

关键词： Triceps skinfold thickness Trajectory All-cause mortality Body mass index Geriatric Nutritional Risk Index