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Renal Vein Thrombosis Suggestive of Extramembranous Glomerulonephritis Associated with Sjögren’s Syndrome (Case Report)

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Open Journal of Internal Medicine 2024年第2期14卷 181-187页

作者： Mariam El Galiou Mina Agrou Rihab Dkhissi Loubna Benamar Naima Ouzeddoun Lamyae Rouass Tarik Bouattar department of Nephrology Dialysis and transplantation IbnSina University Hospital Mohamed V University Rabat Morocco department of Pathological Anatomy IbnSina University Hospital Mohammed V University Rabat Morocco

Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the case of a 44-year-old woman with primary Sjgrens syndrome, confirmed by clinical dryness syndrome, positive anti-SSA and anti-SSB antibodies, and a salivary gland biopsy revealing grade 4 lymphocytic sialadenitis according to CHISHOLMs classification. Later, the patient developed nephrotic syndrome, along with hypertension. Renal function remained normal with a creatinine level of 9.3 mg/l, and hematuria was absent. Only antinuclear antibodies tested positive, while anti-PLA2R antibodies were negative. A renal biopsy was performed, which was complicated on the same day by hemodynamic instability with hematuria. Renal CT scan with contrast injection revealed a posterior perirenal hematoma without contrast extravasation. Additionally, bilateral renal vein thrombosis was incidentally discovered, suggesting extramembranous glomerulonephritis. The patients hemodynamic status stabilized after fluid resuscitation with isotonic saline solution (0.9%), without the need for blood transfusion. Renal biopsy confirmed extramembranous glomerulonephritis with interstitial fibrosis and minimal tubular atrophy. The initial etiological assessment was negative. The patient was started on oral corticosteroids, angiotensin-converting enzyme inhibitors, and therapeutic anticoagulation for renal vein thrombosis. The patients condition improved, with the disappearance of the syndrome and spontaneous regression of the hematoma. Discussion: The association of nephrotic syndrome and renal vein thrombosis primarily suggests glomerulopathy, in particular extra-membranous glomerulonephritis. Sjgrens syndrome can be associated with extra-membranous glomerulonephritis without being its direct cause. Like, it is possible that it is a cause of glomerulonephritis, essentially extra membrano

关键词： Sjögren’s Syndrome Extramembranous Glomerulonephritis Nephrotic Syndrome Anti-PLA2R Antibodies

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Integrated bulk and single-cell transcriptome data identify clinically relevant cell populations in clear cell renal cell carcinoma

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Genes & Diseases 2024年第1期11卷 42-45页

作者： Pingbao Zhang Pu Zhang Jun Gao Xiaosong Li Chengcheng Wei Weihui Liu QingLiu He Yuan Zhang department of Urology Zhongshan HospitalFudan UniversityShanghai 200032China department of Urology Surgery Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhanHubei 430022China department of Liver Surgery and transplantation Liver Cancer InstituteZhongshan HospitalFudan UniversityShanghai 200032China Clinical Molecular Medicine Testing Center The First Affiliated Hospital of Chongqing Medical UniversityChongqing 400016China department of Urology The Second Affiliated Hospital of Fujian Medical UniversityQuanzhouFujian 362000China department of Nephrology Sichuan Provincial People’s HospitalUniversity of Electronic Science and Technology of ChinaChengduSichuan 610072China

Renal cell carcinoma,which consists of three main histological subtypes,namely,clear cell renal cell carcinoma(ccRCC),papillary RCC(pRCC),and chromophobe RCC(chRCC),accounts for most kidney cancer *** accounts for approximately 85%of all RcC cases,1 and it is a malignant tumor with multiple molecular features and a poor *** have shown that ccRCC is one of the most immune and vaso-invasive cancer types.2 Considering that ccRCC is not sensitive to either radiotherapy or chemotherapy,targeted therapies.

关键词： chemotherapy cancer cases

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Characteristics and distinct prognostic determinants of individuals with hepatosplenic T-cell lymphoma over the past two decades

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World Journal of Clinical Oncology 2024年第6期15卷 745-754页

作者： Ayrton Bangolo Pierre Fwelo Shraboni Dey Tanni Sethi Sowmya Sagireddy Jawaria Chatta Ashish Goel Sneha Nagpaul Eric Pin-Shiuan Chen Chiranjeeve Saravanan Sheeja Gangan Joel Thomas Sarah Potiguara Vignesh K Nagesh Daniel Elias Charlene Mansour Prajakta H Ratnaparkhi Priyanshu Jain Midhun Mathew Taylor Porter Shadiya Sultan Shailaja Abbisetty Linh Tran Megha Chawla Abraham Lo Simcha Weissman Christina Cho department of Internal Medicine Palisades Medical CenterNorth BergenNJ 07047United States department of Epidemiology Human Geneticsand Environmental SciencesUTHealth School of Public HealthHoustonTX 77030United States department of Medicine Palisades Medical CenterNorth BergenNJ 07047United States Stem cell transplantation and cellular Therapy John Theurer Cancer CenterHackensackNJ 07601United States

BACKGROUND Hepatosplenic T-cell lymphoma(HSTCL)is a rare and aggressive peripheral T-cell lymphoma with historically dismal outcomes,representing less than one percent of non-Hodgkin *** its rarity,the true incidence of HSTCL is unknown and most data have been extrapolated through case *** the best of our knowledge,the largest and most up to date study addressing the epidemiology and outcomes of patients with HSTCL in the United States covered a period from 1996 to 2014,with a sample size of 122 *** To paint the most updated epidemiological picture of *** A total of 186 patients diagnosed with HSTCL,between 2000 and 2017,were ultimately enrolled in our study by retrieving data from the Surveillance,Epidemiology,and End Results *** analyzed demographics,clinical characteristics,and overall mortality(OM)as well as cancer-specific mortality(CSM)of *** with a P value<0.01 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors,with a hazard ratio of greater than 1 representing adverse prog-nostic *** Overall,the outlook for this rare malignancy is very *** this retrospective cohort study of the United States population,non-Hispanic blacks and the elderly had a higher *** data highlights the need for larger prospective studies to investigate factors associated with worse prognosis in one ethnic group,such as treatment delays,which have been shown to increase mortality in this racial/ethnic group for other cancers.

关键词： Extra nodal lymphoma Mortality Survival Racial disparity Age

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Umbilical cord mesenchymal stem cell exosomes alleviate necrotizing enterocolitis in neonatal mice by regulating intestinal epithelial cells autophagy

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World Journal of Stem cells 2024年第6期16卷 728-738页

作者： Lin Zhu Lu He Wu Duan Bo Yang Ning Li department of Pediatrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430077Hubei ProvinceChina Division of Endocrinology Department of Internal MedicineQilu Hospital of Shandong UniversityJinan 250012Shandong ProvinceChina Shenzhen Research Institute of Shandong University Shenzhen 518000Guangdong ProvinceChina Institute of Organ transplantation Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430077Hubei ProvinceChina department of Physical Education Heze UniversityHeze 274015Shandong ProvinceChina

BACKGROUND Necrotizing enterocolitis(NEC)is a severe gastrointestinal disease that affects premature *** mounting evidence supports the therapeutic effect of exosomes on NEC,the underlying mechanisms remain *** To investigate the mechanisms underlying the regulation of inflammatory response and intestinal barrier function by umbilical cord mesenchymal stem cell(UCMSCs)exosomes,as well as their potential in alleviating NEC in neonatal *** NEC was induced in 5-d-old C57BL/6 pups through hypoxia and gavage feeding of formula containing lipopolysaccharide(LPS),after which the mice received human UCMSC exosomes(hUCMSC-exos).The control mice were allowed to breastfeed with their *** tissues were collected from the mice and analyzed by histopathology and *** tissues were collected from NEC neonates and analyzed by *** biology and cell culture approaches were employed to study the related mechanisms in intestinal epithelial *** We found that autophagy is overactivated in intestinal epithelial cells during NEC,resulting in reduced expression of tight junction proteins and an increased inflammatory *** ability of hUCMSC-exos to ameliorate NEC in a mouse model was dependent on decreased intestinal *** also showed that hUCMSC-exos alleviate the inflammatory response and increase migration ability in intestinal epithelial cells induced by *** These results contribute to a better understanding of the protective mechanisms of hUCMSC-exos against NEC and provide a new theoretical and experimental foundation for NEC *** findings also enhance our understanding of the role of the autophagy mechanism in NEC,offering potential avenues for identifying new therapeutic targets.

关键词： Necrotizing enterocolitis Autophagy Umbilical cord mesenchymal stem cell Exosomes Intestinal epithelial cell Intestinal barrier function

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Crohn’s disease as the intestinal manifestation of pan-lymphatic dysfunction:An exploratory proposal based on basic and clinical data

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World Journal of Gastroenterology 2024年第1期30卷 34-49页

作者： Yu-Wei Zhou Yue Ren Miao-Miao Lu Ling-Ling Xu Wei-Xin Cheng Meng-Meng Zhang Lin-Ping Ding Dong Chen Jian-Guo Gao Juan Du Ci-Liang Jin Chun-Xiao Chen Yun-Fei Li Tao Cheng Peng-Lei Jiang Yi-Da Yang Peng-Xu Qian Peng-Fei Xu Xi Jin department of Gastroenterology The First Affiliated Hospital of Zhejiang University School of MedicineHangzhou 310003Zhejiang ProvinceChina department of Gastroenterology The Second Hospital of JiaxingJiaxing 314000Zhejiang ProvinceChina Endoscopy Center Children’s Hospital of Zhejiang University School of MedicineHangzhou 310003Zhejiang ProvinceChina department of Gastroenterology The Second People’s Hospital of Yuhang DistrictHangzhou 310000Zhejiang ProvinceChina department of Gastroenterology Hangzhou Shangcheng District People’s HospitalHangzhou 310003Zhejiang ProvinceChina department of Colorectal Surgery The First Affiliated Hospital of Zhejiang University School of MedicineHangzhou 310003Zhejiang ProvinceChina department of Gastroenterology The First Affiliated Hospital of Wenzhou Medical UniversityWenzhou 325000Zhejiang ProvinceChina Women’s Hospital and Institute of Genetics Zhejiang University School of MedicineHangzhou 310000Zhejiang ProvinceChina Center of Stem cell and Regenerative Medicine and Bone Marrow Transplantation CenterThe First Affiliated Hospital of Zhejiang University School of MedicineHangzhou 310003Zhejiang ProvinceChina department of Infectious Disease The First Affiliated Hospital of Zhejiang University School of MedicineHangzhou 310003Zhejiang ProvinceChina

Crohn’s disease(CD)is caused by immune,environmental,and genetic *** can involve the entire gastrointestinal tract,and although its prevalence is rapidly increasing its etiology remains *** biological and small-molecule drugs have advanced the treatment of CD;however,a considerable proportion of patients are non-responsive to all known *** achieve a breakthrough in this field,innovations that could guide the further development of effective therapies are of utmost *** this review,we first propose the innovative concept of pan-lymphatic dysfunction for the general distribution of lymphatic dysfunction in various diseases,and suggest that CD is the intestinal manifestation of pan-lymphatic dysfunction based on basic and clinical preliminary *** supporting evidence is fully summarized,including the existence of lymphatic system dysfunction,recognition of the inside-out model,disorders of immune cells,changes in cell plasticity,partial overlap of the underlying mechanisms,and common gut-derived fatty and bile acid *** benefit of this novel concept is that it proposes adopting the zebrafish model for studying intestinal diseases,especially CD,as this model is good at presenting and mimicking lymphatic *** importantly,the ensuing focus on improving lymphatic function may lead to novel and promising therapeutic strategies for CD.

关键词： Inflammatory bowel disease Crohn’s disease Lymphatic system Inside-out model Immune cells Zebrafish

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The non-canonical poly(A)polymerase FAM46C promotes erythropoiesis

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Journal of Genetics and Genomics 2024年第6期51卷 594-607页

作者： Ke Yang Tiangi Zhu Jiaying Yin Qiaoli Zhangg Jing Li Hong Fan Gajing Han Weiyin Xu Nan Liu Xiang Lv Bone Marrow transplantation Center The First Affiliated HospitalZhejiang University School of MedicineHangzhouZhejiang 310003China Liangzhu Laboratory Zhejang UniversityHangzhouZhejang 311121China Institute of Hematology Zhejiang UniversityHangzhouZhejiang 311121China The State Key Laboratory for Complex Severeand Rare DiseasesHaihe Laboratory of Cell EcosystemDepartment of PathophysiologyInstitute of Basic Medical SciencesChinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100005China Medical Epigenetics Research Center Chinese Academy of Medical Sciences&Peking Union Medical CollegeBeijing 100005China

The post-transcriptional regulation of mRNA is a crucial component of gene *** disruption of this process has detrimental effects on the normal development and gives rise to various *** for novel post-transcriptional regulators and exploring their roles are essential for understanding development and *** a multimodal analysis of red blood cell trait genome-wide association studies(GWAS)and transcriptomes of erythropoiesis,we identify FAM46C,a non-canonical RNA poly(A)polymerase,as a necessary factor for proper red blood cell ***46C is highly expressed in the late stages of the erythroid lineage,and its developmental upregulation is controlled by an erythroidspecific *** demonstrate that FAM46C stabilizes mRNA and regulates erythroid differentiation in a polymerase activity-dependent ***,we identify transcripts of lysosome and mitochondria components as highly confident in vivo targets of FAM46C,which aligns with the need of maturing red blood cells for substantial clearance of organelles and maintenance of cellular redox *** conclusion,our study unveils a unique role of FAM46C in positively regulating lysosome and mitochondria components,thereby promoting erythropoiesis.

关键词： FAM46C TENT5C Poly(A)polymerase Erythroblasts Post-transcriptional regulation Erythroid-specific enhancer

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Lipid-nanoparticle-enabled nucleic acid therapeutics for liver disorders

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Acta Pharmaceutica Sinica B 2024年第7期14卷 2885-2900页

作者： Porkizhi Arjunan Durga Kathirvelu Gokulnath Mahalingam Ashish Kumar Goel Uday George Zacharaiah Alok Srivastava Srujan Marepally Center for Stem cell Research(A Unit of inStem Bengaluru)Christian Medical College CampusBagayamVellore 632002Tamil NaduIndia Manipal academy for higher education Mangalore 576104KarnatakaIndia department of Hematology Christian Medical College&HospitalVellore 632004Tamil NaduIndia department of Hepatology Christian Medical College&HospitalVellore 632004Tamil NaduIndia

Inherited genetic disorders of the liver pose a significant public health *** transplantation is often limited by the availability of donor livers and the exorbitant costs of immunosuppressive *** overcome these limitations,nucleic acid therapy provides a hopeful alternative that enables gene repair,gene supplementation,and gene silencing with suitable *** viral vectors are the most efficient and preferred for gene therapy,pre-existing immunity debilitating immune responses limit their *** a potential alternative,lipid nanoparticle-mediated vectors are being explored to deliver multiple nucleic acid forms,including pDNA,mRNA,siRNA,and ***,we discuss the broader applications of lipid nanoparticles,from protein replacement therapy to restoring the disease mechanism through nucleic acid delivery and gene editing,as well as multiple preclinical and clinical studies as a potential alternative to liver transplantation.

关键词： Lipid nanoparticle Liver disorders Gene therapy Gene editing Nucleic acid delivery pDNA mRNA siRNA ASO Clinical trials

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Echerichia coli Infection—Associated Glomerulonephritis in a Kidney Transplant Patient: A Case Report

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Open Journal of Nephrology 2024年第1期14卷 81-85页

作者： Meryem Benbella Zaineb Kaouiri Imane Saidi Naima Ouzedoun Tarik Bouattar department of Nephrology-Dialysis-Renal transplantation Ibn Sina University Hospital in Rabat Rabat Morocco Faculty of Medicine and Pharmacy in Rabat Mohammed V University Rabat Morocco

Post infectious Glomerulonephritis (PIGN) in renal allograft is a rare entity. Only a few Cases have been described in the literature. The post streptococcal glomerulonephritis is the classic example in native kidney. A wide variety of organism has been associated with PIGN in renal allograft such as Staphylococcus, Cytomegalovirus and Polyomavirus. We describe one case of Infection associated glomerulonephritis due to Echericha Coli, developed 5 years after kidney transplantation, in 47 years old female patient. The Clinical presentation was characterized by a peripheral edema and high blood pressure, and biological tests showed a nephrotic syndrome, an acute kidney injury, a consumption of Complement fractions. The renal biopsy revealed a diffuse endocapillary cell proliferation with preponderant deposits of C3. Total recovery was achieved 4 months after Methyprednisolone pulse and Cyclophasphamid with antimicrobial treatment.

关键词： Glomerulonephritis Echerichia coli Kidney Transplant

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B cell development and antibody responses in human immune system mice:current status and future perspective

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Science China(Life Sciences) 2024年第4期67卷 645-652页

作者： Tao Zhang Wentao Liu Yong-Guang Yang Key Laboratory of Organ Regeneration&transplantation of the Ministry of Education The First Hospital of Jilin UniversityChangchun 130061China National-Local Joint Engineering Laboratory of Animal Models for Human Diseases Jilin UniversityChangchun 130061China International Center of Future Science Jilin UniversityChangchun 130061China

Humanized immune system(HIS)mice have been developed and used as a small surrogate model to study human immune function under normal or disease *** variations are found between models,most HIS mice show robust human T cell ***,there has been unsuccessful in constructing HIS mice that produce high-affinity human antibodies,primarily due to defects in terminal B cell differentiation,antibody affinity maturation,and development of primary follicles and germinal *** this review,we elaborate on the current knowledge about and previous attempts to improve human B cell development in HIS mice,and propose a potential strategy for constructing HIS mice with improved humoral immunity by transplantation of human follicular dendritic cells(FDCs)to facilitate the development of secondary follicles.

关键词： humanized immune system mouse humoral immunity germinal center B cell follicular dendritic cell

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Unresolved Excess Accumulation of Myelin-Derived Cholesterol Contributes to Scar Formation after Spinal Cord Injury

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Research 2024年第1期2023卷 17-32页

作者： Bolin Zheng Yijing He Shuai Yin Xu Zhu Qing Zhao Huiyi Yang Zhaojie wang Rongrong Zhu Liming Cheng Key Laboratory of Spine and Spinal Cord Injury Repair and Regeneration Ministry of EducationDepartment of OrthopedicsTongji HospitalSchool of MedicineTongji UniversityShanghai 200092China Frontier Science Center for Stem cell Research School of Life Science and TechnologyTongji UniversityShanghai 200092China Clinical Center for Brain and Spinal Cord Research Tongji UniversityShanghai 200092China

Spinal cord injury triggers complex pathological cascades,resulting in destructive tissue damage and incomplete tissue *** formation is generally considered a barrier for regeneration in the central nervous ***,the intrinsic mechanism of scar formation after spinal cord injury has not been fully ***,we report that excess cholesterol accumulates in phagocytes and is inefficiently removed from spinal cord lesions in young adult ***,we observed that excessive cholesterol also accumulates in injured peripheral nerves but is subsequently removed by reverse ***,preventing reverse cholesterol transport leads to macrophage accumulation and fibrosis in injured peripheral ***,the neonatal mouse spinal cord lesions are devoid of myelin-derived lipids and can heal without excess cholesterol *** found that transplantation of myelin into neonatal lesions disrupts healing with excessive cholesterol accumulation,persistent macrophage activation,and *** internalization suppresses macrophage apoptosis mediated by CD5L expression,indicating that myelin-derived cholesterol plays a critical role in impaired wound *** together,our data suggest that the central nervous system lacks an efficient approach for cholesterol clearance,resulting in excessive accumulation of myelin-derived cholesterol,thereby inducing scar formation after injury.

关键词： healing Spinal impaired

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