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ccdc141 is required for left-right axis development by regulating cilia formation in the Kupffer's vesicle of zebrafish

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Journal of Genetics and Genomics 2024年第9期51卷 934-946页

作者： Pengcheng Wang Wenxiang Shi Sijie Liu Yunjing Shi Xuechao Jiang Fen Li Sun Chen Kun Sun Rang Xu Department of Pediatric Cardiology Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghai 200092China Department of Cardiovascular medicine Heart Failure CenterRuijin HospitalRuijin Hospital Lu Wan BranchShanghai Jiao Tong University School of MedicineShanghai 200025China Scientific Research Center Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghai 200092China Department of Pediatric Cardiology Shanghai Children's Medical CenterShanghai Jiao Tong University School of MedicineShanghai 200127China

Laterality is a crucial physiological process intricately linked to the cilium-centrosome complex during embryo *** in the process can result in severe organ ***-coil domain containing 141(CCDC141)has been previously known as a centrosome-related gene,but its role in left-right(LR)asymmetry has not been *** this study,we utilize the zebrafish model and human exome analysis to elucidate the function of ccdc141 in laterality *** knockdown of ccdc141 in zebrafish disrupts early LR signaling pathways,cilia function,and Kupffer's vesicle *** ccdc141-knockdown embryos exhibiting aberrant LR patterns,ccdc141-null mutants show no apparent abnormality,suggesting a genetic compensation response *** parallel,we observe a marked reduction inα-tubulin acetylation levels in the ccdc141 *** treatment with histone deacetylase(HDAC)inhibitors,particularly the HDAC6 inhibitor,rescues the ccdc141 crispant ***,exome analysis of 70 patients with laterality defects reveals an increased burden of CCDC141 mutations,with in-vivo studies verifying the pathogenicity of the patient mutation *** findings highlight the critical role of ccdc141 in ciliogenesis and demonstrate that CCDC141 mutations lead to abnormal LR patterns,identifying it as a candidate gene for laterality defects.

关键词： Left-right axis Cilia WES Laterality defects Zebrafish CCDC141

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Neiguan(PC6) acupoint stimulation for preventing chemotherapy-induced nausea and vomiting: a cost-effective supplement in guideline-inconsistent chemotherapy-induced nausea and vomiting prophylaxis subgroup

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Journal of Traditional Chinese medicine 2024年第3期44卷 581-585页

作者： YU Wenxi TANG Lina LI Hongtao WANG Yonggang SHEN Zan Department of Oncology Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai200233China

OBJECTIVE: To assess the efficacy and safety of Neiguan(PC6) acupoint acustimulation in preventing chemotherapy-induced nausea and vomiting(CINV),especially for patients with guideline-inconsistent CINV prophylaxis(GICP) due to personal reasons METHODS: From January 2021 to December 2021, 373 patients suffered from solid malignancy were recruited according to the inclusion criteria. Complete response(no emesis and no rescue medication use) rate during the overall phase(0-120 h of each chemo-cycle) was the primary assessment of CINV control. The Functional Living Index-Emesis(FLIE) questionnaire was investigated among these patients as a secondary 'quality of life' objective to assess the impact of CINV on patients' daily life by recording score of nausea and vomiting. RESULTS: With acustimulation of Neiguan(PC6) acupuncture point through a portable, noninvasive and user-friendly device, in terms of complete response rate and scores in nausea/vomiting by FLIE questionnaire, patients achieve a better outcome in highly emetogenic chemotherapy(HEC) induced CINV, especially GICP subgroup. Meanwhile, analysis also demonstrated this tendency existed in other patients with HEC/GCCP(guideline consistent CINV prophylaxis) and moderate emetogenic chemotherapy, although the difference was not significant. CONCLUSION: Considering advantages of Neiguan(PC6) acustimulation such as noninvasive, covered by medical insurance and few side effects, we believe it would be an ideal auxiliary tool in CINV control, especially in patients who receive highly emetogenic chemoprotocol and are reluctant to GCCP for economic reasons.

关键词： acupuncture acustimulation tumor chemotherapy-induced nausea and vomiting

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Detachable string magnetically controlled capsule endoscopy for the noninvasive diagnosis of esophageal diseases:A prospective,blind clinical study

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World Journal of Gastroenterology 2024年第9期30卷 1121-1131页

作者： Yan-Ling Yang Huang-Wen Qin Zhao-Yu Chen Hui-Ning Fan Yi Yu Wei Da Jin-Shui Zhu Jing Zhang Department of Gastroenterology Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200233China

BACKGROUND Traditional esophagogastroduodenoscopy(EGD),an invasive examination method,can cause discomfort and pain in *** contrast,magnetically controlled capsule endoscopy(MCE),a noninvasive method,is being applied for the detection of stomach and small intestinal diseases,but its application in treating esophageal diseases is not *** To evaluate the safety and efficacy of detachable string MCE(ds-MCE)for the diagnosis of esophageal *** Fifty patients who had been diagnosed with esophageal diseases were pros-pectively recruited for this clinical study and underwent ds-MCE and conven-tional *** primary endpoints included the sensitivity,specificity,positive predictive value,negative predictive value,and diagnostic accuracy of ds-MCE for patients with esophageal *** secondary endpoints consisted of visualizing the esophageal and dentate lines,as well as the subjects'tolerance of the *** Using EGD as the gold standard,the sensitivity,specificity,positive predictive value,negative predictive value,and diagnostic accuracy of ds-MCE for esophageal disease detection were 85.71%,86.21%,81.82%,89.29%,and 86%,***-MCE was more comfortable and convenient than EGD was,with 80%of patients feeling that ds-MCE examination was very comfortable or comfortable and 50%of patients believing that detachable string v examination was very *** This study revealed that ds-MCE has the same diagnostic effects as traditional EGD for esophageal diseases and is more comfortable and convenient than EGD,providing a novel noninvasive method for treating esophageal diseases.

关键词： Clinical trial Detachable string magnetically controlled capsule endoscopy Esophagogastroduodenoscopy Noninvasive diagnosis Esophageal diseases

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Anti-metabolic agent pegaspargase plus PD-1 antibody sintilimab for first-line treatment in advanced natural killer T cell lymphoma

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Signal Transduction and Targeted Therapy 2024年第4期9卷 1665-1674页

作者： Jie xiong Shu Cheng xiao Gao Shan-He Yu Yu-Ting Dai xin-Yun Huang Hui-Juan Zhong Chao-Fu Wang Hong-Mei Yi Hao Zhang Wei-Guo Cao Rong Li Wei Tang Yan Zhao Peng-Peng Xu Li Wang Wei-Li Zhao Shanghai Institute of Hematology State Key Laboratory of Medical GenomicsNational Research Center for Translational Medicine at ShanghaiRuijin Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghaiChina Department of Nuclear medicine Shanghai Ruijin HospitalShanghai Jiao Tong University School of MedicineShanghaiChina Department of Pathology Shanghai Ruijin HospitalShanghai Jiao Tong University School of MedicineShanghaiChina Department of Otolaryngology Shanghai Rujin HospitalShanghai Jiao Tong University School of MedicineShanghaiChina Department of Radiation Shanghai Ruijin HospitalShanghai Jiao Tong University School of MedicineShanghaiChina Department of Hematology Navy Medical Center of PLAShanghaiChina Pole de Recherches Sino-Francais en Science du Vivant et Genomique Laboratory of Molecular PathologyShanghaiChina

Natural killer T cell lymphoma(NKTCL)is highly aggressive,with advanced stage patients poorly responding to intensive *** explore effective and safe treatment for newly diagnosed advanced stage NKTCL,we conducted a phase ll study of anti-metabolic agent pegaspargase plus PD-1 antibody sintilimab(NCT04096690).Twenty-two patients with a median age of 51 years(range,24-74)were enrolled and treated with induction treatment of pegaspargase 2500 IU/m2 intramuscularly on day 1 and sintilimab 200 mg intravenously on day 2 for 6 cycles of 21 days,followed by maintenance treatment of sintilimab 200 mg for 28 cycles of 21 *** complete response and overall response rate after induction treatment were 59%(95%Cl,43-79%)and 68%(95%Cl,47-84%),*** a median follow-up of 30 months,the 2 year progression-free and overall survival rates were 68%(95%Cl,45-83%)and 86%(95%Cl,63-95%),*** most frequently grade 3/4 adverse events were neutropenia(32%,n=7)and hypofibrinogenemia(18%,n=4),which were manageable and led to no discontinuation of *** proportion score of PD-L1,peripheral blood high-density lipoprotein cholesterol,and apolipoprotein A-l correlated with good response,while PD-1 on tumor infiltrating lymphocytes and peripheral Treg cells with poor response to pegaspargase plus sintilimab *** conclusion,the chemo-free regimen pegaspargase plus sintilimab was effective and safe in newly diagnosed,advanced stage *** lipid profle and immunosuppressive signature contributed to treatment resistance,providing an alternative therapeutic approach dual targeting fatty acid metabolism and CTLA-4 in NKTCL.

关键词： killer metabolism lymphoma

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A randomized,controlled,open label non-inferiority trial of intravenous ferric carboxymaltose versus iron sucrose in patients with iron deficiency anemia in China

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Frontiers of medicine 2024年第1期18卷 98-108页

作者： Jie Jin Zhihua Ran Emanuele Noseda Bernard Roubert Matthieu Marty Anna Mezzacasa Udo Michael Göring The First affiliated hospital Zhejiang University College of MedicineHangzhou310058China Renji hospital Shanghai Jiao Tong University School of MedicineShanghai200025China Vifor Pharma Glattbrugg8152Switzerland

Iron deficiency(ID)and ID anemia(IDA)pose significant public health concerns in *** iron sucrose(IS)treatment is well-established in the country,ferric carboxymaltose(FCM)offers the advantage of higher doses and fewer *** open label,randomized,controlled,non-inferiority trial was conducted at multiple sites in China to compare the outcomes of FCM(maximum of 2 doses,500 or 1000 mg iron)and IS(up to 11 infusions,200 mg iron)treatments in subjects with *** primary endpoint was the achievement of hemoglobin(Hb)response(an increase of⩾2 g/dL from baseline)within 8 weeks,whereas secondary endpoints included changes in Hb,transferrin saturation,and serum ferritin *** the 371 randomized subjects,a similar percentage of subjects treated with FCM and IS achieved Hb-response(FCM 99.4%,IS 98.3%),thereby confirming the non-inferiority of FCM compared with IS(difference 1.12(−2.15,4.71;95%confidence interval(CI))).Furthermore,a significantly higher proportion of FCM-treated subjects achieved early Hb-response at Week 2(FCM 85.2%,IS 73.2%;difference 12.1(3.31,20.65;95%CI)).Additionally,the increase in TSAT and serum ferritin levels from baseline was significantly greater at all time points for FCM-treated *** safety profiles of FCM and IS were comparable,with the exception of transient hypophosphatemia and pyrexia,which are consistent with FCM’s known safety *** conclusion,FCM proves to be an efficacious treatment for IDA,providing faster Hb-response and correction of ID with fewer administrations than IS.

关键词： iron deficiency anemia intravenous iron ferric carboxymaltose iron sucrose Hb response early response

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Cellular strategies to induce immune tolerance after liver transplantation:Clinical perspectives

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World Journal of Gastroenterology 2024年第13期30卷 1791-1800页

作者： Ai-Wei Zhou Jing Jin Yuan Liu Department of Liver Surgery Renji Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200127China Department of Nursing Renji Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200127China Department of Liver Transplantation Shanghai Immune Therapy InstituteShanghai 200127China

Liver transplantation(LT)has become the most efficient treatment for pediatric and adult end-stage liver disease and the survival time after transplantation is becoming longer due to the development of surgical techniques and perioperative ***,long-term side-effects of immunosuppressants,like infection,metabolic disorders and malignant tumor are gaining more *** tolerance is the status in which LT recipients no longer need to take any immunosuppressants,but the liver function and intrahepatic histology maintain *** approaches to achieve immune tolerance after transplantation include spontaneous,operational and induced *** first two means require no specific intervention but withdrawing immunosuppressant gradually during *** clinical factors or biomarkers so far could accurately predict who are suitable for immunosuppressant withdraw after *** the understanding to the underlying mechanisms of immune tolerance,many strategies have been developed to induce tolerance in LT *** strategy is one of the most promising methods for immune tolerance induction,including chimerism induced by hematopoietic stem cells and adoptive transfer of regulatory immune *** safety and efficacy of various cell products have been evaluated by prospective preclinical and clinical trials,while obstacles still exist before translating into clinical ***,we will summarize the latest perspectives and concerns on the clinical application of cellular strategies in LT recipients.

关键词： Cellular therapy Induced tolerance Liver transplantation Regulatory T cells Regulatory dendritic cells

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Unmet needs in biomarkers for autoimmune pancreatitis diagnosis

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World Journal of Gastroenterology 2024年第6期30卷 523-526页

作者： Bao-Can Wang Jian-Gao Fan Department of Gastroenterology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China

Autoimmune pancreatitis(AIP)is a rare chronic autoimmune *** diagnosis of AIP mainly depends on histopathology,imaging and response to *** immunoglobulin 4(IgG4)is used only as collateral evidence in diagnostic criteria for AIP because of its moderate *** IgG4 levels are normal in 15%-37%of type 1 AIP and most of type 2 AIP *** these patients,the indeterminate imaging and histopathology may lead to the difficulty in definitive diagnosis of ***,discovery of new biomarkers is impor-tant for AIP ***,we provide some views on the progression and challenges in identifying novel serological biomarkers in AIP diagnosis.

关键词： Autoimmune pancreatitis Immunoglobulin G4 Biomarker,Cytokine Autoantibody

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Clinical management of a rare melanoma case arising from congenital melanocytic nevus

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Genes & Diseases 2024年第3期11卷 79-82页

作者： Ruixin Jiang Yan Wang Xuhui Ma xinyang Xuanyuan Yanjie Zhang Bin Jiang Weizhen Zhang Hanlin Zeng Department of Oncology Shanghai Ninth People's HospitalShanghai Jiao Tong University School of MedicineShanghai 201900China Department of Oncology The Third People's Hospital of ZhengzhouZhengzhouHenan 450000China Department of Oral&Maxillofacial-Head and Neck Oncology Shanghai Ninth People's HospitalShanghai Jiao Tong University School of MedicineShanghai 200125China Shanghai Institute of Precision medicine Shanghai Ninth People's HospitalShanghai Jiao Tong University School of MedicineShanghai200125China Department of Dermatology Shanghai Ninth People's HospitalShanghai Jiao Tong University School of MedicineShanghai 200000China

Congenital melanocytic nevi(CMNs)are skin lesions characterized by benign melanocytic proliferations and present at birth or shortly *** and giant CMNs,with a projected adult size≥20 cm and 40 cm in diameter respectively,are more likely to develop into malignant *** most cases,melanoma arising from congenital melanocytic nevus(CMN)is particularly aggressive.

关键词： melanoma congenital melan

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Flexible polydimethylsiloxane pressure sensor with micro-pyramid structures and embedded silver nanowires:A novel application in urinary flow measurement

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Nanotechnology and Precision Engineering 2024年第3期7卷 1-12页

作者： Ben-Song Wang Ang Gao Song-Wei Huang Qi-Hong Ning Cheng Zhou Qi-xiang Song Da-xiangCui school of Electronic Information and Electrical Engineering Shanghai Jiao Tong UniversityShanghai 200240China Department of Urology Renji HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai 200240China

Flexible pressure sensors are lightweight and highly sensitive,making them suitable for use in small portable devices to achieve precise measurements of tiny *** article introduces a low-cost and easy-fabrication strategy for piezoresistive flexible pressure *** embedding silver nanowires into a polydimethylsiloxane layer with micro-pyramids on its surface,a flexible pressure sensor is created that can detect low pressure (17.3 Pa) with fast response (xible pressure sensor is innovatively combined with a micro-rotor to fabricate a novel urinary flow-rate meter (uroflowmeter),and results from a simulated human urination experiment show that the uroflowmeter accurately captured all the essential shape characteristics that were present in the pump-simulated urination *** ahead,this research provides a new reference for using flexible pressure sensors in urinary flow-rate monitoring.

关键词： Flexible pressure sensor Silver nanowires Portable device Uroﬂowmeter Healthcare

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Interactions Between Extracellular Vesicles and Autophagy in Neuroimmune Disorders

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Neuroscience Bulletin 2024年第7期40卷 992-1006页

作者： xiwen Ai Haojun Yu Yu Cai Yangtai Guan Department of Neurology Shanghai Jiao Tong University School of Medicine Affiliated Renji HospitalShanghai 200127China Department of Neurology Renaissance School of MedicineStony Brook UniversityStony BrookNY 11794USA

Neuroimmune disorders,such as multiple sclerosis,neuromyelitis optica spectrum disorder,myasthenia gravis,and Guillain–Barrésyndrome,are characterized by the dysfunction of both the immune system and the nervous *** evidence suggests that extracellular vesicles and autophagy are closely associated with the pathogenesis of these *** this review,we summarize the current understanding of the interactions between extracellular vesicles and autophagy in neuroimmune disorders and discuss their potential diagnostic and therapeutic *** we highlight the need for further research to fully understand the mechanisms underlying these disorders,and to develop new diagnostic and therapeutic strategies.

关键词： Autophagy Extracellular vesicles Neuroimmune disorders Multiple sclerosis Neuromyelitis optica spectrum disorder Myasthenia gravis Guillain-Barrésyndrome

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