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A three-year clinical investigation of a Chinese child with craniometaphyseal dysplasia caused by a mutated ANKH gene

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World Journal of Clinical Cases 2021年第8期9卷 1853-1862页

作者： Jia-Li Wu xiao-Li Li Shu-Mei Chen xiao-ping lan Jin-Jin Chen xiao-Yan Li Wei Wang Department of Otolaryngology and Head and Neck Surgery Shanghai Children’s HospitalShanghai Jiaotong University School of MedicineShanghai 200062China Department of Radiation Oncology Longhua HospitalShanghai University of Traditional Chinese MedicineShanghai 200032China Molecular Diagnostic Laboratory Shanghai Children’s HospitalShanghai Jiaotong University School of MedicineShanghai 200062China Department of Child Healthcare Shanghai Children’s HospitalShanghai Jiaotong University School of MedicineShanghai 200062China Shanghai AhCare Consulting Shanghai 20120China

BACKGROUND Craniometaphyseal dysplasia(CMD)is a rare genetic *** dominant CMD(AD-CMD)is caused by mutations in the ANKH *** individuals typically have distinctive facial features including progressive thickening of the craniofacial *** for AD-CMD primarily consists of surgical intervention to release compression of the cranial nerves and the brain stem/spinal *** alleviate progression of the clinical course and improve the quality of life in children waiting to undergo the necessary surgery,we investigated clinical changes in a diagnosed patient with AD-CMD over three *** SUMMARY A 17-mo-old boy presented with progressive nasal obstruction,snoring and hearing loss *** examination showed enlargement of the head circumference and clinical features such as wide nasal bridge,paranasal bossing,widely spaced eyes with an increased bizygomatic width,and a prominent *** patient underwent otolaryngological examination,endoscopy,hearing test,laboratory examination of phosphorus and bone metabolism,cranial and femoral computed tomography,X-ray and next-generation *** patient was diagnosed with AD-CMD due to ***377 deletion(c.1129_1131del)on exon 9 of the ANKH *** adherence to a prescribed low-calcium diet,the boy’s alkaline phosphatase(ALP)levels continuously decreased to within the normal ***,after 14 mo of dietary intervention,his parents altered his diet to an intermittent low-calcium diet to include milk and *** patient’s ALP was slightly higher than normal after the dietary change but remained close to the normal *** serum osteocalcin changed to within normal levels after dietary regulation for 33 *** serum combined beta C-terminal telopeptide of type I collagen also continuously decreased after the nutritional intervention,although still slightly higher than normal *** fluctuating blood test results,the boy’s nasal symptoms were markedly relieved

关键词： ANKH gene mutation Clinical investigation Craniometaphyseal dysplasia Case report Child

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Cyclic Pulsating Pressure Enhanced Segregating Structuration of Ultra-High Molecular Weight Polyethylene/Graphene Composites as High-performance Light-Weight EMI Shields

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Chinese Journal of Polymer Science 2024年第7期42卷 958-967,I0008页

作者： Yun-Zhi Huang xiao-xiao Liu lan-Wei Li Guang-Ming Huang Zhao-Xia Huang Jin-ping Qu National Engineering Research Center of Novel Equipment for Polymer Processing Key Laboratory of Polymer Processing EngineeringMinistry of EducationGuangdong Provincial Key Laboratory of Technique and Equipment for Macromolecular Advanced ManufacturingSchool of Mechanical and Automotive EngineeringSouth China University of TechnologyGuangzhou 510641China School of Advanced Manufacturing Technology Guangdong Mechanical&Electrical PolytechnicGuangzhou 510550China

Currently,the enhancement in electromagnetic interference(EMI)performance of polymeric composite generally relies on either improving electrical conductivity(σ)for stronger electromagnetic(EM)reflections or tailoring structure for higher EM ***,we proposed a novel technique called cyclic pulsating pressure enhanced segregating structuration(CPP-SS),which can reinforce these two factors *** structural information was supplied by optical microscopy(OM)and scanning electron microscopy(SEM),both of which confirmed the formation and evolution of segregate structured ultra-high molecular weight polyethylene(UHMWPE)/graphene ***,the result showed that CPP-SS can significantly improve theσof ***,advanced specific EMI shielding efficiency of 31.1 d B/mm was achieved for UHMWPE/graphene composite at 1-mm thickness and a low graphene loading of 5 wt%.Meanwhile,it also confirmed that the intrinsic disadvantage of poor mechanical properties of conventional segregated structure composites can be *** work is believed to provide a fundamental understanding of the structural and performance evolutions of segregated structured composites prepared under CPPSS,and to bring us a simple and efficient approach for fabricating high-performance,strong and light-weight polymeric EMI shields.

关键词： Cyclic pulsating pressure Segregated structure Ultra-high molecular weight polyethylene Graphene Electromagnetic interface

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Poly(3-hexylthio) thiophene Field-effect Transistor Device Performance: Impact of the Content of Hexylthio Side Chain on Backbone

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Chinese Journal of Polymer Science 2024年第1期42卷 14-23,I0005页

作者： Hao-Yu Liu Lian-He Han Ling-Wei Kong Li-ping Zhu xiao-lan Qiao Mei-Fang Zhu State Key Laboratory for Modification of Chemical Fibers and Polymer Materials College of Materials Science and EngineeringDonghua UniversityShanghai 201620China School of Electronic Information and Electrical Engineering Shanghai Jiao Tong UniversityShanghai 200240China

Regioregular poly(3-hexylthio)thiopene(P3HTT) has emerged tremendous potential in organic electronic applications due to the strong noncovalent interactions from the sulfur atom linked to thiophene. However, P3HTT generally exhibits low charge mobility mostly due to poor solution processability attributed to dense arrangement of hexylthio side chain in polymer, which led to strong noncovalent interactions among sulfur atoms. To balance the nonvalent interaction and aggregation for P3HTT, herein, we systematically study the effect of hexylthio side chain content in polymer backbone on the structure and properties. A series of regioregular P3HTT-based homopolymers(P3HTT, P3HTT-50,P3HTT-33 and P3HTT-25) were prepared via Kumada catalyst transfer polycondensation method from a set of mono-, bi-, ter-and quarterthiophenes containing different contents of hexylthio side chain. The DFT calculation shows the planarity of polymers backbone could be improved through reducing the density of hexylthio side chain in polymer mainchain. And significant changes in their crystallinity, aggregation and optical properties were observed with the content of hexylthio side chain reducing. The P3HTT-33 displayed the highest field-effect transistor hole mobility of 2.83×10^(-2) cm^(2)·V^(-1)·s^(-1) resulting from a balance between the crystallinity and planarity. This study demonstrates modulating the content of hexylthio side chain in P3HTT is an effective strategy to optimize the opto-electronic properties of polymer obtaining excellent semiconductor device performance.

关键词： Conjugated polymer Polythiophene Noncovalent interaction Molecular aggregation Organic field-effect transistor

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Advances in cell sources of hepatocytes for bioartificial liver

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Hepatobiliary & Pancreatic Diseases International 2012年第6期11卷 594-605页

作者： xiao-ping Pan , lan-Juan Li State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China State Key Laboratory for Diagnosis and Treatment of Infectious Diseases First Affiliated Hospital Zhejiang University School of Medicine Hangzhou 310003 China

BACKGROUND: Orthotopic liver transplantation (OLT) is the most effective therapy for liver failure. However, OLT is severely limited by the shortage of liver donors. Bioartificial liver (BAL) shows great potential as an alternative therapy for liver failure In recent years, progress has been made in BAL regarding genetically engineered cell lines, immortalized human hepatocytes, methods for preserving the phenotype of primary human hepatocytes, and other functional hepatocytes derived from stem cells. DATA SOURCES: A systematic search of PubMed and ISI Web of Science was performed to identify relevant studies in English language literature using the Key words such as liver failure bioartificial liver, hepatocyte, stem cells, differentiation, and immortalization. More than 200 articles related to the cell sources of hepatocyte in BAL were systematically reviewed. RESULTS: Methods for preserving the phenotype of primary human hepatocytes have been successfully developed. Many genetically engineered cell lines and immortalized human hepatocytes have also been established. Among these cell lines the incorporation of BAL with GS-HepG2 cells or alginate encapsulated HepG2 cells could prolong the survival time and improve pathophysiological parameters in an animal model of liver failure. The cBAL111 cells were evaluated using the AMC-BAL bioreactor, which could eliminate ammonia and lidocaine, and produce albumin. Importantly, BAL loading with HepLi-4 cells could significantly improve the blood biochemical parameters, and prolong the survival time in pigs with liver failure. Other functional hepatocytes differentiated from stem cells, such as human liver progenitor cells, have been successfully achieved. CONCLUSIONS: Aside from genetically modified liver cell lines and immortalized human hepatocytes, other functionalhepatocytes derived from stem cells show great potential as cell sources for BAL. BAL with safe and effective liver cells may be achieved for clinical liver fa

关键词： bioartificial liver liver failure hepatocyte liver cell source stem cell differentiation immortalization

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Key challenges to the development of extracorporeal bioartificial liver support systems

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Hepatobiliary & Pancreatic Diseases International 2012年第3期11卷 243-249页

作者： Li-Fu Zhao, xiao-ping Pan and lan-Juan Li State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China State Key Laboratory for Diagnosis and Treatment of Infectious Diseases First Affiliated Hospital Zhejiang University School of Medicine Hangzhou 310003 China

BACKGROUND: For nearly three decades, extracorporeal bioartificial liver (BAL) support systems have been anticipated as promising tools for the treatment of liver failure. However, these systems are still far from clinical application. This review aimed to analyze the key challenges to the development of BALs. DATA SOURCE: We carried out a PubMed search of Englishlanguage articles relevant to extracorporeal BAL support systems and liver failure. RESULTS: Extracorporeal BALs face a series of challenges. First, an appropriate cell source for BAL is not readily available. Second, existing bioreactors do not provide in vivolike oxygenation and bile secretion. Third, emergency needs cannot be met by current BALs. Finally, the effectiveness of BALs, either in animals or in patients, has been difficult to document. CONCLUSIONS: Extracorporeal BAL support systems are mainly challenged by incompetent cell sources and flawed bioreactors. To advance this technology, future research is needed to provide more insights into interpreting the conditions for hepatocyte differentiation and liver microstructure formation.

关键词： bioartificial liver liver failure cell source bioreactor

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Bidirectional effects of the tryptophan metabolite indole-3-acetaldehyde on colorectal cancer

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World Journal of Gastrointestinal Oncology 2024年第6期16卷 2697-2715页

作者： Ze Dai Kai-Li Deng xiao-Mei Wang Dong-Xue Yang Chun-lan Tang Yu-ping Zhou Department of Gastroenterology The First Affiliated Hospital of Ningbo UniversityNingbo 315020Zhejiang ProvinceChina Health Science Center Ningbo UniversityNingbo 315211Zhejiang ProvinceChina School of Traditional Chinese Medicine Southern Medical UniversityGuangzhou 510515Guangdong ProvinceChina Institute of Digestive Disease of Ningbo University Ningbo UniversityNingbo 315020Zhejiang ProvinceChina Ningbo Key Laboratory of Translational Medicine Research on Gastroenterology and Hepatology Ningbo Key LaboratoryNingbo 315020Zhejiang ProvinceChina

BACKGROUND Colorectal cancer(CRC)has a high incidence and *** studies have shown that indole derivatives involved in gut microbiota metabolism can impact the tumorigenesis,progression,and metastasis of *** To investigate the effect of indole-3-acetaldehyde(IAAD)on *** The effect of IAAD was evaluated in a syngeneic mouse model of CRC and CRC cell lines(HCT116 and DLD-1).Cell proliferation was assessed by Ki-67 fluorescence staining and cytotoxicity *** apoptosis was analysed by flow cytometry after staining with Annexin V-fluorescein isothiocyanate and propidium *** was investigated using the transwell *** blotting and real-time fluorescence quantitative polymerase chain reaction were performed to evaluate the expression of epithelial-mesenchymal transition related genes and aryl hydrocarbon receptor(AhR)downstream *** PharmMapper,SEA,and SWISS databases were used to screen for potential target proteins of IAAD,and the core proteins were identified through the String *** IAAD reduced tumorigenesis in a syngeneic mouse *** CRC cell lines HCT116 and DLD1,IAAD exhibited cytotoxicity starting at 24 h of treatment,while it reduced Ki67 expression in the *** results of flow cytometry showed that IAAD induced apoptosis in HCT116 cells but had no effect on DLD1 cells,which may be related to the activation of *** can also increase the invasiveness and epithelial-mesenchymal transition of HCT116 and DLD1 *** low concentrations(<12.5μmol/L),IAAD only exhibited cytotoxic effects without promoting cell *** addition,predictions based on online databases,protein-protein interaction analysis,and molecular docking showed that IAAD can bind to matrix metalloproteinase-9(MMP9),angiotensin converting enzyme(ACE),poly(ADP-ribose)polymerase-1(PARP1),matrix metalloproteinase-2(MMP2),and myeloperoxidase(MPO).CONCLUSION Indole-3-aldehyde can induce cell apoptosis and inhibit cell prol

关键词： Indole-3-acetaldehyde Colorectal cancer Tryptophan metabolism Apoptosis Epithelial-mesenchymal transition

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Emerging mechanisms and implications of c GAS-STING signaling in cancer immunotherapy strategies

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Cancer Biology & Medicine 2024年第1期21卷 45-64页

作者： Jiawen Zhang Sihui Yu Qiao Peng ping Wang lan Fang Tongji University Cancer Center Shanghai Tenth People’s HospitalSchool of MedicineTongji UniversityShanghai 200072China Reproductive Medicine Center Department of Obstetrics and GynecologyShanghai General HospitalShanghai Jiao Tong University School of MedicineShanghai 201620China Department of Obstetrics and Gynecology Zhongshan HospitalFudan UniversityShanghai 200032China

The intricate interplay between the human immune system and cancer development underscores the central role of immunotherapy in cancer *** this landscape,the innate immune system,a critical sentinel protecting against tumor incursion,is a key *** cyclic GMP-AMP synthase(c GAS)and stimulator of interferon genes(STING)pathway has been found to be a linchpin of innate immunity:activation of this signaling pathway orchestrates the production of type I interferon(IFN-α/β),thus fostering the maturation,differentiation,and mobilization of immune effectors in the tumor ***,STING activation facilitates the release and presentation of tumor antigens,and therefore is an attractive target for cancer *** strategies to activate the STING pathway,including use of pharmacological agonists,have made substantial advancements,particularly when combined with immune checkpoint *** approaches have shown promise in preclinical and clinical settings,by enhancing patient survival *** review describes the evolving understanding of the c GAS-STING pathway's involvement in tumor biology and ***,this review explores classical and non-classical STING agonists,providing insights into their mechanisms of action and potential for optimizing immunotherapy *** challenges and complexities,the c GAS-STING pathway,a promising avenue for enhancing cancer treatment efficacy,has the potential to revolutionize patient outcomes.

关键词： cGAS-STING pathway type I interferon cyclic dinucleotide STING agonist cancer immunotherapy

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Burning Numbers of Barbells

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Acta Mathematicae Applicatae Sinica 2024年第2期40卷 526-538页

作者： Hui-qing LIU Rui-ting ZHANG xiao-lan HU Hubei Key Laboratory of Applied Mathematics&Faculty of Mathematics and Statistic Hubei UniversityWuhan 430062China Hubei Key Laboratory of Mathematical Sciences&School of Mathematics and Statistics Central China Normal UniversityWuhan 430079China

Motivated by a discrete-time process intended to measure the speed of the spread of contagion in a graph,the burning number b(G)of a graph G,is defined as the smallest integer k for which there are vertices x1,…xk su... 详细信息

关键词： burning number short/long barbell isometric subtree cycle

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Regulated polarization degree of upconversion luminescence and multiple anti-counterfeit applications

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Rare Metals 2024年第5期43卷 2172-2183页

作者： Dong-ping Wen ping Chen Yi Liang xiao-Ming Mo Cao-Feng Pan Center On Nanoenergy Research Guangxi Key Laboratory for Relativistic AstrophysicsSchool of Physical Science and TechnologyGuangxi UniversityNanning 530004China Key Laboratory of Micronano Energy and Sensor CAS Center for Excellence in NanoscienceBeijing Institute of Nanoenergy and NanosystemsChinese Academy of SciencesBeijing 100140China

Polarization upconversion luminescence(PUCL)of lanthanide ions(Ln^(3+))has been widely used in single particle tracking,microfluidics detection,three-dimensional displays,and so ***,no effective strategy has been developed for modulating *** we report a strategy to regulate PUCL in Ho^(3+)-doped NaYF4single nanorods based on the number of upconversion *** constructing a multiphoton upconversion system for Ho^(3+),we regulate the degree of polarization(DOP)of PUCL from 0.590 for two-photon luminescence to 0.929 for three-photon upconversion luminescence(UCL).Furthermore,our strategy is verified from cross-relaxation between Ho^(3+)and Yb^(3+),excitation wavelength,excitation power density,and local site *** this regulation strategy of PUCL has also been achieved in Tm^(3+),with the DOP ranging from 0.233 for two-photon luminescence to 0.925 for four-photon UCL Besides,multi-dimensional anti-counterfeiting display has been explored with *** work provides an effective strategy for regulating PUCL and also provides more opportunities for the development of polarization display optical encoding,anti-counterfeiting,and integrated optical devices.

关键词： Polarization upconversion luminescence Degree of polarization Population density of excited state Number of upconversion photons Polarization anti-counterfeitingdisplay

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Analysis of China's Electricity Price and Electricity Burden of Basic Industries under the Carbon Peak Target before 2030

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CSEE Journal of Power and Energy Systems 2024年第2期10卷 481-491页

作者： Yue Qiu Suyang Zhou Wei Gu xiao-ping Zhang School of Electrical Engineering Southeast UniversityNanjingChina School of Electronic Electrical and System EngineeringUniversity of BirminghamU.K.

The Chinese government is deepening reformation of electricity prices during the 14th Five Year Plan period and has set a carbon emission reduction target of reaching carbon peak before *** this context,will the carbon emission target influence electricity pricing and will electricity price influence competitiveness of Chinese main industries are two questions needing to be *** paper compares China's electricity price level with the selected major countries in the world,and four typical industries are selected to evaluate their electricity burden ***,the correlation between residential electricity price and industrial electricity price and the influencing factors is analyzed,from the perspectives of scale,structure and *** to the model obtained by regression analysis,the electricity price level and corresponding residential and industrial electricity burden in 2025 and 2030 are *** Terms-Electricity burden,industrial electricity price,regression analysis,residential electricity price.

关键词： Electricity burden industrial electricity price regression analysis residential electricity price

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