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Application of Gene Therapy in Hemophilia

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Current Medical Science 2022年第5期42卷 925-931页

作者： yue-fen HU Yun-hai FANG Yong-rong LAI Xiao-qin fenG Shu-qian XU Department of Hematology Qilu HospitalCheeloo College of MedicineShandong UniversityJinan 250014China Shandong Blood Center Shandong Hemophilia Treatment CenterJinan 250014China Department of Hematology the First Affiliated Hospital of Guangxi Medical UniversityNanning 530021China Nanfang Hospital Southern Medical UniversityGuangzhou 510000China

Gene therapy refers to introducing normal exogenous genes into target cells to correct or compensate for the diseases caused by defective and abnormal genes for the purpose of *** holds out hope of a cure for single-gene genetic diseases such as thalassemia,hemophilia,*** present,gene therapy is performed in two ways:introducing exogenous genes,and gene editing.A great number of clinical trials of gene therapy in hemophilia have been carried out using viral vectors to introduce foreign genes into target ***,the production of neutralizing antibodies following injection and the inability to prepare viral vectors in large quantities limit their *** gene-editing methods like CRISPR avoid the above problems,the potential risks of off-target effects are still *** trials and evidence are needed to elucidate the safety and accuracy of gene *** paper will review the bench and clinical work of gene therapy in hemophilia in recent years,and summarize the challenges and prospects of gene therapy,so as to provide directions for future scientific research in this field.

关键词： hemophilia gene therapy CRISPR-Cas9 application

Esophageal bronchogenic cyst excised by endoscopic submucosal tunnel dissection: A case report

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World Journal of Clinical Cases 2020年第2期8卷 353-361页

作者： fen-Ming Zhang Hong-Tan Chen Long-Gui Ning yue Xu Guo-Qiang Xu Department of Gastroenterology The First Affiliated Hospital Zhejiang University School of MedicineZhejiang University

BACKGROUND Esophageal bronchogenic cyst(EBC) is a rare congenital disease that is difficult to diagnose preoperatively, and treatment remains *** SUMMARY We report a 53-year-old Chinese woman hospitalized in our hospital following the discovery of a submucosal protruding mass of the esophagus by upper endoscopy. A preliminary diagnosis of EBC was made by endoscopic ultrasonography(EUS), and treatment was accomplished by endoscopic submucosal tunnel dissection(ESTD). The pathological results verified the diagnosis. No scar changes or cystic lesion within the original lesion were found under EUS after a 3-mo *** EUS is valuable for the preliminary diagnosis of EBC and surveillance. ESTD is a safe and effective treatment for EBC. Further evaluation of complications and long-term follow-ups are required.

关键词： Esophageal bronchogenic cyst Endoscopic ultrasonography Endoscopic submucosal tunnel dissection Diagnosis and treatment Case report

Anti-Yo antibody-positive paraneoplastic cerebellar degeneration in a patient with possible cholangiocarcinoma:A case report and review of the literature

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World Journal of Clinical Cases 2021年第17期9卷 4423-4432页

作者： yue Lou Shan-Hu Xu Si-Ran Zhang Qin-fen Shu Xiao-Li Liu Department of Neurology Affiliated Zhejiang HospitalZhejiang University School of MedicineHangzhou 310000Zhejiang ProvinceChina

Paraneoplastic cerebellar degeneration(PCD),which is rare in clinical practice,is closely related to *** positive for anti-Yo antibodies(anti-Purkinje cytoplasmic antibody 1)are the main subtype of *** is subacute cerebellar degeneration,and while it progresses over weeks to months,its resultant deficits last much *** patients with anti-Yo antibody-positive PCD are very *** of them are breast cancer or ovarian cancer patients but also occasionally lung cancer *** SUMMARY A 61-year-old woman presented with sudden vertigo,nausea,and vomiting for approximately 10 *** patient's neurological examination showed torsion with downbeat nystagmus and ataxia of the right limb and *** examination found that the patient's cerebrospinal fluid and serum were anti-Yo antibody-positive,positron emission tomography computed tomography showed an increased metabolic rate in the retroperitoneal lymph nodes,and the pathology of lymph node punctures in the retroperitoneum and neck suggested adenocarcinoma of the pancreaticobiliary duct,which strengthens the hypothesis of paraneoplastic *** immunoglobulin(IVIg)0.4 g/kg/d for 5 d and methylprednisolone 160 mg for 3 d were initiated,which was reduced to 80 mg for 3 d and then to 40 mg for 7 *** treatment with IVIg and a steroid,the patient's vertigo and ataxia *** The patient's vertigo and ataxia alleviated after treatment,suggesting that early immunotherapeutic intervention may have certain value in stopping neurological loss.

关键词： Paraneoplastic cerebellar degeneration Anti-Yo antibody Cholangiocarcinoma Case report

Efficacy and safety of non-penetrating glaucoma surgery with phacoemulsification versus non-penetrating glaucoma surgery:a Meta-analysis

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International Journal of Ophthalmology(English edition) 2021年第12期14卷 1970-1978页

作者： Jun-Yan Xiao An-Yi Liang yue-Lin Wang Gang-Wei Cheng Mei-fen Zhang Department of Ophthalmology Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijing 100730China

AIM:To compare the clinical efficacy and safety of non-penetrating glaucoma surgery(NPGS)plus phacoemulsification(Phaco-NPGS)and ***:We systematically searched various databases and reviewed studies that had evaluated the effects of Phaco-NPGS or NPGS-alone for patients with *** outcomes included postoperative intraocular pressure(IOP)and the number of postoperative antiglaucoma *** outcomes were the prevalence of complications,incidence of needling or goniopuncture,and surgical success ***:In total,380 and 424 eyes in NPGS-alone and Phaco-NPGS groups respectively were *** postoperative IOP and number of medications were significantly lowered in the Phaco-NPGS group than that in the NPDS-alone group[weighted mean difference(WMD)=-1.12,95%confidence interval(CI):-2.11 to-0.12,P=0.03;WMD=-0.31,95%CI:-0.53 to-0.09,P=0.006].Moreover,Phaco-NPGS had a significantly lower prevalence of complications and postoperative procedures compared to NPGS-alone,while no significant difference existed for surgical ***:Phaco-NPGS superior to NPGS-alone in the reduction of IOP and ***-NPGS can be recommended for glaucoma patients with coexisting cataracts owing to its superior efficacy,fewer complications,and postoperative procedures.

关键词： cataract glaucoma non-penetrating glaucoma surgery phacoemulsification Meta-analysis

NMR studies of stock process water and reaction pathways in hydrothermal carbonization of furfural residue

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Green Energy & Environment 2018年第2期3卷 163-171页

作者： fen yue Christian Marcus Pedersen Xiuyin Yan Yequn Liu Danlei Xiang Caifang Ning Yingxiong Wang Yan Qiao Institute of Coal Chemistry Chinese Academy of Sciences Institute of Process Engineering Chinese Academy of Sciences Department of Chemistry University of Copenhagen Taiyuan Supervision and Testing Station for Quality of Grain Department of Applied Biology and Chemical Technology The Hong Kong Polytechnic University

Hydrothermal carbonization(HTC) is a valuable approach to convert furfural residue(FR) into carbon material. The prepared biochars are usually characterized comprehensively, while the stock process water still remains to be studied in detail. Herein, a NMR study of the main components in stock process water generated at different HTC reaction conditions was reported. Various qualitative and quantitative NMR techniques(~1H and ^(13)C NMR,~1H-~1H COSY and ~1H-^(13)C HSQC etc.) especially 1D selective gradient total correlation spectroscopy(TOCSY NMR) were strategically applied in the analysis of HTC stock process water. Without separation and purification, it was demonstrated that the main detectable compounds are 5-hydroxymethylfurfural, formic acid, methanol, acetic acid, levulinic acid, glycerol, hydroxyacetone and acetaldehyde in this complicate mixture. Furthermore, the relationship between the concentration of major products and the reaction conditions(180-240 ℃ at 8 h, and 1-24 h at 240 ℃) was established. Finally, reasonable reaction pathways for hydrothermal conversion of FR were proposed based on this result and our previously obtained characteristics of biochars. The routine and challenging NMR methods utilized here would be an alternative other than HPLC or GC for biomass conversion research and can be extended to more studies.

关键词： NMR Hydrothermal carbonization Furfural residue Stock process water

Corrosion resistance of biodegradable polymeric layer-by-layer coatings on magnesium alloy AZ31

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Frontiers of Materials Science 2016年第2期10卷 134-146页

作者： Lan-yue CUI Rong-Chang ZENG Xiao-Xiao ZHU Ting-Ting PANG Shuo-Qi LI fen ZHANG College of Materials Science and Engineering Shandong University of Science and Technology Qingdao 266590 China State key Laboratory of Mining Disaster Prevention and Control Co-founded by Shandong Province and the Ministry of Science and Technology Shandong University of Science and Technology Qingdao 266590 China

Biocompatible polyelectrolyte multilayers （PEMs） and polysiloxane hybrid coatings were prepared to improve the corrosion resistance of biodegradable Mg alloy AZ31. The PEMs, which contained alternating poly（sodium 4-styrenesulfonate）（PSS） and poly（allylamine hydrochloride）（PAH）, were first self-assembled on the surface of the AZ31 alloy substrate via electrostatic interactions, designated as （PAH/PSS）s/AZ31. Then, the （PAH/PSS）5/AZ31 samples were dipped into a methyltrimethoxysilane （MTMS） solution to fabricate the PMTMS films, designated as PMTMS/（PAH/PSS）5/AZ31. The surface morphologies, microstructures and chemical compositions of the films were investigated by FE-SEM, FTIR, XRD and XPS. Potentiodynamic polarization, electro- chemical impedance spectroscopy and hydrogen evolution measurements demonstrated that the PMTMS/（PAH/PSS）s/AZ31 composite film significantly enhanced the corrosion resistance of the AZ31 alloy in Hank＇s balanced salt solution （HBSS）. The PAH and PSS films effectively improved the deposition of Ca-P compounds including Ca3（PO4）2 and hydroxyapatite （HA）. Moreover, the corrosion mechanism of the composite coating was discussed. These coatings could be an alternative candidate coating for biodegradable Mg alloys.

关键词： magnesium alloy polyelectrolyte polysiloxane corrosion layer-by-layer

Role of gut microbiota in primary biliary cholangitis

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Hepatobiliary & Pancreatic Diseases International 2022年第6期21卷 597-599页

作者： Wu-Meng Jin Si-yue Song Xiao-fen Xu Cheng-Ping Wen Chang-feng Hu College of Basic Medical Sciences Zhejiang Chinese Medical UniversityHangzhou 310053China

Primary biliary cholangitis(PBC,formerly known as primary biliary cirrhosis)is a chronic female-predominant inflammatory and cholestatic liver disease with a prolonged course and poor prognosis[1].It is characterized by anti-mitochondrial autoanti-body(AMA),immunoglobulin M and lymphocyte infiltration in the portal area of liver tissue.A recent systemic review has shown a steady rise both in incidence and prevalence of PBC,with 5.31/100000 persons and 40.2/100000 persons,respectively[2].It can cause fatigue,pruritus and is associated with a high risk of cirrhosis and liver failure if left untreated[3].The pathogenesis of PBC may be related to genetic,environmental,immune factors and individual susceptibility,but the specific etiology is not *** previous studies[4,5]have found that gut microbiota plays an important role in a variety of liver diseases,including *** bidirectional crosstalk between liver and microbiome appears to be crucial in maintaining health and could be therapeutically targeted,such as by fecal microbiota transplantation.

关键词： biliary cholangitis cirrhosis

Seasonal response of nitrogen exchange fluxes to crab disturbance at sediment-water interface in coastal tidal wetlands

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Water Science and Engineering 2024年第2期17卷 129-138页

作者： Guo-fen Hua Shang-qing Liu Xiang-dong Liu Jin-li Li yue Fang Wen-ting Xie Xiang Xu College of Water Conservancy and Hydroelectric Power Hohai UniversityNanjing 210098China College of Renewable Energy Hohai UniversityNanjing 210098China

Coastal wetlands are hotspots for nitrogen(N)cycling,and crab burrowing is known to transform N in intertidal marsh ***,the underlying mechanisms remain *** study conducted field experiments and used indoor control test devices to investigate the seasonal response of nitrogen to crab disturbance at the sediment-water interface in coastal tidal flat *** results showed that crab disturbance exhibited significant seasonality with large seasonal differences in cave density and *** to crab disturbance,nitrogen fuxes at the sediment-water interface were much greater in the box with crabs than in the box without *** summer,NH-N showed a positive flux from the sediment to the overlying water,but NO2-N and NOg-N showed positive fluxes from the sediment to the overlying water only in early *** winter,NH-N showed a positive flux from the sediment to the overlying water,but NO-N and NO,-N both exhibited positive and negative *** results indicated that the presence of crab burrows can cause the aerobic layer to move downward by approximately 8-15 cm in summer and directly promote nitrification at the sediment surface.

关键词： Coastal tidal flat wetland Crab disturbance Nitrogen flux Nitrification Denitrification Sediment-water interface

Microstructure evolutions of the W-TiC composite conducted by dual-effects from thermal shock and He-ion irradiation

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Tungsten 2019年第3期1卷 213-219页

作者： Yu-fen Zhou Xiao-yue Tan Lai-Ma Luo yue Xu Xiang Zan Qiu Xu Kazutoshi Tokunaga Xiao-Yong Zhu Yu-Cheng Wu School of Materials Science and Engineering Hefei University of TechnologyHefei 230009China Laboratory of Nonferrous Metal Material and Processing Engineering of Anhui Province Hefei 230009China Institute for Integrated Radiation and Nuclear Science Kyoto UniversityOsaka-fuKyoto 590-0494Japan Research Institute for Applied Mechanics Kyushu UniversityKasugaFukuoka 816-8580Japan National-Local Joint Engineering Research Centre of Nonferrous Metals and Processing Technology Hefei 230009China

Considering that tungsten(W)materials served as the plasma-facing material in the fusion reactor would be exposed to edge-localized modes(ELMs)-like thermal shock loading accompanied with He-ion irradiation,the W-TiC composite produced with a wet-chemical method was conducted by the dual effects from the laser beam thermal shock first and He-ion irradia-tion later in this *** microstructure changes of the W-TiC composite before and after two tests were characterized by scanning electron microscopy or transmission electron *** the laser beam thermal shock test,there was an obvious interface on the exposed surface of the W-TiC *** main cracks and melting areas could be found nearby the interface and center,***,a mixture of tungsten oxide and TiC was easy to aggregate and form into circle areas surrounding the melting *** thermal shock tested that W-TiC composite was then subjected to the He-ion *** typical features of fuzz structures could be detected on the surface of the W-TiC composite apart from the center of the melting ***,several nano-sized He bubbles deeply distributed at grain boundaries in the melting area,owing to the grain boundary functioning as the free path for He diffusion.

关键词： Plasma-facing materials Wet-chemical method W-TiC composite Thermal shock He-ion irradiation