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Characterization of the molecular dysfunctions occurring in Aicardi-Goutieres syndrome patients with mutations in ADAR1

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Genes & Diseases 2024年第3期11卷 116-119页

作者： Sofan Al wardat Loredana Frasinlli Elisa Orecchini Federica Rey Silvia Anna Ciafre Silvia Galardi Jessica Garau Stella Gagliardi Simona Orcesi Davide Tonduti Stephana Carelli cristina Cereda Ernesto Picardi Alessandro Michienzi Department of Biomedicine and Prevention University of Rome Tor VergataRome 00133Italy Pediatric Clinical Research Center"Romeo ed Enrica Invernizzi" Department of Biomedical and Clinical SciencesUniversity of MilanoMilano 20157Italy Center of Functional Genomics and Rare Diseases Department of PediatricsBuzzi Children's HospitalMilano20154Italy Department of Child Neurology and Psychiatry IRccs Mondino FoundationPavia 27100Italy Unit of Pediatric Neurology C.O.A.L.A(Center for Diagnosis and Treatment of Leukodystrophies)Buzzi Children's HospitalMilano 20154Italy Department of Biosciences Biotechnologies and EnvironmentUniversityof Bari"AldoMoro"Bari 70125Italy Institute of Biomembranes Bioenergetics and Molecular Biotechnologies(IBIOM)National Research Council(CNR)Bari 70126Italy Biostructures and Biosystems National Institute(INBB) Rome00136Italy Institute of Biochemistry and Cell Biology IBBC-CNRCampus Adriano Buzzati TraversoRome 00015Italy

Aicardi-Goutieres syndrome(AGS)is a systemic inflammatory disorder caused by mutations in any one of the nine different genes,whose deficiency provokes a type I(interferon)IFN response probably central to pathogenesis.^(1) ADAR1,one of the genes mutated in AGS(AGS6),encodes for an enzyme that belongs to the ADAR family(ADAR1,ADAR2,and ADAR3)that catalyzes the conversion of adenosine to inosine within double-stranded RNAs(dsRNAs)(RNA editing A-to-1).

关键词： ADAR1 interferon res

Epidemiology,therapy and outcome of hepatocellular carcinoma between 2010 and 2019 in Piedmont,Italy

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World Journal of Gastrointestinal Oncology 2024年第3期16卷 761-772页

作者： Christian Bracco Marta Gallarate Marco Badinella Martini Corrado Magnino Salvatore D'Agnano Roberta Canta Giulia Racca Remo Melchio cristina Serraino Valentina Polla Mattiot Giovanni Gollè Luigi Fenoglio Department of Internal Medicine Santa Croce e Carle General HospitalCuneo 12100Italy Department of Medical Sciences "City of Health and Science"University HospitalTorino 10100Italy

BACKGROUND Hepatocellular carcinoma(HCC)is the most common primary liver malignancy and the second leading cause of cancer deaths *** is often diagnosed at an advanced stage and therefore its prognosis remains poor with a low 5-year survival *** patients have increasingly complex and constantly changing characteristics,thus up-to-date and comprehensive data are *** To analyze the epidemiology and main clinical characteristics of HCC patients in a referral center hospital in the northwest of Italy between 2010 and *** In this retrospective study,we analyzed the clinical data of all consecutive patients with a new diagnosis of HCC recorded at"Santa Croce e Carle"Hospital in Cuneo(Italy)between 1 January 2010 and 31 December *** highlight possible changes in HCC patterns over the 10-year period,we split the population into two 5-year groups,according to the diagnosis period(2010-2014 and 2015-2019).RESULTS Of the 328 HCC patients who were included(M/F 255/73;mean age 68.9±11.3 years),154 in the first period,and 174 in the *** C virus infection was the most common HCC risk factor(41%,135 patients).The alcoholic etiology rate was 18%,the hepatitis B virus infection etiology was 5%,and the non-viral/non-alcoholic etiology rate was 22%.The Child-Pugh score distribution of the patients was:class A 75%,class B 21%and class C 4%.The average Mayo end-stage liver disease score was 10.6±3.7.A total of 55 patients(17%)were affected by portal vein thrombosis and 158(48%)by portal *** average nodule size of the HCC was 4.6±3.1 cm.A total of 204 patients(63%)had more than one nodule<3,and 92%(305 patients)had a non-metastatic stage of the *** Barcelona Clinic Liver Cancer(BCLC)staging distribution of all patients was:4%very early,32%early,23%intermediate,34%advanced,and 7%*** survival rate was 1.6±0.3 *** 20%of the patients underwent ***,presence of ascites,BCLC stage and therapy w

关键词： Hepatocellular carcinoma Hepatitis virus Alcoholic hepatitis Non-alcoholic fatty liver disease Diagnosis Etiology Treatment

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大型科技公司与货币政策的信贷渠道

新金融 2024年第9期 23-28页

作者： Fiorella De Fiore Leonardo Gambacorta cristina Manea 国际清算银行货币和经济部

近期,多家大型科技公司已开始在电子商务平台上为中间产品企业(卖家)提供融资服务(大型科技信贷)。与银行依赖抵押品的传统贷款模式不同,这些大型科技公司能够通过将违约企业从其平台上移除等措施来增强贷款的回收能力。研究表明:当大... 详细信息

近期,多家大型科技公司已开始在电子商务平台上为中间产品企业(卖家)提供融资服务(大型科技信贷)。与银行依赖抵押品的传统贷款模式不同,这些大型科技公司能够通过将违约企业从其平台上移除等措施来增强贷款的回收能力。研究表明:当大型科技公司提高其匹配效率时,不仅增强了企业在平台上的交易价值,也提升了大型科技信贷的可获得性;大型科技信贷有助于缓解货币冲击对产出的初始影响,但同时也延长了产出波动的持续时间;尽管大型科技公司通过提升效率为电子商务平台用户带来了好处,但它们从用户那里收取的费用限制了这些效率进一步提升的潜力。

关键词：大型科技公司科技信贷货币政策信贷摩擦

Metastatic pancreatic and lung cancer patient in complete remission following immunotherapy: A case report and review of literature

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World Journal of Gastrointestinal Oncology 2024年第5期16卷 2233-2240页

作者： Joaquina Martínez-Galán cristina Jiménez-Luna Isabel Rodriguez Elisabeth Maza Carlos García-Collado Antonio Rodríguez-Fernández Javier Luis López-Hidalgo Octavio Caba Department of Medical Oncology Virgen de las Nieves University HospitalGranada 18014Spain Instituto de Investigación Biosanitaria Ibs.GranadaGranada 18012Spain Institute of Biopathology and Regenerative Medicine(IBIMER) University of GranadaGranada 18016Spain Pharmacogenetics Unit Virgen de las Nieves University HospitalGranada 18014Spain Department of Medicina Nuclear Virgen de las Nieves University HospitalGranada 18014Spain Department of Anatomía Patológica Virgen de las Nieves University HospitalGranada 18014Spain

BACKGROUND Metastatic pancreatic ductal adenocarcinoma(PDAC)is a lethal malignancy with dispiriting survival *** is a promising approach to many cancer types,but achieves poor outcomes in advanced PDAC due to its immunosuppressive tumor *** describe a case of metastatic PDAC effectively treated with *** SUMMARY We report the case of a 67-year-old woman with unresectable locally advanced PDAC,treated with gemcitabine plus nab-paclitaxel followed by radiotherapy plus *** nine months,pancreatic tumor progression was observed at the level of the hepatic hilum with the appearance of a new pulmonary nodule suggestive of a second primary,confirmed by left lung *** immunotherapy was then initiated with pembrolizumab,an immune checkpoint inhibitor targeting programmed cell death protein-1 that covers the two tumor *** patient showed a complete metabolic response that was maintained throughout the *** patient continues to be disease-free at 5.6 years since the start of *** These results suggest that the administration of pembrolizumab after chemoradiotherapy has a beneficial effect in patients with metastatic *** our knowledge,this is the first reported case of a patient with metastatic PDAC and metastatic lung cancer showing such a long-lasting complete response after pembrolizumab treatment without curative *** studies are required to determine biomarkers that identify PDAC patients most likely to benefit from this immunotherapy.

关键词： Pancreatic ductal adenocarcinoma Lung cancer Immunotherapy Pembrolizumab Programmed cell death protein-1 Case report

Forecasting tropical cyclone coastal and marine hazards and impacts

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TROPICAL CYCLONE RESEARCH AND REVIEW 2024年第1期13卷 1-14页

作者： Kohno, Nadao Fritz, Cody Sharma, Monica Berg, Robbie Greenslade, Diana Telford, Devon Rabitu, Sakeasi Mohapatra, M. Uson, Maria cristina C. Japan Meteorol Agcy Tokyo Japan Natl Hurricane Ctr Miami FL USA Indian Meteorol Dept New Delhi India Bur Meteorol Melbourne Australia Environm & Climate Change Canada Quebec City PQ Canada Fiji Meteorol Serv Nadi Fiji Philippine Atmospher Geophys & Astron Serv Adm Quezon City Philippines

The report highlights the significant progress over various regions with respect to understanding of coastal hazards, numerical modeling techniques and the generation & dissemination of coastal hazard warnings and products. The developments over various regions in the globe during 2014-18 have been discussed in this report as presented during 10th Session of International Workshop on Tropical Cyclones (IWTC-X) at Bali, Indonesia. More specifically, various regions have started to confront the uncertainty that cannot be removed from TC analyses and forecasts and further communicate those hazards within the context of risk [probabilistic] based information. Progress also includes impact-based forecasts such as communicating coastal inundation information relative to total water level instead of storm surge, specifically (i.e., anomaly from astronomical tide and waves). Lastly, updates to model grid configuration, model resolution, and coupled dynamical systems continue to resolve the costal hazards more effectively. Those approaches have likely helped reduce loss of life relative to historical standards. However, regions agree that the generation and dissemination of coastal hazard information still need to be improved in view of growing population along the coast and thus increased exposure of life to coastal hazard. (c) 2024 The Shanghai Typhoon Institute of China Meteorological Administration. Publishing services by Elsevier B.V. on behalf of KeAi Communication Co. Ltd. This is an open access article under the CC BY-NC-ND license (http://***/licenses/by-nc-nd/4.0/).

关键词： Coastal inundation Storm surge Probabilistic forecast STORM-SURGE UNITED-STATES EAST-COAST INUNDATION BAY CANADA BENGAL MODEL

Sea Cucumber Fishery in Aurora, Philippines

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Open Journal of Ecology 2024年第2期14卷 101-113页

作者： Maria cristina B. Cañada Michelle A. Resueño Eusebio V. Angara Department of Forestry and Environmental Sciences Aurora State College of Technology Baler Aurora Philippines

This study was conducted to provide a preliminary assessment of the sea cucumber fishery in Aurora, Philippines. This was conducted from February to June 2013. All sea cucumber gatherers and traders, obtained through snowball sampling, in each identified sea cucumber fishery area in six coastal municipalities (Baler, Dipaculao, Dinalungan, Casiguran, Dilasag and Dingalan) were interviewed using a structured interview schedule and focused group discussions (FGD). Results revealed that gathering sea cucumbers were accomplished by handpicking, skin diving or compressor diving. A total of 15 commercial species were regularly gathered. Out of this, three species are of very high economic value (≥Php 4000/kg) (Holothuria nobilis, Holothuria scabra, and Actinopyga lecanora). The average number of kilograms harvested per hour per trip ranges from 0.21 (Holothuria fuscopunctata) to 2.71 (Holothuria scabra), while the average size of commonly gathered sea cucumber varies among species with Holothuria lecanora showed an average length (29.26 cm) greater than recorded 24 cm. Processing mainly involves washing, slicing, boiling, drying and packing. Processed sea cucumbers or trepan were sold to middlemen or direct buyers who usually offered higher prices (36.36% up to 69.20%) than the former. Problems encountered by sea cucumber gatherers and traders include a decline in total catch, a shift in area of collection from intertidal zones to deeper waters, an increase in the number of hours spent in finding and gathering sea cucumbers, the use of compressors in gathering, the decline of larger species and gathering of smaller sizes in huge amount, the absence of conservation program for sea cucumber, and the lenient implementation of government policies with regards to coastal resources management.

关键词： Assessment Sea Cucumber Trepang Fishery

Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort

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World Journal of Transplantation 2024年第1期14卷 112-119页

作者： Melina Utz Melere Valberto Sanha Marco Farina Carolina Soares da Silva Luiza Nader Cristine Trein Angelica Maria Lucchese cristina Ferreira Antonio Nocchi Kalil Flavia Heinz Feier Department of Hepatology and Liver Transplantation Santa Casa de Porto AlegrePorto Alegre 90050170Brazil Department of Hepato-biliary-pancreatic Surgery and Liver Transplantation Irmandade Santa Casa de Misericórdia de Porto AlegrePorto Alegre 90020-090Brazil Department of Surgical Oncology Santa Rita Hospital/Santa Casa de Misericórdia de Porto AlegrePorto Alegre 90050-170Rio Grande do SulBrazil

BACKGROUND Biliary atresia(BA)is the most common indication for pediatric liver transplantation,although portoenterostomy is usually performed ***,due to the high failure rate of portoenterostomy,liver transplantation has been advocated as the primary procedure for patients with *** is still unclear if a previous portoenterostomy has a negative impact on liver transplantation *** To investigate the effect of prior portoenterostomy in infants un-dergoing liver transplantation for *** This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in *** with BA were divided into two groups:Those undergoing primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior *** variables were compared using the Student’s t-test or the Kruskal-Wallis test,and categorical variables were compared using theχ2 or Fisher’s exact test,as *** Cox regression analysis was performed to determine risk factors for portal vein *** and graft survival analyses were conducted with the Kaplan–Meier product-limit estimator,and patient subgroups were compared using the two-sided log-rank *** Forty-two patients were included in the study(25[60%]girls),23 undergoing liver transplantation without prior portoenterostomy,and 19 undergoing liver transplantation with prior *** with prior portoenterostomy were older(12 vs 8 months;P=0.02)at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores(13.2 vs 21.4;P=0.01).The majority of the patients(35/42,83%)underwent livingdonor liver *** group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis(39 vs 11%),but this result did not reach statistical *** portoenterostomy

关键词： Hepatic portoenterostomy Biliary atresia Liver transplantation Patient outcome assessment Portal vein Survival

Human leukocyte antigen compatibility and incidence of donorspecific antibodies in pediatric liver transplant recipients

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World Journal of Gastroenterology 2024年第33期30卷 3837-3845页

作者： Melina U Melere Flavia H Feier Jorge Neumann Antônio N Kalil Juliana de M Montagner Luiza S Nader Carolina S da Silva Marco Aurélio F Junior Gabriela P Coral Guilherme P Bobsin cristina T Ferreira Department of Hepatology and Liver Transplantation Santa Casa de Porto AlegrePorto Alegre 90050170Rio Grande do SulBrazil Department of Hepato-biliary-pancreatic Surgery and Liver Transplantation Irmandade Santa Casa de Misericórdia de Porto AlegrePorto Alegre 90020-090Rio Grande do SulBrazil Postgraduation Program in Medicine:Hepatology Federal University of Health Sciences of Porto AlegrePorto Alegre 90050-170Rio Grande do SulBrazil Laboratory of Transplantation Immunology Irmandade Santa Casa de Misericórdia de Porto AlegrePorto Alegre 90020-090Rio Grande do SulBrazil

BACKGROUND Antibody-mediated rejection following liver transplantation(LT)has been increasingly recognized,particularly with respect to the emergence of de novo donor-specific antibodies(DSAs)and their impact on graft *** substantial evidence for adult populations exists,research focusing on pediatric LT outcomes remains *** To investigate the prevalence of human leukocyte antigen(HLA)mismatches and DSA and evaluate their association with rejection episodes after pediatric *** A cohort of pediatric LT recipients underwent HLA testing at Santa Casa de Porto Alegre,Brazil,between December 2013 and December *** patients who survived for>30 days after LT with at least one DSA analysis were *** classes I and II and cross-matches were *** presence of de novo DSA(dnDSA)was evaluated at least 3 months after LT using the Luminex®single antigen bead method,with a positive reaction threshold set at 1000 *** episodes were confirmed by liver *** Overall,67 transplanted children were analyzed;61 received grafts from living donors,85%of whom were related to ***-transplant DSA(class I or II)was detected in 28.3%of patients,and dnDSA was detected in 48.4%.The median time to DSA detection after LT was 19.7[interquartile range(IQR):4.3-35.6]*** rejection occurred in 13 patients at follow-up,with C4d positivity observed in 5/13 Liver *** median time to rejection was 7.8(IQR:5.7-12.8)*** presence of dnDSA was significantly associated with rejection(36%vs 3%,P<0.001).The rejection-free survival rates at 12 and 24 months were 76%vs 100%and 58%vs 95%for patients with dnDSA anti-DQ vs those without,*** Our findings highlight the importance of incorporating DSA assessment into pre-and post-transplantation protocols for pediatric LT *** implications may include immunosuppression minimization strategies based on this analysis in pediatric LT recip

关键词： Human leukocyte antigens Donor-specific antibodies Liver transplantation Pediatric Rejection

Hydrocarbons in particulate samples from wildfire events in central Portugal in summer 2010

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Journal of Environmental Sciences 2017年第3期29卷 122-131页

作者： Ana Vicente Ana Calvo Ana P.Fernandes Teresa Nunes cristina monteiro Casimiro Pio Célia Alves Centre for Environmental and Marine Studies (CESAM) Department of EnvironmentUniversity of Aveiro Department of Physics IMARENABUniversity of León

In summer 2010, twenty eight （14 PM=2.5 samples plus 14 samples PM2.5-10） smoke samples were collected during wildfires that occurred in central Portugal. A portable high-volume sampler was used to perform the sampling, on quartz fibre filters of coarse （PM=2.5-10） and fine （PM2.5） smoke samples. The carbonaceous content （elemental and organic carbon） of particulate matter was analysed by a thermal-optical technique. Subsequently, the particulate samples were solvent extracted and fractionated by vacuum flash chromatog- raphy into three different classes of organic compounds （aliphatics, polycyclic aromatic hydrocarbons （PAHs） and carbonyl compounds）. The organic speciation was performed by gas chromatography-mass spectrometry （GC-MS）. Emissions were dominated by the fine particles, which represented around 92% of the PM10. A clear predominance of carbonaceous constituents was observed, with organic to elemental carbon （OC/EC） ratios ranging between 1.69 and 245 in both size fractions. The isoprenoid ketone 6,10,14-trimethyl-2-pentadecanone, a tracer for secondary organic aerosol formation, was one of the dominant constituents in both fine and coarse particles. Retene was the most abundant compound in all samples. Good correlations were obtained between OC and both aliphatic and PAH compounds. Pyrogenic processes, thermal release of biogenic com- pounds and secondary processing accounted for 97% of the apportioned PM2.5 levels.

关键词： WildfiresOrganic carbonOrganic compoundsBiomarkersParticulate matterPortugal