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Symptomatic Val122del mutated hereditary transthyretin amyloidosis: Need for early diagnosis and prioritization for heart and liver transplantation

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Hepatobiliary & Pancreatic Diseases International 2021年第4期20卷 323-329页

作者： Adriano-valerio Schettini Laura Llado JulieK Heimbach JoseGonzalez Costello Marie Tranäng Olivier Van Caenegem Richard C Daly Peter Van den Bergh Carlos Casasnovas Joan fabregat John J Poterucha Maxime foguenne Bo Göran Ericzon Jan Lerut Institute for Experimental and Clinical Research(IREC) Catholic University of Louvain(UCL)Avenue Hippocrate 55Brussels 1200Belgium Liver Transplant Unit Department of SurgeryHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Division of Transplantation Surgery William J.von Liebig Transplant CenterMayo Clinic2001st St SWRochesterMN 55905USA Advanced Heart failure and Transplant Unit Cardiology DepartmentHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain familial Amyloidotic Polyneuropathy World Transplant Registry(fAPWTR) Division of Transplantation SurgeryKarolinska Universitetssjukhuset HuddingeF82Karolinska InstitutetStockholm 14186Sweden Cardiothoracic Intensive Care Unit Department of Cardiovascular DiseasesUniversity Hospitals Saint-Luc-UCLAvenue Hippocrate 10Brussels 1200Belgium Cardiovascular Surgery Department Mayo Clinic2001st St SWRochesterMN 55905USA Neuromuscular Reference Centre University Hospital Saint-Luc-UCLAvenue Hippocrate 10Brussels 1200Belgium Neuromuscular Unit Neurology DepartmentHospital Universitari de BellvitgeCalle de la Feixa LlargaBarcelona 08907Spain Division of Gastroenterology and Hepatology Mayo Clinic2001st St SWRochester MN 08907USA Division of Transplantation Surgery CLINTECKarolinska Universitetssjukhuset HuddingeF82Karolinska InstitutetStockholm 14186Sweden

Background: Hereditary transthyretin(ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding they reassemble as insoluble fibrils(i.e. amyloid). Apart from the common Val30 Met mutation there is a very heterogeneous group of non-Val30 Met mutations. In some cases, the clinical picture is dominated by a rapidly evolving restrictive and hypertrophic cardiomyopathy. Methods: A case series of four liver recipients with the highly clinically relevant, rare and particularly aggressive Val122 del mutation is presented. Medical and surgical therapeutic options, waiting list policy for ATTRv-amyloidosis, including the need for heart transplantation, and status of heart-liver transplantation are discussed. Results: Three patients needed a staged(1 patient) or simultaneous(2 patients) heart-liver transplant due to rapidly progressing cardiac failure and/or neurologic disability. Domino liver transplantation was impossible in two due to fibrotic hepatic transformation caused by cardiomyopathy. After a follow-up ranging from 3.5 to 9.5 years, cardiac(allograft) function was maintained in all patients, but neuropathy progressed in three patients, one of whom died after 80 months. Conclusions: This is the first report in(liver) transplant literature about the rare Val122 del ATTRv mutation. Due to its aggressiveness, symptomatic patients should be prioritized on the liver and, in cases with cardiomyopathy, heart waiting lists in order to avoid the irreversible neurological and cardiac damage that leads to a rapid lethal outcome.

关键词： Hereditary transthyretin amyloidosis Heart transplantation Liver transplantation Non-Val30Met mutation Val122del mutation Domino liver transplantation

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A monolithic single-chip point-of-care platform for metabolomic prostate cancer detection

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Microsystems & Nanoengineering 2021年第2期7卷 81-95页

作者： valerio f.Annese Samadhan B.Patil Chunxiao Hu Christos Giagkoulovits Mohammed A.Al-Rawhani James Grant Martin Macleod David J.Clayton Liam M.Heaney Ronan Daly Claudio Accarino Yash D.Shah Boon C.Cheah James Beeley Thomas R.Jeffry Evans Robert Jones Michael P.Barrett David R.S.Cumming Electronics and Nanoscale Engineering James Watt School of EngineeringUniversity of GlasgowGlasgowG128QQUK Beatson West of Scotland Cancer Centre GlasgowG120YNUK School of Science and Technology Nottingham Trent UniversityNottinghamNG118NFUK School of Sport Exercise&Health SciencesLoughborough UniversityLoughboroughLE113TUUK Glasgow Polyomics College of MedicalVeterinary and Life SciencesUniversity of GlasgowGlasgowG611BDUK Institute of Cancer Sciences Beatson West of Scotland Cancer CentreUniversity of GlasgowGlasgowG120YNUK Institute of Infection Immunity and InflammationUniversity of GlasgowGlasgowG128TAUK

There is a global unmet need for rapid and cost-effective prognostic and diagnostic tools that can be used at the bedside or in the doctor’s office to reduce the impact of serious *** cancers are diagnosed late,leading to costly treatment and reduced life *** prostate cancer,the absence of a reliable test has inhibited the adoption of screening *** report a microelectronic point-of-care metabolite biomarker measurement platform and use it for prostate cancer *** platform,using an array of photodetectors configured to operate with targeted,multiplexed,colorimetric assays confined in monolithically integrated passive microfluidic channels,completes a combined assay of 4 metabolites in a drop of human plasma in under 2 min.A preliminary clinical study using L-amino acids,glutamate,choline,and sarcosine was used to train a cross-validated random forest *** system demonstrated sensitivity to prostate cancer of 94%with a specificity of 70%and an area under the curve of *** technology can implement many similar assay panels and hence has the potential to revolutionize low-cost,rapid,point-of-care testing.

关键词： inhibited specificity hence

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意大利经济史从统一到今天

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2000年

作者：（意）瓦莱里奥·卡斯特罗诺沃（valerio Castronovo）沈珩译

本书叙述了自十九世纪中期意大利统一，经过19世纪末工业革命、两次世界大战直到当代的意大利经济史。

ISBN: (数字)710002918X

ISBN: (纸本)710002918X

本书叙述了自十九世纪中期意大利统一，经过19世纪末工业革命、两次世界大战直到当代的意大利经济史。

关键词：经济史意大利

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Author Correction:Low temperature chemical sintering of inkjet-printed Zn nanoparticles for highly conductive flexible electronic components

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npj flexible Electronics 2021年第1期5卷 276-276页

作者： Subimal Majee Mikael C.f.Karlsson Pawel Jerzy Wojcik Anurak Sawatdee Mohammad Yusuf Mulla Naveed ul Hassan Alvi Peter Dyreklev valerio Beni David Nilsson Published in partnership with Nanjing Tech University

The original version of this Article omitted from the author list the 3rd author Pawel Jerzy Wojcik from redoxme AB,Research&Development Department,Norrköping,Sweden(Affiliation#3)and 7th author Peter Dyreklev from RI... 详细信息

关键词： ping Nor Sweden

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Observatory science with eXTP

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Science China(Physics,Mechanics & Astronomy) 2019年第2期62卷 97-138页

作者： Jean J.M.in 't Zand Enrico Bozzo JinLu Qu Xiang-Dong Li Lorenzo Amati Yang Chen Immacolata Donnarumma Victor Doroshenko Stephen A.Drake Margarita Hernanz Peter A.Jenke Thomas J.Maccarone Simin Mahmoodifar Domitilla de Martino Alessandra De Rosa Elena M.Rossi Antonia Rowlinson Gloria Sala Giulia Stratta Thomas M.Tauris Joern Wilms Xuefeng Wu Ping Zhou Iván Agudo Diego Altamirano Jean-Luc Atteia Nils A.andersson M.Cristina Baglio David R.Ballantyne Altan Baykal Ehud Behar Tomaso Belloni Sudip Bhattacharyya Stefano Bianchi Anna Bilous Pere Blay Joao Braga Sφren Brandt Edward f.Brown Niccolo Bucciantini Luciano Burderi Edward M.Cackett Riccardo Campana Sergio Campana Piergiorgio Casella Yuri Cavecchi frank Chambers Liang Chen Yu-Peng Chen Jér?me Chenevez Maria Chernyakova ChiChuan Jin Riccardo Ciolfi Elisa Costantini Andrew Cumming Antonino D'Aì Zi-Gao Dai filippo D'Ammando Massimiliano De Pasquale Nathalie Degenaar Melania Del Santo valerio D'Elia Tiziana Di Salvo Gerry Doyle Maurizio falanga XiLong fan Robert D.ferdman Marco feroci federico fraschetti Duncan K.Galloway Angelo f.Gambino Poshak Gandhi MingYu Ge Bruce Gendre Ramandeep Gill Diego G?tz Christian Gouiffès Paola Grandi Jonathan Granot Manuel Güdel Alexander Heger Craig O.Heinke Jeroen Homan Rosario Iaria Kazushi Iwasawa Luca Izzo Long Ji Peter G.Jonker Jordi José Jelle S.Kaastra Emrah Kalemci Oleg Kargaltsev Nobuyuki Kawai Laurens Keek Stefanie Komossa Ingo Kreykenbohm Lucien Kuiper Devaky Kunneriath Gang Li En-Wei Liang Manuel Linares francesco Longo fangJun Lu Alexander A.Lutovinov Denys Malyshev Julien Malzac Antonios Manousakis Ian McHardy Missagh Mehdipour YunPeng Men Mariano Méndez Roberto P.Mignani Romana Mikusincova M.Coleman Miller Giovanni Miniutti Christian Motch Joonas Nättilä Emanuele Nardini Torsten Neubert Paul T.O'Brien Mauro Orlandini Julian P.Osborne Luigi Pacciani Stéphane Paltani Maurizio Paolillo Iossif E.Papadakis Biswajit Paul Alberto Pellizzoni Uria Peretz Miguel A.Pérez Torres Emanuele Perinati Chanda Prescod-Weinstein Pablo Reig SRON Netherlands Institute for Space Research Department of Astronomy University of Geneva Key Laboratory for Particle Astrophysics Institute of High Energy PhysicsChinese Academy of Sciences School of Astronomy and Space Science Nanjing University INAf-IASf Bologna ASI Via del Politecnico snc INAf-Istituto di Astrofisica e Planetologie Spaziali Institut für Astronomie und Astrophysik Tübingen Universit?t Tübingen NASA Goddard Space flight Center ICREA&Institute of Space Sciences(IEEC-CSIC) Campus UABCarrer de Can Magrans CSPAR SPA University of Alabama in Huntsville Department of Physics Texas Tech University INAf Osservatorio Astronomico di Capodimonte Leiden Observatory Leiden University Anton Pannekoek Institute University of Amsterdam ASTRON Netherlands Institute for Radio Astronomy Universitat Politecnica de Catalunya(UPC-IEEC) Universitádegli Studi di Urbino‘Carlo Bo’ Max-Planck-Institut für Radioastronomie Dr.Karl-Remeis-Sternwarte and Erlangen Centre for Astroparticle Physics(ECAP) Friedrich Alexander Universit?t Erlangen-Nurnberg Purple Mountain Observatory Chinese Academy of Sciences Instituto de Astrofysíca de Andalucia-CSIC Department of Physics and Astronomy University of Southampton IRAP Universitéde ToulouseCNRSUPSCNES Mathematical Sciences and STAG Research Centre University of Southampton New York University Abu Dhabi Center for Relativistic Astrophysics School of PhysicsGeorgia Institute of Technology Physics Department Middle East Technical University Department of Physics Technion-Israel Institute of Technology INAf Osservatorio Astronomico di Brera Tata Institute of fundamental Research Dipartimento di Matematica e fisica Universitàdegli Studi Roma Tre Instituto de Astrofísica de Canarias Instituto Nacional de Pesquisas Espaciais-INPE National Space Institute Technical University of Denmark(DTU Space) Department of Physics and Astronomy Michigan State University INAf Osservatorio Astrofisico di Arcetri Dipartimento di fisica Universitádegli Studi di Cagliari Department of Physics and A

In this White Paper we present the potential of the enhanced X-ray Timing and Polarimetry(eXTP) mission for studies related to Observatory Science targets. These include flaring stars, supernova remnants, accreting white dwarfs, low and high mass X-ray binaries, radio quiet and radio loud active galactic nuclei, tidal disruption events, and gamma-ray bursts. eXTP will be excellently suited to study one common aspect of these objects: their often transient nature. Developed by an international Consortium led by the Institute of High Energy Physics of the Chinese Academy of Science, the eXTP mission is expected to be launched in the mid 2020s.

关键词： space research in struments nuclear astrophysics flare stars accretion and accretion disks mass loss and stellar winds cataclysmic binaries X-ray binaries supernova remnants active galactic nuclei X-ray bursts gamma-ray bursts gravitational waves

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Electron-ion collider in China

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frontiers of physics 2021年第6期16卷 1-78页

作者： Daniele PAnderle valerio Bertone Xu Cao Lei Chang Ningbo Chang Gu Chen Xurong Chen Zhuojun Chen Zhufang Cui Lingyun Dai Weitian Deng Minghui Ding Xu feng Chang Gong Longcheng Gui feng-Kun Guo Chengdong Han Jun He Tie-Jiun Hou Hongxia Huang Yin Huang KrešImir KumeričKi LPKaptari Demin Li Hengne Li Minxiang Li Xueqian Li Yutie Liang Zuotang Liang Chen Liu Chuan Liu Guoming Liu Jie Liu Liuming Liu Xiang Liu Tianbo Liu Xiaofeng Luo Zhun Lyu Boqiang Ma fu Ma Jianping Ma Yugang Ma Lijun Mao Cédric Mezrag HervéMoutarde Jialun Ping Sixue Qin Hang Ren Craig DRoberts Juan Rojo Guodong Shen Chao Shi Qintao Song Hao Sun PawełSznajder Enke Wang fan Wang Qian Wang Rong Wang Ruiru Wang Taofeng Wang Wei Wang Xiaoyu Wang Xiaoyun Wang Jiajun Wu Xinggang Wu Lei Xia Bowen Xiao Guoqing Xiao Ju-Jun Xie Yaping Xie Hongxi Xing Hushan Xu Nu Xu Shusheng Xu Mengshi Yan Wenbiao Yan Wencheng Yan Xinhu Yan Jiancheng Yang Yi-Bo Yang Zhi Yang Deliang Yao Zhihong Ye Peilin Yin C-PYuan Wenlong Zhan Jianhui Zhang Jinlong Zhang Pengming Zhang Yifei Zhang Chao-Hsi Chang Zhenyu Zhang Hongwei Zhao Kuang-Ta Chao Qiang Zhao Yuxiang Zhao Zhengguo Zhao Liang Zheng Jian Zhou Xiang Zhou Xiaorong Zhou Bingsong Zou Liping Zou Guangdong Provincial Key Laboratory of Nuclear Science Institute of Quantum MatterSouth China Normal UniversityGuangzhou 510006China IRfU CEAUniversitéParis-SaclayF-91191 Gif-sur-YvetteFrance Institute of Modern Physics Chinese Academy of SciencesLanzhou 730000China University of Chinese Academy of Sciences Beijing 100049China Nankai University Tianjin 300071China Xinyang Normal University Xinyang 464000China School of Physics and Materials Science Guangzhou UniversityGuangzhou 510006China Hunan University Changsha 410082China Nanjing University Nanjing 210093China Huazhong University of Science and Technology Wuhan 430074China European Centre for Theoretical Studies in Nuclear Physics and Related Areas(ECT*)and fondazione Bruno Kessler Villa TambosiStrada delle Tabarelle 286I-38123 Villazzano(TN)Italy School of Physics Peking UniversityBeijing 100871China Hunan Normal University Changsha 410081China Institute of Theoretical Physics Chinese Academy of SciencesBeijing 100190China Nanjing Normal University Nanjing 210023China Department of Physics College of SciencesNortheastern UniversityShenyang 110819China Southwest Jiaotong University Chengdu 610000China Department of Physics Faculty of ScienceUniversity of ZagrebBijenička c.3210000 ZagrebCroatia Bogoliubov Laboratory of Theoretical Physics Joint Institute for Nuclear ResearchDubna 141980Russia School of Physics and Microelectronics Zhengzhou UniversityZhengzhou 450001China Lanzhou University Lanzhou 730000China Key Laboratory of Particle Physics and Particle Irradiation(MOE) Shandong UniversityQingdao 266237China Key Laboratory of Quark and Lepton Physics(MOE)and Institute of Particle Physics Central China Normal UniversityWuhan 430079China School of Physics Southeast UniversityNanjing 211189China Key Laboratory of Nuclear Physics and Ion-beam Application(MOE) Institute of Modern PhysicsFudan UniversityShanghai 200433China Shanghai Institute of Applied Physics Chinese Academy of SciencesShanghai 201800China Depa

Lepton scattering is an established ideal tool for studying inner structure of small particles such as nucleons as well as *** a future high energy nuclear physics project,an Electron-ion collider in China(EicC)has been *** will be constructed based on an upgraded heavy-ion accelerator,High Intensity heavy-ion Accelerator facility(HIAf)which is currently under construction,together with a new electron *** proposed collider will provide highly polarized electrons(with a po-larization of 80%)and protons(with a polarization of 70%)with variable center of mass energies from 15 to 20 GeV and the luminosity of(2–3)×1033 cm^(−2)·s^(−1).Polarized deuterons and Helium-3,as well as unpolarized ion beams from Carbon to Uranium,will be also available at the *** main foci of the EicC will be precision measurements of the structure of the nucleon in the sea quark region,including 3D tomography of nucleon;the partonic structure of nuclei and the parton interaction with the nuclear environment;the exotic states,especially those with heavy flavor quark *** addition,issues fundamental to understanding the origin of mass could be addressed by measurements of heavy quarkonia near-threshold production at the *** order to achieve the above-mentioned physics goals,a hermetical detector system will be constructed with cutting-edge *** document is the result of collective contributions and valuable inputs from experts across the *** EicC physics program complements the ongoing scientific programs at the Jefferson Laboratory and the future EIC project in the United *** success of this project will also advance both nuclear and particle physics as well as accelerator and detector technology in China.

关键词： electron ion collider nucleon structure nucleon mass exotic hadronic states quantum chromodynamics 3D-tomography helicity transverse momentum dependent parton distribution generalized parton distribution energy recovery linac polarization spin rotator

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