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Exome sequencing identifies a likely causative variant in 53%of families with ciliopathy-related features on renal ultrasound after excluding NPHP1 deletions

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Genes & Diseases 2024年第5期11卷 40-43页

作者： konstanti. deutsch Verena Klambt Thomas M.Kitzler Tilman Jobst-Schwan Ronen Schneider Florian Buerger Steve Seltzsam Sherif El Desoky Jameela A.Kari Farkhanda Hafeez Maria Szczepanska Loai A.Eid Hazem S.Awad Muna Al-Saffar Neveen A.Soliman Velibor Tasic Camille Nicolas-Frank Kirollos Yousef Luca M.Schierbaum Sophia Schneider Abdul Halawi Izzeldin Elmubarak Katharina Lemberg Shirlee Shril Shrikant M.Mane Nancy Rodig Friedhelm Hildebrandt Division of Nephrology Department of PediatricsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Department of Pediatric Gastroenterology Nephrology and Metabolic DiseasesCharite Universitatsmedizin BerlinBerlin 13353Germany Berlin Institute of Health at Charitee Universitatsmedizin Berlin BIH Biomedical Innovation AcademyBIH Charite Clinician Scientist ProgramBerlin 10178Germany Department of Nephrology and Hypertension FriedrichAlexander-Universitat Erlangen-NurnbergErlangen 91054Germany Department of Pediatrics Faculty of Medicine King Abdulaziz UniversityPediatric Nephrology Center of ExcellenceKing Abdulaziz University HospitalJeddah 21589Saudi Arabia Department of Pediatric Nephrology The Children’s Hospital and Institute of Child HealthLahore 54000Pakistan Department of Pediatrics Faculty of Medical Sciences in ZabrzeMedical University of Silesia in KatowiceKatowice 40-752Poland Dubai Hospital and Al-Jalila Children’s Specialty Hospital Kidney Center of ExcellenceDubai 4545United Arab Emirates Department of Genetics and Genomics UAE UniversityAbu Dhabi 15551United Arab Emirates Department of Pediatrics Division of Genetics and GenomicsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Department of Pediatrics Center of Pediatric Nephrology and TransplantationKasr Al Ainy School of MedicineCairo UniversityCairo 11562Egypt Egypt Center for Research and Regenerative Medicine(ECRRM) Cairo 11511Egypt Medical Faculty Skopje University Children’s HospitalSkopje 1000North Macedonia Department of Genetics Yale University School of MedicineNew HavenCT 06520USA

Nephronophthisis-related ciliopathies(NPHP-RC)represent one of the most common causes of chronic kidney disease in the first three decades of life and are characterized by a broad genetic and clinical heterogeneity.1 To date,more than 90 genes have been identified that cause autosomalrecessive NPHP-RC if mutated,accounting for up to 60%of cases.1 Among these,homozygous deletions of NPHP1 are the most common *** genes localize to primary cilia,basal bodies,or the centrosome and lead to a primary ciliary disruption if mutated,thereby causing a broad phenotypical spectrum.1 Patients that suffer from NPHP-RC can either have an isolated renal phenotype,such as polyuria,polydipsia,decreased urinary concentration ability,and secondary enuresis due to loss of tubular function,or present with extrarenal symptoms including retinal degeneration,cerebellar vermis hypoplasia,or hepatic *** are often diagnosed in early adolescence,reaching end-stage renal disease before 25 years of age,but early onset and rapidly progressive forms of NPHP-RC also exist.1 Renal ultrasound indicates kidneys of normal or reduced renal length with increased echogenicity and corticomedullary cysts.

关键词： degeneration urinary likely

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Adaptive Design of Fluorescence Imaging Systems for Custom Resolution, Fields of View, and Geometries

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Biomedical Engineering Frontiers 2023年第1期4卷 260-273页

作者： Roujia Wang Riley J.deutsch Enakshi D.Sunassee Brian T.Crouch Nirmala Ramanujam Department of Biomedical Engineering Duke UniversityDurhamNCUSA Department of Pharmacology and Cancer Biology Duke University Medical CenterDurhamNCUSA

Objective and Impact Statement:We developed a generalized computational approach to design uniform,high-intensity excitation light for low-cost,quantitative fluorescence imaging of in vitro,ex vivo,and in vivo samples with a single ***:Fluorescence imaging is a ubiquitous tool for biomedical *** extensively modify existing systems for tissue imaging,increasing the time and effort needed for translational research and thick tissue *** modifications are applicationspecific,requiring new designs to scale across sample ***:We implemented a computational model to simulate light propagation from multiple *** a global optimization algorithm and a custom cost function,we determined the spatial positioning of optical fibers to generate 2 illumination *** results were implemented to image core needle biopsies,preclinical mammary tumors,or tumor-derived *** were stained with molecular probes and imaged with uniform and nonuniform ***:Simulation results were faithfully translated to benchtop *** demonstrated that uniform illumination increased the reliability of intraimage analysis compared to nonuniform illumination and was concordant with traditional histological *** computational approach was used to optimize the illumination geometry for the purposes of imaging 3 different fluorophores through a mammary window chamber *** specifically designed for intravital tumor imaging generated higher image contrast compared to the case in which illumination originally optimized for biopsy images was ***:We demonstrate the significance of using a computationally designed illumination for in vitro,ex vivo,and in vivo fluorescence *** illumination increased the reliability of intraimage analysis and enhanced the local contrast of biological *** approach is generalizable across light sources,biological appl

关键词： illumination Fluorescence image

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Impact of the microenvironment on the pathogenesis of mucosaassociated lymphoid tissue lymphomas

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World Journal of Gastrointestinal Oncology 2022年第1期14卷 153-162页

作者： Barbara Uhl Katharina T Prochazka Karoline Fechter Katrin Pansy Hildegard T Greinix Peter Neumeister Alexander JA deutsch Division of Hematology Medical University of GrazGraz 8036Austria

Approximately 8%of all non-Hodgkin lymphomas are extranodal marginal zone B cell lymphomas of mucosa-associated lymphoid tissue(MALT),also known as MALT *** arise at a wide range of different extranodal sites,with most cases affecting the stomach,the lung,the ocular adnexa and the *** small intestine is involved in a lower percentage of *** growth in the early stages is associated with long-lasting chronic inflammation provoked by bacterial infections(e.g.,Helicobacter pylori or Chlamydia psittaci infections)or autoimmune conditions(e.g.,Sjögren’s syndrome or Hashimoto thyroiditis).While these inflammatory processes trigger lymphoma cell proliferation and/or survival,they also shape the ***,activated immune cells are actively recruited to the lymphoma,resulting in either direct lymphoma cell stimulation via surface receptor interactions and/or indirect lymphoma cell stimulation via secretion of soluble factors like *** addition,chronic inflammatory conditions cause the acquisition of genetic alterations resulting in autonomous lymphoma cell ***,novel agents targeting the microenvironment have been developed and clinically tested in MALT lymphomas as well as other lymphoid *** this review,we aim to describe the composition of the microenvironment of MALT lymphoma,the interaction of activated immune cells with lymphoma cells and novel therapeutic approaches in MALT lymphomas using immunomodulatory and/or microenvironmenttargeting agents.

关键词： Mucosa-associated lymphoid tissue lymphoma Tumor microenvironment Microenvironment Helicobacter pylori Activated immune cells

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Stichwort deutsch intensivkurs fur grundstufe,Band I,初级 I.第3版第3版

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2007年

作者： deutsch-Kolleg der Tongji-Universitat

本书为第一册，包括语音教程及基础教程中的1-14课，相当于《出国留学人员德语强化教学大纲》规定的初级阶段I水平，全书语言地道，练习形式丰富多样，旨在全面培养学员听、说、读、写等方面的能力。

ISBN: (纸本)9787560824789

关键词：德语

来源：畅想之星图书光盘版（南... 评论

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Ion stopping in dense plasmas: A basic physics approach

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Matter and Radiation at Extremes 2016年第6期1卷 277-307页

作者： Claude deutsch Gilles Maynard Laboratoire de Physique des Gaz et Plasmas CNRSUniv-Paris SudUniversite Paris-Saclay91405 OrsayFrance

We survey quite extensively the present research status of ion-stopping in dense plasmas of potential importance for initial confinement fusion(ICF)drivenby intense and heavy ion beams,and alsofor warm dense matter(WDM).First,we putemphasis on every possible mechanism involved in the shaping of the ion projectile effective charge,while losing energy in a target plasma with classical ions and partially degenerate ***,we switch to ion stopping by target bound electrons,taking detailed account of mean excitation *** electron stopping has already been given a lot of attention in former works[*** et al.,Recent *** 1(2000)1-23;Open Plasma Phys.J.3(2010)88-115].Then,we extend the usual standard stopping model(SSM)framework to nonlinear stopping including a treatment of the Z 3 Barkas effect and a confronting comparison of Bloch and Bohr Coulomb ***,we document low velocity ion slowing down(LVISD)in single ion plasmas as well as in binary ionic mixtures(BIM),in connection with specific ICF fuels.

关键词： Ion projectile effective charge Mean excited energies Nonlinear stopping Binary ionic mixture

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Hepatocellular carcinoma in the post-hepatitis C virus era: Should we change the paradigm?

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World Journal of Gastroenterology 2019年第29期25卷 3929-3940页

作者： Hadar Meringer Oren Shibolet Liat deutsch Department of Gastroenterology and Hepatology Tel Aviv Sourasky Medical Center and the Sackler Faculty of Medicine Tel Aviv University

Hepatocellular carcinoma (HCC) is a common and deadly malignancy. The disease usually develops on a background of chronic liver disease. Until recently, the most common etiology was infection with the hepatitis C virus (HCV). The advent of direct-acting antiviral (DAA) therapies has been a major breakthrough in HCV treatment. Sustained virologic response can now be achieved in almost all treated patients, even in patients with a high risk for the development of HCC, such as the elderly or those with significant fibrosis. Early reports raised concerns of a high risk for HCC occurrence after DAA therapy both in patients with previous resection of tumors and those without previous tumors. As the World Health Organization’s goals for eradication of HCV are being endorsed worldwide, the elimination of HCV seems feasible. Simultaneous to the decrease in the burden of cirrhosis from HCV, non-alcoholic fatty liver disease (NAFLD) incidence has been increasing dramatically including significant increased incidence of cirrhosis and HCC in these patients. Surprisingly, a substantial proportion of patients with NAFLD were shown to develop HCC even in the absence of cirrhosis. Furthermore, HCC treatment and potential complications are known to be influenced by liver steatosis. These changes in etiology and epidemiology of HCC suggest the beginning of a new era: The post–HCV era. Changes may eventually undermine current practices of early detection, surveillance and management of HCC. We focused on the risk of HCC occurrence and recurrence in the post–HCV era, the surveillance needed after DAA therapy and current studies in HCC patients with NAFLD.

关键词： Hepatocellular carcinoma Hepatitis C virus Direct-acting antivirals Nonalcoholic fatty liver disease Non-alcoholic steatohepatitis

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A linear programming model for long-term mine planning in the presence of grade uncertainty and a stockpile

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International Journal of Mining Science and Technology 2014年第4期24卷 451-459页

作者： Koushavand Behrang Askari-Nasab Hooman deutsch Clayton V. Department of Civil and Environmental Engineering School of Mining and Petroleum Engineering University of Alberta

The complexity of an open pit production scheduling problem is increased by grade uncertainty. A method is presented to calculate the cost of uncertainty in a production schedule based on deviations from the target production. A mixed integer linear programming algorithm is formulated to find the mining sequence of blocks from a predefined pit shell and their respective destinations, with two objectives:to maximize the net present value of the operation and to minimize the cost of uncertainty. An efficient clustering technique reduces the number of variables to make the problem tractable. Also, the parameters that control the importance of uncertainty in the optimization problem are studied. The minimum annual mining capacity in presence of grade uncertainty is assessed. The method is illustrated with an oil sand deposit in northern Alberta.

关键词：不确定性线性规划模型矿区规划生产调度问题储备线性规划算法生产计划混合整数

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ber“Linguistikunterricht in China”-Worauf muβein chinesischer deutsch-Lehrer achten?

ber“Linguistikunterricht in China”-Worauf muβein chines...

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'92对外经济贸易大学学术报告会

作者： Zhang Cailiang,deutsch-Abteilung

＜正＞Zuerst bedanke ich mich dafü dnβich ein Exemplar"Linguistikunterricht in China"bekommen *** betone,daβder Zweck meines Beitrags nicht darin besteht, das Werk von Harro Gross et *** *** wünsche nur,daβmeine Kollegen mir helfen,einige Schwierigkeiten zu l*** folgenden Aspekte sind nicht nur für chinesische deutschlerner,sondern auch für chinesische deutschlehrer

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会计师事务所目前关注的四大优先事项

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新理财（公司理财） 2023年第11期 86-87页

作者： Sophie deutsch 不详

会计师事务所对其优先关注的事项进行了排名,按照顺序分别是效率、客户服务、业务增长和人才。本文阐述了小型、中型和大型会计师事务所分别采取的做法。根据汤森路透(Thomson Reuters)研究所发布的《税务专业人员状况报告》(以下简称... 详细信息

会计师事务所对其优先关注的事项进行了排名,按照顺序分别是效率、客户服务、业务增长和人才。本文阐述了小型、中型和大型会计师事务所分别采取的做法。根据汤森路透(Thomson Reuters)研究所发布的《税务专业人员状况报告》(以下简称《报告》),会计师事务所将效率列为今年最重要的优先事项,其次是客户服务、业务增长和人才。

关键词：会计师事务所客户服务优先事项税务专业汤森路透《报告》

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Older age, longer procedures and tandem endoscopic-ultrasound as risk factors for post-endoscopic retrograde cholangiopancreatography bacteremia

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World Journal of Gastroenterology 2020年第41期26卷 6402-6413页

作者： Liat deutsch Shay Matalon Adam Phillips Moshe Leshno Oren Shibolet Erwin Santo The Sackler Faculty of Medicine Tel Aviv UniversityTel Aviv 6997801Israel Department of Gastroenterology and Hepatology Tel Aviv Sourasky Medical CenterTel Aviv 6423906Israel Department of Gastroenterology Assaf Harofe Medical CenterZeriffin 70300Israel Faculty of Management Tel Aviv UniversityTel Aviv 6997801Israel

BACKGROUND Clinically significant post-endoscopic retrograde cholangiopancreatography(ERCP) bacteremia(PEB) occurs in up to 5% of cases, while antibiotic prophylaxis is recommended only when an ERCP is unlikely to achieve complete biliary drainage. However, the current recommendations may not cover all potential risk factors for *** To identify novel risk factors for PEB and evaluate appropriateness of antibiotic *** A retrospective study of 1082 ERCP procedures performed between January 2012-December 2013 in a single tertiary medical center. Data collection included: Demographic and clinical characteristics such as pre and post procedure antibiotic treatment and bacterial blood cultures. Exclusion criteria were:(1) Age < 18 years;(2) Positive bacterial blood culture before ERCP;(3) Scheduled antibiotic treatment prior to ERCP;(4) Hospitalization longer than 14 d before ERCP;and(5) missing critical data. Stepwise Logistic Regression analysis and Decision Tree algorithms were used for prediction modeling of *** A total of 626 ERCPs performed in 434 patients were included. Mean age 66.49 ± 15.4 years and 46.5% were males. PEB prevalence was 3.7%. Antibiotic prophylaxis was administrated in 139/626(22.2%) cases but was indicated according to the guidelines only in 44/626(7%) cases. In all the PEB cases, prophylaxis was deemed not indicated. A stepwise logistic regression [receiver operating characteristic(ROC), 0.766], identified 3 variables as independent risk factors for PEB: Age at ERCP ≥ 75 years(OR, 3.780, 95%CI: 1.519-9.408, P = 0.004);Tandem EUS/ERCP with fine needle aspiration(FNA)(OR, 14.528, 95%CI: 3.571-59.095, P < 0.001);ERCP duration longer than 60 min(OR, 5.396, 95%CI: 1.86-15.656, P = 0.002). In a decision tree model(ROC, 0.778) the probability for PEB without any risk factors was 1% regardless of prophylaxis *** The prevalence of PEB in our study is similar to previous reports, despite the fact that anti

关键词： Endoscopic retrograde cholangiopancreatography Bacteremia Tandem-procedures Fine needle aspiration Antibiotic prophylaxis Biliary drainage

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