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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： Konstantin 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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Biotransformation of 6:2 fluorotelomer sulfonate(6:2 FTS)in sulfur-rich media by Trametopsis cervina

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Frontiers of Environmental Science & Engineering 2024年第9期18卷 17-26页

作者： Felix Grimberg thomas m Holsen Sujan Fernando Siwen Wang Department of Civil and Environmental Engineering Clarkson UniversityPotsdamNew York 13699USA

Biotransformation of 6:2 fluorotelomer sulfonate(6:2 FTS)by two species of white-rot fungi,Pleurotus ostreatus(***)and Trametopsis cervina(***),was investigated in a sulfurrich medium designed to stimulate production of lignin-degrading *** of 6:2 FTS was observed by *** over the study period of 30 d,but not by *** rates were comparable to those found in other studies investigating mixed culture degradation in nonsulfur limiting media,with approximately 50 mol%of applied 6:2 FTS removed after 30 *** transformation products were short-chain perfluorocarboxylic acids(PFCAs),including PFHxA(2.27 mol%),PFPeA(0.24 mol%),and PFBA(0.28 mol%).The main intermediate products include 5:2 sFTOH(16.3 mol%)and 5:3 FTCA(2.99 mol%),while 6:2 FTCA,6:2 FTuCA,and 5:2 ketone were also identified at low *** 60 mol%of detected products were assigned to the major pathway to 5:2 ketone,and 40 mol%were assigned to the minor pathway to 5:3 *** overall molar balance was found to decrease to 75 mol%by Day 30,however,was closed to near 95 mol%with a theoretical estimation for the volatile intermediates in the headspace,5:2 ketone and 5:2 *** different capabilities of the two white-rot fungal species for 6:2 FTS biotransformation in sulfur-rich media suggest that the enzyme processes of *** to de-sulfonate 6:2 FTS may be unrelated to sulfur metabolism.

关键词： White-rot fungus 6:2 fluorotelomer sulfonate(6:2 FTS) Biotransformation Sulfur-rich medium Intermediate products

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Impact of increasing one-carbon metabolites on traumatic brain injury outcome using pre-clinical models

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Neural Regeneration Research 2024年第8期19卷 1728-1733页

作者： Sanika m.Joshi Theresa Currier thomas Nafisa m.Jadavji College of Osteopathic medicine Midwestern UniversityGlendaleCAUSA Department of Biomedical Sciences Midwestern UniversityGlendaleCAUSA Department of Child Health College of Medicine–PhoenixUniversity of ArizonaPhoenixAZUSA College of Veterinary medicine Midwestern UniversityGlendaleCAUSA Department of Neuroscience Carleton UniversityOttawaCanada

Traumatic brain injury is a major cause of death and disability worldwide,affecting over 69 million individuals ***-carbon metabolism has been shown to have beneficial effects after brain damage,such as ischemic ***,whether increasing one-carbon metabolite vitamins impacts traumatic brain injury outcomes in patients requires more *** aim of this review is to evaluate how one-carbon metabolites impact outcomes after the onset of traumatic brain ***,Web of Science,and Google Scholar databases were searched for studies that examined the impact of B-vitamin supplementation on traumatic brain injury *** search terms included combinations of the following words:traumatic brain injury,dietary supplementation,one-carbon metabolism,and *** focus of each literature search was basic science *** year of publication in the literature searches was not *** analysis of the literature has shown that dietary supplementation of B-vitamins has significantly improved the functional and behavioral recove ry of animals with traumatic brain injury compared to *** r,this improvement is dosage-dependent and is contingent upon the onset of supplementation and whether there is a sustained or continuous delive ry of vitamin supplementation post-traumatic brain *** details of supplementation post-traumatic brain injury need to be further ***,we conclude that B-vitamin supplementation improves behavioral outcomes and reduces cognitive impairment post-traumatic brain injury in animal model *** investigation in a clinical setting should be stro ngly considered in co njunction with current medical treatments for traumatic brain injury-affected individuals.

关键词： folic acid nicotinamide one-carbon metabolism riboflavin traumatic brain injury vitamin B12 vitamin B2 vitamin B3

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James Hogarth Pringle and his time-honored maneuver

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Hepatobiliary Surgery and Nutrition 2024年第3期13卷 391-392页

作者： thomas m.van Gulik Department of Surgery Amsterdam University Medical CenterUniversity of AmsterdamAmsterdamThe Netherlands

The classical method to control blood loss during parenchymal transection of the liver is the Pringle *** his landmark article in the Annals of Surgery in 1908,James Hogarth Pringle occluded the afferent vessels to the liver by compressing the hepatoduodenal ligament between his thumb and index finger(1).He applied this maneuver in eight patients who required emergency laparotomy for major hemorrhage after severe liver *** he succeeded in controlling blood loss,the eight patients he described unfortunately all *** outcome did not prevent the Annals of Surgery from publishing his report after which James Hogarth Pringle was ready to enter the‘Hall of Fame’.Vascular inflow occlusion,applied in a continuous or intermittent fashion,became the mainstay technique for reducing blood loss during liver transection,and has remained so in the present era of laparoscopic and robotic liver resection(2).

关键词： Pringle Surgery patients

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The miracle transplantation of a black leg by the surgeon saints Cosmas and Damian

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Hepatobiliary Surgery and Nutrition 2024年第1期13卷 1-2页

作者： thomas m.van Gulik Department of Surgery Amsterdam University Medical CenterUniversity of AmsterdamAmsterdamThe Netherlands

The miracle of the saints Cosmas and Damian refers to transplantation of a black leg onto a white man that took place in medieval Rome(Figure 1)(1).A sexton of a church in Rome was seriously affected by cancer in his right *** into a delirious fever,he dreamed that the holy surgeons,saint Cosmas and saint Damian,visited him and skilled as they were,decided to remove the affected leg and replace it with a healthy *** went to the grave of a recently deceased Ethiopian man and retrieved the right leg of this black man and took it to the *** sick leg of the sexton was amputated and they transplanted onto him the leg of the black *** their miracle operation,Cosmas and Damian have been elevated to patron saints of the surgical profession and many surgical societies have adopted their names in honor of them.

关键词： black Figure replace

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Applying machine Learning Approach to Explore Childhood Circumstances and Self-Rated Health in Old Age-China and the US,2020-2021

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China CDC weekly 2024年第11期6卷 213-218,I0008-I0013页

作者： Shutong Huo Derek Feng thomas m.Gill Xi Chen Department of Health Society&BehaviorPublic HealthUniversity of CaliforniaIrvineCAUSA Department of Statistics and Data Science Yale UniversityNew HavenCTUS Department of Internal medicine Yale School of MedicineNew HavenCTUS Department of Health Policy and management Yale School of Public HealthNew HavenCTUS Department of Economics Yale UniversityNew HavenCTUS.

Introduction:Childhood circumstances impact senior health,prompting the introduction of machine learning methods to assess their individual and collective contributions to senior ***:Using health and retirement study(HRS)and China Health and Retirement Longitudinal Study(CHARLS),we analyzed 2,434 American and 5,612 Chinese participants aged 60 and *** inference trees and forests were employed to estimate the influence of childhood circumstances on self-rated health(SRH).Results:The conventional method estimated higher inequality of opportunity(IOP)values in both China(0.039,accounting for 22.67%of the total Gini coefficient 0.172)and the US(0.067,accounting for 35.08%of the total Gini coefficient 0.191).In contrast,the conditional inference tree yielded lower estimates(China:0.022,accounting for 12.79%of 0.172;US:0.044,accounting for 23.04%of 0.191),as did the forest(China:0.035,accounting for 20.35%of 0.172;US:0.054,accounting for 28.27%of 0.191).Childhood health,financial status,and regional differences were key determinants of senior *** conditional inference forest consistently outperformed others in predictive accuracy,as demonstrated by lower out-of-sample mean squared error(mSE).Discussion:The findings emphasize the need for early-life interventions to promote health equity in aging *** learning showcases the potential in identifying contributing factors.

关键词： collective inequality estimates

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Energy and blast performance of beryllium in a model thermobaric composition in comparison with aluminum and magnesium

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Defence Technology（防务技术） 2024年第6期36卷 13-19页

作者： thomas m.Klapotke Stanisław Cudziło Waldemar A.Trzcinski Jozef Paszula Energetic materials Research Department of ChemistryUniversity of Munich(LMU)Butenandtstrasse 5-13(D)81377MunichGermany military University of Technology Faculty of Advanced Technologies and ChemistrySylwester Kaliski Str.200-908WarsawPoland

A direct comparison is made between the effectiveness of Al,mg,and Be powders as additional fuels in model thermobaric compositions containing 20%fuel,20%ammonium perchlorate,and 60%RDX(1,3,5-Trinitro-1,3,5-triazacyclohexane)passivated with *** determined calorimetric measurements of the heat of detonation,along with the overpressure histories in an explosion chamber filled with nitrogen,were used to determine the quasi-static pressure(QSP)under anaerobic *** measurements were also performed in a semi-closed bunker,and all blast wave parameters generated after the detonation of 500 g charges of the tested explosives were *** calorimetry results,QSP values,and blast wave parameters(pressure amplitude,specific and total impulses)clearly indicate that Be is much more effective as an additional fuel than either Al or mg in both anaerobic post-detonation reactions as well as the subsequent aerobic *** heat of detonation of the RDXwax/AP/Be explosive mixture is over 40%and 50%higher than that of the mixture containing aluminum and magnesium instead of beryllium,***,the TNT equivalent of the Be-containing composition due to the overpressure in the nitrogen-filled explosion chamber is 1.66,while the equivalent calculated using an air shock wave-specific impulse at a distance of 2.5 m is equal to *** high values of these parameters confirm the high reactivity of beryllium in both the anaerobic and aerobic stages of the thermobaric explosion.

关键词： Thermobaric explosives Beryllium Heat of detonation Quasi-static overpressure Blast wave parameters

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Gabor Analysis on Finite Product of Cyclic Groups

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Journal of mathematical Research with Applications 2024年第4期44卷 521-532页

作者： Jineesh thomas N.m.madhavan NAmBOOTHIRI Ajo JOSE Research Scholar of mathematics St.Thomas College Palai Professor and Principal Government Arts & Science College Idukki

We present a complete characterization of Gabor frame operators on finite dimensional Hilbert space L2（Γ）,where■and m1,m2,...,mpare positive *** notion of generalized B-modulation and generalized B-translation is ... 详细信息

关键词： Gabor frame Gabor frame operator modulation translation

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Utilizing blockchains in opportunistic networks for integrity and confidentiality

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Blockchain(Research and Applications) 2024年第1期5卷 31-38页

作者： Samaneh Rashidibajgan thomas Hupperich Cyber Security Department of Information SystemsUniversity of Münster80539 MünsterGermany

Opportunistic networks(OppNets)are usually a set of smart,wearable,and portable devices or entities with mobility that connect wirelessly without requiring *** a network is of great importance in data transmission,particularly in incidents and disasters,whether man-made or ***,message integrity and confidentiality are of concern when dealing with vital and physiological data transmission under strict privacy *** this work,we propose a structure to classify messages based on their priority in different ***,due to the decentralized architecture of OppNets,we propose a blockchain-based structure for providing security for high-priority *** contains three sequences of functional blocks with a light and simplified implementation that make it suitable for battery-powered wearable devices that are limited in energy consumption and computational *** simulation results show that by increasing the number of nodes in the network,the average of the changes in block sizes is neglectable,which addresses the computation ***,we analyze the performance of the proposed structure in terms of message delivery and network overhead compared with the Epidemic and Prophet routing *** results indicate advancing the overall performance of the proposed algorithm.

关键词： Opportunistic networks Blockchain message integrity message confidentiality

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mEmS-actuated terahertz metamaterials driven by phase-transition materials

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Frontiers of Optoelectronics 2024年第2期17卷 23-32页

作者： Zhixiang Huang Weipeng Wu Eric Herrmann Ke ma Zizwe A.Chase thomas A.Searles m.Benjamin Jungfleisch Xi Wang Department of materials Science and Engineering College of EngineeringUniversity of DelawareNewarkDE 19716USA Department of Physics and Astronomy College of Arts and SciencesUniversity of DelawareNewarkDE 19716USA Department of Electrical and Computer Engineering College of EngineeringUniversity of Illinois ChicagoChicagoIL 60607USA

The non-ionizing and penetrative characteristics of terahertz(THz)radiation have recently led to its adoption across a variety of *** effectively utilize THz radiation,modulators with precise control are *** most recent THz modulators manipulate the amplitude,frequency,or phase of incident THz radiation,considerably less progress has been made toward THz polarization *** methods for polarization control suffer from high driving voltages,restricted modulation depth,and narrow band capabilities,which hinder device performance and broader ***,an ideal THz modulator that offers high modulation depth along with ease of processing and operation is *** this paper,we propose and realize a THz metamaterial comprised of microelectromechanical systems(mEmS)actuated by the phase-transition material vanadium dioxide(VO_(2)).Simulation and experimental results of the three-dimensional metamaterials show that by leveraging the unique phase-transition attributes of VO_(2),our THz polarization modulator offers notable advancements over existing designs,including broad operation spectrum,high modulation depth,ease of fabrication,ease of operation condition,and continuous modulation *** enhanced features make the system a viable candidate for a range of THz applications,including telecommunications,imaging,and radar systems.

关键词： metamaterials mEmS THz VO_(2) Phase-transition material

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