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IFIH1 and DDX58 gene variants in pediatric rheumatic diseases

World Journal of Clinical Pediatrics 2023年第3期12卷 107-114页

作者： Rinat Raupov Evgeny Suspitsin konstantin belozerov.tatiana gabrusskaya Mikhail Kostik Department of Pediatry Saint-Petersburg State Pediatric Medical UniversitySaint-Petersburg 194100Russia Department of Genetics Saint-Petersburg State Pediatric Medical UniversitySaint-Petersburg 194100Russia Department of Gastrointestinal Diseases Saint-Petersburg State Pediatric Medical UniversitySaint-Petersburg 194100Russia

BACKGROUND The IFIH1 gene codes the MDA5 protein and the DDX58 gene codes the RIG-I *** proteins are parts of the interferon(IFN)I signaling pathway and are responsible for antiviral defense and innate immune ***1 and DDX58 polymorphisms are associated with a spectrum of autoimmune *** gain-of-function IFIH1 mutations have been found in Singleton-Merten and Aicardi-Goutières syndrome,while DDX58 mutation can cause atypical Singleton-Merten *** To characterize children with pediatric rheumatic diseases(PRD)carrying DDX58 or IFIH1 *** Clinical exome sequencing was performed on 92 children with different ***1 and DDX58 variants have been detected in 14 ***-I score has been analyzed and the clinical characteristics of patients have been *** A total of seven patients with systemic lupus erythematosus(SLE)(n=2),myelodysplastic syndrome with SLE features at the onset of the disease(n=1),mixed connective tissue disease(MCTD)(n=1),undifferentiated systemic autoinflammatory disease(uSAID)(n=3)have 5 different variants of the DDX58 gene.A common non-pathogenic variant p.D580E has been found in five children.A rare variant of uncertain significance(VUS)p.N354S was found in one patient with uSAID,a rare likely non-pathogenic variant p.E37K in one patient with uSAID,and a rare likely pathogenic variant ***864fs in a patient with *** IFN-I score was detected in 6 of 7 patients with DDX58 *** patients had six different IFIH1 *** were presented with uSAID(n=2),juvenile dermatomyositis(JDM)(n=1),SLElike disease(n=1),Periodic fever with aphthous stomatitis,pharyngitis,and adenitis syndrome(n=1),and systemic onset juvenile idiopathic arthritis(n=1).Three patients have VUS p.E627X,one patient has benign variant *** VUS p.R595H was detected in the JDM *** rare VUS p.L679Ifs*2 and previously not reported variant p.V599Ffs*5 were detected in the patient with uS

关键词： IFIH1 DDX58 Undifferentiated systemic autoinflammatory disease Systemic lupus erythematosus Interferon-I score©The Author(s)2023.Published by Baishideng

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Adding new dimension to non-directed C（sp3）–H functionalizations

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Science China Chemistry 2025年

作者： konstantin P.Bryliakov Laboratory of Selective Oxidation Catalysis N.D.Zelinsky Institute of Organic Chemistry RAS

Aliphatic C–H groups are among the most abundant yet least reactive functional groups in organic molecules. Nature has evolved metalloenzymes such as those of the cytochrome P450 superfamily that efficiently activate individual tertiary（3） and secondary （2） C（sp3）–H bonds in complex molecules with the highest level of regio-and stereoselectivity [1], thus encouraging studies aimed at engineering artificial metalloenzymes for synthetic applications [2].

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Novel Methodologies for Preventing Crack Propagation in Steel Gas Pipelines Considering the Temperature Effect

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Structural Durability & Health Monitoring 2025年第1期19卷 1-23页

作者： Nurlan Zhangabay Ulzhan Ibraimova Marco Bonopera Ulanbator Suleimenov konstantin Avramov Maryna Chernobryvko Aigerim Yessengali Department of Architecture and Urban Planning Mukhtar Auezov South Kazakhstan UniversityShymkent160012Kazakhstan Department of Industrial Civil and Road Construction Mukhtar Auezov South Kazakhstan UniversityShymkent160012Kazakhstan Mechanics Sound&Vibration LaboratoryDepartment of Civil EngineeringCollege of EngineeringNational Taiwan UniversityTaipei10617Taiwan Department of Reability&Dynamic Strength A.Pidhornyi Institute of Mechanical Engineering Problems of National Academy of Sciences of UkraineKharkiv61046Ukraine

Using the software ANSYS-19.2/Explicit Dynamics,this study performedﬁnite-element modeling of the large-diameter steel pipeline cross-section for the Beineu-Bozoy-Shymkent gas pipeline with a non-through straight crack,strengthened by steel wire *** effects of the thread tensile force of the steel winding in the form of single rings at the crack edges and the wires with different winding diameters and pitches were also *** results showed that the strengthening was preferably executed at a minimum value of the thread tensile force,which was 6.4%more effective than that at its maximum *** analysis of the inﬂuence of the winding dia-meters showed that the equivalent stresses increased by 32%from the beginning of the crack growth until the wire *** increment in winding diameter decelerated the disclosure of the edge crack and reduced its length by 8.2%.The analysis of the inﬂuence of the winding pitch showed that decreasing the distance between the winding turns also led to a 33.6%reduction in the length of the straight crack and a 7.9%reduction in the maximum stres-ses on the strengthened pipeline *** analysis of the temperature effect on the pipeline material,within a range from-40℃ to+50℃,resulted in a crack length change of up to 5.8%.As the temperature dropped,the crack length *** such a temperature range,the maximum stresses were observed along the cen-tral area of the crack,which were equal to 413 MPa at+50℃ and 440 MPa at-40℃.The results also showed that the presence of the steel winding in the pipeline signiﬁcantly reduced the length of crack propagation up to 8.4 times,depending on the temperature effect and design parameters of *** work integrated the existing methods for crack localization along steel gas pipelines.

关键词： Crack propagation ﬁnite-element internal pressure prestressing steel gas pipeline temperature effect

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A functional tacrolimus-releasing nerve wrap for enhancing nerve regeneration following surgical nerve repair

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Neural Regeneration Research 2025年第1期20卷 291-304页

作者： Simeon C.Daeschler Katelyn J.W.So konstantin Feinberg Marina Manoraj Jenny Cheung Jennifer Zhang Kaveh Mirmoeini JPaul Santerre Tessa Gordon Gregory HBorschel SickKids Research Institute Neuroscience and Mental Health ProgramTorontoONCanada Institute of Biomaterials and Biomedical Engineering University of TorontoTorontoONCanada Indiana University School of Medicine IndianapolisINUSA Division of Plastic and Reconstructive Surgery the Hospital for Sick ChildrenTorontoONCanada Translational Biology and Engineering Program Ted Rogers Centre for Heart ResearchInstitute of Biomedical EngineeringFaculty of DentistryUniversity of TorontoTorontoONCanada

Axonal regeneration following surgical nerve repair is slow and often incomplete,resulting in poor functional recovery which sometimes contributes to lifelong ***,there are no FDA-approved therapies available to promote nerve *** accelerates axonal regeneration,but systemic side effects presently outweigh its potential benefits for peripheral nerve *** authors describe herein a biodegradable polyurethane-based drug delivery system for the sustained local release of tacrolimus at the nerve repair site,with suitable properties for scalable production and clinical application,aiming to promote nerve regeneration and functional recovery with minimal systemic drug *** is encapsulated into co-axially electrospun polycarbonate-urethane nanofibers to generate an implantable nerve wrap that releases therapeutic doses of bioactive tacrolimus over 31 *** and drug loading are adjustable for applications in small and large caliber nerves,and the wrap degrades within 120 days into biocompatible *** released from the nerve wrap promotes axon elongation in vitro and accelerates nerve regeneration and functional recovery in preclinical nerve repair models while off-target systemic drug exposure is reduced by 80%compared with systemic *** its surgical suitability and preclinical efficacy and safety,this system may provide a readily translatable approach to support axonal regeneration and recovery in patients undergoing nerve surgery.

关键词： biodegradable local drug delivery nerve injury nerve regeneration nerve wrap tacrolimus

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Systemic juvenile idiopathic arthritis–associated lung disease: A retrospective cohort study

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World Journal of Clinical Pediatrics 2024年第1期13卷 73-83页

作者： konstantin E belozerov Natalia M Solomatina Eugenia A Isupova Alla A Kuznetsova Mikhail M Kostik Department of Pediatric Saint-Petersburg State Pediatric Medical UniversitySaint-Petersburg 194100Russia St.Petersburg State Budgetary Institution of Health Care Children's City Polyclinic No.29 of the Kalininsky District of St.PetersburgSt.Petersburg 195274Russia Research Laboratory of Autoimmune and Autoinflammatory Diseases World-Class Research Centre for Personalized MedicineAlmazov National Medical Research CentreSt.Petersburg 197341Russia

BACKGROUND Lung damage in systemic juvenile arthritis(sJIA)is one of the contemporary topics in pediatric *** previous studies showed the severe course and fatal outcomes in some *** information about interstitial lung disease(ILD)in the sJIA is scarce and limited to a total of 100 *** To describe the features of sJIA patients with ILD in *** In the present retrospective cohort study,information about 5 patients less than 18-years-old with sJIA and ILD were *** diagnosis of sJIA was made according to the current 2004 and new provisional International League of Associations for Rheumatology criteria *** was diagnosed with chest computed tomography with the exclusion of other possible reasons for concurrent lung *** activation syndrome(MAS)was diagnosed with HLH-2004 and 2016 EULAR/ACR/PRINTO Classification Criteria and hScores were calculated during the lung *** The onset age of sJIA ranged from 1 year to 10 *** time interval before ILD ranged from 1 mo to 3 *** disease course was characterized by the prevalence of the systemic features above articular involvement,intensive rash(100%),persistent and very active MAS(hScore range:194-220)with transaminitis(100%),and respiratory symptoms(100%).Only 3 patients(60%)developed a clubbing *** patients(100%)had pleural effusion and 4 patients(80%)had pericardial effusion at the disease *** patients(40%)developed pulmonary arterial ***-related reactions to tocilizumab were observed in 3(60%)of the *** patient with trisomy 21 had a fatal disease *** of the remaining patients had sJIA remission and 2 patients had *** disease improved in 3 patients(75%),but 1 of them had initial deterioration of lung *** patient who has not achieved the sJIA remission had the progressed course of *** cases of hyper-eosinophilia were *** patients(80%)received

关键词： Systemic juvenile arthritis Interstitial lung disease Canakinumab Tocilizumab Interleukin-6 Interleukin-1

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The trade-off between COVID-19 and mental diseases burden during a lockdown: Mathematical modeling of control measures

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Infectious Disease Modelling 2023年第2期8卷 403-414页

作者： Alexei Alexeevich Romanyukha konstantin Alexandrovich Novikov konstantin konstantinovich Avilov Timofey Alexandrovich Nestik tatiana Evgenevna Sannikova Marchuk Institute of Numerical Mathematics of the Russian Academy of Sciences Gubkina str.8Moscow119333Russia Institute of Psychology of the Russian Academy of Sciences Yaroslavskaya str.13Moscow129366Russia

Background:During the COVID-19 pandemic,many countries used lockdowns as a containment *** lockdowns successfully contributed to slowing down the contagion,the related mobility restrictions were reportedly associated with an increased risk of major depressive and anxiety *** aimed to quantify the trade-off between the quality-adjusted life years(QALY)gain due to lower COVID-19 incidence as a result of a lockdown and QALY loss due to lockdown-induced mental ***:We developed an agent-based model of COVID-19 epidemic and coupled mental disorder development in the population of a large *** used data sources on the places of living,studying and working,public health and census *** of mental disorders was based on diathesis-stress *** quantified mental and physical health burden in terms of QALY taking into account major depressive and anxiety disorder episodes,lethal and non-lethal cases of COVID-19,and ***:We evaluated the dynamics of new major depressive disorder(MDD)and anxiety disorder(AD)cases during the period between September 2020 and December 2021 in Moscow,*** found that lockdown imposition increases the daily chances of getting MDD or ADD by a vulnerable person by 16.79%(95%CI[12.36%,21.23%]).The QALY loss associated with COVID-19-induced and lockdown-induced mental disorders was estimated to be 18.93%(95%CI[16.94%,19.73%])of the total QALY loss caused by COVID-19,immunization,and all kinds of mental *** a synthetic“strong”lockdown,it had been shown that QALY loss is minimized when about 70%of the population are ***:The burden associated with mental disorders amounts to a considerable part of COVID-19-related *** findings demonstrate that cost-benefit analysis of mobility restriction should include a forecast of mental disorder development in the population.

关键词： COVID-19 Agent-based model Major depressive and anxiety disorders Quality adjusted life years(QALY) Lockdown strictness Optimal lockdown

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Modern developments related to nanotechnology and nanoengineering of concrete

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Frontiers of Structural and Civil Engineering 2016年第2期10卷 131-141页

作者： konstantin SOBOLEV Department of Civil and Environmental Engineering University of gqsconsin-Milwaukee P.O. Box 413 Milwaukee W153201 USA

This paper reports on modem developments related to nanotechnology of cement and concrete. Recent advances in instrumentation and design of advanced nano-composite materials is discussed. New technological directions and historical milestones in nanoengineering and nanomodification of cement-based materials are presented. It is concluded that there is a strong potential of nanotechnology to improve the performance of cement-based materials.

关键词： nanotechnology cement concrete nanoparticle nano-composite nanomodification carbon nanotubes C-S-Hgel photocatalyst

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Large-for-size syndrome prophylaxis in infant liver recipients with low body mass

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World Journal of Transplantation 2025年第1期15卷 72-85页

作者： konstantin Semash Timur Dzhanbekov Department of Mini-Invasive Surgery National Children's Medical CenterTashkent 100171ToshkentUzbekistan

Transplantation of the left lateral section(LLS)of the liver is now an established practice for treating advanced diffuse and unresectable focal liver diseases in children,with variants of the LLS primarily used in ***,the surgical challenge of matching the size of an adult donor's graft to the volume of a child's abdomen remains *** review explores historical developments,various approaches to measuring the required functional liver mass,and techniques to prevent complications associated with large-for-size grafts in infants.

关键词： Pediatric liver transplantation Large-for-size syndrome Preoperative evaluation of donor and recipient Liver volumetry Monosegmental transplantation Left lateral sector graft Reduced size liver graft Abdominal wall reconstruction Liver transplantation Liver resection

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Taxonomic Composition and Spatial Distribution of Meiofauna Community from a Sandy Intertidal Zone in Sishili Bay, Yellow Sea Coast (Northern China)

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Journal of Ocean University of China 2022年第5期21卷 1389-1401页

作者： PAVLYUK Olga N TREBUKHOVA Yulia A SHCHERBAKOV Ilya A TARASOVA tatiana S LUTAENKO konstantin A CHEN Linlin SONG Bo LI Xiaojing LI Baoquan A.V.Zhirmunsky National Scientific Center of Marine Biology Far Eastern BranchRussian Academy of SciencesVladivostok 690041Russia Yantai Institute of Coastal Zone Research Chinese Academy of SciencesYantai 264003China

The meiofauna composition and trophic groups of the nematode communities have been studied at a sandy intertidal zone in Sishili Bay along the Yantai coast(Yellow Sea,China).Nematoda was dominant among the 11 groups of *** meiofaunal densities were low,ranging between 111±47 and 542±131 ind./10 cm^(2).Results obtained from the correlation analysis made it possible to determine several factors that influence species composition and ***,sediment granulometry significantly affected the *** highest abundance was found in fine-grained sediments(318.77±126 ind./10 cm^(2))com-pared to coarse-grained sediments(175±82 ind./10 cm2).Nematodes were predominant in all studied sites.A total of 70 species be-longing to 52 genera and 18 families were identified in the study *** was a significant difference in nematode abundance(ANOVA,F=2.38,P63μm),with dominant species belonging to the genus Bathylaimus,Ptycho-laimellus,Spirinia,and *** trophic groups were dominated by non-selective deposit feeders(40.7%)and epistrate feed-ers(41.29%).In contrast,the species composition in medium grain sands was characterized by a low species diversity index and a high Simpson dominance *** communities showed similar dominant species compositions belonging to a single genus Bathylaimus,Enoplolaimus,and Oncholaimus,and in terms of trophic structure-representatives of deposit feeders(46.15%),preda-tors,and omnivores(64.71%)*** divergence of meiofaunal community structures can be explained by abiotic factors such as sediment grain size,salinity,and organic carbon ***,sediment particle size can explain the diversity of nematodes at the level of in abundance,diversity indices,and trophic *** highest nematode density and diversity coincided with

关键词： meiofauna nematodes sandy intertidal zone sediment grain size Yellow Sea

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Thermodynamic Properties of Li-LiH (LiD, LiT) Systems. The Phase Diagram

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Open Journal of Physical Chemistry 2012年第3期2卷 141-146页

作者： konstantin A. Yakimovich tatiana Biryukova Joint Institute for High Temperatures Russian Academy of Sciences Moscow Russia The Institute of Informatics Problems Russian Academy of Sciences Moscow Russia

The paper presents a comprehensive critical review and detailed analysis of the literature, published in the field of the phase diagram solution Li-LiH (LiD, LiT). Special attention is paid to the completeness of the source information on the paper’s topic resulting in an extended reference list. Particularly, paper reviews rarely used sources including little-known publications from research centers, proceedings of the international scientific meetings and dissertations. These publications were more thoroughly analyzed in order to make the information available to the scientific society.

关键词： Diagram Phase Equilibrium Lithium Hydride Deuteride

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