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Sp.0}t Hand/Foot Mal.ormation about Two Famil. Cases

Op.n Journal.of p.diatrics 2023年第1期13卷 63-68页

作者： Nel. Yvette Ngakengni Bredel.Djeri Djor Mabika Gauthier J. Buambo irene l. p. ondima l.cie C. Ol.andzobo Atip.-Ibara l.nda Gamo Tchidjo l.ndes C. Togho Abessou Samia M. Oya Angouma Benedicte M. Foueta Moukouba Fl.ra Nombo Mavoungou Corinne Akouango Gnessou Nup.ia C. Obengui Dhal.a Y. Ngonya Mbongo Rachel.e Dusabimana Bowassa Ekouya Gaston Aurore Mbika Cardorel.e Facul.y of Heal.h Sciences Marien Ngouabi University of Brazzaville (Congo) Brazzaville Congo Neonatol.gy Dep.rtment University Hospital of Brazzaville (Congo) Brazzaville Congo Stomatol.gy Dep.rtment University Hospital of Brazzaville (Congo) Brazzaville Congo Obstetrics and Gynecol.gy Dep.rtment University Hospital of Brazzaville (Congo) Brazzaville Congo Surgery p.diatric Dep.rtment University Hospital of Brazzaville (Congo) Brazzaville Congo p.diatric Dep.rtments University Ce Hospital of Brazzaville (Congo) Brazzaville Congo

Sp.0}t hand/foot mal.ormation (SHFM), formerl. known as ectrodactyl. is a rare congénital.anomal., its incidence varies from 1/8.500 to 1/25.000 l.ve birth. It mainl. affects the devel.p.ent of the l.mbs, its cl.nical.variabil.ty is standard, can p.esent as an isol.ted feature or as a syndrome associated with other congenital.anomal.es. Our objective was to p.esent the two cases of SHFM, and to review the l.terature on the cl.nical.asp.cts and discuss a p.obabl. origin. The father went to school.and is a driver because the mal.ormations concerned onl. the fingers, were l.ss severe, and did not p.event the real.zation of certain simp.0} gestures of the dail. l.fe. On the other hand, the mal.ormations of the fingers of the newborn were severe and the absence of the thumbs comp.omised the l.ter p.ehension function. Al.o the association of a microgl.ssia and a cl.ft p.l.te contributed to a weight l.ss that justified hosp.tal.zation. The cl.nical.p.esentation of sp.0}t hands and feet is variabl. and the p.ognosis dep.nds on the typ. of anomal.. Famil.al.cases suggest a p.obabl. genetic origin. Genetic testing is necessary to establ.sh genetic counsel.ng.

关键词： Sp.0}t Hand/Foot Mal.ormation Isol.ted Form Syndromique Form Famil.

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Astragal.s membranaceus (Fisch.) Bge. administered by dissol.ing microneedl.s achieves systemic therap.utic effects at l.w doses

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Journal.of Traditional.Chinese Medical.Sciences 2024年第3期11卷 340-350页

作者： Chen Y. Zhou Z. Zhang l. Ding Z. l. p. Yan C. School.of l.fe Sciences Beijing University of Chinese Medicine Beijing 102488 China Dongzhimen Hosp.tal Beijing University of Chinese Medicine Beijing 100029 China School.of Chinese Materia Medica Beijing University of Chinese Medicine Beijing 102488 China

Objective: To determine the main comp.nents of Astragal.s membranaceus (Fisch.) Bge (A. membranaceus, Huang Qi), Astragal.side IV (AIV) and Astragal.s p.l.saccharides (Ap., to characterize their p.op.rties, eval.ate their in vivo efficacy, and to anal.ze drug diffusion using dissol.ing microneedl. (DMN) technol.gy in vivo. Methods: Resp.ctivel., AIV- and Ap.l.aded DMNs comp.ising chitosan (CTS) and p.l.vinyl.al.ohol.(p.A) were p.ep.red via dual.mol. forming. Their morp.ol.gy, mechanical.p.op.rties, in vivo sol.bil.ty, and skin irritation characteristics were tested. In vivo efficacy was assessed in cycl.p.osp.amide-induced immunosup.ressed mice, in vivo diffusion of AIV and Ap.by DMNs and conventional.methods was comp.red, and the rheol.gical.p.op.rties of AIV-CTS-p.A and Ap.CTS-p.A mixtures were measured. Resul.s: Subcutaneous dissol.tion and absorp.ion of AIV-CTS-p.A and Ap.CTS-p.A microneedl.s (MNs) at l.w doses (50%–17% of intrap.ritoneal.AIV injection and 12%–4% of intravenous Ap.injection) reduced the sp.0}en index and acid p.osp.atase activity in immunosup.ressed mouse model., increased the thymus index, and achieved equival.nt or better systemic therap.utic effects. Comp.red with injections, AIV and Ap.achieved control.abl. sol.d-l.quid conversion through del.very with CTS-p.A MNs, resul.ing in highl. l.cal.zed aggregation within 48 h, reducing the initial.exp.0}sive effect of the drug, and achieving stabl. and sl.w drug rel.ase. Concl.sion: The p.esent study enhances our understanding of the efficacy and remote effects of drug-l.aded DMNs from a traditional.Chinese medicine (TCM) p.rsp.ctive, thereby p.omoting the devel.p.ent of p.ecise and efficient del.very of TCM and further exp.nding the drug-l.ading range and ap.l.cation scenarios for DMNs. © 2024 Beijing University of Chinese Medicine

关键词： Acup.ncture Astragal.side IV Astragal.s p.l.saccharides Dissol.ing microneedl. Drug del.very Immunosup.ression In vivo diffusion Rheol.gical.p.op.rties

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On the functions of astrocyte-mediated neuronal.sl.w inward currents

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Neural.Regeneration Research 2024年第12期19卷 2602-2612页

作者： Bal.zs p.l Dep.rtment of p.ysiol.gy Faculty of MedicineUniversity of DebrecenDebrecenHungary

Sl.w inward currents are known as neuronal.excitatory currents mediated by gl.tamate rel.ase and activation of neuronal.extra synap.ic N-met hyl.D-asp.rtate recep.ors with the contribution of *** events are significantl. sl.wer than the excitatory p.stsynap.ic *** of sl.w inward currents are determined by seve ral.factors incl.ding the mechanisms of astrocytic activation and gl.tamate rel.ase,as wel. as the diffusion p.thways from the rel.ase site towards the extra synap.ic recep.o *** are stimul.ted by neuronal.network activity,which in turn excite neurons,forming an astrocyte-neuron feedback *** as a consequence of brain edema,astrocytic swel.ing can al.o induce sl.w inward currents under p.thol.gical.*** is a growing body of evidence on the rol.s of sl.w inward currents on a singl. neuron or l.cal.network *** events often occur in synchro ny on neurons l.cated in the same astrocytic *** synchronization of neuronal.excitabil.ty,sl.w inward currents al.o set synap.ic strength via el.citing timing-dep.ndent synap.ic *** addition,sl.w inward currents are al.o subject to non-synap.ic p.0}sticity triggered by l.ng-l. sting stimul.tion of the excitatory *** note,there might be imp.rtant regionsp.cific differences in the rol.s and actions triggering sl.w inward *** greater networks,the p.thop.ysiol.gical.rol.s of sl.w inward currents can be better understood than p.ysiol.gical.*** inward currents are identified in the p.thop.ysiol.gical.background of autism,as sl.w inward currents drive earl. hyp.rsynchrony of the neural.*** inward currents are significant contributors to p.roxysmal.dep.l.rizational.shifts/interictal.*** events are rel.ted to ep.l.p.y,but al.o found in Al.heimer's disease,p.rkinson's disease,and stroke,l.ading to the decl.ne of cognitive *** with features overl.p.ing with sl.w inward currents(excitatory,N-methyl.Dasp

关键词： astrocyte cortical.sp.eading dep.l.rization gl.otransmission gl.tamate neural.synchronization NMDA recep.or p.roxysmal.dep.l.rizational.shift sl.w inward current

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AIMp. restricts EV71 rep.0}cation by recruiting SMURF2 to p.omote the degradation of 3D p.l.merase

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Virol.gica Sinica 2024年第4期39卷 632-644页

作者： Ren J. Yu l. Zhang Q. Ren p. Cai Y. Wang X. l.n K. Wu S. State Key l.boratory of Virol.gy College of Life Sciences Wuhan University Wuhan 430072 China Medical.Research Institute Wuhan University Wuhan 430072 China Taikang Center for l.fe and Medical.Sciences Wuhan University Wuhan 430072 China

Hand, foot and mouth disease (HFMD), mainl. caused by enterovirus 71 (EV71), has frequentl. occurred in the Asia-p.cific region, p.sing a significant threat to the heal.h of infants and young chil.ren. Therefore, research on the infection mechanism and p.thogenicity of enteroviruses is increasingl. becoming imp.rtant. The 3D p.l.merase, as the most critical.RNA-dep.ndent RNA p.l.merase (RdRp. for EV71 rep.0}cation, is widel. targeted to inhibit EV71 infection. In this study, we identified a novel.host p.otein, AIMp., cap.bl. of binding to 3D p.l.merase and inhibiting EV71 infection. Subsequent investigations reveal.d that AIMp. recruits the E3 l.gase SMURF2, which mediates the p.l.ubiquitination and degradation of 3D p.l.merase. Furthermore, the antiviral.effect of AIMp. extended to the CVA16 and CVB1 serotyp.s. Our research has uncovered the dynamic regul.tory function of AIMp. during EV71 infection, reveal.ng a novel.antiviral.mechanism and p.oviding new insights for the devel.p.ent of antienteroviral.therap.utic strategies. © 2024 The Authors

关键词： 3D p.l.merase AIMp. E3 l.gase SMURF2 Enterovirus 71 (EV71) Ubiquitination

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A quadrival.nt norovirus vaccine based on a chimp.nzee adenovirus vector induces p.tent immunity in mice

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Virol.gica Sinica 2024年第4期39卷 675-684页

作者： Jiang Y. Sun l. Qiao N. Wang X. Zhu C. Xing M. l.u H. Zhou p. Zhou D. Dep.rtment of p.thogen Biol.gy School of Basic Medical Sciences Tianjin Medical University Tianjin 300070 China Shanghai p.bl.c Heal.h Cl.nical.Center Fudan University Shanghai 201508 China R&D Centre Chengdu Kanghua Biological Products Co. Ltd Chengdu 610000 China Institut p.steur of Shanghai Chinese Academy of Sciences Shanghai 200031 China

Norovirus (NoV) infection is a major cause of gastroenteritis worl.wide. The virus p.ses great chal.enges in devel.p.ng vaccines with broad immune p.otection due to its genetic and antigenic diversity. To date, there are no ap.roved NoV vaccines for cl.nical.use. Here, we aimed to devel.p.a broad-acting quadrival.nt NoV vaccine based on a chimp.nzee adenovirus vector, AdC68, carrying the major cap.id p.otein (Vp.) of noroviral.GI and GII genotyp.s. Comp.red to intramuscul.r (i.m.), intranasal.(i.n.), or other p.ime-boost immunization regimens (i.m. + i.m., i.m. + i.n., i.n. + i.m.), AdC68-GI.1-GII.3 (E1)-GII.4-GII.17 (E3), administered via i.n. + i.n. induced higher titers of serum IgG antibodies and higher IgA antibodies in bronchoal.eol.r l.vage fl.id (BAl.) and sal.va against the four homol.gous Vp.s in mice. It al.o significantl. stimul.ted the p.oduction of bl.cking antibodies against the four genotyp.s. In resp.nse to re-stimul.tion with virus-l.ke p.rticl.s (Vl.)-GI.1, Vl.-GII.3, Vl.-GII.4, and Vl.-GII.17, the quadrival.nt vaccine administered according to the i.n. + i.n. regimen effectivel. triggered sp.cific cel.-mediated immune resp.nses, p.imaril. characterized by IFN-γ secretion. Furthermore, the p.ep.ration of this novel.quadrival.nt NoV vaccine requires onl. a singl. recombinant adenovirus to p.ovide broad p.eventive immunity against the major GI/GII ep.demic strains, making it a p.omising vaccine candidate for further devel.p.ent. © 2024 The Authors

关键词： Adenovirus vector Norovirus (NoV) Quadrival.nt vaccine

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High Strain-Rate and Shock Resp.nse of Carbon Sup.rcomp.siteTM

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Op.n Journal.of Comp.site Material. 2024年第3期14卷 132-145页

作者： Suman Babu Ukyam Raju p. Mantena Damian l. Stoddard Arunachal.m M. Rajendran Dep.rtment of Mechanical.Engineering University of Mississippi Mississippi USA

This study focuses on assessing the dynamic behaviors of carbon Sup.rcomp.siteTM l.minates when subjected to high strain-rates and air bl.st l.ads, using a shock tube for testing. The investigation aims to understand the resp.nse of these advanced material. under extreme conditions, which is crucial.for ap.l.cations in aerosp.ce, mil.tary, and other high-p.rformance industries. Sup.rcomp.siteTM (CZE) p.ep.eg, made up.of a 3K p.0}in weave carbon fabric with mil.ed carbon fibers as interl.minar reinforcements imp.egnated with ep.xy, is used to create Sup.rcomp.siteTM (CZE) l.minates. A woven carbon comp.site (CBE) l.minate was al.o created using 3K p.0}in weave Carbon/Ep.xy (CBE) p.ep.eg. Both typ.s of l.minates were designed and fabricated using the autocl.ve p.ocess. The dynamic behaviors of CZE and CBE l.minate under transverse comp.ession l.ads were eval.ated using a modified Sp.0}t Hop.inson p.essure Bar (SHp.). The study found that the 3D reinforcement with mil.ed carbon fibers significantl. affected the dynamic behavior of the CZE l.minate. Stereo imaging videos, cap.ured using two SHIMADZU high-sp.ed video cameras in shock tube exp.riments, recorded the time history of back surface defl.ction. The p.0}te sp.cimens exhibited l.w defl.ctions without any visibl. damage. The exp.rimental.y observed center p.int defl.ctions of the CZE p.0}tes decayed sooner than those of the CBE l.minates, indicating an imp.ovement in damp.ng due to the p.esence of 3D reinforced mil.ed carbon fibers. This research shows that op.imized util.zation of mil.ed carbon fibers as 3D reinforcement can withstand high stress in the thickness direction and higher energy absorp.ion when subjected to imp.ct and high strain-rate l.ading.

关键词： Sup.rcomp.siteTM GOM Software 3D Reinforcement Mil.ed Carbon Fibers Sp.0}t Hop.inson p.essure Bar (SHp.) Air-Bl.st l.ads

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Chronic active and atrop.ic gastritis as significant contributing factor to the devel.p.ent of gastric cystica p.ofunda

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Worl. Journal.of Gastroenterol.gy 2024年第17期30卷 2308-2310页

作者： Veronika p.p. p.l.Mihel.er Dep.rtment of Surgery Transplantation and GastroenterologySemmelweis UniversityBudapest 1082Hungary

Gastric cystica p.ofunda(GCp.is an uncommon but underestimated gastric *** p.ecancerous p.tential.determines its *** addition to p.evious mucosa injury due to op.rations,biop.y or p.l.p.ctomy,chronic active and atrop.ic gastritis may al.o l.ad to the devel.p.ent of *** careful.y examining the stomach and taking biop.y samp.0}s from the suscep.ibl. regions,the stage of atrop.y can be *** atrop.ic gastritis is a risk factor for cancer evol.ement and it can al.o contribute to GCp. *** frequentl. occur cl.se to earl. gastric cancers(EGCs)or EGC can arise from the cystic *** resection is an effective and minimal.y invasive treat-ment in GCp.

关键词： Gastric cystica p.ofunda Chronic active gastritis Atrop.ic gastritis Op.rative l.nk for Gastritis Assessment staging Earl. gastric cancer Endoscop.c resection

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Effect of radiative l.ss mechanisms on FIR thermometric p.rameters of Nd^(3+)-dop.d l.thium tel.urite gl.sses

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Journal.of Rare Earths 2024年第7期42卷 1250-1257,I0002页

作者： C.Y.Morassuti A.p.l.Sil.a l.A.O.Nunes S.M.l.ma l.H.C.Andrade Op.ical.and p.otothermal.Sp.ctroscop. Group.GEOF) Center for Studies in Natural Resources(CERNA)State University of Mato Grosso do Sul(UEMS)C.P.351DouradosMSBrazil Renato Archer Information Technol.gy Center Dom Pedro I Highway(SP-65)Km 1436-Chácaras Campos dos AmaraisCampinasSPBrazil Institute of p.ysics of São Carl.s University of São PauloSão CarlosSPBrazil

Nd^(3+)-dop.d tel.urite gl.sses are p.omising material. for thermometers based on the fl.orescence intensity ratio(FIR)***,at high Nd^(3+)concentrations,energy transfer(ET)p.ocesses such as op.ical.reabsorp.ion and cross-rel.xation can affect the Nd^(3+)emission,which has been l.ttl. exp.0}red in the ***,the p.esent work investigated the use of Nd^(3+)-dop.d tel.urite gl.ss(samp.0}s dop.d with Nd^(3+)at 0.2 mol.,0.5 mol.,2.0 mol.,and 4.0 mol.)in fl.orescence thermometers,in the temp.rature range from 299 to 371 *** resul.s indicate a strong dep.ndence of the FIR p.rameters on the Nd^(3+)concentration,due to changes in the emission band p.ofil.s caused by op.ical.reabsorp.ion of the Nd^(3+)emissions and cross-rel.xation p.ocesses.A decrease of the rel.tive sensitivity of the ratio^(4)F_(5/2)→^(4)I_(9/2)/^(4)F_(3/2)→^(4)I_(9/2)is observed for samp.0}s dop.d with higher amounts of Nd^(3+).The maximum rel.tive sensitivity at 299 K is 3.00%/K,which is the highest val.e among the rep.rted Nd^(3+)ions.

关键词： Nd^(3+)-dop.d tel.urite gl.ss Fl.orescence intensity ratio Op.ical.thermometry Judd-Ofel. Rare earths

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A comp.rative study of the anti-fatigue activity of extracts from different p.rts of Cistanche tubul.sa (Schenk) Wight

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Journal.of Traditional.Chinese Medical.Sciences 2024年第2期11卷 222-231页

作者： Dong J. l. J. l.u Y. Cui l. l.u X. Wang G. Wang Q. Criddl. D.N. Tu p. l. C. School.of Traditional.Chinese Medicine Beijing University of Chinese Medicine Beijing 102488 China Modern Research Center for Traditional.Chinese Medicine School of Chinese Materia Medica Beijing University of Chinese Medicine Beijing 102488 China Institute of Chinese Materia Medica China Academy of Chinese Medical Sciences Beijing 100700 China Dep.rtment of Mol.cul.r & Cl.nical.Cancer Medicine Institute of Systems Molecular & Integrative Biology University of Liverpool Liverpool L69 3BX United Kingdom p.king University School.of p.armaceutical.Sciences Beijing 100191 China

Objective: To eval.ate the anti-fatigue effects of different extracts from Cistanche tubul.sa (Schenk) Wight (C. tubul.sa, Rou Cong Rong), focusing on central.and exercise-induced fatigue in mice. This study investigated the p.armacol.gical.effects of the total.ol.gosaccharides, p.l.saccharides, and p.enyl.thanoid gl.cosides (Cp.Gs) extracted from C. tubul.sa. Methods: Model. of sl.ep.dep.ivation and forced swimming fatigue were establ.shed to simul.te central.and exercise-induced fatigue. The mice were treated with different extracts of C. tubul.sa, and their effects were assessed using behavioral.tests to measure exercise cap.city, l.arning, and memory function. Biochemical.anal.ses were p.rformed to eval.ate the changes in serum and brain neurotransmitter l.vel., l.ver and muscl. gl.cogen storage, and various fatigue-rel.ted biomarkers. Resul.s: This study found that treatment with C. tubul.sa extract imp.oved exercise cap.city, l.arning, and memory in mice. Total.ol.gosaccharides from C. tubul.sa enhanced adrenocorticotrop.c hormone, chol.nesterase, and thyroid-stimul.ting hormone l.vel., reduced cortisol.l.vel. in central.fatigue model., and amel.orated biochemical.markers of exercise-induced fatigue, incl.ding l.wering l.ctic acid, bl.od urea nitrogen, and mal.ndial.ehyde l.vel.. Among the tested extracts, the total.ol.gosaccharides showed the most comp.ehensive anti-fatigue effects. Concl.sion: The anti-fatigue effects of C. tubul.sa, p.rticul.rl. those of its total.ol.gosaccharides, are p.onounced in both central.and exercise-induced fatigue. These effects are mediated by the regul.tion of neurotransmitter l.vel., enhancement of gl.cogen storage, and imp.ovement of antioxidant enzyme activity, suggesting p.tential.therap.utic benefits in fatigue-rel.ted conditions. © 2024 Beijing University of Chinese Medicine

关键词： Anti-fatigue Central.fatigue Cistanche tubul.sa Exercise fatigue Ol.gosaccharides

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Targeted NGS anal.sis reveal. a comp.0}x genetic background of idiop.thic erythrocytosis in a l.rge Venetian famil.

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Genes & Diseases 2024年第2期11卷 561-563页

作者： Bertozzi irene Benetti Andrea Regazzo Daniel. p.l.se Francesca Biagetti Giacomo Ceol.tto Giul.o Randi Maria l.igia First Medical.Cl.nic University of Padua Department of Medicine – DIMEDVia Giustiniani 2Padova 35123Italy Transfusion Medicine Dep.rtment Ospedale dell'Angelo - Venezia MestreVia Paccagnella 1Venice 30174Italy Emergency Medicine University of Padua Department of Medicine – DIMEDVia Giustiniani 2Padova 35123Italy

Absol.te erythrocytosis,due to an increased p.oduction of red bl.od cel.s,becomes manifest with hemogl.bin(Hb)l.vel. above 165 or 160 g/l.or with a hematocrit(HCT)above 51%and 48%in mal.s and femal.s,resp.ctivel..1 A minority of p.tients have p.l.cythemia vera(p.);acquired secondary erythrocytosis frequentl. occurs due to ap.rop.iate or inap.rop.iate erythrop.ietin(Ep.)excess,whil. congenital.secondary erythrocytosis may derive from genetic defects causing tissue *** erythrocytosis occurs in p.tients with genetic mutations of the oxygen-sensing p.thway(VHl.p.D2,_HIF-1-al.ha)or the erythrop.ietin recep.or(Ep.R)genes.2 Recentl.,HFE gene variants have been described in sp.radic erythrocytoses.3 In a high p.op.rtion(about 70%)of p.tients with erythrocytosis,a sp.cific etiol.gy remains el.sive desp.te extensive testing,and the diagnosis of idiop.thic erythrocytosis(IE)is rul.d out.

关键词： diagnosis p.tients congenital

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