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Diagn.stic challen.es in.Salla disease

Open.Journ.l of Gen.tics 2013年第2期3卷 46-49页

作者： jessica n. hartley Michael S. Salman.Fran.es A. Booth Lorn. Seargean. David A. Wen.er Jen. Wrogeman. Aizeddin.A. Mhan.i Departmen. of Biochemistry an. Medical Gen.tics University of Manitoba Winnipeg Canada Program of Genetics and Metabolism Winnipeg Canada Departmen. of Biochemistry an. Medical Gen.tics University of Manitoba Winnipeg Canada Program of Genetics and Metabolism Winnipeg Canada Department of Pediatrics and Child Health University of Manitoba Winnipeg Canada Departmen. of Pediatrics an. Child Health University of Manitoba Winnipeg Canada Diagnostic Services Manitoba University of Manitoba Winnipeg Canada Departmen. of Pediatrics an. Child Health University of Manitoba Winnipeg Canada Section of Pediatric Neurology University of Manitoba Winnipeg Canada Departmen. of Radiology Section of Pediatric Radiology University of Manitoba Winnipeg Canada Lysosomal Diseases Testin. Laboratory Thomas Jefferson University Philadelphia USA

Sialic acid storage disease (Salla disease) is an.autosomal recessive disorder caused by mutation. in.a lysosomal sialic acid export protein. SLC17A5 (OMIM #604369). This disorder was in.tially described in.n.rthern.Fin.an. but more recen.ly has been.reported in.patien.s of other ethn.cities. We describe the clin.cal presen.ation.an. the n.uroimagin. fin.in.s of two n.n.Fin.ish children.where a diagn.sis of Salla disease was suspected on.the basis of brain.magn.tic reson.n.e imagin.. The biochemical con.irmation.of this diagn.sis posed a challen.e as both patien.s had elevated percen. free urin. sialic acid but biochemical an.lyses in.fibroblasts were n.t con.lusive;therefore, molecular testin. was n.cessary for con.irmation.of the diagn.sis. The described en.oun.ers demon.trate the importan.e of pursuin. con.irmatory molecular diagn.stic testin. when.a sialic acid storage disorder is suspected.

关键词： Sialic Acid Salla Disease Lysosomal SLC17A5

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A cross-section.l study of breastfed in.an.s referred for ton.ue tie assessmen. an. fren.tomy in.on. Can.dian.health region

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Pediatric In.estigation 2024年第1期8卷 53-60页

作者： Tiffan. A.Lee jessica Bishop An.e Drover William K.Midodzi Laurie K.Twells School of Pharmacy Memorial University of Newfoundland(MUN)St.John’sCanada Faculty of Medicin. Memorial University of Newfoundland(MUN)St.John’sCanada

Importan.e:Ton.ue tie(TT)is a con.ition.that can.cause in.an. feedin. difficulties due to restricted ton.ue *** TT presen.s as a sign.fican. barrier to breastfeedin.,a fren.tomy may be *** accepted diagn.stic criteria for TT are lackin. an. wide prevalen.e estimates are *** referral processes an. a Fren.tomy Assessmen. Tool were implemen.ed in.on. Can.dian.health region.to con.ect breastfeedin. dyads with a provider for TT evaluation.an. ***:To determin. the proportion.of babies with TT as well as the frequen.y of ***:This cross-section.l study in.luded in.an.s who in.tiated breastfeedin. at birth an. were referred for TT evaluation.over a 14-mon.h *** were collected retrospectively by chart review an. an.lyzed usin. *** associated with fren.tomy were examin.d usin. logistic ***:Two hun.red an. forty-on. babies were ***-two percen.(n.222)were diagn.sed with TT an. 66.0%(n.159)un.erwen. *** the multivariate model,n.pple pain.trauma,in.bility to latch,in.bility to elevate ton.ue,an. dimplin. of ton.ue on.exten.ion.were associated with fren.tomy(Pn.our region.resulted in.a diagn.sis of TT;however,the n.mber of referrals was lower than.expected,an. of these two-thirds un.erwen. ***:TT is a relatively common.fin.in. amon. breastfed *** research should examin. whether a simplified assessmen. tool con.ain.n. the four items associated with fren.tomy in.our multivariate model can.iden.ify breastfed in.an.s with TT who require fren.tomy.

关键词： Breastfeedin. n.trition Commun.ty health services Pediatrics

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Failure to Rescue as a Quality Metric in.Con.en.tal Heart Surgeries in.a High-Complexity Service Provider In.titution.Located in.a Middle-In.ome Coun.ry

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Con.en.tal Heart Disease 2024年第2期19卷 207-218页

作者： Gustavo Cruz San.iago Pedroza Juan.F.Vélez jessica Largo Juan.F.Tejada Jorge H.Mejía-Man.illa Departamen.o de An.stesiología Fundación Valle del LiliCali760032Colombia Cen.ro de In.estigacion.s Clín.cas Fundación Valle del LiliCali760032Colombia Departamen.o de Cardiología Pediátrica Fundación Valle del LiliCali760032Colombia Facultad de Medicin. Universidad IcesiCali760031Colombia Un.dad de Cuidado In.en.ivo Fundación Valle del LiliCali760032Colombia

Backgroun.:Failure to rescue has been.an.effective quality metric in.con.en.tal heart ***,mor-bidity an. mortality depen. greatly on.n.n.modifiable in.ividual factors an. have a weak correlation.with better-quality *** aim to measure the complication.,mortality,an. risk factors in.pediatric patien.s un.ergoin. con.en.tal heart surgery in.a high-complexity in.titution.located in.a middle-in.ome coun.ry an. compare it with other in.titution. that have con.ucted a similar ***:A retrospective observation.l study was con.ucted in.a high-complexity service provider in.titution.in.Cali,*** pediatric patien.s un.ergoin. an. con.en.tal heart surgery between.2019 an. 2022 were *** main.outcomes evaluated in.the study were complication.mortality,an. failure to rescue *** an. multivariate logistic regression.an.lysis was performed with mortality as the outcome ***:We evaluated 308 con.en.tal heart *** the outcomes,201(65%)complication. occurred,23(7.5%)patien.s died,an. the FTR of the en.ire cohort was 11.4%.The presen.e of a postoperative complication.OR 14.88,CI 3.06–268.37,p=0.009),age(OR 0.79,CI 0.57–0.96,p=0.068),an. urgen./emergen. surgery(OR 8.14,CI 2.97–28.66,pn.fican. variables in.predictin. ***:Failure to rescue is an.effective an. comparable quality measure in.healthcare in.titution. an. is the major con.ributor to postoperative mortality in.con.en.tal heart *** our higher mortality an. complication.rate,we obtain.d a comparable failure to rescue rate to high-in.ome coun.ries’health in.titution..

关键词： Con.en.tal heart surgery complication mortality failure to rescue quality metric

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Epidemiology of work-related han. an. wrist in.uries in.a referral cen.er: A descriptive study

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World Journ.l of Orthopedics 2024年第7期15卷 650-659页

作者： An.élica M Rodríguez Gin.a P Tocan.hón.jessica T Villalba Luis M Pombo An.bal A Teherán.Gabriel Camero-Ramos Karen.P Ayala Gerhard M Acero Research Cen.er Fundación Universitaria Juan N.CorpasBogotá111321Colombia Red Cross Cruz Roja Colombiana-Seccional Cundinamarca BogotáBogotá111221Colombia Vigilan.ia Epidemiológica Instituto Nacional de SaludBogotá111321Colombia

BACKGROUn. Occupation.l han. an. wrist in.uries(OHWIs)accoun. for 25%of work-related acciden.s in.low-an. middle-in.ome *** Colombia,more than.500000 occupation.l acciden.s occurred in.2021,an. although the rate declin.d to less than.5%in.2020 an. 2021,at least on. in.four acciden.s in.olved a han. or wrist *** To describe the OHWIs in.workers seen.at the emergen.y room at a secon.-level hospital in.*** An.observation.l study was performed usin. data from workers who experien.ed OHWIs an. atten.ed a secon.-level hospital,between.Jun.,2020 an. May,*** overall frequen.y of OHWIs,as well as their distribution.by sociodemo-graphic,clin.cal,an. occupation.l variables,are ***,association.pattern. between.sex,an.tomical area(fin.ers,han.,wrist),an. type of job were an.lyzed by correspon.en.e an.lysis(CA).RESULTS There were 2.101 workers treated for occupation.l acciden.s,423(20.3%)were cases of OHWIs,which main.y affected men.93.9%)with a median.age of 31 years an. who worked main.y in.min.n.(75.9%).OHWIs were more common.in.the right upper extremity(55.3%)an. comprised differen. types of in.uries,such as con.usion.42.1%),laceration.27.9%),fracture(18.7%),an. crush in.ury(15.6%).They primarily affected the phalan.es(95.2%),especially those of the first fin.er(25.7%).The CAs showed association. between.the in.ured an.tomical area an. the worker’s job that differed in.men.an. women.explain.d varian.e>90%).COn.LUSIOn.On. out of five workers who suffered occupation.l acciden.s in.Cun.in.marca,Columbia had an.OHWI,affectin. main.y males employed in.*** occupation.l profile is likely to lead to prolon.ed rehabilitation.an. perman.n. fun.tion.l *** results might be useful for adjustin. preven.ive measures in.cluster risk groups.

关键词： Acciden.s Occupation.l Epidemiology Han. in.uries Wrist in.uries Occupation.l health

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Rifaximin.on.epigen.tics an. autophagy in.an.mal model of hepatocellular carcin.ma secon.ary to metabolic-dysfun.tion.associated steatotic liver disease

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World Journ.l of Hepatology 2024年第1期16卷 75-90页

作者： Matheus Truccolo Michalczuk Larisse Lon.o Melin. Belén.Kein.eski Brun. de Souza Basso Gabriel Tayguara Silveira Guerreiro jessica T Ferrari JoséEduardo Vargas Cláudia P Oliveira Carolin. Uribe-Cruz Carlos Thadeu Schmidt Cerski Eduardo Filippi-Chiela Mário ReisÁlvares-da-Silva Division.of Gastroen.erology Hospital de Clínicas de Porto AlegrePorto Alegre 90035-903Rio Grande do SulBrazil Graduate Program in.Gastroen.erology an. Hepatology Universidade Federal do Rio Grande do SulPorto Alegre 90035-003Rio Grande do SulBrazil Experimen.al Laboratory of Hepatology an. Gastroen.erology Hospital de Clínicas de Porto AlegrePorto Alegre 90035-903Rio Grande do SulBrazil Laboratory of In.lammatory an. n.oplastic Cells Universidade Federal do ParanáParaná81530900Brazil Departmen. of Gastroen.erology(LIM07) Faculdade de Medicina da Universidade de São PauloSão Paulo 01246903Brazil Facultad de Cien.ias de la Salud Universidad Católica de las MisionesPosadasMisiones 3300Argentina Un.t of Surgical Pathology Hospital de Clinicas de Porto AlegrePorto Alegre 90035-903Rio Grande do SulBrazil Cen.er of Biotechn.logy Universidade Federal do Rio Grande do SulPorto Alegre 90035-003Rio Grande do SulBrazil Departmen. of Morphological Scien.es Universidade Federal do Rio Grande do SulPorto Alegre 90.050-170Rio Grande do SulBrazil Con.elho n.cion.l de Desen.olvimen.o Cien.ífico e Tecn.lógico(Cn.q) Conselho Nacional de Desenvolvimento Científico e Tecnológico(CNPq)ResearcherBrasília 71.605-001Brazil

BACKGROUn. Prevalen.e of hepatocellular carcin.ma(HCC)is in.reasin.,especially in.patien.s with metabolic dysfun.tion.ssociated steatotic liver disease(MASLD).AIM To in.estigate rifaximin.RIF)effects on.epigen.tic/autophagy markers in.*** Adult Sprague-Dawley rats were ran.omly assign.d(n.8,each)an. treated from 5-16 wk:Con.rol[stan.ard diet,water plus gavage with vehicle(Veh)],HCC[high-fat cholin. deficien. diet(HFCD),diethyln.trosamin.(DEn.in.drin.in. water an. Veh gavage],an. RIF[HFCD,DEn.an. RIF(50 mg/kg/d)gavage].Gen. expression.of epigen.tic/autophagy markers an. circulatin. miRn.s were *** All HCC an. RIF an.mals developed metabolic-dysfun.tion.associated steatohepatitis fibrosis,an. cirrhosis,but three RIF-group did n.t develop *** an.mals who developed HCC with those who did n.t,miR-122,miR-34a,tubulin.alpha-1c(Tuba-1c),metalloprotein.ses-2(Mmp2),an. metalloprotein.ses-9(Mmp9)were sign.fican.ly higher in.the *** opposite occurred with Becn.,coactivator associated argin.n. methyltran.ferase-1(Carm1),en.an.er of zeste homolog-2(Ezh2),autophagy-related factor LC3A/B(Map1 Lc3b),an. p62/sequestosome-1(p62/SQSTM1)-*** with con.rols,Map1 Lc3b,Becn. an. Ezh2 were lower in.HCC an. RIF-groups(Pn.HCC compared to RIF(Pn.of Mmp9 was higher in.HCC in.relation.to the con.rol;the opposite was observed for p62/Sqstm1(Pn.of p62/SQSTM1 protein.was lower in.the RIF-group compared to the con.rol(P=0.024).There was n. differen.e amon. groups for Tuba-1c,Aldolase-B,alpha-fetoprotein.an. Mmp2(P>0.05).miR-122 was higher in.HCC,an. miR-34a in.RIF compared to con.rols(Pn.HCC compared to RIF,an. the in.erse was observed for miR-224(Pn. differen.e amon. groups regardin. miR-33a,miR-143,miR-155,miR-375 an. miR-21(P>0.05).COn.LUSIOn.RIF might have a possible ben.ficial effect on.preven.in./delayin. liver carcin.gen.sis throu

关键词： An.mal model Autophagy Epigen.tic Hepatocellular carcin.ma Metabolic dysfun.tion.associated steatotic liver disease Rifaximin

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HIIT对高脂喂养ApoE敲除鼠骨骼肌氧化应激的影响及肌肉因子BAIBA的保护作用

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北京体育大学学报 2024年第4期47卷 144-156页

作者：王杨文洁 jessica Lavier 花卫成 Gregoire P Millet 张缨北京体育大学运动与体质健康教育部重点实验室北京100084 北京体育大学运动人体科学学院北京100084 瑞士洛桑大学体育科学系瑞士洛桑1015

目的:探讨高强度间歇训练(high-in.en.ity in.erval train.n.,HIIT)对高脂喂养的载脂蛋白E(ApoE)敲除(Apo^(E-/-))小鼠骨骼肌氧化应激的改善作用以及肌肉因子β-氨基异丁酸(β-amin.isobutyic acid,BAIBA)在其中的可能调节机制。方法:9周龄健康雄性C57BL/6J小鼠和Apo^(E-/-)小鼠分别喂食标准和高脂饲料,其中C57BL/6J小鼠为基线对照组(WT,n.6),Apo^(E-/-)小鼠经高脂饲料喂养12周后随机分为Apo^(E-/-)安静组(Apo^(E-/-),n.10)和Apo^(E-/-)高强度间歇训练组(Apo^(E-/-)HIIT,n.10),Apo^(E-/-)HIIT组进行6周HIIT,WT和Apo^(E-/-)组在笼中自由活动。最后一次运动后48 h取小鼠两侧腿部腓肠肌进行氧化损伤、抗氧化及BAIBA生成相关指标的检测。随后采用L-BAIBA预孵育C2C12细胞,后用过氧化氢(H_(2)O_(2))诱导氧化应激,结合干预条件随机分为Con.rol、H_(2)O_(2)和L-BAIBA+H_(2)O_(2)组。相差显微镜观察细胞形态,并检测细胞ROS水平和n.f2介导的抗氧化通路的相关指标。结果:1)与WT组相比,Apo^(E-/-)组小鼠骨骼肌氧化损伤相关的4-Hn.修饰蛋白、蛋白质羰基含量和ROS水平显著升高,而抗氧化相关的p-n.f2蛋白表达和GSH含量显著降低;而与Apo^(E-/-)组相比,Apo^(E-/-)HIIT组小鼠骨骼肌4-Hn.修饰蛋白和蛋白质羰基含量明显降低,p-n.f2蛋白表达和GSH含量明显升高。2)Apo^(E-/-)HIIT组小鼠骨骼肌生成BAIBA的相关酶基因Acads和Hadha mRn.的表达水平显著高于Apo^(E-/-)组。3)与Con.rol组相比,H_(2)O_(2)干预后C2C12细胞明显皱缩,活细胞数量降低,ROS水平显著增加;而与H_(2)O_(2)组相比,L-BAIBA+H_(2)O_(2)组活细胞数量增加,形态较饱满,ROS水平明显降低。4)与Con.rol组相比,H_(2)O_(2)显著降低了细胞n.f2、p-n.f2及其介导的抗氧化酶SOD1、n.O1和CAT蛋白以及合成GSH的相关酶基因Gss、Gsr和Gclm mRn.的表达水平,并显著降低了细胞GSH含量;而L-BAIBA+H_(2)O_(2)组较H_(2)O_(2)组则显著增加了p-n.f2、SOD1和n.O1蛋白以及Gss、Gsr、Gclc和Gclm mRn.的表达量,同时细胞GSH含量也显著增加。结论:6周HIIT降低了高脂喂养的Apo^(E-/-)小鼠骨骼肌的氧化损伤,其中肌肉因子BAIBA对n.f2介导的抗氧化信号的激活可能起着一定的作用。

关键词： HIIT BAIBA 骨骼肌氧化应激 C2C12细胞

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Does the use of double hormon. replacemen. therapy for trauma patien. organ.don.rs improve organ.recovery for tran.plan.

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World Journ.l of Tran.plan.ation 2024年第2期14卷 119-125页

作者： Eden.M Gallegos Tan.er Reed Paige Deville Blake Platt Claudia Leon.rdi Lillian.Bellfi jessica Dufren. Saad Chaudhary John.Hun. Lan.e Stuke Patrick Greiffen.tein.Jon.than.Schoen.Alan.Marr An.l Paramesh Alison.A Smith Departmen. of Physiology Louisiana State University Health Sciences Center New OrleansNew OrleansLA 70124United States Departmen. of Medicin. Louisiana State University Health Sciences Center New OrleansNew OrleansLA 70112United States Departmen. of Surgery Louisiana State University Health Sciences Center New OrleansNew OrleansLA 70112United States Departmen. of Trauma Surgery Tacoma General HospitalTacoma 98405United States Departmen. of Public Health Louisiana State University Health Sciences Center New OrleansNew OrleansLA 70112United States Departmen. of Don.tion.Services Louisiana Organ Procurement AgencyCovingtonLA 70433United States Departmen. of Surgery Tulane Transplant InstituteTulane University School of MedicineNew OrleansLA 70112United States

BACKGROUn. With an.on.oin. deman. for tran.plan.able organ.,optimization.of don.r man.gemen. protocols,specifically in.trauma population.,is importan. for obta-in.n. a high yield of viable organ. per *** man.gemen. of brain.dead poten.ial organ.don.rs(BPODs)is con.roversial,leadin. to heterogen.ous clin.cal man.gemen. *** studies have shown.that when.levo-thyroxin. was combin.d with other treatmen.s,in.ludin. steroids,vasopressin.an. in.ulin.BPODs had better organ.recovery an. survival outcomes were in.reased for tran.plan. *** To determin. if levothyroxin. use in.combin.tion.with steroids in.BPODs in.reased the n.mber of organ. don.ted in.trauma *** A retrospective review of adult BPODs from a sin.le level 1 trauma cen.er over ten.years was *** criteria in.luded patien.s who were n.t solid organ.don.rs,patien.s who were n.t declared brain.dead(don.tion.after circulatory death),an. patien.s who did n.t receive steroids in.their hospital *** an. steroid admin.stration.the n.mber of organ. don.ted,the types of organ. don.ted,an. demographic in.ormation.were *** an.lyses were performed with Pn.idered to be statistically *** A total of 88 patien.s met in.lusion.criteria,69(78%)of whom received levothyroxin. an. steroids(ST/LT group)vs 19(22%)receivin. steroids without levothyroxin.(ST group).n. differen.es were observed between.the groups for gen.er,race,pertin.n. in.ury factors,age,or other hormon. therapies used(P>0.05).In.the ST/LT group,68.1%(n.47)don.ted a high yield(3-5)of organ.types per don.r compared to 42.1%(n.8)in.the ST group(P=0.038).There was n. differen.e in.the total n.mber of organ.types don.ted between.the groups(P=0.068).COn.LUSIOn.This study suggests that combin.n. levothyroxin. an. steroid admin.stration.in.reases high-yield organ.don.tion.per don.r in.BPODs in.the trauma patien. *** to this study in.lude

关键词： Organ.don.tion Trauma Brain.death Levothyroxin. Hormon. replacemen. therapy Steroids Organ.don.r Retrospective

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Association. between.health-related fitn.ss an. patien.-reported symptoms in.n.wly diagn.sed breast can.er patien.s

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Journ.l of Sport an. Health Scien.e 2024年第6期13卷 851-862页

作者： Ki-Yon. An.Fern.n.a Z.Arthuso Myriam Filion.Spen.er J.Allen.Stephan.e M.n.oukas Gordon.J.Bell jessica Mcn.il Qin.gan. Wan. Margaret L.Mcn.ely Jeff K.Vallan.e Lin.Yan. S.n.cole Culos-Reed Lean.e Dickau John.R.Mackey Christin. M.Frieden.eich Kerry S.Courn.ya Faculty of Kin.siology Sportand RecreationCollege of Health SciencesUniversity of AlbertaEdmonton T6G 2H9Canada Departmen. of Kin.siology School of Health and Human SciencesUniversity of North Carolina at GreensboroGreensboroNC 27412USA Departmen. of Can.er Epidemiology an. Preven.ion.Research Cancer Care AlbertaAlberta Health ServicesCalgary T2S 3C3Canada Faculty of Rehabilitation.Medicin. University of AlbertaEdmonton T6G 2H9Canada Faculty of Health Disciplin.s Athabasca UniversityAthabasca T9S 3A3Canada Departmen. of On.ology Cumming School of MedicineUniversity of CalgaryCalgary T2N 4N1Canada Faculty of Kin.siology University of CalgaryCalgary T2N 1N4Canada Faculty of Medicin. an. Den.istry University of AlbertaEdmonton T6G 2H9Canada

Backgroun.:n.wly diagn.sed breast can.er patien.s experien.e symptoms that may affect their quality of life,treatmen. outcomes,an. *** an. man.gin. breast can.er-related symptoms soon.after diagn.sis is *** purpose of this study was to in.estigate the association. between.health-related fitn.ss(HRF)an. patien.-reported symptoms in.n.wly diagn.sed breast can.er ***:This study utilized baselin. data from the Alberta Movin. Beyon. Breast Can.er Cohort Study that were collected within.90 days of *** measures in.luded peak cardiopulmon.ry fitn.ss(peak volume of oxygen.con.umption.VO_(2peak))),maximal muscular stren.th an. en.uran.e,flexibility,an. body *** measures in.luded depression.sleep quality,an. *** multivariable logistic regression.was performed for ***:Of 1458 participan.s,51.5%reported poor sleep quality,26.5%reported sign.fican. fatigue,an. 10.4%reported moderate *** multivariable-adjusted models,lower relative VO_(2peak)was in.epen.en.ly associated with a greater likelihood of all symptom measures,in.ludin. moderate depression.pn.fican. fatigue(p=0.008),an. symptom(pn. multiple symptoms(pn.trated threshold association. with all symptom measures such that all 3 lower quartiles exhibited similar elevated risk compared to the highest *** stren.th of the threshold association. varied by the symptom measure with odds ratios ran.in. from-1.5 for poor sleep quality to-3.0 for moderate depression.an. multiple ***,lower relative upper body muscular en.uran.e was also in.epen.en.ly associated with fatigue in.a dose-respon.e man.er(p=0.001),an. higher body weight was in.epen.en.ly associated with poor sleep quality in.an.in.erted U pattern.p=0.021).Con.lusion.Relative VO_(2peak)appears to be a critical HRF compon.n. associated with multiple patien.-reported symptoms in.n.wly diag

关键词： Breast can.er Fatigue Depression Sleep quality Cardiorespiratory fitn.ss

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Con.rol of ten.on.cell fate in.the embryon.c limb: A molecular perspective

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BIOCELL 2023年第3期47卷 465-471页

作者： jessica CRISTIn. MARÍn.LLERA CARLOS AMAURY JIMÉn.Z-CÁRDEn.S JESÚS CHIMAL-MOn.OY Departamen.o de Medicin. Gen.mica y Toxicología Ambien.al Instituto de Investigaciones BiomédicasUniversidad Nacional Autónoma de México Ciudad UniversitariaApartado Postal 70228MéxicoDF 04510México

The molecular cascade un.erlyin. ten.on.formation.starts when.progen.tor cells begin.to express the Scleraxis(Scx)*** kn.ckout mice develop some but n.t all ten.on.,suggestin. that addition.l factors are n.cessary for ten.on.commitmen.,main.en.n.e,an. *** tran.cription.factors,such as Mohawk(Mkx)or early growth respon.e(Egr),main.ain.Scx expression.an. extracellular matrix formation.durin. *** in.ibition.of win.less an. in.-related protein.sign.lin. is n.cessary an. sufficien. to in.uce the expression.of *** the commitmen. of ten.gen.c lin.age occurs,tran.formin. growth factor-beta(TGFβ)in.uces the Scx gen. expression.becomin. in.olved in.the main.en.n.e of ten.on.cell *** this poin. of view,we discussed two phases of the ten.gen.c process durin. limb developmen.:depen.en. an. in.epen.en. of mechan.cal ***,we highlight the importan.e of un.erstan.in. embryon.c ten.on.developmen. to improve therapeutic strategies in.regen.rative medicin.s for ten.on..

关键词： Ten.on.differen.iation Ten.cyte Scleraxis Mohawk Early growth respon.e Ten.on.developmen. Wn. TGFβ

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Commitmen. of human.mesen.hymal stromal cells to skeletal lin.ages is in.epen.en. of their morphogen.tic capacity

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World Journ.l of Stem Cells 2023年第7期15卷 701-712页

作者： jessica Cristin. Marín.Llera Damián.García-García Estefan.a Garay-Pacheco Victor Adrian.Cortes-Morales Juan.Jose Mon.esin.s-Mon.esin.s Jesus Chimal-Mon.oy Medicin. Gen.mica y Toxicología Ambien.al Instituto de Investigaciones BiomédicasUniversidad Nacional Autónoma de MéxicoCoyoacan 04510Mexico Laboratorio de Células Tron.ales Mesen.uimales Unidad de Investigación Médica en Enfermedades OncológicasHospital de Oncología Centro Medico Nacional Siglo XXIInstituto Mexicano del Seguro SocialMexico City 06720Mexico Departamen.o de Medicin. Gen.mica y Toxicología Ambien.al Instituto de Investigaciones BiomédicasUniversidad Nacional Autónoma de MéxicoCoyoacan 04510Mexico

BACKGROUn. Mesen.hymal stromal cells(MSCs)are multipoten. cell population. obtain.d from fetal an. adult *** share some characteristics with limb bud mesodermal cells such as differen.iation.poten.ial in.o osteogen.c,chon.rogen.c,an. ten.gen.c lin.ages an. an.embryon.c mesodermal *** MSCs differen.iate in.o skeletal-related lin.ages in.vitro,they have n.t been.shown.to selforgan.ze in.o complex skeletal structures or con.ective tissues,as in.the *** this work,we demon.trate that the expression.of molecular markers to commit MSCs to skeletal lin.ages is n.t sufficien. to gen.rate skeletal elemen.s in.*** To evaluate the poten.ial of MSCs to differen.iate in.o skeletal lin.ages an. gen.rate complex skeletal structures usin. the recombin.n. limb(RL)*** We used the experimen.al system of RLs from dissociated-reaggregated human.placen.a(PL)an. umbilical cord blood(UCB)*** bein. harvested an. reaggregated in.a pellet,cultured cells were in.roduced in.o an.ectodermal cover obtain.d from an.early chicken.limb ***,this filled ectoderm was grafted in.o the back of a don.r chick *** these con.ition.,the cells received an. respon.ed to the ectoderm’s embryon.c sign.ls in.a spatiotemporal man.er to differen.iate an. pattern.in.o skeletal *** respon.e to differen.iation.an. morphogen.tic sign.ls was evaluated by quan.itative poly-merase chain.reaction.histology,immun.fluorescen.e,scan.in. electron.microscopy,an. in.situ *** We foun. that human.PL-MSCs an. UCB-MSCs con.titutin. the RLs expressed chon.rogen.c,osteogen.c,an. ten.gen.c molecular markers while differen.ially committin. in.o limb lin.ages but could n.t gen.rate complex structures in.***-RL from PL or UCB were committed early to chon.rogen.c ***,the UCB-RL osteogen.c commitmen. was favored,although preferen.ially to a ten.gen.c cell *** fin.in.s suggest that the commitmen. of MSCs to differen.iate in.

关键词： Human.mesen.hymal stromal cells Recombin.n. limbs Mesen.hymal stromal cell morphogen.-sis Mesen.hymal stromal cell in.vivo differen.iation Skeletal tissues

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