简介：AbstractObjectives:To investigate the prevalence of ACADM pathogenic variants, c.985A>G and c.199T>C, for medium chain acyl CoA dehydrogenase deficiency (MCADD) in a healthy population in the southern region of Brazil.Methods:This was an observational cross-sectional study with a convenience sampling strategy. The participants were recruited from the blood bank of the Hospital de Clínicas of Porto Alegre, Brazil. A total of 1000 healthy individuals from the state of Rio Grande do Sul were included. Genotyping for the c.199T>C and c.985A>G variants was performed using real-time polymerase chain reaction (PCR) and the PCR-restriction fragment length polymorphism (RFLP) technique, respectively. Individuals considered heterozygous for c.985A>G were subjected to additional acylcarnitine profile analysis using tandem mass spectrometry. Carrier frequency was obtained by calculating the ratio of heterozygous individuals to the total number of individuals analyzed and reported with a 95% confidence interval. Allele and genotype frequencies were calculated based on the Hardy-Weinberg equilibrium.Results:The c.985A>G variant was detected as heterozygotes in three individuals (frequency of the heterozygous genotype = 1:333, allele frequency= 0.0015, minimum frequency of MCADD= 1:444,444) whose acylcarnitine profiles were within normal limits. The c.199T>C variant was not identified.Conclusions:Considering the small sample size and associated allelic heterogeneity with MCADD, these findings are believed to denote the rarity or underdiagnosis of MCADD in southern Brazil. This study provides evidence for the need for further investigation to ascertain the contribution of these diseases to child morbidity and mortality in the country.

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简介：Fourteennew1-acyl-5-arylbiuretsweresynthesizedbythereactionofarylureaswithacylisocynantes.Thelatterwasobtainedfromacylchlorideandleadcyanateindichloromethane,usingpyridineascatalyst.Thestructuresofcompounds3a-nwereconfirmedbyIR,1HNMRandMSspectra.

简介：BackgroundAlcoholdehydrogenase(ADH)andaldehydedehydrogenase2(ALDH2)arethekeyenzymesforalcoholmetabolism.SeveralgeneticstudieshaveinvestigatedtheassociationbetweenADHandALDH2geneticpolymorphismsandserumlipidprofile(SLP),however,theresultswereinconsistent.MethodsFourteenarticlesinvolving27,917participantswereincludedinthismeta-analysis.Weightedmeandifference(WMD)and95%confidenceintervals(CIs)werepresentedusingrandomeffectsmodel.PublicationbiaswasevaluatedbyfunnelplotandBegg’stest.Inaddition,tofurtherexploretheheterogeneity,subgroupanalysiswereperformed.ResultsOverall,therewasnoassociationbetweenADHgeneticpolymorphismsandSLPwithnoregardfordrinkingstatus.However,comparedwithALDH2wildhomozygousgenotype,ALDH2mutantgenotypeswereassociatedwithsignificantdecreaseinserumhighdensitylipoproteincholesterol(HDLC)(WMD-1.80mg/dL,95%CI-1.88to-1.72,P<0.001)andtotalcholesterol(TC)levels(WMD-1.10mg/dL,95%CI-1.59to-0.62,P<0.001),andsignificantincreaseinserumlowdensitylipoproteincholesterol(LDL-C)level(WMD0.30mg/dL,95%CI0.18to0.43,P<0.001).Althoughtherewasnosignificantdifferenceinserumtriglyceridelevelintheoverallpopulation,subgroupanalysisrevealedthatcomparedwithALDH2*1wildhomozygote,ALDH2*2alleledisplayedasignificantdifferenceinserumtriglyceridelevelbetweenthefemaleandmale(female:WMD1.69mg/dl,95%CI1.08to2.30,P<0.001;male:WMD-6.42mg/dL,95%CI-12.15to-0.68,P=0.028).ConclusionADHgeneticpolymorphismhasnoassociationwithSLP,regardlessofsexcategoryanddrinkingstatus.ALDH2geneticpolymorphismhasslightassociationwithHDL-C,LDL-CandTClevelsandsex-specificassociationwithserumtriglyceridelevel.WhetherornottheassociationbetweenADH2geneticpolymorphismsandSLPisresultedfromalcoholconsumptionneedsfurtherinvestigation.

简介：中华医学会第十三届全国骨科学术会议暨第六届COA国际学术大会日前在国家会议中心隆重举办。来自全球46个国家和地区及80多个国际组织的逾万名骨科医师、专家学者等齐聚国家会议中心。

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简介：在包含离子的液体(IL)的系统的马肝白酒脱氢酶(HLADH)的催化特征(BMIm????????????????猠牵牰獥敳?????????€污敫敮刯?????????ǐ

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简介：Recentstudieshaverevealedthatthepropertyofdrugismainlyassociatedwiththebody'ssubstanceandenergyme-tabolism.ThepresentstudyaimedtoevaluatethedrugpropertyofPoria,calledFuling(FL)intraditionalChinesemedicine(TCM),intermsofitseffectsonthesubstanceandenergymetabolisminratmodelsofcold-deficiencyandheat-deficiencysyndromes,comparedwithAconitiLateralisRadixPraeparaia,calledFuzi(FZ)inTCM,withhotproperty,andAnemarrhenaeRhizoma,calledZhimu(ZM)inTCM,withcoldproperty,asreferencedrugs,respectively.Theappearancescore,toeandrectaltemperaturesoftheanimalstreatedwereassessedatdifferenttimepoints.Severalindicesinvivocorrelatedwithsubstanceandenergymetabolism(glucokinas,phosphoglyceratekinase,cytochromecreductase,cytochromecoxydase,andNa^+-K^+-ATPase),endocrinesystem(triiodothyronine,thyroxine,and17-hydroxycorticosteroid),nervoussystem(acetylcholinesterase),andcyclicnucleotidesystemweredetermined.Thechangesinappearancescoreandindicesinvivosuggestedthesuccessfulestablishmentofcold-deficiencyandheat-deficiencysyndromemodels.FZreversedthedecreasedlevelsofindices(substanceandenergymetabolismandendocrinesystem)andalleviatedthesyndromeofcold-deficiencymodel,andZMshowedobviouslytherapeuticeffectonheat-deficiencysyndrome(appearancescore,substanceandenergymetabolism,andendocrinesystem).FLcouldalleviatecold-deficiencysyndromeandraisethedecreasedlevelsofglucokinas,phosphoglyceratekinase,cytochromecreductaseandtriiodothyronineincold-deficiencymodel,buthadnosignificanteffectonheat-deficiencysyndrome.DrugpropertyofFLwasinferredastrendingto"flatandwarm",whichstillneedfurtherstudy.Itwasadvisabletoadoptbothcold-deficiencyandheat-deficiencymodelstostudythedrugswith"flat"property.

简介：武港国贸总公司近日与川崎汽船签订了延长COA合同协议，该合同将使武钢海运日期租水平大幅下降，有利于国贸降成本目标的实现。2007年武钢与川崎汽船签订了为期5年期的巴西航线COA合同。

简介：Theinvitroandinvivoeffectsofcornpeptides(CPs)preparedfromcornglutenmealbyproteolysiswithanalkalineproteaseandfractionsofCPsfromSephadexG-15andG-10columnsonactivitiesofalcoholdehydroge-nase(ADH)werestudied.TheresultsshowthatCPsandfraction3ofCPsfromSephadexG-10columnenhanceinvitroADHactivity.Furthermore,theinvitroacceleratingeffectofthefraction3ofCPsonADHactivitywassuperiortothatofglutathione,whichwasalsofoundeveninthepresenceofADHinhibitor,suchaspyrazole.Intheinvivoexperiments,theanimalswerefedwithdifferentdosagesofCPsandwithadoseofChinesedistilledspiritorally,andsacrificedforthemeasurementofADHactivity.InvivoexperimentalresultsindicatethatCPSenhancedhepaticADHactivities.TotestthesafetyofCPsashealthfood,30dfeedingtestwasperformed.NoobvioustoxiceffectsweredetectedintreatedWistarrats.

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简介：Threegenericformsofgovernance--hierarchy,hybridandmarketareproposedinthenewinstitutionaleconomics,givinganabstractdescriptionofalternativecoordinationandcontrolmechanismsforgeneraluse.Whilethistheoryisknownasfarawayfrombeingoperationalandspecified,afewresearchersinthemanagementandorganizationareaaretryingtoextendGalbraithand/orMintzberg'sorganizationaldesigntheoriesintotheapplicationoflogisticalcoordinationforobtainingasmoothflowofproductsalongthesupplychainwithinandwithoutthefocalorganization.Inthispaper,wepresentanexplorativecasestudyonthedesignofvariousorganizationalarchitecturesoflogisticsactivitiesandtheirrelevantcoordinationmechanisms,especiallyinternalandexternalcontractsasonenewkindofhybridmechanismsforcoordinatingeffectivesupplychains.

简介：AbstractImportance:There is a high incidence of iron deficiency in children worldwide. Notably, however, while iron deficiency is the most common cause of anemia, little is known about the prevalence and different types of iron deficiency in neuroblastoma patients.Objective:The aim of the present study was to investigate the prevalence of iron deficiency in patients newly diagnosed with neuroblastoma.Methods:A total of 195 newly diagnosed neuroblastoma patients from November 2015 to January 2018 were analyzed retrospectively. The survival analysis was estimated by the Kaplan-Meier method.Results:Of the 195 neuroblastoma patients included in the study, 121 (62.1%) had iron deficiency, 55 (28.2%) had absolute iron deficiency, and 66 (33.9%) had functional iron deficiency. Being aged ≥ 18 months, tumor originating in the abdomen, International Neuroblastoma Risk Group Staging System M, high-risk neuroblastoma, lactate dehydrogenase ≥ 1500 U/L, neuron-specific enolase ≥ 100 U/L, unfavorable histologic category, MYCN amplification, chromosome 1p loss, and bone marrow metastasis were associated with significantly higher rates of functional iron deficiency (P < 0.05).Interpretation:Functional iron deficiency at the time of initial neuroblastoma diagnosis predicted lower event-free survival. Long-term effects of iron supplementation in neuroblastoma patients with different types of iron deficiency need to be further studied.

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简介：Avolume-basedmethodformeasuringparticle-sizedistribution(PSD)fractaldimensionsofporousmediumswasdevelopedbyemployinglasersize-analyzingtechnology.ComparedwithconventionalapproachesofusinghydrometerorscreentodeterminePSD,thismethodcanavoidcalculationerrorsandmeasuresmallersize-scaleporousmedium.Inthispapertheexperimentalporousmediumswerebrownsoil,kaolinandsandsoil.Amicro-orderofmagnitude(10^-5m)inparticle-sizeintervalcouldbeshowninPSDresultsofbrownsoilandkaolin.Theexperimentsindicatedthatbrownsoilhadanearlymono-fractalPSDcharacter,whilekaolinandsandsoilshowedmulti-fractalPSDcharacters.Bytheadsorptionisothermexperiments,thePSDfractaldimensionsofthesandsoilwerealsofoundtokeepalinearlyincreasingrelationwiththelinearadsorptiveparametersofthesoilsindifferentintervalstoadsorbbenzenefromaqueoussolution.

简介：AbstractObjective:Rubicon is an inhibitory interacting protein of the autophagy-related protein Uvrag. We previously showed that Rubicon deficiency promotes autophagic flux in vivo and that autophagy can degrade lipid droplets. This study aimed to investigate the effects of Rubicon deficiency on fasting-induced hepatic steatosis.Methods:Two-month-old wild-type (WT) and Rubicon-deficient mice were subjected to feeding or fasting for 24 hours to induce hepatic steatosis. The distribution of liver lipid droplets was revealed by oil red O staining. Hepatic and plasma triglyceride, non-esterified fatty acid (NEFA), and cholesterol levels were detected using commercially available kits. Real-time reverse transcriptasepolymerase chain reaction was performed to analyze the mRNA expression of genes related to lipid metabolism in the liver. Western blot was conducted to assess autophagy-related protein levels in the liver. The animal experiments were approved by the Institutional Animal Care and Use Committee at Shanghai Jiao Tong University, China.Results:We showed that under fasting conditions, Rubicon-deficient mice had more lipid droplets in the liver than WT controls. Consistent with these results, the hepatic triglyceride, NEFA, and cholesterol levels in fasted Rubicon-deficient mice were significantly higher than those of fasted WT controls. The levels of SREBP-1, a key regulator of lipid synthesis, were significantly lower in livers from fasted WT mice than those of fed WT mice. However, the decrease in SREBP-1 in fasted mice was attenuated by Rubicon deficiency. Western blot analysis demonstrated that the fasting-induced increase in autophagic flux was amplified by Rubicon deficiency. Finally, we showed that Rubicon deficiency in mice led to elevated plasma triglyceride and NEFA acid levels under fasting conditions.Conclusion:Rubicon deficiency exacerbates fasting-induced hepatic steatosis in mice.

简介：AIM:Tocomparethecornealparametersofchildrenwithcongenitalisolatedgrowthhormonedeficiencyandhealthysubjects.METHODS:Inthiscross-sectional,prospectivestudy,50caseswithgrowthhormone(GH)deficiencytreatedwithrecombinantGHand71healthychildrenunderwentacompleteophthalmicexamination.Thecornealhysteresis(CH),cornealresistancefactor(CRF),Goldmann-correlatedintraocularpressure(IOPg)andcorneal-compensatedintraocularpressure(IOPcc)weremeasuredwiththeOcularResponseAnalyzer(ORA).Centralcornealthickness(CCT)wasmeasuredbyaultrasonicpachymeter.RESULTS:Themeanagewas13.0±3.0yearsintheGHdeficiencygroupconsistingof21femalesand29malesand13.4±2.4yearsinthehealthychildrengroupconsistingof41femalesand30males.Therewasnostatisticallysignificantdifferencebetweenthegroupsforgenderorage(Chi-squaretest,P=0.09;independentttest,P=0.28,respectively).ThemeandurationofrecombinantGHtherapywas3.8±2.4yinthestudygroup.ThemeanCH,CRF,IOPgandIOPccvalueswere11.0±2.0,10.9±1.9,15.1±3.3,and15.1±3.2mmHgrespectivelyinthestudygroup.Thesamevalueswere10.7±1.7,10.5±1.7,15.2±3.3,and15.3±3.4mmHgrespectivelyinthecontrolgroup.ThemeanCCTvalueswere555.7±40.6,545.1±32.5μminthestudyandcontrolgroupsrespectively.TherewasnostatisticallysignificantdifferencebetweenthetwogroupsforCH,CRF,IOPg,IOPccmeasurementsorCCTvalues(independentt-test,P=0.315,0.286,0.145,0.747,0.13respectively).CONCLUSION:OurstudysuggeststhatGHdeficiencydoesnothaveaneffectonthecornealparametersandCCTvalues.ThisobservationcouldbebecauseofthedurationbetweenthebeginningofdiseaseandthediagnosisandbeginningofGHtherapy.

简介：Therearetwopartsinthispaper.InthefirstpartweconstructtheMarkovchaininrandomenvironment(MCRE),theskewproductMarkovchainandp-θ~→chainfromarandomtransitionmatrixandatwo-dimensionalprobabilitydistribution,andinthesecondpartweprovethattheinvarianceprincipleforp-θ→chain,amorecomplexnon-homogeneousMarkovchain,istrueundersomereasonableconditions.Thisresultismorepowerful.