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简介：目的:了解我国Leber遗传性视神经病变(Leber'shereditaryopticneuropathy,LHON)线粒体DNA(mtDNA)14484位点突变患者的发病率和临床特征.方法:对来自117个家系的119例临床确诊或疑诊LHON的患者进行mtDNA检测.对3例证实为14484位点突变的家系做深入调查并收集相关病史及临床资料,抽取15例家属的血样进行mtDNA检测.结果:存在线粒体DNA突变的62例(62/119,52.1%)中,14484位点突变仅3例(4.8%).该3例3个家系56例中,28例有眼部症状,外显率50%.发病经过和临床表现类似11778位点突变的LHON,但其中视力恢复者17例(60.7%).15例家属的血样检测再次证实为14484位点突变.结论:我国LHON患者中14484位点突变者少见,其临床表现与11778位点突变者相似,惟视力恢复率高.

简介：AIM:Toinvestigatethepossiblerelationshipbetweentheinfluencingfactorsoccurringbeforeandduringbirthinfull-terminfantsandtheoutcomeofretinopathy.·METHODS:Totally816full-terminfantsadmittedintheneonateintensiveunitofBoaiHospitalofZhongshanbetween1May,2008and30June,2011wereincludedinthestudy.Fundusexaminationwasperformedandevaluatedindividuallyonthemattheageof48hoursafterdelivery,2weeksand1month.Somepossibleriskfactorshappeningprenatallyorduringdeliverysuchaspregnantrelatedhypertension,placentaprevia,placentalabruptionetc,aswellassomeneonatalriskfactorssuchasneonatalasphyxia,hypoxic-ischemicencephalopathy(HIE),lowbirthweightetc,wererecordedandevacuated.Thentheeffectoftheriskfactorsoffull-terminfantsonretinopathywasstudied.·RESULTS:Theincidenceofretinalhemorrhageoffull-terminfantswithprenatalpregnantrelatedhypertension(PRH)ofthemother(43.6%)wassignificantlyhigherthanthatoffull-terminfantswithout(8.0%).(P<0.001).Theincidenceofretinalhemorrhageoffull-terminfantswithneonatalasphyxiaand/orhypoxic-ischemicencephalopathy(HIE)(29.3%)wassignificantlyhigherthanthatofthosewithout(15.7%),butcorrelationwasnotfoundbetweentheseverityofretinahemorrhageandthedegreeofhypoxicdisease.Apalecolorofopticdiscwasassociatedwithalowbirthweightoffull-terminfant.Full-terminfantswithbirthweighlessthan2500ghadasignificanthigherincidenceofretinopathythanthosewithbirthweightequalormorethan2500g(P<0.001).·CONCLUSION:Themaininfluencingfactorswhichleadtoretinopathyofhighriskfull-terminfantsareprenatalfactorssuchasPRH,andsomeneonatalriskfactorssuchasasphyxia,hypoxic-ischemicencephalopathy,andlowbirthweight.

简介：由中国科学评价中心,武汉大学图书馆及中国科教评价网联袂研发的《中国学术期刊评价研究报告》——RCCSE权威期刊,核心期刊排行榜与指南于2011年8月由科学出版社正式出版,这在国内外期刊评价史上具有新的里程碑意义。

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简介：<正>1926年11月生。1954年毕业于中南同济医学院。毕业后留同济医科大学附属协和医院工作,长期从事耳鼻咽喉科的医疗、教学和科研工作,现为主任医师、教授。曾任同济医科大学附属协和医院院长,曾先后在苏联、法、

简介：<正>计算机技术的不断进步,促进了多媒体技术的崛起和飞速发展,其应用已遍及国民经济与社会生活的各个角落,正在对人类的生产方式、工作方式乃至生活方式带来巨大的变革。多媒体技术是以计算机为中心,把语音处理技术、图像处理技术、视听技术集成在一起,把语音信号,图象信号先通过模数转

简介：目的探讨视网膜脱离患者的健康教育形式。方法以视网膜脱离患者为教育对象，通过护士对患者入院时、手术前、手术后、出院后四个不同时期进行有目的、有计划、系统性的教育，使患者掌握健康知识，并使其行为向有利于健康的方向发展。结果视网膜脱离患者术后视力恢复均较满意，且治疗期间未发生并发症。结论健康教育的应用对视网膜脱离患者的康复起到了促进作用，提高了患者适应能力和生活质量，保证了手术的成功率，同时和患者建立了良好的护患关系。

简介：AIM:ToevaluatethecornealendothelialcelldensityandmorphologyinChinesepatientswithpseudoexfoliationsyndrome(PEX).·METHODS:Medicalrecordsof16patients(20eyes)withPEXwhopresentedtoourinstitutionbetweenJuly2008andJune2010wereretrospectivelyreviewed.Thirteeneyeshadcombinedglaucoma.Theinformationoffiveapparentlynormalfelloweyesinthesepatientswasalsorecorded.Lefteyesof20patientswithbilateralsenilecataractsbutnoothereyediseasewereincludedascontrols.Specularmicroscopywasperformedinalleyestoanalyzeforcornealendothelialcelldensityandmorphology.Celldensity,coefficientofvariationincellsize,andpercentageofhexagonalcellsincornealendotheliumwereevaluated.·RESULTS:ThemeancornealendothelialcelldensityinthePEXeyeswas2298±239cells/mm2,significantlylowerthanthatinthecataracteyes(2652±18cells/mm2,P=0.026),buttherewerenosignificantdifferencesincoefficientofvariationofcellsizeandfrequencyofhexagonalitybetweenthesetwogroups.NosignificantdifferencesinthethreeparameterswerefoundbetweentheapparentlynormalfelloweyesandthePEXeyesorthecataracteyes,orbetweenthePEXeyeswithandwithoutglaucoma.·CONCLUSION:CornealendothelialcelldensitymaydecreaseinChinesepatientswithPEX.ThedevelopmentofglaucomainPEXeyesdoesnotseemtoberelatedwiththechangeincornealendothelialcelldensityormorphology.

简介：目的探讨健康教育路径的实施在青光眼手术患者中的作用.方法将64例均行小梁切除术的青光眼患者分成对照组和观察组,对照组实施常规健康教育,观察组实施路径化健康教育,采用问卷调查法调查比较两组健康教育效果、患者依从性及护理满意度.结果健康教育效果观察组各项指标均高于对照组,差异有统计学意义（P＜0.05）;观察组依从性明显好于对照组,护理满意度调查观察组各维度均高于对照组,差异有显著意义（P＜0.01）.结论健康教育路径的实施,使疾病健康教育工作程序化、标准化、具体化,健康教育效果更为明显,有助于患者获得良好疗效,提高患者满意度.

简介：据中国科学技术期刊编辑学会和中国高校科技期刊研究会国际检索系统资讯部报道，数据来源——http：//www．ncbi．nlm．nih．gov／pubmed／citmatch。

简介：马凡氏综合症是一种常染色体不完全外显性遗传病,临床表现多样,主要涉及心血管、骨骼和眼等系统,病死率高。对发病患者应加强健康教育、风险管理,积极治疗护理,给予心理支持。患者生育正常儿和患儿的几率各为50%,这就意味着提高正常儿出生率是医务人员为之奋斗的目标,也是马凡氏综合症患者的福音,所以,加强遗传学、优生优育教育将成为马凡氏综合症健康教育的新动向。

简介：AIM:Tocharacterizetheclinicalfeatures,diagnosis,treatmentandprognosisofuveitisassociatedwithankylosingspondylitis(AS)inChinesepatients.·METHODS:TwohundredandthreepatientswithuveitisassociatedwithASfollowed-upintheThirdMilitaryMedicalUniversityDapingHospitalbetween2005and2010wereretrospectivelyevaluatedinthisstudy.Completeophthalmologicalexaminationswereevaluatedatbaselineandduringthefollow-upperiod.Thegender,age,follow-uptime,meanfrequencyofuveitisonset,andaccompanyingeyeexaminationfindings,history,demographicalparameterswerereviewed.Allthepatientspresentedcompleteclinicalandradiologic(sacroiliac,lumbar,dorsalandcervicalspine,knee,ankle,shoulder,hip,elbow)evaluation.HLA-B27typingwasalsosearched.·RESULTS:Therewere203patientsdiagnosedwithASassociateduveitis.AllshowedsacroiliacX-raychangesindicativeofAS.Therewere184maleand19femalepatients.Theaverageageofpatientswas35±12(range18-50).Meanfollow-upperiodwas2.4years(1-5years).Acuteanterioruveitiswasthemostcommontypeofuveitisinbothgenders.121eyespresentedunilateralinvolvement(55.2%),and92eyespresentedbilateralinvolvement(45.3%)withonsetalternately.22eyesoccurredhypopyon,16eyeswerefoundanteriorvitreouscells,7eyeswerenotedreactivemacularedemaorexudation,29eyespresentedposteriorsynechiaeofiris,and14eyespresentedcataract,9eyespresentedsecondaryglaucoma,2eyespresentedbendcornealdegenerationand1eyespresentedatrophyofeyeball.Atthefinalvisit,uveitiswaswellcontrolledinmostpatients.·CONCLUSION:ASassociatedwithuveitisinChinesepatientsmainlymanifestsasacuteanterioruveitis.AcombinationofcorticosteroidswithothermydriasisagentsiseffectiveformostASassociatedwithuveitispatients.Ingeneral,theprognosisisgoodinthesecases.

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简介：AIM:Toinvestigatethetreatmentstatusandprognosisofspace-occupyinglacrimalglandlesionsatonetertiaryeyecenterinChina.·METHODS:Aretrospectiveclinicalstudywasperformedon95patientswithspace-occupyinglesionsofthelacrimalglandsurgicallytreatedattheEye&ENTHospitalofFudanUniversityfrom2003to2007.Thereviewedclinicaldataincludedage,gender,sideofthelesion,durationofsignsandsymptoms,histopathologicaldiagnosis,treatmentmodality,recurrence(local,regional,anddistantmetastasis)andsurvival.·RESULTS:Ofthe95cases(99eyes),pleomorphicadenomaswerethemostcommonlesions(43cases),followedbylymphoiddisorders(14),inflammatorypseudotumors(11),carcinomaex-pleomorphicadenomas(11),andadenoidcysticcarcinomas(ACC,6).Therewere8patientswithrelapsedpleomorphicadenomas.Fiveofthese8caseshadmalignantpathologicalchanges.AllpatientswithACChadmetastasisandthreeofthemdiedduringtheirfollow-up.·CONCLUSION:Ourstudyindicatedthatthemostcommonlacrimalglandlesionswerepleomorphicadenomas.Multiplerecurrenceandsurgicalproceduresmayincreasetheriskoftumorprogression.ACChadahighincidenceoftumormetastasisandapoorprognosis.

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简介：AIM:ToidentifythegeneticdefectinaChinesefamilywithbilateralprogressivechildhoodposteriorcataract.METHODS:Atwo-generationfamilywasrecruitedinthisstudy.Familyhistoryandclinicaldatawererecorded.AllreportedcandidategenesassociatedwithcongenitalposteriorcataractwerescreenedbydirectDNAsequencing.·RESULTS:Allaffectedindividualspresentedposterioropacitiesinthelens.Directsequencingofthecandidategenesshowedaheterozygousc.2668C>TvariationinEPHA2gene,whichresultedinthereplacementofargininebycysteineatcodon890(p.R890C).Thismutationwasfoundintwoaffectedindividuals,butwasnotobservedin200normalcontrols.·CONCLUSION:Wereportanovelmutation(p.R890C)intheEPHA2receptortyrosinekinasegene.ThefindingexpandsthemutationspectrumofEPHA2inassociationwithposteriorcataract.