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简介：AIM:Todeterminetheclinicalfeatures,diagnosisandtreatmentoftheprimarySjogrensyndrome(SS)relatedopticneuritis.METHODS:Theclinicaldataof8patients(12eyes)withprimarySSrelatedopticneuritiswereanalyzedretrospectively.RESULTS:Eightof128consecutivepatientswithopticneuritisresultedfromvariedcausesfulfilledthediagnosticcriteriafortheprimarySS.Theypresentedinitiallywiththesignsandsymptomsofnon-specificopticneuritis,and5patientspresentingwithoutdrynessshowedachronicinflammationofsubmandibularglandorparotidgland,andlymphocyteinfiltrationwasdemonstratedbylabialglandbiopsyin2patients.Therewereserumpositivetitersforanti-SjogrensyndromeA(SSA)in7patientsandanti-SjogrensyndromeB(SSB)in8patients.Anti-aquaporin-4(AQP4)antibodywasnegativeinallthe8patients.Bothglucocorticoidsandimmunosuppressiveagentwereadministered,andvisualacuityelevatedin8eyes(66.7%),3patients(37.5%)recurredinthefollow-up.CONCLUSION:PrimarySSrelatedopticneuritisislesscommonandeasilymisdiagnosed.Theconventionaltherapiesforopticneuritiscouldnotcontroltherecurrence.

简介：鼻腔结石和鼻内异位牙较罕见,我科近年在门诊中先后遇见4例,现报道如下:

简介：P75神经营养受体是最早发现的神经营养因子受体,既往认为其主要作为高亲和力受体的辅助受体间接发挥作用.近年来研究表明,P75神经营养受体在神经系统的发育中有诱导细胞凋亡作用.现阐述其在视网膜神经细胞生长,分化以及逆向运输神经营养因子等方面的作用.

简介：喉真菌病亦称真菌性喉炎，在临床上较少见，本科收治喉真菌病4例，现报告如下。

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简介：Leber'scongenitalamaurosis(LCA)andrecentgenetherapyadvancementfortreatinginheritedretinopathieswereextensiveliteraturereviewedusingMEDLINE,PubMedandEMBASE.Adeno-associatedviralvectorswerethemostutilisedvectorsforoculargenetherapy.Conephotoreceptorcellsmightuseanalternatepathwaywhichwasnotreliantoftheretinalpigmentepithelium(RPE)derivedretinoidisomerohydrolase(RPE65)toaccessthe11-cisretinaldehydechromophore.Researcheffortsdedicatedontheprogressionofagene-basedtherapyforthetreatmentofLCA2.Suchgenetherapyapproacheswereextremelysuccessfulincanine,porcineandrodentLCA2models.TherecombinantAAV2.hRPE65v2adenoassociatedvectorcontainedtheRPE65cDNAandwasreplicationdeficient.ItsinvitroinjectionintargetcellsinducedRPE65proteinproduction.Thegenetherapytrialsthatweresofarconductedforinheritedretinopathieshavegeneratedpromisingresults.PhaseIclinicaltrialstocureLCAandchoroideremiademonstratedthatadeno-associatedviralvectorscontainingRPEgenesandphotoreceptorsrespectively,couldbesuccessfullyadministeredtoinheritedretinopathypatients.AphaseIIItrialispresentlyongoingandifsuccessful,itwillleadthewaytoadditionalgenetherapyattemptstocuremonogenic,inheritedretinopathies.

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简介：Aim:ToverifywhetherpartialintraoperativeTenon'scapsuleresection(PTCR)withadjunctiveMitomycinCiseffectiveindevelopingthin,avascularblebsineyesundergoingAhmedglaucomavalveinsertionandtoassesstheefficacyandsafetyofthisprocedure.Methods:ThisstudywasconductedinfourLatinAmericacountries(Argentina,Brazil,ColombiaandPeru).AhmedglaucomavalveimplantinsertionwithPTCR(groupA)andwithoutPCTR(groupB)wasperformedinneovascular

简介：小瞳孔白内障手术属于疑难和复杂性手术，约占白内障手术的5．19％。在我科进行超声乳化术的1500例白内障患者中，小瞳孔白内障有48例（52眼）小瞳孔，现将手术情况报告如下。

简介：AIM:ToexplorethemolecularmechanismsinlensdevelopmentandthepathogenesisofPetersanomalyinSmad4defectivemice.METHODS:Le-CretransgenicmouselinewasemployedtoinactivateSmad4inthesurfaceectodermselectively.PathologicaltechniqueswereusedtorevealthemorphologicalchangesoftheanteriorsegmentinSmad4defectiveeye.ImmunohistochemicalstainingwasemployedtoobservetheexpressionofE-cadherin,Ncadherinanda-SMAinanteriorsegmentofSmad4defectivemiceandcontrolmiceatembryonic(E)day16.5.Real-timequantitativepolymerasechainreaction(qPCR)wasperformedtodetecttheexpressionofSnail,Zeb1,Zeb2andTwist2inlensofSmad4defectivemiceandcontrolmiceatE16.5.RESULTS:ConditionaldeletionofSmad4oneyesurfaceectodermresultedincorneaidysplasia,iridocornealangleclosure,corneolenticularadhesionsandcataractresemblingPetersanomaly.LossofSmad4functioninhibitedE-cadherinexpressioninthelensepitheliumcellsandcorneaiepitheliumcellsinSmad4defectiveeye.ExpressionofN-cadherinwasupregulatedincorneaiepitheliumandcorneaistroma.BothE-cadherinandN-cadherinweredown-regulatedatthefuturetrabecularmeshworkregioninmutanteye.TheqPCRresultsshowedthattheexpressionofTwist2wasincreasedsignificantlyinthemutantlens(P<0.01).CONCLUSION:Smad4isessentialtoeyedevelopmentandlikelyacandidatepathogenicgenetoPetersanomalybyregulatingepithelial-mesenchymaltransition.Twist2canberegulatedbySmad4andplaysanessentialroleinlensdevelopment.

简介：目的：探讨眼眶IgG4相关疾病（IgG4-RD）的临床病理特点。方法：收集整理23例35眼眼眶IgG4-RD患者的临床病理资料,对其进行组织学和免疫组织化学观察,总结其临床和病理特点。结果：眼眶IgG4-RD患者23例35眼,其中男8例9眼,女15例26眼,年龄28-72（平均52.1）岁。19例30眼来源于泪腺,4例5眼来源于眶内其他部位。以泪腺区肿胀或眼球突出就诊。单侧11例,双侧12例。病程1mo-10a,平均27mo。1例1眼6mo后复发。大体：灰白色结节状肿物,泪腺表面有很薄的纤维膜包绕。组织学特点：泪腺腺泡、导管组织严重萎缩甚至消失,被大量密集的淋巴细胞、浆细胞及淋巴滤泡替代,伴有不同程度的纤维化。免疫组织化学染色：23例35眼IgG4阳性浆细胞均〉50个/HPF,IgG4/IgG阳性浆细胞比值〉40%。结论：眼眶IgG4-RD主要发生于泪腺组织,通过组织学特点和免疫组织化学IgG4的表达可明确诊断。IgG4-RD应早期筛查、预防和治疗。

简介：AIM:Tocomparethespeedofvisualrecoveryfollowingmyopicthin-flapLASIKwithfourfemtosecondlasers.METHODS:Eighty-eighteyesof46patientswhowereconsecutivelyscheduledforbilateralLASIKwiththeIntraLaseFS60(Group1),FemtoLDVCrystalLine(Group2),WavelightFS200(Group3)andVisuMax(Group4)femtosecondlaserswereenrolledin.Monocularuncorrecteddistancevisualacuity(UDVA),best-correcteddistantvisualacuity(CDVA),refraction,contrastsensitivityandhigher-orderaberrations(HOAs)wereevaluatedat1,3d,1wkand1mopostoperatively.RESULTS:Sixteeneyes(72.7%)achieved20/16and8eyes(36.4%)were20/12.5at1dinGroup2,whichwassignificantlymorethanother3groups.At1wk,20eyes(90.9%)achieved20/16inGroups2and4.At1mo,20eyes(90.9%)achieved20/16inGroup2andGroup4,whichweresignificantlymorethanothertwogroups.Whileby1mo,thedifferenceoftheresidualsphericalequivalent(SE)wasnotstatisticallysignificantamong4groups(P=0.121).Theinductionofsphericalaberration(SA)weresignificantlylessforGroups2,3,4thanforGroup1onedayaftersurgery(P=0.015).Thedifferencesamong4groupswerenotstatisticallysignificantbeforeandaftersurgeryoneverytimepoints(allP>0.05).CONCLUSION:Thethin-flapLASIKprocedureusingtheFemtoLDVCrystalLineandVisuMaxfemtosecondlasershowfastervisualperformancerecovery.

简介：气管切开术后并发无名动脉破裂出血是耳鼻喉科急症，一旦发生，往往危及患者生命。我科自2002年7月至2007年3月收治4例此类患者，均死于未能止住的大出血，现报告如下。

简介：<正>康宁克通—A系合成皮质类固醇类药物,对治疗变应性鼻炎,具有良好的疗效。我科自1993年以来,用此药作下鼻甲和迎香穴注射治疗变应性鼻炎458例,其中男性211例,治疗后出现性功能障碍者4例,现报告如下。

简介：资料患者男性，48岁。因双眼及双侧面部肿胀1年多入院。患者自诉1年多前无明显诱因出现双眼及双侧面部肿胀，局部无发红、压痛，全身无发热、畏寒，无口干、眼干、关节肿痛、口腔溃疡、脱发等症状。病程期间患者未行任何治疗。

简介：AIM:Toevaluatethelong-termresponsetothefixedcombinationofdorzolamide/timololinpatientswithprimaryopenangleglaucoma(POAG)andtheadditionofotherintraocularpressure(IOP)loweringmedicationssuchasprostaglandinanalogsandbrimonidine.METHODS:Aretrospective,non-randomized,anddescriptiveclinicalstudywasperformedwith182eyesdiagnosedwithPOAG.Patientsweredividedintothreegroups:agroupwithfixedcombinationofdorzolamide/timololonly,asecondgroupwithprostaglandinanalogsplusfixedcombinationofdorzolamide/timolol,andathirdgroupwiththeadditionofbrimonidinetothesamefixedcombination.IOPdataweregatheredretrospectivelyandthedifferencesbetweengroupswerecalculated.RESULTS:IOPwasreducedsatisfactorilyinallthreegroups;however,aprogressiveIOPreductionwasnotedinthegroupwiththefixedcombinationplusprostaglandinanalogs.Inthisgroup,aprogressive,significantandmorehomogeneousresponseofthereductionwasnotedincomparisonwiththeothergroups.CONCLUSION:IOPreductionwasefficaciousinallthreegroups.TheadditionofprostaglandinanalogsshowedprogressiveIOPreduction,progressiveresponseandabsenceoflong-termdrift.Brimonidinedidnotshowasignificantadditiveeffect.

简介：3型Stargardt病（STGD3，MIM600110）是一种常染色体显性遗传的早发性黄斑营养不良性疾病，是一种单基因遗传性疾病。目前为止，已知的STGD3致病基因为极长链脂肪酸延长酶4基因（ELOVL4）。ELOVIA是位于内质网上的一种调节极长链饱和及多不饱和脂肪酸生物合成的限速缩合反应中不可或缺的膜蛋白。作为一种遗传性疾病，STGD3目前仍无有效治疗方法。然而，饮食补充极长链多不饱和脂肪酸可能是治疗STGD3的一种可行疗法。对STGD3基因及致病机制的研究可能将有助于发现STGD3的有效治疗方法及进一步了解其他遗传性视网膜变性疾病和更复杂的年龄相关性黄斑变性。

简介：AIMTo评估关于光质量的培植根据包括的decentering.METHODSThis的不同的度估计了的V4c可植入的collamer透镜(洞ICL)的临床的结果收到了常规ICL的49只眼睛和收到了洞ICL的94只眼睛。收到了洞ICL的眼睛根据decentering的度被划分成三个组：组织1，在从学生中心的1条洞直径(HD)以内的中央洞；组织2，在到2HD的1HD以内的中央洞；并且组织3，在到3HD的2HD以内的中央洞。视觉尖酸(VA)，intraocular压力(IOP)，和球形的等价物(SE)价值在1wk被估计，1并且在外科以后的3mo。在surgery.RESULTSThere不是在VA，IOP，和SE的重要差别在之中以后，眼睛的调整转移功能，Strehl比率，客观散布索引，和更高的顺序错误(HOA)在3mo为4公里学生被测量常规并且洞ICL组。关于HOA，为昏迷和球形的错误的价值没显示出差别。全部的HOA和翘摇价值在比在组1的组2是显著地更高的(P=0.02，0.03，分别地)。在surgery.CONCLUSIONOur结果建议洞ICL培植提供等价于由常规ICL提供了那的令人满意的视觉质量以后，在关于在3mo的另外的光优秀参数的组之中没有重要差别，不管decentering的中央洞和度的存在。