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时间:2020-04-18
《更新观念,提高我国肝豆状核变性诊治的临床水平-论文.pdf》由会员上传分享,免费在线阅读,更多相关内容在行业资料-天天文库。
1、杨旭.更新观念,提高我国肝豆状核变性诊治的临床水平905更新观念,提高我国肝豆状核变性诊治的临床水平杨旭(中南大学肝病研究所,湘雅二医院肝病研究中心,长沙410011)摘要:肝豆状核变性是一少见而可治疗的遗传性疾病,介绍本病最新进展和作者多年的经验,讨论当前我国这一疾病临床中值得注意的问题。大量研究表明,近年本病病例增多,年龄范围更广,肝型及不典型病例更多,诊断难度大,误诊误治常见,值得重视。高度警惕是防止误诊的关键,实验检查是诊断本病不可缺少的依据。K—F环、铜蓝蛋白、尿铜、肝铜、基因检查等对本病的诊断均有重要
2、意义。其中肝铜的特异性和敏感性最高,达95%;基因检查特异性100%,敏感性60%一85%。怀疑本病的患者应进行系统铜代谢检查。严格掌握诊断标准,积分/>4可诊断本病,切勿凭某项指标诊断本病。不论病情轻重,确诊后即开始治疗。危重患者不要因为药物副作用放弃治疗,暴发型应尽快进行快速驱铜治疗,治疗无效的急慢性肝衰竭患者可行肝移植。本病长期预后良好。要认识本病新的特点,更新观念,提高我国肝豆状核变性诊治的临床水平。关键词:肝豆状核变性中图分类号:R742.4文献标志码:A文章编号:1001—5256(2013)12—0
3、905—04UpdateondiagnosisandtreatmentofWilsondiseaseinChinaYANGXu.(InstituteofHepatology&LiverDiseaseResearchCenter,SecondXiangyaHospital,SouthCentralUniversity,Changsha410011,China)Abstract:Wilsondisease(WD)isarareandtreatablegeneticdiseasethatisfoundworldwide
4、.ThelatestprogressandauthorSexperienceinthisfieldaredescribed,andsomenotableclinicalissuesconcerningthisdiseaseinChinaarediscussed.Manystudieshaveshownthatinrecentyears,thenumberandagerangeofWDcaseshaveincreased,andmoreandmoreliver—typeandatypicalcaseshavebee
5、nidenti—fied;WDisdifficulttodiagnose,andmisdiagnosedcasesarecommonlyseen.Heightenedalertnessisthekeytothepreventionofmisdiagno—sis,andlaboratorytestingisanindispensiblebasisfordiagnosisofthisdisease.Kayser—Fleischerring,ceruloplasmin,urinarycopper,he—paticcop
6、per,andgenetictestingareofgreatsignificanceforthediagnosisofWD.HepaticcopperhasthehighestspecificityandsensitivityforWD,reaching95%,andgenetictestinghasaspecificityof100%andasensitivityof60%一85%forWD.PatientssuspectedofWDshouldundergosystemiccoppermetabolicex
7、amination.Diagnosticcriteriashouldbestrictlyfollowed,andWDcanbediagnosedifthetotalscoreisnotlowerthan4;diagnosiscannotbebasedonasingletest.Oncethediagnosisisconfirmed,therapyisstartedregardlessofthese—verityofdisease.Severecasesarenotencouragedtodiscontinueth
8、erapyduetothesideeffectsofdrugs.Fulminatingcasesshouldreceiverapiddecoppertreatmentassoonaspossible.LivertransplantationmaybeconsideredforpatientswithacuteOrchronicliverfailureunre—sponsi
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