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Wilson病和非Wilson病肝脏铜中毒患者酒精小体的免疫组化分析
(HEPATOLOGY2004;39;963-969)Wilson病(WD)、印第安儿童肝硬化(ICC)、特发性铜中毒(ICT)患者出现重度肝损伤,其形态学特点为肝细胞气球样变、炎症、细胞骨架改变、酒精小体(MB)形成,最终大多发生小结节性肝硬化。但目前...
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肝豆状核变性
...Fleischer环),伴有血浆铜蓝蛋白缺少和氨基酸尿症。又名Wilson病。【诊断】对于儿童和青年的慢活肝、暴发性肝炎或肝硬化患者,须考虑除外本病。K-F环、血铜蓝蛋白、尿铜测定是必要的诊断步骤。肝组织活检一是观察组织变化...
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Wilson病肾纤维化水平研究进展
肝豆状核变性(hepatolenticulardegeneration,HLD病)又称Wilson病,是一种常染色体隐性遗传的铜代谢障碍性疾病。由于铜沉积于肝、脑、肾等部位,引起相应的器官脏器损伤,如肝硬化、脑损害、肾病等。Wilson病临床上多以肝病和神经精...
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Wilson病1例报告
...值0.21~0.53g/L)。据上述检查诊断为肝豆状核变性,又称Wilson病。则给予D-青霉胺(D-penicillamine),甘草锌胶囊口服,并给保肝、对症、支持等治疗。半月后好转,复查铜蓝蛋白0.02g/L,未再见K-F环。患者好转出院后,继续服用D-...
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Wilson病脑CT表现(附7例报告)
【摘要】目的探讨Wilson病脑CT诊断价值。方法回顾分析7例临床资料完整,并经实验室检查证实的Wilson病的脑CT表现。结果本组7例中患者,脑CT均有豆状核密度减低,6例伴尾状核低密度,4例双侧丘脑同时受累,5例有脑萎缩。结论W...
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肝豆状核变性并发肝硬化腹水死亡1例报告
...部CT报告:肝硬化、腹水、脾大。拟诊为肝豆状核变性(Wilson氏病),肝硬化(失代偿期)并腹水,脾大。入院后,查铜蓝蛋白0.18g/L,角膜K-F环阳性,头颅MRI扫描未见异常,确诊肝豆状核变性(Wilson氏病),肝功能衰竭。给予低...
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南疆地区53例肝豆状核变性分析
【摘要】目的研究南疆地区肝豆状核变性(Wilson病)的特点。方法对53例Wilson病患者的发病年龄、临床特点进行统计分析。结果维吾尔族Wilson病患者具有发病年龄早、肝硬化发生率高的特点。结论维吾尔族Wilson病患者具有发病年...
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原位肝移植手术并发症的预防和治疗
...脾功能亢进8例,其中有上消化道出血史5例,Caroli’s病、Wilson’s病、原发性胆汁性肝硬化、硬化性胆管炎致肝硬化各1例。男16例,女7例,年龄为3~49岁,平均年龄40.8岁。出现各类近期并发症者25例次,6例为较大量腹腔出血,4...
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TSRI揭示HIV脆弱表位
...主任、结构生物学教授、Skaggs化学生物学研究所成员IanA.Wilson说。这一研究发现是由IAVI和NIH资助的一个大型研究工作的组成部分,该项目旨在开发出一种有效的抗HIV疫苗。这种疫苗是通过对HIV病毒上的保守脆弱位点引发强有力且...
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肝豆状核变性39例早期临床表现及治疗分析
...遗传 肝豆状核变性(hepatolenticulardegeneration)又称为Wilson’s病(Wilson’sdisease,WD),是由于铜代谢异常而引起的神经系统变性疾病,本文就近10年来我们收治的被确诊为该病的病例进行回顾性分析,报告如下。 1临床资料...
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4-5参考文献
...斌译)《遗传学经典论文集》P.67,科学出版社,1987年。[2]Wilson,E.B.(1905),《昆虫染色体与性决定的关系》-G.孟德尔等著(梁宏,王斌译)《遗传学经典论文集》P.37,科学出版社,1987年。[3]McClung,C.E.1902.Theaccessorychromosome~sexdeterminan...
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1例罕见的严重脊柱前凸畸形的临床诊治
...的骨条。剩余肋骨埋于皮下。2周后行二期手术。俯卧于Wilson手术架上,后入路剥离椎旁肌,斜形切断部分竖脊肌,彻底松解关节突、横突周围软组织;因见棘间韧带挛缩,而切断T5~L5棘间韧带并取活检。然后于T5、T7、T9、T11、L...
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DirectIdentificationofMajorBloodCulturePathogens,IncludingPseudomonasaeruginosaandEscherichiacoli,byaPanelofFluorescenceInSituHybridizatio
...cher,A.Durbin,D.S.Shapiro,D.R.Schwartz,P.C.DeGirolami,J.Dakos,G.W.Procop,D.Wilson,C.S.Hanna,G.Haase,H.Peltroche-Llacsahuanga,K.C.Chapin,M.C.Musgnug,M.H.Levi,C.Shoemaker,andH.Stender.2003.DirectidentificationofStaphylococcusaureusfrompositivebloodculturebottles.J.Clin.Microbiol.41:889-891.Oliveira,K....
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DepartmentofMicrobiologyandImmunology,UniversityofMichiganMedicalSchool,AnnArbor,MI48109
...sworkwasfundedinpartsbyNationalInstitutesofHealthgrants:P20RR018758-01(P.C.Wilson)andP20RR15577-02(P.C.Wilson).ReferencesGriffiths,G.M.,C.Berek,M.Kaartinen,andC.Milstein.1984.Somaticmutationandthematurationofimmuneresponseto2-phenyloxazolone.Nature.312:271–275.Rajewsky,K.,I.Forster,andA.Cumano.198...
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ImportedCrimean-CongoHemorrhagicFever
...hagicfeverandCongovirus.Proc.Soc.Exp.Biol.Med.131:233-236.Chapman,L.E.,M.L.Wilson,D.B.Halletal.1991.RiskfactorsforCrimean-CongohemorrhagicfeverinruralnorthernSenegal.J.Infect.Dis.164:686-692.Drosten,C.,B.M.Kummerer,H.Schmitz,andS.Gunther.2003.Moleculardiagnosticsofviralhemorrhagicfevers.Antivir.Res....
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StructuralbasisforCD1dpresentationofasulfatidederivedfrommyelinanditsimplicationsforautoimmunity
...udywassupportedbyNationalInstitutesofHealthgrantsGM62116,CA58896(bothtoI.A.Wilson),andCA10066(toV.Kumar),andTheSkaggsInstituteforChemicalBiology(toI.A.Wilson,C.-H.Wong,andD.M.Zajonc).Thisismanuscriptnumber17631-MBofTheScrippsResearchInstitute.ReferencesBendelac,A.1995.MouseNK1+Tcells.Curr.Opin.Immun...
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探索新路径——利用基因组学研究证候实质
...省立儿童医院神经内科科研人员应用PCR技术分别扩增90例Wilson病(肝豆状核变性)患者和30名健康人ATP7B基因的第8外显子,其PCR产物行限制性内切酶MspⅠ酶切分析。结果发现90例Wilson病患者有34例Arg778Leu/Gln点突变,Wilson病患者中医...
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以肌肉疼痛为表现的肝豆状核变性病1例
...院。 2讨论 肝豆状核变性病是1912年英国神经学家Wilson发现,后又称Wilson病(WD),发病率约1/5000~1/30000,是常染色体隐性遗传病,基因定位于13号染色体q14.3。致病因子造成铜蓝蛋白的合成障碍,并影响铜在胆道中的排泄...
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肝干细胞与干细胞移植治疗急慢性肝病的研究进展
...将肝内可能具有分化潜力的细胞描述为卵圆细胞,1958年Wilson和Leduc[2]提出肝干细胞的概念。近年来,人们发现肝干细胞移植对急慢性肝脏疾病有明确的治疗作用,使之成为消化领域的研究热点。本文对肝干细胞及干细胞移植...
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肝豆状核变性1例报告
肝豆状核变性(hepatolenticulardegeneration,HLD),又称Wilson病(Wilson’sdisease,WD),是一种常染色体隐性遗传的铜代谢缺陷病,好发于儿童和青少年,临床特点为肝硬化、大脑基底节软化和变性以及角膜色素环(Kayser-Fleischer环),伴...
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MulticenterEvaluationofaCandidaalbicansPeptideNucleicAcidFluorescentInSituHybridizationProbeforCharacterizationofYeastIsolatesfromBloodCu
...cher,A.Durbin,D.S.Shapiro,D.R.Schwartz,P.C.DeGirolami,J.Dakos,G.W.Procop,D.Wilson,C.S.Hanna,G.Haase,H.Peltroche-Llacsahuanga,K.C.Chapin,M.C.Musgnug,M.H.Levi,C.Shoemaker,andH.Stender.2003.DirectidentificationofStaphylococcusaureusfrompositivebloodculturebottles.J.Clin.Microbiol.41:889-891.Oliveira,K....
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ComparisonofMolecularTestsforDetectionandQuantificationofCell-AssociatedCytomegalovirusDNA
...metrika64:123-128.Razonable,R.R.,R.A.Brown,M.J.Espy,A.Rivero,W.Kremers,J.A.Wilson,C.M.Groettum,T.F.Smith,andC.V.Paya.2001.Comparativequantitationofcytomegalovirus(CMV)DNAinsolidorgantransplantrecipientswithCMVinfectionbyusingtwohigh-throughputautomatedsystems.J.Clin.Microbiol.39:4472-4476.Razonable,...
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StructureandbindingkineticsofthreedifferenthumanCD1d–-galactosylceramide–specificTcellreceptors
...icityofTcellvaccines.J.Exp.Med.201:1243–1255.Garcia,K.C.,L.Teyton,andI.A.Wilson.1999.StructuralbasisofTcellrecognition.Annu.Rev.Immunol.17:369–397.Al-Lazikani,B.,A.M.Lesk,andC.Chothia.2000.CanonicalstructuresforthehypervariableregionsofTcellreceptors.J.Mol.Biol.295:979–995.Rudolph,M.G.,andI.A....
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StructuralandkineticbasisforheightenedimmunogenicityofTcellvaccines
...ticmelanoma.Nat.Med.4:321–327.Apostolopoulos,V.,M.Yu,I.F.McKenzie,andI.A.Wilson.2000.Structuralimplicationsforthedesignofmolecularvaccines.Curr.Opin.Mol.Ther.2:29–36.Apostolopoulos,V.,M.Yu,A.Corper,L.Teyton,G.Pietersz,I.McKenzie,I.Wilson,andM.Plebanski.2002.Crystalstructureofanon-canonicallow-af...
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MolecularPhylogeneticsoftheGenusTrichosporonInferredfromMitochondrialCytochromebGeneSequences
...udy,4thed.Elsevier,Amsterdam,TheNetherlands.Brown,W.M.,M.George,Jr.,andA.C.Wilson.1979.RapidevolutionofanimalmitochondrialDNA.Proc.Natl.Acad.Sci.USA76:1967-1971.Chikuni,K.,Y.Mori,T.Tabata,M.Saito,M.Monma,andM.Kosugiyama.1995.Molecularphylogenybasedonthekappa-caseinandcytochromebsequencesinthemammali...
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ComparativeRecoveryofMicroorganismsfromBacT/ALERTPlasticandGlassFAandFNBloodCultureBottles
...fromchildren.J.Clin.Microbiol.43:445-447.Weinstein,M.,S.Mirrett,L.Reimer,M.Wilson,S.Smith-Elekes,C.Chuard,K.Joho,andL.Reller.1995.ControlledevaluationofBacT/AlertstandardaerobicandFANaerobicbloodculturebottlesfordetectionofbacteremiaandfungemia.J.Clin.Microbiol.33:978-981.Wilson,M.,M.Weinstein,S.Mir...
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74例肝豆状核变性患者中ATP7B基因七种新突变的发现
...6Vol.39No.10P.673-67710(北京)为了分析中国人肝豆状核变性(Wilsondisease,WD)患者ATP7B基因突变的分布特征,建立利用变性高效液相色谱(DHPLC)技术对Wilson病进行基因诊断的方法,并评价其在临床的应用价值。研究者对临床确诊为Wilson...
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Contrastingmodelsofpromiscuousgeneexpressionbythymicepithelium
...sease.Immunol.Rev.204:156–164.Liston,A.,D.H.Gray,S.Lesage,A.L.Fletcher,J.Wilson,K.E.Webster,H.S.Scott,R.L.Boyd,L.Peltonen,andC.C.Goodnow.2004.Genedosage–limitingroleofAireinthymicexpression,clonaldeletion,andorgan-specificautoimmunity.J.Exp.Med.200:1015–1026.Kuroda,N.,T.Mitani,N.Takeda,N.Ishim...
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RapidIdentificationofStaphylococcusaureusinBloodCulturesbyaCombinationofFluorescenceInSituHybridizationUsingPeptideNucleicAcidProbesandF
...cher,A.Durbin,D.S.Shapiro,D.R.Schwartz,P.C.DeGirolami,J.Dakos,G.W.Procop,D.Wilson,C.S.Hanna,G.Haase,H.Peltroche-Llacsahuanga,K.C.Chapin,M.C.Musgnug,M.H.Levi,C.Shoemaker,andH.Stender.2003.DirectidentificationofStaphylococcusaureusfrompositivebloodculturebottles.J.Clin.Microbiol.41:889-891.Oliveira,K....
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PolyphasicCharacterizationRevealsthattheHumanPathogenMycobacteriumperegrinumTypeIIBelongstotheBovinePathogenSpeciesMycobacteriumsenegalense
...nment.J.Clin.Microbiol.18:816-824.Brown,B.A.,B.Springer,V.A.Steingrube,R.W.Wilson,G.E.Pfyffer,M.J.Garcia,M.C.Menendez,B.Rodriguez-Salgado,K.C.Jost,S.H.Chiu,G.O.Onyi,E.C.Bttger,andR.J.Wallace,Jr.1999.Mycobacteriumwolinskyisp.nov.andMycobacteriumgoodiisp.nov.,twonewrapidlygrowingspeciesrelatedtoMycoba...