α1-抗胰蛋白酶的缺乏与治疗.docx

1、综述，al-抗胰蛋白酶的缺乏与治疗郭玉婷综述；蒋琳审校兰州生物制品研究所有限责任公司甘谱省疫苗工程技术研究中心，甘肃兰州730046摘要:al-抗胰蛋白酶缺乏症(al-antitrypsindeficiency,AATD)是遗传性疾病，可引起肝脏疾病，也与早发型肺气.肿等肺部疾病有关。编码抗胰蛋白酶(al-antitrypsin,AAT)的基因具有复杂的单核甘酸多态性，并以常染色体共显性方式遗传。临床上，以混合人血浆为来源的静注AAT可提高AATI)患者的血清AAT水平。除这种增补疗法外,新的治疗方法也在不断发展，包括基因治疗及干细胞治疗等。关11词:al我胰蛋白酶缺乏症;al-抗胰蛋白酶;肺

2、气肿;补充剂中图分类号：Q55文献标志码：A文章编号：1005-5673(2015)03-0072-04DOI：10.13309/ki.pmi.2015.03.016Deficiencyandtherapeuticapproachesofal-antitrypsinGUOYu-ting,JIANGLinLanzhouInstituteofBiologicalProductsCo.,Ltd.,CenterforGansuProvincialVaccineEngineeringResearch,Lanzhou,730046,GtmsuProvince,ChinaCorrespondingautho

3、r:JIANGLin,E-mail:ljiangl&Abstract:Alpha1-antitrypsindeficiency(AATD)isahereditarydisease,whichrelatestohepaticdiseasesandsomepulmonarydiseaseslikeearly-onsetemphysema.Geneof-antitrypsin(AAT)hascomplexsinglenucleotidepolymorphisms,andinheritancemaKDA,EvansDI.,FinchJT.efal.ThemechanismofZal-antitryps

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