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自从Sharp等于1969年首先报告在6个不同亲缘的家庭内,发现有10例小儿肝硬化,经实验室检查结果均伴有α_1-抗胰蛋白酶(α_1-antitrypsin简称α_1AT)缺乏以来,α_1AT缺乏与小儿肝病的密切关系已引起世界各地学者们的注意。什么是α_1AT 在人的血浆和组织中含有一定数量的各种蛋白溶解酶,这些酶在体内又受另外一些酶所对抗,后者称蛋白酶抑制系统((Protease inhibitor system简称Pi系统)。在此系统中共有6种不同的抗蛋白酶物质,其中以α_1AT的
Since Sharp was first reported in 1969 in 6 families of six different relatives, 10 cases of pediatric cirrhosis were found and the lack of alpha 1-AT was associated with a lack of alpha 1-antitrypsin (alpha 1 -AT) in laboratory tests The close relationship of pediatric liver disease has attracted the attention of scholars all over the world. What is α_1AT in human plasma and tissue contains a number of various proteolytic enzymes, these enzymes in the body and by some other enzyme confrontation, the latter known as the protease inhibitor system ((Protease inhibitor system referred to as Pi system) There are altogether 6 different antiproteolytic substances in the system, among them α_1AT