Sharp H L
Semin Liver Dis. 1982 Nov;2(4):314-28. doi: 10.1055/s-2008-1040718.
alpha 1AT should no longer be ignored by hepatologists with regard to its role in protecting the liver from injury. This protective role needs further definition with regard to protease inhibition, immunologic control, and functional inactivation. Severe deficiency of alpha 1AT clearly predisposes to definable types of liver disease not only in infants and children, but at the other end of the spectrum in elderly adults. It is unfortunate that the pathophysiology of human and animal alpha 1AT related liver injury is not as readily apparent as in pulmonology conditions. However, it is time to digest the tremendous progress that has evolved in that field in relating alpha 1AT to emphysema. Cirrhosis of the liver should be studied in relationship to the pathophysiology demonstrated by our colleagues to be important in "cirrhosis of the lung." The diagnosis of alpha 1AT deficiency and the course of the associated liver disease have been reviewed and updated. Prevention and cure have been demonstrated to be possible but neither method has universal applicability. In contrast, other potential therapeutic endeavors, although not presently proved to be efficacious, with time and research will provide methodologies for the treatment of this disorder as well as insight into mechanisms of general liver injury not previously appreciated.
就α1抗胰蛋白酶在保护肝脏免受损伤方面的作用而言，肝病学家不应再忽视它。关于蛋白酶抑制、免疫控制和功能失活，这种保护作用需要进一步明确。α1抗胰蛋白酶严重缺乏不仅在婴幼儿和儿童中，而且在老年人这一极端情况下，显然易引发特定类型的肝病。遗憾的是，与α1抗胰蛋白酶相关的人类和动物肝脏损伤的病理生理学不像在肺病情况中那样容易显现。然而，现在是时候消化该领域在将α1抗胰蛋白酶与肺气肿联系起来方面取得的巨大进展了。应结合我们的同事所证明的在“肺纤维化”中很重要的病理生理学来研究肝纤维化。α1抗胰蛋白酶缺乏症的诊断及相关肝病的病程已得到回顾和更新。已证明预防和治愈是可能的，但这两种方法都没有普遍适用性。相比之下，其他潜在的治疗努力虽然目前尚未证明有效，但随着时间的推移和研究，将为治疗这种疾病提供方法，并深入了解以前未认识到的一般肝损伤机制。
