α1-抗胰蛋白酶缺乏症——一篇文献综述及一例慢性阻塞性气道疾病合并肝硬化患者的病例报告。

Alpha1-antitrypsin deficiency--a literature review and a case report of a patient with chronic obstructive airways disease and cirrhosis.

作者信息

Coughlin G P, van Deth A G, Ey G T, Grant A K

出版信息

Aust N Z J Med. 1977 Aug;7(4):400-3. doi: 10.1111/j.1445-5994.1977.tb04404.x.

DOI:10.1111/j.1445-5994.1977.tb04404.x

Abstract

A patient who manifested both pulmonary and hepatic disease associated with alpha1-antitrypsin deficiency is described. The biological function of alpha1-antitrypsin is discussed, as well as the inheritance of deficiency states and the spectrum of disorders which may ensue. Severe deficiency of alpha1-antitrypsin has been linked with pulmonary emphysema in adult life, progressive liver disease in childhood, adult cirrhosis but rarely with both pulmonary and liver disease.

摘要

本文描述了一名同时患有与α1-抗胰蛋白酶缺乏相关的肺部和肝脏疾病的患者。文中讨论了α1-抗胰蛋白酶的生物学功能，以及缺乏状态的遗传情况和可能随之出现的一系列疾病。α1-抗胰蛋白酶的严重缺乏与成年期的肺气肿、儿童期的进行性肝病、成年期肝硬化有关，但很少同时与肺部和肝脏疾病相关。