Tucker L E, Rawson P G
Division of Gastroenterology, St. John's Mercy Hospital, Washington, Missouri 63090.
Mo Med. 1993 Jan;90(1):27-30.
Alpha-1-antitrypsin is a glycoprotein which inactivates proteolytic enzymes, especially neutrophil elastase. Infants deficient in this enzyme commonly develop neonatal hepatitis. In adults, the deficiency typically results in emphysema. Only rarely will an adult manifest liver disease. We present a case of adult liver cirrhosis due to Alpha-1-antitrypsin deficiency in a 63-year-old man. Manifestations of alpha-1-antitrypsin deficiency and liver disease are discussed.
α-1抗胰蛋白酶是一种糖蛋白,可使蛋白水解酶失活,尤其是中性粒细胞弹性蛋白酶。缺乏这种酶的婴儿通常会患新生儿肝炎。在成年人中,这种缺乏通常会导致肺气肿。成年人很少会出现肝脏疾病。我们报告一例63岁男性因α-1抗胰蛋白酶缺乏导致的成人肝硬化病例。本文讨论了α-1抗胰蛋白酶缺乏和肝脏疾病的表现。