Resendes M
West J Med. 1987 Jul;147(1):48-53.
alpha(1)-Protease inhibitor (alpha(1)-Pi) deficiency is associated with emphysema, neonatal hepatitis and cirrhosis. The deficiency associated with emphysema has multiple alleles. Cigarette smoke may influence the onset of emphysema in a twofold manner: by overwhelming the concentration of alpha(1)-Pi by increasing elastase release, and by inactivating the alpha(1)-Pi active site through oxidation. alpha(1)-Pi-associated hepatic disease occurs primarily in children with the allele PiZZ, most of whom are asymptomatic although in a small percentage severe obstructive jaundice and fatal junvenile cirrhosis develop. Pharmacologic intervention and alpha(1)-Pi replacement therapy are being tested against alpha(1)-Pi-associated emphysema.
α1-抗胰蛋白酶（α1-Pi）缺乏与肺气肿、新生儿肝炎和肝硬化有关。与肺气肿相关的缺乏有多个等位基因。香烟烟雾可能以两种方式影响肺气肿的发病：通过增加弹性蛋白酶释放来超过α1-Pi的浓度，以及通过氧化使α1-Pi活性位点失活。α1-Pi相关的肝脏疾病主要发生在携带PiZZ等位基因的儿童中，尽管有一小部分会发展为严重的阻塞性黄疸和致命的青少年肝硬化，但大多数人没有症状。针对α1-Pi相关的肺气肿正在测试药物干预和α1-Pi替代疗法。
