[α-1抗胰蛋白酶缺乏症]
[Alpha-1 antitrypsin deficiency].
作者信息
Kauppi Paula, Jokelainen Kalle
机构信息
HYKS, iho- ja allergiasairaala, allergiayksikkö.
出版信息
Duodecim. 2011;127(18):1911-8.
PMID:22034728
Abstract
Alpha-1 antitrypsin deficiency is a hereditary metabolic disorder predisposing its carrier to lung and liver damage. Organ damage results from decreased secretion of alpha-1 antitrypsin from hepatocytes to circulation, caused by a genetic mutation. Decreased alpha-1 antitrypsin level predisposes to early-onset pulmonary emphysema. Unsecreted alpha-1 antitrypsin accumulating into hepatocytes may in turn lead to an inflammatory reaction, increase in fibrous tissue and finally to liver cirrhosis.
摘要
α-1抗胰蛋白酶缺乏症是一种遗传性代谢紊乱疾病，会使其携带者易患肺和肝损伤。器官损伤是由基因突变导致肝细胞向循环系统分泌α-1抗胰蛋白酶减少引起的。α-1抗胰蛋白酶水平降低易引发早发性肺气肿。未分泌的α-1抗胰蛋白酶在肝细胞中积累反过来可能导致炎症反应、纤维组织增加，最终导致肝硬化。
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