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α-1-抗胰蛋白酶缺乏症:门脉周围的红色玻璃样小体


过碘酸-雪夫(PAS)染色时,门脉周围的红色玻璃样小体是α-1-抗胰蛋白酶缺乏症(pi-ZZ纯合子基因型患者)的特征表现。肝细胞未排泌的α-1-抗胰蛋白酶在肝内聚集,形成小球。它最终可导致大结节性肝硬化。这些患者还可能发生全小叶性肺气肿。
The periportal red hyaline globules seen here with periodic acid-Schiff (PAS) stain are characteristic for alpha-1-antitrypsin deficiency (a person with homozygous pi-ZZ genotype). The globules are intrahepatic collections of alpha-1-antitrypsin that is not being excreted from the hepatocytes. This may eventually lead to a macronodular cirrhosis. These patients are also prone to develop panlobular emphysema of lungs.

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