门腔分流术后肝脏α-1-抗胰蛋白酶沉积的逆转
Reversal of hepatic alpha-1-antitrypsin deposition after portacaval shunt.
作者信息
Starzl T E, Porter K A, Francavilla A, Iwatsuki S
出版信息
Lancet. 1983 Aug 20;2(8347):424-6. doi: 10.1016/s0140-6736(83)90390-2.
Abstract
End-to-side portacaval shunts were carried out in three children with the liver disease of alpha-1-antitrypsin deficiency and complications of portal hypertension. Their clinical courses have been stable for 3 1/2 to almost 7 years. Postoperative liver biopsy material from two of the patients showed the typical histopathological changes caused by portal diversion, as well as an apparent reduction in the quantity of alpha-1-antitrypsin particles in the hepatocytes. The metabolic changes caused by portal diversion have apparently created a more favourable equilibrium between the synthesis and excretion of the abnormal alpha-1-antitrypsin.
摘要
对三名患有α-1抗胰蛋白酶缺乏症肝病并伴有门静脉高压并发症的儿童进行了端侧门腔分流术。他们的临床病程在3年半至近7年的时间里一直保持稳定。两名患者的术后肝脏活检材料显示了由门静脉分流引起的典型组织病理学变化，以及肝细胞中α-1抗胰蛋白酶颗粒数量的明显减少。门静脉分流引起的代谢变化显然在异常α-1抗胰蛋白酶的合成和排泄之间创造了更有利的平衡。
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