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α1-抗胰蛋白酶缺乏的单卵双生杂合子婴儿中的维生素E与肝损伤

Vitamin E and liver damage in MZ heterozygous infants with alpha 1-antitrypsin deficiency.

作者信息

Pittschieler K

机构信息

Department of Pediatrics, Regional Hospital, Bolzano/Bozen, Italy.

出版信息

Acta Paediatr. 1993 Mar;82(3):228-32. doi: 10.1111/j.1651-2227.1993.tb12647.x.

Abstract

Low levels of alpha 1-antitrypsin can predispose affected infants to develop a wide spectrum of liver diseases. Heterozygous PiMZ carriers can be affected by a subclinical liver involvement in the first six months of life. The liver damage appears to be mediated by the activity of toxic oxygen waste products originating from partially unchecked proteases which can cause enough damage to impair hepatic function significantly. In the present study it was found that the antioxidant properties of vitamin E were able to reduce the frequency of liver dysfunction in PiMZ carriers at two but not at five months of age. Liver damage is highly related to low levels of alpha-tocopherol in the plasma. These findings show that oxidative free radicals can promote liver damage in inadequately protected young infants, such as those affected by alpha 1-antitrypsin deficiency. The protective role of vitamin E in relation to the developmental expression of other antioxidant scavengers is discussed.

摘要

低水平的α1-抗胰蛋白酶会使受影响的婴儿易患多种肝脏疾病。杂合子PiMZ携带者在生命的前六个月可能会受到亚临床肝脏受累的影响。肝脏损伤似乎是由部分未受控制的蛋白酶产生的有毒氧废物的活性介导的,这些蛋白酶会造成足够的损伤,严重损害肝功能。在本研究中发现,维生素E的抗氧化特性能够降低PiMZ携带者在两个月大而非五个月大时肝功能障碍的发生率。肝脏损伤与血浆中α-生育酚水平低高度相关。这些发现表明,氧化自由基可促进未得到充分保护的幼儿(如受α1-抗胰蛋白酶缺乏影响的幼儿)的肝脏损伤。本文还讨论了维生素E相对于其他抗氧化清除剂发育表达的保护作用。

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