Suppr超能文献

移植后肝脏同种异体移植物中发现的偶发性α-1抗胰蛋白酶缺乏症:两例报告。

Incidental alpha-1-antitrypsin deficiency found in post-transplant liver allografts: Report of two cases.

作者信息

Lee Jonathan, Yoshida Eric M, Mattman Andre, Marquez Vladimir, Bukhari Hussam, Farnell David, Yang Hui-Min

机构信息

Department of Pathology and Laboratory Medicine, Vancouver General Hospital, Vancouver, BC, Canada.

Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.

出版信息

Hepatol Forum. 2021 Jan 8;2(1):31-33. doi: 10.14744/hf.2020.2020.0013. eCollection 2021 Jan.

Abstract

Alpha-1 antitrypsin deficiency is an autosomal recessive disease most commonly caused by misfolding of the Alpha-1-antitrypsin protein, which prevents its release from hepatocytes into the systemic circulation. This results in increased lifetime risk of liver and lung disease. Due to its variable penetrance, presentation and natural history, patients with alpha-1 antitrypsin deficiency are often underdiagnosed. In this report, we present two cases of alpha-1 antitrypsin deficiency in deceased-donor liver transplant allografts diagnosed post-transplant. There is currently no known adverse outcome directly linked to alpha-1 antitrypsin deficiency in the immediate post-transplant follow-up period. Thus, these allografts should not be excluded from transplantation.

摘要

α-1抗胰蛋白酶缺乏症是一种常染色体隐性疾病,最常见的病因是α-1抗胰蛋白酶蛋白错误折叠,这阻止了它从肝细胞释放到体循环中。这导致肝脏和肺部疾病的终生风险增加。由于其可变的外显率、临床表现和自然病史,α-1抗胰蛋白酶缺乏症患者常常被漏诊。在本报告中,我们介绍了两例在移植后诊断出的已故供体肝移植同种异体移植物中α-1抗胰蛋白酶缺乏症的病例。目前在移植后的随访期内,尚无已知的与α-1抗胰蛋白酶缺乏症直接相关的不良后果。因此,这些同种异体移植物不应被排除在移植之外。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/569a/9138925/b957155c4d29/hf-2-31-g001.jpg

相似文献

4
Alpha1-Antitrypsin Deficiency: A Cause of Chronic Liver Disease.α1-抗胰蛋白酶缺乏症:一种慢性肝病的病因。
Clin Liver Dis. 2020 Aug;24(3):483-492. doi: 10.1016/j.cld.2020.04.010. Epub 2020 Jun 2.
5
Lung and liver transplantation in patients with alpha-1 antitrypsin deficiency.α-1抗胰蛋白酶缺乏症患者的肺和肝移植
Ther Adv Chronic Dis. 2021 Jul 29;12_suppl:20406223211002988. doi: 10.1177/20406223211002988. eCollection 2021.
9
Lung Transplantation in Alpha-1-Antitrypsin Deficiency.α1-抗胰蛋白酶缺乏症中的肺移植
COPD. 2016;13(2):146-52. doi: 10.3109/15412555.2015.1048850. Epub 2015 Oct 21.
10
Liver Transplantation in Alpha-1 Antitrypsin Deficiency.α-1抗胰蛋白酶缺乏症的肝移植
Clin Liver Dis. 2017 May;21(2):355-365. doi: 10.1016/j.cld.2016.12.008. Epub 2017 Feb 13.

本文引用的文献

1
Liver disease in adults with severe alpha-1-antitrypsin deficiency.成人严重α-1-抗胰蛋白酶缺乏症相关肝病。
J Gastroenterol. 2019 Jun;54(6):541-548. doi: 10.1007/s00535-019-01548-y. Epub 2019 Jan 24.
3
Alpha-1-Antitrypsin Deficiency Liver Disease.α1-抗胰蛋白酶缺乏症肝病。
Clin Liver Dis. 2018 Nov;22(4):643-655. doi: 10.1016/j.cld.2018.06.010. Epub 2018 Aug 22.
4
Role of alpha-1 antitrypsin in human health and disease.α-1 抗胰蛋白酶在人类健康和疾病中的作用。
J Intern Med. 2014 Oct;276(4):311-35. doi: 10.1111/joim.12239. Epub 2014 May 13.
6
Transient elastography (FibroScan).瞬时弹性成像(FibroScan)。
Gastroenterol Clin Biol. 2008 Sep;32(6 Suppl 1):58-67. doi: 10.1016/S0399-8320(08)73994-0.
8
Acquired PiZZ alpha-1 antitrypsin deficiency in a liver transplant recipient.
Transplantation. 2004 Jun 27;77(12):1918-9. doi: 10.1097/01.tp.0000131602.35978.98.

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验