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为什么一些患有PiMZ α1-抗胰蛋白酶的成年人会发展为支气管扩张?[已修正]

Why do some adults with PiMZ α1-antitrypsin develop bronchiectasis? [corrected].

作者信息

Aggarwal Nupur, Delgado Beatriz Martinez, Salipalli Sandeep, Matamala Nerea, Rademacher Jessica, Schwerk Nicolaus, Welte Tobias, Janciauskiene Sabina, Ringshausen Felix C

机构信息

Dept of Respiratory Medicine, University Children's Hospital, Hannover Medical School, Endstage and Obstructive Lung Disease Hannover (BREATH), Member of the German Center for Lung Research (DZL), Hannover, Germany.

Molecular Genetics Unit, Instituto de Investigación de Enfermedades Raras (IIER), Instituto de Salud Carlos III (ISCIII), Madrid, Spain.

出版信息

ERJ Open Res. 2016 Jun 6;2(2). doi: 10.1183/23120541.00021-2016. eCollection 2016 Apr.

DOI:10.1183/23120541.00021-2016
PMID:27730187
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5005170/
Abstract

http://ow.ly/iJsF300kbyV.

摘要

http://ow.ly/iJsF300kbyV.(这本身就是网址,无法进行常规翻译,保留原样即可)

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81c1/5005170/b5208a611641/00021-2016.01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81c1/5005170/b5208a611641/00021-2016.01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/81c1/5005170/b5208a611641/00021-2016.01.jpg

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Why do some adults with PiMZ α1-antitrypsin develop bronchiectasis? [corrected].为什么一些患有PiMZ α1-抗胰蛋白酶的成年人会发展为支气管扩张?[已修正]
ERJ Open Res. 2016 Jun 6;2(2). doi: 10.1183/23120541.00021-2016. eCollection 2016 Apr.
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Erratum: "Why do some adults with PiMZ α-antitrypsin develop bronchiectasis?" Nupur Aggarwal, Beatriz Martinez Delgado, Sandeep Salipalli, Nerea Matamala, Jessica Rademacher, Nicolaus Schwerk, Tobias Welte, Sabina Janciauskiene and Felix C. Ringshausen. 2016; 2: 00021-2016.
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Alpha-1 antitrypsin deficiency as a common treatable mechanism in chronic respiratory disorders and for conditions different from pulmonary emphysema? A commentary on the new European Respiratory Society statement.α-1抗胰蛋白酶缺乏症作为慢性呼吸系统疾病及与肺气肿不同病症的一种常见可治疗机制?对欧洲呼吸学会新声明的评论
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本文引用的文献

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α1-Antitrypsin Combines with Plasma Fatty Acids and Induces Angiopoietin-like Protein 4 Expression.α1-抗胰蛋白酶与血浆脂肪酸结合并诱导血管生成素样蛋白4表达。
J Immunol. 2015 Oct 15;195(8):3605-16. doi: 10.4049/jimmunol.1500740. Epub 2015 Sep 11.
2
A Comprehensive Analysis of the Impact of Pseudomonas aeruginosa Colonization on Prognosis in Adult Bronchiectasis.铜绿假单胞菌定植对成人支气管扩张症预后影响的综合分析。
Ann Am Thorac Soc. 2015 Nov;12(11):1602-11. doi: 10.1513/AnnalsATS.201506-333OC.
3
Identification of a novel SERPINA-1 mutation causing alpha-1 antitrypsin deficiency in a patient with severe bronchiectasis and pulmonary embolism.
Description of a new rare alpha-1 antitrypsin mutation in Naples (Italy): PI*M S-Napoli.
意大利那不勒斯一种新的罕见α-1抗胰蛋白酶突变的描述:PI*M S-那不勒斯
Ann Thorac Med. 2018 Jan-Mar;13(1):59-61. doi: 10.4103/atm.ATM_234_17.
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A New SERPINA-1 Missense Mutation Associated with Alpha-1 Antitrypsin Deficiency and Bronchiectasis.一种与α-1抗胰蛋白酶缺乏症和支气管扩张相关的新型丝氨酸蛋白酶抑制剂A-1(SERPINA-1)错义突变。
Lung. 2017 Oct;195(5):679-682. doi: 10.1007/s00408-017-0033-2. Epub 2017 Jul 1.
在一名患有严重支气管扩张症和肺栓塞的患者中鉴定出一种导致α-1抗胰蛋白酶缺乏的新型SERPINA-1突变。
Int J Chron Obstruct Pulmon Dis. 2015 May 7;10:891-7. doi: 10.2147/COPD.S80173. eCollection 2015.
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Lancet Respir Med. 2015 May;3(5):341-3. doi: 10.1016/S2213-2600(15)00094-6.
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Occup Environ Med. 2014 Apr;71(4):234-40. doi: 10.1136/oemed-2013-101592. Epub 2013 Nov 8.
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Mol Med. 2011 Sep-Oct;17(9-10):865-74. doi: 10.2119/molmed.2011.00089. Epub 2011 Apr 11.
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British Thoracic Society guideline for non-CF bronchiectasis.英国胸科学会非 CF 支气管扩张症指南。
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