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威廉姆斯-坎贝尔综合征的临床表现、诊断与治疗

The clinical manifestations, diagnosis and management of williams-campbell syndrome.

作者信息

Noriega Aldave Adrian Pedro, William Saliski DO

机构信息

Department of Internal Medicine, University of Alabama at Birmingham Health Center Montgomery, Montgomery, Alabama, USA.

Pulmonary-Critical Care Medicine, Baptisti South Medical Center Hospital, Pulmonary Montgomery Consultants, Montgomery, Alabama, USA.

出版信息

N Am J Med Sci. 2014 Sep;6(9):429-32. doi: 10.4103/1947-2714.141620.

DOI:10.4103/1947-2714.141620
PMID:25317385
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4193147/
Abstract

Williams-Campbell syndrome is a rare congenital syndrome characterized by the absence of cartilage in subsegmental bronchi leading to formation of bronchiectasis distal to the affected bronchi. The differential diagnosis of bronchiectasis is broad and the rarity of the disease poses a diagnostic and management challenge for clinicians. This present review aims to help the understanding of the clinical manifestations, pathophysiological features, diagnostic modalities, management and differential diagnosis of Williams-Campbell syndrome. A MedLine/PubMed search was performed identifying all relevant articles. No restrictions were used for publication dates. The author used the keywords "Williams-Campbell syndrome," "non-cystic fibrosis bronchiectasis" and "congenital bronchiectasis" finding 503, 195 and 489 articles, respectively.

摘要

威廉姆斯-坎贝尔综合征是一种罕见的先天性综合征,其特征是亚段支气管中缺乏软骨,导致受累支气管远端形成支气管扩张。支气管扩张的鉴别诊断范围广泛,且该疾病的罕见性给临床医生带来了诊断和管理方面的挑战。本综述旨在帮助理解威廉姆斯-坎贝尔综合征的临床表现、病理生理特征、诊断方法、管理及鉴别诊断。进行了MedLine/PubMed检索以识别所有相关文章。对发表日期未作限制。作者使用关键词“威廉姆斯-坎贝尔综合征”、“非囊性纤维化支气管扩张”和“先天性支气管扩张”,分别找到503篇、195篇和489篇文章。

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The clinical manifestations, diagnosis and management of williams-campbell syndrome.威廉姆斯-坎贝尔综合征的临床表现、诊断与治疗
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本文引用的文献

1
Williams-Campbell syndrome presenting in an adult.成人期出现的威廉姆斯-坎贝尔综合征。
BMJ Case Rep. 2012 Sep 18;2012:bcr2012006775. doi: 10.1136/bcr-2012-006775.
2
Lung transplantation for Williams-Campbell syndrome with a probable familial association.肺移植治疗威廉姆斯-坎贝尔综合征合并可能的家族相关性。
Respir Care. 2012 Sep;57(9):1505-8. doi: 10.4187/respcare.01484. Epub 2012 Feb 17.
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Williams-Campbell syndrome: a case report.威廉姆斯-坎贝尔综合征:一例报告。
Int J Gen Med. 2012;5:41-4. doi: 10.2147/IJGM.S28447. Epub 2012 Jan 11.
4
Bronchiectasis in a diverse US population: effects of ethnicity on etiology and sputum culture.美国不同人群中的支气管扩张症:人种对病因和痰培养的影响。
Chest. 2012 Jul;142(1):159-167. doi: 10.1378/chest.11-1024.
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Bronchiectasis.支气管扩张症
BMJ Clin Evid. 2011 Aug 16;2011:1507.
6
Respiratory failure of Williams-Campbell syndrome is effectively treated by noninvasive positive pressure ventilation.无创正压通气可有效治疗威廉姆斯-坎贝尔综合征的呼吸衰竭。
Intern Med. 2011;50(16):1729-32. doi: 10.2169/internalmedicine.50.4971. Epub 2011 Aug 15.
7
Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome.肺非结核分枝杆菌病:一种独特的既往存在综合征的前瞻性研究
Am J Respir Crit Care Med. 2008 Nov 15;178(10):1066-74. doi: 10.1164/rccm.200805-686OC. Epub 2008 Aug 14.
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Aetiology in adult patients with bronchiectasis.成人支气管扩张症患者的病因
Respir Med. 2007 Jun;101(6):1163-70. doi: 10.1016/j.rmed.2006.11.008. Epub 2007 Jan 12.
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The role of spiral multidetector dynamic CT in the study of Williams-Campbell syndrome.螺旋多排动态CT在威廉姆斯-坎贝尔综合征研究中的作用
Acta Radiol. 2006 Oct;47(8):798-800. doi: 10.1080/02841850600849084.
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International guidelines for the selection of lung transplant candidates: 2006 update--a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation.国际肺移植受者选择指南:2006年更新——国际心肺移植学会肺科学委员会共识报告
J Heart Lung Transplant. 2006 Jul;25(7):745-55. doi: 10.1016/j.healun.2006.03.011.