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.
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.
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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