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Year : 2014  |  Volume : 6  |  Issue : 9  |  Page : 429-432

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

1 Department of Internal Medicine, University of Alabama at Birmingham Health Center Montgomery, Montgomery, Alabama, USA
2 Department of Pulmonary-Critical Care Medicine, Baptisti South Medical Center Hospital, Pulmonary Montgomery Consultants, Montgomery, Alabama, USA

Correspondence Address:
Adrian Pedro Noriega Aldave
2055, East S Blvd, Suite 200, Montgomery, Alabama-36116
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1947-2714.141620

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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.

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