Open AccessThe clinical manifestations, diagnosis and management of williams-campbell syndrome
Author(s) -
Adrian Pedro Noriega Aldave,
DO William Saliski
Publication year - 2014
Publication title -
north american journal of medical sciences
Language(s) - English
Resource type - Journals
eISSN - 2250-1541
pISSN - 1947-2714
DOI - 10.4103/1947-2714.141620
Subject(s) - bronchiectasis , medicine , differential diagnosis , modalities , disease , intensive care medicine , pathology , pediatrics , lung , social science , sociology
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.