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REVIEW
Bronchiectasis in congenital diseases: Pathogenesis, imaging, diagnostic approach
Konstantinos Pappas 1
,
 
Alexandra Pentheroudaki 1
,
 
Emmanuel Ferdoutsis 1
,
 
Georgios Meletis 1
,
 
Maria Kokkinaki 2
,
 
Georgios Patsourakis 1
,
 
Nikolaos Bachlitzanakis 1
 
 
 
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1
Pneumonology Department, “Venizeleio-Pananeio” General Hospital, Herakleion, Crete
 
2
Department of Computed Tomography, “Venizeleio-Pananeio” General Hospital, Herakleion, Crete
 
 
Corresponding author
Konstantinos Pappas   

Pneumonology Department, “Venizeleio-Pananeio” General Hospital, Herakleion, Crete Knossos Avenue, POB 44, GR-71300, Herakleion, Crete
 
 
Pneumon 2011;24(3):248-262
 
Article (PDF)
References (110)
 
KEYWORDS
congenital bronchiectasis
cystic fibrosis
- primary ciliary dyskinesia-Kartagener’s syndrome
Young’s syndrome
 primary immunodeficiency
alpha-1-antitrypsin deficiency
Williams-Campbell syndrome
Mounier-Kuhn syndrome
bronchial atresia
intralobar sequestration
yellow nail syndrome
 
ABSTRACT
Bronchiectasis is defined as the abnormal, irreversible dilatation of the cartilage containing airways in the context of repeated cycles of airway infection and inflammation. Many pulmonary syndromes and systemic diseases have been described that have bronchiectasis as an integral feature. Contrary to earlier perceptions that it is an “orphan disease”, bronchiectasis appears to be underdiagnosed, especially in childhood, and new disease associations continue to be described. With the advent of high resolution computerized tomography (HRCT) scanning, bronchiectasis has been demonstrated as an important cause of respiratory disease that may predispose to premature mortality. This long-neglected illness should receive more research attention in order to provide better understanding of its aetiology, pathogenesis and multimodality treatment.
 
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