July - September 2011: 
Volume 24, Issue 3

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Pneumon 2011, 24(3):248-262
Bronchiectasis in congenital diseases. Pathogenesis, imaging, diagnostic approach

SUMMARY. 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. Pneumon 2011, 24(3):248-262.