Non-cystic fibrosis bronchiectasis: its diagnosis and management

Abstract

An increase in the frequency of diagnosing non-cystic fibrosis bronchiectasis in children is due to heightened awareness of the disease and the wider availability of high-resolution computed tomography. The most common underlying conditions leading to bronchiectasis include infections, immunodeficiency, aspiration and primary ciliary dyskinesia. Treatment centres on airway clearance with aggressive antibiotic regimens and physiotherapy; more specific approaches are available for some of the underlying conditions. A high index of suspicion that a child may have underlying bronchiectasis must be maintained in the presence of prolonged or recurrent "wet/productive" cough. The classic definition of bronchiectasis is of irreversible bronchial dilatation; however, at the milder end of the spectrum it appears that radiographic changes may be reversible. Untreated, in its severest form bronchiectasis can progress to end stage pulmonary failure in adult life. In this article, we review its pathogenesis and diagnosis and the evidence base for available treatments.