Non cystic fibrosis bronchiectasis

Abstract

Bronchiectasis (Bx), an old disease, was a popular topic of study mid last century producing a series of articles in 1956– 1963describing thecharacteristicsof affectedchildrenbefore falling into relative obscurity. However a resurgence in the literature since the late 1990s has resulted in similar descriptions demonstrating clear concern about the incidence of children affected, particularly in certain communities – Alaskan native, Aboriginal, Maori & Pacific Islanders, Turkish with rates (likely underestimated) at between 0.2–14.7/ 1,000. There have only been 2 national studies of incidence in children to date – Finland at 0.5/100,000 and New Zealand at 3.7/100,000. Diagnosis has moved from autopsy tobronchography toHRCTscanwith criteria defined as lack of normal tapering, bronchi with an internal diameter greater than the external diameter of the accompanying artery, or visualization of bronchi within 1 cm of the pleura. Concern also surrounds the severity of the disease in those diagnosed. The paediatric studies that describe the degree of lung involvement give this as 39–82% with 4 or more lobes involved, 61–93% with bilateral disease and only 9%, 29% and 43% with single lobe involvement. TB, pertussis, measles and certain types of adenovirus are traditionally implicated. In the more recent series, aetiology has been ascribed to infection 25–95%, unknown 0–50% (noting that those less willing to ascribe to infectious disease without definitive proof had higher numbers in the unknown category), aspiration including foreign body 4–10%, immunodeficiency 0–17%, cilia abnormalities 0–13%. However, recognise with these figures that not all investigations were done in all the populations. The infections above are still apparent, but more now are secondary to pneumonia with organisms unknown. A history of hospitalized pneumonia gave a relative risk of 15.2 for developing Bx, and associations were also strong with recurrent hospitalised pneumonia,