Bronchiectasis in children: orphan disease or persistent problem?

Abstract

More than a decade ago, bronchiectasis unrelated to cystic fibrosis was termed an "orphan disease", because it had become an uncommon clinical entity among children in the developed world. Bronchiectasis is more common among children in lower socioeconomic classes and in developing countries, presumably due to more frequent and recurrent respiratory infections, environmental airway irritants, poor immunization rates, and malnutrition. Reports from the Southern Pacific and from Alaska Native children reveal persistently high rates of childhood bronchiectasis. Better epidemiologic data throughout the world are needed to reassess the importance of this condition. The pathophysiology includes airway inflammation, mucus production, and regional airway obstruction, yet the reasons why some children develop bronchiectasis while other do not is unclear. The coexistence of asthma with bronchiectasis is associated with more severe disease, yet the impact of asthma therapy in children with both disorders has not been studied. Similarly, the pattern of antibiotic use for children with bronchiectasis varies by region with little data to justify one particular approach. It may be that public health measures aimed at improving living conditions for children and prevention of respiratory infections with antiviral vaccines will have more impact on childhood bronchiectasis than medical treatments in the future.