Childhood Bronchiectasis around the World Gregory J. Redding M.D. Professor of Pediatrics, University of Washington School of Medicine Chief, Pulmonary and Sleep Medicine Division, Seattle Children's Hospital Seattle, Washington, USA Key words: bronchiectasis Mailing Address: Pulmonary and Sleep Medicine Division, Office A-5937 Seattle Children's Hospital 4800 Sand Point Way N.E. Seattle, Washington, USA e-mail: gredding@u.washington.edu Telephone: 206-987-2174 Fax: 206-987-2639 Bronchiectasis has been described as an orphan disease in developed countries around the world, most often associated with conditions that compromise pulmonary host defenses, such a immunodeficiencies, cystic fibrosis (CF), or disorders of ciliary motility. However, worldwide, the most common etiology for bronchiectasis is probably lung injury following acute respiratory infections. The prevalence of bronchiectasis should therefore parallel the distribution of childhood mortality and hospitalization for pneumonias in very young children worldwide.[1] The lungs response to infection varies with the etiology but both viruses and bacteria can lead to chronic airway injury. Certain infections, e.g. adenoviruses of certain serotypes, produce bronchiolitis obliterans after a single infection with subsequent bronchiectasis. Severe pneumococcal pneumonia can also produce bronchiectasis as a sequelae.[2] More often, children with bronchiectasis have had recurrent acute lung infections or they experience combinations of lung infections and conditions predisposing to lung injury or airway mucus production. Chronic productive cough is more common among children passively exposed to tobacco smoke and the frequency of bronchiectasis in some populations is coincident with crowding, a high prevalence of lower respiratory tract infections, and high prevalences of smoking in families. Chronic productive cough is the most common clinical symptom associated with bronchiectasis. In some populations, the prevalence of chronic productive cough reaches up to 20% of middle school children.[3] Chronic productive cough can occur with or without associated wheezing. Bronchiectasis is associated with chronic obstructive lung disease but focal bronchiectasis does not necessarily produce air trapping or reduced airflows. In general, the more diffuse the disease, the more likely obstructive lung disease coexists. Also, approximately 40% of children and adults with bronchiectasis will have a reversible component, responsive to short acting bronchodilator treatment. Worldwide prevalences of chronic cough and bronchiectasis in children do not exist. There is likely a similar distribution of bronchiectasis to frequency of severe LRIs in children and smoking rates among families. Several infectious epidemics also contribute to the frequency distribution of childhood bronchiectasis. Human Immunodeficiency virus (HIV) and tuberculosis prevalences vary across the world, these conditions both can result in recurrent or chronic pulmonary infections, and lead to bronchiectasis. Similarly the prevalence of indoor pollution, such as indoor biomass combustion, is likely to predispose to chronic airway mucus production and chronic productive cough long term. It is unlikely that the distribution of bronchiectasis prevalences will emerge soon as the diagnostic gold standard is a high resolution computerized tomographic (CT) scan of the lungs.[2] The reason bornchiectasis has been described so well in certain populations is because those populations, however rural, have access to CT scans nearby. Actionable items to reduce bronchiecatsis worldwide will be population based. Immunization programs both for pneumococcal and perhaps influenza infections must be in place. New polyvalent pneumococcal vaccines covering more than 7 serotypes are in clinical trials in young children.[4] Improved water access for hand washing will reduce the prevalence of acute respiratory infections, as well reduced crowding. Indoor tobacco and other smoke exposure needs to be reduced. Whether medical treatments such as antibiotics can alter the evolution of bronchiectasis after acute lung infections remains unclear. Safe feeding practices may reduce the risk of aspiration and these practices are amenable to public health initiatives. A better description of the magnitude of the problem is essential if chronic suppurative lung disease in childhood is to become a priority. References 1. Redding GJ, Byrnes CA. Chronic respiratory symptoms and desieases among indigenous children. Ped Clin N Am 2009; 56:1323-1342. 2. Chang AB, Masel JP, Boyce NC, et al. Non-CF bronchiectasis: Clinical and HRCT evaluation. Pediatr Pulmonol 2003; 35:477-483. 3. Lewis TC, Stout JW, Martinez P, et al. Prevalence of asthma and chronic respiratory symptoms among Alaska Native children. Chest 2004; 125:16651673. 4. Singleton RJ, Hennessey T, Bulkow LR, et al. Invasive pneumococcal disease caused by non-vaccine serotypes among Alaskan Native children with high levels of 7-valent pneumococcal conjugate vaccine coverage. JAMA 2007; 297:17841792.