Management of bronchiectasis in children without cystic fibrosis
- Khoulood Fakhoury, MD
Khoulood Fakhoury, MD
- Assistant Professor of Pediatrics
- Baylor College of Medicine
- Adaobi Kanu, MD
Adaobi Kanu, MD
- Associate Professor of Pediatrics
- Texas Tech Health Sciences Center
Bronchiectasis is a structural abnormality characterized by abnormal dilation and distortion of the bronchial tree, resulting in chronic obstructive lung disease. This condition is typically the end result of a variety of pathophysiologic processes that render the bronchial walls weakened, easily collapsible, chronically inflamed, and plugged with mucus secretions. Associated findings include atelectasis, emphysema, fibrosis, and hypertrophy of the bronchial vasculature.
In resource-rich countries, cystic fibrosis (CF) is the most common cause of bronchiectasis in children. The management of CF-related bronchiectasis is discussed in detail in separate topic reviews. (See "Cystic fibrosis: Overview of the treatment of lung disease" and "Cystic fibrosis: Antibiotic therapy for lung disease".)
Bronchiectasis can be caused by a variety of disease processes other than CF, most of which include some combination of bronchial obstruction and infection (table 1). The types of disorders that cause bronchiectasis vary among populations and age groups. Infections and acquired causes of bronchiectasis are more common in adults and in resource-limited countries, whereas congenital anomalies of the bronchi or immune system are more prominent in children and in resource-rich countries.
This topic review will describe the management and prevention of non-CF-related bronchiectasis in children. The causes, clinical manifestations, and diagnosis of non-CF-related bronchiectasis in children, and the treatment of bronchiectasis in adults are discussed elsewhere. (See "Causes of bronchiectasis in children" and "Clinical manifestations and evaluation of bronchiectasis in children" and "Treatment of bronchiectasis in adults".)
Management of bronchiectasis is aimed at treating the underlying cause (eg, removal of an airway foreign body, or treatment of aspiration or humoral immunodeficiency), improving mucociliary clearance, treating and preventing infection, and controlling inflammation.
- Kapur N, Masters IB, Morris PS, et al. Defining pulmonary exacerbation in children with non-cystic fibrosis bronchiectasis. Pediatr Pulmonol 2012; 47:68.
- Kapur N, Grimwood K, Masters IB, et al. Lower airway microbiology and cellularity in children with newly diagnosed non-CF bronchiectasis. Pediatr Pulmonol 2012; 47:300.
- Kapur N, Mackay IM, Sloots TP, et al. Respiratory viruses in exacerbations of non-cystic fibrosis bronchiectasis in children. Arch Dis Child 2014; 99:749.
- Sethi GR, Batra V. Bronchiectasis: causes and management. Indian J Pediatr 2000; 67:133.
- Hnin K, Nguyen C, Carson KV, et al. Prolonged antibiotics for non-cystic fibrosis bronchiectasis in children and adults. Cochrane Database Syst Rev 2015; :CD001392.
- Brodt AM, Stovold E, Zhang L. Inhaled antibiotics for stable non-cystic fibrosis bronchiectasis: a systematic review. Eur Respir J 2014; 44:382.
- Lee AL, Burge AT, Holland AE. Airway clearance techniques for bronchiectasis. Cochrane Database Syst Rev 2015; :CD008351.
- O'Donnell AE, Barker AF, Ilowite JS, Fick RB. Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group. Chest 1998; 113:1329.
- Redding GJ. Bronchiectasis in children. Pediatr Clin North Am 2009; 56:157.
- Bilton D, Tino G, Barker AF, et al. Inhaled mannitol for non-cystic fibrosis bronchiectasis: a randomised, controlled trial. Thorax 2014; 69:1073.
- Nicolson CH, Stirling RG, Borg BM, et al. The long term effect of inhaled hypertonic saline 6% in non-cystic fibrosis bronchiectasis. Respir Med 2012; 106:661.
- Zhuo GY, He Q, Xiang-Lian L, et al. Prolonged treatment with macrolides in adult patients with non-cystic fibrosis bronchiectasis: meta-analysis of randomized controlled trials. Pulm Pharmacol Ther 2014; 29:80.
- Fan LC, Lu HW, Wei P, et al. Effects of long-term use of macrolides in patients with non-cystic fibrosis bronchiectasis: a meta-analysis of randomized controlled trials. BMC Infect Dis 2015; 15:160.
- Valery PC, Morris PS, Byrnes CA, et al. Long-term azithromycin for Indigenous children with non-cystic-fibrosis bronchiectasis or chronic suppurative lung disease (Bronchiectasis Intervention Study): a multicentre, double-blind, randomised controlled trial. Lancet Respir Med 2013; 1:610.
- Sharma R, Florea VG, Bolger AP, et al. Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax 2001; 56:746.
- Shepherd RW, Holt TL, Thomas BJ, et al. Nutritional rehabilitation in cystic fibrosis: controlled studies of effects on nutritional growth retardation, body protein turnover, and course of pulmonary disease. J Pediatr 1986; 109:788.
- Marchand V, Baker SS, Stark TJ, Baker RD. Randomized, double-blind, placebo-controlled pilot trial of megestrol acetate in malnourished children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2000; 31:264.
- Jean-Baptiste E. Clinical assessment and management of massive hemoptysis. Crit Care Med 2000; 28:1642.
- Lewiston NJ. Bronchiectasis in childhood. Pediatr Clin North Am 1984; 31:865.
- Wilson JF, Decker AM. The surgical management of childhood bronchiectasis. A review of 96 consecutive pulmonary resections in children with nontuberculous bronchiectasis. Ann Surg 1982; 195:354.