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 developed nations, 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 developing nations, whereas congenital anomalies of the bronchi or immune system are more prominent in children and in industrialized nations.
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.
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