Chapter 393 Bronchiectasis
Bronchiectasis, a disease characterized by irreversible abnormal dilatation and anatomic distortion of the bronchial tree, likely represents a common end stage of a number of nonspecific and unrelated antecedent events. Its incidence has been decreasing overall in developed countries, but it persists as a problem in developing countries and among some ethnic groups in industrialized nations. In at least 1 series of children with bronchiectasis (not due to cystic fibrosis), the male : female ratio was 2 : 1.
Pathophysiology and Pathogenesis
In the developed world, cystic fibrosis (Chapter 395) is the most common cause of clinically significant bronchiectasis. Other conditions associated with bronchiectasis include primary ciliary dyskinesia, foreign body aspiration, aspiration of gastric contents, immune deficiency syndromes (especially humoral immunity), and infection, especially pertussis, measles, and tuberculosis. Bronchiectasis can also be congenital, as in Williams-Campbell syndrome, in which there is an absence of annular bronchial cartilage, and Marnier-Kuhn syndrome (congenital tracheobronchomegaly), in which there is a connective tissue disorder. Other disease entities associated with bronchiectasis are right middle lobe syndrome (chronic extrinsic compression of right middle lobe bronchus by hilar lymph nodes) and yellow nail syndrome (pleural effusion, lymphedema, discolored nails).
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