Anatomic Pathology: Pulmonary Pathology

121) Which of the following statements is TRUE regarding the disease process in these explanted lungs?

• Bronchiectasis is a description of proximal/bronchial dilatation, which is the result of injury to the bronchial wall. This injury prevents proper tone in the airway wall and, therefore, chronic dilatation. Mucostasis results in repeated infections that damage the airway wall further. Abnormalities in mucous and mucociliary elevator function are common causes of the initial mucostasis leading to airway damage.

• Causes of bronchiectasis include infections, foreign body aspiration, cystic fibrosis, and immotile cilia syndromes. It can be congenital or acquired.

• Cystic fibrosis is a genetic disease caused by mutations in thecystic fibrosis transmembrane conductance receptor gene. It is autosomal recessive. Patients develop pulmonary disease with bronchiectasis. Aggressive management of respiratory secretions and prevention of infection are central in prolonging lung function.

• Treatment for bronchiectasis is limited. In some instances, bronchiectasis confined to one lobe may require surgical intervention.

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