Certified Pediatric Nurse Exam Practice Questions Related

Review Certified Pediatric Nurse Exam Practice Questions related questions and content

What is the pathophysiologic mechanism of cystic fibrosis leading to obstructive lung disease?

  • A. Fibrosis of mucous glands and destruction of bronchial walls
  • B. Destruction of lung parenchyma from inflammation and scarring
  • C. Production of secretions low in sodium chloride and therefore thickened mucus
  • D. Increased serum levels of pancreatic enzymes that are deposited in the bronchial mucosa
Correct Answer: C

Rationale: Cystic fibrosis (CF) is caused by mutations in the CFTR gene, leading to altered transport of sodium and chloride ions in and out of epithelial cells. This results in the production of secretions that are low in sodium chloride, leading to thickened mucus. The abnormally thick, abundant secretions from mucous glands in the respiratory tract can obstruct the airways, leading to chronic, diffuse obstructive pulmonary disease in almost all patients with CF. This process impairs mucociliary clearance, predisposing individuals to recurrent lung infections, inflammation, and ultimately bronchiectasis. This mechanism is a key factor in the pathophysiology of cystic fibrosis-associated obstructive lung disease.