A 33 year old man has progressive dyspnea and decreased exercise tolerance. He has never smoked. Physical examination shows hyperresonance to percussion and decreased breath sounds. His father died of cirrhosis in his forties. Imaging shows basilar predominant emphysematous change. Which of the following is the most likely underlying mechanism?
- A)Inhaled silica causing activation of alveolar macrophages
- B)Autoimmune destruction of alveolar basement membrane collagen
- C)Defect in chloride transport causing mucus plugging
- D)Bronchiolar smooth muscle hypertrophy due to atopy
- E)Loss of antiprotease activity leading to uninhibited elastase in the lungGABARITO
Explicação
Loss of antiprotease activity leading to uninhibited elastase in the lung is correct. Alpha 1 antitrypsin deficiency predisposes to panacinar emphysema at a young age, especially in the lower lobes, and also causes liver disease because the abnormal protein ac... Ver explicação completa e trilha adaptativa →