Which treatments are indicated for cystic fibrosis?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene, leading to dysfunctional chloride channels in epithelial cells. This results in thick, sticky mucus that can obstruct airways and pancreatic ducts, making patients susceptible to respiratory infections and nutritional deficiencies.

The correct answer includes N-acetylcysteine, which is a mucolytic agent that helps to break down mucus, making it easier to clear from the lungs. This is particularly important for cystic fibrosis patients who struggle with mucus accumulation. Additionally, antipseudomonal prophylaxis is crucial in CF management as patients are at high risk for infections caused by Pseudomonas aeruginosa. Prophylactic antibiotics, such as inhaled tobramycin, help prevent or mitigate these chronic infections, slowing disease progression and improving lung function.

In contrast, other treatment options listed do not adequately address the unique challenges posed by cystic fibrosis. Beta agonists may provide bronchodilation but do not directly address mucus clearance or infection control. Glucocorticoids may reduce inflammation but do not manage the underlying mucus production problem. Nasogastric feeding may be necessary for nutritional support but does not treat the disease itself. Gene therapy, while a

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