A 40 year old man presented with repeated episodes of bronchospasm and hemoptysis. Chest X-ray revealed perihilar bronchiectasis. The most likely diagnosis is :
**Core Concept**
The patient's symptoms of bronchospasm, hemoptysis, and perihilar bronchiectasis on chest X-ray suggest a chronic inflammatory airway disease with bronchiectasis, a condition characterized by irreversible dilatation of bronchial airways due to destruction of the bronchial wall.
**Why the Correct Answer is Right**
The presence of perihilar bronchiectasis and hemoptysis is highly suggestive of **Cystic Fibrosis (CF)**, an autosomal recessive genetic disorder caused by mutations in the CFTR gene, leading to abnormal chloride transport in epithelial cells. This results in thickened mucus that clogs the airways and traps bacteria, leading to chronic inflammation, bronchiectasis, and recurrent infections. The bronchospasm is likely due to airway hyperresponsiveness.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect as it does not directly relate to the patient's symptoms and radiographic findings. While cystic fibrosis can present with respiratory symptoms, the absence of a family history and other systemic symptoms makes this option less likely.
**Option B:** This option is incorrect as it does not explain the patient's hemoptysis and perihilar bronchiectasis. While chronic bronchitis can present with cough and sputum production, it does not typically cause hemoptysis or bronchiectasis.
**Option C:** This option is incorrect as it does not account for the patient's genetic predisposition and family history. While alpha-1 antitrypsin deficiency can cause chronic lung disease, it is not the most likely diagnosis in this case.
**Clinical Pearl / High-Yield Fact**
Cystic fibrosis is a classic example of a genetic disorder that can present with respiratory symptoms, but also has associated extrapulmonary symptoms such as pancreatic insufficiency, meconium ileus, and male infertility.
**Correct Answer:** B. Cystic Fibrosis