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Answers on p 692
A 26 year man, a non-addict, was referred to us as he had a right sided spontaneous pneumothorax. His past medical history included surgery done at the age of 12 years for bilateral inguinal hernia and aphakia of both eyes. On physical examination there was elasticity of skin over the abdomen. Cardiac examination revealed a systolic murmur over the apex.
Routine blood tests were within normal limits. Chest radiography revealed a right sided pneumothorax, which was treated with intercostal tube drainage after which his lung expanded completely. The underlying lungs revealed the presence of bilateral bullous change (fig 1); this was confirmed on high resolution computed tomography (HRCT), which showed extensive bilateral bullous disease, especially marked in left where bullae virtually replaced the lung parenchyma (fig 2). His α1-antitrypsin levels were 199 IU/l (normal 93–224). Spirometry showed obstructive airway disease with poor bronchodilator reversibility, and increased residual volume (residual volume/total lung capacity, 92% observed) 383% predicted. Two dimensional echocardiography showed mild to moderate pulmonary hypertension and mild prolapse of mitral valve leaflets. Arterial blood gases revealed compensated type 2 respiratory failure.
A 34 year man, an engineer and non-smoker, was referred to us to rule out drug resistant tuberculosis because of worsening of his lesions on the chest radiograph. He had symptoms of dry cough and dyspnoea for the past five years and he was treated with varying courses of antituberculosis treatment in view of the upper lobe lesions, which were progressive.
On physical examination he was a tall individual with a wide arm span and a high arched palate. There was grade 2 clubbing. On auscultation there were bilateral crepitations. On investigation baseline blood tests were within normal limits. Chest radiography revealed the presence of bilateral upper lobe fibrosis with hyperinflated lung fields (fig 3). Sputum stain and culture for acid fast bacilli were negative. HRCT demonstrated the presence of fibrosis, cavities in the upper lobes, with the presence of an aspergilloma in the right upper lobe. In addition there were bilateral bullae in the rest of the lung fields (fig 4). Two dimensional echocardiography revealed the presence of mitral regurgitation and mild pulmonary hypertension. Radiography of both hands (fig 5) was done to calculate the metacarpal index (MCI), which was 8.6 (n-MCI = 5.4 to 7.9).
There was extensive cystic bullous involvement of lungs in both the cases; what could be the possible diagnosis?
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