Pulmonary cysts in BHD are elliptical and lentiform-shaped, often subpleural, and distributed predominantly in the lower lung zones [6,10]. Emphysema due to alpha-1 antitrypsin deficiency and infectious causes like Pneumocystis carinii can also mimic the appearance of cystic lung diseases, and the medical team should be mindful of them too. This evidence concerns the gene SERPINA1 and hyperinsulinemic hypoglycemia, familial, 4.