Of note, over a 6-year Fup we observed a high frequency of pneumothorax (8.7% of cases) when compared to other severe chronic bronchopneumopathies, such as cystic fibrosis or patients with alpha-1 antitrypsin deficiency, where pneumothorax occurred with a frequency of 2–3% over a 10-year period [39, 40]. The gene discussed is SERPINA1; the disease is hyperinsulinemic hypoglycemia, familial, 4.