ELANE and hyperinsulinemic hypoglycemia, familial, 4: α1-Antitrypsin deficiency is mostly known to result in an early onset of chronic obstructive pulmonary disease (COPD), due to the lack of inhibition of neutrophil elastase, and liver cirrhosis due to polymerization of mutant α1-antitrypsin in the endoplasmic reticulum, resulting in hepatocyte damage [1–3].