Nonetheless, we strongly believe that alpha 1-antitrypsin accumulation diagnosed histologically with PAS/D-PAS and confirmed by immunohistochemical staining for alpha 1 anti-trypsin should prompt the treating clinician to perform a work-up for alpha 1-antitrypsin deficiency. The gene discussed is PSMA6; the disease is hyperinsulinemic hypoglycemia, familial, 4.