Recently, several cohorts showed the natural history of patients with serine protease inhibitor gene (Kazal type 1: SPINK1) germline-related pancreatitis and HP caused by PRSS1 gene mutation, indicating a high progression rate of pancreatic exocrine insufficiency and diabetes mellitus, as well as a significantly increased risk of pancreatic cancer [3,7]. Here, SPINK1 is linked to exocrine pancreatic insufficiency.