It was first described in 1952 by Comfort and Steinberg[1] but it was not until 1996 that Whitcomb et al[2] isolated the first responsible mutation in the trypsinogen gene (PRSS1) on the long arm of chromosome seven (7q35).

For example, HP typically has an earlier age of pancreatitis onset; although malabsorption and diabetes mellitus occur at a later stage in the disease progression.

The mechanism by which these genetic mutations cause pancreatitis is not yet known; but is likely to be the result of increased autoactivation[17] or reduced deactivation[18] of trypsinogen.

Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications, such as pseudocysts, bile duct or duodenal obstruction.

(PMC1774562)[citation needed] A 2009 study which followed 189 patients found no excess mortality despite the increased risk of pancreatic cancer.