Hereditary pancreatitis (HP) is a rare autosomal dominant genetic disorder of the pancreas with incomplete penetrance. HP typically presents with acute pancreatitis in early adolescence with a high rate of progression to chronic pancreatitis (CP) by early adulthood. Genetically, HP is generally caused by gain-of-function mutations in the cationic trypsinogen gene (PRSS1), although rare kindreds have been identified with other known or unknown etiologies. While HP is similar to RAP and CP of other etiologies, the onset of HP is dramatically earlier. A lifetime of pancreatic injury and inflammation increases the risk for the most adverse late complication of HP -pancreatic cancer. Furthermore, the natural history of HP cases varies substantially between individuals and families due to gene-gene and geneenvironment interactions. Patients with HP should be closely evaluated for pancreatic endocrine and exocrine insufficiency, counseled on lifestyle changes that may reduce severity and progression, and may be evaluated for total pancreatectomy with islet autotransplantation (TP-IAT) when chronic pain does not respond to therapeutic interventions.
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