Hereditary Pancreatitis in the United States: Survival and Rates of Pancreatic Cancer

OBJECTIVES: Hereditary pancreatitis (HP), an autosomal dominant disease typically caused by mutations inPRSS1, has a broad range of clinical characteristics and high cumulative risk of pancreatic cancer. We describe survival and pancreatic cancer risk in the largest HP cohort in the US. METHODS: HP probands and family members prospectively recruited from 1995 to 2013 completed medical and family history questionnaires, and provided blood for DNA...