Dataset Information

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

ABSTRACT: OBJECTIVES:Hereditary pancreatitis (HP), an autosomal dominant disease typically caused by mutations in PRSS1, 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 testing. Overall survival (until 12/31/2015) was determined from the Social Security Death Index (SSDI), National Death Index (NDI), and family members. Cause of death was obtained from the NDI. RESULTS:217 PRSS1 carriers (181 symptomatic) formed the study cohort. The most frequently detected mutations were p.R122H (83.9%) and p.N29I (11.5%). Thirty-seven PRSS1 carriers (30 symptomatic, 7 asymptomatic) were deceased at conclusion of the study (5 from pancreatic cancer). Median overall survival was 79.3 years (IQR 72.2-85.2). Risk of pancreatic cancer was significantly greater than age- and sex- matched SEER data (SIR 59, 95% CI 19-138), and cumulative risk was 7.2% (95% CI 0-15.4) at 70 years. DISCUSSION:We confirm prior observations on survival and pancreatic cancer SIR in PRSS1 subjects. Although risk of pancreatic cancer was significantly high in these patients, its cumulative risk was much lower than previous reports.

SUBMITTER: Shelton CA

PROVIDER: S-EPMC6708435 | biostudies-literature | 2018 Sep

REPOSITORIES: biostudies-literature

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

Shelton Celeste A CA Umapathy Chandraprakash C Stello Kimberly K Yadav Dhiraj D Whitcomb David C DC

The American journal of gastroenterology 20180718 9

<h4>Objectives</h4>Hereditary pancreatitis (HP), an autosomal dominant disease typically caused by mutations in PRSS1, 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.<h4>Methods</h4>HP probands and family members prospectively recruited from 1995 to 2013 completed medical and family history questionnaires, and provided blood for DNA testing. Overall survival (until 12/ ...[more]

PMID: 30018304

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Dataset Information

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

Publications

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

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