ArticleThe American journal of gastroenterology2018
Hereditary Pancreatitis in the United States: Survival and Rates of Pancreatic Cancer.
Article in The American journal of gastroenterology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers, 3 of them syntheses that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
50 citing papers in PubMed, 3 syntheses or guidelines pooled it, 107 citations in OpenAlex.
- The Epidemiology of PRSS1 Hereditary Pancreatitis and Its Clinical Implications: A Systematic Review.Pancreas · 2026Pooled it
- Endoplasmic stress-inducing variants in CPB1 and CPA1 and risk of pancreatic cancer: A case-control study and meta-analysis.International journal of cancer · 2022Pooled it
- Germline Variants and Risk for Pancreatic Cancer: A Systematic Review and Emerging Concepts.Pancreas · 2018Pooled it
- Hereditary Pancreatic Cancer: Genetic Risk, Surveillance Strategies, and Therapeutic Implications.International journal of molecular sciences · 2026Review
- Early-Onset and Rapidly Progressive Hereditary Pancreatitis Associated with PRSS1 Mutations in a Romanian Pediatric Cohort.Maedica · 2026Article
- Toward Timely Diagnosis of Pancreatic Cancer: Revolutionizing Early Detection Through Genomics, Artificial Intelligence, and Noninvasive Biomarkers.Journal of gastroenterology and hepatology · 2026Review
- Germline genetic profiling utilizing multigene panel testing in Jordanian patients with pancreatic cancer: a comprehensive cancer center experience.BMC cancer · 2026Article
- Approach to the Patient With Pancreatogenic Diabetes.The Journal of clinical endocrinology and metabolism · 2026Review
- Challenges of early detection of pancreatic cancer.The Journal of clinical investigation · 2025Review
- MisfoldingAmerican journal of physiology. Gastrointestinal and liver physiology · 2025Article
- Current Approaches of Pancreatic Cancer Surveillance in High-Risk Individuals.Journal of gastrointestinal cancer · 2025Review
- Enhancing Early Detection of Pancreatic Cancer in Genetically Predisposed Individuals: Integrating Advanced Imaging Modalities with Emerging Biomarkers and Liquid Biopsy.Biologics : targets & therapy · 2025Review
- Case Report: a family report of hereditary pancreatitis caused by SPINK1 and PRSS1 gene mutations.Frontiers in genetics · 2025Article
- Genetic and other risk factors for pancreatic ductal adenocarcinoma (PDAC).Familial cancer · 2024Review
- [Pancreatic cancer-screening or surveillance?]Radiologie (Heidelberg, Germany) · 2023Review
- A narrative review on the role of genetics in children with acute recurrent pancreatitis and chronic pancreatitis.Pediatric investigation · 2023Review
- Pancreatic Cancer: Changing Epidemiology and New Approaches to Risk Assessment, Early Detection, and Prevention.Gastroenterology · 2023Article
- Epidemiologic risk factors for patients admitted with chronic pancreatitis and pancreatic ductal adenocarcinoma in the United States.World journal of clinical oncology · 2022Article
- Endoplasmic stress-inducing variants in carboxyl ester lipase and pancreatic cancer risk.Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] · 2022Article
- Positivity resonance in long-term married couples: Multimodal characteristics and consequences for health and longevity.Journal of personality and social psychology · 2022Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
objectivesHereditary 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.
methodsHP 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.
results217 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.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.