ArticleJournal of gastroenterology2018
Nationwide survey of hereditary pancreatitis in Japan.
Article in Journal of gastroenterology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers, 1 of them a synthesis that pooled it.
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Who cites it
26 citing papers in PubMed, 1 synthesis or guideline pooled it, 50 citations in OpenAlex.
- The Epidemiology of PRSS1 Hereditary Pancreatitis and Its Clinical Implications: A Systematic Review.Pancreas · 2026Pooled it
- Hereditary Pancreatic Cancer: Genetic Risk, Surveillance Strategies, and Therapeutic Implications.International journal of molecular sciences · 2026Review
- Genotype-Phenotype Correlations in Pediatric Hereditary Pancreatitis: Evidence from a Romanian Retrospective Cohort.Journal of clinical medicine · 2026Article
- TRPV6-related pancreatitis: natural history and the impact of the pancreas-specific deletion on pancreatitis in mice.Journal of gastroenterology · 2026Article
- Expanding the ethnic and geographic spectrum of hereditary pancreatitis: first report of a Tibetan case with compoundHepatobiliary surgery and nutrition · 2025Article
- Article
- A narrative review on the role of genetics in children with acute recurrent pancreatitis and chronic pancreatitis.Pediatric investigation · 2023Review
- Spectrum ofSovremennye tekhnologii v meditsine · 2023Article
- Evidence-based clinical practice guidelines for chronic pancreatitis 2021.Journal of gastroenterology · 2022Review
- Disease-related protein co-expression networks are associated with the prognosis of resectable node-positive pancreatic ductal adenocarcinoma.Scientific reports · 2022Article
- Current status of inherited pancreatic cancer.Hereditary cancer in clinical practice · 2022Review
- Imaging Modalities for Early Detection of Pancreatic Cancer: Current State and Future Research Opportunities.Cancers · 2022Review
- Hereditary pancreatic cancer.International journal of clinical oncology · 2021Review
- An Autopsy Case of Anaplastic Carcinoma of the Pancreas in a 39-Year-Old Woman that Developed from Hereditary Pancreatitis.The American journal of case reports · 2021Article
- Endosonographic features in patients with non-alcoholic early chronic pancreatitis improved with treatment at one year follow up.Journal of clinical biochemistry and nutrition · 2021Article
- Genetic Abnormalities in Pancreatitis: An Update on Diagnosis, Clinical Features, and Treatment.Diagnostics (Basel, Switzerland) · 2020Review
- Endoscopic Ultrasonography Findings of Early and Suspected Early Chronic Pancreatitis.Diagnostics (Basel, Switzerland) · 2020Review
- Nationwide epidemiological survey of chronic pancreatitis in Japan: introduction and validation of the new Japanese diagnostic criteria 2019.Journal of gastroenterology · 2020Article
- Article
- Diabetes of the Exocrine Pancreas Related to Hereditary Pancreatitis, an Update.Current diabetes reports · 2020Review
Corrections and comments
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Authors and funding
10 authors at 4 institutions in 1 country.
Funding
Abstract
backgroundHereditary pancreatitis (HP) is a rare cause of chronic pancreatitis. We here report a nationwide survey to clarify the epidemiological, genetic, and clinical features of HP in Japan.
methodsTarget subjects were patients with HP and their family members who had visited selected hospitals between 2005 and 2014. This study consisted of two-stage surveys; patients with HP were identified by the first questionnaire and their clinical features were assessed by the second questionnaire.
resultsTwo hundred seventy-one patients (153 males and 118 females) in 100 families diagnosed based on the Japanese criteria or 231 patients (131 males and 100 females) patients in 80 families based on the EUROPAC criteria were reported. Of the families undertaking genetic tests, 41% had the PRSS1 mutations (p.R122H 33%, p.N29I 8%) and 36% had the SPINK1 mutations (p.N34S 22%, c.194+2T>C 14%, p.P45S 1%). The mean age at symptom onset was 17.8 years. The cumulative rates of pancreatic exocrine insufficiency and diabetes mellitus were 16.1 and 5.5% at 20 years old, and 45.3 and 28.2% at 40 years, respectively. Forty-four percent of the patients underwent endoscopic treatment and/or surgery. The cumulative rate of pancreatic cancer diagnosis was 2.8% at 40 years old, 10.8% at 60 years, and 22.8% at 70 years.
conclusionsHP was characterized by early disease onset, frequent development of pancreatic exocrine insufficiency and diabetes mellitus, requirement of endoscopic treatment and/or surgery, and increased risk of pancreatic cancer. PRSS1 and SPINK1 mutations serve as genetic background for HP in Japan.
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