ReviewOrphanet journal of rare diseases2007
Hereditary chronic pancreatitis.
Review in Orphanet journal of rare diseases, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 224 citations in OpenAlex.
- Understanding the Pathophysiology of Chronic Pancreatitis: A Comprehensive Review Unraveling Pain Mechanisms and the Role of Psychosocial Factors.Journal of clinical medicine · 2026Review
- Acute Pancreatitis as an Initial Presentation of Hereditary Chronic Calcific Pancreatitis with Pancreatic Duct Stones: A Case Report.Clinical medicine insights. Case reports · 2026Article
- Nutritional Management in Chronic Pancreatitis: From Exocrine Pancreatic Insufficiency to Precision Therapy.Nutrients · 2025Review
- Innate Immunity and Platelets: Unveiling Their Role in Chronic Pancreatitis and Pancreatic Cancer.Cancers · 2025Review
- Minimally invasive total pancreatectomy with islet autotransplantation for chronic pancreatitis: the robotic approach.Surgical endoscopy · 2024Article
- Ayurvedic Treatment Protocol for Hereditary Pancreatitis: A Case Report Demonstrating Disease Arrestation.Cureus · 2023Article
- The clinical and genetic features of hereditary pancreatitis in South Australia.The Medical journal of Australia · 2022Article
- Diabetes of the Exocrine Pancreas Related to Hereditary Pancreatitis, an Update.Current diabetes reports · 2020Review
- PRSS1 (R122H) mutation in an Indian family with low penetrance is associated with pancreatitis phenotype.Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology · 2018Article
- Review
- Hereditary Pancreatitis Associated With the N29T Mutation of the PRSS1 Gene in a Brazilian Family: A Case-Control Study.Medicine · 2015Observational
- Pharmacological attenuation of chronic alcoholic pancreatitis induced hypersensitivity in rats.World journal of gastroenterology · 2015Article
- The genetic predisposition and its impact on the diabetes mellitus development in patients with alcoholic chronic pancreatitis.Gastroenterology research and practice · 2015Article
- Hereditary pancreatitis: dilemmas in differential diagnosis and therapeutic approach.Journal of gastrointestinal cancer · 2014Review
- Animal models of pancreatitis: can it be translated to human pain study?World journal of gastroenterology · 2013Review
- Two cases of chronic pancreatitis associated with anomalous pancreaticobiliary ductal union and SPINK1 mutation.Korean journal of pediatrics · 2013Article
- Idiopathic chronic calcific pancreatitis in a child: An uncommon entity.Journal of natural science, biology, and medicine · 2013Article
- Genetic risk for alcoholic chronic pancreatitis.International journal of environmental research and public health · 2011Review
- Abdominal pain in a 5-year-old boy.BMJ case reports · 2011Article
- Acute pancreatitis complicated with choledochal duct rupture.Case reports in gastrointestinal medicine · 2011Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary chronic pancreatitis (HCP) is a very rare form of early onset chronic pancreatitis. With the exception of the young age at diagnosis and a slower progression, the clinical course, morphological features and laboratory findings of HCP do not differ from those of patients with alcoholic chronic pancreatitis. As well, diagnostic criteria and treatment of HCP resemble that of chronic pancreatitis of other causes. The clinical presentation is highly variable and includes chronic abdominal pain, impairment of endocrine and exocrine pancreatic function, nausea and vomiting, maldigestion, diabetes, pseudocysts, bile duct and duodenal obstruction, and rarely pancreatic cancer. Fortunately, most patients have a mild disease. Mutations in the PRSS1 gene, encoding cationic trypsinogen, play a causative role in chronic pancreatitis. It has been shown that the PRSS1 mutations increase autocatalytic conversion of trypsinogen to active trypsin, and thus probably cause premature, intrapancreatic trypsinogen activation disturbing the intrapancreatic balance of proteases and their inhibitors. Other genes, such as the anionic trypsinogen (PRSS2), the serine protease inhibitor, Kazal type 1 (SPINK1) and the cystic fibrosis transmembrane conductance regulator (CFTR) have been found to be associated with chronic pancreatitis (idiopathic and hereditary) as well. Genetic testing should only be performed in carefully selected patients by direct DNA sequencing and antenatal diagnosis should not be encouraged. Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications, such as pseudocysts, bile duct or duodenal obstruction. The disease course and prognosis of patients with HCP is unpredictable. Pancreatic cancer risk is elevated. Therefore, HCP patients should strongly avoid environmental risk factors for pancreatic cancer.
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Registered trials
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.