ReviewWorld journal of clinical pediatrics2022
Hereditary pancreatitis: An updated review in pediatrics.
Review in World journal of clinical pediatrics, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed.
- Article
- Early-Onset and Rapidly Progressive Hereditary Pancreatitis Associated with PRSS1 Mutations in a Romanian Pediatric Cohort.Maedica · 2026Article
- Infantile exocrine pancreatic insufficiency due to a homozygousJPGN reports · 2026Article
- Genotype-Phenotype Correlations in Pediatric Hereditary Pancreatitis: Evidence from a Romanian Retrospective Cohort.Journal of clinical medicine · 2026Article
- Gene, genetics and genetic medicines in gastroenterology: Current status and its future.World journal of gastroenterology · 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
- Article
- Case Report: a family report of hereditary pancreatitis caused by SPINK1 and PRSS1 gene mutations.Frontiers in genetics · 2025Article
- Editorial: Insights in pediatric gastroenterology, hepatology & nutrition.Frontiers in pediatrics · 2025Article
- Turkish Society of Gastroenterology: Pancreas Working Group, Acute Pancreatitis Committee Consensus Report.The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology · 2024Review
- Diagnosis of Hereditary Pancreatitis Following the Initial Acute Episode With Multiple Pseudocyst Complications.Cureus · 2024Article
- Puestow surgery in a SPINK mutation chronic pancreatitis: a rare case report.Journal of surgical case reports · 2024Article
- Identification of RAD17 as a candidate cancer predisposition gene in families with histories of pancreatic and breast cancers.BMC cancer · 2024Article
- Pancreatic Crosstalk in the Disease Setting: Understanding the Impact of Exocrine Disease on Endocrine Function.Comprehensive Physiology · 2024Review
- A case of early-onset idiopathic chronic pancreatitis associated with a loss-of-function TRPV6 p.R483Q variant successfully treated by pancreatic duct stenting.Clinical journal of gastroenterology · 2023Article
- Hyperthermia combined with chemotherapyWorld journal of clinical oncology · 2023Article
- Persistent Eosinophilia and Fever in Pancreatitis: A Clinical Conundrum.Journal of investigative medicine high impact case reportsArticle
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hereditary Pancreatitis (HP) has emerged as a significant cause of acute, acute recurrent and chronic pancreatitis in the pediatric population. Given that it presents similarly to other causes of pancreatitis, a positive family history and/or isolation of a gene mutation are vital in its designation. Inheritance patterns remain complex, but mutations involving the PRSS1, SPINK1, CFTR and CTRC genes are commonly implicated. Since being first described in 1952, dozens of genetic alterations that modify the action of pancreatic enzymes have been identified. Among children, these variants have been isolated in more than 50% of patients with chronic pancreatitis. Recent research has noted that such mutations in PRSS1, SPINK1 and CFTR genes are also associated with a faster progression from acute pancreatitis to chronic pancreatitis. Patients with HP are at increased risk of developing diabetes mellitus, exocrine pancreatic insufficiency, and pancreatic adenocarcinoma. Management follows a multi-disciplinary approach with avoidance of triggers, surveillance of associated conditions, treatment of pancreatic insufficiency and use of endoscopic and surgical interventions for complications. With significant sequela, morbidity and a progressive nature, a thorough understanding of the etiology, pathophysiologic mechanisms, diagnostic evaluation, current management strategies and future research considerations for this evolving disease entity in pediatrics is warranted.
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