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Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden
Sarah Jane Schwarzenberg1, Melena Bellin1, Sohail Z Husain2
1University of Minnesota Children's Hospital, Minneapolis, Minnesota, USA.
Insights
Chronic pancreatitis in children presents early with genetic or obstructive factors. This condition significantly impacts their quality of life, leading to frequent pain, school absences, and medical interventions.
Area of Science:
- Pediatric Gastroenterology
- Pancreatology
- Clinical Research
Background:
- Chronic pancreatitis (CP) is a debilitating condition in adults, but its characteristics in children are less understood.
- Pediatric pancreatitis presents unique challenges due to ongoing growth and development.
Purpose of the Study:
- To define the clinical presentation, diagnostic factors, risk factors, and disease burden of chronic pancreatitis in pediatric patients.
- To identify key differences and commonalities in the presentation of pediatric chronic pancreatitis.
Main Methods:
- A cross-sectional study utilizing data from the "In Search for a Cure" registry, which collects information on pediatric acute recurrent and chronic pancreatitis.
- Statistical analysis using the Wilcoxon rank-sum test to compare groups.
Main Results:
- Of 170 subjects, 76 (45%) had chronic pancreatitis, diagnosed at a median age of 9.9 years. Genetic mutations (67%) and obstructive factors (33%) were common causes.
- Abdominal pain affected 77%, with 28% experiencing constant pain requiring narcotics. Significant disease burden included frequent emergency visits (median 3), hospitalizations (median 2), and school absenteeism (34% missing ≥3 days).
- Interventional procedures like endoscopic retrograde pancreatography (43%) and pancreatic surgeries (39%) were common.
Conclusions:
- Pediatric chronic pancreatitis manifests at a young age with specific clinical features.
- Genetic and obstructive etiologies are prevalent in children with CP.
- The disease imposes a substantial burden on children, affecting their daily lives and requiring significant medical management.
Objective:
To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis.
Study Design:
We performed a cross-sectional study of data from the International Study Group of Pediatric Pancreatitis: In Search for a Cure, a registry of children with acute recurrent pancreatitis and chronic pancreatitis. Between-group differences were compared using Wilcoxon rank-sum test.
Results:
Among 170 subjects in the registry, 76 (45%) had chronic pancreatitis; 57% were female, 80% were white; median age at diagnosis was 9.9 years. Pancreatitis-predisposing genetic mutations were identified in 51 (67%) and obstructive risk factors in 25 (33%). Toxic/metabolic and autoimmune factors were uncommon. Imaging demonstrated ductal abnormalities and pancreatic atrophy more commonly than calcifications. Fifty-nine (77%) reported abdominal pain within the past year; pain was reported as constant and receiving narcotics in 28%. Children with chronic pancreatitis reported a median of 3 emergency department visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic endoscopic retrograde pancreatography; one or more pancreatic surgeries were performed in 30 (39%).
Conclusions:
Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.
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