The Epidemiology of PRSS1 Hereditary Pancreatitis and Its Clinical Implications: A Systematic Review.
PancreasSep 1, 2026 (epub)
Denghao Wu, Gorjana Radisic, Sunita M C De Sousa et al.
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In 30 seconds
This systematic review examined the epidemiology and clinical implications of PRSS1 hereditary pancreatitis (HP) across 68 studies. The findings indicate that over 70% of affected individuals present before age 18, highlighting early disease onset. Pain and reduced quality of life are significant concerns, with diabetes prevalence in adults reported at 14%-26%.
Key findings
- Over 70% of individuals with PRSS1-associated HP presented before age 18.
- Diabetes prevalence ranged from minimal in children to 14%-26% in adults.
- Pain and reduced quality of life were the most frequently reported clinical features.
- PRSS1 R122H and N29I were the most common pathogenic variants identified.
Why it matters
Understanding the epidemiology and clinical features of PRSS1 hereditary pancreatitis is crucial for early diagnosis and management. Clinicians can better anticipate complications and tailor care strategies for affected patients.