Hereditary Pancreatitis.

Authors

Mantej Sehmbhi, Aimee Lee Lucas

Published in

Gastroenterology clinics of North America. Volume 55. Issue 4. Pages 693-706. Epub Jul 10, 2026.

Abstract

There are multiple genetic defects associated with hereditary pancreatitis, which have variable penetrance. Important genes include PRSS1, serine protease inhibitor Kazal type 1, and cystic fibrosis transmembrane conductance regulator. Disease pathogenesis is typically through trypsin dysregulation, intracellular stress, or altered pancreatic duct physiology. There is considerable variability in epidemiology, clinical manifestation, and prognosis between the known variants. Management should include environmental risk factor modification, monitoring for endocrine and exocrine insufficiency and malnutrition, screening for pancreatic cancer if appropriate, and a multidisciplinary approach to address long-term complications and quality of life for patients and families.

PMID:
42933941
Bibliographic data and abstract were imported from PubMed on 27 Oct 2026.

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