Orphanet: Hereditary chronic pancreatitis

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Hereditary chronic pancreatitis

Disease definition

A rare gastroenterologic disease characterized by recurrent acute pancreatitis and/or chronic pancreatitis in at least 2 first-degree relatives, or 3 or more second-degree relatives in 2 or more generations, for which no predisposing factors are identified. This rare inherited form of pancreatitis leads to irreversible damage to both exocrine and endocrine components of the pancreas.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood, Adolescent
  • ICD-10: K86.1
  • OMIM: 167800
  • UMLS: C0238339  C0341474
  • MeSH: -
  • GARD: 6632
  • MedDRA: -

Detailed information


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