/hereditary chronic pancreatitis

hereditary chronic pancreatitis

Rare Disease

MONDO:0008185

Also Known As

hereditary chronic pancreatitishereditary pancreatitisHPCHpPCTTautosomal dominant hereditary pancreatitisfamilial pancreatitispancreatitis, calcificpancreatitis, calcific, includedpancreatitis, chronicpancreatitis, chronic pancreatitis, chronic, susceptibility to, includedpancreatitis, chronic, protection againstpancreatitis, chronic, protection against, includedpancreatitis, chronic, susceptibility topancreatitis, hereditary

Definition

Hereditary chronic pancreatitis (HCP), a rare inherited form of pancreatitis is defined as recurrent acute pancreatitis and/or chronic pancreatitis in two first-degree relatives or 3 or more second-degree relatives in 2 or more generations, for which no predisposing factors are identified. HCP is characterized by irreversible damage to both exocrine and endocrine components of the pancreas.