Hereditary pancreatitis: report of an affected Canadian kindred and review of the disease.

Hereditary pancreatitis is an autosomal dominant disease with no other known cause. It usually begins in childhood and is characterized by recurrent attacks of abdominal pain of variable intensity and duration, followed by symptom-free periods. The diagnosis is usually made in early adult life, when pancreatic insufficiency and calcifications appear. Complications are less frequent than in nonhereditary chronic pancreatitis. There are also differences between the two forms of chronic pancreatitis in sex incidence, etiologic factors and life expectancy. In a Canadian kindred three generations are affected with hereditary pancreatitis; there are four definite and four suspected cases. More than 40 affected kindreds, including 195 proven cases and 190 suspected cases, have now been reported in the literature. Thus, hereditary pancreatitis should be considered in the differential diagnosis of chronic relapsing pancreatitis of unknown cause as well as recurrent abdominal pain in childhood.