[Hereditary pancreatitis]

Hereditary pancreatitis (HP) is a rare cause of chronic pancreatitis. Recurrent abdominal pain is the most common clinical manifestation, with onset in childhood or adolescence. PURPOSE--Report of a case of HP with atypical presentation and a review of the literature. METHODS--A non-alcoholic patient, without history of abdominal pain, with steatorrhea and malnutrition was investigated. The diagnostic evaluation revealed severe chronic pancreatitis. Two close relatives with early onset calcifying pancreatitis were detected. Epidemiologic and clinical features of HP were reviewed. CONCLUSION--Although uncommon, HP should be regarded in the differential diagnosis of chronic pancreatitis. Familial screening of suspected cases should be routinely performed.