Abstract

Hereditary pancreatitis is the second most common cause of chronic pancreatitis in children.1 In this entity, pancreatitis is clustered in families and inherited throughout the generations, involving many family members. The disease is transmitted as an autosomal dominant trait which is not sex-linked and has limited penetrance.1 Unfortunately, the etiology of this familial disease is unknown. The most common symptom in hereditary pancreatitis is recurrent attacks of abdominal pain due to repeated episodes of acute pancreatitis, which later may result in exocrine and/or endocrine pancreatic insufficiency. In cases in which pancreatic histology had been reported, normal pancreatic tissue was replaced by diffuse fibrosis and chronic inflammation.2

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