Hereditary pancreatitis

Last revised by Jeremy Jones on 20 Sep 2021

Hereditary pancreatitis is a rare subtype of chronic pancreatitis that has an autosomal dominant inheritance and is one of the main causes of pancreatitis in childhood. 

Clinical presentation

As a congenital condition, a presentation with acute pancreatitis attacks typically occurs in childhood, and features of chronic pancreatitis with parenchymal and ductal calcifications and atrophy are commonly seen when patients become adolescents.

The diagnosis is clinically considered when dealing with idiopathic chronic pancreatitis in young patients, especially when family history is present. 

The condition has been reported with a 50-70-fold increased risk of pancreatic cancer 1.

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