Hereditary pancreatitis is characterised by all of the following except
Hereditary pancreatitis: Is a autosomal dominant condition. Hereditary factors are increasingly being recognized as a significant cause of pancreatitis. Hereditary pancreatitis is characterized by recurrent attacks of severe acute pancreatitis often beginning in childhood and ultimately leading to chronic pancreatitis. The disorder is genetically diverse, but the shared feature of most forms is a defect that increases or sustains the activity of trypsin. Three genes implicated in hereditary pancreatitis deserve special note: PRSS1, SPINK1, and CFTR. Of note, patients with hereditary pancreatitis have a 40% lifetime risk of developing pancreatic cancer. Autosomal dominant condition have high degree of penetrance.
Autosomal recessive inheritance
Increased risk of pancreatic cancer
80% penetrance
Increased risk of chronic pancreatitis
JIPMER 2017