True regarding hereditary pancreatitis? – Surgery MCQ

Which of the following is true regarding hereditary pancreatitis?
A. Autosomal recessive disorder
B. Has 80% penetrance
C. Accounts for about 30% of cases of chronic pancreatitis
D. IgG4 concentrations are elevated

Correct answer : B. Has 80% penetrance

Chronic pancreatitis - CT scanCT scan of chronic pancreatitis. Calcification is visible.

  • Hereditary pancreatitis is an autosomal dominant disorder.
  • It has 80% penetrance.
  • It is associated with mutations in the cationic trypsinogen gene located on chromosome 7.
  • IgG4 concentrations are elevated in autoimmune pancreatitis.

Ref: Bailey and Love’s Short Practice of Surgery, 25th edition, p 1146.
Image credits: Hellerhoff. https://en.wikipedia.org/wiki/File:Chronische_Pankreatitis_mit_Verkalkungen_-_CT_axial.jpg Licensed under the Creative Commons Attribution-Share Alike 3.0 Unported license.