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As an educator, I appreciated the thorough discussion in "Stopping the Destruction of Acute Pancreatitis" (June 2007).* I wish to add another dimension. My students and I recently cared for a patient with a history of chronic pancreatitis with many past admissions for acute episodes. We assumed that he also had a history of alcoholism, but his social history was negative for alcohol use. The attending physician told us that our patient's disease was hereditary and that several members of his family had it.


Although more than 80% of cases of chronic pancreatitis are due to alcoholism, hereditary pancreatitis accounts for 1% of pancreatitis cases worldwide.1 An autosomal dominant disease, hereditary pancreatitis may cause symptoms in childhood, but a patient may not be diagnosed until recurrent episodes of acute pancreatitis have damaged the pancreas. Hereditary pancreatitis carries a 40% cumulative lifetime risk of pancreatic cancer.2


Some areas have clusters of families (called "kindreds") with the genetic predisposition. Pockets of people with hereditary pancreatitis have been documented in Japan, Korea, Thailand, Australia, and France. In the United States, over 100 kindreds have been identified in the central Appalachian region; kindreds also appear in northern California.


Any patient with a family history of acute pancreatitis (or unexplained abdominal pain) should be considered at risk for hereditary pancreatitis, and family members with the disease should be documented in the patient's record.




Morristown, Tenn.




1. Levy P, et al. Estimation of the prevalence and incidence of chronic pancreatitis and its complications. Gastroenterologie Clinique et Biologique. 30(6-7):838-444, June-July 2006. [Context Link]


2. Charnley RM. Hereditary pancreatitis. World Journal of Gastroenterology. 9(1):1-4, January 2003. [Context Link]


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