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Patients > Hereditary Pancreatitis
Hereditary Pancreatitis Research
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OMIM
Entry on Hereditary Pancreatitis
OMIM
Entry on SPINK1 Mutations
Hereditary Pancreatitis (HP) is a rare genetic condition characterized
by recurrent episodes of pancreatic attacks, which can progress
to chronic pancreatitis. Symptoms include abdominal pain, nausea,
and vomiting. Onset of attacks typically occurs between within the
first two decades of life, but can begin at any age. In the United
States, it is estimated that at least 1,000 individuals are affected
with hereditary pancreatitis.
HP has also been linked to an increased lifetime risk of pancreatic
cancer. Pancreatic cancer is the 4th most leading cause of cancer
deaths among Americans. Individuals with hereditary pancreatitis
appear to have a 40% lifetime risk to develop pancreatic cancer.
This increased risk is heavily dependent upon the duration of chronic
pancreatitis and environmental exposures to alcohol and smoking.
One recent study suggested that individuals with chronic pancreatitis
for more than 25 years had a higher rate of pancreatic cancer when
compared to individuals in the general population. This increased
rate appears to be due to the prolonged chronic pancreatitis rather
than having a gene mutation (all cationic trypsinogen mutations).
It is important to note that these risk values may be higher than
expected because these studies on pancreatic cancer use a highly
selective population rather than a randomly selected population.
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