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Hereditary pancreatitis is a rare genetic condition that causes multiple episodes of pancreatitis. The first episode of pancreatitis usually occurs in late childhood. Signs and symptoms may include stomach pain, fever, nausea, or vomiting. Episodes often lasts from one to three days, but may be longer. Repeat episodes of pancreatitis leads to chronic pancreatitis (a constantly inflamed pancreas), calcium deposits in the pancreas, and over years, pancreas scarring. At this stage signs and symptoms may include fatty stool, weight loss, and protein and vitamin deficiencies. People with hereditary pancreatitis are also at an increased risk for type 1 diabetes and pancreatic cancer, typically in adulthood. In most cases, hereditary pancreatitis is caused by mutations in the PRSS1 gene and passed through families in an autosomal dominant pattern. While there is not a cure for hereditary pancreatitis, treatments are available (visit the Pancreas Foundation to learn more about treatment). With treatment people with hereditary pancreatitis tend to have a normal life expectancy.
Source: GARD Last updated on 05-01-20
No. While people with the genetic condition, cystic fibrosis, are at an increased risk for both inguinal hernias and pancreatitis, we are otherwise unaware of an association between these conditions. However both hernias and pancreatitis can independently cluster in families. As a result, your risk for inguinal hernia increases if you have a close relative, such as a parent or sibling, who has the condition. Likewise your risk for pancreatitis increases if a close relative has pancreatitis. However having a family history of pancreatitis does not put you at an increased risk for hernia and vice versa. There are ongoing studies to learn more about the genetic risk component of these relatively common conditions.
Other risk factors for hernias, include chronic cough, chronic constipation, excess weight, pregnancy, doing heavy physical labor, premature birth, and being male.
Causes of pancreatitis, include abdominal injury, infection, having high triglyceride and/or calcium levels, gallstones, cigarette smoking, alcohol consumption, certain medications, and abdominal surgery.
Last updated on 05-01-20
Hereditary pancreatitis is rare, affecting roughly 1 in 300,000 people. This diagnosis is considered when two or more closely related members (e.g., parent and sibling) of the same family have chronic pancreatitis or recurring acute pancreatitis typically beginning at a young age. This diagnosis is also considered in a family who has three or more second-degree relatives (e.g., grandparent, grandchild, uncle, aunt, nephew, niece, half-sibling) in 2 or more generations with pancreatitis that can not be explained by other causes.
If you have a family history suggestive of hereditary pancreatitis, we recommend that you discuss this concern with your doctor. You can also consider meeting with a genetics professional. Genetics professionals are a source of information for individuals and families regarding genetic conditions, treatment, inheritance, and genetic risks to other family members. More information about finding and visiting a genetics professional is available from the Genetic Consultation Handbook developed by Genetics Home Reference.
To find a genetics clinic, we recommend that you contact your primary healthcare provider for a referral. The following online resources can also help you find a genetics professional in your community:
Last updated on 05-01-20
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