This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Hereditary pancreatitis causes multiple episodes of inflammation of the pancreas (pancreatitis), an important digestive organ. Symptoms usually begin in childhood and may last a few days or longer. Signs and symptoms may include stomach pain, nausea, or vomiting. People with hereditary pancreatitis develop chronic pancreatitis, a constantly inflamed pancreas. This leads to symptoms which may include fatty stools, weight loss, and poor absorption of nutrients from food. Adults with hereditary pancreatitis are at an increased risk for type 1 diabetes and pancreatic cancer. In most cases, hereditary pancreatitis is due to a PRSS1 gene that is not working correctly and is inherited in an autosomal dominant pattern. Diagnosis is based on the symptoms, a clinical history and exam, and the results of genetic testing. Treatment is focused on managing the symptoms and may include medications, surgery, and surveillance for diabetes and cancer.
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