Hereditary pancreatitis
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| Hereditary pancreatitis Classification and external resources | |
| OMIM | 167800 |
|---|---|
| DiseasesDB | 30026 |
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Hereditary Pancreatitis is a genetic disease affecting enzyme production in the pancreas. In the pancreas, a genetic mutation causes the enzyme trypsinogen to be made in a way which renders it resistant to inactivation through autolysis. Normally this autolysis mechanism prevents trypsinogen from being activated within the pancreas. However, when the abnormal trypsinogen is activated, it causes a chain reaction where all the trypsinogen in the pancreas is activated, effectively digesting the pancreas from the inside.
Symptoms and Treatment
Symptoms are generally the same as acute pancreatitis, including severe abdominal pain, vomiting, and diarrhea. There is no treatment for the disease, although complications such as malnutrition can be treated with supplimentary pancreatic enzymes.
Most estimates place the number of individuals with hereditary pancreatitis in the United States at 1000.
References
- Whitcomb D (1999). "Hereditary pancreatitis: new insights into acute and chronic pancreatitis". Gut 45 (3): 317-22. PMID 10446089.
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
