Hereditary pancreatitis
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This article needs additional citations for verification. Please help improve this article by adding reliable references. Unsourced material may be challenged and removed. (June 2007) |
| Hereditary pancreatitis Classification and external resources |
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| OMIM | 167800 |
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| DiseasesDB | 30026 |
Hereditary Pancreatitis is a genetic disease affecting enzyme production in the pancreas. In the pancreas, a genetic mutation causes the enzyme cationin 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.
[edit] 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.[citation needed]
[edit] References
- Whitcomb D (1999). "Hereditary pancreatitis: new insights into acute and chronic pancreatitis". Gut 45 (3): 317–22. PMID 10446089.
