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Hereditary pancreatitis



Hereditary pancreatitis
Classification & external resources
OMIM 167800
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.

Additional recommended knowledge

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]

References

  • Whitcomb D (1999). "Hereditary pancreatitis: new insights into acute and chronic pancreatitis". Gut 45 (3): 317-22. PMID 10446089.
 
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Hereditary_pancreatitis". A list of authors is available in Wikipedia.
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