Hereditary pancreatitis: Difference between revisions

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	Hereditary pancreatitis;
OMIM	167800
DiseasesDB	30026

VisualWikitext

Revision as of 20:29, 5 September 2012

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Hereditary Pancreatitis is a genetic disease affecting enzyme production in the pancreas.

Pathophysiology

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.

Epidemiology and Demographics

Prevalence

Most estimates place the number of individuals with hereditary pancreatitis in the United States at 1000.

Symptoms

Symptoms are generally the same as acute pancreatitis, including severe abdominal pain, vomiting, and diarrhea.

Treatment

There is no treatment for the disease, although complications such as malnutrition can be treated with supplimentary pancreatic enzymes.

References

Template:Gastroenterology

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+'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
+{{CMG}}
 ==Overview==
-'''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.
+'''Hereditary Pancreatitis''' is a genetic disease affecting enzyme production in the pancreas.
-==Symptoms and Treatment==
+==Pathophysiology==
-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.
+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.
+==Epidemiology and Demographics==
+===Prevalence===
 Most estimates place the number of individuals with hereditary pancreatitis in the United States at 1000.
+==Symptoms==
+Symptoms are generally the same as [[acute pancreatitis]], including severe abdominal pain, vomiting, and diarrhea.
+==Treatment==
+There is no treatment for the disease, although complications such as malnutrition can be treated with supplimentary pancreatic enzymes.
 ==References==
-* {{cite journal |author=Whitcomb D |title=Hereditary pancreatitis: new insights into acute and chronic pancreatitis |journal=Gut |volume=45 |issue=3 |pages=317-22 |year=1999 |pmid=10446089 |url=http://gut.bmjjournals.com/cgi/content/full/45/3/317}}
+{{Reflist|2}}
 {{Gastroenterology}}