Hereditary pancreatitis
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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.
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.
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Digestive system - Gastroenterology (primarily K20-K93, 530-579) |
| Esophagus |
Esophagitis - GERD - Achalasia - Boerhaave syndrome - Nutcracker esophagus - Zenker's diverticulum - Mallory-Weiss syndrome - Barrett's esophagus |
Stomach/
duodenum |
Peptic (gastric/duodenal) ulcer - Gastritis - Gastroenteritis - Duodenitis - Dyspepsia - Pyloric stenosis - Achlorhydria - Gastroparesis - Gastroptosis - Portal hypertensive gastropathy |
| Hernia |
Inguinal (Indirect, Direct) - Femoral - Umbilical - Incisional - Diaphragmatic - Hiatus |
| Noninfective enteritis and colitis |
IBD (Crohn's, Ulcerative colitis) - noninfective gastroenteritis |
| Other intestinal |
vascular (Abdominal angina, Mesenteric ischemia, Ischemic colitis, Angiodysplasia) - Ileus/Bowel obstruction (Intussusception, Volvulus) - Diverticulitis/Diverticulosis - IBS
other functional intestinal disorders (Constipation, Diarrhea, Megacolon/Toxic megacolon, Proctalgia fugax) - Anal fissure/Anal fistula - Anal abscess - Rectal prolapse - Proctitis (Radiation proctitis) |
| Liver/hepatitis |
Alcoholic liver disease - Liver failure (Acute liver failure) - Cirrhosis - PBC - NASH - Fatty liver - Peliosis hepatis - Portal hypertension - Hepatorenal syndrome |
| Accessory digestive |
Gallbladder (Gallstones, Choledocholithiasis, Cholecystitis, Cholesterolosis, Rokitansky-Aschoff sinuses)
Biliary tree (Cholangitis, Cholestasis/Mirizzi's syndrome, PSC, Biliary fistula, Ascending cholangitis)
Pancreas (Acute pancreatitis, Chronic pancreatitis, Pancreatic pseudocyst, Hereditary pancreatitis) |
| Other/general |
Appendicitis - Peritonitis (Spontaneous bacterial peritonitis)
Malabsorption (celiac, Tropical sprue, Blind loop syndrome, Whipple's)
postprocedural: Gastric dumping syndrome - Postcholecystectomy syndrome
bleeding: Hematemesis - Melena - Gastrointestinal bleeding (Upper, Lower) |
| See also congenital |
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