Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1389183 (autodigestion)
 317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hereditary pancreatitis (HP) is a rare, early-onset genetic disorder characterized by epigastric pain and often more serious complications. We now report that an Arg-His substitution at residue 117 of the cationic trypsinogen gene is associated with the HP phenotype. This mutation was observed in all HP affected individuals and obligate carriers from five kindreds, but not in individuals who married into the families nor in 140 unrelated individuals. X-ray crystal structure analysis, molecular modelling, and protein digest data indicate that the Arg 117 residue is a trypsin-sensitive site. Cleavage at this site is probably part of a fail-safe mechanism by which trypsin, which is activated within the pancreas, may be inactivated; loss of this cleavage site would permit autodigestion resulting in pancreatitis.
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PMID:Hereditary pancreatitis is caused by a mutation in the cationic trypsinogen gene. 884 Nov 72

We report a case of the mediastinal teratoma associated with acute cardiac tamponade followed by emergency operation. This tumor causes a variety of complications, but reports of acute cardiac tamponade have been rare. 20-year-old male admitted into our hospital with complaint of the chest pain, who was diagnosed with mediastinal tumor. She was admitted due to sudden fever up, and 5 days after admission, the complaint of severe epigastralgia and abdominal swelling were occurred. A chest X-ray film and chest computed tomography (CT) revealed cardiac tamponade. We carried out emergency surgical pericardial drainage and extirpation of the tumor. The tumor was composed of cystic part involving of dark green fluid macroscopically. The amylase level in this fluid was 42 U/l. We observed a small hole on the pericardial sac beside the base of ascending aorta. The amylase level in pericardial effusion was 19 U/l. Histopathological diagnosis was mature teratoma perforating into pericardiac sac, which included skin, bronchus and pancreatic tissue. We concluded that the progress of the tumor and the mechanical inflammation were major cause of perforation of the tumor into the pericardial sac, and autodigestion by enzyme from pancreas was minor cause of perforation.
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PMID:[Perforating mediastinal mature teratoma associated with acute cardiac tamponade; report of a case]. 1536 80