Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011854 (type 1 diabetes)
 20,749 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Poly arteritis nodosa (PAN) is a systemic vasculitis with a male: female ratio of 2:1 and a peak incidence in the fifth decade. Small to medium-sized arteries are involved by focal transmural inflammatory necrosis. Aneurysms with inflammatory destruction of the media also occur. The most frequently involved organs are the kidney, heart, lung, liver, and gastrointestinal tract. There are few reported cases of ischemic necrosis of the intestine and even fewer survivors. A 22-year-old woman was transferred to St. Thomas Hospital (Nashville, TN) after resection of 80 per cent of the small bowel for ischemic necrosis. She had a history of juvenile onset diabetes mellitus, recurrent abdominal pain, and splinter hemorrhages. Emergency aortogram and selective mesenteric arteriogram were performed. The celiac artery was not visualized and small aneurysms were present in the mesenteric and renal arteries. The patient was successfully resuscitated from a cardiac arrest in x ray from a cardiac tamponade. Laparotomy was performed to determine the viability of the bowel. The celiac, hepatic, and splenic arteries were found to be chronically occluded. Pathology of these arteries revealed a nonspecific arteritis. At a third operation, several more inches of small bowel were removed. Characteristic changes of PAN were present on all small bowel specimens. She was treated with high-dose cyclophosphamide and steroids for 6 months and has continued on low-dose cyclophosphamide. She is now 36 months from her original operation and is doing well on oral nutrition. Intestinal hemorrhage from aneurysm rupture or gangrene with perforation are gastrointestinal complications of PAN that the surgeon may be called upon to treat.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surviving gastrointestinal infarction due to polyarteritis nodosa: a rare event. 134 8

Type II autoimmune polyglandular syndrome (APS), a relatively common endocrine disorder, includes primary adrenal insufficiency coupled with type 1 diabetes mellitus and/or autoimmune primary hypothyroidism. Autoimmune serositis, an associated disease, may present as symptomatic pericardial effusion. We present a case of a 54-year old male with APS who developed pericarditis leading to cardiac tamponade with a subacute loculated effusion. After urgent pericardiocentesis intrapericardial pressure dropped to 0, while central venous pressures remain elevated, consistent with acute effusive constrictive pericarditis. Contrast computerized tomography confirmed increased pericardial contrast enhancement. The patient recovered after prolonged inotropic support and glucocorticoid administration. He re-accumulated the effusion 16 days later, requiring repeat pericardiocentesis. Effusive-constrictive pericarditis, an uncommon pericardial syndrome, is characterized by simultaneous pericardial inflammation and tamponade. Prior cases of APS associated with cardiac tamponade despite low volumes of effusion have been reported, albeit without good demonstration of hemodynamic findings. We report a case of APS with recurrent pericardial effusion due to pericarditis and marked hypotension with comprehensive clinical and hemodynamic assessment. These patients may require aggressive support with pericardiocentesis, inotropes, and hormone replacement therapy. They should be followed closely for recurrent tamponade.
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PMID:Cardiac tamponade due to low-volume effusive constrictive pericarditis in a patient with uncontrolled type II autoimmune polyglandular syndrome. 2455 26