Cases reported "Pancreatitis"

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1/58. Complete agenesis of the dorsal pancreas.

    Pancreatic anomalies are occasionally reported, but complete agenesis of the dorsal pancreas is extremely rare. We report a 47-year-old woman with complete agenesis of the dorsal pancreas. This patient initially presented with jaundice. Computed tomography did not reveal the pancreatic corpus or tail. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography did not visualize the dorsal pancreatic duct. Choledochojejunostomy was performed because she had obstructive jaundice. At laparotomy, there was an enlarged pancreatic head, but no distal pancreas was seen. Histological examination of the pancreatic biopsy specimen showed scattered islets of langerhans in diffuse fibrosis, with destruction of the glandular parenchyma. This case was diagnosed as complete agenesis of the dorsal pancreas.
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2/58. Hepatobiliary and pancreatic infections in AIDS: Part II.

    In the first part of our review, we discussed the general evaluation and clinical presentation of the various hepatic infections occurring in patients with AIDS. In addition, we focused on specific hepatic parenchymal infections. In this article, we will discuss the major clinical syndromes arising from opportunistic infections affecting the gallbladder (acalculous cholecystitis), biliary tree (AIDS-cholangiopathy), and pancreas (pancreatitis). acalculous cholecystitis can develop in patients with AIDS who have not experienced the severe precipitating physiologic stresses normally required in patients without AIDS. The most common presentation is with right upper quadrant (RUQ) pain and tenderness. The diagnosis is a clinical one since there is no standard test, other than surgery. cholecystectomy is the treatment of choice. The most common AIDS-associated infective complication of the biliary tree is AIDS-cholangiopathy. This is best viewed as a form of secondary sclerosing cholangitis resulting from a variety of opportunistic infections within the biliary tree. Affected persons present with RUQ pain and have marked elevations in the canalicular enzymes, alkaline phosphatase, and gamma-glutamyl transferase. Morphologic abnormalities are identified by endoscopic retrograde cholangiopancreatography. These include stricturing, dilatation, and beading of the biliary tract. Endoscopic sphincterotomy of the papilla of Vater may provide symptomatic relief for patients with papillary stenosis. opportunistic infections within the pancreas gland have been documented in both pre- and postmortem studies. However, the true incidence of pancreatitis related to infections is unknown. The presentation is similar to that of pancreatitis from other causes. A computerized tomogram of the abdomen is the investigation of choice. Tissue aspiration or biopsy of the pancreas is required to demonstrate the presence of an opportunistic infection. The management is usually supportive, as it is rare that a specific infection is identified and treated.
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3/58. Heterotopic pancreatitis: gastric outlet obstruction due to an intramural pseudocyst and hamartoma.

    Heterotopic pancreas, usually a silent gastrointestinal malformation, may become clinically evident when complicated by chronic inflammation. We report a case of pancreatitis and extensive pseudocyst formation in the gastric antrum, which caused gastric outlet obstruction. The diagnosis was obscured by a history of emesis during pregnancy and a previously resected gastric polyp. The nature of the obstructive lesion was not diagnosed preoperatively in spite of endosonographic evaluation. Intraoperatively, a cystic tumor of the stomach wall was found, the lesion was excised, and a pyloroplasty was performed to close the excision site. histology revealed heterotopic pancreatic tissue with chronic inflammation, fibrosis and pseudocyst formation and adjacent to this lesion a myoglandular hamartoma. The patient is symptom-free two years after surgery and no recurrence was found. The nature of heterotopic pancreatic tissue, its diagnosis and management are discussed.
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4/58. Secondary pancreatic involvement by diffuse large B-cell lymphoma presenting as acute pancreatitis: treatment and outcome.

    We report the case of a 33-year-old man who presented with a large B-cell non Hodgkin's lymphoma presenting as acute pancreatitis. Abdominal CT showed diffuse swelling of the pancreas, with two distinct masses in the corpus and the tail. Thoracic CT showed a markedly enlarged mediastinum, with a voluminous mass in the middle mediastinum. Direct biopsy of this mass revealed a large B-cell lymphoma. Chemotherapy followed by peripheral blood cell autotransplantation led to complete disappearance of the pancreatic and mediastinal masses. Fatty diarrhea occurred after chemotherapy, probably owing to gland destruction by lymphomatous infiltration. Twenty-six months later, the patient is disease-free but continues to require pancreatic enzyme supplements.
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5/58. Issues of nutritional support for the patient with acute pancreatitis.

    Management strategies in the nutritional support of the patient with acute pancreatitis have changed dramatically over the past 10 years. Prospective randomized trials show that maintaining gut integrity is equally as important as placing the pancreas at rest while inflammation within the gland resolves. In comparison to total parenteral nutrition and gut disuse, enteral feeding attenuates disease severity, reduces oxidative stress, and improves patient outcome. Nasojejunal feeds infused at or below the Ligament of Treitz should be provided to those patients with severe pancreatitis, as identified by a number of standardized scoring systems such as Ranson Criteria, apache II, Glasgow, and Imrie scores. Total parenteral nutrition should be reserved only for the patient with severe pancreatitis, initiated 4 to 5 days after peak inflammation in whom intolerance to enteral feeding has been shown and/or enteral access cannot be obtained. Vigilant monitoring is required to assure safe and effective delivery of enteral nutrients.
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6/58. A primary sjogren's syndrome patient with marked swelling of multiple exocrine glands and sclerosing pancreatitis.

    A 55-year-old man suffered from nasal obstruction, swelling of the salivary glands, and diplopia caused by markedly enlarged lacrimal glands. The diagnosis of primary sjogren's syndrome was made by a positive Schirmer's test and nasal mucosal biopsy with severe lymphocyte infiltration. He was also found to have swelling of the whole pancreas and increased wall thickness in the common bile duct and the gall bladder. His serum was positive for an anticarbonic anhydrase II antibody. Since carbonic anhydrase ii is present in the ductal cells of various exocrine organs, this autoantibody is considered to be related to the pathogenesis of primary sjogren's syndrome with a marked swelling of multiple exocrine organs.
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7/58. Chronic pancreatitis and maldigestion.

    patients with chronic pancreatitis may suffer from maldigestion and malnutrition. Longstanding inflammation and fibrosis in the gland can destroy exocrine tissue, leading to inadequate delivery of digestive enzymes to the duodenum in the prandial and postprandial period and subsequent maldigestion. Maldigestion is augmented by inadequate bicarbonate delivery to the duodenum, with secondary inactivation of enzymes and bile acids by gastric acid. abdominal pain, sitophobia, nausea, vomiting, postprandial satiety, and on-going alcohol abuse may contribute to poor oral intake. Gastric dysmotility and mechanical gastric outlet obstruction from fibrosis in the pancreatic head may contribute to malnutrition and clinical decline. patients with chronic pancreatitis may at times experience profound steatorrhea and weight loss. In this article, we examine the natural history of exocrine insufficiency in chronic pancreatitis, outline the important nutritional issues in these patients, review the methods of diagnosis of maldigestion, and discuss the approach to therapy.
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8/58. Wegener's granulomatosis: parotid involvement and associated pancreatitis with C.T. findings.

    Parotid enlargement is an uncommon physical sign and even less commonly the reason for referral for computerised tomography (CT) as the aetiology is usually self-evident. In cases referred for CT examination Wegener's granulomatosis should be considered, particularly if the pathology appears bilateral. This diagnosis may be confirmed by biopsy and/or supported by Anti-Neutrophil Cytoplasmic Antibody (ANCA) assay. A case of Wegener's granulomatosis with parotid gland involvement and temporally related pancreatitis with pseudocyst formation is reported. This is the second reported case of possible pancreatic involvement in Wegeners granulomatosis and approximately the tenth reported case of salivary gland involvement.
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9/58. Pancreatic pseudotumor with sclerosing pancreato-cholangitis: is this a systemic disease?

    OBJECTIVE AND METHOD: Primary sclerosing cholangitis (PSC) is a disease that predominantly affects the biliary tree, although the pancreas may also be affected. A review of the presenting features of all patients given a diagnosis of PSC at a single center was conducted. The aim was to clarify the presentation of patients with pseudotumor of the pancreas in this patient population. RESULTS: Seventy-two patients were diagnosed with PSC either by ERCP (63/72 = 88%) or by liver biopsy (9/72 = 12%). The diagnosis of PSC was made following referral for abnormal liver tests (67%), jaundice (17%), and acute cholangitis (5%). Inflammatory bowel disease (IBD) (60%), non-insulin-dependent diabetes mellitus (NIDDM) (13%), thyroid disease (8%), and pancreatic disease (7%) were the major coexistent extrahepatic diseases. Three patients, all with marked weight loss, who presented with jaundice, abdominal pain, and/or diarrhea were found to have a pancreatic mass at first presentation. Clinical and radiological findings suggested pancreatic malignancy, and only later was advanced sclerosing cholangitis identified. The biopsy of the pancreas in two of these three patients revealed chronic pancreatitis. The long-term follow-up and good clinical response to medical therapy confirmed lack of pancreatic malignancy. These three patients all had other evidence of systemic involvement: submandibular gland fibrosis and urethral stricture in one, fibromuscular dysplasia of the renal artery in another, and retroperitoneal fibrosis in the third. None had IBD. CONCLUSION: Pancreatic pseudotumor with sclerosing pancreato-cholangitis may be a manifestation of a systemic disease characterized by nonmalignant strictures and multifocal fibroinflammatory processes, unlike classical PSC.
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10/58. Overlap syndrome of autoimmune pancreatitis and cholangitis associated with secondary sjogren's syndrome.

    In approximately 25% of patients with acute or chronic pancreatitis the cause remains unclear. Despite progress in understanding so-called idiopathic pancreatitis, more diagnostic criteria are needed. We report on a patient who presented with jaundice, but without pain or fever. Under the assumption of pancreatic cancer the patient underwent hemipancreatoduodenectomy. Histological examination showed chronic sclerosing inflammation of the pancreas and bile ducts without any signs of malignancy. Ten weeks later he developed bilateral parotid swelling and recurrent bouts of fever. Again liver enzymes were elevated and unsuccessfully treated with antibiotics for bacterial cholangitis. Further biopsies from submandibular gland, lymph nodes and liver again showed chronic sclerosing inflammation with lymphoplasmacytic infiltration. For sicca symptoms the diagnosis of a primary sjogren's syndrome was proposed. However, with corticosteroid treatment the patient improved remarkably but after tapering he relapsed. On the basis of established criteria, we diagnosed autoimmune pancreatitis with (1) diffuse swelling of the pancreas, (2) irregularities of the pancreatic duct, (3) lymphoplasmacytic infiltration, (4) response to corticosteroids, (5) hypergammaglobulinaemia, and (6) disproportionately raised IgG4. In addition, the patient fulfilled the criteria for secondary sjogren's syndrome. Autoimmune pancreatitis may present as an isolated or syndromic disease. It is an autoimmune disorder of unknown cause and should be included in the differential diagnosis of pancreatic disorders.
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