Cases reported "Cholangitis, Sclerosing"

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1/24. Symptomatic chronic calcific pancreatitis in a patient with idiopathic ulcerative colitis and sclerosing cholangitis.

    Although sclerosing cholangitis is well recognized to occur in patients with idiopathic inflammatory bowel disease, pancreatitis as a complication of ulcerative colitis is uncommon. We describe a patient who had idiopathic ulcerative colitis, primary sclerosing cholangitis and calcific pancreatitis with endocrine pancreatic deficiency, a rare combination.
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2/24. 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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3/24. Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis.

    BACKGROUND/AIMS: We encountered one group of primary sclerosing cholangitis cases which met classic criteria, but demonstrated better clinical courses. METHODOLOGY: We evaluated the clinical characteristics of several cases of primary sclerosing cholangitis, especially those associated with chronic pancreatitis. RESULTS: Of a total of 25 cases, eight had a background of pancreatitis with unusual irregularly segmental or diffuse narrowing features on pancreatography. Abdominal ultrasonography and computed tomography also revealed segmental or diffuse enlargement of the pancreas. Pathological findings of surgical specimens in two cases pointed to lymphoplasmacytic sclerosing pancreatitis. The walls of the lower biliary ducts were also thickened with a similar appearance to the pancreatic ducts. In seven cases, cholangiography displayed stenosis of the lower part of the common bile duct and in one case, only the hepatic hilar region was affected. In this case both biliary and pancreatic lesions responded to steroid therapy simultaneously. One case developed hepatic hilar stenosis four months after a lesion in the lower part of the common bile duct had disappeared. The cases of primary sclerosing cholangitis with pancreatitis demonstrated better clinical courses than did typical cases of primary sclerosing cholangitis. Six cases were characterized by autoimmunological abnormalities. CONCLUSIONS: We concluded that similar etiological agents might impact on both the pancreas and biliary tract, either simultaneously or in sequence. We propose new criteria for these atypical primary sclerosing cholangitis cases.
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4/24. Autoimmune pancreatitis associated with primary sclerosing cholangitis: MR imaging findings.

    Autoimmune pancreatitis is a relatively rare type of chronic pancreatitis that may be associated with other autoimmune disorders. The imaging features of this entity may be misleading and suggest the presence of a malignant tumour. We present a case in which MR imaging allowed us to diagnose autoimmune pancreatitis associated with primary sclerosing cholangitis, which is another autoimmune-related disease. Typical MR characteristics of autoimmune pancreatitis include focal or diffuse enlargement of the pancreas, the absence of parenchymal atrophy and significant dilation proximal to the site of stenosis, the absence of peripancreatic spread, the clear demarcation of the lesion and the presence of a peripancreatic rim.
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5/24. Secondary sclerosing cholangitis and portal hypertension after O157 enterocolitis: Extremely rare complications of hemolytic uremic syndrome.

    The authors experienced an extremely rare case of secondary sclerosing cholangitis and portal hypertension developed as late complications of hemolytic uremic syndrome (HUS) owing to escherichia coli o157:H7 in a 2-year-old boy. HUS after E coli O157 infection is the most frequent cause of acute renal failure in childhood and occasionally is accompanied by extrarenal complications such as encephalopathy, cardiomyopathy, ischemic colitis, and pancreatitis. Rarely, late colonic stenosis may develop secondary to the ischemic damage. Sclerosing cholangitis and subsequent cirrhosis with portal hypertension are very uncommon as late complications of HUS. To our knowledge, such a case has not been previously reported in the literature. J Pediatr Surg 36:1838-1840.
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6/24. Bacterial cholangitis causing secondary sclerosing cholangitis: a case report.

    BACKGROUND: Although bacterial cholangitis is frequently mentioned as a cause of secondary sclerosing cholangitis, it appears to be extremely rare, with only one documented case ever reported. CASE PRESENTATION: A 48-year-old woman presented with an episode of acute biliary pancreatitis that was complicated by pancreatic abcess formation. After 3 months she had an episode of severe pyogenic (E. Coli) cholangitis that recurred over the subsequent 7 months on a further two occasions. Initially, cholangiography suggested the presence of extra-biliary intrahepatic abcesses while repeated investigations demonstrated development of multiple segmental biliary duct strictures. After maintenance antibiotic treatment was started, no episodes of cholangitis occurred over a 14-month period. CONCLUSIONS: Sclerosing cholangitis can rapidly develop after an episode of bacterial cholangitis. Extra-biliary involvement of the hepatic parenchyma with abcess formation may be a risk factor for developing this rare but particularly severe complication.
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7/24. Bile duct involvement in a case of autoimmune pancreatitis successfully treated with an oral steroid.

    In the case reported here, the characteristic features of AIP were evaluated by ultrasonography, computed tomography and endoscopic retrograde cholangiopancreatography, initially in the intrahepatic- and extrahepatic bile ducts, and later in the pancreas. In addition, histological examination revealed lymphocytic sclerosis around the intralobular bile ducts, as is reported in AIP, without chronic nonsuppurative destructive cholangitis or onion-skin-like appearance. immunohistochemistry identified the infiltrating lymphocytes as T cells. Although hypergammaglobulinemia was observed with elevation of hepatobiliary and pancreatic enzymes, no other serological or physiological abnormalities suggestive of other systemic autoimmune diseases were detected. These findings progressed over a three-month period and were dramatically resolved within one month by steroid therapy. These observations support a novel clinical entity characterized by the presence of bile duct lesions similar to the pancreatic involvement seen in AIP that is distinct pathophysiologically, histologically, and therapeutically from the so-called autoimmune cholangitis or primary sclerosing cholangitis.
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8/24. Primary sclerosing cholangitis associated with autoimmune pancreatitis.

    We present 2 cases of primary sclerosing cholangitis complicated with autoimmune pancreatitis with narrowing of the pancreatic duct. In both cases, endoscopic retrograde cholangiography showed the characteristic findings of primary sclerosing cholangitis. In addition, positivity for autoantibody, hypergamma-globulinemia and narrowing of the pancreatic duct shown on endoscopic retrograde pancreatography were noticed in both. They were thu diagnosed as autoimmune pancreatitis. Whereas it is well known that primary sclerosing cholangitis is often complicated with chronic pancreatitis, it has rarely been studied whether the chronic pancreatitis is autoimmune pancreatitis or not. In English literature, possibly 7 equivalent cases have been reported so far. In those cases, the onset was in the post-prime of life, and the disease was frequently accompanied with sjogren's syndrome, but not with ulcerative colitis; frankly the nature of the disease was slightly different from what has traditionally been thought as a typical primary sclerosing cholangitis. In primary sclerosing cholangitis complicated with autoimmune pancreatitis, the autoimmune mechanism seems to be much involved, and thus steroid therapy might be effective as in Case 2 in our series.
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9/24. Lymphoplasmacytic sclerosing pancreatocholangitis successfully treated by pancreatoduodenectomy.

    A 68-year-old man complaining of jaundice was admitted to our hospital in October 1996. Radiological imaging studies, including dynamic computed tomography, endoscopic retrograde cholangiography, and angiography, were highly suggestive of pancreatic head cancer, and laparotomy was performed on October 25, 1996. On gross examination, the pancreas appeared firm, as in chronic pancreatitis, with a mass lesion in the pancreatic head measuring 35 x 35 x 25 mm. A pylorus-preserving pancreatoduodenectomy was carried out. Histological findings were characterized by uniform fibrosis with diffuse lymphoplasmacytic infiltration and lymph follicles in the thickened wall of the bile duct and in and around the pancreas, with acinar atrophy. The histological diagnosis was lymphoplasmacytic sclerosing pancreatocholangitis. Approximately 5 years postoperatively, the patient was alive and well without adjunctive corticosteroid therapy. This positive result suggests that pancreatoduodenectomy may be effective for lymphoplasmacytic sclerosing pancreatocholangitis that is localized in the pancreatic head.
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10/24. 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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